UWorld Fun Facts Flashcards
(481 cards)
Chronic bronchitis -- Chronic bronchitis vs emphysema --- Chronic bronchitis vs bronchiectasis
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
Aspergillosis
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
Chorioamnionitis
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
Bone tumors: giant cell tumor of bone osteoid osteoma osteosarcoma Ewing sarcoma
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
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
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
Hereditary Hemochromatosis
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
Pseudogout
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
B12 deficiency / Pernicious anemia
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)
Esophageal varices
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
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
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
Acute pancreatitis
Chronic pancreatitis
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
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
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
Effusions / Lights criteria
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
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Normal pleural pH = 7.6
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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
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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
Aortic injury
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
Hereditary cancer syndromes: Lynch FAP vHL MEN 1 MEN 2 BRCA 1/2
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
Vasculitidies: Wegener Churg-Strauss HSP Takayasu Buerger Polyarteritis nodosa
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
Hereditary spherocytosis
- 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
Paroxysmal nocturnal hemoglobinuria
- 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)
Celiac disease vs pancreatic insufficiency
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
Precocious puberty
Premature adrenarche
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
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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
Classic vs nonclassic CAH
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
Serous otitis media
- 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
PCOS vs testosterone secreting tumor
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
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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
Acute mediastinitis
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