UWorld Fun Facts Flashcards

(481 cards)

1
Q
Chronic bronchitis
--
Chronic bronchitis vs emphysema
---
Chronic bronchitis vs bronchiectasis
A

Chronic bronchitis (intact alveoli and capillary walls) vs emphysema (destruction of alveolar walls): they’re both obstructive lung diseases (FEV1/FVC <70% predicted)
- DLCO (diffusion capacity of the lung for CO; measures gas exchange between pulm capillaries and alveoli) - normal in bronchitis, decreased in emphysema
- CXR - prominent bronchovascular markings and flattening of diaphragm in bronchitis; decreased bronchovascular markings and hyperinflated lungs in emphysema
- more pronounced hypoxemia in bronchitis than emphysema

Chronic bronchitis: chronic productive cough for 3+ months in 2 successive years; can produce hemoptysis

Bronchiectasis: irreversible dilation and destruction of bronchi, resulting in chronic cough and inadequate mucus clearance. Hx of recurrent respiratory tract infections and chronic cough with daily production of copious mucopurulent sputum
Cycle: infectious insult/impaired bacterial clearance –> bacterial overgrowth –> neutrophil infiltration –> inflammation –> tissue damage/structural airway changes –> bacterial overgrowth

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2
Q

Aspergillosis

A

Invasive pulmonary aspergillosis - RF=immunocompromised; triad of fever, pleuritic chest pain, and hemoptysis. CT chest shows nodules with surrounding ground-glass opacities (halo sign). Tx: 1-2 weeks IV voriconazole + echinocandin –> prolonged therapy with oral voriconazole

Chronic pulmonary aspergillosis: RF = lung disease/damage; 3+ months of weight loss, cough, hemoptysis, fatigue, cavitary lesion +/- fungus ball, positive IgG serology. Tx: resect aspergilloma, voriconzole

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3
Q

Chorioamnionitis

A

Dx clinically by presence of maternal fever and 1+ of the following: uterine tenderness, maternal or fetal tachycardia, malodorous amniotic fluid, or purulent vaginal discharge
PROM = RF
NOT an indication for CS

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4
Q
Bone tumors:
giant cell tumor of bone
osteoid osteoma
osteosarcoma
Ewing sarcoma
A

Giant cell tumor of bone: benign, locally aggressive; presents with pain, swelling, dec ROM at involved site; osteolytic lesion with soap-bubble appearance (interspersed large osteoclast giant cells) on radiographs in the epiphyseal regions of the long bones; most commonly involves the distal femur and prox tibia around the knee joint

Osteoid osteoma: sclerotic cortical lesion with central nidus of lucency. Typically causes pain worse at night and unrelated to activity. Pain controlled with NSAIDs

Osteosarcoma: primary bone cancer in the metaphyses of long bones; Codman’s triangle in the metaphyses of long bones, “sunburst” pattern

Ewing sarcoma: primary bone cancer; onion skinning; diaphyses of long bones

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5
Q
Genetic immuno diseases:
Wiskott-Aldrich syndrome
Hyper-IgM syndrome
Ataxia telangiectasia
Chronic granulomatous disease CGD
SCID (ADA deficiency)
Leukocyte adhesion deficiency 
X-linked agammaglobulinemia
Complement deficiency
Selective IgA deficiency
Primary ciliary dyskinesia
Chediak-Higashi syndrome
Common variable immunodeficiency
A

Wiskott-Aldrich syndrome: x-linked recessive defect in WAS protein gene –> impaire cytoskeleton regulation in leukocytes/platelets
Presentation: eczema, microthrombocytopenia, recurrent infections
Tx: stem cell transplant

Hyper-IgM syndrome: lack of CD40 ligand –> T cells cannot bind to the CD40 receptor on B cells –> B cells cannot switch the antibody isotype they produce

Ataxia telangiectasia: T cell deficiency associated with a defect in DNA repair –> immune dysfunction, progressive cerebella degeneration, high risk for cancer

Chronic granulomatous disease: x-linked recessive gene defect in NADPH oxidase enzyme complex –> inability of phagocytes to produce hydrogen peroxide in their lysosomes –> recurrent pulmonary and cutanoues infections; abscesses due to fungi or catalase-positive bacteria (Staph aureus, Burkholderia, aspergillus)
Dx: dihydrorhodamine 123 test, nitroblue tetrazolium test; these tests test neutrophil function via detection or absence of an oxidative burst
Ppx: antimicrobial ppx with TMP-SMX and itraconazole
Tx: immunomodulator therapy

SCID: severe T cell deficiency due to lack of maturation of T cells in the thymus –> severe B cell deficiency/dysfunction too
- ADA deficiency = AR form of SCID –» deficient formation of mature B and T lymphocytes
Tx: stem cell transplant

Leukocyte adhesion deficiency: recurrent skin and mucosal bacterial infections - lack of neutrophil migration –> no pus, poor wound healing although there is leukocytosis with neutrophilia; associated with delayed umbilical cord separation (>21 days)

X-linked agammaglobulinemia aka Bruton’s agammaglobulinemia: recurrent sinopulmonary and GI infections with absent B cell and Ig concentrations; exam shows absence of lymphoid tissue; normal T cell count

Complement deficiency: increased risk for disseminated bacterial infections particularly with encapsulated bacteria like Strep pneumo and Neisseria meningitidis

Selective IgA deficiency: recurrent sinopulmonary infections, atopy, anaphylactic reactions during blood transfusion
associated with celiac dz (people with both will be negative for IgA anti-tissue transglutaminase and IgA anti-endomysial antibodies)

Primary ciliary dyskinesia: AR; absent or dysmotile cilia and poor mucociliary clearance
recurrent sinopulmonary infections, bronchiectasis
+/- situs inversus = Kartagener syndrome

Chediak-Higashi syndrome: AR; partial oculocutaneous albinism and recurrent cutaneous infections with Staph aureus and Strep pyogenes

Common variable immunodeficiency CVID - most common primary immunodeficiency in adults; abnormal b-cell differentiation results in deficiency of multiple immunoglobulin classes. Manifestations: recurrent resp and GI infections, autoimmune disease, chronic lung disease, GI disorders.
Tx: Ig replacement therapy

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6
Q

Hereditary Hemochromatosis

A

Clinical manifestations:

  • hyperpigmented skin (bronze DM)
  • arthralgia, arthropathy, chondrocalcinosis (suspect in pseudogout; hemochromatosis induced iron deposition in the synovial fluid promotes CPPD)
  • elevated LFT’s, cirrhosis, inc risk for HCC
  • restrictive or dilated cardiomyopathy and conduction abnormalities

Evaluation: serum iron studies
Confirm with genetic tests

Long term management: serial phlebotomy to deplete excess iron stores

Vibrio, yersinia, and listeria - love iron - ppl with hemachromatosis are susceptible to these infections

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7
Q

Pseudogout

A

Acute arthritis induced by the release of CPPD (calcium pyrophosphate dihydrate) crystals from sites of chondrocalcinosis (calcification of articular cartilage) into the joint space. Often occurs in the setting of recent surgery or other medical illness.

Patients dx’d with pseudogout should be evaluated for 2’ causes such as hyperparathyroidism, hypothyroidism, and hemochromatosis

Synovial fluid analysis: inflammatory effusion (15-30k wbc), rhomboid shaped, positively birefringent crystals

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8
Q

B12 deficiency / Pernicious anemia

A

Sx of B12 deficiency: macrocytic anemia, glossitis, neurologic changes including peripheral neuropathy

Pernicious anemia = autoimmune disorder where the body makes anti-intrinsic factor antibodies - leading cause of B12 deficiency esp in whites of northern European backround; associated with other autoimmune disease
Patients with pernicious anemia also develop a chronic atrophic gastritis which increases the risk of intestinal type gastric cancer and gastric carcinoid tumors by 2-3x
–> monitor for development of gastric cancer (periodic stool testing for presence of blood)

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9
Q

Esophageal varices

A

Patients with varices should receive primary prophylaxis to prevent bleeding - can be achieved either with endoscopic variceal ligation or nonselective beta blocker like propranolol or nadolol

TIPSS = used as salvage therapy in patients with refractory ascites or esophageal varices who have failed endoscopic or medical management

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10
Q
Hashimoto thyroiditis
Subacute thyroiditis 
TSH-secreting pituitary adenoma
RAIU high vs low
Hyperthyroidism
Graves
Euthyroid sick syndrome
Congenital hypothyroidism 
Follicular thyroid cancer
Papillary thyroid cancer
Medullary thyroid cancer
Thyroid lymphoma 
Painless thyroiditis
Hypothyroidism 
Struma ovarii
A

Hashimoto:
Autoimmune thyroid disease - antithyroid peroxidase antibodies and antithyroglobulin antibodies
Present with palpable goiter and clinical hypothyroidism
Occasionally will present with transient hyperthyroid phase but RAIU is usually increased
Can see Hurthle cells although not specific
High titers of anti-TPO are associated with inc risk of miscarriage

Subacute thyroiditis (de Quervain's thyroiditis): fever, neck pain, thyroid tenderness, elevated ESR/CRP; hyperthyroid sx fade in <8 weeks as thyroid gland becomes depleted of preformed hormone; RAIU is decreased, elevated ESR and CRP; likely postviral inflammatory process
Tx: symptomatic - beta blockers, NSAIDs

Suppurative infection of the thyroid gland (infectious thyroiditis) - high grade fever, tender thyroid gland; may have abscess formations

TSH-secreting pituitary adenoma: elevated TSH, free T4, normal or increased RAIU

High RAIU = suggestive of de novo hormone synthesis - can be seen in Graves disease (diffuse uptake) or toic nodular disease (nodular uptake)

Low RAIU = suggestive of either release of preformed thyroid hormone or exogenous thyroid hormone intake. Thyroglobulin level helps make the distinction - elevated = endogenous thyroid hormone release; decreased = exogenous or factitious thyrotoxicosis

Hyperthyroidism:
excess thyroid hormone increases osteoclast activity –> rapid bone loss and osteoporosis
can see muscle weakness and atrophy
In pts with significant sx and thyroid hormone levels >2-3x normal, an anti thyroid drug with beta blocker is initially recommended to stabilize the pt before definitive tx with RAI or thyroidectomy

Graves:
diffuse goiter, ophthalmopathy, nail clubbing (bulbous fingertip enlargement), onycholysis

Euthyroid sick syndrome:
in the setting of acute, severe illness
Low T3, normal T4 and TSH
Due to lack of peripheral conversion due to low calorie, glucocorticoid use, etc

Congenital hypothyroidism:
Most common cause = thyroid dysgenesis

Follicular thyroid cancer - 2nd most common thyroid epithelial malignancy after papillary cancer
peak incidence 40-60. Presents as firm thyroid nodule (cold nodule). Characterized by invasion of the tumor capsule and/or blood vessels –> can spread hematogenously to distant tissues; lymph node involvement not common; can see Hurthle cells (large polygonal cells with eosinophilic cytoplasm containing large quantities of mitochondria) but not specific finding

Papillary thyroid cancer - slow spread into local tissues and regional lymph nodes; associated with psammoma bodies

Medullary thyroid cancer: calcitonin-producing tumor of the thyroid parafollicular C cells; often occurs as a component of MEN2A and MEN2B, which are also associated with pheochromocytoma. Patients with MTC should be screened for pheochromocytoma prior to thyroidectomy with a plasma fractionated metanephrine assay

Thyroid lymphoma - uncommon but incidence is 60x greater in patients with preexisting Hashimoto thyroiditis
Typical presentation = rapidly enlarging, firm goiter associated with compressive sx (dysphagia, hoarseness), may have B sx as well. Gland is usually fixed to the surrounding structures and does not move up when swallowing. Retrosternal extension of the tumor is common and can result in venous compression with distended neck veins and facial plethora.
+ pemberton sign - raising the arms causes compression of the subclavian and R IJ vein between the clavicles and enlarged thyroid leading to more prominent venous distension and facial redness

Painless thyroiditis = variant of Hashimoto thyroiditis. + TPO antibody. Hyperthyroidism + low RAIU and nontender goiter. Characterized by self-limited hyperthyroid phase due to release of preformed thyroid hormone, followed by hypothyroid phase or return to euthyroid state. Does not require specific therapy but beta blocker can be prescribed to control sx

Hypothyroidism:
pts have an inc requirement for levothyroxine after starting oral estrogen (replacement therapy or OCP’s) or in pregnancy - E decreases clearance of TBG, leading to elevated TBG levels –> higher dose of synthroid is needed to bind them
Hypothyroidism causes additional metabolic abnormalities like hyperlipidemia, hyponatremia, and asymptomatic elevations of CK and transaminases.
severe cases can cause paranoia and hallucinations “myxedema madness”

Struma ovarii: very rare cause of thyrotoxicosis; due to production of thyroid hormone by an ovarian teratoma. Suspect in women over age 40 with a pelvic mass, ascites, or abd pain. Thyroid gland is not enlarged

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11
Q

Acute pancreatitis

Chronic pancreatitis

A

Dx: 2 of the following - acute onset severe epigastric pain radiating to back, inc amylase or lipase >3x upper limit of normal, characteristic abdominal imaging findings (focal or diffuse pancreatic enlargement)

Severe pancreatitis causes local release of activated pancreatic enzymes that enter the vascular system and increase vascular permeability within and around the pancreas –> large amts of volume migrate from vascular system to the surrounding retroperitoneum
Systemic inflammation due to inflammatory mediators enter the vascular system
NET EFFECT = widespread vasodilation, capillary leak, shock, and associated end organ damage
Tx: several liters of IVF

Chronic pancreatitis: recurrent bouts of upper abd pain, diarrhea/steatorrhea, and weight loss
Dx: presence of pancreatic calcifications on CT or plain film

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12
Q
Germ/sex cell tumors:
Leydig cell tumor
Choriocarcinoma
Teratoma
Seminoma
Yolk sac tumor (endodermal sinus tumor)
Theca lutein cyst
Granulosa cell tumor
Dysgerminoma
Serous cystadenomas
A

Leydig cell tumor:
most common type of testicular sex cord stromal tumor, may occur in all age groups; Leydig cells = principal source of testosterone and are capable of estrogen production. Sx: precocious puberty, gynecomastia

Choriocarcinoma:
gestational trophoblastic neoplasia - malignancy that arises from placental trophoblastic tissue and secretes b-hCG. Can occur after a hydatidiform mole, normal gestation, or spontaneous abortion. Typically presents <6 months after a pregnancy.
Sx: irregular vaginal bleeding, enlarged uterus, pelvic pain
Most common metastatic spread is to the lungs (chest pain, hemoptysis, dyspnea)
Dx: elevated b-hCG
Tx: chemo

Mature cystic Teratoma (dermoid ovarian cyst):
AFP and b-HCG can be elevated, US findings include hyperechoic nodules and calcifications in dermoid cysts

Seminoma:
serum tumor markers usually normal, may see elevated b-HCG

Nonseminoma:
elevated AFP, with a considerable number also having an elevated b-hCG

Yolk sac tumor/endodermal sinus tumor:
germ cell tumor, elevated AFP

Theca lutein cyst: present during pregnancy; due to ovarian stimulation by high b-hCG levels and resolve after these levels decline; causes hyperandrogenism in pregnancy; US: multiseptated b/l cystic masses and do not have calcifications or hyperechoic nodules

Luteoma: yellow or yellow-brown masses of large lutein cells that cause hyperandrogenism in pregnancy; see solid ovarian masses on US; regress spontaneously after delivery; high risk of fetal fetal virilization

Krukenberg tumor: b/l solid ovarian masses on US; mets from primary GI tract cancer; causes hyperandrogenism in pregnancy; high risk of fetal virilizationb

Granulosa cell tumor: malignant ovarian neoplasms that secrete estrogen; causes precocious puberty in children and endometrial hyperplasia/bleeding in postmenopausal women

Dysgerminoma: ovarian tumor most commonly occurs in women age <30, secretes LDH or b-hCG

Serous cystadenoma: most common benign ovarian neoplasms; hormonally inactive; does not cause precocious puberty

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13
Q

Effusions / Lights criteria

A

Transudates: due to imbalance between hydrostatic and oncotic pressures that increases fluid movement across the capillaries into the visceral pleura and pleural space

  • -> treat the underlying dz
  • lab values: low protein (<3 g/dL), normal glucose and pH of 7.4-7.55

Exudates: due to pleural and lung inflammation resulting in increased capillary and pleural membrane permeability
Lights criteria define an exudate as having at least 1 of the following:
- pleural fluid protein/serum protein ratio > 0.5
- pleural fluid LDH/serum LDH ratio > 0.6
- pleural fluid LDH > 2/3 of the upper limit of normal for serum LDH

Bacteria pneumonias cause pleural effusions (exudates). Usually they are small, sterile, free-flowing, and resolve with antibiotics if they are uncomplicated. But if they are complicated (bacteria persistently invade the pleural space) or an empyema develops, patients have continued sx (fever, pleuritic pain) despite antibiotics and CXR shows loculation (walled-off pleural fluid). Thoracentesis of empyema shows exudate characterized by low glucose (<60), low pH (<7.2), high protein, and neutrophilic leukocytosis
Empyema vs complicated parapneumonic effusion: presence of gross pus or bacteria on gram stain in empyema

Chylothorax: exudative; leakage of chyle into the thoracic space, often from obstruction of the thoracic duct. Can cause a lymphocytic predominant effusion with turbid or milky white fluid; pleural fluid analysis demonstrates milky-white fluid with lymphocyte predominance and increased triglycerides. Chyle is composed of T cell lymphocytes, Ig, and lipid-transporting chylomicrons that contain triglycerides

TB effusion: exudative; very high protein levels >4, lymphocytic leukocytosis, low glucose, markedly elevated LDH >500; +ADA

Malignant effusion: exudative; low pH (<7.2), low glucose (<60), very high LDH
most common causes are breast cancer and lung cancer


Normal pleural pH = 7.6

Hepatic hydrothorax: transudative pleural effusions due to small defects in the diaphragm which permit peritoneal fluid to pass into the pleural space
Tx: salt restriction and diuretic administration
Definitive tx: liver transplant

high amylase in pleural fluid = either from esophageal perforation or from pancreatitis


hemothorax = potential complication of a thoracentesis; suspect in any pt who develops rapid reaccumulation of pleural effusion, difficulty breathing, and hemodynamic instability shortly after the thoracentesis is performed

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14
Q

Aortic injury

A

Widened mediastinum, large left-sided hemothorax, deviation of the mediastinum to the R and disruption of normal aortic contour
Dx: CT scan
Management: antihypertensive therapy and immediate operative repair

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15
Q
Hereditary cancer syndromes:
Lynch
FAP
vHL
MEN 1
MEN 2
BRCA 1/2
A

Lynch: CRC, endometrial, ovarian

FAP: CRC, desmoids/osteomas, brain

vHL: hemangioblastomas, clear cell renal carcinoma, pheochromocytoma

MEN 1: parathyroid, pituitary, pancreatic

MEN 2a: pheochromocytoma, parathyroid hyperplasia, medullary thyroid cancer

MEN 2b: pheochromocytoma, medullary thyroid cancer, marfanoid habitus/mucosal neuroma

BRCA1/2: breast, ovarian

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16
Q
Vasculitidies:
Wegener
Churg-Strauss
HSP
Takayasu
Buerger
Polyarteritis nodosa
A

Granulomatosis with polyangiitis (wegener granulomatosis):
- necrotizing/granulomatous vasculitis of small and medium sized vessels
+ c-ANCA
- Upper resp tract = most common site of disease (chronic rhinosinusitis)
- Lower resp tract may lead to tracheal narrowing with ulceration
- CXR: multiple lung nodules with cavitation
- Renal involvement: glomerulonephritis
- Cutaneous manifestations including purpura with ulceration, urticaria, livedo reticularis, pyoderma gangrenosum

Henoch-Schonlein purpura:

  • IgA mediated leukocytoclastic vasculitis
  • Palpable purpura, arthritis/arthralgia, abd pain, renal dz, SCROTAL PAIN
  • Lab findings: hematuria +/- RBC casts +/- proteinuria; normal platelet count
  • Tx: supportive

Churg-Strauss syndrome / eosinophilic granulomatosis with polyangiitis:

  • chronic rhinosinusitis, nasal polyps, asthma
  • prominent eosinophilia

Takayasu arteritis: large artery granulomatous vasculitis common in young Asian women. Primarily involves the aorta and its branches; characterized by mononuclear infiltrates and granulomatous inflammation of the vascular media, leading to arterial wall thickening with aneurysmal dilation or narrowing and occlusion. Initial symptoms include fever, arthralgias, and weight loss –> later features present with arterio-occlusive manifestations (claudication, distal ulcers) particularly in the upper extremities –> BP discrepancies, pulse deficits, bruits. CT and MRI reveal aneurysm formation or lumenal narrowing. Tx include systemic glucocorticoids

Buerger disease (thromboangiitis obliterans) - men who are heavy smokers –> superficial thrombophlebitis and ischemia and gangrene of the digits

Polyarteritis nodosa - skin lesions and myalgias; necrotizing vasculitis

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17
Q

Hereditary spherocytosis

A
  • AD
  • hemolytic anemia, jaundice, splenomegaly
  • lab findings: elevated MCHC and RDW, spherocytes (small hyperchromic red cells without central pallor) on peripheral smear, neg Coombs test, inc osmotic fragility on acidified glycerol lysis test, abnormal eosin-5-maleimide binding test
  • tx: folate supplementation, blood transfusions, splenectomy
  • complications: pigment gallstones –> cholecystitis, aplastic crises from parvovirus B19
  • splenectomy: most feared long-term complication: overwhelming sepsis with encapsulated bacteria, namely Strep pneumo - risk is present up to 30 years and longer –> anti-pneumococcal, Haemophilus, and meningococcal vaccines several weeks before operation, and daily oral penicillin ppx for 3-5 years following splenectomy or until adulthood
  • encapsulated pathogens are largely eliminated via the humoral immune response with antibody-mediated phagocytosis (opsonization) and antibody-mediated complement activation - much of this is dependent on splenic macrophages and the generation of splenic opsonizing antibodies
  • -> ppl with asplenia are at high risk for fulminant infection with encapsulated organisms
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18
Q

Paroxysmal nocturnal hemoglobinuria

A
  • dx: genetic defect in RBC CD55 and CD59 surface proteins which normally inhibit activation of complement on RBC –> absence allows complement membrane attack complex to form and cause hemolysis
  • hemolytic anemia (elevated bilirubin and LDH), cytopenias, hypercoagulability
  • dx: flow cytometry to assess absence of CD55 and CD59 on RBC surface
  • tx: iron and folate supplementation. Eculizumab (monoclonal antibody that inibits complement activation)
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19
Q

Celiac disease vs pancreatic insufficiency

A

D-xylose = monosaccharide that can be absorbe din the proximal small intestine without degradation by pancreatic or brush border enzymes

Celiac disease: ingestion of D-xylose –> urinary and venous D-xylose levels will be low since it is not absorbed and secreted into the urine

Malabsorption from enzyme deficiencies: normal absorption of D-xylose

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20
Q

Precocious puberty

Premature adrenarche

A

If low LH –> Gonadotropin independent (peripheral) or Gonadotropin dependent (central)?
Measure LH before and after GnRH stimulation test
If LH still low –> peripheral precocious puberty
If LH high –> central precocious puberty –> MRI brain

Tx for central precocious puberty = GnRH agonist therapy to prevent premature epiphyseal plate fusion and maximize adult height potential

Premature adrenarche: early activation of adrenal androgen release from the adrenal glands –> pubarche, axillary hair, acne, and body odor in girls <8 and boys <9
Generally a benign condition but a significant RF for PCOS, type 2 DM, and metabolic syndrome esp in those who are obese

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21
Q

Classic vs nonclassic CAH

A

Classic: presents in neonatal period with adrenal insufficiency and/or ambiguous genitalia

Non-classic/late-onset: manifests in late childhood with signs of androgen excess; normal electrolytes

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22
Q

Serous otitis media

A
  • most common middle ear pathology in patients with AIDs
  • due to auditory tube dysfunction arising from HIV lymphadenopathy or obstructing lymphomas
  • presence of middle ear effusion without evidence of an acute infection
  • sx: conductive hearing loss, tinnitus, sensation of pressure
  • exam: dull tympanic membrane that is hypomobile on pneumatic otoscopy
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23
Q

PCOS vs testosterone secreting tumor

A

Elevated estrogen
Normal FSH
Elevated LH
Elevated GnRH

Inc in testosterone –> inc in peripheral conversion to estrogen
–> Persistently elevated (not pulsatile) GnRH –> preference for LH instead of FSH (to make testosterone)

Tx: weight loss = first line; decreases peripheral estrogen conversion
Clomiphene citrate - SERM that blocks E receptors at the hypothalamus and inhibits negative feedback mechanism –> pulsatile GnRH secretion –> normalize LH and FSH levels, creating an LH surge resulting in ovulation induction
OCP’s - menstrual regulation

Hirsutism in PCOS is typically chronic and slowly progressive
Testosterone secreting tumors have rapid onset hirsutism –> must check both testosterone levels and DHEAS levels
Elevated testosterone with normal DHEAS –> ovarian source of neoplasm (more common)
Elevated DHEAS –> adrenal source of neoplasm

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24
Q

Acute mediastinitis

A

Complication of cardiac surgery - usually due to intraoperative wound contamination
fever, cp, leukocytosis
CXR: widened mediastinum

Tx: drainage, surgical debridement with immediate closure, prolonged antibiotic therapy

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25
Atopic dermatitis vs Seborrheic dermatitis
Atopic: recurrent rash; severely pruritic; family or personal history of atopic disorders like asthma and allergic rhinitis Seborrheic: erythematous plaques with yellow greasy scales; spontaneous remission is common; 1st line tx options include emollients and nonmedicated shampoos
26
Nephrolithiasis
Most common renal stones are calcium stones Inc Na intake enhances Ca excretion --> hypercalciuria Dec Na intake promotes Na and Ca reabsorption --> patients with recurrent renal calculi should be advised to restrict Na intake Dietary recc for patients with renal calculi: - inc fluid intake - dec sodium intake - normal dietary calcium intake - thiazide/amiloride diuretics - volume depletion caused by thiazides leads to a compensatory rise in reabsorption of Na and water --> inc passive reabsorption of calcium Calcium oxalate stones: envelope shaped; small bowel disease, surgical resection, or chronic diarrhea can lead to malabsorption of fatty acids and bile salts which predisposes to formation of calcium oxalate stones (unabsorbed fatty acids chelate calcium, making oxalic acid free for absorption) --- Uric acid stones: most commonly seen in patients with low urine pH and hyperuricosuria; radiolucent but may be seen on US or CT scan Tx: hydration, alkalinization of urine with oral potassium citrate, low-purine diet -- Stones measuring less than 5mm in diameter typically pass spontaneously with conservative management, including a fluid intake of greater than 2L daily
27
Pulmonary HTN
Mean pulmonary arterial pressure of 25+ mmHg at rest (normal <20 mmHg) Can be idiopathic or 2' to L heart dz, chronic lung dz, or chronic thromboembolism
28
ARDS
Mechanical ventilation improves oxygenation by providing an inc FiO2 and PEEP to prevent alveolar collapse Goal is to maintain PaO2 at 55-80 mmHg, which roughly corresponds to O2 sat >88% Prolonged high FiO2 can cause O2 toxicity so FiO2 levels <60% are generally considered safe Dx: new resp distress within 1 week of clinical insult, b/l lung opacities on imaging, pulmonary edema not explained by heart failure, hypoxemia with PaO2/FiO2 ratio <300mmHg
29
Indications for renal and bladder US in children
- infants and children under 2 years with first febrile UTI - recurrent febrile UTI's in children of any age - UTI in a child of any age w FH of renal or urologic dz, HTN, poor growth - children who don't respond to appropriate antibiotic tx
30
Laryngomalacia
Increased laxity of supraglottic structures Presentation: inspiratory stridor that worsens when supine and when feeding/URI's, peaks at age 4-8 months Dx: clinical; confirm with flexible laryngoscopy for moderate/severe cases - omega-shaped epiglottis and collapse of supraglottic structures during inspiration Management: reassurance; supraglottoplasty for severe sx
31
Ankylosing spondylitis
Chronic progressive back pain and stiffness; pain relief with activity; enthesitis, dactylitis, anterior uveitis *most common extraarticular manifestation of AS; limited chest expansion --> restrictive PFT's Plain x-rays of pelvis showing sacroiliitis confirm diagnosis; can also see fusion of vertebral bodies with ossification of intervertebral discs (bamboo spine) HLA-B27 is NOT specific for ankylosing spondylitis (whereas >90% of patients with AS have HLA-B27, only 5% of patients with HLA-B27 have AS) Complications: vertebral fractures, osteoporosis, aortic regurgitation, cauda equina syndrome
32
Renal pathology: | Alport's syndrome
Alport's syndrome: familial disorder that presents in childhood with gross hematuria and proteinuria and sensorineural deafness. EM findings: alternating areas of thinned and thickened capillary loops with splitting of the glomerular basement membrane
33
Lateral epicondylitis
Tennis elbow Due to noninflammatory angiofibroblastic tendinosis at the common extensor origin; caused by repetitive, forceful extension at the wrist. Exam: tenderness at the lateral epicondyle and reproduction of pain with passive flexion or resisted extension at the wrist
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Leukemoid reaction vs CML
CML = myeloproliferative neoplasm caused by the BCR-ABL fusion gene --> dramatic leukocytosis (>100k), absolute basophilia, preponderance of early immature neurotrophil precursors; presents with fatigue, weight loss, night sweats, abd fullness, thrombocytosis, anemia. LAP score = LOW (neutrophils are cytochemically and functionally abnormal) Leukomoid reaction = leukocytic reaction to severe infection. Leukocyte count >50k, mature neutrophil precurosrs. LAP score = HIGH LAP = leukocyte alkaline phosphatase.
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``` Breast tumors: FIbroadenoma Inflammatory breast carcinoma Intraductal papilloma Infiltrating ductal carcinoma Mammary Paget disease (paget disease of the breast) Fibrocystic change ```
RF's: hormone replacement therapy, nulliparity, inc age at first live birth, alcohol consumption, genetic mutation, white race, increasing age, early menarche or later menopause Fibroadenoma: benign palpable breast mass most commonly found in young women, estrogen-sensitive, firm and mobile with regular borders and spherical shape Intraductal papilloma: benign condition; unilateral bloody nipple discharge; no other sx or skin changes SHould still workup with mammo/US to rule out carcinoma Inflammatory breast carcinoma: rare but aggressive cancer, rapid onset edematous cutaneous thickening with peau d'orange appearance, breast is edematous, erythematous, and painful Mammary Paget disease: suspect when a persistent, eczematous, and/or ulcerating rash is localized to the nipple and spreads to the areola. 85% of pts with Paget dz have an underlying breast cancer --> most common type is adenocarcinoma. --> mammo and biopsy Fibrocystic change: multiple diffuse nodulocystic masses, cyclic premenstrual tenderness
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Atlantoaxial instability in Down's patients | Complete AV septal defect in Down's patients
Atlantoaxial instability = malformation seen in 10-15% of pts with Down syndrome but only symptomatic in 1-2% Due to excessive laxity in the posterior transverse ligament which causes increased mobility between the atlas (C1) and the axis (C2) --> sx progress over several weeks and result from compression of spinal cord Sx: behavioral changes, torticollis, urinary incontinence, vertebrobasilar sx like dizziness vertigo and diplopia, UMN symptoms Dx: lateral radiographs of the cervical spine in flexion, extension, and neutral position Tx: surgical fusion of C1 and C2 ---- Complete atrioventricular septal defect: most common congenital heart defect in patients with Down syndrome - due to failure of the endocardial cushions to merge --> VSD, ASD, and common AV valve due to poor mitral and tricuspid valve development --> heart failure --> loud S2 due to pulmonary HTN, systolic ejection murmur from inc flow across the pulmonary valve from the left to right shunt across the ASD, holosystolic murmur of the VSD ToF is also associated with Down syndrome
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Primary polydipsia, Central DI, Nephrogenic DI
Primary polydipsia: low serum Na Central DI (dec ADH release from pituitary): high serum Na (>150) - due to impaired thirst mechanism Nephrogenic DI (ADH resistance in kidney): normal serum Na - intact thirst mechanism and adequate water intake
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Felty syndrome
- RA - Splenomegaly - Neutropenia
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Caplan syndrome
- RA - Pneumoconiosis - Lung nodules
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``` Adverse effects of RA Medications: Methotrexate Anti-TNF Hydroxychloroquine Sulfasalazine Rituximab Gold salts ```
Methotrexate - hepatotoxicity, pulmonary toxicity, bone marrow suppression, stomatitis Anti-TNF - reactivation of TB Hydroxychloroquine - retinal toxicity Sulfasalazine - rash, hemolysis in G6PD deficiency Rituximab - infection Gold salts - nephrotic syndrome
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Jarisch-Herxheimer reaction
Seen primarily in patients with early syphilis tx'd with antibiotic meds Rapid destruction of spirochetes --> acute febrile illness typically within 12 hrs of tx Sx: HA, myalgias, rigors, sweating, hypotension, worsened syphilitic rash Self limited, resolves spontaneously within 48 hrs
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Hypertensive retinopathy
no acute vision loss On fundoscope: focal spasm of arterioles, followed by progressive sclerosis and narrowing; may see arteriovenous nicking, copper or silver wiring, exudates, and/or hemorrhages
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Placental abruption Umbilical cord prolapse Placenta previa
Retroplacental bleeding - between the placenta and uterine decidua --> can cause placental detachment and vaginal bleeding - the larger the area of placental detachment, the greater the risk for maternal complications such as DIC and hypovolemic shock Potential fetal complications include: hypoxia and preterm delivery --- Umbilical cord prolapse: occurs after ROM, can cause abrupt onset of persistent fetal variable decels or severe bradycardia On exam, the umbilical cord can be palpated below the cervix in the vagina --- Placenta previa = contraindication to labor and vaginal delivery. Undergo CS at 36-37 weeks.
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``` Cyanotic congenital heart diseases: ToF (tetralogy of fallot) Transposition of the great vessels Tricuspid atresia Truncus arteriosus Total anomalous pulmonary venous return with obstruction ```
ToF can increase the risk for hematogenous spread of infection into the brain from ex) sinusitis The R to L shunt of venous blood in the heart (through a VSD) bypasses the pulmonary circulation, where bacteria in the bloodstream are typically filtered and removed by phagocytosis Exam: harsh pulmonic stenosis murmur, VSD murmur CXR: boot-shaped heart (RV hypertrophy) Tet spell: things like exertion, feeding, and agitation can increase PVR, resulting in complete RVOT obstruction --> diversion of blood from the RV into the aorta instead of the pulmonary artery results in acute hypoxemia and cyanosis Transposition of the great vessels: most common congenital cyanotic heart disease in the neonatal period. single S2 (aorta is anterior to pulmonary artery), +/- VSD murmur. CXR: egg on a string heart (narrow mediastinum) After delivery, mixing of deoxygenated and oxygenated blood is necessary for survival thru PDA or VSD or PFO (does not cause a murmur) If suspected, prostaglandins should be initiated to keep the ductus arteriosus open --> echo Tricuspid atresia: single S2, VSD murmur. CXR: minimal pulmonary blood flow Truncus arteriosus: single S2, systolic ejection murmur. CXR: increased pulmonary blood flow, edema TAPVR: severe cyanosis, respiratory distress. CXR: pulmonary edema, "snowman" sign (enlarged supracardiac veins and SVC)
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Serum sickness-like reaction
type III hypersensitivity occurs 1-2 weeks after administration of beta lactams or TMP-SMX Manifestations: fever, urticara, polyarthralgia Lab findings characteristic of typical type III hypersensitivity: nonspecific hypocomplementemia, elevated inflammatory markers Removal of the offending agent is generally sufficient to relieve sx; more severe cases may require glucocorticoid therapy
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CF | Cystis fibrosis
defective chloride transport --> viscous secretions in the lungs, sinuses, and pancreas --> growth failure, recurrent sinopulmonary infections, pancreatic insufficiency (inability to absorb fats and fat soluble vitamins ADEK leading to steatorrhea, failure to thrive, and vitamin deficiencies) Vitamin K deficiency causes prolonged prothrombin time with easy mucosal bleeding and bruising Infectious etiology: infants and young children: MCC = Staph aureus Adults: MCC = Pseudomonas
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SVC syndome
obstruction of SVC impedes venous return from the head, neck, and arms to the heart Sx: dyspnea, venous congestion, swelling of the head neck and arms, headache that is worse when leaning forward, facial swelling, JVP engorgement without peripheral edema Malignancy = most common cause of obstruction CXR to identify cause Tx: radiation therapy as a palliative measure
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PBC | Primary biliary cholangitis
cholestasis with autoimmune destruction of intrahepatic bile ducts (RUQ shows no biliary tract dilation) pruritus, fatigue, jaundice, hepatomegaly, cirrhosis, hyperlipidemia --> cutaneous xanthomas and xanthelasmas + antimitochondrial antibody elevated alk phos, normal hepatic enzymes, normal RUQ US Complications: malabsorption with associated nutrient deficiencies, metabolic bone disease (osteoporosis, osteomalacia), HCC Tx: ursodeoxycholic acid, liver transplant
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Thyroid hormone production in pregnancy
thyroid hormone production increases during pregnancy - estrogen stimulates synthesis of tyroxine binding globulin TBG and decreases TBG clearance --> inc pool of bound thyroid hormone --> patients subsequently increase thyroid hormone production to maintain free hormone levels - hCG which shares a similar structure to TSH subunits directly stimulates TSH receptors --> increased hormone production with feedback suppression of pituitary TSH release
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Study designs
Cohort: observational study in which groups are chosen based upon presence or absence of one or more risk factors --> subjects are studied over time for development of the disease of interest, allowing estimation of incidence within the total population and comparison of incidences between groups. --> allows for calculation of relative risk Case series: study involving only patients already diagnosed with a condition of interest - helpful in determining the natural history of uncommon conditions Case-control: subjects with dz of interest (cases) are compared to a similar group of disease-free subjects (controls). Info is then collected about exposure to risk factors --> retrospective Clinical trial: compares the therapeutic benefit of diff interventions in patients already diagnosed with a particular dz Cross-sectional: takes sample of individuals from a population at a given point in time. Allows determination of a disease's prevalence. Factorial design - involves 2+ experimental interventions, each with 2+ variables that are studied independently Cluster analysis - grouping of different data points into similar categories --> randomization at the level of groups rather than at the level of individuals Cross-over study: group of participants is randomized to 1 tx for a period of time and the other group is given an alternate tx for the same period of time. At the end of the time period the 2 groups then switch treatments for another set period of time
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Amyloidosis
extracellular deposition of insoluble polymeric protein fibrils in tissues/organs Can be primary (AL) or secondary (AA) to chronic inflammatory conditions like inflamma rthritis, chronic infections, ibd, malignancy, vasculitis Manifestations: nephrotic syndrome, restrictive cardiomyopathy, hepatomegaly, peripheral neuropathy, visible organ enlargement like macroglossia, bleeding diathesis, waxy thickening/easy bruising of the skin Dx: Tissue biopsy can confirm diagnosis by showing amyloid deposits (abd fat pad, bone marrow, rectum, kidney, endomyocardial) tx and ppx = colchicine; tx of underlying disease for 2' amyloidosish --- nephrotic syndrome Renal biopsy: amyloid deposits in the glomerular BM, blood vessels, and interstitium of the kidneys that stain with Congo red; demonstrate apple-green birefringence under polarized light; deposits can be visualized on electron microscopy as randomly arranged thin fibrils Deposits may consist of light chains (AL amyloidosis) or abnormal proteins (AA amyloidosis) AL amyloidosis: associated with multiple myeloma, waldenstrom macroglobulinemia; amyloid = light chains, usually lambda AA amyloidosis: associated with chronic inflammatory conditions and infections (RA, IBD, osteomyelitis, TB); amyloid = abnormally folded proteins (beta-2 microglobulin, apolipoprotein, transthyretin)
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Charcot joint
Neurogenic arthropathy due to DM
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EBV (infectious mononucleosis)
- exudative pharyngitis - TENDER cervical lymphadenopathy - fatigue, rash - splenomegaly - acute airway obstruction = rare but potentially serious complication; sx: throat tightness, difficulty swallowing, severe tonsillar enlargement, difficulty swallowing, labored breathing. Tx: corticosteroids Dx: positive heterophile antibody (monospot) test
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Gonococcal pharyngitis | Disseminated gonococcal infection
- inoculation of the pharynx during orogenital contact - pharyngeal edema NO exudate, NONTENDER cervical lymphadenopathy Dx: PCR + fever, lower abd pain --> associated PID --- Disseinated gonococcal infection: purulent monoarthritis and/or triad of tenosynovitis, dermatitis, asymmetric migratory polyarthralgias
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Neonatal thyrotoxicosis
aka neonatal Graves dz due to transplacental passage of anti-TSH receptor antibodies during the 3rd trimester Elevated anti-TSH receptor antibody levels can occur despite maternal tx for Graves and euthyroidism or hypothyroidism during pregnancy Sx: fetal tachycardia, hydrops, growth restriction, poor feeding, warm moist skin Tx: methimazole + beta blocker; self resolves as maternal antibody disappears from infant circulation
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OCP risks and benefits
Benefits: pregnancy prevention, endometrial and ovarian cancer risk reduction, menstrual regulation with reduction in IDA, reduction in risk of benign breast dz Risks: VTE, HTN (due to increased angiotensinogen synthesis by estrogen during hepatic first-pass metabolism), hepatic adenoma (benign; well-demarcated, hyperechoic lesions; remove/watch for malignent transformation) , stroke and MI; slight increase in risk for cervical and breast cancer (risk returns to normal within 10 yrs of OCP discontinuance)
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Aspirin exacerbated respiratory disease
A pseudoallergic rxn to NSAIDs (NOT IgE mediated but occurs in patients with comorbid asthma, chronic rhinosinusitis with nasal polyps, chronic urticaria) presents with asthmatic symptoms and ocular symptoms and facial flushing with 30 min to 3 hrs after NSAID ingestion Tx: avoidance of NSAIDs, desensitization if required, use of leukotriene receptor antagonists like montelukast
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Osteonecrosis Legg-Calve-Perthes disease Transient synovitis
Common complication of long-term glucocorticoid use --> bone and bone marrow infarction Legg Calve perthes disease: Idiopathic avascular necrosis of the femoral capital epiphysis Most commonly affects boys 4-10 Can be managed conservatively with observation and bracing See collapse of the ipsilateral femoral head on plain pelvic x rays As dz progresses, internal rotation and abduction at the hip joint can become markedly limitedd --- Transient synovitis = most common cause of hip pain in children, boys 3-10. Cause is unknown but usually follows a viral infection or mild trauma. Affected hip is typically flexed, slightly abducted, and externally rotated. Tx: rest and NSAIDs
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``` Transfusion reactions Transfusion-related acute lung injury Febrile nonhemolytic reaction Acute hemolytic Delayed hemolytic ```
TRALI: Respiratory distress within 6 hrs of transfusion Signs of noncardiogenic pulmonary edema CXR shows b/l interstitial infiltrates Caused by donor anti-leukocyte antibodies Febrile nonhemolytic reaction: fever and chills within 1-6 hrs of transfusion caused by cytokine accumulation during blood storage Acute hemolytic: fever, flank pain, hemoglobinuria, renal failure, DIC within 1 hr of transfusion; positive direct Coombs test, caused by ABO incompatibility Delayed hemolytic: mild fever and hemolytic anemia within 2-10 days after transfusion; positive direct Coombs, caused by anamnestic antibody response
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Primary hyperaldosteronism (Conn)
Suspect in patient who gets weakness/leg cramps (hypokalemia) after initiation of a thiazide diuretic in uncontrollable HTN Best screening test = early morning plasma aldosterone concentration to plasma renin activity ratio Aldosterone:renin ratio >20 with plasma aldosterone > 15 suggests primary hyperaldosteronism and requires further confirmatory adrenal suppression testing Adrenal suppression testing: salt loading and documenting inability to suppress serum aldosterone if + --> CT to image adreals Usually due to b/l adrenal hyperplasia or aldosterone-producing adrenal adenoma If no discrete mass on CT scan --> adrenal vein sampling is recommended to differentiate between hyperplasia and adenoma Hyperplasia --> medical management; adenoma --> surgery Tx: spironolactone, eplerenone spironolactone = progesterone and androgen receptor antagonist that can cause significant side effects whereas eplerenone = selective mineralocorticoid antagonist with very low affinity for p or a receptors
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Antiphospholipid syndrome
Lupus anticoagulant prolongs PTT partial thromboplastin time - lab artifact false positive RPR since syphilis antigen used in RPR contains cardiolipin
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Aortic regurgitation | Mitral regurgitation
AR: early decrescendo diastolic murmur, best heard along left sternal border at the 3rd and 4rth ICS while pt is sitting up, leaning forward, and holding a breath in full expiration (brings aortic valve closer) congenital bicuspid aortic valve = most common cause of isolated AR in young adults bounding pulse/water hammer pulse, quincke's pinkies, when AR is due to valvular disease, the early diastolic murmur is best heard along the left sternal border; when AR is due to aortic root disease, it is best heard along the R sternal border --- MR: Mitral valve prolapse is the most common cause of chronic MR. MVP occurs due to myxomatous degeneration of the mitral valve leaflets and chordae and causes a mid-systolic click followed by a mid-to-late systolic murmur. As the severity of leaflet dysfunction and MR worsens, the murmur becomes holosystolic and the click may not be audible.
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``` Cardiomyopathies: Tachycardia-mediated cardiomyopathy Hypertrophic obstructive cardiomyopathy Alcoholic cardiomyopathy Dilated cardiomyopathy 2/2 viral myocarditis ```
Tachycardia-mediated cardiomyopathy: any chronic tachyarrhythmia (afib, aflutter, v tach, etc) can cause structural changes in the heart including LV dilation and myocardial dysfunction Tx: aggressive rate control or restoration of nsr; cardiomyopathy potentially reversible HOCM: autosomal dominant genetic disorder - mutation on genes encoding myocardial contractile proteins Harsh crescendo-decrescendo systolic murmur heard best at the apex and LLSB Valsalva or abrupt standing decreases preload and increases the intensity of the murmur Handgrip (inc afterload), squatting (inc after and preload), leg elevation (inc preload) that increases venous return and preload decreases the intensity of the murmur MCC of sudden death in young people, NOT long QT syndromes systolic anterior motion of the mitral valve Alcoholic cardiomyopathy - a form of dilated cardiomyopathy Cessation of drinking can reverse cardiomyopathy Dilated cardiomyopathy 2/2 viral myocarditis: due to a number of viruses: parvovirus B19, HHV6, coxsackievirus, adenovirus, influenza virus, HIV - most commonly affects young adults <60 and presents with signs/sx of decompensated HF DIagnostic gold standard: viral PCR in endomyocardial biopsy
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Minimal change dz
Children age <10 with isolated nephrotic syndrome Normal renal architecture Electron microscopy shows diffuse effacement of foot processes of podocytes = confirmatory Respond rapidly to corticosteroid therapy with complete remission Pathogenesis: t cell mediated injury to podocytes causes inc molecular permeability to albumin -- most common form of nephrotic syndrome in patients with Hodgkin lymphoma also associated with NSAID use
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``` Rapidly progressive glomerulonephritis Membranous glomerulonephritis Membranoproliferative glomerulonephritis Goodpasture's syndrome IgA nephropathy ```
Rapidly progressive glomerulonephritis: Crescent formation in the glomeruli Severe condition with rapid decline in renal function Membranous glomerulonephritis: thickened basement membrane and subepithelial spikes associated with adenocarcinoma of the breast/lung, NSAID use, hep B esp in unvaccinated immigrant children, and SLE Membranoproliferative glomerulonephritis: associated with hep B (less common than membranous) and C mesangial hypercellularity; presents with nephritic syndrome rather than nephrotic syndrome Characteristic microscopic finding: dense intramembranous deposits that stain for C3 - caused by IgG antibodies (C3 nephritic factor) directed against C3 convertase of the alternative complement pathway --> persistent complement activation and kidney damage Goodpasture's syndrome: IgG Anti-GBM antibodies; lungs (cough, dyspnea, hemoptysis) kidneys (nephritic range proteinuria, ARF, dysmorphic red cells/red cell casts on UA)m IgA nephropathy: usually within 5 days (vs PSGN which begins 10-21 days after respiratory tract infection) of upper respiratory tract infection, more common in young adult men (age 20-30), recurrent gross hematuria ; normal complement levels, mesangial IgA deposits seen on kidney biopsy PSGN: most cases follow strep skin infections like impetigo as opposed to pharyngitis
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MAC infection
HIV, CD4<50 Sx: fever, cough, abd pain, diarrhea, night sweats, weight loss + splenomegaly, + elevated alk phos Dx: bx cx Tx: clarithromycin, azithromycin Ppx: CD4<50 --> azithromycin
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DIaphragmatic injury
L diaphragm is more prone to injury than the right due to congenital weakness in the diaphragm's L posterolateral region and the liver's protective effects on the R side Some patients esp children with traumatic diaphragmatic injury may intiially have no sx or signs and can have a delayed presentation with expansion of the diaphragmatic defect and herniation of abd organs Initial test: CXR Confirmatory test: CT
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Osteogenesis imperfecta
connective tissue disorder, AD Varying spectrum: type 1 = mild, fatal = type 2, moderate types 3 and 4 Osteopenia, blue sclerae, recurrent fx, easy bruisability, hypotonia, hearing loss, dentinogenesis imperfecta (discolored dentin shining through the translucent and weak enamel) Type 2: growth restriction, multiple limb fractures, hypoplastic thoracic cavity, shortened femur
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Alpha-1 antitrypsin deficiency
Smoking-induced centriacinar emphysema most commonly causes dz in the upper lobes whereas panacinar emphysema of AAT deficiency classically results in greater destruction of the lower lobes Smokers present in their 30's, nonsmokers present in their 40's AAT deficiency is frequently associated with liver dz Dx: serum AAT levels, PFT's Tx: IV supplementation with human AAT
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Primary sclerosing cholangitis | PSC
inflammation, fibrosis, stricturing of intrahepatic and extrahepatic bile ducts co-exist with UC fatigue, pruritus Labs: elevated alk phos with small transaminitis Liver biopsy not necessary but classically shows intrahepatic ductular obliteration with lymphocytic infiltration and periductular onion skin fibrosis
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``` Genetic Diseases: Friedreich ataxia Hereditary telangiectasia NF 1 and 2 Prader-Willi Angelman Klinefelter Fragile X Beckwith-Wiedemann Phenylketonuria Hereditary fructose intolerance Galactosemia Potter sequence McCune-Albright syndrome Sturge-Weber disease Peutz-Jeghers syndrome Riley-Day syndrome Tuberous sclerosis ```
Friedreich ataxia - AR excessive number of trinucleotide repeats GAA; ataxia; associated w/ necrosis and degen of cardiac muscle fibers leading to myocarditis, myocardial fibrosis, and cardiomyopathy; scoliosis, high arched foot (pes cavus) Hereditary telangiectasia - AD: cutaneous AVM's, recurrent epistaxis, telangiectasias NF1 (von recklinghausen dz) - AD, chromosome 17; cafe au lait spots, multiple neurofibromas (peripheral nerve sheatbh tumors), freckles, lisch nodules. optic pathway glioma in 15% of patients under age 6 (usually asymptomatic but can cause dec visual acuity, proptosis, optic nerve atrophy) NF2 (central neurofibromatosis) - AD, chromosome 22; b/l acoustic neuromas, cataracts Prader-Willi: maternal uniparental disomy (inherit both copies of 15 from mom) --> poor suck, feeding problems, binge-eating, obesity Angelman: paternal uniparental disomy (inherit both copies of 15 from dad) --> short stature, ID, smiling/laughter, hand-flapping, ataxia, seizures Klinefelter: XXY; primary hypogonadism; small testes and tall stature; normal external genitalia and no apparent dysmorphic features Fragile X: x-linked trinucleotide expansion CGG; ID, prominent forehead, macroorchidism Beckwith-Wiedemann: dysregulation of imprinted gene expression in chromosome 11. Macroglossia, rapid growth, hemihyperplasia, umbilical hernia or omphalacele, transient hypoglycemia Complications - Wilms tumor, hepatoblastoma - must be monitored closely with abd US Surveillance - serum afp, abd/renal US PKU: AR mutation in phenylalanine hydroxylase --> severe ID, seizures, musty body odor, hypopigmentation; Tx: dietary restriction of phenylalanine Hereditary fructose intolerance: aldolase B deficiency; vomiting, poor feeding, lethargy, seizures, encephalopathy; when fruits and vegetables are introduced into the diet Galactosemia: absence of galactose-1-phosphate uridyl transferase activity --> presents in the first few days after birth with jaundice, hepatomegaly, FTT after consumption of breast milk or regular infant formula, b/l cataracts, hypoglycemia, convulsions Galactokinase deficiency: cataracts only; otherwise asymptomatic Potter sequence: lethal fetal anomaly; presents with pulmonary hypoplasia, limb deformities (clubfoot, hipdislocation), oligohydramnios; most commonly due to urinary tract abnormalities (eg b/l renal agenesis, polycystic kidney dz) Pathway: urinary tract anomaly (ex. posterior urethral valves) --> anuria/oliguria in utero --> oligohydramnios --> pulmonary hypoplasia, flat facies, limb deformities McCune-Albright syndrome: precocious puberty, cafe au lait spots, multiple bone defects (polyostotic fibrous dysplasia). Defect in the G-protein cAMP-kinase function Sturge-Weber disease: MR, seizures, visual impairment, port-wine stain over trigeminal nerve territory (congenital unilateral cavernous hemangioma); skull x-rays reveal gyriform intracranial calcifications that resemble a tramline Peutz-Jeghers syndrome: GI tract polyposis, mucocutaneous pigmentation (melanotic spots on lips and skin); inc frequency of breast, gonadal, and pancreatic cancer; can develop an estrogen-secreting tumor, leading to precocious puberty Riley-Day syndrome (familial dysautonomia): AR, seen predominantly in children of Ashkenazi jewish ancestry; due to gross dysfunction of the autonomic nervous system with severe orthostatic hypotension Tuberous sclerosis: neurocutaneous genetic syndrome; associated with intracranial tumors (hamartomas, giant cell astrocytomas, subependymal nodules), hypopigmented macules (ash leaf spots), facial angiofibromas, cardiac rhabdomyomas, renal angioleiomyomas, MR, seizures
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Hep C in pregnancy
usually patients with chronic hep C are asymptomatic acute viral hepatitis can be life threatening though esp for patients with preexisting chronic viral hepatitis --> all patients with chronic HCV should be immunized against hep A and B if not already immune - both are inactivated vaccines and safe in pregnancy
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Gestational DM
Target blood glucose: fasting < 95 1 hr post prandial < 140 2 hr post prandial < 120 Tx: 1st line - dietary 2nd line - insulin > metformin, glyburide
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Varicocele
Tortuous dilation of pampiniform plexus More common on L side - L gonadal vein drains to L renal vein which passes between SMA and aorta --> vulnerable to compression beneath the SMA (nutcracker effect) R gonadal vein drains directly into IVC so R sided varicoceles are relatively rare and can be a sign of malignant compression or thrombosis
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Sickle cell disease/trait
Hyposthenuria/isosthenuria (impairment of kidney to concentrate urine) - associated w/ SCD and SCT Results from RBC sickling to the vasa rectae of the inner medulla which impairs countercurrent exchange and free water reabsorption SCT - most common renal issue is painless hematuria. hyposthenuria/isosthenuria also common. SCD: pts are more susceptible to infection with encapsulated organisms like strep pneumo h flu and n meningitidis. Vaccination with PCV13 (conjugated strep pneumo vaccine) decreases the incidence of invasive infections Twice daily administration of prophylactic penicillin should also be given to children with SCD until they reach 5 years of age - SCD = most common cause of pediatric stroke Hydroxyurea - beneficial effect = increase HbF Primary dose limiting side effect = myelosuppression (neutropenia, anemia, thrombocytopenia)
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CCB therapy
Peripheral edema = common side effect - due to preferential dilation of precapillary vessels (arteriolar dilation) --> increased capillary hydrostatic pressure and fluid extravasation into the interstitium Dihydropyridine CCBs like amlodipine and nifedipine are potent arteriolar dilators and cause more peripheral edema than non-dihydropyridine CCBs like verapamil and diltiazem ACEi and ARBs cause post-capillary venodilation and can normalize the icnreased capillary hydrostatic pressure caused by CCB therapy --> combo of CCB and ACEi's can significantly lower the risk of peripheral edema
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Wilms tumor | Neuroblastoma
Wilms tumor: nephroblastoma Proliferation of metanephric blastema Presents with HTN, abd mass, fever, hematuria Neuroblastoma: neural crest-derived adrenal medullary tumor, secretes catecholamines in excess; palpable abdominal mass, hepatomegaly, blue nodules; calcifications and hemorrhages are seen on plain x ray and CT
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Torsades de pointes
a form of polymorphic ventricular tachycardia Tx: unstable patients --> immediate defibrillation stable patients --> IV magnesium
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Hydatid cyst from echinococcus granulosus | Amebic liver abscess from Entamoeba histolytica
Echinococcus granulosus: Due to echinococcus granulosus (tapeworm) infection Humans contract infection from dogs Causes unilocular cystic lesions that can occur in any organ; smaller daughter cysts may be present Usually diagnosed incidentally CT scan shows eggshell calcification Tx: surgical resection under albendazole Entamoeba histolytica: protozoan found in developing counties transmitted through contaminated food/water Can be asymptomatic or cause colitis or liver abscess (single subcapsular cyst) Dx: colitis - stool ova and parasites, stool antigen testing; liver abscess - E histolytica Tx: metronidazole and intraluminal antibiotic like paromomycin
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Renal artery stenosis
ACEi and arbs are initial therapy for pts with HTN and renal artery stenosis Stenting and surgical revascularization is reserved for patients with resistant HTN or recurrent flash pulm edema and/or refractory heart failure due to severe HTN
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DiGeorge syndrome
22q11.2 microdeletion syndrome --> T cell concentrations markedly low characterized by thymic dysplasia, dysmorphic facies, hypocalcemia, cardiac defects (truncus arteriosus, transposition of the great arteries), and FTT in addition to recurrent infections CATCH-22 once digeorge is suspected, get echo and calcium level right away
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Meningitis
Bacterial, fungal, and TB meningitis: elevated protein, low glucose, elevated WBC counts bacterial - neutrophilic pleocytosis TB - lymphocytic pleocytosis Viral: elevated WBC, normal RBC and glucose, protein levels normal to slightly elevated *exception: HSV which has high RBC (due to hemorrhagic destruction of the frontotemporal lobes) and protein levels Guillain-barre: elevated protein, everything else normal Tx: IVIG or plasmapheresis after dx is suspected, must assess pulmonary function by serial spirometry; a decline in FVC indicates impending respiratory arrest (peak flow meter testing if spirometry is not available) -- In children: <3 months: GBS, E coli, listeria, hsv 3m-10yr: Strep pneumo, neisseria meningitidis >11 yrs: neisseria meningitidis -- Cryptococcal meningoencephalitis: CD4 < 100; due to inhalation of spores dx: CSF - high opening pressure, low glucose, high protein, lymphocytic pleocytosis + cryptococcal antigen testing, +India Ink stain or culture on Sabouraud agar
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Neuroanatomy of the lower limbs
Femoral nerve - innervates muscles of the anterior compartment of the thigh, responsible for knee extension and hip flexion, provides sensation to the anterior thigh and medial leg TIbial nerve - supplies muscles of the posterior compartment of the thigh, posterior compartment of the leg, and plantar muscles of the foot --> flexion of the knee and digits and plantar flexion of the foot, sensation to the leg minus the medial side and plantar foot Obturator nerve - innervates the medial compartment of the thigh and controls adduction of the thigh, provides sensation over the medial thigh Common peroneal nerve --> superficial and deep peroneal nerves --> supply the muscles of the anterior and lateral leg, provide sensation to the anterolateral leg and dorsum of the foot
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TTP | HUS
TTP Classic pentad: thrombocytopenia, MAHA, renal insufficiency, neurologic changes, fever HUS: thrombocytopenia, MAHA, renal insufficiency Shiga toxin induces endovethelial damage in renal glomeruli, leading to platelet activation, microthrombi formation, and MAHA
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Benzos
Elderly metabolize more slowly, likely to experience confusion and incr risk of falls Another adverse effect = paradoxical agitation - inc agitation, confusion, aggression, and disinhibition typically within an hr of administration
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PCP
PCP cannot be cultured; dx requires exam of respiratory samples using microscopy with specialized stains induced sputum = least invasive method, if it does not work bronchoscopy with bronchoalveolar lavage is required suspect in CXR with b/l diffuse interstitial infiltrates and elevated LDH Corticosteroids are used in patients with impaired oxygenation (pulse ox < 92%, PaO2 < 70mmHg, A-a gradient >35 mmHg on RA)
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Leukemias and lymphomas
CLL - predominant type in elderly; smudge cells; diagnosed by flow cytometry showing clonality of mature B cells; rituximab (monoclonal antibody against CD20 expressed on B lymphocytes) ALL - predominant type in children 2-10 y/o. Lymphoblasts seen on peripheral smear. 25% lymphoblasts on bone marrow biopsy = confirmation. Lymphoblasts lack peroxidase positive granules but contain cytoplasmic aggregates of PAS periodic acid-Schiff material. +TdT (terminal deoxynucleotidyltransferase) expressed only by pre B and pre T lymphoblasts Burkitt lymphoma - neoplasm of mature B cells. Associated with EBV. Present with mass in mandible or abdominal viscera. High mitotic index is typical. Histo: starry sky appearance. Aggressive but responds well to high dose chemotherapy AML - Auer rods CML - imatinib (BCR-ABL tyrosine kinase inhibitor)
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Splenic abscess
Fever, leukocytosis, LUQ abd pain Can also develop L sided pleuritic chest pain, L pleural effusion, and splenomegaly RF's: infection (endocarditis) with hematogenous spread, immunosuppression, IV drug use, trauma, hemoglobinopathy Infective endocarditis is most commonly associated with splenic abscess
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Hep C
Chronic infection is associated with many extrahepatic manifestations like fatigue, arthralgias, porphyria cutanea tarda Porphyria cutanea tarda presents with fragile photosensitive skin that develops vesicles and bullae with trauma or sun exposure Screening test: serology Confirmation: 2 step process that requires both positive serologic test for HCV antibody and a confirmatory molecular test for the presence of circulating HCV RNA
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Prenatal testing
1st trimester combined test: analyzes risk for trisomy 21 and 18 by measuring PAPP, b-hCG, and nuchal lucency; done at 9-13 weeks [1st trimester US with crown-rump length measurement = most accurate method of determining GA. After 20w, fundal height can be measured in centimeters which correlates with the GA +/- 3w] Cell free fetal DNA: >10 weeks; high sensitivity/specificity for aneuploidy offer in all women 35+; can also identify fetal sex abnormal results can be confirmed by CVS at 10-12 weeks or amniocentesis at 15-20 weeks CVS: 10-13 weeks; definitive karyotypic diagnosis; invasive, risk of spontaneous abortion 2nd trimester quadruple screen: weeks 15-22; measures AFP, estriol, b-hCG, and inhibin A; screens for neural tube defects and aneuploidy; not indicated as follow-up to an abnormal first trimester screen Trisomy 18: dec AFP, b-hCG, estriol. normal inhibin A Trisomy 21: dec AFP, estriol. inc b-hCG, inhibin A Neural tube or abd wall defect: inc AFP, normal b-hCG, estriol, inhibin A. Amniocentesis: 15-20 weeks; definitive karyotypic diagnosis; invasive, risk of membrane rupture, fetal injury and pregnancy loss 2nd trimester US: 18-20 weeks; measures fetal growth, evaluates fetal anatomy, confirms placenta position -- Initial prenatal visit: Rh(D) type, antibody screen, hgb/hmc/MCV, HIV, VDRL/RPR, HBsAg, rubella, varicella, Pap test if screening is indicated, chlamydia PCR, urine culture, urine protein. Gonorrhea screen is indicated only for HIGH RISK patients (age<25, new or multiple partners, history of STI's)
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Ascites fluid
neutrophils: <250 --> no peritonitis; >250 = peritonitis Total protein: >2.5 --> high protein ascites (causes: CHF, constrictive pericarditis, TB, fungal) <2.5 --> low protein ascites (Causes: cirrhosis, nephrotic syndrome) SAAG (serum to ascites albumin gradient; differentiates between portal and non-portal hypertensive etiologies): >1.1 = portal HTN (cardiac ascites, cirrhosis) --> inc capillary hydrostatic pressure <1.1 = other causes (malignancy, pancreatitis, nephrotic syndrome, tb) --> inc capillary permeability
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Whipple's disease | Tropical Sprue
Infection with gram positive bacillus Tropheryma whippelii Presentation: chronic malabsorptive diarrhea, protein-losing enteropathy, weight loss, ARTHRALGIA, lymphadenopathy, low grade fever Dx: small intestinal biopsy and PCR Small intestinal biopsy shows PAS periodic acid-Schiff positive macrophages in the lamina propria containing non-acid-fast gram-positive bacilli Tx: TMP-SMX, doxycycline --- Tropical Sprue - like celiac sprue but infectious etiology and responds to antibiotics
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Paget disease of bone
Most patients are asymptomatic; can present with bone pain and deformity Due to osteoclast dysfunction and increased bone turnover Lab tests: elevated alk phos and bone turnover markers, calcium and phos usually normal Imaging: x-ray shows osteolytic or mixed lytic/sclerotic lesions bone scan shows focal increase in uptake Tx: bisphosphonates
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Acute cholangitis
Charcot triad: fever, jaundice, RUQ pain | Reynolds pentad: + hypotension, AMS
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Cyanide poisoning
In the setting of fires (burning of rubber or plastic) or occupational exposure Acute toxicity: HA, vertigo, dizziness, hyperventilation, tachycardia, n, v --> coma, seizures, bradycardia, hypotension, cardiorespiratory arrest, permanent neurologic deficits * BITTER ALMOND BREATH = characteristic of inhaled cyanide Skin has pinkish-red hue (like carbon monoxide poisoning but unlike methemoglobinemia which has blue hue) Antidotes: hydroxocobalamin or sodium thiosulfate, which directly binds cyanide molecules; or nitrites which induce methemoglobinemia (Fe3+ binds cyanide) Cyanide = potent inhibitor of cytochrome oxidase a3 in the mitochondrial ETC --> binds Fe3+ inhibiting its reduction to Fe2+ and blocks production of ATP from oxidative phosphorylation --> cells switch to anaerobic metabolism --> inc lactic acid formation (>10) and metabolic acidosis -- Nitroprusside = vasodilatory with quick onset/offset of action, commonly used for rapid BP control in pts with hypertensive emergency Metabolism of nitroprusside releases nitric oxide and cyanide ions Cyanide toxicity can occur in patients receiving prolonged infusions or higher doses, most common with renal insufficiency
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Acute liver failure
most common causes = drug tox and acute hep infection Diagnostic requirements: - severe acute liver injury (ALT and AST > 1000) - Signs of hepatic encephalopathy (confusion, asterixis) - synthetic liver dysfunction (prolonged PT with INR >1.5)
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Extrapyramidal symptoms | EPS
Acute dystonia - tx: benztropine or diphenhydramine Akathisia - tx: beta blocker or benztropine Parkinsonism - tx: benztropine or amantadine Tardive dyskinesia - tx: no definitive treatment (but clozapine may help)
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Biostat tests
2 sample z test and t tests: compare 2 means Chi-square: compares the proportions of a categorized outcome ANOVA = compares the means of 3 or more variables
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``` Genetic blood diseases: Fanconi's anemia Diamond-Blackfan anemia Transient erythroblastopenia of childhood G6PD deficiency ```
Fanconi's anemia: AR; pancytopenia (aplastic anemia), congenital anomalies (hypopigmentation, cafe au lait spots, microcephaly, microphthalmia, short stature, horseshoe kidneys, absent thumbs); 4-12 y/o; thrombocytopenia, neutropenia, macrocytic anemia; due to chromosomal breaks; most common congenital cause of anemia Diamond-Blackfan anemia: congenital pure red cell aplasia; presents in first 3 months of life with pallor and poor feeding and congenital anomalies (webbed neck, cleft lip, shield chest, triphalangeal thumbs, pale MM); normocytic or macrocytic anemia with reticulocytopenia; WBC and platelet counts normal; can see elevated fetal Hb levels on electrophoresis Primary pathology: intrinsic defect of erythroid progenitor cells --> inc apoptosis Tx: corticosteroids Transient erythroblastopenia of childhood: acquired red cell aplasia which occurs in healthy children between 6m and 5 years; normocytic normochromic anemia with extremely low reticulocyte count; no congenital abnormalities G6PD deficiency: X-linked recessive. AA men. Episodic hemolysis in response to oxidant drugs, infections, or fava beans. G6PD = enzyme involved in creating NADPH, a cofactor required to create glutathione and prevent the oxidation of hemoglobin. Without G6PD, hemoglobin becomes oxidized and denatures into Heinz bodies --> bite cells, which disrupts RBC membranes and cause hemolysis. Typically occurs in response to oxidant drugs and infection. + prussian blue stain which indicates hemosiderin; found in urine during hemolytic episodes G6PD activity can be used as a screening test, but has reduced sensitivity during an acute hemolytic episode. since the erythrocytes with G6PD deficiency are hemolyzed early and reticulocytes which have normal G6PD levels are circulating at abnormally high levels. it is best to wait 3 months before retesting. Tx is supportive, with removal or management of the underlyin ginsult
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Hypertensive nephropathy | Diabetic nephropathy
Hypertensive: renal vasculature is sensitive to damages incurred by systemic HTN --> as HTN progresses there is progressive decrease in renal blood flow and GFR --> nephrosclerosis (hypertrophy and intimal medial fibrosis of renal arterioles) to glomerulosclerosis (progressive loss of the glomerular capillary surface area with glomerular and peritubular fibrosis) Diabetic: inc GFR --> GBM thickening, hypertrophy, and mesangial volume expansion with GFR returning to normal --> microalbuminuria Histo: diffuse glomerulosclerosis and nodular glomerulosclerosis (pathognomonic) with Kimmelstiel-Wilson nodules DM = leading cause of ESRD in the US Beneficial effect of ACEI in slowing the progression - reduces urinary albumin excretion and the decline in creatinine clearance due to the reduction in BP as well as the direct effect on reducing intraglomerular pressure Most effective intervention for slowing the progression of diabetic nephropathy is tight BP control <140/90; pts with diabetic nephropathy should be treated toward a target BP of 130/80 Intensive glycemic management (Hba1c <7) has been shown to reduce the occurrence and progression of microalbuminuria
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Rickets
Vitamin D or calcium deficiency RF's: exclusive breastfeeding, increased skin pigmentation Manifestations: craniotabes (ping pong ball skull), delayed fontanelle closure, enlarged skull (frontal bossing), enlarged costochondral joints (rachitic rosary), enlarged long bone joints, genu varum X-ray findings: cupping and fraying oft he metaphyses of the long bones Tx: infants who do not ingest fortified baby food or formula should receive vitamin D supplementation of 400IU daily
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Angiodysplasia
painless GI bleeding of maroon colored stools (differentiates from diverticulosis which has bright red blood) Dilated submucosal veins and AVM's most common in the R colon More frequently diagnosed in pts with advanced renal dz and von willebrand dz More common in pts with aortic stenosis Dx: endoscope (upper GI, colonoscopy) - however can be missed on colonoscopy due to poor bowel prep or location behind a haustral fold Asymptomatic pts don't require tx Pts with anemia or gross or occult bleeding can be treated endoscopically with cautery
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Blastomyces Coccidioidomycoses Histoplasma
Blasto: fever, pneumonia, violaceous skin lesions with scrapings showing yeast, bone and prostate also involved; location: midwest, southern mississippi valley Coccidioidomycosis: valley fever (CAP, arthralgias, erythema nodosum/multiforme), Southwest/California Histo: Mississippi and Ohio River valleys; immunocompetent pts usually asymptomatic; immunocompromised pts present with disseminated dz with papular crusting skin lesions, fever, pulmonary, and reticuloendothelial (lymphadenopathy, hepatosplenomegaly) manifestations. Lab findings: pancytopenia, elevated aminotransferase and LDH levels. CXR: diffuse interstitial or reticulonodular infiltrates. Dx: confirm with serum or urine Histoplasma antigen Tx: amphotericin B then transition to oral itraconazole for maintenance therapy
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Reye syndrome
Pediatric aspirin use during influenza or varicella infection Clinical features: acute liver failure, encephalopathy Lab findings: elevated transaminases, coagulopathy, hyperammonemia Liver biopsy: microvesicular steatosis
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Methemoglobinemia | Carbon Monoxide poisoning
methemoglobinemia: excessive exposure to an oxidizing agent Fe2+ --> Fe3+ decreased affinity for oxygen Cyanosis and respiratory depression due to impaired oxygen delivery to the tissues Skin and MM are cyanotic, have dark chocolate-colored blood, and have bluish discoloration *UNLIKE carbon monoxide and cyanide poisoning Supplemental oxygen does not improve cyanosis, blood color, or pulse ox ABG: analyzes unbound arterial oxygen (as opposed to hemoglobin-bound oxygen) --> PaO2 is normal and overestimates the degree of true oxygen saturation Tx: methylene blue --- Carbon monoxide poisoning: headache, nausea, abdominal discomfort skin has pinkish-red hue (like cyanide toxicity but unlike methemoglobinemia which has blue hue)
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Cutaneous larva migrans
hookworm larvae humans are incidental hosts barefoot contact with contaminated sand or soil intensely pruritic, migrating, serpiginous, reddish brown tracks tx: antihelmintic (ivermectin)
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Preterm delivery / short cervix
Strongest RF = previous preterm delivery Other RF's: multiple gestation, hx of cervical surgery (causes cervical scarring/stenosis and incompetence - TVUS measurement of cervical length = gold standard for evaluation of cervical incompetency) --- Short cervix: <2cm without a hx of preterm birth or <2.5cm with history of preterm birth TVUS during second trimester = strong predictor of preterm delivery During pregnancy, progesterone maintains uterine quiescence and protects the amniotic membranes against premature rupture. Supplementation with exogenous progesterone decreases the rate of preterm delivery in pts with short cervices or a hx of preterm birth Short cervix + no history of preterm delivery = vaginal progesterone History of preterm delivery = IM progesterone starting in T2 and undergo serial TVUS Cerclage may be indicated
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``` Neonatology: Neonatal Respiratory Distress Anemia SGA Maternal estrogen effects Apgar scores Physiologic jaundice of the newborn Polycythemia NEC Maternal diabetes complications IVH Erythema toxicum neonatorum Anemia of prematurity ```
Healthy neonates normally lose up to 7% of their birth weight in the first 5 days of life due to excretion of excess fluid acquired in utero and during labor. Birth weight should be regained by age 10-14 days. Weight triples and height doubles by age 1. Appearance of pink stains or brick dust in neonatal diapers = uric acid crystals --- Common causes of neonatal respiratory distress: TTN, respiratory distress syndrome, persistent pulmonary HTN TTN - inadequate alveolar fluid clearance at birth --> mild pulmonary edema; tachypnea begins shortly after birth and resolves by day 2; CXR shows b/l perihilar linear streaking Respiratory distress syndrome - surfactant deficiency --> alveolar collapse and diffuse atelectasis; severe resp distress and cyanosis after premature birth; CXR shows diffuse reticulogranular (ground glass appearance), air bronchograms, and low lung volumes RF's: prematurity, maternal diabetes (delays maturation of pulmonary surfactant production) IUGR, maternal HTN, and chronic intrauterine stress from PROM decrease the risk of RDS because intrauterine stress is thought to stimulate early fetal lung maturity Tx: postnatal surfactant, antenatal corticosteroids Persistent pulmonary HTN - high pulmonary vascular resistance results in R to L shunting and hypoxia; tachypnea and severe cyanosis; CXR shows clear lungs with decreased pulmonary vascularity --- Full term infants are born with adequate iron stores to prevent anemia for the first 4-6m of life regardless of dietary intake Preterm infants are at significant inc risk for IDA. Iron supplementation should be started at birth in exclusively breastfed preterm infants and continued until age 1. All exclusively breastfed infants should also be started on vitamin D supplementation --- SGA infants have a weight under the 10th percentile for GA at birth and may have complications such as hypoxia, polycythemia, hypoglycemia, hypothermia, and hypocalcemia --- Maternal estrogen effects in newborns: breast hypertrophy in both boys and girls, swollen labia, physiologic leukorrhea (whitish vaginal discharge), uterine withdrawal bleeding --- Apgar scores = quick measure of overall neonatal status and response to resuscitation NOT correlation with prognosis, measured at 1 and 5 minutes of life. Scores 7-9 require no intervention. <7 may require further evaluation and resuscitation. -- Physiologic jaundice of the newborn: common, usually benign. - due to elevated RBC concentration and high hemoglobin turnover at birth, slow bilirubin clearance bc hepatic uridine diphosphogluconurate glucuronosyltransferase has not reached adult levels (Asian newborns have decreased UGT activity), increased enterohepatic recycling as sterile newborn gut cannot break down bilirubin to urobilinogen for fecal excretion Newborns should be monitored for persistent or worsening jaundice as high levels can cause brain damage --> frequent feeding should be encouraged to promote gut colonization and fecal excretion Rapidly rising hyperbilirubinemia requires phototherapy for kernicterus prevention Exchange transfusion is indicated for T bili levels > 20-25 mg/dL --- Polycythemia in neonates: hematocrit >65%. Common cause = delayed clamping of the umbilical cord, resulting in excess transfer of placental blood. Other predisposing conditions: in-utero hypoxia (maternal HTN, smoking) or poor placental gas exchange (ie maternal diabetes) Can be asymptomatic and appear ruddy/plethoric. But as hematocrit rises, the viscosity of the blood increases and impairs blood flow to various organs. Most common sx: lethargy, irritability, and jitteriness. Other manifestations: respiratory distress, tachypnea, cyanosis, poor feeding, hypoglycemia, hypocalcemia Asymptomatic neonates require only hydration Symptomatic neonates require partial exchange transfusion, in which blood is removed in exchange for normal saline to normalize the hematocrit --- NEC: associated with prematurity pneumatosis intestinalis on upper GI series --- complications of maternal DM: RDS, prematuriy, macrosomia, TGA, sacral agenesis/caudal regression, hypoglycemia, polycythemia (due to hyperinsulinemia), asphyxia --- IVH: intraventricular hemorrhage; from bleeding in the germinal matrix; occurs predominantly in premature and low birth weight infants; exposure to vascular perfusion injuries is also a RF; many cases are asymptomatic; presentation: pallor, cyanosis, hypotension, seizures, focal neurologic signs, bulging or tense fontanella, apnea, bradycardia Transfontanel ultrasound is necessary for all newborns with predisposing RF's Communicating hydrocephalus (nonobstructive) is a complication in one third of cases; these patients are at greatest risk of death Those who survive high grade IVH's suffer from significant neurodevelopmental disability (cerebral palsy) Prevention of preterm labor and antenatal administration of maternal corticosteroids are the only interventions that can reduce the incidence of IVH and improve overall mortality dx: cranial doppler tx: shunts/drains --- erythema toxicum neonatorum - asymptomatic, scattered erythematous macules, papules, and pustules throughout the body; common in full term neonates; occurs in the first 2 weeks of life; no treatment, will resolve spontaneously --- all vaccines should be administered according to chronologic rather than gestational age in preterm infants; exception: weight should be >2kg (4lb6oz) before first hep B vaccine --- retinopathy of prematurity: caused by neoangiogenesis --> blindness, retinal detachment dx: see on eye exam tx: photoablation f/u: glaucoma --- NEC: premature; dead gut; presents with bloody bowel movement x-ray shows pneumatosis intestinalis tx: NPO, IV abx against gram neg, TPN --- Physiologic RBC nadir in term infants: occurs at age 2-3 months; due to dec EPO due to inc oxygen concentration after birth --> dec reticulocyte production Anemia of prematurity: low EPO levels are exacerbated by short RBC life span and frequent phlebotomy in the NICU --> early onset anemia
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Stable angina
Beta blockers = first line therapy | Also CCB's and nitrates
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Thiazide adverse effects
Hyperglycemia (thiazides impair insulin release), increased LDL cholesterol, triglycerides, hyperuricemia, hyponatremia, hypokalemia, hypomagnesemia, hypercalcemia Adverse effects occur more frequently with chlorthalidone than with other thiazides
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Torus palatinus
Fleshy nontender immobile mass on the midline hard palate | No intervention required unless growth becomes symptomatic or interferes with speech or eating
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Cancer-related anorexia/cachexia syndrome
hypercatabolic state associated with weight loss, anorexia, and an excessive reduction in skeletal muscle Pharma intervention: progesterone analogues (megestrol acetate) or corticosteroids - increases appetite, causing weight gain, and improving well-being
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Postpartum findings | PPH
Normal postpartum findings: Shivering - thermal imbalance Uterus contracts and becomes firm and globular Lochia rubra; after 3-4d lochia serosa, after 2-3w lochia alba Signs of postpartum hemorrhage: boggy uterus, heavy vaginal bleeding, unstable vital signs >500mL after vaginal delivery, >1000mL after CS Hemostasis after placental delivery is achieved by clotting and by compression of the placental site blood vessels by myometrial contraction - disruption of either of these processes can lead to PPH most commonly caused by uterine atony - when the uterus becomes fatigued (as in prolonged labor), over-distended (fetal weight >4000g 8.8lb) or unresponsive to oxytocin from oxytocin receptor saturation Other RF's for atony include operative vaginal delivery and HTN disorders Tx: bimanual uterine massage, oxytocin; uterotonics (methylergonovine, carboprost, misoprostol); embolization; hysterectomy
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Juvenile idiopathic arthritis aka Juvenile RA
symmetric arthritis for at least 6 weeks Lab findings: elevated inflammatory markers, hyperferritinemia, hypergammaglobulinemia, thrombocytosis, anemia (due to inflamm cytokines leading to inc production of hepcidin which inhibits iron absorption across the small intestine and iron release) systemic-onset JIA presents with 2+ weeks of fever occurring once a day, arthritis of 1+ joints, and a pink macular rash that worsens during fever. Joint pain/stiffness classically is worse in the morning and improves throughout the day.
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Spondylolisthesis
Developmental disorder characterized by forward slip of vertebrae (usually L5 over S1) Results in chronic back pain and neuro dysfxn if significant Palpable "step off" detected on exam
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Head and neck cancer
Squamous cell carcinoma of the mucosa of the head and neck Common in ppl with significant history of alc and tobacco use 1st manifestation usually a palpable cervical lymph node Best initial test is panendoscopy (triple endoscopy - esophagoscopy, bronchoscopy, laryngoscopy)
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Vitamin deficiencies | Homocysteine
Vitamin A deficiency - night blindness, photophobia, dry scaly skin, dry conjunctiva/cornea Hypervitaminosis A - anorexia, pruritis, lack of weight gain, inc irritability, fissuring of the corners of the mouth Riboflavin deficiency - due to anorexia, malabsorptive syndromes; sore throat, hyperemic oropharyngeal mucous memrbanes, cheilitis, stomatitis, glossitis Pyridoxine deficiency (B6) - irritability, depression, dermatitis, stomatitis, elevated serum homocysteine (RF for venous thromboembolic dz and atherosclerosis) Niacin deficiency (B3) - pellagra: diarrhea, dermatitis, dementia Homocysteine: B6, folate, and B12 deficiency will all raise levels of homocysteine f
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PPROM (preterm premature ROM)
<34 weeks or 34-37 weeks? 34+ weeks --> DELIVER; penicillin should be administered for patients who are GBS+ or unknown <34 weeks + signs of infection or fetal compromise --> DELIVER + corticosteroids + penicillin <34 weeks and no signs of infection/fetal compromise --> SURVEILLANCE; + corticosteroids + penicillin
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Scabies Chagas disease Strongyloidiasis Onchocerciasis
Scabies: Dx: confirmed by light microscopy of skin scrapings Tx: topical permethrin or oral ivermectin Chagas disease: trypanosoma cruzi Cardiomyopathy, megacolon/megaesophagus Tx: Benznidazole Strongyloidiasis: Urticaria, abdominal pain, respiratory problems Tx: ivermectin Onchocerciasis: river blindness ocular lesions, dermatitis Tx: ivermectin
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Milk-alkali syndrome
Due to excessive intake of calcium and absorbable alkali --> hypercalcemia, renal insufficiency, metabolic acidosis Hypercalcemia causes renal vasoconstriction and decreased glomerular blood flow Inhibition of Na-K-2Cl cotransporter (due to activation of calcium-sensing receptors in the thick ascending loop) and impaired ADH activity lead to loss of Na and free water --> hypovolemia and inc reabsorption of bicarb Meds that raise the risk of milk alkali syndrome: thiazide, ACEi/arbs, NSAIDs Sx: n, v, constipation, polyuria, polydipsia, neuropsych sx Lab findings: hypercalcemia, metabolic alkalosis, AKI, suppressed PTH Tx: discontinuation of causative agent, isotonic saline followed by furosemide
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Prosthetic joint infection
Early onset (<3 months) - Staph aureus (due to infectious organism perioperatively) Delayed onset (3-12 months) - Staph epi (due to less virulent organism perioperatively) Late onset (>12 months) - Staph aureus, gram neg rods (usually due to hematogenous spread of distant infection)
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``` CMV retinitis HSV retinitis HIV retinopathy Diabetic retinopathy Toxo chorioretinitis Syphilitic chorioretinitis Contact lens-associated keratitis Retinal detachment Trachoma ```
CMV retinitis: full-thickness retinal inflammation; fundoscopy shows yellow-white fluffy hemorrhagic lesions along the vasculature; sx: blurred vision, floaters, photopsia HSV retinitis: b/l necrotizing retinitis with keratitis and conjunctivitis; funduscopy shows widespread pale peripheral lesions and central necrosis of the retina i HIV retinopathy: cotton-wool retinal lesions, rarely hemorrhagic and tend to resolve over weeks to months Diabetic retinopathy: microaneurysms, hemorrhages, exudates, retinal edema, cotton wool spots, newly formed vessels Toxo chorioretinitis: eye pain, decreased vision; retinal lesions in nonvascular distribution Syphilitic chorioretinitis: uveitis, diminished visual acuity Contact lens-associated keratitis: painful, red eye and opacification and ulceration of the cornea, most cases due to gram neg organisms like pseudomonas and serratia Retinal detachment: photopsia (flashes of light), floaters (spots in the visual field) "curtain coming down over my eye"; ophthalmoscopic exam reveals grey elevated retina inciting event can occur months before retinal detachment. Myopia or trauma (cataract surgery) can cause retinal breaks, through which fluid seeps in and separates the retinal layers tx: laser therapy and cryotherapy to create permanent adhesion Trachoma: conjunctival injection, tarsal inflammation, pale follicles leading cause of blindness worldwide spreads effectively in crowded or unsanitary conditions Follicular conjunctivitis and pannus (neovascularization) formation in the cornea; often concomitant nasopharyngeal infection repeated or chronic infection leads to scarring of the eyelids and inversion of the eyelashes (trichiasis); the lashes rub on the eye and cause ulcerations and blindness
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Muscular dystrophies
Myotonic muscular dystrophy: - AD expansion of CTG trinucleotide - Presentation: 12-30 onset. Facial weakness, hand grip myotonia, dysphagia - Comorbidities: arrythmias, cataracts, balding, testicular atrophy/infertility - Prognosis: death from respiratory or heart failure Duchenne DMD: - X-linked recessive deletion of dystrophin gene on chromosome X (no dystrophin) - Presentation: 2-3 onset. progressive weakness, Gower maneuver, calf pseudohypertrophy - Comorbidities: scoliosis cardiomyopathy - Prognosis: wheelchair dependent by adolescence, death by age 20-30 from respiratory or heart failure - Dx: screening - elevated serum CPK and aldolase (shows some kind of muscular dystrophy, not specific for DMD). Fibrosis and fatty infiltration on calf muscle biopsy support the diagnosis. Genetic studies = gold standard Becker BMD: - X-linked recessive deletion of dystrophin gene on chromosome X (reduced dystrophin)s - Presentation: 5-15 onset. milder weakness compared to Duchenne - Comorbidities: cardiomyopathy - Prognosis: death by age 40-50 from heart failure
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Trousseau's syndrome
Aka migratory superficial thrombophlebitis A hypercoagulable disorder that usually presents with unexplained superficial venous thrombosis at unusual sites (arm, chest area, etc). Usually diagnosed prior to or at the same time as an occult visceral malignancy. Associated with cancer involving the pancreas (most common), lung, prostate, stomach, and colon, and acute leukemias.
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Cerebral amyloid angiopathy
Amyloid angiopathy = MCC of spontaneous lobar hemorrhage, particularly in adults age > 60 Due to beta-amyloid deposition in the walls of small to medium size cerebral arteries, resulting in vessel wall weakening and predisposition to rupture Affects occipital and parietal lobes most often
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Zenker diverticulum
- usually age >60, males - dysphagia, halitosis, regurgitation/aspiration, variable neck mass - develops above the upper esophageal sphincter with posterior herniation between the fibers of the cricopharyngeal muscle. Upper esophageal sphincter dysfunction and esophageal dysmotility are believed to be the cause of Zenker - dx: barium esophagram, esophageal manometry - tx: open/endoscopic surgery, cricopharyngeal myotomy
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Alcohol abuse = MCC of __ deficiency in the US
Folate --> megaloblastic anemia
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Methamphetamine use
Associated with cardiomyopathy; can lead to decompensated heart failure
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Intrauterine fetal demise
Fetal death > 20 weeks prior to expulsion from mom Pts present with decreased/absent fetal movement Inability to find FHR by doppler is not diagnostic; could be due to fetal malpresentation or maternal obesity Must be confirmed by absence of fetal cardiac activity on US induction of labor for vaginal delivery = best option; delivery can be delayed to allow time for parental acceptance but retention of the fetus for several weeks can lead to coagulopathy
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Gastric ulcer Duodenal ulcer Jejunal ulcer
Gastric ulcer: pain worse after eating due to increased acid secretion Duodenal ulcer: pain worse on empty stomach (unopposed gastric acid emptying into the duodenum), improves with food (due to alkaline fluid secretion into the duodenum) Jejunal ulcer: suggestive of excess gastric acid that cannot be fully neutralized in the duodenum --> suspect gastrinoma/Zollinger-Ellison syndrome (associated with MEN1) Dx: fasting serum gastrin level (off PPI therapy for 1 week): <110 rules it out, >1000 is diagnostic. 110-1000 is nondiagnostic and requires secretin-stimulation test Secretin stimulates release of gastrin by gastrinoma cells. Normal gastric G cells are inhibited by secretin; therefore, secretin administration should not cause a rise in serum gastrin concentrations in patients with other causes of hypergastrinemia.
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Bath salts
Amphetamine analogs Cause severe agitation, combativeness, delirium, psychosis In contrast to other stimulants and hallucinogens which have a much shorter duration of effect, the effects of bath salt intoxication may take several days to weeks to subside
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Legionella
High grade fever, GI symptoms, neurologic symptoms CXR shows interstitial infiltrates Hyponatremia, hepatic dysfunction Gram neg rod that stains poorly bc it is primarily intracellular --> sputum Grain stain show neutrophils but no organisms Definitive dx: culture in combo with urinary antigen Tx: macrolides, fluoroquinolones
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Alpha-1 antitrypsin deficiency
Genetic disease with sx of COPD and liver dz Smokers present in their 30's, non-smokers present in their 40's Consider in pts with COPD at young age, COPD with minimal or no history of smoking, or family hx of emphysema or liver dz Dx: AAT levels, PFT's Tx: IV supplementation of human AAT
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Interstitial lung disease
Asbestos: pneumoconiosis - inc incidence in mining, shipbuilding, insulation, pipework, plumbing - CXR: insterstitial abnormalities in lower lobes, pleural plaques = hallmark - PFT: restrictive pattern - bronchogenic carcinoma = most common malignancy in ppl with asbestos esp if smoker as well; more common than pleural mesotheliomas
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Sideroblastic anemia
Acquired: due to defective heme synthesis (ex isoniazid = pyridoxine antagonist and there are pyridoxine-dependent steps in protoporphyrin synthesis) - Microcytic hypochromic anemia simulating IDA - Can usually see dimorphic RBC population (hypochromic and normochromic) - Iron studies: inc serum iron concentration, decreased TIBC - Bone marrow sampling confirms dx by demonstrating ringed sideroblasts
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Iron poisoning | Lead poisoning
Iron poisoning: acute: abd pain, HEMATEMESIS, hypovolemic shock, anion gap metabolic acidosis Source: pre-natal vitamins, iron supplements Antidote = deferoxamine Iron is radioopaque and can be seen on xray Confirm: serum iron levels Tx: deferoxamine; whole bowel irrigation is sometimes instituted but other methods of decontamination are not recommended Lead poisoning: RF's: infants/toddlers living in homes built before 1978 asymptomatic, cognitive/behavioral problems, irritability, poor appetite, headaches, abd pain, anemia Dx: capillary (fingerstick) blood specimens are widely used as screening however false positive results are common --> confirm with a venous lead measurement if screening capillary lead level is >5. Tx: succimer, calcium EDTA In adults - GI manifestations (abd pain, constipation), neuropsychiatric manifestations (forgetfulness), motor and sensory neuropathies (extensor weakness, stocking-glove distribution), hematologic manifestations (lead disrupts heme synthesis --> microcytic anemia with basophilic stippling) Occupation: battery manufacturing, plumbing, home restoration, distillation of alcohol through parts with lead soldering)
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Myoglobinuria
from rhabdomyolysis Suspect when test results demonstrate large amount of blood on UA but relative absence of RBC's on urine microscopy nearly 20% of cocaine overdoses are complicated by rhabdo
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Niacin
side effects: cutaneous flushing, generalized pruritis Due to drug-induced release of histamine and prostaglandins (not a true hypersensitivity) Low-dose aspirin can reduce or prevent cutaneous flushing and pruritis if taken 30 min before niacin Sx usually improve after 2-4 weeks of therapy -- pellagra can result from carcinoid syndrome (serotonin is synthesized in carcinoid cells from tryptophan which is also used in the production of niacin)
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Hearing loss
Presbycusis: sensorineural hearing loss that occurs with aging; symmetrical high frequency hearing loss; difficulty hearing in crowded or noisy environments Otosclerosis: conductive hearing loss due to bony overgrowth of the stapes; begins with low frequency hearing loss Serous otitis media: Meniere's disease: tinnitus, vertigo (fullness due to lots of endolymph - endolymph hydrops), sensorineural hearing loss tx: benzos, anticholinergics (scopolamine), antihistamines - for acute stuff furosenemide - long term Cholesteatoma: abnormal skin growth in the middle ear. Suspect in any patient with continued ear drainage for several weeks despite appropriate antibiotic therapy. Chronic middle ear disease leads to formation of a retraction pocket in the tympanic membrane which can fill with granulation tissue and skin debris Complications: hearing loss, CN palsy, vertigo, brain abscess or meningitis; should be referred to ENT
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AAA
Repair can lead to bowel ischemia/infarction Strongest predictors of AAA expansion/rupture = large aneurysm diameter, rapid rate of expansion, and current cigarette smoking Indications for repair: size > 5.5 cm, rapid rate of expansion (>0.5cm in 6 months or >1cm in a year), presence of sx regardless of size
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Fetal exposure
Valproic acid: inc risk of neural tube defects, cardiac anomalies, dysmorphic facies Fetal hydantoin syndrome (phenytoin): nail/digit hypoplasia, dysmorphic faces, MR, growth deficiency Fetal alcohol syndrome: growth deficiency, long smooth philtrum, small palpebral fissures, thin upper lip, MR Cocaine: jitteriness, excessive sucking, hyperactive Moro reflex Neonatal abstinence syndrome (opioids): irritability, high pitched cry, poor sleeping, tremors, seizures, sweating sneezing, v, d
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Refeeding syndrome
Due to surge in insulin activity as body resumes anabolism Carb ingestion causes pancreatic insulin secretion and cellular uptake of phosphorus, potassium and magnesium Phosphorus = primary deficient electrolyte as it is required for energy (ATP) Potassium and magnesium deficiencies potentiate cardiac arrhythmias and cardiopulmonary failure
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Babesiosis Ehrlichiosis Dengue fever Chikungunya fever
- Tick bite (Ixodes) - Fever, fatigue, myalgias, headache - Lab findings: anemia, thrombocytopenia, inc bilirubin/LDH/LFT's - Dx: blood smear showing intraerythrocytic rings aka maltese cross - Tx: atovaquone and azithromycin Ehrlichiosis - Tick bite (Amblyomma americanum) - Fever, myalgias, headache (flu-like illness) * * NO RASH ** - Lab findings: leukopenia, thrombocytopenia, elevated aminotransferases and LDH - Dx: clinical; visualization of intracytoplasmic morulae in WBC's or thru PCR - Tx: doxycycline Dengue fever: - Vector: aedes mosquito - Can be asymptomatic, but some pts develop fever and severe myalgias/headache (breakbone fever) - Lab findings: leukopenia, thrombocytopenia Chikungunya fever: - Central/S America, Africa, S Asia - Vector: aedes mosquito - HA, myalgias, conjunctivitis, maculopapular rash - Lab findings: lymphopenia, thrombocytopenia, elevated liver enzymes - Tx: supportive care
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Sialadenosis
Benign noninflammatory nontender enlargement of the submandibular glands Commonly found in patients with advanced liver disease (eg alcoholic and nonalcoholic cirrhosis) and ppl with altered dietary patterns or malnutrition (eg diabetes, bulimia)
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Coarctation of the aorta
Clinical features: - upper body: well developed, HTN - lower body: underdeveloped, claudication - brachial-femoral pulse delay - upper and lower extremity BP differential - systolic murmur; can be continuous if collateral vessels are present Diagnostic studies: ecg - LV hypertrophy cxr - inferior notching of the posterior third of the 3rd to 8th ribs (bony erosion due to enlarged intercostal arteries), "3 sign" due to aortic indentation
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Turner syndrome
Streak ovaries --> low E --> risk of osteoporosis Associated with kyphoscoliosis Associated with bicuspid aortic valve (20-30%), coarctation of the aorta (3-10%), and aortic root dilation with inc risk of aortic dissection
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Attributable risk percent
aka etiologic fraction represents the excess risk in a population that can be explained by exposure to a particular risk factor ``` ARP = (risk in exposed - risk in unexposed) / risk in exposed ARP = (RR-1)/RR ```
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Gallstone ileus
a form of SBO n, v, pneumobilia, hyperactive bowel sounds, dilated loops of bowels dx: confirmed by abd CT scan tx: surgical removal of stone and either simultaneous or delayed CCY
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Bronchial rupture
Suspect in persistent pneumothorax and significant air leak despite chest tube placement in a patient who has sustained blunt chest trauma Other findings: pneumomediastinum, subcutaneous emphysema
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Analgesic nephropathy
Chronic analgesic use can cause CKD due to chronic tubulointerstitial nephritis and hematuria due to papillary necrosis from analgesic-induced vasoconstriction of medullary blood vessels (vasa recta)
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Toxic megacolon
Associated with UC and c diff presents with total or segmental nonobstructive colonic dilation, severe bloody diarrhea, and systemic findings Dx: confirmed by plain abd x rays showing dilated right or transverse colon, possible multiple air fluid levels, and thick haustral markings that do not extend across the entire lumen; and >3 of the following: fever, tachycardia, leukocytosis, anemia also, abdominal distension medical emergency tx: IVF, antibiotics, bowel rest, IV corticosteroids for IBD-induced toxic megacolon
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Wilson dz
AR --> hepatic copper accumulation --> leak from damaged hepatocytes --> deposits in tissues Atrophy of the lenticular nucleus Hepatic, neurologic, and psychiatric manifestations Dx: decreased ceruloplasmin, inc urinary copper excretion tx: chelators (D-penicillamine, trientine); zinc (interferes with copper absorption)
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Tourettes
Most effective nonpharm tx = habit reversal training, a form of behavioral therapy Alpha-2 adrenergic receptor agonists (eg clonidine, guanfacine) in mild cases Risperidone or other dopamine blocking agents in more severe cases
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Trisomy 18 Trisomy 13 CHARGE syndrome
Trisomy 18 / Edwards syndrome: IUGR, microcephaly, VSD, closed fists with overlapping fingers, micrognathia, prominent occiput, rocker-bottom feet, severe ID Trisomy 13 / Patau syndrome: cutis aplasia (absence of epidermis over the skull) and microphthalmia, midline defects (holoprosencephaly, omphalocele) CHARGE: coloboma, heart defects, atresia choanae, retardation of growth/development, genitourinary anomalies, ear abnormalities/deafness Choanal atresia: failure to pass a catheter through the nares into the oropharynx; dx is confirmed by CT
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The only indication for hormone replacement therapy
for vasomotor symptoms (hot flash) in women age <60 who have undergone menopause within the past 10 years NOT indicated for prevention of chronic disease
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Priapism
most common drug that causes priapism = prazosin | also, trazodone
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Valvular heart dz Aortic Regurg Supravalvular aortic stenosis ASD
AR: Exertional dyspnea, pounding heart sensation (inc in LV size brings the ventricular apex close to the chest wall), widened pulse pressure, water hammer or Corrigan pulse Most common cause of AR in developing countries is rheumatic heart disease; in developed countries it is often due to aortic root dilation or congenital bicuspid valve Supravalvular aortic stenosis: 2nd most common type of AS; congenital LV outflow tract obstruction due to discrete or diffuse narrowing of the ascending aorta. Causes a systolic murmur in the FIRST right ICS, higher than where valvular aortic stenosis is best heard; may have unequal carotid pulses or differential BP in the upper extremities --> LV hypertrophy over time; associated also with coronary artery stenosis --> can lead to cardiac ischemia during exercise, etc ASD: wide, fixed splitting of the 2nd heart sound. Systolic murmur is sometimes heard at the LUSB from increased flow across the pulmonary valve
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Bacillary angiomatosis
Bartonella infection seen primarily in patients with HIV with CD4 counts <100 Vascular cutaneous lesions that begin as small papules that evolve into friable pedunculated or nodular lesions Constitutional symptoms Dx: tissue biopsy with histopathology and microscopic ID of organisms Tx: antibiotics (doxycycline, erythromycin) and initiation of antiretroviral therapy usually 2-4 weeks later
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Palpable breast mass - next step?
Age < 30 --> US +/- mammo | Age > 30 --> mammo +/- US
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Warfarin-associated intracerebral hemorrhage | Warfarin-induced skin necrosis
Reverse anticoagulation immediately Initial therapy - IV vitamin K (takes 12+ hrs to be effective though) Prothrombin complex concentrate (contains vit K dependent clotting factors that offer rapid and short term reversal) FFP - considered if PCC is not available --- Warfarin-induced skin necrosis: occurs within the first few days of warfarin therapy (usually at large loading doses). Warfarin decreases protein C anticoagulant activity to 50% within the first day while levels of procoagulant factors (2, 9, 10) decline more slowly, leading to a transient hypercoagulable state --> inc the risk for VTE and skin necrosis, esp in pts with underlying hereditary protein C deficiency tx: stop warfarin, administer protein C concentrate
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Behcet disease
``` Recurrent, painful oral aphthous ulcers Genital ulcers Eye lesions (uveitis) Skin lesions (eg erythema nodosum, acneiform lesions) Thrombosis --> major cause of morbidity ``` Evaluation: Pathergy (exaggerated skin ulceration with minor trauma eg needlestick) Biopsy
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Adverse effects of antithyroid drugs, radioiodine ablation, and surgery
Antithyroid drugs: agranulocytosis - if pt complains of sore throat/fever/etc, drug should be discontinued promptly and WBC measured. Total WBC count < 1000 warrents permanent discontinuation of the drug. If total is more than 1500, antithyroid drug toxicity is unlikely the cause methimazole - 1st trimester teratogen, cholestasis PTU - hepatic failure, ANCA-associated vasculitis Radioiodine ablation: permanent hypothyroidism, WORSENING OF OPHTHALMOPATHY, possible radiation side effects Surgery: permanent hypothyroidism, risk of recurrent laryngeal nerve damage, risk of hypoparathyroidism
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Over the counter cold medicine (antihistamines, alpha-adrenergic agents, dextromethorphan)
Can cause hallucinations/psychosis as a side effects
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Adenomyosis
Endometrial glands become trapped within the myometrium and continue to cyclically shed in that new location Typical presentation: dysmenorrhea with heavy menstrual bleeding with progression to chronic pelvic pain PE: boggy (soft/flaccid), tender, uniformly enlarged uterus Dx: pelvic US and/or MRI - initial gold standard = histopathologic exam of hysterectomy specimen Tx: hormonal methods (OCP's, levonorgestrel IUD) hysterectomy = definitive
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Epiglottitis Croup Retropharyngeal abscess
Epiglottitis - thumbprint sign Croup (laryngotracheitis) - steeple sign (subglottic edema); parainfluenza viral infection of the larynx and trachea features: inspiratory stridor, barking cough, hoarseness tx: mild (no stridor at rest) - humidified air +/- corticosteroids moderate/severe (stridor at rest) - corticosteroids + nebulized racemic epinephrine Retropharyngeal abscess - prevertebral widening; see air-fluid level on scans; infection within the retropharyngeal space can drain into the superior mediastinum and extend through the alar fascia into the posterior mediastinum and result in acute necrotizing mediastinitis
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FSGN
- classically seen in HIV and heroin use, obesity, AA and hispanic ethnicity - presentation: proteinuria and azotemia with rapid development of renal failure
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Stroke | Lacunar strokes
Lacunar: due to microatheroma formation and lipohyalinosis in the small penetrating arteries of the brain --> often affect the internal capsule and result in pure motor hemiparesis
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Cardiac tamponade Pulsus paradoxus Constrictive pericarditis
Cardiac tamponade: decrease in cardiac output bc the pressure exerted on the heart by the pericardial fluid is greater than the venous pressure that fills the R atrium during diastole. can be a postop complication of CABG ---> beck triad, pulsus paradoxus Urgent echo confirms the diagnosis --> immediate percutaneous or surgical (pericardial window) drainage of pericardial fluid Cardiac catheterization in patients with tamponade typically reveal elevated and equilibrated intracardiac diastolic pressures. Pulsus paradoxus: exaggerated drop in SBP (>10 mmHg) during inspiration most commonly seen in patients with cardiac tamponade but can also occur in severe asthma and COPD, hypovolemic shock, and infrequently with constrictive pericarditis cardiac filling in cardiac tamponade can only occur during deep inhalation when the neg intrathoracic pressure allows for a pressure diff between the vena cava and the R atrium can manifest as a loss of palpable radial pulse during inspiration Constrictive pericarditis = important cause of right heart failure
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Hypomagnesemia
Associated with chronic alcoholism Commonly occurs with hypokalemia (hypokalemia that cannot be corrected with K replacement) Intracellular mag is thought to inhibit K secretion by renal outer medullary potassium channels in the collecting tubules of the kidney --> low intracellular magnesium concentrations result in excessive renal K loss and refractory hypokalemia Normalize mag levels to allow for restoration K
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TORCH infections
Rubella: PDA, sensorineural hearing loss, cataracts Toxo: chorioretinitis, hydrocephalus, intracranial calcifications CMV: chorioretinitis, periventricular calcifications Syphilis: hepatomegaly, nasal discharge/snuffles, osteoarticular destruction, maculopapular rash
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Nephrotoxic and ototoxic drugs
Aminoglycosides, vancomycin, furosemide (loop diuretics)
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Trihexyphenidyl
Anticholinergic meds used in Parkinson's disease, generally in younger patients where tremor is the primary sx
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Schatzki ring | Plummer-Vinson syndrome
``` Schatzki ring: scarring/tightening of the distal esophagus (peptic stricture) causing intermittent dysphagia from acid reflux associated with hiatal hernia tx: pneumatic dilation ``` Plummer-Vinson syndrome: esophageal webs (more proximal), IDA, dysphagia, spoon-shaped nails, glossitis/cheilitis tx: iron replacement
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Airway pressures / Physiology
Peak airway pressure = sum of airway resistance + plateau pressure Plateau pressure = sum of elastic pressure + PEEP Calculated by performing the end-inspiratory hold maneuver PEEP is calculated with the end-expiratory hold maneuver Elastic pressure = tidal volume / compliance Decreased compliance (eg pulmonary fibrosis) causes stiffer lungs and higher elastic pressure
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Cortical laminar necrosis
Hallmark of prolonged seizures / status epilepticus Increased risk of developing permanent injury due to excitatory cytotoxicity Can lead to persistent neurologic deficits and recurrent seizures
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Brain death
Absent cortical and brain stem functions Spinal cord may still be functioning though so DTR's may still be present Confirm using EEG = isoelectric EEG
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SLE
known RF for accelerated atherosclerosis and premature CAD hematologic manifestations include anemia, leukopenia, thrombocytopenia, and antiphospholipid syndrome Pancytopenia is most commonly due to concurrent peripheral immune-mediated destruction of all 3 cell lines Focal proliferative glomerulonephritis = subset of class III SLE nephritis
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Angina
If suspected and stable --> exercise ECG = initial stress test for dx and risk stratification Coronary angiography is performed in pts with high risk findings on initial stress test
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``` Dermatology: Actinic keratosis Seborrheic dermatitis Seborrheic keratosis Vitiligo Tinea versicolor Acanthosis nigricans Senile purpura Porphyria cutaneous tarda SCC/BCC Melanoma Impetigo SJS/TEN Exfoliative dermatitis SSSS Ecthyma gangrenosum Bullous pemphigoid vs Pemphigus valgaris Idiopathic guttate hypomelanosis Piebaldism Pyoderma gangrenosum Keratoacanthoma ```
Actinic keratosis: scaly papules or plaques; chronic sun exposure = major RF; premalignant lesion for SCC although likelihood is low Seborrheic dermatitis: scaly, oily erythematous rash affecting the skinfolds around the nose, eyebrows, ears, and scalp Seborrheic keratosis: stuck on appearance found on elderly people *sudden onset of a lot of these = sign of malignancy Vitiligo: regional destruction of melanocytes most likely due to autoimmune etiology; associated with other autoimmune conditions Tinea versicolor: hypopigmented macules (partial depigmentation when compared to vitiligo); HIV infection inc risk for tinea versicolor Acanthosis nigricans: benign - seen in younger people, associated with insulin resistance malignant - associated with underlying neoplasms esp of the GI and GU. Sudden appearance of these skin changes in older people is suggestive of underlying malignancy Senile purpura: Noninflammatory disorder most common in the elderly but can also be seen in middle aged patients with extensive sunlight exposure; presents with skin fragility, ecchymosis, and normal lab studies Caused by loss of elastic fibers in perivascular connective tissue Minor abrasions that would merely stretch the skin in younger patients can rupture superficial blood vessels in the elderly --> subsequent extravasation of blood leads to ecchymosis over vulnerable areas; can see brownish discoloration from hemosiderin deposition Porphyria cutanea tarda: most common porphyria due to deficiency of uroporphyrinogen decarboxylase Can be triggered by ingestion of ethanol or estrogens Frequently associated with hep C*, HIV, excessive alcohol consumption, estrogen use, smoking Manifestations: photosensitivity with blisters and skin fragility SCC = enlarging nodule in sun-exposed areas. lesions often become keratinized or ulcerate with crusting and bleeding. Can display early perineural invasion, causing regional neurologic symptoms. Common in patients with hx of organ transplant and on chronic immunosuppressive therapy. ; most common malignancy of the lip, with 95% cases occurring in the lower lip vermilion histology: invasive cords of squamous cells with keratin pearls associated with burn wound = marjolin ulcer BCC typically affects the upper lip Melanoma - suspect if substantially diff from others (ugly ducking sign), itches or bleeds, or develops new nodularity --> excisional biopsy with initial margins of 1-3mm normal tissue Impetigo: limited skin involvement - topical antibiotics like mupirocin extensive skin involvement - oral antibiotics like cephalexin, dicloxacillin, clindamycin SJS/TEN: SJS <10% BSA; 10-30% SJS/TEN; TEN >30% TEN - inflammatory hypersensitivity reaction to drugs or infections - coalescing erythematous macules, bullae, desquamation, and mucositis. + Nikolsky's sign Exfoliative dermatitis / erythroderma: widespread, scaly eruption of the skin; may be drug-induced, idiopathic, or 2/2 underlying dermatological or systemic dz SSSS: toxin produced by Staph aureus; skin has sandpaper-like diffuse erythematous rash; bullae, + Nikolsky's sign, facial edema, perioral crusting, dehydration Ecthyma gangrenosum: associated with Pseudomonas aeruginosa; rapid progression from small erythematous macule to larger, nontender nodules with necrosis; caused by bacterial invasion through the media and adventitia of blood vessels; seen in patients who are immunocompromised Bullous pemphigoid: tense bullae; no oral lesions or acantholysis; subepidermal cleavage with linear IgG against hemidesmosomes at the basement membrane; tx: high potency topical glucocorticoids Pemphigus vulgaris: acantholysis and intraepidermal cleavage; intercellular IgG against desmogleins; involvement of oral cavity Idiopathic guttate hypomelanosis: common finding with aging; characterized by smal macules in sun-exposed areas Piebaldism: AD; patchy absence melanocytes; usually noticed at birth and confined to the head and trunk Pyoderma gangrenosum: neutrophilic ulcerative skin disease. Starts off as an inflammatory papule pustule vesicle or nodule and progresses to form an expanding ulcer with a purulent base and ragged violaceous borders; associated with underlying systemic disorder like IBD, arthropathies, and hematologic conditions. Diagnosed clinically after exclusing other disorders; skin biopsy. Tx requires local or systemic corticosteroids Keratoacanthoma: benign cutaneous tumor with dome-shaped nodules with central keratin plug
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Diabetes Meds
Metformin: initial therapy, weight neutral, low risk of hypoglycemia, lactic acidosis = life threatening complication so avoid in pts with renal insufficiency Sulfonylurea: main side effects are weight GAIN and hypoglycemia Pioglitazone (TZD's): side effects include weight GAIN, edema, CHF, bone fracture, bladder cancer; CAN be used in renal insufficiency DPP-4 inhibitors (sitagliptin): low risk of hypoglycemia, WEIGHT NEUTRAL. CAN be used in renal insufficiency GLP-1 receptor (exenatide): causes WEIGHT LOSS, low hypoglycemia risk
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Trochanteric bursitis vs OA of the hip
Trochanteric bursitis: superficial unilateral hip pain exacerbated by external pressure to the upper lateral thigh OA of hip: pain deep within the joint referred to the inguinal area or to the knee; internal rotation of the hip worsens the pain
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Sleep syndromes: Delayed sleep phase syndrome Advanced sleep phase syndrome Shift work sleep disorder
Delayed: circadian rhythm disorder inability to fall asleep at "normal" bedtimes --> sleep-onset insomnia, excessive daytime sleepiness can sleep normally if allowed to follow internal circadian rhythm and sleep until late morning Advanced: circadian rhythm disorder inability to stay awake in the evening (usually after 7PM) --> social functioning is difficult early morning insomnia due to early bedtime Shift work disorder: recurrent pattern of sleep interruption due to shift work --> work schedule that is incongruent with a NORMAL circadian clock
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TCA overdose
mental status change, seizures, tachycardia, hypotension, cardiac conduction delay, anticholinergic effects, and CARDIOTOXICITY due to blockade of cardiac fast sodium channels --> QRS prolongation and risk of developing ventricular arrhythmia ECG should be obtained immediately and monitored QRS > 100ms = associated with increased risk for ventricular arrhythmia and seizures; used as an indication for sodium bicarb therapy TCA's inhibit fast sodium channels in the His-Purkinje system and myocardium. This decreases conduction velocity, increases duration of repolarization, and prolongs absolute refractory periods. --> hypotension, QRS prolongation, and ventricular arrhythmias Sodium bicarb increases serum pH and extracellular sodium, which alleviates depressant actions on myocardial sodium channels
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Pseudocyesis
RF = history of infertility, prior pregnancy loss Somatization of stress that affects the HPO axis and Causes early pregnancy symptoms or when bodily changes like weight gain and amenorrhea are misinterpreted --> nonpsychotic patient who believes she is pregnant Management: psych eval and tx
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Alloimmunization
Mom is alloimmunized / sensitized when her anti-D antibody titer is greater than 1:8. To prevent this, mom is supposed to be given anti-D IG at 28 weeks gestation and repeated within 72 hrs of delivery 50% of Rh negative women will need a higher dose than standard dose after delivery, placental abruption, procedures, etc. Kleihauer-Betke (KB) test is used to determine the dose --> RBC from mom's circulation are fixed on a slide, then exposed to an acidic solution where the adult hemoglobin lyses. Dose of anti-D IG is calculated from the % of remaining fetal hemoglobin
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``` Lung cancers: Adenocarcinoma Small cell lung cancer Squamous cell lung cancer Large cell lung cancer Pancoast tumor ```
Adenocarcinoma: 40-50% incidence. Peripheral. Associated with clubbing and hypertrophic osteoarthropathy Small cell: 10-15%. Central. SIADH, Lambert-Eaton, Cushing Squamous cell: 20-25%. Central, necrosis and cavitation. PTHrP Large cell: 5-10%. Peripheral. Associated with gynecomastia, galactorrhea Pancoast tumor = superior pulmonary sulcus tumor typical lung cancer sx but + shoulder pain due to invasion of brachial plexus and adjacent structures, horner syndrome, C8-T2 neuro involvement, supraclavicular lymph node enlargement. CXR = initial test
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Primary amenorrhea
Measure FSH If high --> karyotype analysis If low --> brain MRI
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Diabetic ophthalmoplegia
ischemic neuropathy of CN III due to poorly controlled DM CN 3 has 2 components: - inner somatic fibers (EOM's and levator muscle) - superficial parasympathetic fibers (sphincter of iris and ciliary muscles) Blood supply runs through the middle of the nerve so ischemic injury will first present with ptosis and down and out gaze with preserved pupillary response
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Secondary bacterial pneumonia
Most common complication of influenza Most occur in age >65, rare in young people with exception of community acquired MRSA CA-MRSA causes severe, necrotizing pna that is rapidly progressive and often fatal. manifestations = high fever, productive cough with hemoptysis, leukopenia, multilobar cavitary infiltrates Requires admission to ICU with broad spectrum empiric abx
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Prevalence and PPV/NPV
High prevalence --> high PPV, low NPV | Low prevalence --> low PPV, high NPV
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Compartment syndrome
Ischemia-reperfusion syndrome is a form of compartment syndrome - due to interstitial edema and possibly intracellular swelling following tissue ischemia and subsequent reperfusion
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Narcolepsy
Modafinil = first line - nonamphetamine medication that promotes wakefulness, reduces daytime sleepiness, generally well tolerated, and has less abuse potential compared with traditional stimulants
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External cephalic version
External cephalic version: Maneuvers to convert a breech presentation into a vertex presentation for delivery; done between 37 weeks gestation and the onset of labor Internal podalic version: Performed in twin delivery to convert the 2nd twin from a transverse/oblique presentation to a breech presentation for subsequent delivery
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Recurrent cystitis in children
Often caused by constipation as fecal retention can cause rectal distension which in turn compresses the bladder and prevents complete voiding --> residual urine is a potential breeding ground for bacteria that ascend to the urethra from the perineum
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Torus palatinus
benign bony growth (exostosis) located on the midline suture of the hard palate surgery is indicated for patients in whom the mass becomes symptomatic, interferes with speech or eating, or causes problems with fitting of dentures later in life
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Trendelenburg sign
Drooping of the contralateral hemipelvis below its normal horizontal level during monopedal stance due to weakness or paralysis of the gluteus medius and minimus muscles, which are innervated by the superior gluteal nerve
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Alkalotic urine
urine pH > 8 If in the setting of cystitis/pyelo, presence raises suspicion for a urease-producing bacteria such as Proteus and Klebsiella. Urease spits urea into ammonia and carbon dioxide; ammonia then converts to ammonium and alkalinizes the urine. High urine pH reduces the solubility of phosphate, raising risk for development of struvite stones (magnesium ammonia phosphate)
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Hypercalcemia | Calcium physio
aggressive saline hydration calcitonin (works quickly, can be administered with saline) bisphosphonates (effect is delayed, takes 2-4 days) --- Calcium physio: Plasma calcium exists in 3 forms: ionized (45%), albumin-bound (40%), and bound to inorganic and organic anions (15%) Ionized calcium is the physiologically active form Homeostasis of these forms is influenced by the extracellular pH level Increased extracellular pH (like in resp alkalosis) causes H ions to dissociate from albumin molecules, thereby freeing up albumin to bind with calcium --> decreased levels of ionized calcium --> can result in clinical manifestations of hypocalcemia even though total calcium is unchanged
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Rhinoplasty complications
Most common complications: patient dissatisfaction, nasal obstruction, epistaxis Nasal septum complications are less common but more serious - cartilage has poor blood supply, relies completely on the overlying mucosa for nourishment by diffusion --> poor regenerating capacity of the septal cartilage --> trauma or surgery on the septum may result in septal perforation. Typical post-op presentation of septum perforation = whistling noise heard during respiration, typically due to a septal hematoma or a septal abscess
200
Benzo withdrawal
Can appear as early as 24 hrs after cessation | Can present with seizures, tremors, anxiety, perceptual disturbances, and psychosis
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Acute angle closure glaucoma
gold standard for diagnosis = gonioscopy (specialized lens to visualize the angle) Ocular tonometry measures IOP and can provide additional info if needed or if ophthalmologic consultation is not immediately available Tx: narcotics - pain control inc IOP - reduced with mannitol, acetazolamide, timolol, pilocarpine AVOID ATROPINE (anti-cholinergic) and other mydriatics since it can dilate the pupil and worsen
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Dysthymia (persistent depressive disorder)
Dx requires persistent depressive symptoms for 2+ years and that the individual has never been without depressive symptoms for more than 2 months at a time
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Normal pressure hydrocephalus
wet, wobbly, wild --> abnormal gait, incontinence, dementia Pathogenesis: increase in ICP due to either diminished CSF absorption at the arachnoid villi or obstructive hydrocephalus --> ventricular enlargement --> pressure returns to normal
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Leprosy
Etiology: chronic granulomatous disease of the skin/peripheral nerves caused by mycobacterium leprae; primarily developing world, from respiratory droplets/armadillos Manifestations: macular, anesthetic skin lesions with raised borders. Nodular, painful nearby nerves with loss of sensory/motor function Dx: full thickness biopsy Tx: dapsone + rifampin. + clofazimine if severe
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Supplemental oxygen in pts with COPD
Can worsen hypercapnia due to combo of increased dead space perfusion causing VQ mismatch, dec affinity of oxyhemoglobin for CO2, reduced alveolar ventilation. Goal oxyhemoglobin saturation in these patients = 90-93% -- Antibiotics are indicated in acute exacerbations when: - moderate to severe COPD exacerbation (2 of the following: inc dyspnea, inc cough, sputum production change) - mechanical ventilation requirement -- Criteria for initiating long term home oxygen therapy: resting PaO2 < 55Hg or SaO2 < 88%; OR PaO2 < 59 or SaO2 < 89% in pts with corpulmonale, evidence of R heart failure, or hematocrit > 55%
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Colon polyps
Neoplastic features: adenomatous, villous features, large, high number, sessile (nonpedunculated)
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HTN complications
``` HTN urgency: Severe HTN (usually >180/120) with no sx or acute end-organ damage ``` HTN emergency: HTN urgency + end organ damage Malignant HTN - severe HTN with retinal hemorrhages, exudates, or papilledema HTN encephalopahty - severe HTN with cerebral edema and non-localizing neuro signs and sx
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Factor V Leiden
activated protein C resistance
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Zinc deficiency Copper deficiency Selenium deficiency
Zinc = trace mineral obtained from meat, nuts, and fortified cereal in the diet Mostly absorbed in the duodenum and jejunum Deficiency can be due to malabsorption (Crohn's, celiac), bowel resection, gastric bypass, poor nutritional intake, pts dependent on TPN Clinical manifestations: hypogonadism, impaired wound healing, IMPAIRED TASTE, immune dysfunction, alopecia, skin rash Copper deficiency: fragile hair, skin depigmentation, neurologic dysfunction that can mimic vitamin B12 deficiency, sideroblastic anemia Selenium deficiency: thyroid dysfunction, cardiomyopathy
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Sjogren syndrome
Keratoconjunctivitis sicca, dry mouth, salivary hypertrophy, xerosis of skin Extraglandular features: raynaud phenomenon, cutaneous vasculitis, arthralgia/arthritis, interstitial lung disease Diagnostic findings: decreased lacrimation on Echirmer test, positive anti-Ro (SSA) and anti-La (SSB), salivary gland biopsy with focal lymphocytic sialoadenitis Can be a primary syndrome or secondary to another autoimmune disease
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Lateral medullary syndrome (wallenberg syndrome)
ischemia in lateral medulla --> blockage of PICA Manifestations: Loss of pain/temp sensation on the contralateral body and ipsilateral face, CN X dysfunction/bulbar muscle weakness (dysphagia, slurred speech), vertigo/nystagmus (vertibular nuclei), and Horner syndrome (ascending sympathetic fibers)
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Myasthenia gravis
Associated with thymoma Myasthenic crisis: tx with intubation, IVIG or plasmapheresis in addition to corticosteroids
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Rotator cuff tear
Rotator cuff muscles: supraspinatus, infraspinatus, teres minor, and subscapularis ``` Supraspinatus = most commonly injured due to degeneration of the tendon with age and repeated ischemia induced by impingement between the humerus and the acromion during abduction Supraspinatus = responsible for initiating the first 15 degrees of abduction ``` Complete supraspinatus tear causes weakness of abduction which can be appreciated with drop arm test. Dx: MRI Tx: surgery
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Klumpke palsy | Erb-Duchenne palsy
Klumpke palsy: injury to the C8 and T1 cervical roots (lower trunk of brachial plexus) - associated with shoulder dystocia and LGA Affects muscles supplied by the ulnar nerve --> weakness and atrophy of the hypothenar and interosseous muscles and "claw hand" deformity --> absent grasp reflex Associated damage to the sympathetic fibers that run along C8 and T1 that manifests as ipsilateral miosis and ptosis Erb-Duchenne palsy - most common type of brahcial plexus injury; involves C5 and C6, leading to waiter's tip posture. affected arm may have dec or absent moro reflexes, but grasp reflex should remain intact
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Cryoglobulinemia Cryoglobulinemia vs cold agglutinins Autoimmune hemolytic anemias: warm agglutinin vs cold agglutinin
Cryoglobulinemia - immune complex deposition in blood vessels, leading to endothelial injury and end-organ damage Dx: serology (serum cryoglobuilns, complement); skin/renal biopsy Tx: address the underlying disease, can include plasmapheresis and immunosuppression Type I: lymphoproliferative of hematologic disease associations asymptomatic; hyperviscosity/thrombosis, livedo reticularis, purpura complement levels are normal Type II and III: associated with chronic HCV, HIV, SLE fatigue, arthralgia, renal (glomerulonephritis HTN), dyspnea, pleurisy, palpable purpura, leukocytoclastic vasculitis low C4 levels --- Cryoglobulinemia: immunoglobulin proteins that become insoluble and precipitate at reduced temps --> can block blood vessels and cause gangrene Cold agglutinins: RBC's that become insoluble and precipitate at reduced temps due to autoimmune IgM against RBC's --- Warm agglutinin AIHA etiology: drugs, viral infections, autoimmune, immunodeficiency states, lymphoproliferative Can present from asx to life threatening anemia + direct Coombs test with anti-IgG, anti-C3, or both Tx: corticosteroids, splenectomy for refractory dz Complications: VTE, lymphoproliferative disorders Cold agglutinin AIHA etiology: infections (mycoplasma, mono, etc), lymphoproliferative diseases Presentation: anemia, livedo reticularis and acral cyanosis with cold exposure that disappear with warming + direct Coombs test with anti-IgM or anti-C3 Tx: avoid cold temps, rituximab +/- fludarabine Complications: ischemia, peripheral gangrene, lymphoproliferative disorders
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Shin splints vs stress fractures
Shin splints aka medial tibial stress syndrome --> anterior leg pain; seen in casual runners; characterized by diffuse area of tenderness (not point tenderness); more common in overweight people Stress fractures: Tibial stress fractures are caused by repeated tension or compression without adequate rest. Findings: localized activity-related swelling and point tenderness. Tx = rest with exception of stress fracture of the fifth metatarsal -- surgery due to risk of avascular necrosis
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Diarrhea: Osmotic diarrhea Secretory diarrhea
Stool osmotic gap = plasma osmolality - 2 x (stool sodium + stool potassium) Osmotic: ingestion of osmotically active, poorly absorbable substances, characterized by an elevated stool osmotic gap (>125) ex) lactose intolerance Lactose intolerance: + hydrogen breath test, positive stool test for reducing substances, low stool pH and increased stool osmotic gap Secretory: large daily stool volumes (>1L/day), diarrhea that occurs even during fasting or sleep, due to bacterial infections (cholera), viral infections (rotavirus, congenital disorders of ion transport (CF), early ileocolitis, postsurgical changes (bowel resection, CCY); reduced SOB (<50)
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Erysipelas vs Cellulitis
Erysipelas: Strep pyogenes - superficial dermis and lymphatics (involvement of external ear = suggestive of erysipelas as this skin lack a lower dermis level) - raised, sharply demarcated edges - rapid spread and onset - fever early in course Cellulitis: - deep dermis and subcutaneous fat - flat edges with poor demarcation - indolent - localized (fever later in course)
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HIV-associated dementia
in patients with untreated and/or long-standing HIV and CD4 < 200 Early subcortical symptoms (basal ganglia, nigrostriatal) --> slowed movement, difficulty with smooth limb movement Followed by significant cortical neuronal loss and memory decline
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Primary vs central adrenal insufficiency | Addison's disease
Primary AI: elevated ACTH, low aldosterone, significant electrolyte effects (hyponatremia, hyperkalemia) - etiology: autoimmune, infections (tb, HIV, disseminated fungal), hemorrhagic infarction (eg meningococcemia, anticoagulants), metastatic cancer (eg lung) Central adrenal insufficency ex) secondary to chronic supraphysiologic doses of glucocorticoid use --> suppresses CRH and ACTH --> low ACTH, cortisol. Normal aldosterone. Initial test: early-morning cortisol levels Cosyntropin test = ACTH stimulation test - to differentiate central vs peripheral adrenal insufficiency
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Chancroid | LGV
``` Chancroid (painful ulcer, painful LAD) --> azithromycin LGV (painless ulcer, painful LAD) --> doxycycline Syphilis chancre (painless ulcer, painless LAD) ```
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Acute Interstitial Nephritis Acute Tubular Necrosis Chronic renal failure
AIN: + WBC casts, usually due to infection or NSAIDs or antibiotics like penicillin, cephalosporin, and TMP-SMX Other features - fever, rash, arthralgias, eosinophiluria ATN: + muddy brown casts, usually due to hypovolemia, ischemia or toxins like IV contrast and myoglobin Chronic renal failure: + broad waxy casts
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Prerenal azotemia
BUN/Cr > 20 FeNa < 1% UNa < 10 FeUrea < 35% (if pt is on diuretic and FeNa can't be measured)
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Indications for dialysis | Uremic pericarditis
``` Acidosis Electrolytes (K) Intoxication* Overload Uremia ``` * Dialyzable substances: SLIME - Salicylates - Lithium - Isopropanol - Methanol/Magnesium - Ethylene glycol (**CALCIUM OXALATE CRYSTALS IN URINE) --- Uremic pericarditis - BUN > 60; present with pleuritic chest pain and pericardial friction rub. Classic ECG finding of diffuse ST elevation is typically absent. Indication for dialysis. 50% cases are accompanied by pericardial effusion so cardiac tamponade should be ruled out prior to dialysis
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Ludwig angina
Rapidly progressive bilateral cellulitis of the submandibular and sublingual spaces, most often arising from an infected mandibular molar
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Endometrial biopsy indications
> 45 --> abnormal uterine bleeding, postmenopausal bleeding < 45 --> abnormal uterine bleeding PLUS any of the following: - unopposed estrogen exposure (obesity, chronic anovulation) - failed medical management - lynch syndrome (HNPCC) Age >35 --> atypical glandular cells on Pap (could be due to either cervical or endometrial cancer) If endometrial biopsy reveals no evidence of malignancy or hyperplasia, hormonal tx with OCP's or cyclic/continuous progestins is appropriate
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Dacryocystitis Episcleritis Hordeolum Chalazion
Dacryocystitis: Infection of the lacrimal sac; occurs in infants and adults over the age of 40 sudden onset of pain and redness in the medial canthal region +/- purulent discharge Usually Staph aureus or strep pyo - will respond to systemic antibiotic therapy Episcleritis: infection of the episcleral tissue between the conjunctiva and sclera - acute onset of mild to moderate discomfort, photophobia, and watery discharge. Diffuse or localized bulbar conjunctival injection Hordeolum: abscess located over the upper and lower eyelid, usually caused by Staph aureus. localized red, tender swelling over the eyelid Chalazion: lid discomfort. Chronic granulomatous inflammation of the meibomian gland. Appears as a hard, painless lid nodule
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Confounding vs effect modification
Confounding: alcohol use associated with bladder cancer? stratify by smoking --> in both smokers and non-smokers, there is no association between alc and bladder cancer --> smoking = confounding variable Effect modification: lung cancer associated with people exposed to asbestos stratify by smoking --> smoking augments the risk of asbestos and lung cancer
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PMS/PMDD | Premenstrual syndrome / Premenstrual dysphoric disorder
PMDD = more severe Sx severity must reach point of socioeconomic impact to qualify SSRI's= first line OCP's are another tx but do not give in patients who are contraindicated (eg migraines with aura)
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Winter's formula
Measures whether there is appropriate respiratory compensation for primary metabolic acidosis expected PaCO2 = 1.5 (serum HCO3) + 8 +/- 2 If higher than expected, this suggests an independent primary respiratory acidosis causing a mixed acid-base disorder
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Interstitial lung disease
Can be due to known causes (infections, connective tissue disease) or idiopathic (idiopathic pulmonary fibrosis) CXR shows reticular or nodular opacities CT shows fibrosis, honeycombing, bronchiectasis Causes pulmonary fibrosis -- excessive collagen deposition in the extracellular matrix around the alveoli --> scarring which reduces the TLC, FRC, and RV Alveolar fibrosis increases elastic recoil in the airways and results in a restrictive lung dz pattern on PFT's There is impaired gas exchange resulting in reduced diffusion capacity of carbon monoxide and increased alveolar-arterial gradient
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ALS
neurodegenerative disease + UMN (spasticity, bulbar sx, hyperreflexia) and LMN lesions (fasciculations, weakness, hyporeflexia) Riluzole = glutamate inhibitor
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Jejunal atresia | Ileum atresia
Thought to occur due to a vascular accident in utero that causes necrosis and resorption of the fetal intestine, sealing off and leaving behind blind proximal and distal ends of intestine RF's: maternal use of vasoconstrictive meds or drugs like cocaine and tobacco Jejunal atresia = triple bubble sign and gasless colon Tx: resuscitation/stabilization, followed by surgical correction
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Aortic dissection
suspect in pt with chest/neck pain, syncope, HTN, mediastinal widening on chest radiograph, pericardial effusion Type A (ascending aorta) dissections can lead to aortic rupture into the pericardial space and hemopericardium which can rapidly progress to cardiac tamponade and cardiogenic shock Dx: CT angio = initial study in stable patients and no renal dysfunction TEE = preferred study in unstable patients or with renal insufficiency Tx: IV beta blockers = initial therapy to lower HR and BP and reduce LV contractility
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Ureteral stone
Uncomplicated stones <1 cm can be managed conservatively with hydration, analgesics, and alpha blockers Alpha receptors are found on the distal ureter, base of the detrusor, bladder neck, and urethra. Sympathetic activation stimulates these alpha receptors to maintain high muscular tone for normal urinary continence Tamsulosin = alpha 1 antagonist; relaxes ureteral muscle and decreases intraureteral pressure --> facilitates stone passage and reduces the need for analgesics
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Hemorrhagic stroke
Basal ganglia (putamen / adjacent internal capsule) - contralateral hemiparesis and hemisensory loss, homonymous hemianopsia, conjugate gaze deviation towards side of the lesion (opposite of hemiparesis) Cerebellum - facial weakness, ataxia and nystagmus, occipital headache and neck stiffness Thalamus - contralateral hemiparesis and hemisensory loss, nonreactive miotic pupils, upgaze palsy, eyes deviate Towards hemiparesis Pons - deep coma and total paralysis, pinpoint reactive pupils
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Normal anion gap acidosis | type 4 RTA
``` Hyperalimentation Acetazolemide and other carbonic anhydrase inhibitors Renal tubular acidosis Diarrhea Ureteroenteric fistula Pancreaticoduodenal fistula ``` -- type 4 RTA - hyperkalemia. Seen in poorly controlled DM.
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Clubbing
Due to lung malignancy, CF, R to L cardiac shunts Pathophys: megakaryocytes that skip the normal route of fragmentation within the pulmonary circulation (due to circulatory disruption from tumors, chronic lung inflammation) to enter systemic circulation --> become trapped in the distal fingertips due to large size and release PDGF and VEGF which have growth-promoting properties that increase connective tissue hypertrophy and capillary permeability and vascularity, ultimately leading to clubbing Presence of clubbing should prompt a search for malignancy
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TB
biggest RF = substance abuse (tobacco and IV drug use) other RF's = immigrant, travel, incarceration, immunosuppression (CKD, DM, HIV, hematologic malignancy, chronic immunosuppressant medication) Miliary tubercolosis = hematogenous spread; can be primary or reactivation. See diffuse reticulonodular pattern (millet seed) on radiograph
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Ischemic hepatic injury
Occurs in the setting of hypotension Manifests as acute, massive increases in AST and ALT with milder increases in T bili and alk phos If pts survive inciting condition enzymes typically return to normal within a few weeks
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Peripheral artery disease
CAD risk equivalent --> statin, aspirin, smoking cessation, HTN, DM Tx: supervised exercise program = initial therapy. Pharmacologic therapy with cilostazol and percutaneous or surgical revascularization should be reserved for those with persistent sx despite adequate supervised exercise therapy ABI: <9 = abnormal .91-1.30 = normal +1.3 = suggestive of calcified and uncompressible vessels; additional vascular studies should be considered
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Live vaccines in HIV Opportunistic infections Diarrhea in AIDS
Live vaccines are contraindicated if CD4<200 ``` Opportunistic infections and ppx PCP - CD4 < 200; TMP-SMX Histo - CD4 < 150; Itraconazole Toxo - CD4 < 100; TMP-SMX MAC - CD4 < 50; Azithromycin --- ``` Diarrhea in AIDS: Cryptospordium CD4 < 180 - watery diarrhea, LOW grade fever, weight loss; stool exam with modified acid-fast stain reveals cryptosporidial oocytes. Tx: supportive and ART Microsporidium/Isosporidium CD4 < 100 - watery diarrhea, crampy abd pain, weight loss, FEVER IS RARE MAC CD4 < 50 - watery diarrhea, HIGH grade fever, weight loss CMV CD4 < 50 - frequent small volume diarrhea, hematochezia, abd pain, low grade fever, weight loss
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Subcutaneous emphysema
Can be 2' to severe coughing paroxysms where air leaks from the chest wall into the subcutaneous tissues due to the high intraalveolar pressure provoked by the cough. Pneumothorax is a possible complication; anyone with subcutaneous emphysema should have an emergent CXR to r/o pneumothorax
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Functional hypothalamic amenorrhea
suspect in athletic patient (caloric restriction, chronic illness) with secondary amenorrhea progestin challenge test (eg medroxyprogesterone acetate) can be performed to confirm low estrogen levels; presence of E causes proliferation of the endometrium with subsequent sloughing after the withdrawal of P Patients without E will have none or minimal bleeding as there is no endometrial lining to shed Patients will demonstrate signs/sx of hypoestrogenemia - infertility, breast atrophy, dec bone mineral density
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Rheumatic fever
All patients with an initial diagnosis of rheumatic fever should be treated with antibiotic therapy to eradicate group A strep regardless of the presence or absence of pharyngitis at the time of dx Pts with a hx of rheumatic fever are at high risk for recurrence and progression of rheumatic heart disease with repeated episodes of GAS pharyngitis --> all patients should receive continuous antibiotic prophylaxis to prevent recurrent GAS pharyngitis Regimen: IM pen G every 4 weeks Duration: RF w/o carditis: 5 years or until 21 years old RF with carditis but no residual heart or valvular dz by clinical or echo: 10 years or until 21 years old RF with carditis and persistent heart or valular dz: 10 years or until 40 years old
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Cor pulmonale
Right sided heart failure from pulmonary HTN COPD = most common cause of cor pulmonale Exertional sx (dyspnea, angina, syncope), loud P2, tricuspid regurg murmur, elevated JVP, peripheral edema, hepatomegaly due to hepatic congestion, possible ascites, R axis deviation/R BBB, RV hypertrophy, RA enlargement Dx: right heart catheterization = gold standard, shows elevated CVP, RV end-diastolic pressure, and mean pulmonary artery pressure without left heart disease
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Shy-Drager syndrome (multiple system atrophy)
degenerative disease characterized by: 1. Parkinsonism 2. Autonomic dysfunction (postural hypotension, abn sweating, disturbance of bowel/bladder control, etc) 3. Widespread neurological signs (cerebellar, pyramidal, lower motor neuron) Tx: Intravascular volume expansion with fludrocortisone, salts supplementation, alpha-adrenergic agonists, application of constrictive garments to the lower body NOT anti-parkinsonism drugs
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Hypertensive crisis 2/2 MAOI use
MAOIs inhibit the metabolism of monoamines epinephrine, dopamine, and serotonin Also inhibits tyramine metabolism. Tyramine = sympathomimetic monoamine. If patient taking MAOI eats foods containing tyramine - can result in a severe HTN crisis (HA, intracranial bleeding, stroke, death)
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Exercise-induced bronchoconstriction
Occurs in response to mast cell degranulation triggered by the passage of high volumes of dry, cold air Can occur in asthmatics but can occur in in non-asthmatics too Short-acting beta-adrenergic agonists should be used 10-20 min prior to exercise. 1st line therapy if used only intermittently Antileukotriene agent can be used 15-20 min prior for those unable to tolerate albuterol
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Gluconeogenesis
Main substrates to glucose: gluconeogenic amino acids (glutamine, alanine), lactate, glycerol 3-phosphate lactate and alanine --> pyruvate --> glucose glycerol-3-phosphate --> glucose glutamine --> TCA --> glucose
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Septic pelvic thrombophlebitis
RF: CS, pelvic surgery, endometritis, PID, pregnancy, malignancy Pathophys: thrombosis of the deep pelvic or ovarian veins that becomes infected Presentation: post partum patient with persistent fever that is resistant to endometritis empiric antibiotics and with negative infectious evaluation --> septic pelvic thrombophlebitis is a dx of exclusion Tx: anticoagulation, broad spectrum antibiotics
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Polyp Syndromes Gardner syndrome Turcot Syndrome Juvenile Polyposis
Gardner: colon cancer + osteomas, desmoid tumors, and other soft tissue tumors Turcot: colon cancer + CNS malignancy (turcot turban) Juvenile Polyposis: colon cancer + multiple hamartomatous polyps
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Anesthetics and side effects ``` Succinylcholine Halothane Etomidate Nitrous oxide Propofol ```
Succinylcholine = often used during rapid-sequence intubation as it has rapid onset/offset. It is a depolarizing neuromuscular blocker. Works by binding to postsynaptic Ach receptors to trigger influx of sodium ions and efflux of potassium ions through ligand-gated ion channels. Can cause life-threatening hyperkalemia in patients with a condition leading to upregulation of postsynaptic Ach receptors (eg skeletal muscle trauma, burn injury, stroke). Nondepolarizing neuromuscular blocking agents (eg vecuronium, rocuronium) should be used with these patients Halothane: can lead to acute liver failure Etomidate: inhibits 11-hydroxylase and can lead to adrenal insufficiency. Elderly and patients with critical illness like sepsis are typically susceptible. Nitrous oxide: inactivates vitamin B12 --> neurotoxicity (peripheral neuropathy) can result in patients with preexisting vitamin B12 deficiency Propofol: severe hypotension due to myocardial depression. use with extreme caution in patients with ventricular systolic dysfunction
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Flail chest
3+ contiguous ribs fractured in 2+ locations See paradoxical chest wall motion with respiration Chest pain, tachypnea, rapid shallow breaths CXR: rib fractures +/- contusion/hemothorax Management: pain control, supplemental oxygen Mechanical positive pressure ventilation corrects the paradoxical motion of the flail segmenet by replacing the normal negative intrapleural pressure with positive intrapleural pressure and forcing the segment to move outward with the rest of the rib cage during inspiration --> improves oxygenation
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Inherited long QT syndromes: Jervell-Lange-Nielsen syndrome Romano-Ward syndrome
Jervell-Lange-Nielsen syndrome: AR, FH of sudden death, congenital sensorineural deafness, QT ~600ms Due to molecular defects in K channels Romano-Ward syndrome: AD Patients with inherited long QT syndromes are at high risk of syncope, life-threatening ventricular arrhythmias such as torsades and sudden death Tx: refrain from vigorgous exercise, avoid meds that can lengthen the QT interval, maintaining normal electrolyte levels, and pharmacotherapy with beta blockers Symptomatic patients require beta blocker + long-term placemaker placement
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Lichen sclerosus
Chronic inflammatory condition of the anogenital region that can affect women at any age --> skin becomes thin white and crinkled like cigarette paper Perianal skin may be involved, resulting in a figure of 8 appearance Sx include intense pruritus, dyspareunia, dysuria, and painful defecation Lichen sclerosis is a vulvar premalignant lesion - vulvar SCC occurs with greater frequency in these patients --> vulvar punch biopsy to rule out malignancy
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Rabies
Bite from infected mammal US: bats = most common; also raccoons, skunks, and foxes Developing world: dogs Sx: encephalitic - hydrophobia and aerophobia - feeling of water or air cause pharyngeal spasm paralytic - ascending flaccid paralysis Post exposure prophylaxis: rabies IG and rabies vaccine immediately after exposure
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Sarcoidosis
noncaseating granulomatous inflammation Can affect multiple organs Heart: granuloma infiltration of the myocardium --> serious arrhythmia, cardiomyopathy, heart failure, and sudden cardiac death. can result in conduction defects (AV block), restrictive cardiomyopathy, dilated cardiomyopathy, valvular dysfunction, and heart failure Eyes: uveitis Lungs: dyspnea, syncope Hypercalcemia due to granulomatous activity of 1-alpha hydroxylase causing increased production of 1,25-dihydroxyvitamin D and inc intestinal calcium absorption. Hypercalciuria occurs too and can result in nephrolithiasis Patients with sx disease are treated with systemic glucocorticoids
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Asthma
Intermittent: SABA/sx frequency <2 days/week. <2 nighttime awakenings a month --> SABA Mild persistent: >2 SABA days a week but not daily. 3-4 nighttime awakenings a month --> + inhaled corticosteroid Moderate persistent: daily SABA use. 1+ a week but not nightly nighttime awakenings --> + LABA Severe persistent: SABA use throughout the day. 4-7 nighttime awakenings a week. --> + oral corticosteroid
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Metabolic syndrome
when 3 of the 5 following criteria are met: 1. abdominal obesity (men with waist circumference > 40 inches, women with waist circumference > 35 inches) 2. fasting glucose > 100-110 3. BP > 130/80 4. Triglycerides > 150 5. HDL (men <40; women <50) Due to insulin resistance
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Vitreous hemorrhage
Sudden loss of vision and onset of floaters Most common cause = diabetic retinopathy Exam: fundus is hard to visualize/details are obscured, also see floating debris, dark red glow Immediate ophthalmologic consultation is required
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Secondary causes of HTN
- Renal parenchymal disease - Renovascular disease - Primary aldosteronism - Pheochromocytoma (tx with alpha and beta adrenergic blockade. Alpha blockers like phenoxybenzamine should be initiated first because administration of beta blockers alone can cause unopposed alpha adrenergic effects, leading to severe peripheral vasoconstriction and a paradoxical rise in BP.) - Cushing syndrome - Hypothyroidism - Primary hyperparathyroidism - Coarctation of the aorta
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Preventative screening tests for patients at avg risk
breast cancer: women 50-75 - mammo every 2 years cervical cancer: women 21-65 - pap every 3 years colon cancer: 50-75 - FOBT yearly or colonoscopy every 10 years HIV: 15-65. HIV antibody screen 1 time (p24 antigen and antibodies) Hyperlipidemia: men 35+. Lipid panel every 5 years HTN: 18+. BP every 2 years. Osteoporosis: women 65+. DEXA.
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Vibrio vulnificus
Gram negative; free living in marine environments Infection from ingestion (oysters) or wound infection Increased risk in those with liver disease (cirrhosis, hepatitis) Rapidly progressive septicemia (septic shock, bullous lesions) and cellulitis (hemorrhagic bullae, nec fasc) Dx: blood and wound cultures Tx: empiric since this infection is highly fatal; ceftriaxone and doxycycline
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Vertigo
Meniere Disease: vertigo, sensorineural hearing loss, tinnitus. Due to increased volume and pressure of endolymph (endolymphatic hydrops), likely due to defective resorption of endolymph --> damage to the vestibular and cochlear components of the inner ear BPPV: brief episodes triggered by head movement; due to displacement of otoliths from their normal location in the utricle into the adjacent semicircular canals. Dix-Hallpike maneuver causes nystagmus. Most beneficial tx includes using certain maneuvers to reposition the displaced otoliths such as the epley maneuver Vestibular neuritis: acute single episode that lasts days, often follows viral syndrome Abnormal head-thrust test Migraine: vertigo associated with HA or aura. Sx resolve completely between episodes Brainstem/cerebellar stroke: sudden onset, persistent vertigo; usually other neurologic sx
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Breastfeeding contraindications | breastfeeding benefits
Maternal: - active, untreated TB - maternal HIV infection (in developed countries where formula is available) - herpetic breast lesions - active varicella infection - chemotherapy or radiation therapy - active substance abuse - marijuana has been associated with dec muscle tone, poor suckling, and sedation Infant: - galactosemia -- Benefits: - more rapid uterine involution and decreased postpartum bleeding - faster return to prepartum weight - improved child spacing - improved mom-infant bonding - reduced risk of breast and ovarian cancer babies: - improved immunity, GI function - prevention of infectious diseases: otitis media, gastroenteritis, respiratory illnesses, UTI - dec risk of childhood cancer, type 1 DM, NEC
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Hereditary hyperbilirubinemia syndromes
Unconjugated: defect in conjugation Gilbert's, Criggler-Najjar Conjugated: defect in hepatic secretion of conjugated bilirubin into the biliary system Rotor's, Dubin-Johnson Normal AST, ALT, alk phos See + urine bilirubin
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Marfan syndrome | Homocystinuria
Marfan syndrome: AD disorder of the fibrillin-1 gene, normal intellect, aortic root dilation --> diastolic murmur = aortic regurg --> must monitor for development of aneurysms and aortic arch dissection, upward lens dislocation Homocystinuria: AR, ID, thrombosis, downward lens dislocation, megaloblastic anemia, fair complexion Overlapping features: pectus deformity, tall, arachnodactyly, joint hyperlaxity, skin hyperelasticity, scoliosis
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Use dependence in antiarrhythmics
``` Use dependence = enhanced pharmacologic effects of a drug during faster heart rates Seen with class I (especially 1C) and class IV (CCB) antiarrhythmics. Class 1C (flecainide, propafenone) cause a progressive decrease in impulse conduction with faster heart rates, leading to an increase in the QRS complex duration - this is because they have the slowest rate of drug binding and dissociation from the sodium channel receptor and in pts with faster heart rates, the drug has less time to dissociate from the sodium channels --> higher number of blocked channels --> progressive decrease in impulse conduction --> widening of the QRS ```
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Loop diuretics
Side effects: hypokalemia, metabolic alkalosis, prerenal kidney injury, hypomagnesemia Hypokalemia and metabolic alkalosis: increase in Na in the urine --> inc in K and H in the urine Prerenal kidney injury: due to volume contraction from diuresis --> inc in aldosterone --> further loss of H in the urine Hypokalemia and hypomagnesemia can lead to v tach Hypokalemia potentiates side effects of digoxin which toxicity can lead to v tach as well
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Post-MI complications
RV failure - acute - RCA artery - hypotension, clear lungs, Kussmaul sign - echo: hypokinetic RV Papillary muscle rupture - acute within 3-5 days - RCA artery - acute severe pulmonary edema, new holosystolic murmur - echo: severe mitral regurg with flail leaflet Interventricular septum rupture/defect - acute within 3-5 days - LAD, RCA - shock and chest pain, new holosystolic murmur, biventricular failure - echo: L to R shunt in ventricle, step up in oxygen between RA and RV Free wall rupture - within first 5d to 2w - LAD - shock and chest pain, JVD, distant heart sounds - echo: pericardial effusion with tamponade
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Mediastinal tumors
Anterior - thymoma, retrosternal thyroid, lymphoma 4 T's for anterior mediastinal mass differential: thymoma, teratoma + other germ cell tumors, thyroid neoplasm, and terrible lymphoma Middle - bronchogenic cyst, tracheal tumor, pericardial cyst, lymphoma, lymph node enlargement, aortic aneurysm Posterior - neurogenic tumors including meningocele, enteric cysts, lymphomas, diaphragmatic hernias, esophageal tumors, aortic aneurysm
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Asymptomatic bacteriuria
Growth of >100,000 colony-forming units of a single type of bacteria Always treated in pregnant patients on initial prenatal visit Most common pathogen = E. coli. 1st line antibiotics include cephalexin, amoxicillin-clavulanate, nitrofurantoin In pregnancy, increased progesterone levels cause smooth muscle relaxation and ureteral dilation --> increased risk for ascending infection Associated with an increased risk of preterm labor and low birth weight
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Latent/active phases of labor | Disorders of the active phase of labor
1st stage of labor begins with onset of regular contraction, ends when pt is 10 cm dilated Latent phase = until 6cm dilation Active = 6-10 cm -- Disorders of the active phase of labor: Protraction: cervical change that is slower than expected +/- inadequate contractions. Tx = oxytocin Arrest: no cervical change for 4+ hours with adequate contractions OR no cervical change for 6+ hours with inadequate contractions. Tx = CS Adequate contractions = 200MVU in 10 min / forceful contractions every 2-3 min
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Steppage gait
Footdrop - due to weakness in foot dorsiflexion Most commonly caused by L5 radiculopathy (weak foot inversion and plantar flexion) or neuropathy of the common peroneal nerve (compression of the nerve at the lateral aspect of the fibula, associated with paresthesias and sensory loss over the dorsum of the foot)
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Osgood-Schlatter disease
traction apophysitis of the tibial tubercle (quadriceps tendon puts traction on the apophysis of the tibial tubercle where the patellar tendon inserts) PE: edema and tenderness over the tibial tubercle. Firm mass can sometimes be felt due to heterotopic bone formation. Pain can be reproduced by extending the knee against resistance. X ray: nonspecific, can include anterior soft tissue swelling, lifting of tubercle from the shaft, irregularity or fragmentation of the tubercle Tx: activity restriction, stretching exercises, NSAIDs
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Clubfoot
Equinus and varus of the calcaneum and talus, varus of the midfoot, and adduction of the forefoot Tx: immediate stretching, manipulation, and casting
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Venous ulcers
Usually medial
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Cryptorchidism
failure of testicular descent from the abdomen into the scrotum Testicles that have not descended by 6 months are unlikely to descend spontaneously and require surgery Almost all patients have an associated patent processus vaginalis that is repaired concurrently to prevent inguinal hernia Orchiopexy = optimally performed before 1 year of age Removes risk of testicular torsion Can improve risk of infertility but sperm count and quality remain substandard Decreases but does not eliminate risk for testicular cancer
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Maternal adaptations in pregnancy
Cardiac: increased cardiac output and plasma volume decreased systemic vascular resistance Respiratory: hyperventilation (progesterone directly stimulates the respiratory centers) --> increased tidal volume, minute ventilation --> chronic respiratory alkalosis which lowers the PaCO2 to 27-32 mmHg decreased functional residual capacity (elevation of diaphragm) Renal: inc renal blood flow, GFR, and basement membrane permeability --> dec BUN, creatinine; inc renal protein excretion (>300 mg is considered abnormal vs >150 mg in nonpregnan patients heme: hypercoagulable state: dec protein S activity, inc in fibrinogen, inc in resistance to activated protein C mildly decreased platelet counts (ingestational thrombocytopenia)
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Ovarian cancers | Epithelial ovarian carcinoma
Epithelial ovarian carcinoma = malignancy involving the ovary, fallopian tube, and peritoneum - due to abnormal proliferation of ovarian or tubal epithelium or peritoneum Presentation: dyspnea, obstipation/constipation with vomiting, abdominal distension, pelvic/abdominal pain, bloating, early satiety, asymptomatic adnexal mass Lab findings: increased CA-125 US findings (confirmation): solid mass, thick septations, ascites Management: ex lap Specificity of CA-125 levels are much greater in postmenopausal women - any ovarian mass in a postmenopausal patient requires investigation
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Preeclampsia HELLP syndrome Intrahepatic cholestasis of pregnancy Acute fatty liver of pregnancy
Preeclampsia: New-onset HTN (SBP>140 and/or DBP >90) at > 20 weeks gestation + Protein level - > 0.3g/24hr urine specimen, 0.3 protein/cr ratio, +1 Severe features: SBP >160, DBP>110, thrombocytopenia, inc creatinine, inc transaminases, pulmonary edema, visual or cerebral sx Management: without severe features delivery at >37 weeks. with severe features delivery at >34 weeks. mag sulfate (seizure prophylaxis). antihypertensives (hydralazine, labetalol, nifedipine)s HELLP: Variation of severe preeclampsia - preeclampsia, nausea/vomiting, RUQ abd pain Lab findings: MAHA, elevated LFT's, low platelets Tx: delivery, magnesium for seizure ppx, antihypertensive drugs pulmonary edema = rare complication These processes can cause liver swelling with distension of the hepatic capsule (Glisson's capsule), resulting in RUQ or epigastric pain Intrahepatic cholestasis of pregnancy: functional disorder of bile formation that develops in the 2nd and 3rd trimesters Clinical manifestations: intense, intolerable generalized pruritus that is especially significant on the palms and soles and worsens at night. Serum total bile acids are increased. LFT's may be suggestive of cholestasis. Tx: ursodeoxycholic acid = symptom relief; pruritis resolves in teh weeks following delivery Acute fatty liver of pregnancy: rare, serious disease that presents in the 3rd trimester. Malaise, abd pain, n/v, ultimately liver failure and extrahepatic complications such as ascites, jaundice, hypoglycemia, encephalopathy, severe coagulopathy, and AKI. Lab studies suggestive of liver failure.
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Hemolytic uremic syndrome (HUS)
Enterocolitis from Shiga toxin-producing bacteria, most common being E coli O150:H7, Shigella) Presentation: bloody diarrhea, lethargy/irritability, bruising/petechiae, oliguria, edema Lab findings: hemolytic anemia, thrombocytopenia, inc in creatinine, hematuria, proteinuria, casts, inc in bilirubin Tx: fluid and electrolyte management, blood transfusions, dialysis
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CAH | congenital adrenal hyperplasia
Enzyme deficiency, hormonal abnormalities, symptoms 21-hydroxylase deficiency: dec cortisol and aldosterone, inc testosterone and 17--hydroxyprogesterone Sx - ambiguous genitalia in girls, salt wasting Inc in 17-hydroxyprogesterone 11-hydroxylase deficiency: dec cortisol and aldosterone, inc testosterone and 11-deoxycorticosteone (weak mineralocorticoid) and 11-deoxycortisol Sx - ambiguous genitalia in girls, fluid and salt retention, HTN 17-hydroxylase deficiency: dec cortisol and testosterone, inc mineralocorticoids and corticosterone (weak glucocorticoid) Sx - all patients phenotypically female; fluid and salt retention, HTN
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Pruritic urticarial papules and plaques of pregnancy (PUPPP)
Skin condition that develops in the 3rd trimester Classic finding: red papules within striae with sparing around the umbilicus, sometimes extending to the extremities. Palms, soles, face rarely involved. No lab or liver abnormalities associated.
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Chronic prostatitis/chronic pelvic pain syndrome
Chronic pelvic pain (including perineum and testes) for 3+ months without an identifiable cause, can present as voiding difficulties, irritative voiding sx (frequency, urgency, hesitancy), pain with ejaculation, or blood in the semen. Patients generally afebrile and have little or no prostate tenderness; normal UA and negative urine culture results. Diagnosis of exclusion Although bacterial cause is not know, antibiotics are helpful in symptom relief. Other interventions include alpha-adrenergic inhibitors like tamsulosin and 5-alpha-reductase inhibitors like finasteride.
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Gonococcal conjunctivitis | Chlamydia conjunctivitis
Gonococcal: 2-5 days. Copious exudate and eyelid swelling. Tx with 1 dose of IM ceftriaxone or cefotaxime. Prevent with Erythromycin at birth Chlamydia: 5-14 days. Milder chemosis and eyelid swelling and watery discharge than gonococcal.
288
Hyponatremia in CHF
Parallels the severity of heart failure; an independent predictor of adverse clinical outcomes. Caused by increased levels of renin, norepinephrine, and ADH. Tx involves fluid restriction, ACE inhibitors, and loop diuretics.
289
Sympathetic ophthalmia
"Spared eye injury" Immune-mediated inflammation of one eye (the sympathetic eye) after a penetrating injury to the other eye Manifestations: anterior uveitis, panuveitis, papillary edema, blindness Pathophysiological mechanism is believed to be the uncovering of "hidden" antigens
290
Atrial fibrillation Atrial flutter Atrioventricular nodal reentry tachycardia Atrioventricular reentrant tachycardia
A fib - ectopic foci within the pulmonary veins A flutter - reentrant circuit around the tricuspid annulus Atrioventricular nodal reentry tachycardia - reentrant circuit formed by 2 separate conducting pathways within the AV node Atrioventricular reentrant tachycardia - reentrant circuit involving an accessory atrioventricular bypass tract
291
Aphasia: Broca Wernicke Conduction
Broca - comprehension preserved; speech and repetition impaired; can see associated R hemiparesis (face and upper limb) Wernicke - speech preserved but lacks meaning; comprehension and repetition impaired; can see R superior visual field defect Conduction - speech and comprehension preserved; repetition very poor
292
Juvenile myoclonic epilepsy (JME) | Lennox-Gastaut syndrome
JME: generalized seizures during adolescence, most commonly myoclonic jerks most prominent in the first hour after awakening. Absence seizures and GTC seizures may also be seen. Lennox-Gastaut syndrome: presents by age 5 with ID and severe seizures of varying types. EEG demonstrates slow spike-wave pattern.
293
Meniscal tears
etiology: younger patients - rotational force on planted foot older patients - degeneration of meniscal cartilage Sx: acute popping sensation, catching locking and reduced ROM, slow-onset joint effusion Exam: joint line tenderness pain or catching in provocative tests (mcmurray, thessaly - pt stand on affected leg with knee flexed 20 degrees with + pain/catching/palpable crepitus on internal/external rotation of the knee Dx: MRI, arthroscopy
294
Septic abortion
Retained products from elective abortion with nonsterile technique or missed/incomplete abortion 9rare) Sx: fever, abd pain, purulent discharge, boddy tender uterus, dilated cervix Pelvic US: retained POC, thick endometrial stripe Tx: IVF, broad-spectrum antibiotics, suction curttage
295
Popliteal cyst (Baker cyst)
Asymptomatic bulge behind the knee that diminishes with flexion. Can cause posterior knee pain, swelling, stiffness Complications: venous compression (swelling), dissection into calf, rupture Rupture: can cause posterior knee and calf pain, with tenderness and swelling of the calf resembling DVT Arc of ecchymosis is visible distal to the medial malleolus (crescent sign) US to rule out DVT and confirm popliteal cyst
296
Heparin-induced thrombocytopenia (HIT)
Type 1 HIT: nonimmune direct effect of heparin on platelet activation; presents within the first 2 days of heparin exposure; platelet count then normalizes with continued heparin therapy and there are no clinical consequences Type 2 HIT: antibodies to platelet factor 4 (PF4) complexed with heparin --> platelet aggregation, thrombocytopenia, and thrombosis (both arterial and venous) - usually presents 5-10 days after heparin initiation. Elevated aPTT is suggestive of unfractionated heparin use. Enoxaparin does not prolong aPTT and the risk of HIT is lower with enoxaparin than with unfractionated heparin therapy If HIT is suspected, all heparin products should be discontinused immediately and anticoagulation should be initiated with a non-heparin medication (eg argatroban, fondaparinux)
297
vesicoureteral reflux | chronic pyelo
see blunted calyces and polar scars VUR = most common pediatric urologic problem and is present in ~30-45% of children with UTI's. It is a risk factor for recurrent UTI's, which in turn place a patient at significant risk for renal scarring. Dx: voiding cystourethrogram Children with first UTI at age 2-24 months should get renal and bladder US. VCUG usually not recommended unless there is recurrent UTI
298
Lactation suppression
wearing a comfortable, supportive bra, avoidance of nipple stimulation and manipulation, application of ice packs to the breasts, and NSAIDs to reduce inflammation and pain breast binding is not recommended for lactation suppression due to the risk of mastitis, plugged ducts, and increased pain oral dopamine agonist therapy would suppress lactation but is no longer approved by the FDA due to side effects
299
Preterm labor and management
34-37 weeks: +/- betamethasone, penicillin if GBS pos or unknown 32-34 weeks: betamethasone, tocolytics (indomethacin, nifedipine), penicillin if GBS pos or unknown <32 weeks: betamethasone, tocolytics, magnesium sulfate (fetal neuroprotection), penicillin if GBS positive or unknown
300
Pronator drift
Indicative of UMN or pyramidal/corticospinal tract disease.
301
Breast milk
ideal form of nutrition for term infants | major protein source is whey, which is more easily digested than casein and helps to improve gastric emptying.
302
CJD
rapidly progressive dementia | myoclonus and sharp, triphasic, synchronous discharges on EEG
303
Syringomyelia
Fluid-filled cavity in the spinal cord that may represent dilation of the central canal or a separate cavity within the spinal parenchyma, usually located within the cervical and thoracic spine but can also involve the brainstem Most commonly associated with Arnold Chiara malformation type 1 Presentation: loss of pain/temp in cape distribution, due to disturbance of the crossing spinothalamic tracts in the anterior commissure. Vibration/proprioception is preserved as the dorsal spinal column is not usually affected As cavity enlarges there can be interruption of the anterior horn gray matter, resulting in LMN signs in the upper limbs (areflexic weakness)
304
Herpangina vs HSV-1
herpangina: coxsackie A virus; age 3-10; summer/early fall features - fever, pharyngitis, gray vesicles/ulcers on the posterior oropharynx tx - supportive HSV-1: 6 mo-5 years; no seasonality features - fever, pharyngitis, erythematous gingiva, clusters of small vesicles on anterior oropharynx
305
Constrictive pericarditis
Etiology: idiopathic, viral, cardiac surgery, radiation therapy, TB Presentation: fatigue, dyspnea, peripheral edema, ascites, inc JVP, pericardial knock, pulsus paradoxus, Kussmaul's sign Dx: pericardial thickening/calcification, JVP tracing shows prominent x & y descents
306
Emergency contraception
Copper IUD - 99% effective; up to 5d after intercourse; copper causes inflammatory reaction that is toxic to sperm and ova and impairs implanatation Ulipristal pill - >85% effective; up to 5 d after intercourse; antiprogestin that delays ovulation Levonorgestrel pill - 85% effective; up to 3d after intercourse; progestin, delays ovulation OCP's - 75% effective; up to 3d after intercourse; progestin, delays ovulation
307
HACEK
``` Haemophilus aphrophilus Aggregatibacter actinomycetemcomitans Cardiobacterium hominis Eikenella corrodens Kingella Kinae ``` Responsible for 3% of infectious endocarditis E corrodens = gram neg anaerobe, constituent of normal human oral flora See in the setting of poor dentition and/or periodontal infection, along with dental procedures that involve manipulation of the gingival or oral mucosa
308
Sphincter of oddi dysfunction
can develop following any inflammatory process (eg surgery, pancreatitis). Encompasses 2 separate physiologic entities: dyskinesia and stenosis of the sphincter It causes a functional biliary disorder --> episodic recurrent RUQ pain with aminotransferanse and alk phos elevations Opioids cause sphincter contraction and precipitate sx Manometry = gold standard for diagnosis; sphincterotomy = tx of choice
309
Foreign body aspiration | Foreign body ingestion
Aspiration --> bronchoscopy | Ingestion --> endoscopy
310
Transferrin
Serum iron/TIBC In IDA, iron is low and TIBC is high --> transferrin is low
311
Bartholin duct cyst | Gartner duct cyst
bartholin: soft mobile cystic mass found at the 4 or 8 o clock position at the base of the labium majus. If asymptomatic, do nothing. If symptomatic, I&D. Gartner: due to incomplete regression of the wolffian duct during fetal development. These cysts may be single or multiple and are submucosal along the lateral (parallel) aspects of the upper anterior vagina.
312
Flow-volume loops
Obstructive lung disease = scooped out pattern Restrictive lung disease = everything gets smaller Fixed upper-airway obstruction = flattening of the top and bottom of the flow-volume loop
313
IDA Anemia of chronic disease Sideroblastic anemia
IDA: RDW IS HIGH TRANSFERRIN (IRON/TIBC) IS LOW --- Anemia of chronic disease: decreased TIBC --- Sideroblastic anemia: inc serum iron levels, normal TIBC
314
Primary dysmenorrhea
Cramping during the first few days of menses | Caused by uterine contractions triggered by prostaglandin release from the sloughing endometrium
315
Aspiration pneumonitis vs aspiration pneumonia
Aspiration pneumonitis: acute lung injury due to aspiration of acidic and sterile stomach contents (ex. ppl who vomit during intubation/anesthesia); gastric acid induces a chemical burn and consequent inflammatory response, usually within HOURS of the aspiration event. Bilateral infiltrates can sometimes be seen on CXR. Sx resolve rapidly within 2 days with supportive management Aspiration pneumonia: usually occurs in individuals with impaired level of consciousness (eg following overdose, seizure, or anesthetic use); caused by inhalation of pathogenic bacteria that colonize oropharyngeal secretions. Typical presentation would be that of an institutionalized elderly patient who has dysphagia and develops fever and cough DAYS following an aspiration event. Tx requires antibiotics (amoxicillin/clavulanate, ampicillin/sulbactam, clinda). In supine patients, the posterior segments of the upper lobes and superior segments of the lower lobes are most commonly affected In erect patients, the bases of the lower lobes and the right middle lobe are most affected.
316
Fibromuscular dysplasia
systemic noninflammatory nonatherosclerotic disease that typically affects the renal and internal carotid arteries and vertebral arteries --> arterial stenosis, aneurysm, and dissection 90% cases occur in women Internal carotid artery stenosis --> recurrent HA, pulsatile tinnitus, TIA, stroke Renal artery stenosis - secondary HTN, flank pain Exam: subauricular systolic bruit, abdominal bruit Diagnosis: imaging (US, CTA, MRA); catheter-based arteriography Tx: antihypertensives, PTA, surgery
317
Nonalcoholic fatty liver disease (NAFLD, NASH)
hepatic steatosis on imaging or biopsy - looks like alcohol liver disease but minimal alcohol use mostly asymptomatic associated with metabolic syndrome and insulin resistance severity can range from bland steatosis to steatohepatitis to fibrosis and cirrhosis
318
Chemo drugs and toxicity/side effects
Cyclosporine: calcineurin inhibitor Side effects: nephrotoxicity, hyperkalemia, HTN, gum hypertrophy, hirsutism, and tremor Tacrolimus: calcineurin inhibitor Side effects: nephrotoxicity, hyperkalemia, HTN, tremor NOT hirsutism or gum hypertrophy Azathioprine: purine analog enzymatically converted to 6-MP (inhibits purine synthesis) Side effects: dose-related diarrhea, leukopenia, and hepatotoxicity Mycophenolate: inhibits inosine monophosphate-dehydrogenase, the rate limiting enzyme in de novo purine synthesis Side effects: bone marrow suppression
319
HIV in pregnancy
Antepartum: - initiation/continuation of HAART - viral load monthly until undetectable, then every 3 months - CD4 count every 3 months - avoidance of amniocentesis if viral load is detectable Intrapartum: - avoidance of artificial ROM - viral load < 1000 ---> continuation of HAART and vaginal delivery - viral load > 1000 ---> zidovudine and CS Postpartum: - mom: continuation of HAART - infant: zidovudine for 6+ weeks + serial HIV testing *there is no such thing as HIV IG
320
Posterior urethral valves
MCC of urinary tract obstruction in newborn boys Abnormal folds in the distal prostatic urethra obstruct urinary flow, resulting in progressive dilation of the bladder, ureters, and kidneys US: bladder distention, b/l hydroureters, b/l hydronephrosis Poor urine output in utero results in oligohydramnios as fetal urine is a major source of amniotic fluid
321
Autosomal dominant polycystic kidney disease (ADPKD)
HTN, palpable b/l abdominal masses, microhematuria Extra-renal complications: - hepatic cysts (most common) - intracranial berry aneurysm - valvular heart disease (most often MVP and AR) - colonic diverticula - abd wall and inguinal hernia
322
Disseminated gonorrhea
Triad of polyarthralgia, tenosynovitis, and painless vesiculopustular skin lesions
323
Graft versus host disease
Common complication after bone marrow transplant Due to recognition of host major and minor HLA-antigens by donor T-cells and consequent cell-mediated immune response Target organs for GVHD are the skin (maculopapular rash involving the palms, soles, and face that may generalize), intestine (blood-positive diarrhea), and the liver (abnormal liver function tests and jaundice)
324
Ileus
most commonly due to abd surgery but can also be seen in other conditions like retroperitoneal/abdominal hemorrhage or inflammation, intestinal ischemia, and electrolyte abnormalities sx: n, v, abd distention, failure to pass flatus or stool, hypoactive or absent bowel sounds dx: clinical; abd x rays may reveal dilated gas-filled loops of bowel with no transition point Management: conservative, bowel rest, supportive, tx of secondary causes
325
Volvulus
Adults - most common sites are the cecum/proximal ascending colon and the sigmoid colon Neonate - midgut volvulus; suspect in any neonate with bilious vomiting; classic finding on upper GI series is a corkscrew-shaped duodenum that is abnormally located in the R abdomen
326
Postcholecystectomy syndrome
persistent abd pain or dyspepsia that occurs either postop or months to years after a cholecystectomy Can be due to biliary or extrabiliary causes Lab findings: elevated alk phos, abnormal serum aminotransferases, dilated CBD on abd ultrasound Endoscopic US, ERCP, or MRCP to follow for final diagnosis and guiding therapy
327
Digoxin toxicity
Atrial tachycardia with AV block is the arrhythmia most specific for toxicity - digoxin toxicity causes increased ectopy and increased vagal tone GI sx (anorexia, n, v, abd pain), neuro sx (lethargy, fatigue, confusion), visual (changes in color vision, scotomas, blindness) Amiodarone, verapamil, quinidine, and propafenone increase the serum levels of digoxin - decrease the dose by 25-50% when initiating amiodarone therapy with close monitoring of digoxin levels once weekly
328
Constitutional growth delay
MCC of short stature and pubertal delay in adolescents Individuals have normal birth weight and height but between 6m to 3 years of age, the height growth velocity slows and the child drops percentiles on their growth curve. Puberty and adolescent growth spurt are delayed but eventually occur. Child will have a normal growth spurt and reach a normal adult height. Bone age radiographs show a bone age that is delayed compared to the chronological age.
329
Rheumatoid arthritis
Increased levels of proinflammatory cytokines, corticosteroid therapy, and lack of physical activity may contribute to local or generalized loss of bone mass --> inc risk of osteopenia, osteoporosis, and bone fractures Management includes adequate physical activity, optimization of calcium and vitamin D intake, minimization of corticosteroid therapy, and consideration for bisphosphonate tx
330
Neuroimaging in diseases
Schizophrenia: loss of cortical tissue volume, enlargement of cerebral ventricles (lateral ventricular enlargement = most common), decreased volume of the hippocampus/amygdala Huntington disease: atrophy of the caudate Autism: accelerated head growth during infancy, inc total brain volume OCD: structural abnormalities in the orbitofrontal cortex and basal ganglia
331
Dermatitis herpetiformis
Intensely pruritic erythematous papules, vesicles, and bullae that occur symmetrically in grouped clusters on the extensor surfaces of the elbows, knees, back, and buttocks Associated with celiac disease Skin biopsy in DH shows subepidermal microabscesses at the tips of the dermal papillae; immunofluorescence studies show deposits of anti-epidermal transglutaminase IgA in the dermis Initial tx: dapsone Long term tx: gluten-free diet
332
Proteinuria in children
Can be transient, orthostatic, or persistent Transient proteinuria = most common cause; should be reevaluated with a repeat urine dipstick test on 2 separate occasions to rule out persistent proteinuria, which requires further evaluation for underlying renal disease Orthostatic proteinuria: very common in adolescent boys; inc protein when the patient is in an upright position that returns to normal when the patient is recumbent
333
DVT
most frequent source of PE lower extremity DVT is divided into 2 categories: - proximal/thigh (iliac, femoral, popliteal) - source of 90% of acute PE's, probaby due to their large caliber and proximity to the lungs - distal/calf - less likely to embolize and more likely to spontaneously resolve
334
De Quervain tenosynovitis
clasically affects new moms who hold their infants with the thumb outstretched (abducted/extended) Caused by inflammation of the abductor pollicis longus and extensor pollicis brevis tendons Finkelstein test is +
335
Restless legs syndrome
associated with IDA -- iron supplementation | pharmacotherapy with dopamine agonists (pramipexole) or alpha-2-delta calcium channel ligands (gabapentin)
336
Rheumatic heart disease | Infective endocarditis
Rheumatic heart disease - mitral stenosis Infective endocarditis - mitral valve prolapse/mitral regurgitation perivalvular abscess is seen in approx 30-40% of patients with infective endocarditis; the development of a new conduction abnormality in patients with IE should raise suspicion for a perivalvular abscess extending into the adjacent cardiac conduction pathways
337
Bupropion
Norepinephrine and dopamine reuptake inhibitor used as a first-line treatment for major depression Preferred by patients because it does not cause weight gain or sexual dysfunction Seizures = potential adverse effects Contraindications to bupropion include seizure disorders, bulimia nervosa, anorexia nervosa, and use of MAOIs in the past 2 weeks
338
SCFE
Exam: loss of abduction and internal rotation of the hip as well as external rotation of the thigh while the hip is being flexed Dx: frog-leg lateral-view x-ray of the hip Tx: surgical pinning of the slipped epiphysis in order to dec the risks of avascular necrosis of the femoral head and chondrolysis
339
Reactive arthritis
a type of seronegative spondyloarthropathy urethritis, asymmetric oligoarthritis, conjunctivitis tx: NSAIDs
340
Stridor
Acute: croup vs foreign body aspiration Chronic: Laryngomalacia vs vascular ring Laryngomalacia: inspiratory stridor, worse when supine, improves when prone Vascular ring: inspiratory and expiratory stridor that improves with neck extension; ring can encircle the trachea and/or esophagus and present with resp and esophageal symptoms
341
Allergic rhinitis
Typical allergy symptoms May also have nasal creases (allergic salute), pharyngeal cobblestoning, conjunctival edema, or thick green nasal discharge Tx: intranasal corticosteroid
342
Beta-2 agonists
like Albuterol reduce serum potassium levels by driving potassium into cells Clinically significant hypokalemia can result, causing muscle weakness, arrhythmias, and EKG changes
343
TCA overdose
CNS depression, hypotension, anticholinergic effects like dilated pupils, hyperthermia, and intestinal ileus. QRS prolongation on EKG, leaving patient susceptible to ventricular arrhythmias. Patients suspected of TCA overdose should first undergo the ABC's and sodium bicarbonate should then be administered to improve BP, shorten the QRS interval, and prevent arrhythmia
344
Protein gap
Elevated protein gap = total protein - albumin > 4 Indicatese elevated nonalbumin protein in the serum; can be seen with polyclonal gammopathies, excessive monoclonal protein In multiple myeloma, initial test = SPEP (m-spike). confirm with bone marrow biopsy (>10% clonal plasma cells)
345
Reactive NST
- baseline of 110-160 - moderate variability (6-25/min) - 2+ accelerations in 20 minutes, each peaking >15/min above baseline and lasting >15 seconds
346
Spontaneous bacterial peritonitis SBP
Diagnosis from ascitic fluid: PMNs > 250 Positive culture, often with gram neg organisms Protein <1 g/dL SAAG > 1.1 g/dL Tx: empiric antibiotics (3rd gen cephalosporins) and fluoroquinolones for ppx Enteric bacteria are thought to translocate across the intestinal wall and seed asciti fluid within the peritoneal cavity
347
complications of gastric bypass surgery
Early complications: - anastomotic leak --> sepsis - bowel ischemia --> diffuse abdominal pain Late complications: - anastomotic stricture --> dysphagia, bowel obstruction - marginal ulcer --> abd pain, bleeding, perianastomotic perf - cholecystitis --> RUQ pain (rapid weight loss promotes gallstone formation) - dumping syndrome --> diarrhea, crampy abd pain, vasomotor sx)
348
Secondary hyperparathyroidism
CKD --> dec production of calcitriol (1,24 dihydroxyvitamin D) leads to decreased intestinal absorption of calcium. Also as GFR decreases, the kidney cannot adequately excrete phosphate --> hyperphosphatemia Hypocalcemia and hyperphosphatemia stimulate PTH release. Overtime this results in parathyroid hyperplasia and secondary hyperparathyroidism. Effects on bone: renal osteodystrophy (pain, inc risk for fracture) Chronic parathyroid stimulation may also lead to the development of autonomously functioning adenomas (tertiary hyperparathyroidism) with very high PTH levels and hypercalcemia Management: low-phosphate diet and/or phosphate binders (eg calcium carbonate, calcium acetate, sevelamer) and supplementation of calcium and vitamin D
349
Hepatic encephalopathy
Precipitating factors: - drugs - hypovolemia - electrolyte changes - inc nitrogen load (eg GI bleed) - infection - portosystemic shunting (TIPS) Presentation: sleep pattern changes, AMS, ataxia, asterixis Tx: correct precipitating cause. decrease blood ammonia concentration with lactulose and rifaximin
350
Astrocytoma
most common brain tumor in adults usually primary prognosis is most affected by tumor grade
351
Pancreatic cancer
Advanced cancer can cause jaundice and pruritus by obstructing the common bile duct (extrahepatic cholestasis) Treatment is usually palliative with endoscopic common bile duct stent placement to relieve the obstruction in most patients
352
Meckel diverticulum
painless GI bleeding in children diagnostic test: meckel scan aka 99mTc-pertechnetate scan, which shows increased uptake if there is ectopic gastric mucosa present tx: surgical
353
DIC
most common coagulopathy in patients with malignancies Can be seen in gastric, breast, and lung cancer Labs: thrombocytopenia, decreased fibrinogen, increased INR, anemia due to MAHA
354
Primary renal causes of nephrotic syndrome
FSGS - AA/Hispanics, obesity, HIV, heroin Membranous nephropathy - adenocarcinoma, NSAIDs, hep B, SLE Membranoproliferative glomerulonephritis - hep B and C, lipodystrophy Minimal change disease - NSAIDs, lymphoma IgA nephropathy - URI Nephrotic syndrome is complicated by hypercoagulation - renal vein thrombosis is the most common manifestation, but arterial thrombosis and PE may also occur
355
Gout drugs
Colchicine - used for short term therapy to prevent gouty attacks while patients start urate lowering drugs like allopurinol Long term colchicine use can cause neuropathy or myopathy which may occur more often in pts taking statins Febuxostat and allopurinol - xanthine oxidase inhibitors that decrease uric acid production; allopurinol is preferred Probenecid - uricosuric med that increases uric acid excretion in the kidneys
356
Gaze palsies
Parinaud's syndrome aka dorsal midbrain syndrome: vertical gaze palsy due to impingement on superior colliculus, upper eyelid retraction, pupils not reactive to light but reactive to accommodation can be from pinealoma or obstructive hydrocephalus Progressive supranuclear palsy: supranuclear palsy, severe dysphagia and dysarthria Olivopontocerebellar atrophy: cerebellar fingins + parkinsonism
357
Spondylosis Spondylolisthesis Spondylolysis
Spondylosis - degenerative arthritis of the spine Spondylolisthesis - anterior slippage of the superior over inferior vertebrae Spondylolysis - stress fracture of the vertebral arch
358
Myelodysplastic syndrome
Hematopoietic stem cell neoplasm that interferes with the production of functional blood cell lines --> dysplasia and cytopenias Patients can be asymptomatic, have sx of anemia, or granulocytopenia Lab findings: normocytic or macrocytic anemia with insufficient reticulocytosis Leukopenia with immature granulocytes and thrombocytopenia also common Peripheral blood smear notable for dysplastic erythrocytes (eg ovalomacrocytosis) and granulocytes (hyposegmention, hypogranulation) Bone marrow biopsy is required for diagnosis and usually shows a hypercellular marrow Tx: transfusions for symptomatic cytopenias, chemotherapy, hematopoietic stem cell transplant
359
HAART side effects
Efavirenz - NNRTI; can cause significant CNS and psych side effects (dizziness, insomnia, vivid or bizarre dreams, depression, anxiety, confused thinking, and aggression); use cautiously in pts with hx of psychiatric illness Abacavir, Lamivudine = NRTI's; associated with lactic acidosis, hepatotoxicity, insomnia, depression Abacavir is associated with a potentially fatal hypersensitivity reaction if you have a certain gene Dolutegravir - integrase inhibitor;associated with hyperglycemia, inc transaminases, insomnia, depression
360
Migraine therapies
Abortives: triptans, NSAIDs, acetaminophen, antiemetics (metoclopramide, prochlorperazine), ergots (dihydroergotamine) *Both triptans and ergots are serotonin agonists - combo can cause severe prolonged vasoconstriction due to overaction of serotonin receptors --> elevated BP, MI, stroke Preventatives: topiramate, divalproex sodium, TCA's, beta blockers
361
Cataract
vision impairing opacification of the lens caused by oxidative damage Sx: painless blurring of vision, flare, halos around lights, worsening of distance vision (myopic shift); red reflex is lost and retinal detail becomes less visible as cataract gets worse
362
MS
``` periventricular demyelination (dawson's fingers) optic neuritis - painful monocular vision loss that evolves over hrs to days and improves after several weeks; heat exposure exacerbates symptoms (uhthoff phenomenon) ```
363
TMP-SMX
contraindicated in pregnancy because TMP fucks with folate (binds DHFR)
364
Secondary thrombocytosis
Commonly seen in ppl with splenectomy. Bc the spleen removes senescent platelets, patients often have dramatic thrombocytosis following splenectomy which in most patients resolve within weeks or months. However a minority will have persistent thrombocytosis for years.
365
beta blockers proven to improve sx and long term survival in HF
metoprolol succinate, carvedilol, bisoprolol
366
Tocolytics and their side effects
- Indomethacin: COX inhibition. First line tocolytic for <32 weeks. Side effects: mom- gastritis, platelet dysfunction; fetal - oligohydramnios, closure of PDA - Nifedipine: CCB. First line tocolytic for 32-34 weeks. Side effects: mom- tachycardia/palpitations, n, flushing, HA - Terbutaline: beta agonist. Short term tocolytic for inpatient use. Side effects: mom - tachycardia/arrhythmias, hypotension, hyperglycemia, pulmonary edema
367
Central vs peripheral cyanosis
Cyanosis = significant amount of deoxygenated hemoglobin in the blood Peripheral - bluish discoloration is only seen in the distal extremities - cool clammy extremities; normal arterial oxygen saturation and increased oxygen extraction due to sluggish blood flow through capillaries Central - bluish discoloration in lips, tongue, nail beds; due to low arterial oxygen saturation
368
Anti-lipid medications
Statins - inhibit rate limiting step of cholesterol biosynthesis indications to start: clinically significant ASCVD, LDL >190, age 40-75 with diabetes, estimated 10 year ASCVD risk >7.5%s Fenofibrate - fibric acid derivation; used to treat hypertriglyceridemia. Cholestyramine - bile acid-binding resin that helps reduce LDL levels; may inc triglyceride levels Ezetimibe - prevents absorption of dietary cholesterol from the gut Niacin -raises HDL
369
Torsades de pointes
suspect in rapid onset LOC without preceding prodrome usage of antiarrhythmic drugs that prolong QT like sotalol, structural heart disease, hypokalemia and/or hypomagnesemia are all potential predisposing factors Normal QT
370
RTA | Renal tubular acidosis
All types can present as growth failure Suspect in person who is acidotic and has low serum bicarb and hyperchloremia --> normal anion gap metabolic acidosis Urine pH and electrolytes helps distinguish between the types of RTA
371
Catatonia
immobility or excessive purposeless activity, mutis, stupor, negativism, catalepsy, posturing, waxy flexibility, echolalia, echopraxia Tx: benzodiazepines (lorazepam), ECT
372
Crystal induced AKI
acyclovir, sulfonamides, methotrexate, ethylene glycol, protease inhibitors due to low urine solubility --> precipitation in renal tubules, causing intratubular obstruction and direct renal tubular toxicity
373
Dermatomyositis
proximal muscle inflammatory myopathy heliotrope, Gottron's papules Elevated CPK + Anti-Jo-1, anti-Mi-2 Associated with malignancies --> must be regularly screened
374
Dementia with lewy bodies
visual hallucinations, spontaneous parkinsonism with severe sensitivity to potent dopamine antagonists, fluctuating cognition
375
Bleeding disorders
von Willebrand disease: AD; prolonged mucosal bleeding (oropharyngeal, GI, uterine) Hemophilia: x-linked; bleedin ginto deep tissues (hemarthrosis, intramuscular hematomas) hemophilic arthropathy = caused by iron;hemosiderin deposition leading to synovitis and fibrosis within the joint --> chronic worsening joint pain and swelling and can result in contractures of the joint and limited ROM; most common in patients with history of recurrent hemarthroses
376
Side effects of erythropoietin therapy
- worsening of HTN - headaches - flu-like syndrome - red cell aplasia Anemia of chronic kidney disease is due to erythropoietin deficiency. One must be careful to ensure adequate iron stores prior to epo replacement because the epo-induced surge in RBC production can precipitate an iron-deficient state
377
Constrictive pericarditis
Etiology: idiopathic, viral, cardiac surgery, radiation, tb
378
Retroperitoneal hematoma
a vascular complication due to bleeding from the arterial access site after recent cardiac catheterization with/without use of anticoagulants (other complications: bleeding, arterial dissection, acute thrombosis, pseudoaneurysm, av fistula formation) can present with hemodynamic instability Dx: non-contrast CT abdomen pelvis or abdominal US Tx: supportive with bed rest, intensive monitoring, IV fluids/transfusions
379
Genital warts / condylomata acuminata
Small lesions may be treated with trichloroacetic acid or podophyllin resin Larger lesions can be removed with excisional therapy Recurrence rates are high no matter what
380
Milk or soy induced colitis
age 2-8 weeks non-IgE mediated immunologic response to proteins in formula or breast milk causes rectal and colonic inflammation --> eczema, regurg/vomiting, and/or painless bloody stools Tx: mom needs to eliminate dairy and soy from her diet and continue breastfeeding formula fed infants should be switched to a hydrolyzed formula (containing predigested proteins)
381
zofran/ondansetron
serotonin receptor antagonist that targets the 5HT3 receptor first line for chemotherapy induced nausea
382
Hazard ratio
<1 = event is more likely to occur in the control group >1 = event is more likely to occur in the treatment group Close to 1 implies little difference between the 2 groups
383
Burr cells Spur cells Target cells
Burr cells aka echinocytes spiculated RBC's with serrated edges seen in liver disease and ESRD Spur cells (acanthocytes) RBCs with irregularly sized and spaced projections most commonly seen in liver disease Target cells: seen in hemoglobinopathy (thalassemia) or chronic liver disease
384
Scleroderma renal crisis
occurs in 20% of diffuse systemic sclerosis Present with acute renal failure and malignant HTN. UA may show mild proteinuria Peripheral blood smear can show MAHA and fragmented RBCs and thrombocytopenia
385
clear cell adenocarcinoma
associated with DES exposure in utero --> cervical and uterine malformations as well as ifficulty conceiving and maintaining pregnancy males exposed in utero are at risk of cryptorchidism, microphallus, hypospadias, testicular hypoplasia
386
Hypopituitarism with mild to moderate increase in prolactin
Nonfunctioning adenoma (gonadotroph adenoma)
387
Complications after acute MI
``` Reinfarction - hrs to 2 days Ventricular septal rupture - hrs to 1 week Free wall rupture - hrs to 2 weeks Postinfarction angina - hrs to 1 month papillary muscle rupture - 2d to 1 week Pericarditis - 1 day to 3 months LV aneurysm - 5 days to 3 months ```
388
Chemo regimens
Induction therapy - initial dose of tx to rapidly kill tumor cells and send patient into remission (<5% tumor burden) Adjuvant therapy = tx given in addition to standard therapy Consolidation therapy - given after induction therapy with multidrug regimens to further reduce tumor burden Neoadjuvant therapy - tx given before standard therapy Maintenance therapy - given after induction to kill residual cells and keep patient in remission Salvage therapy - tx when standard tx fails
389
Genital HSV in pregnancy | Genital HSV
pregnant women with a history of genital HSV infection should receive prophylactic acyclovir or valacyclovir beginning at 36 weeks. This reduces the risk of an outbreak around the time of delivery and the need for a CS. --- classic presentation with vesicles often absent. Look for painful ulcerations associated with inguinal lymphadenopathy
390
Leriche syndrome
Arterial occlusion at the bifurcation of the aorta into the common iliac arteries Triad of bilateral hip, thigh and buttock claudication, impotence and symmetric atrophy of the bilateral lower extremities due to chronic ischemia. Men with a predisposition for atherosclerosis such as smokers are at the greatest risk of this condition
391
Kallmann syndrome Mullerian agenesis Androgen insensitivity
Kallman: X- linked; disorder of migration of fetal gonadotropin releasing hormone GnRH and olfactory neurons --> hypogonadotropic hypogonadism and rhinencephalon hypoplasia. Affected children have normal genotype and internal reproductive organs but congenital absence of GnRH secretion results in short stature and delayed or absent puberty Hypogonadism + anosmia/hyposmia Girls: primary amenorrhea, no breasts Boys: eunuchoid appearance with small external genitalia and absent secondary sex characteristics Lab findings: low FSH and LH Hormonal tx can help facilitate secondary sex characteristics and improve fertility Mullerian agenesis 46, XX: hypoplastic or absent mullerian duct system due to failure to differentiate into a uterus, cervix, and upper vagina Absent or rudimentary uterus and upper vagina; normal ovaries; normal breast development; normal axillary/pubic hair Androgen insensitivity 46, XY: defective androgen receptor that results in end-organ resistant to androgens. These patients have testicular secretion of anti-Mullerian hormone, which results in male internal genitalia. but testicular testosterone is converted to Estrogen, resulting in breast development.
392
Tetanus
Pts with significant puncture wounds who have not received revaccination for tetanus within 5 years should be vaccinated Also, tetanus Ig should be given to patients with significant or dirty wounds who have not previously received at least 3 doses of tetanus vaccine or whose vaccine status is uncertain
393
Cushing syndrome
Initial dx: late-night salivary cortisol assay, 24-hour urine free cortisol measurement, and/or overnight low-dose dexamethasone test If hypercortisolism is confirmed, ACTH levels are measured to differentiate ACTH-dependent from ACTH-independent causes
394
VIPoma
watery diarrhea, hypo or achlorhydria due to decreased gastric acid secretion, flushing, lethargy, n, v, muscle weakness/cramps Labs: hypokalemia (inc intestinal K secretion), hypercalcemia (inc bone resorption), hyperglycemia, secretory diarrhea with increased sodium and osmolal gap < 50 mOsm/kg Dx: watery diarrhea with VIP level >75. Abd CT or MRI to localize tumor suspect MEN
395
Inclusion body myositis
Adult onset distal muscular weakness and atrophy; dysphagia --> poor water intake
396
trial of labor after CS TOLAC
contraindications: classical vertical CS and adominal myomectomy with uterine cavity entry Not contraindicated in horizontal incision or abdominal myomectomy without uterine cavity entry
397
C diff
RF's: recent antibiotics, hospitalization, PPI Complications: fulminant colitis, toxic megacolon Dx: Stool PCR Tx: oral metronidazole or vancomycin
398
WPW
Accessory pathway that bypasses the AV node and directly connects the atria to the ventricles A fib occurs in 10-30% of individuals and is a potentially life threatening emergency bc it can bypass the usual rate-limiting function of the AV node, leading to very rapid ventricular response rate which can deteriorate into v fib unstable --> cardiovert stable --> anti-arrhythmic like procainamide or ibutilide
399
Intussusception
Usually ileo-colic When associated with HSP, intussusceptions are more likely to be small-bowel or ileo-ileal - which cannot be seen on contrast enema and are diagnosed by the presence of a target sign on US Lead points: - congenital malformation of the intestines (eg meckel diverticulum) - HSP - celiac disease - intestinal tumor - polyps - MOST COMMON IN KIDS AGE<2: gastroenteritis causing hypertrophy of the Peyer patches in the lymphoid-rich terminal ileum
400
Neutropenia
ANC < 1500 | Febrile neutropenia = medical emergency - start early empiric antibiotic therapy
401
Tumor lysis syndrome
Hyperureicemia, hyperkalemia, hyperphosphatemia, HYPOCALCEMIA (phosphate binds and precipitates calcium)
402
Pediatric tumors: Astrocytoma Medulloblastoma Ependymoma
Astrocytoma: most common brain tumor; seizures, weakness, sensory changes Medulloblastoma: 2nd most common arise in cerebellar vermis present with ataxia and truncal instablity from hydrocephalus and mass effect Ependymoma: glial cell tumor that arises from the ependymal cell lining of the ventricles and spinal cord --> obstruction of cerebrospinal fluid and inc ICP
403
Progressive multifocal leukoencephalopathy | JC virus
HIV detected on PCR testing of CSF Neuroimaging shows patchy areas of white matter consistent with demyelination
404
Acute mania
Antipsychotics, lithium, valproate
405
Pancreatic pseudocyst
expectant management - minimal sx | endoscopic drainage - sx
406
``` Lysosomal storage disorders Niemann-Pick Tay-Sachs Krabbe Gaucher Hurler ```
Niemann-Pick: AR; sphingomyelinase deficiency; hypotonia, loss of motor milestones, feeding difficulties, cherry red macula, HEPATOSPLENOMEGALY, AREFLEXIA Tay-Sachs: AR; beta-hexosaminidase deficiency; loss of motor milestones, hypotonia, feeding difficulties, cherry red macula, HYPERREFLEXIA Krabbe disease: AR; galactocerebrosidase deficiency; developmental regression, hypotonia, areflexia Gaucher: glucocerebrosidase deficiency; anemia, thrombocytopenia, hepatosplenomegaly Hurler: lysosomal hydrolase deficiency; coarse facial features, inguinal or umbilical hernias, corneal clouding, hepatosplenomegaly
407
Polycythemia vera
pruritus triggered by hot baths, headaches, hepatosplenomegaly --> inc cell turnover due to clonal hyperproliferation in all 3 primary bone marrow lineages --> gout Low epo level +JAK2 mutation Complications: thrombosis, myelofibrosis, acute leukemia Tx: phlebotomy, hydroxyurea (if increased risk of thrombus)
408
ITP
test for hep C and HIV as thrombocytopenia may be initial presentation -- Tx: Children - skin manifestations --> observe; bleeding --> IVIg or glucocorticoids Adults - >30,000 without bleeding --> observe; <30,000 or bleeding --> IvIg or glucocorticoids
409
COPD
mainstay treatment for symptom management = inhaled anti-muscarinic agents like ipratropium
410
Calcium correction
Total calcium + 0.8 (4 - albumin) patients with hypoalbuminemia can have decreased total serum calcium however ionized calcium (physiologically active form) is hormonally regulated and remains stable
411
ECT use in depression
- tx resistance - psychotic features - emergency conditions: pregnancy refusal, to eat or drink, imminent risk for suicide
412
Crohn vs UC
Crohn: noncaseating granulomas, cobblestoning, linear mucosal ulcerations, creeping fat; complications: fistulas, strictures, abscesses UC: no granulomas, mucosal and submucosal inflammation, pseudopolyps; complication: toxic megacolon
413
Anterior STEMI
puts you at risk for acute limb ischemia from arterial occlusion major cardiac sources of arterial emboli include: LV THROMBUS, thrombus in LA due to a fib, aortic atherosclerosis --> get echo
414
Thyroid storm
can be triggered by thyroid or non-thyroid surgery, acute illness, childbirth, or acute iodine load ie from undergoing CT with contrast
415
Medication-induced esophagitis
Tetracyclines, aspirin, NSAIDs, bisphosphonates, potassium chloride, iron
416
Parotid neoplasm
parotid gland = 2 lobes separated by the facial nerve which courses directly through the substance of the gland the facial nerve may need to be sacrificed with a parotidectomy in parotid neoplasms
417
Acute intermittent porphyria
Hereditary disorder involving alterations in heme biosynthesis. Suspect in new onset of psychiatric and neurologic abnormalities (neuropathy, anxiety, mood changes, psychosis), accompanied by unexplained acute abdominal pain and a family history of similar symptoms Abdominal pain is neuropathic Acute sx may last for days to weeks and will resolve between attacks Age of onset typically 30-40's Urinary porphobiliogen is elevated
418
Antisocial personality disorder
Must be 18+ to diagnosis and have history of conduct disorder sx before age 15
419
Growing pains
bilateral lower extremity pains that occur at night in children age 2-12 years Observation, massage, OTC pain meds
420
Brown-Sequard syndrome
Hemisection of the spinal cord: ipsilateral hemiparesis and diminished proprioception, vibratory sensation, and light touch at the level of the spinal cord injury and below diminished pain and temp sensation contralaterally, usually 1-2 levels below the cord injury, due to damage of the lateral spinothalamic tract
421
Carcinoid syndrome
well-differentiated neuroendocrine tumors most commonly found in the distal SI, proximal colon, and lung, with a strong propensity for metastasis to the liver. these tumors can secrete several products including histamine, serotonin, and VIP that are metabolized in the liver episodic pounding sensation (flushing, tachycardia), secretory diarrhea, weight loss, valvular heart disease with tricuspid regurg, cutaneous telangiectasias, bronchospasm Pathognomonic plaque-like deposits of fibrous tissue occur most commonly on the endocardium on the R side of the heart --> tricuspid regurg and R sided HF Dx: elevated 24 urine 5-HIAA
422
Indications for carotid endarterectomy
Men: asymptomatic - 60%+ stenosis Symptomatic - 50%+ Women: asymptomatic and symptomatic: 70%+
423
Anterior vs Posterior shoulder dislocation
Anterior: arm is abducted and externally rotated Posterior: arm is adducted and internally rotated
424
Congenital muscular torticollis | Cystic hygromas
Postural deformity that presents between age 1-6 months with limited ROM of the neck Exam may show a palpable well circumscribed mass that does not transilluminate in the inferior portion of SCM Flattening of the head with ipsilateral anterior displacement of the ear and forehead, known as positional plagiocephaly RF's: crowding in the uterus like multiple gestation, breech positioning, and oligohydramnios Tx: positioning (increased tummy time), passive stretching, and physical therapy -- Cystic hygromas - congenital malformations of the lymphatic system and are located in the posterior triangle of the neck; detected prenatally; high association with aneuploidy; postnatal exam reveals a fluctuant mass that transilluminates associated with Turner'sme
425
Isoniazid
Peripheral neuropathy, hepatotoxicity, sideroblastic anemia
426
Morton neuroma
Mechanically induced neuropathic degeneration of the interdigital nerves that causes numbness, aching, and burning in the distal forefoot from the metatarsal heads to the 3rd and 4rth toes when palpating the space between the 3rd and 4rth toes while squeezing the metatarsal joints
427
Aortic aneurysm
Ascending: due to cystic medial necrosis or connective tissue disorders Descending: due to atherosclerosis CXR: widened mediastinal silhouette, increased aortic knob, tracheal deviation
428
Window period Hep B
The time between the disappearance of HBsAg and the appearance of anti-HBs IgM anti-HBc may be the only detectable marker for acute hep B during this period HBsAg and anti-HBc are the most appropriate diagnostic tests for acute hep B as these are both elevated during initial infection and anti-HBc will remain elevated during the window period
429
Treatment schema of acne vulgaris
Comedonal acne: topical retinoids, salicylic, benzoyl peroxide Inflammatory acne: + topical antibiotics (erythromycin, clindamycin) Nodular cystic acne: + oral antibiotics --> oral isotretinoin
430
Tick-borne paralysis
Progressive ascending paralysis over hours to days - can be localized or more diffuse Due to neurotoxin release from the tick; tick needs to feed for 4-7 days for the realse Vs guillain-barre: no autonomic dysfunction in tick-borne paralysis CSF is normal
431
Interstitial cystitis
Painful bladder syndrome More common in women, associated with psych disorders and pain syndromes Presentation: bladder pain with filling, relief with voiding; inc frequency and urgency; dyspareunia Dx: bladder pain with no other attributable cause for 6+ weeks; normal UA Tx: not curative, focus is on quality of life; behavioral modification and trigger avoidance; amitriptyline; analgesics for exacerbations
432
Ischemic colitis
Acute abdominal pain followed by lower GI bleed after an episode of hypotension most commonly involved segments: splenic flexure (superior and inferior mesenteric arteries) and rectosigmoid junction (sigmoid and superior rectal) Abd CT with IV contrast may show thickened bowel wall although may just be nonspecific; colonoscopy confirms the diagnosis CT should be performed urgently to identify patients who need immediate surgical intervention
433
Varicella zoster
Kids get immunized at age 1 and age 4 All kids who are nonimmune age >1 year with varicella exposure should receive postexposure prophylaxis with the varicella vaccine Patients who are at-risk who cannot receive the vaccine should get Ig instead Infants <1 yr outside the neonatal period are not eligible for the VZV vaccine and do not require Ig as they are at lower risk than neonates or older children
434
Inferior MI | Posterior MI
Inferior MI: RCA or LCX - STE in leads II, III, aVF Associated with hypotension, bradycardia, and AV block Posterior MI: LCX or RCA - ST depression in leads V1-V3 LCX - STE in I and aVL RCA - ST depression in I and aVL
435
Pulmonary auscultation
Consolidation: increased breath sounds, increased tactile fremitus, dullness to percussion (Sound travels faster through solids/liquids) Pleural effusion/Atelectasis: dec or absent breath sounds, dec fremitus, dullness to percussion Pneumothorax/Emphysema: dec breath sounds, dec fremitus, hyperresonant to percussion
436
Open angle glaucoma
more common in AA's can see cupping of optic disc with loss of peripheral vision beta blockers like timolol eye drops = initial management laser trabeculoplasty and surgical trabeculectomy are done if needed
437
Diverticulosis
strongly associated with chronic constipation incidence of acute diverticular complications is lower in ppl with high intake of fruit and vegetable fiber and ppl who exercise; inc risk of complications = heavy meat consumption, aspirin, NSAIDs, obesity, possibly smoking
438
Cardiac myxoma
``` most often located in LA constitutional sx cardio complications: valvular abnormalities (mitral disease), HF due to anatomic obstruction, myocardial invasion causing arrhythmias, heart block, or pericardial effusion can embolize can invade the lungs ``` TEE = most sensitive test Tx: surgical resection
439
Dissociative disorders: Depersonalization/derealization disorder Dissociative amnesia Dissociative identity disorder
Depersonalization/derealization disorder: feelings of detachment from self or experiencing surroundings as unreal; INTACT REALITY TESTING Dissociative amnesia: inability to recall important personal information, usually of a traumatic or stressful nature + dissociative fugue - travel or bewildered wandering Dissociative identity disorder: discontinuity in identity and loss of personal agency with fragmentation into >2 distinct personality states; associated with severe trauma/abuse
440
Epididymitis
Unilateral testicular pain, epididymal edema; dysuria, frequency Age <35 - sexually transmitted (chlamydia, gonorrhea) Age >35 - bladder outlet obstruction (coliform bacteria) Tx: ceftriaxone/doxycycline if STI; levofloxacin if coliform bacteria
441
Glycogen storage diseases: | Glucose-6-phosphatase deficiency G6P deficiency
``` Type I (von Gierke disease): G6P deficiency - impaired conversion of glycogen to glucose, leading to glycogen accumulation in the affected organs Present at 3-4 months with hypoglycemia (seizures) and lactic acidosis (buildup in the liver), hyperuricemia, hyperlipidemia, doll-like face with round cheeks, thin extremities, short stature, and a protuberant abdomen due to hepatomegaly ```
442
Cushing reflex
HTN, bradycardia, and respiratory depression | indicates intracranial pressure
443
Bias
Hawthorne effect - tendency of study population to affect the outcome becuase they are aware they are being studied sample distortion bias - study sample not representative of target population Information bias - imperfect assessment of association between exposure and outcome due to errors in measurement
444
Familial hypocalciuric hypercalcemia (FHH)
benign AD disorder caused by a mutation of the calcium-sensing receptor higher calcium concentrations are required to suppress PTH release. And defective CaSR leads to increased reabsorption of calcium in renal tubules --> in evaluation of hypercalcemia, an elevated or inappropriately normal PTH level suggest either primary hyperparathyroidism or FHH Urine calcium/creatinine clearance ratio is usually <0.01 in FHH compared to >0.02 in primary hyperparathyroidism
445
Weber and Rinne
Weber: conductive hearing loss - lateralizes to affected ear sensorineural hearing loss - lateralizes to unaffected ear Rinne: conductive - BC > AC in affected ear, AC > BC in unaffected ear sensorineural - AC > BC in both earso
446
Hepatorenal syndrome
Severe liver cirrhosis --> inc nitric oxide generation in the splanchnic circulation secondary to portal hypertension --> systemic vasodilation, which reduces peripheral vascular resistance and BP, causing renal hypoperfusion --> compensatory pathways (RAAS, ADH) --> worsen volume overload anything that may reduce glomerular capillary pressure like hypotension from GI bleed causes an acute decline in glomerular filtration and can precipitate hepatorenal syndrome Lab results are similar to those of prerenal azotemia But patients do not respond to IVF and withdrawal of diuretics and renal function continues to decline
447
Ototoxic antibiotics
Aminoglycosides, chemo agents (cisplatin), aspirin, loop diuretics
448
Zollinger-Ellison syndrome
due to gastrin-producing tumor associated with MEN1 uncontrolled gastrin secretion leads to pareital cell hyperplasia, with excessive production of gastric acid --> excess gastric acid in the small intestine can cause diarrhea and steatorrhea due to inactivation of pancreatic enzymes and injury to the mucosal brush border
449
Meds to withhold prior to cardiac stress testing
hold for 48 hrs: beta blockers, CCB's, nitrates, dipyridamole hold for 12 hrs: caffeine-containing food or drinks Continue: ACEI's, ARB's, digoxin, statins, diuretics
450
Pneumonia physio
Pneumonia causes hypoxemia due to R to L intrapulmonary shunting and an extreme V/Q mismatch. Increased concentration of inspired oxygen does not correct hypoxemia caused by intrapulmonary shunting
451
Developmental dysplasia of the hip
RF's: breech positioning, positive FH, excessively tight swaddling Clinical findings: red flags - positive ortolani test, dislocated hip, limited hip abduction supportive findings - limb length discrepancy, asymmetric gluteal/inguinal/thigh, creases, limited hip abduction Management: red flags - refer to ortho supportive findings or risk factors: age <4 months: hip ultrasound age >4 months: hip radiograph s
452
Primary nocturnal enuresis
Urinary incontinence age > 5 and occurs at least twice a week ``` Management: UA - rule out secondary causes lifestyle change - minimize fluid intake before bed, avoid sugary/caffeinated beverages, void before bedtime, institute reward system enuresis alarm desmopressin therapy (ADH)c ```
453
Pneumococcal vaccine
Pneumococcal polysaccharide vaccine (PPSV23) - contains capsular material from 23 serotypes. Polysaccharides alone cannot be presented to T cells --> vaccine induces a relatively T cell independent B cell response that is less effective in young children and the elderly Pneumococcal conjugate vaccine (PCV13) consists of capsular polysaccharides from 13 serotypes that have been covalently attached to inactivated diphtheria toxin protein --> induces a T cell dependent B cell response, resulting in improved immunogenicity due to the formation of higher-affinity antibodies and memory cells
454
Biliary atresia
elevated conjugated bilirubin | Early tx with a Kasai procedure dramatically improves outcomes
455
Aspirin and beta blockers in ppl with asthma
Can trigger bronchoconstriction patients with asthma
456
Late term / postterm pregnancy complications
Fetal: oligohydramnios, meconium aspiration, stillbirth, macrosomia, convulsions Maternal: CS, infection, PPH, perineal trauma
457
Platelet dysfunction
most common cause of abnormal hemostasis in patients with chronic renal failure PT, PTT, and platelet count are normal; bleeding time is prolonged. DDAVP is usually the treatment of choice if needed - it increases the release of factor VIII:von Willebrand factor multimers from endothelial storage sites
458
Shoulder dystocia RF's
fetal macrosomia, maternal obesity, excessive pregnancy weight gain, gestational diabetes, post-term pregnancy
459
Brain abscess
Microbiology: Viridans streptococci, Staph aureus, Gram neg organisms
460
High output heart failure
Congenital: PDA, angiomas, pulmonary AVF, CNS AVF Acquired: trauma (AVF), iatrogenic (femoral catheterization), atherosclerosis (aortocaval fistula), cancer, thyrotoxicosis, Paget disease, anemia, thiamine deficiency mi Shunting of a large amount of blood through a fistula and bypassing the capillary beds decreases SVR, increases cardiac preload, and increases cardiac output to meet the oxygen requirements of the peripheral tissues - cardiac function can decompensate over time and result in heart failure despite high cardiac output bc the circulation is unable to meet the oxygen demand of the peripheral tissues. Clinical signs: widened pulse pressure, strong peripheral arterial pulsation, systolic murmur, tachycardia, flushed extremities
461
Hemoptysis
Massive: >600 mL/24 hrs or 100 mL/hr danger = asphyxiation due to the airway flooding with blood Patient should be placed with the bleeding lung in the dependent position (lateral position) to avoid blood collection in the airways of the opposite lung Bronchoscopy = initial procedure of choice - can localize the bleeding site, provide suctioning ability to improve visualization, and include other therapeutic interventions
462
Angioedema
edema in the face, mouth, lips, tongue, glottis, and larynx - due to the pro-inflammatory action of bradykinin, which promotes edema, inflammation, and sesation of pain ACEI = most common cause of acquired; can occur anytime within taking the medications both hereditary and acquired is due to C1 inhibitor deficiency, dysfunction, or destruction --> elevated levels of edema producting factors C2b and bradykinin C1q levels are normal in hereditary angiodema and depressed in acquired forms C4 levels are depressed in all forms
463
Dysphagia
Oropharyngeal dysphagia: difficulty initiating swallowing with cough, choking, or nasal regurgitation --> videofluoroscopic modified barium swallow Esophageal dysphagia: --> barium swallow
464
Hyperemesis gravidarum
RF's: multiple gestation, hydatidiform mole, and a history of esophageal reflux unresponsive to oral antiemetics; weight loss is typically >5% of prepregnancy weight differentiated from typical nausea and vomiting of pregnancy by the presence of ketones on UA, lab abnormalities, and changes in volume status severe HG is an indication for hospital admission for IV antiemetics, rehydration, and electrolyte repletion
465
Laxative abuse
Frequent watery nocturnal diarrhea Dx: positive laxative screen or colonoscopy with characteristic finings of melanosis coli (dark brown discoloration with pale patches of lymph follicles)
466
Amiodarone
Antiarrhythmic Broad range of adverse effects including hypo and hyperthyroidism, hepatotoxicity, bradycardia, heart block, pneumonitis, neurologic symptoms, and visual disturbances Patients taking amiodarone should be monitored with periodic thyroid and hepatic function markers
467
Placenta accreta
RF's: prior CS, hx of D&C, maternal age >35 typically diagnosed by antenatal US findings that include irregularity or absence of the placental-myometrial interface and intraplacental villous lakes Antenatally diagnosed placenta accreta is delivered by planned cesarean hysterectomy Undiagnosed placenta accreta presents as difficulty with placental delivery - placenta does not detach from the uterus and often results in cord avulsion and necessitates a manual extraction, which can then be complicated by placental adherence and severe hemorrhage
468
Hydrocele
fluid collection within the processus vaginalis/tunica vaginalis, a peritoneal projection that accompanies the testis during its descent into the scrotum. When this fails to obliterate, peritoneal fluid may accumulate within the processus vaginalis causing a communicating hydrocele most hydroceles will resolve spontaneously by the age of 12 months and can be safely observed during that period Communicating hydroceles that persist beyond 1 yr of age are unlikely to resolve spontaneously and put the patient at increased risk of indirect inguinal hernia. Surgical intervention is indicated in such cases
469
Bronchiolitis
primarily caused by RSV in older children, it is usually self limiting and a mild URI in children <2 years they tend to get lower resp tract involvement --> wheezing, crackles, apnea, respiratory failure Palivizumab = monoclonal antibody against RSV that is used for prophylaxis in children age <2 years who are at exceptionally high risk of complications (preterm <29 weeks gestation), chronic lung dz of prematurity, hemodynamically significant congenital heart disease)
470
Cephalohematoma vs caput succedaneum
Cephalohematoma: subperiosteal hemorrhage, presents a few hours after birth as scalp swelling limited to one cranial bone most cases don't require treatment and resorb spontaneously within 2 weeks to 3 months Caput succedaneum: diffuse, sometimes ecchymotic, swelling of the scalp. Usually involves the portion of the head presenting during vertex delivery. May extend across the midline and across suture lines
471
Vasovagal syncope
Tx: reassurance, education, avoid triggers, use physical counterpressure maneuvers during prodromal phase to abort or delay syncope (supine position with leg raising, leg crossing with tensing of muscles, handgrip, tensing of arm muscles with clenched fists) - improve venous return and cardiac output
472
Aromatase deficiency
normal internal genitalia, external virilization, undetectable estrogen levels --> delayed puberty, osteoporosis, high concentrations of gonadotropins that result in polycystic ovaries
473
Ovarian hyperthecosis
virilization typically diagnosed in postmenopausal women; signs of insulin resistance; low/normal LH and FSH typical US findings: solid-appearing, enlarged ovaries rather than multiple cystssu
474
Organophosphate
bradycardia, MIOSIS, bronchorrhea, MUSCLE FASCICULATIONSm, salivation, lacrimation, diarrhea, urination counteract: atropinep
475
Urinary incontinence
Stress - dec urethral sphincter tone, urethral hypermobility Urge - detrusor hyperactivity Overflow - impaired detrusor contractiliy, bladder outlet obstruction
476
Cauda equina syndrome vs conus medullaris syndrome
``` Cauda equina syndrome: LMN signs as the nerve roots are part of the peripheral nervous system b/l severe radicular pain saddle hypo/anesthesia asymmetric motor weakness hyporeflexia/areflexia late onset bowel and bladder dysfunction ``` Conus medullaris syndrome: UMN and LMN signs as the conus is part of the spinal cord sudden onset severe back pain perianal hypo/anesthesia symmetric motor weakness hyperreflexia early-onset bowel and bladder dysfunctiona
477
Progressive multifocal leukoencephalopathy PML
``` JC virus reactivation severe immunosuppression (eg untreated AIDS CD4 <200) ``` manifestations: slowly progressive; confusion, paresis, ataxia, seizure Dx: MRI brain - white matter lesions with no enhancement/edema LP - CSF PCR for JC virus Tx: often fatal; if HIV --> antiretroviral therapy
478
Hypovolemic shock
Lowers central venous pressure, elading to decreased venous return to the RA and therefore decreased cardiac output PPV causes an acute increase in intrathoracic pressure, which in a severely hypovolemic patient with low central venous pressure, can collapse venous capacitance vessels (IVC) and cut off venous return --> sudden loss of RV preload --> acute circulatory failure and cardiac arrest Sedatives used prior to intubation cause relaxation of venous capacitance vessels and can also contribute to decreased venous return
479
Alzheimer's
First line: donepezil, rivastigmine, galantamine - reversible acetylcholinesterase inhibitors
480
Mumps
Viral infection that presents with fever and parotitis after a nonspecific prodrome self-limited condition but can lead to complications: aseptic meningitis, orchitis
481
CRC screening in high risk patients
IBD: 8 years post diagnosis; colonoscopy with biopsies every 1-2 years FAP: begin 10-12 colonoscopy yearly HNPCC: begin 20-25, colonoscopy 1-2 years