Path Flashcards
1
Q
Fibrocystic change
A
usually benign, some changes associated with increased risk for invasive carcinoma cystic breasts blue dome gross appearance
2
Q
Anitschkow cells are plump macrophages seen in what dx?
A
Rheumatic heart disease (beta hemolytic, group A streptococcus) Anitschkow cells are part of the Aschoff bodies lesions
3
Q
What drugs cause interstitial nephritis?
A
sulfonamides, penicillins, cephalosporins, the fluoroquinolone antibiotics ciprofloxacin and norfloxacin, and the antituberculous drugs isoniazid and rifampin. Acute tubulointerstitial nephritis also can occur with use of thiazide and loop diuretics, cimetidine, ranitidine, omeprazole, and nonsteroidal anti-inflammatory drugs. The disease manifests about 2 weeks after the patient begins to use the drug.
4
Q
What is the effect of the following teratogen?
Phenytoin
A
Digit hypoplasia and cleft lip/palate
5
Q
Anti-DNA topoisomerase antibody is seen in:
A
scleroderma (mainly affects vasculature of kidney)
6
Q
What is the diagnosis? Benign cystic tumor with abundant lymphocytes and germinal centers; Almost always arises in the parotid
A
Warthin tumor 2nd most common tumor of the salivary gland
7
Q
Name the dx:
The orifice of the prepuce cannot allow retraction over the glans penis, and can strangulate the glans and impede the outflow of venous blood
A
Phimosis
Complication when blood flow is obstructed is paraphimosis
8
Q
Same small lymphocytes as CLL, but appearing in tissue and not blood; widespread lymphadenopathy; liver and spleen enlargement; lymphocytosis
A
Small lymphocytic lymphoma
9
Q
Lobular Carcinoma In Situ (LCIS)
A
discohesive cells lacking E-cadherin
no mass or calcifications
treatment with tamoxifen
**LCIS, more than DCIS, serves as a marker for enhanced risk of subsequent invasive carcinoma in both breasts
10
Q
Volvulus is found in the elderly in:
A
sigmoid colon
11
Q
Neurogenic bladder
A
Control of micturition is disrupted by spinal cord transection above the sacral cord, and detrusor hyperreflexia can occur in this instance.
Blockade of muscarinic acetylcholine receptors with agents like tolterodine and other antispasmodics can decrease this parasympathetic-related hyperactivity.
12
Q
Invasive Lobular Carcinoma
A
second most common form of invasive breast cancer
can be a discrete firm mass or not
single strands or lines of cells with stroma in between
13
Q
Labs Hep B:
A
ALT>AST, in the thousands; Bili high
14
Q
Name the diagnosis:
recent acute Hep B infection; ANCA (+); Proteinuria; Fever, muscle pain, weight loss; Lesions on legs - reddish-blue; Elevated ESR
A
Polyarteritis nodosa
small to medium arteries
fibrinoid necrosis = eosinophilic mass that stains for fibrin
15
Q
Frataxin is essential for what?
A
Mitochondrial iron regulation;
loss results in iron build-up with free radical damage;
this is the gene impaired in Friedreich Ataxia
Frataxin, Fe, Free radical damage, Friederich Ataxia
16
Q
CK activity does what after an MI?
A
CK activity begins to increase 2 to 4 hours after an MI, peaks at about 24 to 48 hours, and returns to normal within 72 hours. Total CK activity is a sensitive marker for myocardial injury in the first 24 to 48 hours.
17
Q
Hep C is Sexual?
A
Not really, Hep B is sexually transmitted Hep C is blood (tattoos, IV drug use)
18
Q
Most common breast cancer in men:
A
Invasive ductal carcinoma (still very rare)
BRCA2 plays a role
19
Q
Name the disorder characterized by: 1. Platelet destruction secondary to heparin therapy 2. May cause thrombosis from destroyed platelets activating remaining platelets
A
Heparin-induced thrombocytopenia
20
Q
Desquamative interstitial pneumonitis is related to what predisposition?
A
Desquamative interstitial pneumonitis (DIP) is an uncommon smoking-related interstitial disease in which monocytes gather to form intra-alveolar macrophages.
21
Q
Necrotizing vasculitis occurs in pulmonary vasculature in association with:
A
Necrotizing vasculitis occurs in pulmonary vasculature in association with anti–neutrophil cytoplasmic autoantibody (ANCA)–associated granulomatous vasculitis.
22
Q
Dental caries are most often caused by:
A
Strep mutans
Prevention is fluoride
23
Q
A 10-year-old girl develops subcutaneous nodules over the skin of her arms and torso 3 weeks after a bout of acute pharyngitis. She manifests choreiform movements and begins to complain of pain in her knees and hips, particularly with movement. A friction rub is heard on auscultation of her chest. An abnormality detected by which serum laboratory finding is most characteristic of the disease affecting this girl?
A
anti–streptolysin O (ASO) titer
Acute rheumatic fever can involve any or all layers of the heart. Because rheumatic fever follows group A streptococcal infections, the antihyaluronidase, anti-DNase, and anti–streptolysin O (ASO) titers are often elevated.
24
Q
What is the anatomical (as opposed to clinical) result of an ischemic stroke?
A
Liquefactive necrosis
Eosinophilic change –> Necrosis –> fluid-filled cystic space surrounded by gliosis
25
Chronic inflammatory disease of the salivary and lacrimal glands that may be restricted there or be part of a systemic collagen vascular disease
Sjogren syndrome
26
Name the diagnosis corresponding to this autoantibody:
Anti-basement membrane
Goodpasture syndrome
27
PJP in neonates
Premature infant with respiratory distress and a biopsy showing **surfactant-rich exudate and silver-staining cysts:**
Pneumocystis jirovecii
The surfactant-rich exudate is due to the **replication of type 2 pneumocytes.**
28
Dx: Liver infarction due to hepatic vein obstruction; Painful hepatomegaly and ascites
Budd-Chiari syndrome
29
Dx: Waxing and waning RUQ pain; Due to gallbladder contracting against stone lodged in cystic duct
Biliary colic
Complication of gallstones
30
Subepithelial electron-dense humps represent immune complexes seen in:
Postinfectious GN
31
The most important gene in familial cases of _________ is that of cyclin-dependent kinase inhibitor 2 (CDKN2A), which encodes several tumor suppressors including p16/INK4A.
dysplastic nevus syndrome
32
The finding of pulmonary hypertension in a young individual without any known pulmonary or cardiac disease is typical for primary pulmonary hypertension. With what gene is this associated?
BMPR-2 - bone morphogenic receptor 2 BMPR2, a cell surface protein belonging to the TGF-β receptor superfamily, causes inhibition of vascular smooth muscle cell proliferation and favors apoptosis. In the absence of BMPR2 signaling, smooth muscle proliferation occurs, and pulmonary hypertension ensues.
33
Give the dx:
Abnormal type IV collagen in the glomerular BM;
Hematuria presents early in life;
Proteinuria;
progressive renal failure and HTN develop later in life
Hereditary nephritis (Alport syndrome)
34
Mutations in fibrillin gene
Marfan Syndrome
35
Give the dx: Chronic immune complex disease; Granular deposition of immunoglobulin and complement in glomerular capillary loops and mesangium
Membranoproliferative glomerulonephritis type 1 (MPGN-1)
36
Patients with autoimmune chronic gastritis are at increased risk for
Gastric adenocarcinoma (from intestinal metaplasia)
37
Facial hyperpigmentation in a pregnant woman
Melasma
38
Apolipoprotein B-100 function
make VLDL and LDL
39
Androgenital syndrome
congenital 21-hydroxylase deficiency virilization in females a congenital adrenal hyperplasia high levels of androgens lead to premature closure of epiphyses
40
Name the diagnosis: Vasculitis with cryoglobulin immune deposits affecting small vessels (predominantly capillaries, venules, or arterioles) and associated with serum cryoglobulins. Skin, glomeruli, and peri- pheral nerves are often involved.
Cryoglobulinemic vasculitis (CV)
41
Cause of von-hippel lindau syndrome
mutated VHL tumor suppressor gene
42
Dx: Giant cell pneumonia with "Warthin-Finkeldey giant cells"
Measles pneumonia
43
What is the diagnosis? Bilateral parotitis with increased serum amylase; Complications include meningoencephalitis, unilateral orchitis or oophoritis and pancreatitis
Mumps (paroxymyxovirus)
44
A 62-year-old woman has had increasing dyspnea for the past 2 years. She now awakens at night with air hunger and cough productive of frothy sputum. On examination, she has rales in all lung fields. Her point of maximal impulse is strong and displaced laterally. Echocardiography shows a decreased ejection fraction of 30% with concentric increase in left ventricular wall size. The valves appear normal. What underlying diseases does she have?
HTN
Left-sided congestive heart failure leads to pulmonary congestion and edema.
Systemic hypertension is common and leads to pressure load with predominantly concentric left ventricular hypertrophy with systolic dysfunction.
45
What is Sheehan syndrome?
Hypopituitarism caused by infarction (ischemic necrosis) of the anterior pituitary Can happen during pregnancy when it enlarges
46
A 50-year-old man with a history of infective endocarditis has increasing fatigue. He receives a bileaflet tilting disk mechanical mitral valve prosthesis. After surgery, he is stable, and an echocardiogram shows no abnormal valvular or ventricular function. What pharmacologic agent should he receive regularly after this surgical procedure?
Warfarin Anticoagulant therapy is necessary for patients with mechanical prostheses to prevent potential thrombotic complications.
47
PTEN tumor suppressor gene
hormonally regulated in the normal endometrium
biomarker for endometrial carcinogenesis
48
Hemorrhage and contraction bands in necrotic myocardial fibers are most likely to be seen with infarction in what clinical setting?
Thrombolytic therapy
Reperfusion of an ischemic myocardium by spontaneous or therapeutic thrombolysis changes the morphologic features of the affected area.
Reflow of blood into vasculature injured during the period of ischemia leads to mitochondrial dysfunction, followed by leakage of blood into the tissues (hemorrhage).
49
What is the diagnosis? Heliotropic rash on the upper eyelids, face and trunk; Increased serum levels of creatinine kinase; Thought to be of autoimmune origin
Dermatomyositits
50
What is the diagnosis? Benign papillary tumor of the vocal cord; Due to HPV 6 and 11; Presents with hoarseness
Laryngeal Papilloma
51
Common autoimmune blistering disease;
Acantholysis (loss of intercellular connections) absent;
IgG antibodies are directed against 2 basement membrane proteins, BPAG1 and BPAG2;
immunofluorescent studies reveal linear deposition of C3 and IgG along epidermal BM zone
Bullous pemphigoid
52
Patients with H. Pylori chronic gastritis are at increased risk for what 3 things?
1. ulceration 2. gastric adenocarcinoma 3. MALT lymphoma
53
CD19 is a:
B-cell marker
54
What is the diagnosis? Hypersensitivity reaction against mycoplasma or sulfa drugs
Erythema multiforme
called Stevens-Johnson syndrome when it involves the mouth
55
Name the disorder characterized by: 1. Genetic G2b-3a deficiency 2. Impaired platelet aggregation
Glanzmann Thrombasthenia
Glanzmann thrombasthenia is a rare autosomal recessive disorder with defective platelet aggregation from deficiency or dysfunction of glycoprotein IIb/IIIa.
56
Achondroplasia
dwarfism
overexpression of FGF3
autosomal dominant
failure of normal epiphyseal cartilage formation
poor endochondral bone formation - long bones cant grow
57
Hypercalcemia occurs with myeloma because:
myeloma cells produce MIP1-α that up-regulates RANKL production and increased osteoclastic activity; punched-out lytic bone lesions are typical of multiple myeloma.
58
T/F: Diabetes affects both the sensory and the motor portions of the peripheral nervous system.
True
Common cause of distal polyneuropathy
59
Name the kind of cancer:
Peripheral subpleural mass
Nonsmoker
Glandular appearance on H&E
Adenocarcinoma
Cancers that arise in nonsmokers are pathogenetically distinct from those that occur in smokers. They may have either **EGFR** mutations or **KRAS** mutations. Most are adenocarcinomas. Twenty-five percent of lung cancers worldwide occur in nonsmokers. Primary adenocarcinomas in the lung tend to be small, peripheral masses that are amenable to surgical excision and have a better overall prognosis than other forms of lung cancer.
60
Small emboli, notably those composed of fat or air, occlude capillaries and cause \_\_\_\_\_\_\_
petechiae
61
What is the diagnosis?
Airway obstruction causes CO2 retention (respiratory acidosis) leading to hypoxemia; PaO2 down, O2 sat down, PaCO2 up
Complications include pulmonary HTN and right ventricular hypertrophy and secondary polycythemia (RBC hyperplasia due to hypoxemia)
Obstructive Sleep Apnea
Obesity is the most common cause
Tonsillar hypertrophy is another cause
62
First signs of medulloblastoma
symptoms of hydrocephalus, truncal ataxia and wide based gate rapid and regionally infiltrative tumor of the cerebellum
63
in rare cases pleomorphic adenomas can become malignant resulting in parotid adenocarcinoma. What clinical symptom would be indicative of possible malignancy and why?
diffuse unilateral upper and lower facial pain -- facial nerve runs through parotid gland
64
Name two common extrarenal findings in adults with ADPKD.
Berry aneurysm; Liver cysts
65
What is the cause of exophthalmos (proptosis) in hyperthyroidism?
Swelling of the extraocular muscles If severe, can include corneal exposure with subsequent ulceration and optic nerve compression
66
What kind of shunt is PDA?
Although often not causing a large shunt defect, a patent ductus arteriosus can produce a significant murmur and predispose to infection. This left-to-right shunt may eventually result in pulmonary hypertension.
67
Choriocarcinoma
malignant tumor or cytotrophoblasts and syncytiotrophoblasts
rapid breast enlargement/precocious puberty in younger patients
mimics placental tissue,
villi absent
small and hemorhhagic tumor with early hematogenous spread
b-HCG elevated\*\* due to syncytial cells
poor response to chemo
68
Intracerebral hemorrhage is bleeding into the brain parenchyma. It's classically due to:
Rupture of Charcot-Bouchard microaneurysms of the lenticulostriate vessels; complication of HTN;
69
Volvulus is found in the young in:
Cecum
70
Folic acid is mostly reabsorbed in
Jejunum
71
An elevated serum B-type natriuretic peptide (which is measured instead of atrial natriuretic peptide) is consistent with \_\_\_\_\_\_\_\_
An elevated serum B-type natriuretic peptide (which is measured instead of atrial natriuretic peptide) is consistent with heart failure.
72
Name the diagnosis:
Arteritis associated with the mucocutaneous lymph node syndrome and predominantly affecting medium and small arteries.
Coronary arteries are often involved.
Aorta and large arteries may be involved.
Usually occurs in infants and young children.
Kawasaki disease
73
What is croup? What is the most common cause?
inflammation of the upper airway (aka laryngotracheaobronchitis) parainfluenza virus
74
Name the dx:
Renal retention of phosphate --\> hypocalcemia --\> bone deformities such as bone pain, fractures, cysts, "brown tumors"
Osteitis fibrosa cystica
due to secondary hyperparathyroidism (from renal insufficiency)
75
Give the dx:
Glomerular scarring; Initially involves only part of a glomerular tuft;
Segmental obliteration of capillary loops by collagen and increased lipids/proteins;
Cause of nephrotic syndrome in 30% of adults (and 10% of children);
Proteinuria
Focal segmental glomerulosclerosis (FSGS)
\*\*Most common renal complication of IV drug use
76
Apolipoprotein B-48 dies:
makes chylomicrons (no chylomicrons --\> malabsorption) seen in Abetalipoproteinemia
77
What is the disease? Pathogenic auto-IgG antibodies recognize voltage sensitive calcium channels that are expressed in both the motor nerve terminals and the cells of the associated cancer. These calcium channels are necessary for the release of ACh, thus their destruction is associated with weakness.
Lambert Eaton Myasthenic Syndrome aka myasthenic myopathic syndrome
78
biopsy of a nasopharyngeal carcinoma would likely show what two histological features?
pleomorphic keratin-positive (poorly differentiated) epithelial cells Background of lymphocytes
79
What is the most common site for a stroke due to hypertension?
basal ganglia/thalamic area (65%)
80
Watershed zone most commonly affected by deminished cerebral blood flow (e.g. prolonged hypotension)
overlap zone between terminal branches of middle cerebral and anterior cerebral (watershed zone) - results in parasaggital infarcts
81
Give the dx:
LACK of immune deposits; periorbital edema; BUN:creatinine \>10:1; Oliguria with cloudy brown appearance; focal necrosis in glomeruli with glomerular BM breaks and no crescents; Anti-neutrophil cytoplasmic antibody detected in serum
Pauci-immune crescentic glomerulonephritis
Cases can be idiopathic, associated with ANCAs, and limited to the kidney.
82
DCIS
does not usually produce a mass
calcification on mammography
Paget Disease indicates underlying carcinoma
83
Mutations in the lysyl hydroxylase gene
Ehlers-Danlos Syndrome
84
PJP
Pneumocystis jirovecii (formerly known as Pneumocystis carinii) is classified as a fungus, based on nucleic acid and biochemical analysis. To prevent confusion in the medical community, when using the "pneumonia" abbreviation, P. jirovecii pneumonia is still abbreviated as PCP.
PCP is still the **most common AIDS-defining opportunistic infection.**
85
Differential for bilateral parotid gland swelling includes?
Sjogren syndrome; Mumps virus infection; Warthin cysts (15% of cases bilateral)
86
An elevated D-dimer level suggests:
a microangiopathic hemolytic anemia
87
What is the diagnosis? Inflammation of the upper airway; Most common cause is parainfluenza virus; Barking cough and inspiratory stridor
Croup aka Laryngotracheobronchitis
88
Name the dx: Follicular hyperplasia with scalloping of colloid of thyroid gland
Graves disease
89
Give the diagnosis for the following auto-antibody:
p-ANCA (MPO-ANCA)
Microscopic Polyangiitis
Churg-Strauss syndrome
90
High lipase is most specific for:
Pancreatitis (moreso than amylase)
91
What is sialadenitis?
Inflammation of the salivary gland; Most commonly due to an obstructing stone --\> staph aureus Usually unilateral
92
Give the dx: long-standing ulcerative colitis; jaundice; inflammation and obliterative fibrosis of intrahepatic and extrahepatic bile ducts; Beaded biliary tree on radiography
Primary sclerosing cholangitis
93
Cyanosis is a feature of what pathological cardiac shunt?
Right to left
94
What is the diagnosis? Notched teeth
Congenital syphilis Treponma Palladium Incisors are notched and tapered like pegs, molars look like mulberries
95
Give the dx: bilaterally small kidneys with barely visible medullary cysts concentrated at the corticomedullary junction; mutation in NPHP1;
Nephronophthisis; most common genetic cause of end-stage renal disease in children and adolescents; AR
96
Fatty bone marrow, think:
aplastic anemia fatigue, severe pancytopenia, NO reticulocyte response to the anemia
97
A 73-year-old woman with hyperhomocystinemia has noted poor circulation in her left leg for the past 2 years. On examination her dorsalis pedis and posterior tibial pulses are barely palpable. CT angiography shows 90% stenosis of the left iliac artery. What procedure would be most effective in treating this woman?
Endovascular stent Endovascular stent placement can be done without major surgery, because the graft can be deployed percutaneously.
98
Rapidly progressive GN is principally associated with:
Hematuria, crescents
99
Centrilobular emphysema results from damage to the central part of the lung acinus, with dilation that primarily affects what part of the lung?
Centrilobular emphysema results from damage to the central part of the lung acinus, with dilation that primarily affects the respiratory bronchioles.
100
HMB-45
marker for melanoma used to diagnose brain metastasis
101
Name the two medium vessel vasculitides.
Polyarteritis nodosa Kawasaki disease
102
What is the effect of the following teratogen?
Cocaine
Intrauterine growth retardation;
Placental abruption
103
What is the dx? A fold of vascularized cornea; grows horizontally in the shape of an insect wing; Not due to an infectious agent
Pterygium Frequently recurs after resection; Often associated with pinguecula
104
Progressive multifocal leukoencephalopathy (PML) is a destructive disease caused by JC virus. What are the symptoms?
Dementia Visual loss Progressive weakness Can follow cadaveric transplant (due to immunosuppression) Lesions of demyelination near gray-white junction in cerebral hemispheres and brainstem
105
Enlarged supraclavicular lymph node; common in HIV population; tumor cells express B-cell antigens and are EBV +
Large B-cell lymphoma/high-grade B-cell lymphoma ie Burkitt; bcl2 gene rearrangements often seen, as with follicular lymphoma
106
Periductal mastitis
inflammation of subareolar ducts smokers subareolar mass with nipple retraction
107
What is the diagnosis? Arising from pre-existing Barrett esophagus; usually involves lower 1/3 of esophagus lymph node spread commonly celiac and gastric; Malignant proliferation of glands
Adenocarcinoma of the esophagus Most common esophageal carcinoma of the West
108
Marfan syndrome is a risk for aortic dissection starting in what area of the aorta?
Marfan syndrome is a risk for aortic dissection starting in a dilated ascending aorta.
109
Ankylosing Spondylitis
HLA-B27 axial skeleton involvement seronegative spondylarthropathy more common in young men uveitis
110
What is a common cause of death in HD?
Suicide
111
What is a common cause of Budd-Chiari syndrome?
Hepatocellular carcinoma
112
Old tx Hep C?
Pegelated interferon and ribavarin
113
What is the diagnosis? Tumor that originates in the lining of the cavities that contain CSF; Most common site for this tumor is the 4th ventricle --\> creates an obstruction which leads to hydrocephalus; Not easily resectable
Ependymoma Cells have an "epithelial" appearance May also originate from the lining of the central canal of the spinal cord and the filum terminale
114
PAS + hyaline microthrombi in arterioles and capillaries, principally in the heart, brain and kidneys. This indicates what disorder?
TTP thrombotic thrombocytopenic purpura
115
CAP
Treatment
The treatment of choice for community-acquired pneumonia in a patient with no comorbidities is a macrolide (such azithromycin) or doxycycline.
116
The indirect antiglobulin (Coombs) test detects:
antibody in the plasma
117
Peripapical granuloma is the most common sequel to \_\_\_\_\_
Pulpitis Peripapical granuloma is chronically inflamed periapical granulation tissue
118
Renin secretion is inhibited by:
Aldosterone
119
Dx: Epigastric pain that worsens with meals
Gastric ulcer usually located on the lesser curvature of the antrum
120
What makes a gastric ulcer benign?
Small; punched out appearance; No heaping of mucosa around it or inflammation
121
Endometrial Carcinoma
most common invasive carcinoma of female genital tract postmenopausal bleeding endometrioid histology - hyperplasia pathway sporadic pathway - papillary serous adenocarcinoma - serous histology (surface epithelium) with psammoma bodies (laminated calcified concretions) p53 mutation common BRCA-1 mutation common
122
T/F: Smoking protects against ulcerative colitis.
True Smoking increases the risk for Crohn disease
123
What is the diagnosis?
Child with progressive decline in motor and sensory functions; X-linked disorder; Dysfunction of the adrenal cortex; Demyelination of the nervous system are associated with high levels of saturated very long-chain fatty acids in tissues and body fluid; Body quickly reduced to vegetative state
ALD - Adrenoleukodystrophy
Defect in peroxisomal membrane prevents the normal activation of free fatty acids by the addition of CoA --\> fatty acids accumulate in gangliosides and myelin
124
Chylothorax is a potential complication of what neoplastic process, resulting in widened mediastinum, dyspnea and thoracentesis that shows high protein content; microscopy shows many lymphocytes and fat globules?
Non-Hodgkin lymphoma with lymphatic obstruction
Disruption of the thoracic duct in the posterior chest is most likely to cause chylothorax, and malignant neoplasms, such as a non-Hodgkin lymphoma, are most likely to do this.
125
Give an example of a type III hypersensitivity rxn in the kidney.
SLE: immune-mediated glomerular injury with antigen-antibody complex deposition --\> decreased levels of complement --\> nephritis
126
What is the most common cause of progressive hearing loss in the elderly
Osteosclerosis
127
A 61-year-old woman has noted increasing dyspnea and a nonproductive cough for 5 months. On physical examination, her temperature is 37.7° C. A chest radiograph shows prominent hilar lymphadenopathy with reticulonodular infiltrates bilaterally. A transbronchial biopsy is performed, and the microscopic findings include interstitial fibrosis and small, noncaseating granulomas. One granuloma contains an asteroid body in a Langhans giant cell. The medical history indicates that she smoked cigarettes for 10 years, but stopped 5 years ago. What is the most likely cause of her illness?
T-cell mediated response to unknown antigen The clinical and morphologic features strongly suggest sarcoidosis. This granulomatous disease has an unknown cause, but the presence of granulomas and activated T cells in the lungs indicates a delayed hypersensitivity response to some inhaled antigen. Lung involvement, occurring in about one third of cases, may be asymptomatic or may lead to restrictive lung disease. Sarcoidosis can involve multiple organs, particularly those of the mononuclear phagocyte system, especially lymph nodes.
128
Avascular Necrosis
DCS ischemic necrosis of bone, bone marrow can also be caused by trauma children and adolescents can be affected
129
What is the effect of the following teratogen?
Isotretinoin
Spontaneous abortion;
hearing and visual impairment
130
t(11;14) translocation, in which the cyclin D1 gene (BCL1) is overexpressed, is seen in:
Mantle cell lymphoma B-cell lymphoma, more aggressive than follicular lymphoma
131
Incubation period Heb A:
3-4 wks Prodrome is low grade temp, nausea, anorexia, fatigue, mylagia, malaise
132
Persistent epidermal hyperplasia; chronic, runs in families; erythematous, scaly plaques, commonly on the dorsal extensor cutaneous surfaces; \*Neutrophils\* migrate into epidermis
Psoriasis
133
Most common cause of death from ALS
respiratory insufficiency ALS is a degenerative disease of motor neurons of the brain and spinal cord (atrophy of ventral roots, motor cortex, and motor nuclei in brainstem) that results in weakness and wasting of the muscles of the hands (can be unilateral) accompanied by fasciculations and pain from muscle wasting, rapid decline and death, usually in 6 years or less
134
Children with medulloblastoma are first seen with what neurologic symptoms?
Cerebellar dysfunction (gait and balance); Hydrocephalus
135
What is the most common source of group B strep and E coli infection that leads to meningitis in neonates
exposure in vaginal canal during delivery group B strep is tested in 3rd trimester for prophylaxis
136
Dx: Longstanding cholelithiasis; Rokitansky-Aschoff sinuses; Vague RUQ pain (esp after eating)
Chronic cholecystitis
137
Name the dx: Most common tumor of the thyroid; Patient is euthyroid; Benign; Isolated small nodule; Proliferation of thyroid follicles
Follicular adenoma
138
Name the dx: Verrucae or condylomata; Can be on the shaft of the penis; Epidermal hyperkeratosis, paraketasosis, acanthosis, papillomatosis
HPV (genital warts); Can cause penile cancer
139
Hemolytic Uremic Syndrome is classically seen in children with what infection?
E. Coli O157:H7 - dysentery from undercooked beef Verotoxin damages endothelial cells --\> HUS --\> Microangiopathic Hemolytic Anemia \*Renal insufficiency common in HUS
140
Reidel thyroiditis
Riedel thyroiditis is characterized by **destructive thyroid fibrosis that can cause a hypothyroid state** in the patient.
The dense fibrosis can extend into the adjacent tissues, producing neck tightness or pressure, dyspnea, dysphagia, hoarseness, choking, and coughing.
Riedel thyroiditis may **mimic malignancy and usually produces "rock-hard," painless goiter.**
Patients with Riedel thyroiditis can also have fibrosclerotic disease in other organs including pancreas, liver, kidney, and salivary gland tissue.
141
Give the dx: Proteinuria from effacement of the visceral epithelial cell foot processes; NO morphologic abnormalities seen by light microscopy; most common cause of nephrotic syndrome in children; Fusion of foot processes can be seen on EM;
Minimal change disease (aka lipoid nephrosis); Treat with corticosteroids; Will not progress to renal failure
142
Jarisch-Herxheimer reaction
The Jarisch-Herxheimer reaction is characterized by an acute febrile reaction that is often associated with headache and myalgias within the first 24 hoursof treatment.
Endotoxin (bacterial lipopolysaccharide) causes its pathology by the direct stimulation of macrophages, which results in the overproduction of macrophage mediators such as interleukin (IL)-1, IL-6, and tumor necrosis factor (TNF)-alpha.
Endotoxin is bound to macrophages by CD14, the endotoxin receptor. **IL-1 and IL-6 are pyrogens, responsible for fever (IL-1) and the acute-phase response (IL-6)**.
143
Occlusion of the vertebral artery results in infarcts in what part of the brain?
Cerebellum
144
Raised, erythematous nodular lesions; often on anterior shins; commonly associated with coccidiomycosis
Erythema nodosum
145
A positive P-ANCA (MPO) result suggests what disease?
A positive P-ANCA (MPO) result suggests microscopic polyangiitis
146
Name the dx: Congenital absence of testes.
Anorchia
147
Scalded child syndrome is caused by:
staph aureus exfoliatin toxin
148
Dx: "Leather bottle" appearance of thickened stomach; no true tumor seen; Extensive fibrosis in the submucosa and muscularis of the stomach wall; Signet ring cells
Linitis plastica (when the entire stomach is involved) - gastric adenocarcinoma Poor prognosis
149
Give the dx: Facial edema; Headaches, malaise, dizziness; Oliguria; Hematuria; HTN; Usually seen in children who have recently been ill; Subepithelial and subendothelial depositsof complement that look like "humps"
Nephritic syndrome from poststreptococcal glomerulonephritis
150
Nitrite positive sources of UTI
Enterobacter sp.
Escherichia coli
Klebsiella pneumoniae
and Proteus mirabilus
can cause urinary tract infections and are members of the Enterobacteriaceae, which would be nitrite-positive
151
Genetic (AD); mild hyperkeratosis and reduced or absent keratohyaline granules in the epidermis; scaly skin results from increased cohesiveness of the stratum corneum
Ichthyosis vulgaris
152
CD3 is a:
T-cell marker
153
Dx: Fistula formation between gall bladder and small bowel; Gallstone enters and obstructs the small bowel
Gallstone ileus
154
A 3-year-old child from Osaka, Japan, has developed a fever and a rash and swelling of her hands and feet over the past 2 days. On physical examination, her temperature is 37.8° C. There is a desquamative skin rash, oral erythema, erythema of the palms and soles, edema of the hands and feet, and cervical lymphadenopathy. The child improves after a course of intravenous immunoglobulin therapy. What is most likely to be a complication of this child’s disease if it is untreated?
Mucocutaneous lymph node syndrome, or Kawasaki disease, involves large, medium-sized, and small arteries. Cardiovascular complications occur in 20% of cases and include thrombosis, ectasia, and aneurysm formation of coronary arteries.
155
Malignant lymphoma of the testes occurs in what population?
\> 60 years; usually in the context of systemic disease (not usually primary); poor prognosis
156
Dx: Acidic damage to mucosa due to imbalances between mucosal defenses and acidic environment
Acute gastritis
157
Congenital failure of cerebellar vermis to develop resulting in massively dilated 4th ventricle very diminished or absent cerebellum; often accompanied by hydrocephalus.
Dandy-Walker malformation
158
TdT is a marker of:
pre–T cells and pre–B cells
159
Must test what to dx Hep C?
Hep C Ab; can't tell from AST/ALT because it's normal
160
What is the diagnosis? Malignant, high-grade astrocyte tumor; "Butterfly" lesion because tumor arises in the cerebrum and crosses corpus callosum; tumor cells are GFAP positive
Glioblastoma Multiforme
161
A 50-year-old man cuts his right index finger on a sharp metal shard while cleaning debris out of the gate in an irrigation canal. The cut stops bleeding within 3 minutes, but 6 hours later he notes increasing pain in the right arm and goes to his physician. On physical examination, his temperature is 38° C. Red streaks extend from the right hand to the upper arm, and the arm is swollen and tender when palpated. Multiple tender lumps are noted in the right axilla. A blood culture grows group A β-hemolytic streptococcus. What process is occurring in this patient’s right arm?
Lymphangitis
The red streaks represent lymphatic channels through which the acute infection is draining to axillary lymph nodes, and these nodes drain to the right lymphatic duct and into the right subclavian vein (lymphatics from the lower body and left upper body drain to the thoracic duct).
162
Give the diagnosis for the following auto-antibody:
c-ANCA (PR3-ANCA)
Granulomatosis with Polyangiitis
163
DM causes what kidney changes?
Nodular hyaline mesangial deposits - diabetic nephropathy A diabetic patient with nephrotic syndrome is likely to have nodular (and diffuse) glomerulosclerosis or diffuse thickening of the basement membrane.
164
What is the diagnosis? Nonbacterial vegetations on the undersurface of the mitral valve; Fibrinoid necrosis of small vessels; Focal degeneration of interstitial tissue
Libman-Sacks endocarditis; Associated with SLE ANA+ anti-DS-DNA+
165
Syphilis (positive VDRL test result) produces endarteritis obliterans of the:
Syphilis (positive VDRL test result) produces endarteritis obliterans of the aortic vasa vasorum, which weakens the wall and predisposes to aortic aneurysm formation.
166
An epidemiologic study shows that a highly pathogenic strain of influenza A virus with the antigenic type H5N1 that normally causes disease in birds has been increasingly found to cause influenza in humans. Unlike other strains of influenza A virus, this H5N1 virus is associated with a 60% mortality rate. The enhanced pathogenicity of this avian flu virus is primarily due to mutation in its genome that enables it to do what?
Enter many types of host cells H5N1 virus has much broader tissue tropism because its hemagglutinin can be cleaved by proteases present in many tissues.
167
What is the diagnosis? Malignant tumor composed of mucinous and squamous cells; Most common malignancy of the salivary gland; Involves facial nerve; Usually arises in parotid
Mucoepidermoid Carcinoma
168
A 46-year-old man has noted increasing abdominal enlargement over the past 15 months. Physical examination shows multiple skin lesions on the upper chest that have central pulsatile cores and measure, from core to periphery, 0.5 to 1.5 cm. Pressing on a core causes a radially arranged array of subcutaneous arterioles to blanch. Laboratory studies show serum glucose of 112 mg/dL, creatinine of 1.1 mg/dL, total protein of 5.8 g/dL, and albumin of 3.4 g/dL. What underlying diseases is most likely to be present in this patient?
Micronodular cirrhosis Spider telangiectasias are a feature of micronodular cirrhosis, typically as a consequence of chronic alcohol abuse. They are thought to be caused by hyperestrogenism (estrogen excess) that results from hepatic damage with reduced clearance of circulating steroids.
169
Charcot-Leyden crystals represent the breakdown products of \_\_\_\_\_\_\_\_.
The Charcot-Leyden crystals represent the breakdown products of eosinophil granules. Asthma, particularly extrinsic (atopic) asthma, is driven by a type I hypersensitivity response and is associated with an excessive TH2 and TH17 cell-mediated immune response. Genetic factors are important in the pathogenesis of atopic asthma and linkage to cytokine genes that map on 5q are strongly associated with development of asthma and other atopic allergies.
170
The development of polyneuropathy in patients with T2DM (and T1DM) results from:
Hyperglycemia
171
A Cushing ulcer (increased intracranial pressure) is a risk factor for:
Acute gastritis Cushing ulcer (increased intracranial pressure) --\> increased vagal stimulation
172
Name the dx: Large masses of the thyroid gland; Poorly circumscribed; Frequently extends into the soft tissue of the neck
Anaplastic carcinoma of the thyroid
173
What is the diagnosis? Degenerative disorder of the cerebellum and spinal cord; Subsequent ataxia; Loss of vibratory sense and proprioception, muscle weakness in the lower extremities, loss of deep tendon reflexes; Presents in early childhood; Complication is hypertrophic cardiomyopathy
Friedreich Ataxia Autosomal recessive; expansion of unstable trinucleotide repeat (GAA) in the frataxin gene
174
What Apo allele is associated with DECREASED risk for Alzheimer's Disease?
E2 E4 - increased risk
175
can cause death secondary to truama resulting in diffuse lesions/petechia in white matter
fat emboli - primarily affect capillaries - high density of capillaries at white matter/grey matter interface
176
Steroids have what role in Hep A tx?
None
177
Name the disorder characterized by:
1. Pathologic formation of platelet microthrombi in small vessels
2. Schistocytes
3. Associated with thrombotic thrombocytopenic purpura (TTP) and hemolytic uremic syndrome (HUS)
4. Labs: Thrombocytopenia Increased bleeding time Normal PT/PTT Anemia w/ schistocytes
5. High megakaryocytes in bone marrow
Microangiopathic Hemolytic Anemia
178
Name the dx: Soft, shiny, erythematous plaques on the glans and foreskin
Erythroplasia of Querat A carcinoma in situ of the penis
179
Vesicles that rupture and result in shallow painful red ulcers - often occurring on the lips. What is it what causes it?
Reactivation of HSV-1 inefection that caused by stress or exposure to sunlight
180
T/F: Diffuse large-cell lymphoma of B cells often involve extranodal sites.
True aggressive but responsive to chemotherapy;
181
An experiment studies early atheroma development. Lipid streaks on arterial walls are examined microscopically and biochemically to determine their cellular and chemical constituents and the factors promoting their formation. Early lesions show increased attachment of monocytes to endothelium. The monocytes migrate subendothelially and become macrophages; these macrophages transform themselves into foam cells. What substance is most likely to be responsible for the transformation of macrophages?
Oxidized LDL --can be taken up by a special “scavenger” pathway in macrophages; it also promotes monocyte chemotaxis and adherence. Macrophages taking up the lipid become foam cells that begin to form the fatty streak. Smoking, diabetes mellitus, and hypertension all promote free radical formation, and free radicals increase degradation of LDL to its oxidized form. About one third of LDL is degraded to the oxidized form; a higher LDL level increases the amount of oxidized LDL available for uptake into macrophages.
182
Name the dx: Most frequent cause of hyperthyroidism in young adults; Diffuse goiter, exopthalmous; stimulating IgG antobodies that bind to TSH receptors on the pm of thyrocytes
Graves disease
183
Auer rods are seen in myeloblasts in:
AML
184
The most common sites for ___________ are the thyroid (in Hashimoto thyroiditis), the salivary glands (in Sjögren syndrome), or the stomach (in Helicobacter pylori infection).
MALT - marginal zone - lymphoma
185
What is the dx?
Increasing night blindness; Constriction of the peripheral visual field; Loss of retinal photoreceptors; Pigment accumulation within the retina
Retinitis pigmentosa
runs in families
186
How do children with Reye syndrome present?
Hypoglycemia; Elevated liver enzymes; Nausea and vomiting
187
What is the likely diagnosis? skin eruptions; frequent recent ear infections; bone lesions with "Birbeck" granules in histiocytes; seen in children; exophthalmous sometimes seen, leading to diabetes insipidus from pressure on pituitary stalk
Langherhans cell histiocytosis
188
Lesion on dorsal surfaces of hands or feet; 1 or multiple; no malignant potential; Papules bleed easily; Epithelial-lined fronds with fibrovascular cores
Verruca vulgaris - common wart; HPV-2, HPV-4, other HPVs
189
"smudge cells" think
CLL chronic lymphocytic leukemia
190
Name the diagnosis: HTN, hypernatremia and hypokalemia due to aldosterone secretion; Severe HA, weakness and dizziness
Conn syndrome
Adrenal cortical adenoma
191
Cause of compression of midbrain, CNIII, PCA, parasympathetic fibers that results in Duret hemorrhages, down and out dilation (mydriatic) with hemmoragic infarction of occipital lobe
Uncal herniation (medial temporal lobe pushes through tentorium cerebelli)
192
Leptomeningitis refers to an inflammatory process localized to the:
pia and arachnoid
193
Haptoglobin is:
a serum protein that binds to free hemoglobin. Ordinarily, circulating hemoglobin is contained within RBCs, but hemolysis can release free hemoglobin. The haptoglobin is used up as the amount of free hemoglobin increases.
194
Osteoporosis
reduction in trabecular bone mass DIP \> PIP involvement porous bone, fracture risk labs normal postmenopausal women --\> estrogen withdrawal --\> increased # of osteoclasts (type 1) OR, decreased osteoblast activity (type 2)
195
Ventricular fibrosis with ventricular wall thinning can be caused by what infection?
Chagas disease
196
What is the result of hyperinsulinemia secondary to insulin resistance in T2DM?
Downregulation of the number of insulin receptors on the plasma membrane.
197
Chronic gastritis is what type of hypersensitivity reaction?
Type 4 T-cell mediated damage antibodies against parietal cells or intrinsic factor
198
Anti–glomerular basement membrane (anti-GBM) disease Cryoglobulinemic vasculitis (CV) IgA vasculitis (Henoch-Scho ̈nlein) (IgAV) Hypocomplementemic urticarial vasculitis (HUV) (anti-C1q vasculitis) These all make up what kind of vasculitides?
Small vessel vasculitis Immune complex SVV
199
Pancytopenia; splenomegaly; peripheral blood smear reveals atypical lymphoid cells displaying tartare-resistant acid phophatase activity
Hairy cell leukemia; clonal B-cell proliferation of small to medium lymphocytes that exhibit lots of cytoplasm and hair-like protrusions on the cell membrane; bone marrow-spleen-liver; middle-aged to elderly, male:female ratio is 5:1
200
Central pontine myelinolysis is a rare demyelinating disorder of the pons seen in what population?
Alcoholics
201
Older patients with nontoxic goiter sometimes go on to develop:
Hyperthyroidism
202
Hydrocephalus in a neonate associated with seizures and periventricular calcifications. Histology shows pink intranuclear/intracellular inclusions. What was the cause?
Vertical transmission of Cytomegalovirus accross the placenta in utero. TORCH = Toxoplasmosis, Rubella, Cytomegalovirus, Herpes simplex
203
The most important histologic feature of Mycosis Fungoides (CTCL) is:
the presence of lymphocytes in the epidermis aka epidermotropism
204
Recurrent urinary tract infections with urea-splitting organisms such as Proteus can lead to formation of:
Magnesium ammonium phosphate stones known as "staghorn calculi"
205
Parietal epithelial cells line the Bowman capsule and may proliferate with severe glomerular injury to produce:
crescents
206
Ingestion of strong acids produces what kind of reaction?
Coagulative necrosis in the esophagus --\> protective eschar that limits injury and further chemical penetration Dx: chemical esophagitis
207
tumor derived from endothelial cells; painful, purple nodules on hands and/or feet; neoplastic cells, poorly demarcated lesion
Kaposi's sarcoma
208
Poliomyelitis due to poliovirus results in flaccid paralysis and muscle atrophy, fasciculations, impaired reflexes and a negative Babinski sign (lower motor neuron signs). Where is the damage in the spinal cord?
Degeneration of anterior motor horn
209
Name the diagnosis: Full-thickness defect of lip or palate; Failure of facial prominences to close.
Cleft lip and palate They usually occur together
210
The rapid plasma reagin test is used to diagnose \_\_\_\_\_\_
The rapid plasma reagin test is used to diagnose syphilis
211
A 33-year-old woman from Victoria, British Columbia, goes to the physician because of increasingly severe dyspnea, orthopnea, and swelling of the legs for the past 2 weeks. She has no previous history of serious illness or surgery. On physical examination, her temperature is 37.8° C, pulse is 83/min, respirations are 20/min, and blood pressure is 100/60 mm Hg. An ECG shows episodes of ventricular tachycardia. An echocardiogram shows right and left ventricular dilation, but no valvular deformities. An endomyocardial biopsy shows focal myocyte necrosis and lymphocytic infiltrate. What organism most likely caused the infection?
Coxsackie A virus Focal myocardial necrosis with a lymphocytic infiltrate is consistent with viral myocarditis. This is uncommon, and many cases may be asymptomatic. In North America, most cases are caused by coxsackieviruses A and B. This illness may often be self-limited. Less often, it ends in sudden death or progresses to chronic heart failure.
212
What is the diagnosis? Right ventricular hypertrophy and dilation secondary to pulmonary hypertension; often arises from COPD
Cor pulmonale (Pulmonary stenosis is a rare cause of cor pulmonale)
213
Fatal infantile disease associated with autosomal recessive diffeciency in galactocerebrocide beta-galactosidases
Krabbe disease
214
What is the diagnosis? Degenerative disease of the frontal and temporal cortex; Spares the parietal and occipital lobes
Pick Disease
215
Cotton-wool spots are seen in what patient populations?
Diabetics and patients with HTN Fluffy white patches on the retina
216
What are the risk factors for laryngeal carcinoma?
Cigarette smoking Alcohol use HPV 6 and 11 Majority on true vocal cords and are keratinizing SCC Presents with persistent hoarseness and cervical lymphadenopathy
217
Most invasive urothelial cell carinomas arise from:
carcinoma in situ
218
the presence of peroxidase-positive blasts (myeloblasts) filling the marrow indicates what disease?
AML
219
Uncommon causes of pancreatitis:
Trauma; Hypercalcemia (calcium is an activator of enzymes) and hyperlipidemia; Drugs; Mumps; Scorpion sting; Rupture of posterior duodenal ulcer
220
Name the diagnosis: Recurrent aphthous ulcers Genital ulcers Uveitis Immune complex vasculitis involving small vessels
Behcet's Syndrome
221
Give an example of a type II hypersensitivity rxn in the kidney.
Goodpasture syndrome, other anti-glomerular BM diseases
222
Biopsy, ie of stomach, reveals extranodal lymphocytic infiltrate invading glandular tissue; Cancer originates in mucosa-associated lymphoid tissue; H. Pylori-associated, commonly arise in the context of chronic inflammation or autoimmunity
MALT lymphoma/Marginal zone lymphoma cell proliferation of small to medium lymphocytes; appear to originate from marginal-zone B-cells
223
x-linked inborn error of metabolism high levels of long-chain fatty acids in tissues and body fluids dysfunctional adrenal glands and bilaterally symetric demyelination, gliosis, and perivascular lymphocytic infiltrates Disease is _____ caused by \_\_\_\_
Adrenoleukodystrophy caused by defective peroxisomes
224
Marked leukocytosis and immature myeloid cells in the peripheral blood can represent an exaggerated response to infection (leukemoid reaction), or it can be a manifestation of \_\_\_\_\_\_\_\_.
CML Normal maturation of myeloid cells in the marrow rules out CML.
225
Plexiform lesions of the lung are characteristic for \_\_\_\_\_\_\_\_\_
Plexiform lesions are characteristic for pulmonary hypertension.
226
Give the dx: Malaise and periorbital edema; Afebrile; Proteinuria but no hematuria or gluccosuria; Numerous oval fat bodies seen on u/a; elevated serum creatinine; no improvement on steroid therapy; 50% affected glomeruli
FSGS Manifests with nephrotic syndrome; May be linked with NPHS gene mutations
227
A 25-year-old man suffers a sudden cardiac arrest. He is resuscitated. On examination his vital signs are normal. Echocardiography shows that the left ventricle is normal but there is marked thinning with dilation of the right ventricle. MR imaging of his chest shows extensive fibrofatty replacement of the myocardium, but no inflammation. What is the most likely cause for his findings?
Cardiomyopathy Arrhythmogenic right ventricular cardiomyopathy (arrhythmogenic right ventricular dysplasia) is most likely an autosomal dominant inherited condition with abnormal desmosomal adhesion proteins in myocytes.
228
Osteoma
Gardner syndrome benign tumor surface of facial bones
229
Dx: Persistent herniation of the bowel into the umbilical cord; Failure of herniated intestines to return to the body cavity during development;
Omphalocele Covered by peritoneum and amnion (how you can tell the difference between omphalocele and gastroschisis)
230
Give the dx: WBC casts in the urine; Most commonly caused by E. Coli (gram-negative); Bacteriuria; Diabetic patients are at increased risk; Patients present with fever, chills, malaise and flank pain
Pyelonephritis
231
What kind of cell is abundant in parathyroid neoplasm?
Chief cell
232
Multiple sclerosis is associated with what HLA?
HLA-DR2
233
Name the dx: C-cell-derived medullary thyroid carcinoma and chromaffin cell-derived pheochromocytoma; Hirschsprung disease (congenital megacolon); Variety of neural crest tumors; Elevated serum levels of PTH possible causing fractures
MEN-2A aka Sipple syndrome Mutations in RET protooncogene
234
Krukenberg Tumor
metastatic mucinous tumor from GI cancer (gastric adenocarcinoma) involves both ovaries "signet ring" cell appearance pseudomyxoma peritonei - massive amounts of mucus in the peritoneum (from tumor of appendix)
235
Virus type Heb B
DS DNA enveloped
236
Name the responsible pathogen: Lobar pneumonia Productive cough with purulent sputum High percentage of neutrophils
Streptococcus pneumoniae
237
symptoms of hydrocephalus in children
vomiting, sleepiness/lethargy, irritability, downward deviation of the eyes (also called "sunsetting"), and seizures
238
What is the dx? Cotton-wool spots; Capillary closure; Microaneurysms; Retinal neovascularization
Diabetic retinopathy
Narrowing or occlusion of retinal arterioles from arteriosclerosis or platelet and lipid thrombi --\> ischemia
239
ZE syndrome causes:
Duodenal ulcers
240
Associate what diseases with Crohn disease?
Ankylosing spondylitis;
Sacrolitis;
Migratory polyarthritis;
Erythema nodosum;
Uveitis
241
What are teardrop RBC's?
Teardrop RBCs are misshapen RBCs that are seen when marrow undergoes fibrosis, as in myelofibrosis. Myelofibrosis with myeloid metaplasia is a myeloproliferative disorder that is also a stem cell disorder in which neoplastic megakaryocytes secrete fibrogenic factors leading to marrow fibrosis.
242
Hand-Schuller-Christian disease
young children (2-5)
proliferation of Langerhans cells
radiolucent bony lesions
lesion may produce exopthalmos
infiltration of the hypothalamic stalk
can lead to DI weepy skin lesions ie at hairline
243
Lethal complication of multiple myeloma:
renal failure
244
Development of recurrent hematuria after a viral illness in a child or young adult is typically associated with
IgA nephropathy A renal biopsy specimen will show diffuse mesangial proliferation and electron-dense deposits in the mesangium. In these patients, some defect in immune regulation causes excessive mucosal IgA synthesis in response to viral or other environmental antigens. IgA complexes are deposited in the mesangium and initiate glomerular injury.
245
Adenocarcinoma in situ (AIS) presents often as:
Single lesion Well differentiated Mistaken for pneumonia initially Often peripheral
246
When do tropinin I levels rise after MI?
Troponin I levels begin to increase at about the same time as CK and CK-MB, but remain elevated for 7 to 10 days.
247
Tense bullae
Bullous pemphigoid
248
Name the diagnosis corresponding to this autoantibody:
Anti-Jo-1, anti-SRP, anti-Mi-2
Polymyositis, Dermatomyositis
249
reddish plaque on tongue that cannot be scraped away . What is this? What is it a strong indicator of? Is it cancerous, pre-cancerous or benign?
Erythroplakia squamous cell dysplasia of the oral mucosa per-cancerous ==\> can lead to squamous cell carcinoma Alcohol and tobacco are biggest risk factors
250
Skin issue associated with gluten hypersensitivity
dermatitis herpetiformis
251
Poliovirus has tropism for what part of the brain?
Brainstem nuclei
252
A 50-year-old man has had a chronic cough for the past 18 months. Physical examination shows nasopharyngeal ulcers, and the lungs have diffuse crackles bilaterally on auscultation. Laboratory studies include a serum urea nitrogen level of 75 mg/dL and a creatinine concentration of 6.7 mg/dL. Urinalysis shows 50 RBCs per high-power field and RBC casts. His serologic titer for C-ANCA (proteinase 3) is elevated. A chest radiograph shows multiple, small, bilateral pulmonary nodules. A transbronchial lung biopsy specimen shows a necrotizing inflammatory process involving the small peripheral pulmonary arteries and arterioles. What is the most likely diagnosis?
GPA, Granulomatosis with polyangiitis Anti–neutrophil cytoplasmic autoantibody (ANCA)–associated vasculitis (granulomatosis with polyangiitis) is a form of hypersensitivity reaction to an unknown antigen characterized by necrotizing granulomatous inflammation that typically involves small to medium-sized vessels, although many organ sites may be affected. Pulmonary and renal involvement can be life-threatening. C-ANCAs (antibodies mainly directed against neutrophil proteinase 3) are found in more than 90% of cases.
253
Quadrivalent HPV vaccine
6, 11, 16, 18
254
Give an example of a type II crescentic GN.
Immune complex disease: Type II crescentic GN can occur in systemic lupus erythematosus, in Henoch-Schönlein purpura, and after infections.
255
What leads to injury to pancreatic cells in T1DM?
Cell-mediated immunity: Cytotoxic T lymphocytes sensitized to beta cells are the real reason for beta cell destruction. The antibodies come as a result of the damaged cells.
256
What is the diagnosis? Women; painless movable mass at the angle of the jaw; may become malignant epithelial cells intermixed with myxomatous and cartilaginous stroma
Pleomorphic adenoma
257
Lacunar stroke is due to:
Hyaline arteriosclerosis Most commonly involves the lenticulostriate vessels, resulting in small, cystic areas of infarction; involvement of the internal capsule = pure motor stroke; involvement of the thalamus = pure sensory stroke
258
Besides gout, in what diagnoses is hyperuricemia seen?
Leukemias and lymphomas that have a high proliferative rate and are treated with chemotherapy
259
Infection with the liver fluke Clonorchis sinensis predisposes to what kind of cancer?
Cholangiocarcinoma
260
version of Lagherhans Cell Histiocytosis seen in children 2-5; classic triad of: diabetes insipidus, proptosis, defects in membranous bones
Hand-Schuller-Christian disease typically multifocal and indolent otitis media is a common finding painful lytic bone lesions common
261
A 45-year-old man has had poorly controlled hypertension ranging from 150/90 mm Hg to 160/95 mm Hg for the past 11 years. Over the past 3 months, his blood pressure has increased to 250/125 mm Hg. On physical examination, his temperature is 36.9° C. His lungs are clear on auscultation, and his heart rate is regular. There is no abdominal pain on palpation. A chest radiograph shows a prominent border on the left side of the heart. Laboratory studies show that his serum creatinine level has increased during this time from 1.7 mg/dL to 3.8 mg/dL. What vascular lesions is most likely to be found in this patient’s kidneys?
Necrotizing arteriolitis Malignant hypertension can suddenly complicate and be superimposed on less severe, benign essential hypertension. The arterioles undergo concentric thickening and luminal narrowing with malignant hypertension, called hyperplastic arteriolosclerosis, and fibrinoid necrosis is a prominent feature.
262
What is the diagnosis? Congenital downward displacement of cerebellar vermis and tonsils through the foramen magnum; Obstruction of CSF flow commonly results in hydrocephalus; Often seen with meningomyelocele
Arnold-Chiari Malformation (Type II)
263
What is the most common cause of bacterial meningitis in neonates?
Group B strep, E coli, Listeria monocytogenes
264
Oval, uniformly pigmented macular lesion
Junctional nevus
265
Erythematous, annular plaque
Granuloma annulare
266
Name the diagnosis corresponding to this autoantibody:
Antimitochondrial
Primary biliary cirrhosis
267
T/F ALS usually appears in mid-adulthood and is typically not caused a genetically inherited mutation
True -- SOD1 mutation is present in a fraction of familial cases but these are a vast minority of total ALS cases
268
How are neural tube defects usually detected during pregnancy?
elevated AFP levels in amniotic fluid or maternal blood
269
What is the treatment for pyloric stenosis?
Myotomy
270
What is the diagnosis? Atrophy of type I muscle fibers; Hypertrophy of type II muscle fibers
Myotonic dystrophy
271
Antiphospholipid antibodies pose a risk for \_\_\_\_\_\_\_.
Antiphospholipid antibodies pose a risk for thrombosis. Over half of persons with chronic pulmonary thromboembolism with pulmonary hypertension do not have a history of recurrent pulmonary embolism.
272
Dohle bodies or toxic granulation in mature neutrophils on peripheral blood smear
Leukemoid reaction Benign alk phos HIGH in leukemoidrxn, LOW in CML CML would not have mature neutrophils....
273
What differentiates neurofibromatosis type I vs type II?
Both have neurofibromas but only type 2 has acustic neuromas - intracranial shwannoma of the 8th cranial nerve
274
Primary Sclerosing Cholangitis carries increased risk for what cancer?
Cholangiocarcinoma (bile ducts)
275
Symmetrically smaller kidneys are seen in:
Chronic GN; often no inciting cause; surfaces of kidneys become granular; HTN develops due to renal ischemia
276
45 YO chinese pt. presents with swollen cervical lymph node, blood in saliva, bloody discharge from the nose, nasal congestion, hearing loss, frequent ear infections, headaches. What is a likely diagnosis? What might have caused this?
nasopharyngeal carcinoma secondary to EBV infection
277
What is the diagnosis? Paraneoplastic disorder (usually associated with small cell carcinoma of the lung; Muscle weakness/wasting/fatigability; Responds to corticosteroid treatment;
Lambert Eaton Myasthenic Syndrome aka myasthenic myopathic syndrome
278
A 20-year-old, previously healthy man is jogging one morning when he trips and falls to the ground. He suddenly becomes markedly short of breath. On examination in the emergency room there are no breath sounds audible over the right side of the chest. A chest radiograph shows shift of the mediastinum from right to left. A chest tube is inserted on the right side, and air rushes out. What underlying disease is most likely to have produced this complication?
Distal acinar emphysema Distal acinar (paraseptal) emphysema is localized, beneath pleura typically in an upper lung lobe, and may occur in an area of fibrosis or scar formation. Although the lesions are usually less than 2 cm in diameter, they are prone to rupture spontaneously or with minor trauma, leading to pneumothorax. They can be a cause for spontaneous pneumothorax in young adults. A “ball valve” effect can lead to air trapping in pleura, producing tension pneumothorax, as in this case.
279
What is the diagnosis? Focal demyelination of the pons; Can result in "locked-in" syndrome (bilateral paralysis); Can be due to rapid correction of hyponatremia;
Central Pontine Myelinolysis
280
Bronchial dilation with inflammatory destruction is a feature of \_\_\_\_\_\_\_\_\_.
Bronchial dilation with inflammatory destruction is a feature of bronchiectasis.
281
Sertoli-Leydig cell tumor
hirsutism virilization sex-cord stromal tumors
282
Defining histological change in the brain from ALS
loss of motor neurons, atrophy of motor cortex, gliosis
283
HPV E6 protein
increased destruction of p53
284
Atypical lymphocytes are characteristic in the peripheral blood of individuals with:
infectious mononucleosis
285
Lumbar puncture in the diagnosis of MA would reveal?
Increased lymphocytes; Myelin basic protein; Increased immunoglobulins with oligoclonal IgG bands on high resolution electrophoresis
286
Uncorrected VSD carries what complication?
Pulmonary HTN
287
What is the main culprit of meningitis in newborns?
E. Coli
288
What is the diagnosis? Syndrome; Bilateral tumors of the 8th cranial nerve (acoustic neuromas); meningiomas and gliomas common;
Neurofibromatosis type 2
289
Dx: Serrated appearance on microscopy; Most common type of polyp; Usually in L colon (rectosigmoid); Benign
Hyperplastic polyp
Hyperplasia of glands
Cancerous = adenomatous polyp
290
Common causes of pancreatitis
Alcoholism; Gallstones from contraction of sphincter of Oddi
291
Neuroendocrine tumors such as carcinoid tumors are positive for:
Chromogranin
292
hereditary disorder in which cerebellar hemangioblastomas are associated with retinal angiomas and other turmors like renal cell carcinoma
von-hippel lindau syndrome
293
What is the diagnosis? Longitudinal laceration of mucosa at the GE junction; Caused by severe vomiting (alcoholism, bulimia implicated here); \*Painful\* hematemesis
Mallory-Weiss syndrome risk of Boerhaave syndrome due to laceration: rupture of esophagus leading to air in the mediastinum and subcutaneous emphysema
294
What is the dx? Fundoscopy reveals flame-shaped hemorrhages; elevated intraocular pressure
Retinal occlusive vascular disease - central retinal vein occlusion
295
What would you expect to see on urinalysis of a patient with SLE and nephritic syndrome?
RBC casts, RBCs
296
The lymphocyte depletion variant of Hodgkin lymphoma has what features?
an abundance of Reed-Sternberg cells and a paucity of lymphocytes.
297
15% of patients with myasthenia gravis have \_\_\_\_\_\_
thymoma 1/3 pf patients with thymoma will develop MG
298
What is the diagnosis? Impaired addition of Co-A to long chain fatty acids; X-linked
Adrenoleukodystrophy Accumulation of fatty acids damages adrenal glands and white matter of the brain.
299
Name the diagnosis: Necrotizing vasculitis, with few or no immune deposits, predominantly affecting small vessels (i.e., capillaries, venules, arterioles, and small arteries), associated with myeloperoxidase (MPO) ANCA or proteinase 3 (PR3) ANCA. Not all patients have ANCA. Add a prefix indicating ANCA reactivity, e.g., MPO-ANCA, PR3-ANCA, ANCA- negative.
ANCA-associated vasculitis (AAV)
300
Give the dx: Malaise, nausea and reduced urine output for 3 days; Afebrile, normotensive; creatinine 7.5; Hematurea, no pyuria or glucosuria; renal biopsy shows crescents
Rapidly progressive GN Crescentic GN is divided into three groups on the basis of immunofluorescence: type I (anti–glomerular basement membrane [GBM] disease); type II (immune complex disease); and type III (characterized by the absence of anti-GBM antibodies or immune complexes).
301
Effacement of the foot processes seen in minimal change disease results in:
Selective proteinuria for low molecular weight proteins, for example, albumin
302
Rhabdomyosarcoma
most common malignant soft tissue tumor in children rhabdomyoblast desmin + head and neck common, vagina in young girls common
303
Name the disease associated with the presence of hairy projections from neoplastic leukocytes in the peripheral blood smear and coexpression of B-cell (CD19, CD20) and monocyte (CD11c) markers.
Hairy cell leukemia B-cell leukemia
304
Linear array of suppurating subcutaneous nodules
lymphocutaneous sporotrichosis
305
Complications of acute pancreatitis
Shock; DIC ARDS Pancreatic abscess Pancreatic pseudocyst
306
What is the diagnosis? "Sulfur granules" in pus contain gram + branching filamentous anaerobic bacteria ; often follows extraction of an abscessed tooth
Cervicofacial actinomycosis Actinomyces israelii
307
The CD30 antigen is a marker for:
activated T and B cells
308
Dx: Congenital malformation of the abdominal wall, leads to exposure of abdominal contents
Gastroschisis
309
Fine needle aspirate of bone reveals numerous eosinophils; Otitis media is a common finding; scalp bone pain; Calvarial bone defects; Exopthalmous possible
Langerhans cell histiocytosis
310
What is the diagnosis? Baby turns blue when breast feeding, cannot breathe through nose
Choanal Atresia Can be unilateral or bilateral or membranous septum between nose and pharynx
311
What is the exotoxin responsible for the development of poststreptococcal glomerulonephritis?
Pyogenic exotoxin B (SpeB) Most children with poststreptococcal GN recover, although 1% develop a rapidly progressive GN characterized by crescent formation. Progression to chronic renal failure occurs in 40% of affected adults.
312
Thecoma
postmenopausal women functional ovarian tumor estrogen production yellow on gross exam lipid-laden theca cells endometrial hyperplasia common, can progress to cancer
313
Necrosis of pyrimidal neurons of Sommer's sector in the hippocampus occurs as a consequence of:
global anoxia
314
What is a complication of repeated bouts of rhinitis?
Nasal polyps
315
What is the diagnosis? Benign tumor of astrocytes; Usually arises in the cerebellum; Imaging reveals a cystic lesion with a mural nodule; Biopsy shows Rosenthal fibers; Cells are GFAP positive
Pilocytic astrocytoma
316
Name the dx: Seen in children; Edematous, mucosal, polyploid masses with a "cluster of grapes" appearance;
Embryonal rhabdomyosarcoma aka sarcome botryoides
317
An 8-year-old child has had abdominal pain and dark urine for 10 days. Physical examination shows blotchy purple skin lesions on the trunk and extremities. Urinalysis shows hematuria and proteinuria. Serologic test results are negative for MPO-ANCA (P-ANCA) and PR3-ANCA (C-ANCA). A skin biopsy specimen shows necrotizing vasculitis of small dermal vessels. A renal biopsy specimen shows immune complex deposition in glomeruli, with some IgA-rich immune complexes. What is the most likely diagnosis?
In children, Henoch-Schönlein purpura is the multisystemic counterpart of the IgA nephropathy seen in adults. The immune complexes formed with IgA produce the vasculitis that affects mainly arterioles, capillaries, and venules in skin, gastrointestinal tract, and kidney.
318
A 3-year-old child is developing normally. Physical examination reveals a low-pitched cardiac murmur. An echocardiogram shows the presence of an ostium secundum, with a 1-cm defect. What abnormality is most likely to be found in this child?
Left to right shunt A persistent ostium secundum is the most common form of atrial septal defect. Because atrial pressures are low, the amount of shunting from the left atrium to the right atrium is small, and this lesion may remain asymptomatic for many years. Eventually, pulmonary hypertension can occur, with reversal of the shunt.
319
What is the dx? Anterior chamber angle is open and appears normal; Characteristic excavation of the optic nerve head; Progressive loss of visual field sensitivity; Increased ocular pressure; Degenerative changes in the retina;
Open-angle glaucoma No other underlying ocular disease
320
papillary cystadenoma lymphomatosum and adenolymphoma other names for what? Why is it helpful to remember this?
Warthin's tumor - useful because cystic glandular benign tumor (cystadenoma) with lymph node-like stroma consisting of abundant lymphocytes and germinal centers (lymphomatosum ). Cystic spaces are surrounded by two uniform rows of cells with centrally placed pyknotic nuclei papillary - b/c epithelium surrounding cystic spaces exhibits "papillary infolding" adenolymphoma - useful to remember because neoplasm of glandular tissue (adenoma) with lymph-node like structures (lymphoma) \*only type of salivary gland tumor more common in men than women, 15% of cases are bilateral
321
Sjogren's syndrome
Rheumatoid arthritis is often associated with other autoimmune disorders, including Sjögren syndrome.
Sjögren syndrome presents with dry eyes and a dry mouth (lack of saliva production is involved in the development of caries).
Sjögren syndrome is diagnosed with a **positive SS-A (Ro) and SS-B (La) antibody titer.**
322
What is the serum tumor marker for hepatocellular carcinoma?
Alpha fetoprotein
323
Give the dx: GN; Prominent ribbon-like deposits along the lamina densa of the glomerular BM; Hematuria, proteinuria
Dense deposit disease Half of all cases end in chronic renal failure
324
PDA causes what kind of heart murmur?
A patent ductus arteriosus causes a shrill systolic murmur.
325
Spontaneous abortion
most common is trisomy 16, hypercoagulable state, congenital infection, teratogen exposure usually in first 2 weeks gestation
326
Congenital downward displacement of cerebellar vermis and tonsils through the foramen magnum that can obstruct CSF flow and cause hydrocephalus - very commonly associated with meningomyelocele
Arnold-Chiari malformation Type II meningomyelocele is protrusion of meninges and spinal cord in spinal bifida
327
What is the diagnosis? Autoimmune destruction of the salivary glands and lacrimal glands
Sjogren syndrome
328
Associate negri bodies with what diagnosis?
Rabies
tendency to aspirate fluids = "hydrophobia"
perivascular cuffing by lymphocytes
329
Mutations in ______ are related to development of pulmonary alveolar proteinosis.
Mutations in GM-CSF are related to development of pulmonary alveolar proteinosis.
330
Associate acanthocytosis or "spur cells" (RBCs look like suns with multiple projections coming out of them) with:
chronic liver disease; free cholesterol is deposited in the RBC membrane
331
What is the diagnosis? Mass of accumulated keratin and squamous mucosa that results from growth of squamous epithelium from the external ear canal through the perforated eardrum to the middle ear; Results from multiple bouts of otitis media
Cholesteatoma
332
Name the dx: thyroiditis featuring fibrosis and chronic inflammatory infiltrate; Progressive fibrosis in other locations possible; Patients complain of hoarseness or stridor;
Reidel thyroiditis
333
Charcot-Bouchard aneurysms can be seen in what diagnosis?
HTN cerebral lipohyalinosis --\> weakening of arterial walls --\> cerebral microaneurysms
334
Dx: Fibrosis of pancreatic parenchyma secondary to acute pancreatitis
Chronic Pancreatitis Alcoholics and CF patients OR idiopathic
335
Bradyarrhythmias less than 50/min suggest a disorder of what part of the heart?
Bradyarrhythmias less than 50/min suggest an SA node disorder. SA node dysfunction may worsen with cardioactive drugs, such as cardiac glycosides, β-adrenergic blockers, calcium channel blockers, and amiodarone.
336
Dx: Signet ring cells infiltrate gastric wall; Linitis plastica; Not associated with H. Pylori, intestinal metaplasia or nitrosamines
Diffuse type of gastric cancer NO ulcers, instead diffusely thickened wall of stomach
337
Name the dx: Ovulatory failure; Progsterone deficiency; Irregular and excessive menstrual bleeding; ED/oligospermia in men Mxedema; Mental status changes; Coarse, brittle hair; Muscle weakness
Autoimmune thyroiditis
338
Cervical Intraepithelial Neoplasia (CIN)
Koilocytic change looks like a mosaic, inlaid woodwork
carcinoma in situ is dysplasia involving the full thickness of the epithelium
progression from CIN I to CIS is not inevitable
339
Give the diagnosis for the following auto-antibody:
IgA antiendomysial, IgA anti-tissue transglutaminase
Celiac disease
340
Labs Hep D?
HD-Ag HD-sAg
341
Subdural hematoma is blood where?
UNDER the dura,
blood covering surface of brain;
presents with progressive neurologic signs;
trauma;
tearing of bridging veins between dura and arachnoid;
Herniation is a lethal complication
342
lymphocytic infiltrates at the dermal-epidermal junction; positive CD4+ immunohistochemical staining; scaly skin lesions/plaques
Mycosis fundoides - cutaneous T-cell lymphoma
343
What is the diagnosis? Squamous cell carcinoma, usually arises from the epithelial lining of the vocal cord; Risk factors are alcohol and tobacco
Laryngeal Carcinoma
344
Intensely pruritic cutaneous eruption; Wheal-like lesions with small vesicles Urticaria-like plaques and vesicles over extensor surfaces of the body; possible development of IgA antibodies to componenets of gluten
Dermatitis herpetiformis
345
Toxic granulations, which are coarse and dark primary granules, and Döhle bodies, which are patches of dilated endoplasmic reticulum, represent:
reactive changes of neutrophils that are most indicative of overwhelming inflammatory conditions, such as bacterial sepsis.
346
Give the dx: Serum positive for C-ANCA; Biopsy shows glomerular crescents and damage to small arteries; BUN:Creatinine about 10:1; ABSENCE of immune complexes of anti-GBM antibodies
GPA - granulomatosis with polyangiitis A Necrotizing granulomatous vasculitis Granulomatosis with polyangiitis (ANCA-associated vasculitis) is a cause for rapidly progressive glomerulonephritis (GN) characterized by epithelial crescents in Bowman space.
347
Centriacinar emphysema arises in respiratory bronchioles and is seen in what population?
Smokers
348
Name the diagnosis corresponding to this autoantibody:
Anti-cardiolipin, lupus anticoagulant
SLE
Antiphospholipid syndrome
349
frequently pigmented, elevated papules or plaques; scales easily rubbed off; microscopic: broad, anastomosing cords of mature stratified squamous epithelium with small cysrs of keratin (horn cysts)
seborrheic keratoses
350
Bowen disease
Bowen disease, which is one form of penile squamous carcinoma in situ, typically described as a **thick flat white lesion.**
The other forms of penile squamous cell carcinoma in situ are Bowenoid papulosis (multiple reddish-brown raised papular lesions) and erythroplasia of Queyrat (single or multiple shiny red plaques).
Untreated Bowen disease will, over a period of years, progress to frank invasive carcinoma in 10 to 20% of patients.
Bowen disease can affect the genital region of both men and women.
351
What lab values are commonly seen in nonalcoholic fatty liver disease?
ALT \> AST
352
LFTs in Hep A
ALT\>AST before jaundice (then 500-600s) ALT is more liver specific
353
Inflammation of the salivary gland usually due to an obstructing stone and subsequent staph aureus infection; usually unilateral
sialadenitis
354
Name the diagnosis: Necrotizing vasculitis, with few or no immune deposits, predominantly affecting small vessels (i.e., capillaries, venules, or arterioles). Necrotizing arteritis involving small and medium arteries may be present. Necrotizing glomerulonephritis is very common. Pulmonary capillaritis often occurs. Granulomatous inflammation is absent.
Microscopic polyangiitis
355
Bence-Jones proteinuria is characteristic of:
Multiple myeloma Amyloid deposition enlarges these kidneys
356
Why do some newborns develop jaundice?
Liver is still developing, cannot conjugate bilirubin fast enough
357
What is the diagnosis?
Benign tumor composed of stromal (cartilage) and epithelial tissue;
Most common tumor of the salivary gland;
Arises in parotid;
Mobile, painless, well-circumscribed mass at the angle of the jaw.
Pleomorphic adenoma
rarely turns into carcinoma;
high rate of recurrence (prob due to not excising enough)
358
Pregnant patients with diabetes may give birth to babies with what complication?
Hypoglycemia; increased birth weight
359
Upper lobe cavitation suggests what infection?
Upper lobe cavitation suggests secondary tuberculosis.
360
Give the diagnosis: Necrotizing arteritis of medium or small arteries without glomerulonephritis or vasculitis in arterioles, capillaries, or venules, and not associated with ANCA
Polyarteritis nodosa Medium artery necrotizing arteritis seen on biopsy, negative ANCA, no MCLNS, and no evidence of glomerulonephritis
361
A Krukenberg tumor is commonly seen with what cancer?
Diffuse type of gastric carcinoma distant metastases are bilateral ovaries
362
X-linked (Bruton) agammaglobulinemia
X-linked (Bruton) agammaglobulinemia:
Caused by a **block in the B-cell maturational process** due to mutations in **Bruton tyrosine kinase (btk)**
B-cell maturation stops at the point and cells will not develop beyond the point at which they can produce cytoplasmic mu heavy chains.
363
Give the dx: Deafness; Corneal erosions (lens dislocation possible); Hematuria; Inherited defects in BM collagen
Alport syndrome Proteinuria may be in the nephrotic range; Chronic renal failure in adulthood
364
Mutations involving NADPH oxidase lead to immunodeficiency in what disease?
chronic granulomatous disease
365
Chondroblastoma
chicken wire appearance adolescents primitive chondroblasts and cartilage matrix
366
What is the Diagnosis? Malignant tumor of ependymal cells; Usually seen in children; Commonly arises in the 4th ventricle --\> can present with hydrocephalus; Perivascular Pseudorosettes on biopsy
Ependymoma
367
Most common infectious agent in acute suppurative tonsilitis?
strep pyogenes
368
Thiazolidinediones
Thiazolidinediones, such as pioglitazone, decrease triglycerides, increase HDL cholesterol, decrease C-reactive protein levels, and decrease serum glucose.
They are associated with the development of heart failure, hepatotoxicity, and weight gain.
369
Name the offending organism associated with these symptoms: Mild fever Nonproductive cough HA Myalgias 1 week duration CXR shows extensive bilateral patchy infiltrates Cold agglutinin titer is elevated
This primary atypical pneumonia is caused by Mycoplasma pneumoniae, a cell wall–deficient organism that is difficult to culture. Often, a diagnosis is made empirically. The findings are similar to those of other viral infections, and serologic testing shows the specific organism.
370
Reactive arthritis
HLA-B27 arthritis, urethritis, conjunctivitis Chlamydia or GI infection
371
\_\_\_\_\_\_\_ are present early in the pathogenesis of atherosclerotic lesions and are believed to activate monocytes, endothelial cells, and smooth muscle cells by secreting cytokines.
T-cells T cells are present early in the pathogenesis of atherosclerotic lesions and are believed to activate monocytes, endothelial cells, and smooth muscle cells by secreting cytokines. T cells adhere to VCAM-1 on activated endothelial cells and migrate into the vessel wall. These T cells, activated by some unknown mechanism, secrete various proinflammatory molecules that recruit and activate monocytes and smooth muscle cells and perpetuate chronic inflammation of the vessel wall.
372
Name two cell-markers (suggestive of medulloblastomas, ependoma or pineal parenchymal tumors) that rule out lymphoma, metastatic epithelial tumor or neuroectodermal malignancy
synaptophysin or nestin
373
T/F: The risk of acute cystitis in females is higher in pregnancy.
True
374
In carcinoid syndrome, why does only the right heart develop fibrosis?
Because the serotonin in the circulation goes to the lung and is converted to 5-HIAA by monoamine oxidase which is nontoxic to the heart
375
actinic keratoses indicative of:
precursor to squamous cell carcinoma atypia in basal keratinocytes
376
Name the two large vessel vasculitides.
Giant cell arteritis Takayasu arteritis
377
What is the diagnosis? Vermilion border on the lower lip
Leuko/erythroplakia
378
Goodpasture syndrome
Goodpasture syndrome, a form of anti-glomerular basement antibody disease (anti-GBM antibody disease), is characterized by **autoantibodies (usually IgG)** that are directed against the basement membranes found in the renal glomeruli and pulmonary alveoli. The antigen is a component of a non-collagenous domain of one of the chains of type IV collagen in the glomerular basement membrane. The immunologic reaction against the basement membranes predisposes for both **rapidly progressive (crescentic) glomerulonephritis and pulmonary hemorrhage**.
With immunofluorescent techniques, these antibodies can be detected as **linear deposits** following the basement membranes in both renal and pulmonary biopsies (see photomicrograph of immunofluorescence of a glomerulus in the kidney below). Goodpasture syndrome formerly had a dismal prognosis, with deaths related to either renal failure or pulmonary hemorrhage; however the prognosis has improved with intensive use of plasma exchange.
379
What causes DiGeorge syndrome?
Failure of development of the third and fourth branchial pouches resulting in agenesis or hypoplasia of the thymus and parathyroid glands, congenital heart defects, dysmorphic facies etc. Parathyroid agenesis --\> convulsions (hypocalcemia) Thymic aplasia --\> loss of T cells --\> deficiency of cell-mediated immunity, particular susceptibility to candida infections
380
Lysyl hydroxylase is required for cross-linking collagen, and its loss gives rise to one form of \_\_\_\_\_\_\_\_\_
Lysyl hydroxylase is required for cross-linking collagen, and its loss gives rise to one form of Ehlers-Danlos syndrome.
381
Give the dx: Eosinophils in the urine
Allergic nephritis
382
What is the likely diagnosis? high blood pressure; NO hepatosplenomegaly or lymphadenopathy; high platelet count; occult blood in stool; high Hgb and Hct; afebrile
polycythemia vera (PCV), a myeloproliferative disorder characterized by an increased RBC mass, with hematocrit concentrations typically exceeding 60%. Although the increased RBC mass is responsible for most of the symptoms and signs, these patients also have thrombocytosis and granulocytosis. This occurs because, similar to other myeloproliferative disorders, PCV results from transformation of a multipotent stem cell.
383
Thrombotic stroke is due to:
rupture of an atherosclerotic plaque Atherosclerosis commonly develops at branchpoints, for example the bifurcation of IC and MCA in the circle of willis; results in pale infarct at the periphery of the cortex
384
What is the diagnosis? Benign tumor of arachnoid cells; More common in women; May present as seizures; Histology shows a whorled pattern; Psammoma bodies may be present
Meningioma
385
Vulvar Carcinoma
HPV 16, 18, 31, 33
presents as leukoplakia
rare VIN (vulvar intraepithelial neoplasia)
cytokeratins - tumor markers can have **keratin pearls** on histo
**koilocytic change** on histology
386
Reduced lower esophageal sphincter tone is due to:
GERD Heartburn, asthma and cough, damage to enamel of teeth Late complications are Barrett esophagus and ulceration with stricture
387
What is the most common cause of death in cirrhotics?
Rupture of esophageal varices Arise secondary to portal HTN; Left gastric vein backs up into the esophageal vein, resulting in dilation Asymptomatic until/unless rupture Presents with \*painless\* hematemesis
388
What is the diagnosis? Failure to thrive; Respiratory insufficiency; Accumulation of cerebroside in the white matter of the brain and peripheral nerves; Accumulation of arylsulfate (no functioning arylsufatas)
MLD - Metachromatic Leukodystrophy inborn error of metabolism
389
Wernicke syndrome is secondary to thiamine deficiency. Atrophy of what parts of the brain would be seen on autopsy?
Mamillary bodies and hypothalamus Periaqueductal regions of the midbrain and the tegmentum of the pons
390
Goodpasture syndrome leads to renal and pulmonary lesions produced by an antibody directed against an antigen common to the basement membrane in glomerulus and alveolus. What kind of hypersensitivity reaction then ensues?
Type II
391
Bartholin Cyst
unilateral, painful lesion near vaginal canal
392
generally, megaloblastic anemias are caused by:
impaired DNA synthesis ie folic acid deficiency
393
consequence of chonric suppurative otidis media with rupture of typanic membrane
cholesteatoma
394
Name the disease: Bilateral inflamed parotid glands; Orchitis, pancreatitis, aseptic meningitis may also be present
Mumps \*Serum amylase is increased due to salivary gland or pancreatic involvement
395
T/F polio virus damages the anterior horn of the spinal cord producing lower motor neuron lesion symptoms
true - syndrome called poliomyelitis flaccid paralysis, muscle atrophy, fasciculations, weakness, decreased muscle tone, impaired reflexes, negative babinski sign
396
T/F: Primary disorders of hemostasis do not involve the coagulation cascade, therefore PT/PTT will be normal.
True
397
Granulosa-Theca Cell Tumor
Sex-cord stromal tumor
precocious puberty, but usually occurs in postmenopausal women
malignant but not aggressive
398
Give the diagnosis for the following auto-antibody:
Anti-TSH receptor
Graves disease
399
syndrome marked by recurrent aphthous ulcers, genital ulcers and uveitis What is it? What causes symptoms?
Bechet syndrome small vessel immune-complex vasculitis
400
Partial Thromboplastin Time (PTT) measures:
intrinsic factor (factors XII, XI, IX, VIII) and common pathways (factors II, V, X and fibrinogen) of the coagulation cascade
401
KCNQ1 mutations may be seen with what cardiac disease?
KCNQ1 mutations may be seen with long QT syndrome
402
In patients who develop adenocarcinoma of the prostate, what is the precursor?
Prostatic intraepithelial neoplasia
403
Flat-topped violaceous papules
Lichen planus
404
Myxedema Down syndrome Acromegaly Systemic amyloidosis Mucosal neuromas in multiple endocrine neoplasia syndrome IIb These are all causes of what oral finding?
Macroglossia (enlarged tongue)
405
Most likely type of brain tumor in AIDS patient
primary deep cerebral (B-cell) lymphoma with bilateral perivascular distribution
406
Hirschsprung disease is often seen in:
Down syndrome
407
Yellow-white papules and pustules surrounding an erythematous flare newborns
Erythema toxicum
408
Myoglobinuria most often results from:
rhabdomyolysis, such as after severe crush injuries
409
Mutation in the gene for _________ occurs in Marfan syndrome.
Mutation in the gene for fibrillin-1 (FBN1) occurs in Marfan syndrome.
410
What is the diagnosis? Trinucelotide repeat pathology; Mild scoliosis; New onset difficult walking; Lower-limb areflexia; dysarthria; cardiomyopathy
Friedreich Ataxia combined ataxia of upper and lower limbs extensor plantar reflexes and sensory loss also common; scoliosis, diabetes mellitus; autosomal recessive - inherited hypertrophic cardiomyopathy is common cause of death
411
Degenerative disorder of upper and lower motor neurons of the corticospinal tract that typically appears in middle age adults, marked by early atrophy and weakness of the hands and overall lack of sensory impairment.
ALS familial cases have SOD1 mutation
412
Dx: Cirrhosis Neurological manifestations Kayser-Fleisher rings on cornea; Increased risk of hepatocellular carcinoma
Wilson disease
413
Lichen Simplex Chronicus
Thick, leathery vulva hyperplasia of vulvar squamous epithelium benign
414
Sliding hiatal hernia is most often associated with:
Reflux esophagitis
415
Cause of syndrome defined by bilateral benign tumors of the 8th cranial nerve (acustic neuromas), which are commonly associated with meningeioms and gliomas
Neurofibromatosis type II deletion of NF2 gene
416
benign mobile painless and circumscribed mass in the parotid gland with high recurrence rate after surgical excision
pleomorphic adenoma
417
Name the dx: thyroiditis featuring multinucleated giant cells; granulomatous; caused by a viral infection; self-limited
subacute aka DeQuervain thyroiditis
418
What is the diagnosis? Infant or neonatal presentation; Autosomal recessive; Severe hypotonia and areflexia; Enlarged tongue; Cardiomegaly; Often, death by cardiac failure within the first 2 years of life
Pompe Disease, a glycogen storage disease Inability to degrade glycogen Pompe is the most severe form Serum CK slightly to moderately increased
419
What is the diagnosis? Melanin pigmentation of the lips and oral mucosa
Peutz-Jeghers syndrome could also be Addison disease (on buccal mucosa) could also be lead poisoning
420
Clear Cell Adenocarcinoma of the Vagina
complication of DES-associated vaginal adenosis
421
Associate neutrophilic patchy infiltrates with productive cough with what viral pneumonia infection?
Haemophilus influenzae
422
A monoclonal IgM spike is a feature of:
lymphoplasmacytic lymphoma (Waldenström macroglobulinemia)
423
Lack of sensory impairment distinguishes ALS from what other diagnosis?
Syringomyelia
424
Dx: AD; From mouth through GI tract; results in bleeding
Hereditary Hemorrhagic Telangectasia
425
Clincal signs of Von-Hippel Lindau syndrome
headache, unsteadiness in walking (wide based gait), unilateral impaired coordinated movements in arm and leg (disdiodokinesia), intentional tremor
426
Associate Lewy bodies with what diagnosis?
Parkinson's disease Eosinophilic inclusions composed of filamentous aggregates of alpha-synuclein
427
What is a lethal complication? Infant with severe GI fluid loss ie. from diarrhea
Venous sinus thrombosis in the brain
428
Give the dx:
Focal hemorrhages and necrosis in kidney WITHOUT a granulomatous component;
BP usually high
hyperplastic arteriosclerosis
429
Genetic predisposition to melanoma
CDKN2A tumor suppressor gene (p16) mutation encodes an inhibitor of CDK that normally inhibits cell proliferation
430
bone pain and tenderness; most common malignancy seen in children; anemia; thrombocytopenia; presence of blasts in the peripheral blood and bone marrow that \*have\* nucleoli; petechial hemorrhages possible;
Acute lymphocytic leukemia
431
What disorders are in the differential when a high platelet count is given?
chronic myelogenous leukemia; myelofibrosis; polycythemia vera; essential thrombobyctosis (non-neoplastic)
432
What is the ristocetin test?
Ristocetin induces platelet agglutination by causing vWF to bind to platelet gP1b. Lack of vWF (in Von Willebrand Disease) --\> impaired agglutination --\> abnormal test result
433
A 50-year-old man with muscle pain and fever for a month now notes darker colored urine for the past 2 weeks. On physical examination he has palpable purpuric lesions of his skin. Urinalysis shows hematuria and proteinuria. Serum laboratory findings include a mixed cryoglobulinemia with a polyclonal increase in IgG, as well as a high titer of anti–neutrophil cytoplasmic autoantibodies, mainly antimyeloperoxidase (MPO-ANCA, or P-ANCA). A skin biopsy is performed. What pathologic finding is most likely to be observed in this biopsy?
Medial fibrinoid necrosis Microscopic polyangiitis involves small vessels, typically capillaries. Kidneys and lungs are commonly involved, but many organs can be affected. There may be an underlying immune disease, chronic infection, or drug reaction.
434
Cystic medial necrosis is associated with what?
Dissecting aortic aneurysm
435
Accumulation of blood between the calvaria and the dura is what kind of hematoma?
Epidural Commonly associated with trauma - bone fracture severs middle meningeal artery
436
Relative likelihood of intracranial metastasis Colon cancer Breast cancer Lung cancer Melanoma Renal cancer
Melanoma\>breast/lun\>renal/colon
437
Name the dx: Precocious puberty in a male child (but can occur at any age); If in older age group, can be associated with feminization or gynecomastia Tumor secretes androgens, estrogens, or both
Leydig (interstitial) cell tumor;
438
A 54-year-old woman has had a mild fever with cough for a week. Her symptoms gradually improve over the next 10 days. She then begins to have increasing fever, cough, shortness of breath, and malaise. Now, on physical examination, her temperature is 37.9° C. There are inspiratory crackles on auscultation of the chest. A chest radiograph shows bilateral, patchy, small alveolar opacities. Chest CT scan shows small, scattered, ground-glass and nodular opacities. A transbronchial biopsy specimen shows polypoid plugs of loose fibrous tissue and granulation tissue filling bronchioles, along with a surrounding interstitial infiltrate of mononuclear cells. She receives a course of corticosteroid therapy, and her condition improves. What is the most likely diagnosis?
Cryptogenic organizing pneumonia Bronchiolitis obliterans is a feature of cryptogenic organizing pneumonia, an uncommon, nonspecific reaction to a lung injury, such as an infection or toxic exposure.
439
Dx: Outpouching of all three layers of bowel wall; Arises due to failure of vitelline duct to involute
Meckel Diverticulum
440
Complications of acute pancreatitis
Liquefactive necrosis AND fat necrosis of pancreas from autodigestion
441
Pneumonia in Smokers
Haemophilus influenzae, Legionella pneumophila, and Moraxella catarrhalis are the most common causes of bronchitis in smokers.
H. influenzae is an encapsulated, gram-negative, pleomorphic rod.
Legionella does not Gram-stain well, and Moraxella is a gram-negative coccus.
442
What kind of heart disease leads to endocardial and valvular collagenous thickening?
Carcinoid heart disease leads to endocardial and valvular collagenous thickening. release of active tumor products --\> flushing, wheezing, skin lesions, diarrhea
443
Endometrial stromal sarcoma
usually neoplastic cells surrounding a blood vessel rare
444
Red, papular lesions
Cherry angioma
445
Risk factors for pancreatic carcinoma
Smoking; Chronic pancreatitis
446
CSF findings from viral meningitis?
lymphocytes with normal CSF glucose
447
Name the diagnosis corresponding to this autoantibody:
Anti-desmoglein
Pemphigus vulgaris
448
Porcelain gallbladder is a risk factor for the formation of:
gall bladder cancer (adenocarcinoma) Poor prognosis
449
Familial Fatal Insomnia is what kind of disease?
Spongiform Encephalopathy Inherited form of prion disease
450
Protect babies from Hep B vertical infection by:
immune globulin administration within 12 hours of being born
451
ITP can be seen in what autoimmune disorder?
SLE antibody-mediated platelet destruction
452
Medullary carcinoma (of the breast)
nests of cells lymphocytes, dense lymphoid infiltrate fleshy, bulky tumors no calcifications better prognosis than infiltrating ductal or lobular carcinoma
453
"popcorn" cell
Reed-Sternberg cell aka lacunar histiocyte "crippled" B cell bc of mutations in Ig gene malignant B-cell; bi-nucleated does not stain with CD-20, CD45- NO immunoglobulin on its surface CD15+ CD30+
454
Osteochondroma
overlying cartilage cap lateral projection of growth plate (metaphysis)
455
What is the diagnosis? Most common cancer of the upper lip Associated with UVB exposure
Basal cell carcinoma
456
Minimal change disease is aka:
lipoid nephrosis; no glomerular changes seen by light microscopy
457
Within the airspace are multiple large cells with prominent purple intranuclear inclusions, indicative of what infection?
CMV immunocomprimised marked interstitial pneumonitis
458
Dx: Elevation of serum lipase and amylase; Epigastric abdominal pain that radiates to the back; Hypocalcemia
Acute pancreatitis
459
What is the most common cause of acute epiglottitis , especially in nonimmunized children?
H. influenzae type B
460
Give the diagnosis for the following auto-antibody:
Anti-smooth muscle
Autoimmune hepatitis
461
Aflatoxin is a well-known:
hepatotoxin, associated with hepatocellular carcinoma
462
What is the diagnosis? Tumor of epithelial odontogenic origin; Most common clinically significant odontogenic tumor; Slow-growing, generally benign
Ameloblastoma
463
Papillomatosis
risk for developing cancer proliferative fibrocystic change ie increased cells lining the dilated terminal ducts (ductal epithelial hyperplasia)
464
Name the dx: Congenital abnormality in which one or both testes are not found in their normal position in the scrotum; Most common urologic condition in infants; Increased incidence of infertility AND germ cell neoplasia (ie seminoma)
Cryptorchidism (undescended testicle) Fix w surgery
465
A 62-year-old man is a smoker with a 10-year history of cough productive of copious mucopurulent sputum. Over the past 6 months, he has developed progressive dyspnea. Physical examination shows bilateral pedal edema and a soft but enlarged liver. A chest radiograph shows bilateral pleural effusions and a prominent right heart border. Arterial blood gas values are Po2, 60 mm Hg; Pco2, 52 mm Hg; pH, 7.30; and HCO3−, 29 mEq/L. He is intubated and placed on a ventilator, and he requires increasing amounts of oxygen. What microscopic finding is most likely to be present in the affected lungs?
Hypertrophy of bronchial submucosal glands Chronic bronchitis can be complicated by pulmonary hypertension and cor pulmonale. There are few characteristic microscopic features of chronic bronchitis, so it is mainly defined clinically by the presence of a persistent cough with sputum production for at least 3 months in at least 2 consecutive years.
466
Surface Epithelial Ovarian tumor
most common serous and mucinous BRCA1 mutations increase risk for serous carcinoma of ovary and fallopian tube malignant tumors - cystadenocarcinoma - complex cysts with thick, shaggy lining postmenopausal women poor prognosis (usually caught late) CA-125 serum marker Brenner tumor - benign - bladder-like epithelium
467
What is the diagnosis? Inability to metabolize long-chain fatty acids due to an inability to transport these FAs to into the mitochondria (where they undergo beta-oxidation)
Carnitine Palmityl Transferase Deficiency
468
α-Fetoprotein is a marker for:
some germ cell tumors that contain yolk sac elements;
liver cancer
469
Give the dx: Patients usually develop kidney stones, infection, or recurrent hematuria in the third or fourth decade; often bilateral; cystic dilation of 1 to 5 mm is present in the inner medullary and papillary collecting ducts.
Medullary sponge kidney
470
Name the dx: Adenoma of the pituitary; Parathyroid hyperplasia or adenoma; islet cell tumorsof the pancreas (insulinoma and gastrinoma)
MEN-1 aka Werner syndrome Mutation of the MEN-1 tumor suppressor gene
471
What is the dx?
Fundoscopy reveals cotton-wool spots, macular star, retinal hemorrhages, edema of the optic nerve, arteriovenous nicking of the retinal arterioles
Hypertensive retinopathy
472
Patients with sickle cell disease that undergo autosplenectomy are then at risk for what kind of sequelae?
Pneumonia, infections with encapsulated bacteria is strep pneumo; renal papillary necrosis due to low pH and low O2
473
Decreased serum ceruloplasmin suggests what diagnosis?
Wilson disease
474
What is an ameloblastoma?
Benign tumor of the epithelium Arises from enamel organ epithelium or a dentigerous cyst; Located in the mandible --\> "soap bubble" appearance; Locally invasive but does not metastasize
475
Chronic gastritis involved what parts of the stomach?
Body and fundus if autoimmune (antrum if H. pylori) Knocking out parietal cells --\> knocking out the ability to produce acid
476
Most common form of melanoma in dark-skinned people; generally limited to palms, soles and subungual regions
Acral lentiginous melanoma
477
Name the disorder characterized by: 1. Multiple coagulation factor dysfunction 2. Deficiency in newborns 3. Can be induced by long-term antibiotic therapy 4. Can be found in malabsorptive processes 5. Can be found in alcoholics
Vitamin K deficiency
478
Preeclampsia
HTN, proteinuria, edema usually arising in 3rd trimester DIC is a major complication - fibrin thrombi in liver, brain, kidneys HELLP (hemolysis, elevated liver enzymes, low platelets) abnormality of maternal-fetal vascular interface in placenta
479
Parasitic infection of the bladder, such as with schistosomiasis, causes what kind of cancer?
Squamous cancer of the bladder epithelium (starts as metaplasia)
480
A "butterfly like" lesion in the brain is suggestive of what diagnosis?
Glioblastoma multiforme Can invade the contralateral hemisphere across the corpus callosum (looks like a butterfly)
481
What is the most common cause of bacterial meningitis in infants?
H influenza (particularly in non-vaccinated instants)
482
What is the diagnosis? Malignant tumor of oligodendrocytes; Calcified tumor of white matter usually in frontal lobe; "Fried egg" appearance of cells on biopsy
Oligodendroglioma
483
Transposition of the great arteries results in:
Transposition of the great arteries results in a right-to-left shunt with cyanosis.
484
Anticentromere antibody is characteristic of \_\_\_\_\_\_
Anticentromere antibody is characteristic of limited scleroderma, which does not have significant pulmonary involvement, in contrast to diffuse scleroderma.
485
The fibrosis found in cirrhosis is mediated by:
TGF-beta from stellate cells
486
A 26-year-old woman with postpartum sepsis is afebrile on antibiotic therapy, but she has had worsening oxygenation over the past 3 days. Her chest radiograph shows scattered bilateral pulmonary opacifications. A ventilation-perfusion scan shows areas of mismatch. What microscopic finding is most likely to be present in her lungs?
Alveolar hyaline membranes She has acute lung injury with noncardiogenic pulmonary edema and development of diffuse alveolar damage (DAD), clinically known as acute respiratory distress syndrome (ARDS). Inciting sepsis, trauma, or other forms of lung injury leads to a vicious cycle of inflammation with ongoing damage, mainly through the action of neutrophils. Though ARDS may eventually proceed to fibrosis, most patients do not survive that long.
487
Rupture of the paramedian artery leads to what?
Duret (brainstem) hemorrhage A complication of uncal herniation
488
Marrow fibrosis accompanied by megakaryocytosis and granulopoesis:
chronic idiopathic myelofibrosis 15% of cases transform to AML
489
Endometrial Polyp
side effect of tamoxifen hyperplastic protrusion of endometrium (hypersensitive to estrogen stimulation) endometrial glands, fibrous endometrial stroma perimenopausal women
490
Charcot-Leyden crystals form from eosinophil granules in individuals with \_\_\_\_\_\_\_.
Charcot-Leyden crystals form from eosinophil granules in individuals with allergic asthma.
491
CD10 is what kind of cell marker?
B-cell
492
What is the diagnosis? Most common leukodystrophy; Deficiency in arylsulfatase; Autosomal recessive
Metachromatic Leukodystrophy, a lysosomal storage disease
493
Chrondrosarcoma
intermedullary pelvis or central skeleton malignant
494
T/F: The frequency of proliferative retinopathy correlates with the degree of glycemic control.
True. The better-controlled the sugar, the lower the rate of retinopathy.
495
Placenta Accreta
improper implantation of placenta into myometrium Difficult delivery of placenta, postpartum bleeding Often requires hysterectomy
496
Acute otitis media
The most common bacterial pathogen in acute otitis media is Streptococcus pneumoniae.
The second and third most common are nontypeable Haemophilus influenzae and Moraxella catarrhalis, respectively.
497
What is the treatment for Wilson disease?
D-penicillamine
498
Tumor that contains nests of deeply basophilic epithelial cells with narrow rims of cytoplasm that are attached to the epidermis and protrude into the subjacent papillary dermins
Basal cell carcinoma
499
What lung cancer is most likely to produce paraneoplastic hypercalcemia?
Squamous cell carcinoma strong association with smoking. These tumors also can undergo central necrosis—hence a cavity may form. Localized squamous cell carcinomas, in contrast to small cell carcinomas, may be cured by surgery.
500
Name the diagnosis: Vasculitis with moderate to marked vessel wall deposits of immunoglobulin and/or complement components predominantly affecting small vessels (i.e., capillaries, venules, arterioles, and small arteries). Glomerulonephritis is frequent.
Immune complex vasculitis
501
Chancroid
vesiculopapular lesion(s) purulence, bleeds, fever, malaise haemophilus ducreyi granulomatous inflammation, acute transmission (~5 days)
502
Most common cause of acute epiglottitis?
H influenza type B (especially among non-immunized)
503
Giant Cell Tumor
young adults epiphysis of long bones soap-bubble appearance RANK ligand aggressive
504
Inherited degeneration of the anterior motor horn; autosomal recessive, presents as "floppy baby", usually fatal within several years of life
Werdnig-Hoffman disease
505
\_\_\_\_\_\_\_\_\_ is seen mainly in children and involves the aorta (particularly the arch) and branches such as the coronary and renal arteries, causing granulomatous inflammation, aneurysm formation, and dissection.
Takayasu arteritis is seen mainly in children and involves the aorta (particularly the arch) and branches such as the coronary and renal arteries, causing granulomatous inflammation, aneurysm formation, and dissection. Takayasu arteritis leads to “pulseless disease,” because of involvement of the aorta (particularly the arch) and branches such as coronary, carotid, and renal arteries, which results in granulomatous inflammation, aneurysm formation, and dissection. Fibrosis is a late finding, and the pulmonary arteries also can be involved.
506
What is the diagnosis? Thin protrusion of esophageal mucosa; Most often the upper esophagus; Dysphagia for poorly chewed food
Esophageal web Increased risk for esophageal squamous cell carcinoma
507
Reactivation of latent measles virus can cause what lethal viral infection?
SSPE - subacute sclerosing panencephalitis Intranuclear inclusions in neurons and oligodendroglia, marked gliosis in affected gray and white matter, patchy loss of myelin, perivascular lymphocytes and macrophages.
508
Name the disorder characterized by: 1. Genetic factor IX deficiency 2. Postsurgical, deep tissue and joint bleeding 3. Labs: Increased PTT normal PT Low factor VIII Normal platelet count Normal bleeding time 4. Treat with recombinant factor XI
Hemophilia B
509
What is the classic triad of symptoms in meningitis?
Headache, nuchal rigidity and fever -- other common symptoms are photo-phobia, vomiting, and altered mental status
510
A 41-year-old woman has had increasing dyspnea for the past week. On physical examination, temperature is 37.3° C, pulse is 85/min, respirations are 20/min, and blood pressure is 150/95 mm Hg. There is dullness to percussion over the lung bases. A chest radiograph shows large bilateral pleural effusions and a normal heart size. Laboratory findings include serum creatinine, 3.1 mg/dL; urea nitrogen, 29 mg/dL; troponin I, 0.1 ng/mL; WBC count, 3760/mm3; hemoglobin, 11.7 g/dL; and positive ANA and anti–double-stranded DNA antibody test results. What cardiac lesion is most likely to be present in this patient?
Libman-Sacks endocarditis Libman-Sacks endocarditis is an uncommon complication of systemic lupus erythematosus (SLE) that has minimal clinical significance because the small vegetations, although they spread over valves and endocardium, are unlikely to embolize or cause functional flow problems.
511
What is frataxin?
protein essential for mitochondrial regulation of iron; loss results in iron buildup and free radical damage
512
Name the diagnosis: Arteritis, often granulomatous, predominantly affecting the aorta and/or its major branches. Onset usually occurs before the age of 50 years
Takayasu arteritis Onset usually occurs before the age of 50 years, which is a major distinction from giant cell arteritis, whose onset usually occurs after age 50.
513
Acute Interstitial Nephritis
Acute interstitial nephritis (AIN) is associated with the development of oliguria, fever, and rash that starts days to weeks after starting the drug.
**Eosinophils** are seen in the urine.
The drugs most commonly associated with the development of interstitial nephritis are: antibiotics (e.g., penicillins, cephalosporins, sulfa drugs), NSAIDs, diuretics (e.g., thiazides, furosemide), allopurinol, cimetidine, and proton pump inhibitors.
514
T/F: Atherosclerosis is accelerated with diabetes mellitus.
True
515
A 48-year-old, previously healthy woman reports having suddenly lost consciousness four times in the past 6 months. In three instances, she was unconsciousness for only a few minutes. After the fourth episode 1 month ago, she was unconscious for 6 hours and had weakness in her right arm and difficulty speaking. On physical examination, she is afebrile, and her blood pressure is normal. No murmurs are auscultated. She has good carotid pulses with no bruits. What cardiac lesion is most likely to be present in this woman?
Left atrial myxoma
Atrial myxoma is the most common primary cardiac neoplasm. On the left side of the heart, it can produce a ball-valve effect that intermittently occludes the mitral valve, leading to syncopal episodes and possible strokes from embolization to cerebral arteries.
516
Where does the deposition of copper occur in the brain that leads to the neurologic deficits associated with Wilson disease, such as lack of coordination and tremor?
Corpus striatum
517
Name the diagnosis corresponding to this autoantibody:
Anti-ACh receptor
Myasthenia Gravis
518
Pt presents with bilaterally inflammed parotid glands, testicular pain, feeling of bloating after eating with diffuse left upper abdominal pain that radiates to the back, painful headache, fever, sensitivity to light, nausea and vomiting. Labs show high serum amylase. What do they have? What is the greatest risk of complications in teenagers?
Mumps virus infection with orchitis, pancreatitis and aseptic meningitis
sterility is risk if orchitis is bilateral
519
What are the poor prognostic markers for ALL?
Poor prognostic markers for acute lymphoblastic leukemia/lymphoma are: T-cell phenotype; patient age younger than 2 years; WBC count \>100,000; presence of t(9;22) MLL gene mutations; presentation in adolescence and adulthood
520
Nitrosamines, present in smoked foods, help explain the increased incidence of:
Gastric cancer (in Japan)
521
Retinopathy of prematurity is associated with what toxicity?
Oxygen toxicity
522
Legg-Calve-Perthes disease
avascular necrosis in the femoral head of children
523
What is the diagnosis? Increased CSF in dilated ventricles; Can cause dementia in adults - wet, wobbly, wacky
Normal Pressure Hydrocephalus
524
Give the expected BUN:creatinine ratio for azotemia due to dilated cardiomyopathy.
\>20:1 Azotemia - high SG, low FE-Na, high BUN:creatinine ratio, pre-renal when associated with HF
525
What is a complication of chronic cholecystitis?
Porcelain gallbladder - calcifications - can see on xray
526
Corticosteroid-resistant hematuria and proteinuria leading to hypertension and renal failure is typical for:
FSGS - dysfunction of podocyte slit-diaphragm apparatus - corticosteroid resistant Mutations in genes affecting several proteins, including nephrin and podocin, have been found in inherited cases of FSGS; podocyte dysfunction, possibly caused by cytokines or unknown toxic factors, may be responsible for acquired cases of FSGS.
527
Histologic presentation of a miningeoma
well circumscribed, firm, "bossylated" masses of variable size with whirled pattern of meningothelial cells
528
Pt. 16 YO male presents with recurrent epistaxis - what could be the cause?
Nasopharyngeal angioblastoma benign but locally aggressive vascular tumor that grows in the back of the nasal cavity (consists of large blood vessels and fibrous CT). Most commonly affects adolescent males. Usually presents with one-sided nasal obstruction and recurrent bleeding.
529
Maculopapular rash that blanches with pressure
Roseola infantum (HHV6)
530
What is the diagnosis? Malignant proliferation of squamous cells; Usually in middle or upper third of the esophagus; Lymph node spread is cervical if upper, Mediastinal or tracheobronchial if middle; Risk factors - very hot tea, alcohol, tobacco, Plummer-Vinson syndrome, esophageal web and esophageal injury ie ingesting lye
Squamous cell carcinoma of the esophagus Most common esophageal carcinoma worldwide Presents late, poor prognosis, progressive dysphagia, weight loss, hematemesis, hoarse voice and cough
531
What is the diagnosis? Necrosis of interdental papilla with punched out lesions covered by grayish pseudomembrane
Acute necrotizing gingivitis Anaerobic bacterial infection of the gingiva
532
Heb B serum markers:
Hep B surface ag Hep Be ag - responsible for virus replication Core - Hep Bc-ag and back Once HepB ag, immune
533
What feature is similar about Warthin's tumor, pleomorphic adenoma, and mucoepidermoid carcinoma?
all commonly localize to the parotid gland
534
Microscopic examination of a yolk sac tumor would reveal:
Interlacing strands of epithelial cells surrounded by loose connective stroma;
Glomeruloid structures called "Schiller Duval" bodies;
These tumors are malignant; treat with orchiectomy;
First 4 years of life;
Tumors produce alpha-fetoprotein
535
Adenomyosis
Presence of endometrial glands and stroma in the myometrium 1/5 of uteri have this characteristic can be associated with pelvic pain and bleeding, dyspareunia
536
What is the characteristic lung presentation of an infection with legionella?
Legionella is likely to produce a widespread bronchopneumonia with alveolar neutrophilic exudates.
537
Acanthosis nigricans is an uncommon condition with hyperpigmented areas in skin folds; it may occur in association with:
endocrinopathies neoplasms
538
Most common causes of epithelial dysplasia on true vocal cords that transisions to squamous cell carcinoma of the larynx (laryngeal carcinoma)?
Alcohol and tobacco
539
What is the diagnosis? Glossitis associated with bilateral white excrescences on lateral border of tongue
Hairy leukoplakia Pre-AIDS defining lesion
540
Give the expected BUN:creatinine ratio for BPH
BPH - post-renal (obstructive) 10:1 - 20:1
541
Chromosomal translocation involving 8q24 which harbors myc oncogene; most common childhood malignancy in Central Africa; may be induced by immunodeficiency; "starry sky" macrophage
Burkitt Lymphoma EBV caused
542
Secondary parathyroid hyperplasia is encountered principally in patients with :
Chronic renal failure; --chronic hypocalcemia --\> renal rention of phosphate --\> compensatory hypersecretion of PTH --\> parathyroid hyperplasia However... Vitamin D deficiency, intestinal malabsorption, Fanconi syndrome, renal tubular acidosis also possible
543
Name the dx: Purely follicular thyroid cancer; Well-defined, encapsulated tumor; Resembles follicular adenoma, but with more microfollicles or trabecular pattern
Follicular thyroid carcinoma (FTC)
544
Give the diagnosis for the following auto-antibody:
Rheumatoid factor (antibody, most commonly to IgM, specific to IgG Fc region),
anti-CCP
Rheumatoid arthritis
545
Painful superficial ulceration of the oral mucosa that is characterized a greyish base surrounded by erythema What condition is this? What causes it?
Aphthous ulceration arises in relation to stress, spontaneously resolves
546
ring sideroblasts, elderly patient think:
myelodysplastic syndrome clonal stem cell defect
547
Name the dx: Sex-linked disease with severe immunodeficiency; Hypoplastic thymus; Eczema; Thrombocytopenia; Chronic RTIs, GI infections, Petechiae
Wiskott-Aldrich syndrome
548
Acute, disseminated variant of Langherhans cell histiocytosis seen in children
Letterer-Siwe disease otitis media is a common finding painful lytic bone lesions common
549
Dx: Tarry stool; Upper GI pain; Firm, submucosal, ulcerated mass in the stomach; Spindle cells in cytoplasm
GIST - Gastrointestinal stromal tumor derived from pacemaker cells of Cajal
550
Inheritance of three abnormal α-globin chains leads to:
hemoglobin H disease, with tetramers of β chains; survival to adulthood is possible
551
What is the most common cause of a malignant turmor of the nasopharyngeal epithelium? Who is most susceptible?
Nasopharyngeal carcinom - caused by EBV infection African CHILDREN or Chinese ADULTS
552
Give the dx: Diffuse BM thickening; Granular deposits of IgG and C3 common; Antibody targeting M-type phospholipase A2 receptor antigen; Proteinuria
Membranous nephropathy causing nephrotic syndrome
553
Hyalinzation of the islets of Langherhans shows wahat material in patients with T2DM?
Amyoid (from amylin secreted by the beta cell)
554
Leiomyosarcoma
arises de novo unilateral, usually one mass, necrosis and hemorrhage postmenopausal women Actin+ 10+ mitosis per HPF or 5+ mitoses per 10 HPF Nuclear atypia, necrosis possible
555
What is the effect of the following teratogen?
Tetracycline
Discolored teeth
556
What is the dx? Anterior chamber is shallower than normal and the angle is abnormally narrow; Characteristic excavation of the optic nerve head; Progressive loss of visual field sensitivity; Increased ocular pressure; Degenerative changes in the retina;
Closed-angle glaucoma Affects persons whose iris is displaced anteriorly
557
CSF findings from fungal meningitis?
lymphocytes with decreased CSF glucose
558
tumor with characteristic "epithelial appearance" may feature "perivascular pseudorosettes" of tumor cells surrounding blood vessels typically associated with central canal of spinal cord, walls of 4th ventricle, and often results in hydrocephalus
ependoma
559
What is the diagnosis? Chronic, contagious conjunctivitis caused by Chlamydia trachomatis; Scarring of conjunctiva; Can cause blindness in adults, but usually heals spontaneously in children
Trachoma corneal lesion = pannus
560
What is the most common adult muscular dystrophy?
Myotonic dystrophy Autosomal dominant Slowing of muscle relaxation and wasting; Involves many muscle systems including the heart
561
Tx Chronic Hep B?
Antivirals ie epivir, tenofovir, adefovir, entecovir 1 per day for a year
562
What is the diagnosis? Direct muscle cell damage due to cytotoxic T cells; Association with anti-Jo1 antibody; Interstitial lung disease; Raynaud's phenomenon; Nonerosive arthritis
Polymyositis
563
85% of patients with Hep C become...
chronic
564
Glucose is converted to sorbitol, which is toxic, in what cycle, that can contribute to the formation of cataracts?
Aldose reductase pathway
565
Podocytes are visceral epithelial cells that form what part of the kidney?
The filtration barrier
566
What is anencephaly?
Anencephaly is a neural tube defect that results in absence of the skull and brain - disruption of the cranial end of the neural tube;
"Frog-like" appearance of fetus;
Maternal polyhydramnios because fetus can't swallow amniotic fluid
567
A 5-year-old girl who is below the 5th percentile for height and weight for age has exhibited easily fatigability since infancy. On physical examination, she appears cyanotic. Her temperature is 37° C, pulse is 82/min, respirations are 16/min, and blood pressure is 105/65 mm Hg. Pulse oximetry shows decreased oxygen saturation. One month later, she has fever and obtundation. A cerebral CT scan shows a right parietal, ring-enhancing, 3-cm lesion. What congenital heart diseases is the most likely diagnosis?
Truncus arteriosus Cyanosis at this early age suggests a right-to-left shunt. Truncus arteriosus, transposition of the great arteries, and tetralogy of Fallot are the most common causes of cyanotic congenital heart disease.
568
Give the diagnosis for the following auto-antibody:
Anti-SSA, Anti-SSB (Anti-Ro, Anti-La)
Sjogren's syndrome
569
What is the effect of the following teratogen?
Alcohol
Most common cause of mental retardation;
Also leads to facial abnormalities and microcephaly
570
Fibroadenoma
tumor of fibrous tissue and glands (epithelial and stromal elements) surgical removal is curative well-circumscribed, mobile, marble-like mass estrogen sensitive\*\*\* lifetime risk for cancer is doubled
571
CD56 is a marker for
NK cells
572
What are symptoms of an upper motor neuron lesion?
result from damage to descending motor systems; include paralysis, spasticity, and a positive Babinski sign.
573
FSH
granulosa cells convert androgen to estrogen
574
Name the dx: Enlargement of the prostate gland leading to obstruction of urine flow; Proliferation of the glands and stroma; Repeated bouts of cystitis; Urinary retention leading to bladder diverticula and/or bladder stones
Nodular prostatic hyperplasia
575
What is the diagnosis? Fever Nasal congestion with or without purulent discharge Pain over the affected sinuses Painful teeth, cough from postnasal discharge, periorbital cellulitis
Sinusitis inflammation of the mucous membranes lining one or more of the paranasal sinuses; most often in the maxillary sinus in adults (ethmoid in children); Causes include URIs, deviated nasal septum, allergic rhinitis, barotrauma, cigarette smoking --strep pneumonaie Diagnose with CT scan
576
Pernicious anemia can have what CNS complication?
Lesions in the posterolateral spinal cord --\> degeneration --\> paresthesias and burning sensations in feet, weakness of legs Once these effects hit, it is not commonly reversible
577
Midline cerebellar tumor characterized histologically by small hyperchromatic cells with neuroblastic rosettes
medulloblastoma
578
Rough whitish patch on the lateral tongue, usually arising in immunocompromised Pts, cannot be scraped off. What is it? What causes it? Is it cancerous?
Hairy leukoplakia Caused by EBV which induces squamous cell hyperplasia (not dysplasia!) not pre-malignant
579
The t(8;14) translocation is typical of:
Burkitt lymphoma MYC gene\*\*\*
580
Endometriosis
ovary most common site chocolate cysts gun-powder nodules increased risk of carcinoma at side of endometriosis (in ovary esp) adenomyosis when uterine myometrium is involved
581
Name the diagnosis: Arteritis, often granulomatous and usually affecting the aorta and/or its major branches, with a predilection for the branches of the carotid and vertebral arteries. Giant cells are frequently but not always observed in biopsy specimens
Giant cell arteritis (temporal arteritis if temporal arteries are involved)
582
CD68 is a:
macrophage (histiocyte) marker
583
large cells, some of which contain horseshoe-shaped nuclei and voluminous cytoplasm; tumor cells cluster around venules and infiltrate sinuses; most often in children and young adults; characteristic gene rearrangement on chromosome 2p23 that results in production of anaplastic lymphoma kinase (ALK) with tyrosine kinase activity
anaplastic large-cell lymphoma
584
\_\_\_\_\_\_\_\_\_\_ is the most common form of non-Hodgkin lymphoma among adults in Europe and North America.
Follicular lymphoma The neoplastic B cells mimic a population of follicular center cells and produce a nodular or follicular pattern. Nodal involvement is often generalized, but extranodal involvement is uncommon.
585
Osteoid Osteoma
small, painful lesion surrounded by reactive bone formation
radiolucent core
typically young people (5-25)
**aspirin resolves pain (osteoblastoma does NOT respond to aspirin)**
cortex of long bones (diaphysis) ie femur
benign tumor of osteoblasts (produce osteoid)
586
When tooth discoloration occurs in children as a result of medication, what is the implicated medication?
Tetracycline When tooth discoloration is not due to tetracycline, it is usually excess fluoride (chalky white) OR could be congenital erythropoetic porphyria (reddish brown)
587
Pseudoarthrosis
joint like tissue formation at a site of fracture nonunion healing surgical removal to properly heal fracture
588
Give the dx: Undifferentiated tubular structures surrounded by primitive mesenchyme; Often seen with cysts; Palpable flank mass seen shortly after birth; Usually unilateral
Renal dysplasia (multicystic is one form)
589
Hunner ulcers are seen in what condition?
Chronic interstitial cystitis Hunner ulcer is an intense, acute, inflammatory reaction
590
rapidly progressive gangrenous cellulitis of the soft tissues of the neck and floor of the mouth that results in swelling of the tongue, neck pain, and breathing problems - commonly associated with dental procedures - culture produces mixoid aeorobic and non-aerobic bacterial flora
Ludwig's angina (cellulitis = spreading bacterial infection under skin; angina = strangling)
591
What makes a gastric ulcer malignant?
Piling up of mucosa around the ulcer; large; irregular border
592
Juxtaglomerular cells secrete:
renin
593
How does microvascular disease contribute to extremity ulcers in diabetic patients?
Slow healing wounds due to ischemia; Increased deposition and glycosylation of basement membrane proteins
594
Osteoarthritis
Progressive disease Herberden nodes - DIP - osteophyte formation Bouchard nodes - PIP - osteophyte formation morning stiffness, worsens during day
595
Multiple myeloma produces mass lesions of plasma cells that lead to bone lysis and pain; radiograph shows typical punched-out lytic lesions, produced by expanding masses of what kind of cells?
Plasma cells
596
What kind of brain tumor is commonly associated with diabetes insipidus?
Craniopharyngioma --\> destruction of the posterior pituitary (neurohypophysis)
597
Name the diagnosis corresponding to this autoantibody:
Antihistone
Drug-induced lupus
598
Associate "saddle nose deformity" with what diagnosis?
"Wegener's" granulomatosis aka GPA (granulomatosis with polyangiitis) \*\*antibodies to neutrophil cytoplasm\*\*
599
Hytidaform Mole - Complete Mole
empty ovum, 2 sperm 46 chromosomes absent fetal tissue choriocarcinoma risk snowstorm on ultrasound, fetal HR neg bHCG much higher than expected
600
Fat necrosis
trauma calcifications and giant cells
601
Name the dx: Excessive secretion of PTH, such as from parathyroid adenoma; Stones in renal pelvis and/or ureter causing acute flank pain; Excessive loss of Ca from bones and enhanced Ca resorption by renal tubules; Can also be asymptomatic; Can be accompanied by mental changes such as depression, emotional lability, poor mentation and memory defects
Primary hyperparathyroidism
602
Fatal familial insomnia and spongiform encephalopathy are caused by \_\_\_\_\_\_\_\_
prion disease
603
T/F layngeal papillomas are usually multiple in children and single in adults
true
604
Name the dx: A cyst formed by the protrusions of widened efferent ducts of the rete testis or epididymis; Manifests as a hilar paratesticular nodule or as a fluctuating mass with milky fluid containing spermatozoa in various stages of degeneration
Spermatocele
605
Involucrum
periosteal new bone forms around the sequestrum
606
What is truncus arteriosus?
Truncus arteriosus is an anomalous, incomplete separation of the pulmonic and aortic trunks.
607
Name the dx: thyroiditis featuring lymphocytic infiltrates
Hashimoto thyroiditis
608
What is a Zenker Diverticulum?
Outpouching of pharyngeal mucosa through an acquired defect in the muscular wall; Arises above the upper esophageal sphincter (at the junction of the esophagus and the pharynx; Presents with dysphagia, obstruction, halitosis
609
What is the most common chronic CNS disease among young adults in the US?
Multiple sclerosis
End-stage in brain: astrogliosis, thick-walled blood vessels, perivascular inflammation, secondary loss of axons
610
Acute mastitis
staph
611
What defines nephrotic syndrome?
Podocyte injury, the loss of \>3.5g protein per day in the urine
612
What is the diagnosis? Sore, beefy, red tongue with or without papillary atrophy
Glossitis (inflammation of the tongue) Causes include: long-standing iron deficiency B12 or folate deficiency Scurvy Pellagra (niacin deficiency) Scarlet fever EBV-associated hairy leukoplakia
613
CSF is formed principally by what brain area?
Choroid plexus
614
Microscopic polyangiitis (MPA) Granulomatosis with polyangiitis (Wegener’s) (GPA) Eosinophilic granulomatosis with polyangiitis (Churg-Strauss) (EGPA) These all make up what kind of vasculitides?
Small vessel vasculitis Antineutrophil cytoplasmic antibody (ANCA)–associated vasculitis (AAV)
615
Give the dx: Immune complexes against DNA, RNA and autologous proteins get deposited along the BM of the glomeruli ad form a similar pattern to idiopathic membranous nephropathy; Mesangial and subendothelial deposits of immunoglobulins also present
Membranous nephropathy of SLE
616
Posttransplant lymphoproliferative disorder (PTLD) is associated with immunosuppression and what virus?
EBV
617
What are the oral manifestations of HIV?
1. Candidiasis 2. Aphthous ulcers 3. Hairy leukoplakia - glossitis due to EBV 4. Kaposi sarcoma - HHV8
618
Salmon-colored plaques covered by white to silver colored scales; Nail pitting
Psoriasis
619
Name the dx: Dysuria, urgency, urethral discharge; Suppurative urethritis; Acute onset, related to sexual intercourse
Gonorrhea (neisseria gonorrhoeae)
620
Dx: Migratory thrombophlebitis
Trousseau syndrome May accompany adenocarcinoma of the pancreas (as well as other malignancies)
621
New tx Hep c?
Sofosbuvir (genotype 1 and 2 - 12 weeks); simeprevir
622
Name the dx: Most common testicular cancer (germ cell tumor); Men 30-40; Additional fragment of chromosome 12 seen; Solid, rubbery, firm mass
Seminoma Neoplastic cells are arranged as nests that are separated by fibrous septae and infiltrated with chronic inflammatory cells (lymphocytes, plasma cells, macrophages) treat with radiation
623
Name the dx: Virilization of external genitalia; Still 46, XX; Often associated with CAH (21-hydroxylase deficiency); Adrenal hyperplasia; Ambiguous genitalia seen at birth
Female pseudohermaphroditism
624
Favored sites of aneurysms and most common consequence
internal corotid, vertibral, basilar arteries thrombosis \> hemorrhage /dissection
625
Name the diagnosis: Primary infection usually occurs in childhood; Dormancy in trigeminal ganglia; vesicles in oral mucosa rupture and leave shallow ulcerations
HSV-1 (usually) - oral herpes Stress and sunlight can reactivate the virus
626
What is the difference between Neurofibromatosis type 1 and 2?
NF2 exhibits acoustic neuromas, but NF1 does not
627
Complement lysis is enhanced in paroxysmal nocturnal hemoglobinuria, which results from mutations in the _____ gene.
PIGA Paroxysmal nocturnal hemoglobinuria (PNH) is a disorder that results from an acquired stem cell membrane defect produced by a PIGA gene mutation that prevents the membrane expression of certain proteins that require a glycolipid anchor. These include proteins that protect cells from lysis by spontaneously activated complement. As a result, RBCs, granulocytes, and platelets are exquisitely sensitive to the lytic activity of complement. The RBC lysis is intravascular, so patients can have hemoglobinuria (dark urine). Defects in platelet function are believed to be responsible for venous thrombosis. Recurrent infections can be caused by impaired leukocyte functions. Patients with PNH may develop acute leukemia or aplastic anemia as complications.
628
Name the dx: Pain in the scrotum; Small, tender nodule attached to the testis; Caused by infection (chlamydia in young men, E. Coli in old);
Epididymitis
629
What is Gottron's sign? What disease is it used to detect?
Descrete erythematous, scaly eruptions occurring in symmetric fashion overlying the metacarpal and interphalangeal joints Dermatomyositis
630
Pearly gray-white hyperkeratoic lesion
Actinic (solar) keratosis
631
see lacunar cells, think....
Hodgkin Lymphoma
seen with Reed-Sternberg cells
632
Achalasia is associated with what disease in the tropics?
Chagas disease
633
Give the dx: Enlarged, multicystic (in the parenchyma) kidneys; Often mutation in the gene that codes for polycystin; Half of all patients develop end-stage renal failure; Bilateral flank and abdominal masses; Passage of blood clots in the urine; Azotemia progressing to uremia is also common
Autosomal dominant polycystic kidney disease \*\*1/3 of patients also have hepatic cysts
634
"Hutchinson melanotic freckle"; large pigmented macule; occurs on sun-damaged skin; fair-skinned, elderly, white people
Lentigo maligna melanoma
635
The cystic medial necrosis that occurs in Marfan syndrome most often involves what section of the aorta?
The cystic medial necrosis that occurs in Marfan syndrome most often involves the ascending aorta and predisposes to dissection that could involve coronary arteries, although with external compression.
636
Dx: Intractable gastric hypersecretion; Severe peptic ulceration of the duodenum and sometimes jejunum; Elevated gastrin levels
Zollinger-Ellison syndrome tumor responsible is pancreatic gastrinoma made of G cells
637
Osteitis Fibrosa Cystica
hyperparathyroidism complication
638
postnatal viral infection that causes unilateral deafness
mumps
639
Dx: Necrotizing migratory erythema; Mild hyperglycemia and anemia
Glucagonoma Hypersecretion of glucacon by alpha cell tumors
640
Proteins C and S normally inactivate factors:
5 and 8
641
Name the likely diagnosis: recurrent miscarriages; arterial and venous thromboses; anticardiolipin antobody +
Antiphospholipid syndrome (APS)
642
Associated disease: highly vascularized cerebellar tumor that features endothelium lined cannals interspersed with "plump cells" that may secrete eyrethropoin and induce polycythemia
Von-Hippel Lindau syndrome
643
Osteomalacia
impaired mineralization of osteoid can be associated with autoimmune disorders (ie Chrons, celiac) pidgeon-breast deformity frontal bossing rachitic rosary low vitamin D in adults, Ca down, phosphate down, PTH up, alk phos up
644
Human metapneumovirus is seen in what population?
Children similar to RSV bilateral diffuse interstitial infiltrates on CXR generally self-limiting
645
Give the dx: Multiple small cysts in the renal papillae
Medullary sponge kidney
646
Alpha-1-antitrypsin deficiency
Alpha-1-antitrypsin is a **genetically-acquired form of emphysema.**
Heterozygous patients may only develop symptomatic disease later in life if they are cigarette smokers. However, homozygous patients will develop severe symptomatic emphysema at a young age and may also be at risk for disease in other organ systems, specifically---for example, hepatic cirrhosis.
Histologic examination of an emphysematous lung shows **loss of elastic fibers and alveolar walls with destruction of capillary beds and coalescence of air spaces**. This loss of lung tissue reduces the elastic recoil of the lung and increases the pulmonary compliance (i.e., increases the distensibility of the lungs).
647
Give the dx: Deposition of antiglomerular basement membrane antibody
Goodpasture syndrome
648
An alcoholic aspirates and develops a lung abscess. What organisms are likely responsible and why?
Staphylococcus aureus and Bacteroides fragilis Bacterial organisms are most likely to produce abscesses, and the infection may be polymicrobial. The most common pathogen is Staphylococcus aureus, but anaerobes such as Bacteroides, Peptococcus, and Fusobacterium spp. also may be implicated. These anaerobes normally are found in the oral cavity and are readily aspirated. The purulent, liquefied center of the abscess can produce the radiographic appearance of an air-fluid level.
649
Give the diagnosis for the following auto-antibody:
Anti-U1 RNP (ribonucleoprotein)
Mixed connective tissue disease
650
Inability to relax the lower esophageal sphincter (LES) is called:
Achalasia Damaged ganglion cells in myenteric plexus; Can be idiopathic or due to Chagas disease (Trypanosoma cruzi) "Bird-beak" sign - narrowing of esophagus at sphincter, can see on barium swallow study; Presents with dysphagia, putrid breath, high LES pressure on esophageal manometry Increases risk for esophageal squamous cell carcinoma
651
What is the diagnosis? Single muscle fiber necrosis and regeneration; Lymphocytic inflitration of muscle fibers;
Inclusion Body Myositis Inclusions are stained with Congo red and represent an intracellular amyloid
652
What is the most likely complication of bronchiectasis?
Bronchiectasis results from inflammation with destruction of bronchi; hemoptysis is the most likely complication. Bronchiectasis results in airway dilation from destructive bronchial wall inflammation.
653
Associate p-ANCA with
Ulcerative colitis; Churg-Strauss; Microscopic polyangiitis
654
"slapped face" appearance
Erythema infectiosum
655
Metronidazole causes what rxn with alcohol?
Disulfiram-like rxn
656
most clinically significant odontogenic tumor that benign tumor of odontogenic epithelium more commonly appearing in the lower jaw than the upper jaw. Rarely metastatic but progress slowly, resulting in lesions can cause severe abnormalities of the face and jaw
Ameloblastoma
657
Coning of cerebellar tonsils that produces cardiorespiratory arrest
tonsilar herniation
658
What is the dx? Characteristic excavation of the optic nerve head; Progressive loss of visual field sensitivity; Increased ocular pressure; Degenerative changes in the retina;
Glaucoma
659
macrocytosis with hypersegmentation of neutrophils
pernicious anemia deficiency of B12 or folic acid
660
Polycystic Ovarian Disease (PCOS, PCOD)
LH: FSH \> 2 unopposed acyclic estrogen secretion androgen converted to estrone in adipose tissue --\> increased risk for endometrial carcinoma T2DM common low FSH - cystic degeneration of follicles common (5% of repro age women) oligomenorrhea, hirsutism aka Stein-Leventhal Syndrome
661
Transmission Hep E?
Fecal-oral
662
Patients with Goodpasture syndrome (type I anti-glomerular BM disease) require what kind of treatment?
Plasmapheresis
663
Most common transmission Heb B
Perinatal (vagina) NOT placenta
664
Invasive Ductal Carcinoma
most common invasive cancer of the breast irregular nests of cells and cords of epithelial cells can be tubular (well-differentiated tubules w/o myoepithelium), mucinous, medullary (large, high grade cells with lymphocytes, plasma cells) or inflammatory increased in BRCA1 carriers
665
Dx: Necrotizing arteritis of medium-sized vessels that lead to pseudoaneurysm formation, renal thrombosis, inflammation and hemorrhage; A possible complication of chronic HBV infection due to circulating immune complexes
Polyarteritis nodosa
666
Anemia of chronic disease is often mild to moderate, with what size RBCs?
Microcytic "poor utilization of iron stores" low serum iron LOW iron binding capacity
667
The most common primary lung cancers for smokers to develop are:
Small cell - invasive perihilar mass, poorly differntiated Squamous cell
668
Name the dx: Collection of serous fluid in the scrotal sac between two layers of the tunica vaginalis; Congenital version is often associated with inguinal hernia
Hydrocele
669
What are symptoms of a lower motor neuron lesion?
flaccid paralysis, muscle atrophy, fasciculations, weakness, decreased muscle tone, impaired reflexes, negative babinski sign
670
Classic examples of microangiopathic hemolytic anemia:
DIC TTP both feature generalized thrombosis of capillary vessels
671
Dx: Rash, arthralgias, transient defect in erythropoiesis; Virus; Spread by respiratory secretions; Erythema infectiosum
Parvovirus B19 - DNA virus Can cause aplastic crisis in people with hemolytic anemia/SCD.
672
Thrombotic Thrombocytopenic Purpura (TTP) is due to decreased \_\_\_\_\_, an enzyme that normally cleaves vWF into monomers for degradation.
ADAMS-TS-13 \*CNS abnormalities common in TTP
673
What is the likely offending organism?
A 71-year-old woman Smoker w/ COPD increasing dyspnea 3-day time course of worsening symptoms Febrile Productive cough Severe dyspnea Rales and Expiratory wheezes Neutrophilia
Moraxella Catarrhalis
The short time course and acute inflammatory response are consistent with bacterial pneumonitis.
Moraxella is in the differential diagnosis of both upper and lower respiratory infections, particularly in persons with chronic obstructive pulmonary disease (COPD).
Moraxella is an **aerobic gram-negative diplococcus that is oxidase positive.**
often causing sinusitis, otitis, and upper respiratory infections.
674
Gomori methenamine silver is used to identify what organism that has a predilection for the immunocomprimised?
Pneumocystis jiroveci Often associated with AIDS patients
675
Incubation Hep C:
60 days
676
Associate Alexander disease with:
Rosenthal fibers Alexander disease is a rare neurologic disorder of infants; mutation in gene encoding glial fibrillary acidic protein
677
What is the diagnosis? Hydrocephalus in a newborn; Enlarging head circumference due to dilation of the ventricles; Accumulation of CSF in the ventricular space
Cerebral Aqueduct Stenosis Congenital stenosis of the channel that drains CSF from the 3rd to the 4th ventricle
678
Placental Abruption
separation of placenta from decidua prior to delivery of fetus reason for stillborn 3rd trimester bleeding, fetal insufficiency
679
Give the dx: Pathologic changes in some glomeruli but not others; Intermittent hematuria;
Focal proliferative glomerulonephritis - includes: --lupus nephritis --Henoch-Schonlein purpura --Berger disease (IgA nephropathy)
680
Name the dx: Branching papillae lined by epithelial cells with clear (ground glass or Orphan Annie) nuclei and fibrovascular cores; Psamomma bodies (calcospherites) also possible
Papillary carcinoma of the thyroid
681
Glanzmann thrombasthenia
Glanzmann thrombasthenia is a rare autosomal recessive disorder caused by **deficiency or abnormality of glycoprotein IIb/IIIa receptors for fibrinogen.**
This results in a functional defect in platelets that **prevents them from aggregating.**
682
Target lesions with three zones of color
erythema mutiformae
683
Tea-colored urine suggests?
Icteric phase Hep B infection
684
Sezary syndrome is disseminated \_\_\_\_\_\_\_
mycosis fungoides (a variant of cutaneous T-cell lymphoma)
685
slow growing tumor arising in the white matter marked by a dense patterning of neoplastic cells with small rounded nuclei, pleomorphic features, and randomly distributed (radiographically observable) "Calcospherites"
Oligodendroglioma
686
LH surge
caused by estradiol surge
687
Auer rods, think
Acute promyelocytic leukemia (APL)
688
Parkinson's disease
T: tremor (3-5 Hz resting tremor; initially often unilateral and later becomes bilateral although often remains asymmetric)
R: rigidity (increased resistance to passive movement; can see cogwheel rigidity– ratchet-like interruptions of tone)
A: akinesia (difficulty initiating movements, and bradykinesia –reduced range and speed of movements)
P: postural instability (falls are common, stooped posture, festinating gait-small shuffling steps)
**Lewy bodies are eosinophilic cytoplasmic inclusions, primarily composed of alpha-synuclein and ubiquitin, and are a common pathologic feature of PD.**
689
Name the diagnosis: Sterile vegetations or normal cardiac valves; associated with cancer or other wasting disease; increased blood coagulability and immune complex deposition are suggested for pathogenesis
Marantic endocarditis
690
What kind of gallstones show up on xray?
Bilirubin stones - radiopaque
691
What is the MOA of hydroxyurea that makes it effective for treatment of sickle cell disease?
hydroxyurea therapy can increase the concentration of hemoglobin F in RBCs, which interferes with the polymerization of hemoglobin S. However, the therapeutic response to hydroxyurea often precedes the increase in hemoglobin F levels. Hydroxyurea also has an anti-inflammatory effect, increases the mean RBC volume, and can be oxidized by heme groups to produce nitric oxide that promotes vasodilation.
692
Numerous pigmented lesions
Dysplastic nervus syndrome
693
What is the cause of minimal change disease?
The most likely cause of foot process fusion is a primary injury to visceral epithelial cells caused by T cell–derived cytokines.
694
Blood monocytes become tissue macrophages that evolve into epithelioid cells and giant cells of what kind of inflammation?
Granulomatous
695
Dx: Congenital hypertrophy of pyloric smooth muscle; Projectile vomiting; Visible peristalsis; Olive-like mass in abdomen More common in males
Pyloric stenosis
696
Pancreatic insufficiency is associated with
chronic pancreatitis --\> no enzymes --\> malabsorption w/ steattorhea, fat soluble vitamin deficiencies (A, D, E, K) also seen: dystrophic calcification
697
Proliferative phase
estrogen driven
698
Where is the most common place for Syringomyelia to occur?
C8 to T1 Syringomyelia is cystic degeneration of the spinal cord Sensory loss of pain and temperature, spares fine touch and position sense (cape-like distribution)
699
Papillary structures with psammoma bodies would characterize:
a cystadenocarcinoma
700
What lab test is often seen in Primary Sclerosing Cholangitis?
Positive p-ANCA
701
What is the diagnosis? Galactocerebroside accumulates in macrophages
Krabbe disease Autosomal recessive deficiency of galactocerebrosidase
702
Tendency to form blisters at sites of minor trauma; Mutations of genes encoding cytokeratin IFs; can be minor, or widespread and life-threatening; blisters almost always noted at birth or shortly after; blister formation in \*basement membrane\* zone; no inflammatory cells in blisters, no antibody deposits
Epidermolysis Bullosa
703
In a child with nasal polyps, what diagnosis do you want to rule out?
Sweat test for cystic fibrosis Nasal polyps: Noncancerous often associated with CF due to thickness of secretions Nasal smear shows numerous eosinophils Most often seen in adults with IgE mediated allergies
704
Peritonsillar abscess can be a complication of:
Tonsillitis (strep pyogenes) uvula deviates from contralateral side; "hot potato" voice foul-smelling breath
705
Causes of endothelial cell damage include:
1. Vasculitis 2. Atherosclerosis 3. High levels of homocysteine
706
Fibroma (of the ovary)
benign solid, firm, white tumor of fibroblasts pleural effusion, ascites (Meigs syndrome)
707
What is a viral cause of meningitis?
Enteroviruses (e.g. coxsackievirus) are most common causes (fecal oral transmission, particularly affects children)
708
Pigmented skin with slightly papillomatous appearing surface
Compound nevus
709
Tumor in the nasal cavity that can cause "sinus pressure". Marked by epithelial nests protruding into the submucosa with uniform proliferation and no evidence of cellular atypia.
sinonasal/nasolaryngeal inverted papilloma - caused by HPV 6,11 Inverted papilomas feature inversion of the epithelium into the underlying stroma encased by intact basement membane (so tumor can protrude into the nasal sinuses - think opposite of a polyp).
710
T/F: A 46,XX karyotype is found in some patients with classical signs of Klinefelter syndrome.
True
711
A 10-year-old girl who is normally developed has chronic progressive exercise intolerance. On physical examination, temperature is 37.1° C, pulse is 70/min, respirations are 14/min, and blood pressure is 100/60 mm Hg. A chest radiograph shows cardiomegaly and mild pulmonary edema. An echocardiogram shows severe left ventricular hypertrophy and a prominent interventricular septum. The right ventricle is slightly thickened. During systole, the anterior leaflet of the mitral valve moves into the outflow tract of the left ventricle. The ejection fraction is abnormally high, and the ventricular volume and cardiac output are both low. What is the most likely cause of the cardiac abnormalities in this patient?
Beta-myosin heavy chain mutation Hypertrophic cardiomyopathy is familial in \>70% of cases and is usually transmitted as an autosomal dominant trait. The mutations affect genes that encode proteins of cardiac contractile elements. The most common mutation in the inherited forms affects the β-myosin heavy chain.
712
Name the diagnosis: Vasculitis affecting glomerular capillaries, pulmonary capillaries, or both, with GBM deposition of anti-GBM autoantibodies. Lung involvement causes pulmonary hemorrhage, and renal involvement causes glomerulonephritis with necrosis and crescents.
Anti–glomerular basement membrane (anti-GBM) disease
713
Crateriform tumor with a central keratin plug
keratoacanthoma
714
Name the dx: Congenital defect; Absence of the anterior abdominal wall; Is associated with epispadias in some male infants; Increased risk of bladder carcinoma (squamous) later in life
Exstrophy of the urinary bladder
715
Chronic gastritis is the most common cause of:
Pernicious anemia (B12 deficiency)
716
A 66-year-old man has had cough and worsening shortness of breath for 3 years. On examination, there is dullness to percussion at both lung bases and poorly audible breath sounds. On physical examination, pulse is 77/min and BP is 110/80 mm Hg. He does not have anginal pain. His liver span is increased to 14 cm. He has pitting edema to his knees. Jugular venous distention is noted to the angle of the jaw at 45-degree elevation of his head while lying down. What is most likely causing his heart disease?
Smoking These findings are consistent with right-sided congestive heart failure leading to peripheral edema, body cavity effusions (pleural effusions in this case), passive congestion of the liver, and jugular venous distension. Pure right-sided failure is less common than left-sided failure, and the former most often follows pulmonary disease (cor pulmonale). Chronic obstructive pulmonary disease (COPD) is more common than restrictive lung disease, and smoking leads to COPD.
717
The t(14;18) translocation is a feature of:
follicular lymphomas, which are distinctive B-cell tumors that involve the nodes and produce a follicular pattern.
718
Name the dx: Accumulation of blood between the layers of the tunica vaginalis; May develop after trauma or hemorrhage into hydrocele; Can also be caused by testicular tumors and infections
Hematocele
719
What is the diagnosis? Hereditary syndrome; Cerebellar hemangioblastomas are associated with retinal angiomas, renal cell carcinomas;
Von Hippel-Lindau syndrome Mutation in the VHL tumor suppressor gene
720
Responsible organism: Erythema chronicum migrans, then migratory musculoskeletal pain, then cardiac or neurologic abnormalities like meningitis or facial nerve palsy.
Borrelia burgdorferi - spirochete; Lyme disease
721
Intermittent claudication is seen in patients with T2DM, caused by what?
Atherosclerosis
722
BCL6 rearrangements or mutations in 30% of cases; staining pattern indicates a B-cell proliferation (CD19+, CD10+); T-cell (CD3) and monocytic (CD15) markers are negative; enlargement of the Waldeyer ring of oropharyngeal lymphoid tissue; \*\*no hepatosplenomegaly
diffuse large B-cell lymphoma
723
Patient with chronic Hep B; numerous red lesions; purpuric small papules, do not blanch with pressure; necrotizing leukocytoclastic venulitis; immune complex deposition in vascular walls
Hypersensitivity Angiitis aka Cutaneous necrotizing vasculitis (CNV) C5a attracts neutrophils --\> endothelial damage and fibrin disruption; can be primary or associated with something chronic
724
What is nephrotic syndrome?
Heavy proteinuria; Hypoalbuminemia; Hyperlipidemia; Edema;
725
BMPR2 is associated with development of what pathology?
BMPR2 is associated with development of primary pulmonary hypertension.
726
The symptoms of hypoparathyroidism (decreased serum calcium and decreased PTH) result from hypocalcemia, and cause:
Increased neuromuscular excitability: mild tingling in hands and feet, severe muscle cramps, laryngeal stridor, convulsions, as well as neuropsychiatric manifestations such as depression, paranoia, psychoses
727
Name the diagnosis: Kids; IgA deposits in walls of small blood vessels; purpuric skin lesions; glomerulonephritis
Henoch-Schonlein purpura
728
Clonal rearrangement of immunoglobulin genes; CD19+, CD5+, and deoxynucleotidyl transferase negative (TdT−); blood smear shows monotonous population of small, round, mature-looking lymphocytes; elderly patient
chronic lymphocytic leukemia (CLL), a clonal B-cell neoplasm in which immunoglobulin genes are rearranged, and T-cell receptor genes are in germline configuration. There is typically a tissue component of small lymphocytic lymphoma (SLL).
729
What is the diagnosis?
Sudden onset worst HA of a person's life; Nuchal rigidity; Lumbar puncture shows xanthochromia (yellow hue from bilirubin);
Subarachnoid hemorrhage
Can be due to rupture of a berry aneurysm (most common), AV malformations, anticoagulated state;
Most frequently in the anterior circle of willis at branch points of ACA;
Can be seen in Marfan syndrome and AD polycystic kidney disease
730
Hypopigmented macules are an early finding of:
Tuberculoid leprosy mycobacterium leprae granulomas present positive lepromin test
731
translocation in PML/RAR (retinoic acid receptor)
APL 15;17
732
Name the disorder characterized by: 1. Genetic vWF deficiency 2. Labs: Increased bleeding time High PTT Normal PT Decreased factor VIII half-life Abnormal ristocetin test 3. Treatmet is desmopressin which releases vWF from Weibel-Palade bodies
Von Willebrand Disease
733
The Ig genes in myeloma cells always show evidence of somatic hypermutation. Patients with myeloma often have infections with ____________ because of decreased production of IgG, required for opsonization.
encapsulated bacteria
734
Dx: Hepatic tumor of hepatocytes; Associated with oral contraceptive use - regresses when drug is stopped; Risk of rupture and intraperitoneal hemorrhage, especially during pregnancy
Hepatic adenoma
735
Give the dx: Widespread involvement of the glomeruli; Diffuse proliferation of mesangial and endothelial cells (biopsy shows hypercellularity); Immune complex deposition present on both sides of the BM, in the mesangium and capillary loops; Glomeruli with thickened basement membranes called "wire loop" lesions;
Proliferative glomerulonephritis (severe form of lupus nephritis)
736
What is the diagnosis? Wickham striae on the buccal mucosa (fine white lacey lesions)
Lichen planus May be associated with SCC or dysplasia
737
An infant born at term is noted to have cyanosis during the first week of life. On examination a heart murmur is auscultated. Abnormal findings with echocardiography include an overriding aorta, ventricular septal defect, right ventricular thickening, and pulmonic stenosis involving the fetal heart. This infant is most likely to have an inherited mutation involving which gene?
NOTCH2 There are a number of mutations in genes linked to congenital heart disease that encode proteins in transcription or signaling pathways. The NOTCH pathway plays a role in modulation of vascular development, including cardiac outflow tracts. NOTCH2 mutations are associated with tetralogy of Fallot, as in this infant.
738
Antibodies are seen against what part of the BM in Goodpasture syndrome?
The noncollagenous domain of the alpha-3 chain of type IV collagen
739
What is the diagnosis? Metaplasia of lower esophageal mucosa from stratified squamous to nonciliated columnar with goblet cells
Barrett esophagus May progress to dysplasia and adenocarcinoma
740
What is the effect of the following teratogen?
Thalidomide
Limb defects
741
HMB-45 is a useful tumor marker for:
Melanoma Mets can go to the brain - test for HMD-45 in the brain
742
Dx: Bird to human transmission; Interstitial pneumonia; Persisten dry cough, fever, HA etc.
Psittacosis - pneumonia; Chlamydia psittaci
743
Accumulation of mucopolysaccarides
Hurler disease
744
A deficiency of ADAMTS13, from an acquired antibody to this metalloproteinase or a genetic mutation in the encoding gene, can lead to accumulation of large von Willebrand multimers that promote platelet microaggregate formation, resulting in what disorder?
TTP that is marked by a pentad of: microangiopathic hemolytic anemia, fever, neurologic changes, thrombocytopenia, renal failure.
745
Painless jaundice is a frequent initial symptom of:
Pancreatic cancer
746
Dx: Inflammation and fibrosis of intrahepatic and extrahepatic bile ducts; Periductal fibrosis with an onion-skin appearance; Beaded appearance on imaging
Primary Sclerosing Cholangitis Patients present with obstructive jaundice
747
Does basal cell carcinoma metastasize?
No common location is the nose
748
Why do patients with acute tubular injury excrete large volumes of urine?
the glomerular filtrate cannot be adequately reabsorbed by the damaged tubular epithelium
749
Surgical removal of what organ may be curative for many myasthenia gravis patients?
Thymus Many have thymoma that contributes to their illness.
750
Pt. is a singer who presents with hoarseness that resolves with resting of voice - what might they have?
vocal cord nodule - nodules on true vocal cords that develop from overuse, usually bilaterally, composed of degenerative(mixoid) CT. not a laryngeal papilloma (also has hoarseness) because resolves with non-use
751
Lichen Sclerosis
Postmenopausal women
Benign, slight increased risk for squamous cell carcinoma
Leukoplakia on vulva
Can be associated with autoimmune disorders
painful intercourse
752
Pleomorphic adenoma is known for what characteristic?
Local recurrence after excision
753
What is Dressler dyndrome?
Postmyocardial infarction syndrome; delayed form of pericarditis that develops 2-10 weeks postMI; pain appears anginal
754
Menstrual phase
loss of progesterone support
755
Anti-dsDNA, anti-Smith
SLE
756
Schilling test tests:
B12 normal - no B12 deficiency
757
The foamy change in the tubular epithelial cells and ultrastructural alterations of the basement membrane are characteristic features of:
Alport syndrome The genetic defect results from mutation in the gene for the α5 chain of type IV collagen.
758
Toxic megacolon is a complication of:
C. Diff, ulcerative colitis
759
Renin is release by the:
Juxtaglomerular apparatus
760
Lithium causes goiter how?
Suppresses thyroid hormone synthesis (nontoxic goiter)
761
What is the difference between oral candidiasis and leukoplakia?
Oral candidiases can be scraped away easily. Leukoplakia cannot. The latter represents squamous cell dysplasia
762
Placenta Previa
implantation of placenta in lower uterus,
blocks os
3rd trimester bleeding
often requires C-section
763
Thrombotic thrombocytopenic purpura (TTP)
Thrombotic thrombocytopenic purpura (TTP) is a rare disorder. It is frequently seen in women and is characterized by a pentad:
Thrombocytopenia with purpura
Fever
Neurologic symptoms (altered mental status)
Microangiopathic hemolytic anemia
Acute renal failure
Rare disorder due to **deficiency of ADAMTS13,** which normally cleaves large von Willebrand multimers Increased bleeding time, decreased platelet count, elevated indirect bilirubin and LDH
Schistocytes in the blood smear
**Normal PT and PTT**
764
Name the diagnosis: Vasculitis characterized by ocular inflammatory lesions, including interstitial keratitis, uveitis, and episcleritis, and inner ear disease, including sensorineural hearing loss and vestibular dysfunction. Vasculitic manifestations may include arteritis (affecting small, medium, or large arteries), aortitis, aortic aneurysms, and aortic and mitral valvulitis.
Cogan's syndrome
765
Dx: Postprandial pain and weight loss; Bloody diarrhea
Ischemic colitis
766
Subacute Sclerosing Panencephalitis is a progressive encephalitis leading to death, and is a complication of:
Measles virus
767
What kind of emphysema involves most of the lung lobule and can be seen in all lobes; α1-antitrypsin deficiency is the most likely antecedent?
Panacinar (panlobular) emphysema involves most of the lung lobule and can be seen in all lobes; α1-antitrypsin deficiency is the most likely antecedent.
768
Osteosarcoma
highly malignant proliferation of osteoblasts mutations in Rb protein (some p53, but mostly Rb) teenagers, elderly lung mets Codman triangle ("sunburst appearance") arises in metaphysis of long bones or knee region pathologic fracture or bone pain
769
Give the dx: Children, facial puffiness; Kidney looks normal on light microscopy but foot processes fused on EM;
Minimal change disease
770
Give the dx: rare congenital disease and leads to bilateral, symmetric renal enlargement manifested in utero, with renal failure evident at birth.
Autosomal recessive polycystic kidney disease (ARPKD)
771
Synovial sarcoma
highly malignant soft tissue tumor spindle-shaped mesenchymal cells and cuboidal, epithelial-like cells keratin +
772
Subtype of Hodgkin Lymphoma; Seen in young adults, mainly women; thick bands of fibrosis; mediastinal involvement common
Nodular sclerosis
773
A 12-year-old girl has a 7-year history of coughing and wheezing and repeated attacks of difficulty breathing. The attacks are particularly common in the spring. During an episode of acute respiratory difficulty, a physical examination shows that she is afebrile. Her lungs are hyperresonant on percussion, and a chest radiograph shows increased lucency of all lung fields. Laboratory tests show an elevated serum IgE level and peripheral blood eosinophilia. A sputum sample examined microscopically also has increased numbers of eosinophils. What histologic feature is most likely to characterize the lung in her condition?
Remodeling of airways with smooth muscle hyperplasia Atopic asthma is a type I hypersensitivity reaction in which there are presensitized, IgE-coated mast cells in mucosal surfaces and submucosa of airways. Contact with an allergen results in degranulation of the mast cells, with both immediate release (minutes) of mediators such as histamine to promote bronchoconstriction, and delayed release (an hour or more) of leukotrienes and prostaglandins via the arachidonic acid pathway; these attract leukocytes, particularly eosinophils, and promote bronchoconstriction. The characteristic histologic changes in the bronchi, including remodeling of airways and smooth muscle hyperplasia, result from the episodes of inflammation.
774
Dx: Autoimmune, granulomatous destruction of the intrahepatic bile ducts; Antimitochondrial antibody seen in labwork
Primary Biliary Cirrhosis
775
Infectious Arthritis
DGI or staph warm joint, limited ROM, fever, ESR elevated usually knee
776
What is the main way to control microvascular disease in diabetic patients?
Control of blood sugar
777
Dx: Adenoma of pituitary leading to acromegaly; Hyperplasia of parathyroids leading to hypercalcemia; Adenoma of exocrine pancreas (gastrinoma)
MEN type 1 Can result in Z-E syndrome Mutations in MEN1 tumor suppressor gene
778
Does FSGS affect renal vessels?
No, but GPA does
779
IV drug users that devlop endocarditis can have kidneys that display what pathology?
Wedge-shaped regions of yellow-white cortical necrosis --emboli infarcting the cortex of the kidneys septic emboli can produce abscesses where they lodge in the vasculature
780
What is the most common cause of constrictive pericarditis?
Constrictive pericarditis follows a previous suppurative or tuberculous pericarditis.
781
inflamation of the epiglottis that results in high fever, sore throat, drooping with dysphagia, muffled voice, inspiratory stridow, and risk of airway obstruction
acute epiglottitis
782
Ewing Sarcoma
neuroectoderm derivation onion-skin appearance kids small, round blue cells on biopsy diaphysis of long bones t 11;22 translocation
783
HPV E7 protein
increased destruction of Rb
784
25% chance of WHAT in pregnant women with acute Hep E?
Death
785
What is the dx? Yellow plaque of lipid-containing macrophages usually located on the eyelids; Seen in patients with hypercholesterolemia
Xanthelasma
786
Non smokers who develop hemoptysis and bronchial obstruction (increasing cough) may have what benign lung lesion?
Carcinoid tumor
Most pulmonary carcinoids are central obstructing masses involving a large to medium-sized bronchus.
These neuroendocrine tumors have unpredictable behavior, but many are localized, resectable, and follow a benign course.
787
Protrusion of the meninges is what form of spina bifida?
Meningocele
788
What kind of cancer is associated with chew tobacco?
Verrucous carcinoma of the oral cavity
789
What is the diagnosis? Esophageal web; Beefy red tongue due to atrophic glossitis; iron-deficiency anemia
Plummer-Vinson syndrome
790
The second most common carcinoma of the kidney in adults is papillary renal cell carcinoma, associated with what gene mutations?
MET can be familial or sporadic
791
What is most common cause of bacterial meningitis in children and teenagers?
Niseria meningitidis - enters through nasopharynx and then gets to blood then from blood gets to the meninges
792
Congenital atresia of ______ is the most common cause of congenital hydrocephalus.
Aqueduct of Sylvius
793
What is the diagnosis? Facial port-wine stain (Nevus Flammeus); Angiomas of the leptomeninges
Sturge-Weber syndrome Nonfamilial congenital disorder
794
Name the diagnosis corresponding to this autoantibody:
Antinuclear antibodies
SLE (nonspecific)
795
What bug is associated with meningitis and Waterhouse-Friderichsen syndrome?
Neisseria meningitidis
796
How can you differentiate aphthous ulcers from primary HSV-1 infections?
primary HSV-1 doesn't have grey base of lesion and usually occurs in childhoord. HSV-1 reactivations don't have grey base and also occur on the lips
797
inborn error of metabolism/lysosomal storage disorder caused by autosomal recessive mutation in lysosomal enzyme arylsulphatase. Causes accumulation of the cerebroside galactosyl sulphatide in white matter leading to astrogliosis, failure to thrive and death (~6 months) from respiratory insufficiency
multichromatic leukodistrophy - most common leukodystrophy accumulation of sufatides (breakdown product of myelin-sulfatides) in lysosomes and "metachromatic material" in white matter
798
Disease marked by degeneration of corpus striatum resulting in extrapyramidal symptoms (lack of coordination, tremor), chronic hepatitis/cirrhosis, deposition of copper in the iris causing Keyser-Feischer ocular lesions
Wilson disease
799
Calcific aortic stenosis leads to what cardiac pathology?
Calcific aortic stenosis leads to left-sided congestive heart failure (CHF), and the extra workload of the left ventricle may cause angina pectoris.
800
Name the disorder characterized by: 1. Autoimmune IgG against platelet antigens is GP2B-3A 2. Thrombocytopenia due to ab-bound platelets being consumed by splenic macrophages 3. Acute and Chronic versions --\> acute - children --\> chronic - usually women of childbearing age; can be associated with SLE 4. Labs: Low platelets Normal PT/PTT High megakaryocytes in bone marrow 5. Treat with corticosteroids and splenectomy for refractory cases. IVIG used for short-term therapy.
ITP Immune Thrombocytopenic Purpura
801
focally ulcerated reddish lesion ~2mm - 1cm located on the gingiva of the mouth with highly vascularized granulation tissue
pyogenic granuloma
802
What is the diagnosis? Vomiting, polyhydraminos, abdominal distention and aspiration
Tracheoesophageal fistula Congenital defect resulting in connection between trachea and esophagues; Proximal esophageal atresia with the distal esophagus arising from the trachea - common form
803
Membranous nephropathy is most likely to produce nephrotic syndrome (\>3.5 proteinuria) with or without crescents?
Without
804
Herpes simplex type 1 targets has tropism for what parts of the brain?
Temporal lobes
805
Acute cystitis involves what symptoms:
Pelvic pain; Hemorrhage; Stromal edema; Neutrophilic infiltrate on urinalysis
806
An unsuccessful dexamethasone test suggests a tumor where?
Adrenal cortex fasiculata - glomerulosa - aldosterone
807
What is the diagnosis? Cerebral calcifications and convulsions in a neonate; Intranuclear and intracytoplasmic inclusions in neurons and astrocytes; TORCH infection
CMV
808
Common cause of meningitis in immunocompromised patients?
fungal infections of criptoccocus and aspergillus
809
Dx: Recurrent bouts of bloody diarrhea; Abdominal pain
Chronic IBD Dx of exclusion
810
A crescent-shaped lesion on head CT suggests?
Subdural hematoma
811
Condyloma
HPV 6 and 11
warty neoplasm, not risk for cancer histology - koilocytic change
812
Name the diagnosis corresponding to this autoantibody:
Anti-hemidesmosome
Bullous pemphigoid
813
Name the dx: Developmental abnormality resulting in a vesicle-umbilical fistula or urachal diverticulum
Failure of the urachus to involute
814
Name the dx: Congenital anomaly in which the urethra opens on the upper side of the penis
Epispadias
815
visual disturbances; dizziness; headache; Raynaud phenomena; bone marrow is infiltrated with plasmacytoid lymphocytes that have stored immunoglobulins in their cytoplasm (Russell bodies)
Hyperviscosity syndrome associated with lymphoplasmacytic lymphoma (Waldenström macroglobulinemia). In this disorder, neoplastic B cells differentiate to IgM-producing cells; there is a monoclonal IgM spike in the serum. These IgM molecules aggregate and produce hyperviscosity, and some of them agglutinate at low temperatures and produce cold agglutinin disease.
816
A choriocarcinoma with trophoblastic cells is usually gestational in origin and has a:
hemorrhagic appearance
817
What is the most common testicular tumor in children?
Mature teratoma
818
T/F: Coarctation of the aorta is associated with cyanosis.
False
819
Pus in the pleural space is called \_\_\_\_\_\_\_\_\_, and typically complicates an existing pneumonia.
Pus in the pleural space is called empyema, and typically complicates an existing pneumonia.
820
Name the dx; Most common primary tumor of the bladder; Painless hematuria; Epithelial tumor; Papillary fronds lined by urothelial epithelium; Minimal cytologic atypia on histology
Exophytic papilloma;
821
Give the diagnosis for the following auto-antibody:
Anti-Scl-70 (anti-DNA topoisomerase I)
Scleroderma (diffuse)
822
Primary Sclerosing Cholangitis is associated with what other autoimmune disorder?
Ulcerative colitis
823
Duret hemorrhage and causes
small midbrain bleeds cause by downward movement of brainstem or compression of midbrain
824
Associate what diseases with Ulcerative colitis?
Primary sclerosing cholangitis; p-ANCA positivity
825
What is most common cause of bacterial meningitis in adults and elderly?
Strep pneumoniae
826
How does infection with Nocardia present?
Nocardiosis of the lung appears mainly as chronic abscessing inflammation.
827
What is myopia?
Near-sightedness Light from the visualized object at a point in front of the retina because of a longer than usual anteroposterior diameter of the eye
828
T/F: In children, primary tumors are usually infratentorial.
True In adults, primary tumors are usually supratentorial
829
Dx: Bacterial infection of bile ducts; Usually due to ascending infection with enteric gram-neg bacteria; Presents as sepsis, jaundice, abdominal pain; Increased incidence w/ choledocholithiasis
Ascending cholangitis
830
A dexamethasone suppression test that reduces corticotropes suggests what kind of tumor?
Pituitary (ACTH-secreting) adenoma
831
Tumors composed exclusively of malignant chorionic epithelium are termed:
Choriocarcinomas - a kind of testicular cancer - a "nonseminomatous germ cell tumor" trophoblastic cells give rise to multinucleatied giant cells --\> hemorrhage hCG levels high
832
Immune complex deposition in adults is/is not steroid responsive?
Is not
833
Name the dx: Malignant tumor of the neural crest; Can originate in the adrenal medulla or sympathetic ganglia; Pseudorosettes featuring tumor cells frequently seen clustering around small vessels, "blue cell" appearance; Tumor cells may secrete catecholamines (excreted as vannilylmandelic acid in the urine);
Neuroblastoma \*\*amplification of n-myc
834
Light chains in urine (Bence Jones proteins) are a feature of:
Multiple myeloma
835
Dx: Starts in rectum and extends to colon; Mucosal and submucosal ulcers; LLQ pain w/ bloody diarrhea; Loss of haustra (lead pipe sign on imaging); Crypt abscess w/ neutrophils
Ulcerative Colitis
836
Cause of subacute sclerosing panencephalitis
reactivation of latent measles virus
837
Acute Endometritis
usually due to retained products of conception,
incompete abortion is nidus for inection bacterial infection --\> fever, pelvic pain
838
Where is the villous damage in tropical sprue?
Most prominent in the jejunum and ileum (in celiac, damage is in duodenum); Respond to antibiotic tx
839
What is the most common endocrinopathy associated with pituitary adenoma?
Hyperprolactinemia Functional lactotrope adenomas lead to amenorrhea, galactorrhea and infertility Can cause loss of libido in men
840
Following an uncomplicated pregnancy, a term infant appears normal at birth, but at 1 day of life the infant develops respiratory distress. On physical examination the infant has tachypnea, tachycardia, and cyanosis. There is an S1 ejection click and a split S2 with prominent P sound. A radiograph shows normal heart size but prominent hilar vascular markings. Echocardiography shows a small left atrium, large right atrium, normally sized ventricles, widely patent foramen ovale, and normally positioned aorta and pulmonary trunk. What type of congenital heart disease does this infant most likely have?
**Total anomalous pulmonary venous connection**
This shunt results from **abnormal confluence of pulmonary veins leading to the right atrium** (or systemic veins), and not the left.
Obstruction is often present, as in this case, with pulmonary congestion.
Deoxygenated systemic and oxygenated pulmonary venous blood mix in the right atrium.
841
Acute angle glaucoma
Patients with angle-closure glaucoma often present with severe ocular pain accompanied by the presence of halos around lights, headache, nausea, vomiting, eye redness, corneal edema, and elevated intraocular pressure (IOP).
Agents that cause pupillary dilation can lead to angle-closure glaucoma.
Pupillary dilation "narrows the angle" in the anterior chamber of the eye, thus decreasing drainage of aqueous humor.
Two muscles control the iris of the eye: the pupillary sphincter (constrictor) muscle and the radial dilator muscle.
842
What kind of cell proliferates in membranoproliferative glomerulonephritis?
Mesangial cells phagocytic fx and can proliferate in response to injury
843
HIV is best associated with what neoplasms?
HIV is best known to be associated with non-Hodgkin lymphomas and with Kaposi sarcoma
844
Area of black discoloration newborns
Mongolian spot
845
Dx: Excess body iron leads to deposition in the tissues and organ damage; Damage is mediated by generation of free radicals
Hemochomatosis May be primary or secondary Increased risk of hepatocellular carcinomina
846
Aflatoxins from Aspergillus raise the risk for:
Hepatocellular carcinoma Aflatoxins induce p53 mutations
847
What is the dx? Yellowish conjunctival lump usually located nasal to the corneoscleral limbus
Pinguecula
848
What is the diagnosis? Benign tumor of Schwann cells; Involves cranial or spinal nerves most commonly CN VIII at the cerebellopontine angle (loss of hearing or tinnitus); Tumor cells are S-100 positive; Bilateral tumors are seen in Neurofibromatosis Type II
Schwannoma
849
non-familial congenital disorder featuring facial "port wine stain" (nevus flammeus) and leptominingeal angiomas
Sturge-Weber syndrome
850
Granulosa cell tumor
sex cord tumor prototypical functional neoplasm of the ovary associated with estrogen secretion usually postmenopausal women (in young girls, precocious puberty is an indicator) Exner bodies (look like flowers) on histology
851
A 67-year-old woman with glucose intolerance, hypertension, central obesity, and hyperlipidemia has increasing dyspnea from worsening congestive heart failure. Echocardiography shows a left ventricular ejection fraction of 25%. Percutaneous transluminal coronary angioplasty is performed with placement of a left anterior descending arterial stent containing paclitaxel. What long-term complications in her coronary artery is most likely to be prevented by paclitaxel?
Proliferative restenosis She has metabolic syndrome, a risk for coronary atherosclerosis. Following angioplasty, there is often intimal thickening that causes restenosis. The wire stent holds the lumen open and the paclitaxel limits smooth muscle hyperplasia
852
What is farmer's lung?
Farmer’s lung is a form of hypersensitivity pneumonitis caused by inhalation of actinomycete spores in moldy hay. These spores contain the antigen that incites the hypersensitivity reaction. Because type III (early) and type IV immune hypersensitivity reactions are involved, granuloma formation can occur. The disease abates when the patient is no longer exposed to the antigen. Chronic exposure can lead to more extensive interstitial lung disease.
853
A 41-year-old woman has been awakened at night with “air hunger” for the past year. She notes sleeping better while sitting up in bed. Her serum B-type natriuretic peptide is \>400 pg/mL (very high). What cardiac disease best explains her condition?
Rheumatic valvulitis Paroxysmal nocturnal dyspnea is a feature of left-sided congestive heart failure, and rheumatic heart disease most often involves the mitral, aortic, or both valves, and left-sided valvular disease leads to pulmonary edema.
854
What is a more common cause of PSGN in the US than Strep pyogenes?
Staph Aureus (with IgA antibodies)
855
What kind of E. Coli causes HUS?
verocytoxin-producing, O157:H7 This Shiga toxin damages endothelium, reducing nitric oxide, promoting vasoconstriction and necrosis, and promoting platelet activation to form thrombi in small vessels.
856
Name the disorder characterized by: 1. Resembles DIC 2. Increased bleeding 3. Labs: increased PT/PTT increased bleeding time normal platelet count increased fibrinogen split products w/o d-dimer 4. Treatment is with aminocaproic acid which blocks activation of plasminogen
Disorders of fibrinolysis (removing the clot)
857
Name the dx: Most common thyroid tumor in younger women; tendency to metastasize to regional lymph nodes; Surgery is usually curative
Papillary carcinoma of the thyroid
858
Bacillary angiomatosis produces a focal vascular proliferation, typically on the skin, of an immunocompromised person infected with:
Bacillary angiomatosis produces a focal vascular proliferation, typically on the skin, of an immunocompromised person infected with Bartonella spp.
859
What is the diagnosis? Cellulitis involving the submaxillary and sublingual space
Ludwig Angina follows facial planes due to aerobic or anaerobic strep eikenella or corrodens causes are trauma or dental work
860
What is the diagnosis? Painful vesicles or small white papules occur on an erythematous base typically at the junction of the soft and hard palate
Herpangina due to coxackievirus When vesicles located in mouth and distal extremities, it's hand, foot and mouth disease
861
Strep pneumo
Streptococcus pneumoniae is the most common cause of lobar pneumonia in adults. It is a **gram-positive, catalase-negative diplococcus.**
It is **alpha-hemolytic, bile-soluble, and inhibited by Optochin.** For outpatient therapy of community-acquired pneumonia in adults, the recommended treatment is a macrolide (azithromycin or clarithromycin) or doxycycline.
862
T/F: Most acute infections of Hep C go unnoticed.
True, 80% subclinical
863
Give the dx: Most common type of glomerulonephritis in adults; Deposition of IgA-dominant immune complexes causes nephropathy; Exacerbations occur after RTIs or GI bugs; Renal biopsy shows intense mesangial IgA, almost always with C3; Mesangial cell proliferation; Patients present with hematuria, proteinuria, some RBC casts
Berger disease (a kind of focal proliferative glomerulonephritis)
864
The anti-streptolysin O titer is increased in what kidney disease?
poststreptococcal GN --granular pattern of immune complex deposition
865
What is the diagnosis? Most common malignant salivary gland tumor; Most commonly in the parotid gland; Mixture of neoplastic squamous and mucus-secreting cells
Mucoepidermoid carcinoma
866
What kinds of malaria are not able to form hypnozoites (dormant forms in the liver)?
P. falciparum;
P. malariae
Therefore incapable of causing relapses
867
Dx: Multiple small diverticula in the wall of the esophagus
Intramural pseudodiverticulum
868
Irregular border and dark red nodular mass at the center of the lesion
Nodular melanoma
869
How does hyperparathyroidism cause peptic ulcer disease?
Higher PTH --\> higher serum calcium --\> gastrin secretion up --\> peptic ulcer disease
870
The majority of pancreatic carcinomas arise from what cell type?
Ductal cells
871
When do CK-MB levels rise after MI?
2-4 hours Specific, but not very sensitive
872
What is the diagnosis? Benign tumor of nasal mucosa composed of large blood vessels and fibrous tissue; Classically seen in adolescent males; Presents with profuse epistaxis
Angiofibroma
873
what causes benign tumors on the vocal cords that present with hoarseness and does not get better with resting voice?
HPV 6 or 11 laryngeal papilloma
874
Amiodarone is used to treat \_\_\_\_\_\_\_\_\_\_\_\_
Amiodarone is used to treat intractable arrhythmias.
875
What is the diagnosis? Men; Benign parotid gland tumor; Smokers at increased risk; Heterotropic salivary gland tissue is trapped in a lymph node
Warthin tumor (papillary cystadenoma lymphomatosum)
876
In all forms of HL except ____________ type, the Reed-Sternberg cells and variants stain with CD15.
lymphocyte predominance type
877
Small cell carcinomas are frequently associated with what other symptoms?
Paraneoplastic syndromes such as Cushing syndrome and SIADH Cushing syndrome is a paraneoplastic syndrome resulting from ectopic corticotrophin production (most often from a pulmonary small cell carcinoma), which drives the adrenal cortices to produce excess cortisol. syndrome of inappropriate secretion of antidiuretic hormone (SIADH), marked by free water retention with hyponatremia
878
Erythematous scaling rash thickening of skin possible (lichenification)
Atopic dermatitis
879
What kind of granulomas are found in TB?
Caseating - soft core surrounded by epithelioid macrophages, Laghans giant cells, lymphocytes, peripheral fibrosis
880
Hep A virus phylogeny:
SS RNA nonenveloped
881
X-linked MD
dystrophin deletion replacement of skeletal muscle by adipose tissue
882
Cervical Carcinoma
40-50 yrs
post-coital bleeding
high risk HPV infection
cervical carcinoma is AIDS-defining
most is squamous cell carcinoma, but both squamous cell and adenocarcinoma are associated with HPV
**hydronephrosis w/ postrenal failure is a common cause of mortality**
CIN to cancer 10-20 years
883
Carcinosarcoma
aggressive mixed mesodermal tumor poor prognosis
884
Give the dx: Congenital failure of small bowel to canalize - duodenum is ending in a blind loop; Polyhydraminos; "Double bubble" sign; Bilious vomiting
Duodenal Atresia
885
invasive brain tumor that exhibits vascular changes ("arteric obliterateration"), pallasading pleiomorphic, multinuclear tumor cells around patches of yellow necrosis, and redzones of hemorrhage
glioblastoma multiforme
tumor cells comprised of astroyctes can present with "butterfly lesion",
large tumor extending bridge of malignant cells across corpus collosum can result in ipsilateral symptoms to large tumor.
886
A 19-year-old man has a history of recurrent mucoid rhinorrhea with chronic sinusitis and otitis media since childhood. He has experienced multiple bouts of pneumonia. His temperature is 37.7° C. On examination of his chest, there is tactile fremitus, rhonchi, and rales in lower lung fields. Nasal polyps are noted. A chest radiograph shows bronchial dilation with bronchial wall thickening, focal atelectasis, and areas of hyperinflation; his heart shadow appears mainly on the right. What abnormality is he most likely to have?
Ciliary dyskinesia He has Kartagener syndrome (sinusitis, bronchiectasis, and situs inversus associated with ciliary dyskinesia). There is an abnormality of ciliary dynein arms that diminishes the mucociliary function of the respiratory epithelium, predisposing to recurrent and chronic infections of both upper and lower respiratory tract.
887
Blisters leave extensive denuded or crusted areas; separation of the stratum spinosum and basal layer; IgG on direct immunofluorescence; autoantibody to desmoglein-3; blister contains moderate #s of lymphocytes, macrophages, eosinophils and neutrophils
Pemphigus vulgaris
888
pruritic, purple, polygonal papules indicate what disease?
lichen planus
889
Labarynthitis
same as otitis interna - infection of inner ear and vestibular system commonly caused by viruses (e.g. mumps, rubella
890
Most common malignant tumor in pale-skinned people; locally aggressive; metastases rare;
Basal cell carcinoma
891
Histologic features of subacute sclerosing panencephalopathy
patchy loss of myelin, prominant intranuclear inclusions in neurons and oligodendrocytes, marked gliosis affecting grey and white matter, perivascular lymphocytes and macrophages
892
What is the cause of heart failure in rheumatic heart disease?
Myocarditis leads to dilation of the ventricle so severe that the mitral valve becomes incompetent.
893
Give the dx: High proteinuria; Deposition of kappa or lambda light chains in the glomerular basement membranes and mesangial matrix; Amorphous material blocks glomerular capillaries
Amyloid nephropathy Renal amyloidosis leads to nephrotic syndrome
894
IgA nephropathy (mesagial IgA staining byimmunofluroescence) occurs with increased frequency in patients with:
Celiac disease, liver disease
895
enlarged lymph nodes; mild hepatosplenomegaly; small lymphocytes with plasmacytoid lymphotcytes containing Dutcher and Russell bodies; cytoplasmic accumulation of IgM; mainly affects the elderly
Waldenstrom disease - lymphoplasmacytic lymphoma; neoplastic proliferation of small lymphocytes; IgM secreting lymphocytes
896
What is the genetic defect responsible for Freidrich Ataxia
autosomal recessive mutation of the frataxin gene due to the expansion of an unstable trinucleotide repeat (GAA)
897
vesicles in the oral mucosa that rupture and result in shallow painful red ulcers. Usually seen in children. What is this? What causes it?
Primary HSV-1 infection Lesions heal but virus remains dormant in trigeminal nerve ganglion
898
Dx: Failure to form or early destruction of extrahepatic biliary tree; Leads to biliary obstruction within first 3 months of life; Presents with jaundice and progresses to cirrhosis
Biliary atresia
899
Secretory phase
progesterone driven
900
Left ventricular failure leads to \_\_\_\_\_\_
Left ventricular failure leads to pulmonary edema.
901
Honey-colored crusts over an erythematous base
Impetigo Mostly staph aureus some strep pyogenes
902
Kayser-Feischer rings
ocular lesions from deposition of copper in the Descemet membrane of the iris
903
Solid cystic lesion displacing the region of the hypotalamus (located above stella turcica) that arises from the Rathke pouch and compresses the medial aspect of the optic chiasm resulting in bitemporal hemianopsia, headaches, and sometimes pituitary failure that can result in diabetes insipidus
Cranialpharyngioma Rathke pouch = part of embyronic nasopharynx that migrates cephalad to give rise to the anterior lobe of the hypothalamus
904
Lymphogranuloma venereum
Chlamydia serotype enlarged inguinal lymph nodes genital elephantiasis rectal strictures granulomatous inflammation \*with\* inclusion bodies\*\*
905
Name the diagnosis: Exquisitely painful red, raised lesion; Benign; Branching vascular channels in connective tissue stroma and aggregates of specialized ____ cells
Glomus tumor
906
Asherman Syndrome
Secondary amenorrhea due to loss of stratum basalis
overaggressive D&C cause
907
Where are stellate cells found in the liver?
Beneath the endothelial cells that line the sinusoids
908
Give an example of a type IV hypersensitivity rxn in the kidney.
Renal transplant rejection; SOME cases of drug-induced acute interstitial nephritis Transplant rejection - T-cell injury with tubulitis
909
What is pseudotumor cerebri?
ideopathic intracranial hypertension (increased pressure without evidence of tumor/obstruction) [+] papilloedema [-] mental status changes [-] focal neurologic findings
910
Protrusion of edematous, inflammed mucosa that usually arises secondary to repeated bouts of rhinitis but also occurs in cystic fibrosis and aspirin-intolerant asthma.
Nasal polyp
911
T/F laryngeal papillomas are typically associated with epithelial dysplasia
False - not typically, only rarely pre-cancerous! But does occur - HPV 6 , 11 "Low risk" for cancer -- though can lead to laryngeal carcinoma in rare cases
912
Aschoff bodies are found in what diagnosis?
Rheumatic myocarditis is characterized by minimal myocardial necrosis with foci of granulomatous inflammation (Aschoff bodies).
913
Immune complex deposition of granular or linear in Goodpasture syndrome?
Linear
914
What are Mallory bodies and when are they seen?
Mallory bodies are damaged IFs within the hepatocytes and they are seen in alcoholic hepatitis
915
Name the diagnosis: Vasculitis that can affect arteries or veins. Characterized by recurrent oral and/or genital aphthous ulcers accompanied by cutaneous, ocular, articular, gastrointestinal, and/or central nervous system inflammatory lesions. Small vessel vasculitis, thromboangiitis, thrombosis, arteritis, and arterial aneurysms may occur.
Behcet disease
916
Intraductal Papilloma
bloody nipple discharge in premenopausal woman
\*subareolar (whereas Paget Disease is areola and nipple)
must distinguish from papillary carcinoma
fibrovascular projections lined by epithelium WITH myoepithelial cells (cancer has no myoepithelial cells)
917
Fibrosis and tightening of the skin; dysphagia; structural and functional involvement of other organs ie lungs and kidneys; Raynaud's phenomenon common
Scleroderma
918
An acute proliferative postinfectious GN would show what on light microscopy?
Hypercellular glomeruli with neutrophils \*\*NO BM thickening
919
8;14 translocation
Burkitt Lymphoma
920
white Koplick spots on buccal mucosa
Rubeola (measles)
921
C3NeF is an autoantibody directed against C3 convertase, and it is seen in:
Membranoproliferative GN
922
Protrusion of edematous, inflammed mucosa in a child should be checked for what? In adults?
cystic fibrosis Aspirin intolerant asthma
923
Dynein arms are absent or abnormal in what disease, which leads to bronchiectasis?
Dynein arms are absent or abnormal in Kartagener syndrome, which leads to bronchiectasis.
924
The C-ANCA test (PR3) result is often positive in what disease?
The C-ANCA test (PR3) result is often positive in granulomatosis with polyangiitis
925
Bernard Soulier
Bernard-Soulier syndrome is a rare autosomal recessive disorder characterized by the **absence of platelet glycoprotein Ib, a receptor for von Willebrand factor.**
926
\_\_\_\_\_\_\_\_\_\_ is a rare condition in which muscular arteries become occluded in the lower extremities in smokers.
Thromboangiitis obliterans (Buerger disease) is a rare condition in which muscular arteries become occluded in the lower extremities in smokers.
927
Fibromuscular dysplasia is a hyperplastic medial disorder, usually involving renal and carotid arteries; on angiography, it appears as what?
Fibromuscular dysplasia is a hyperplastic medial disorder, usually involving renal and carotid arteries; on angiography, it appears as a “string of beads” caused by thickened fibromuscular ridges adjacent to less involved areas of the arterial wall.
928
Give the dx: Systemic, necrotizing vasculitis; Granulomatous lesions in the nose, sinuses, lungs; Renal glomerular disease is often present (hematuria with RBC casts, focal glomerular necrosis with crescents and vasculitis affecting the aterioles and venules); C-ANCA often present
Granulomatosis with Polyangiitis (Wegener's) --can result in rapidly progressive glomerulonephritis
929
slow growing benign tumor that may displace brain tissue and erode contiguous bone of the skull. Does not infiltrate the brain.
meningeoma
930
Name the disorder characterized by: 1. Genetic GP1b deficiency 2. Impaired platelet adhesion 3. Blood smear: mild thrombocytopenia w/ enlarged platelets
Bernard-Soullier Syndrome
931
What is the diagnosis? Squamous cell carcinoma, nonkeratinizing squamous carcinoma, or undifferentiated cancer; Increased in Chinese and African populations Causal relationship with EBV
Nasopharyngeal carcinoma Most common malignant tumor of the nasopharynx
932
Name the diagnosis: Kids; Skin rash; Mucosal inflammation; Lymphadenopathy; Aneurysm formation on coronary arteries and HF re rare complications
Kawasaki disease aka mucocutaneous lymph node syndrome
933
Myositis ossificans
young people benign usually from blunt trauma occurs near a bone, often mistaken for a malignant bone thing
934
How do you tell if someone has had Hep B in the past?
Hep B CORE antiody - not part of vaccine series
935
Endometrial Hyperplasia
glands \>\> stroma consequence of unopposed estrogen (ie PCOS, obesity) atypia is the key word for progression to cancer postmenopausal uterine bleeding
936
Chronic Hep B is a major risk factor for hepatocellular carcinoma in what cultural group?
Asians
937
What is the diagnosis?
Shaggy gray pseudomembrane in posterior pharynx and upper airways
Diphtheria
Corynebacterium diphtheriae
938
A 26-year-old woman has had a fever for 5 days. On physical examination, her temperature is 38.2° C, pulse is 100/min, respirations are 19/min, and blood pressure is 90/60 mm Hg. A cardiac murmur is heard on auscultation. Her sensorium is clouded, but there are no focal neurologic deficits. Laboratory findings include hemoglobin, 13.1 g/dL; platelet count, 233,300/mm3; and WBC count, 19,200/mm3. Blood cultures are positive for gram-positive bacteria. Urinalysis shows hematuria. An echocardiogram shows a 1.5-cm vegetation on the mitral valve. What condition is this patient most likely to develop?
Cerebral arterial mycotic aneurysm She developed bacterial septicemia followed by infective endocarditis of the mitral valve. Thus she has a high risk for developing complications of infective endocarditis. Such valvular vegetations are destructive of the valve. The impaired functioning of the mitral valve (most likely regurgitation) would give rise to left atrial dilation and left ventricular failure with pulmonary edema. Septic emboli from the mitral valve vegetation could reach the systemic circulation and give rise to abscesses. Infection of an arterial wall can weaken the wall, resulting in aneurysm formation and the potential for rupture.
939
Most sporadic clear cell carcinomas show loss of both alleles of the ___ gene.
VHL
940
What does malakoplakia show on histology?
Chronic inflammatory cell infiltrate composed mostly of large macrophages with abundant eosinophilic cytoplasm containing PAS-positive granules. Some of these macrophages exhibit laminated basophilic calcopherites called "Michaelis-Gutmann" bodies
941
A 35-year-old woman has experienced multiple bouts of severe necrotizing pneumonia since childhood, with Haemophilus influenzae, Staphylococcus aureus, Pseudomonas aeruginosa, and Serratia marcescens cultured from her sputum. She now has a cough productive of large amounts of purulent sputum. On physical examination, there is dullness to percussion with decreased breath sounds over the right mid to lower lung fields. A chest radiograph shows areas of right lower lobe consolidation. A bronchogram shows marked dilation of right lower lobe bronchi. What mechanism is the most likely cause of her disease?
Recurrent inflammation with bronchial wall destruction Bronchiectasis is a chronic obstructive airway disease from irreversible dilation of bronchi that results from inflammation and destruction of bronchial walls after prolonged infections or obstruction. Serious bouts of pneumonia can predispose to bronchiectasis.
942
What is a possible complication of malignant hyperthermia after administration of an anesthetic such as halothane?
Rhabdomylysis
943
High peripheral reticulocyte count
Hemolytic anemia
944
Liposarcoma
lipoblast is characteristic cell
945
Vaginal Adenosis
focal persistence of columnar epithelium in the upper vagina
increased in females with **DES exposure in utero**
946
What is Polymicrogyria?
Common cause of mental retardation Surface of brain shows multiple small, irregularly sized, randomly-distributed gyral folds
947
Name the disorder characterized by: 1. Long, slender fingers 2. Mental retardation 3. Vessel thrombosis 4. Lens dislocation
Homocystinuria cystathionine beta synthase (CBS) deficiency resulting in high levels of homocysteine
948
What causes subacute slclerosing panencephalopathy? How is disease different in children vs adults?
reactivated measles virus In children time course is protracted and primarily affects cerebral grey matter in adults time course is more rapid
949
Oat cell cancers are central tumors and are strongly associated with:
Smoking
950
Hep E - what kind of virus?
nonenveloped ssRNA
951
What "pathogen" produces ferruginous bodies in the lungs?
Asbestos The ferruginous bodies are long, thin crystals of asbestos that have become encrusted with iron and calcium. The inflammatory reaction incited by these crystals promotes fibrogenesis and resultant pneumoconiosis.
952
Classic polyarteritis nodosa (PAN) can produce small microaneurysms in small arteries, most often where?
Classic polyarteritis nodosa (PAN) can produce small microaneurysms in small arteries, most often renal and mesenteric. --segmental involvement of medium-sized arteries with aneurysmal dilation in the renal and mesenteric vascular beds (e.g., abdominal pain, melena). PAN can affect many organs at different times. Although the cause of PAN is unknown, about 30% of patients have hepatitis B surface antigen that presumably forms immune complexes that damage vascular walls. In contrast to microscopic polyangiitis, PAN has less of an association with anti–neutrophil cytoplasmic autoantibody (ANCA).
953
Renin is stimulated when:
There is a decrease in Na and Cl delivery to the macula densa
954
Dx: Developmental malformation; Pancreas forms ring around the duodenum; Risk of duodenal obstruction
Annular pancreas
955
Name the dx: Lesions on the penis - shiny, soft, erythematous plaques on the glans and foreskin; Neoplastic epitheliam cells connected by intercellular bridges, with invasion to the dermis
Squamous cell carcinoma of the penis
956
Phyllodes Tumor
fibroadenoma-like tumor leaf-like projections on biopsy (benign growth of ductal structures) sharply circumscribed, glistening stroma has high mitotic activity postmenopausal women sometimes malignant (when they are, they are less sharply circumscribed and much more stromal components as compared with ductal components
957
Normal pressure hydrocephalus
Enlarged ventricles and normal pressure on lumbar puncture
Triad of **dementia, urinary incontinence, and gait abnormalities**
Relief of CSF pressure can be obtained by placing a ventricular shunt, which usually improves clinical symptomatology. A ventriculoperitoneal shunt drains excess CSF to the abdomen, where it is absorbed.
958
Uncontrolled prolifertion of megakaryocytes; increased platelets; episodes of thrombosis or hemorrhage; NO fibrosis in the bone marrow
Essential thrombocythemia
959
What is the diagnosis? deficiency of galactocerebroside beta-galactosidase activity; disease of white matter - accumulation of "globoid cells"
Krabbe disease rapidly progressive; fatal; AR disorder; presence of perivascular aggregates of mononuclear and multinucleated cells in the white matter
960
What is the diagnosis? Tumor that arises from epithelial remnants of Rathke's pouch; Presents as a supratentorial mass in a child or young adult; May compress optic chiasm --\> bitemporal hemianopsia; Calcifications seen on imaging (derived from "tooth-like" tissue); Benign but recurs after resection
Craniopharyngioma
961
What is the diagnosis: Gross appearance is Firm, glistening, bluish white cut surface; Single lesion in lung;
Hamartomas are uncommon but benign peripheral lesions of the lung. They are composed of benign-appearing epithelial cells and connective tissue, typically with a large component of cartilage. They are included in the differential diagnosis of a “coin lesion” that also includes carcinoma and granuloma.
962
What is an expected complication of corpulmonale?
Pleural effusions
963
Pseudogout / Chondrocalcinosis
aspirate has rhomboid "coffin-like" crystals (CPPD) and neutrophils seen in the elderly gout-like symptoms + biferingence
964
Osteopetrosis
inherited defect of bone resorption abnormally thick, heavy bones marrow can be compromised, tx is bone marrow replacement aka Albers-Shonberg disease disorder of osteoCLASTS (failed resorption)
965
Dx: Enlarged rugal folds of stomach; Severe loss of plasma proteins including albumin from altered gastric mucosa; in adults - overexpression of TGF-alpha
Menetrier disease aka hyperplastic hypersecretory gastropathy
966
cystic degeneration of the spinal cord caused by the presence of a fluid filled cavity that usually occurs between C8-T1 and results in sensory loss of pain and temperature with sparing of fine touch and position sense in the upper exremities that follows a "cape-like" distribution. As fluid cavity expands, progresses to involve muscle atrophy and weakness with decreased muscle tone and impaired reflexes, ptosis, miosis, and anhidrosis
Syringomyelia
967
What is the dx?
Type I diabetes; Accumulation of sorbitol in the lens (derived from glucose); Subsequent imbibition of water and enlargement of lens
Snowflake Cataract
associated with diabetic retinopathy
968
When dermatomyositis is seen in middle-aged men, it is often associated with what other disease?
Increased risk for epithelial cancer, most commonly of the lung
969
Name the diagnosis: Known autoimmune disease (ie Sjogren's); immune complex deposition in dermal venules causing a purpuric skin rash
Hypersensitivity vasculitis
970
Dx:
Cirrhosis
Secondary DM
Bronze skin
Hemochromatosis
971
The t(14;18) translocation, which is characteristic of \_\_\_\_\_\_\_, causes overexpression of the BCL2 gene by juxtaposing it with the IgH locus; the cells are resistant to apoptosis.
Follicular lymphoma follicular lymphomas are indolent tumors that continue to accumulate cells for 7 to 9 years. IgH and bcl2 genes
972
When is fibrinous pericarditis seen?
2-10 weeks after a transmural MI
973
JAK2 mutations are found in what cancers?
polycythemia vera and other myeloproliferative diseases
974
What are the amyloid proteins and their associations?
AA
AF
AL
ACal
The following are the mnemonics for amyloid proteins:
AA (Acute-phase reactant)
AF (Familial and old Fogies)
AL (Light chain, Multiple myeloma and Lymphoma)
A Cal (Calcitonin)
975
What serious associated medical condition accompanies friedrich ataxia?
hypertrophic cardiomyopathy
976
Epidural hematoma is blood where?
BEWTEEN dura and skull; trauma; fracture of temporal bone --\> rupture of middle meningeal artery; Herniation is a lethal complication
977
\_\_\_\_\_\_\_\_\_ is the most common cause of sudden unexplained death in young athletes.
Hypertrophic cardiomyopathy is the most common cause of sudden unexplained death in young athletes.
978
Autosomal recessive degenerative disorder of the spinal cord and cerebellum that presents in early childhood and leads to loss of vibratory sense and proprioception, muscle weakness in the lower extremities, loss of deep tendon reflexes and ataxia
Friedreich's ataxia
979
What is the complication of BPH untreated?
Hydroureter and hydronephrosis Can lead to renal failure
980
JC virus infection of oligodendrocytes is called:
PML - Progressive Multifocal Leukoencephalopathy can happen as the result of immunosuppression; rapidly progressive neurologic signs and death
981
A 71-year-old woman has had a 10-kg weight loss accompanied by severe nausea and vomiting of blood for the past 8 months. On physical examination, she is afebrile. Laboratory studies show hemoglobin, 8.4 g/dL; platelet count, 227,100/mm3; and WBC count, 6180/mm3. Biopsy specimens obtained by upper gastrointestinal endoscopy show adenocarcinoma of the stomach. CT scan of the abdomen shows multiple hepatic masses. CT scan of the head shows a cystic area in the right frontal lobe. Her condition is stable until 2 weeks later, when she develops severe dyspnea. A chest CT scan shows areas of decreased pulmonary arterial attenuation. What cardiac lesion is most likely to be present in this woman?
Nonbacterial thrombotic endocarditis So-called marantic vegetations may occur on any cardiac valve, but tend to be small and do not damage the valves. They have a tendency to embolize, however. They can occur with hypercoagulable states that accompany certain malignancies, especially mucin-secreting adenocarcinomas.
982
What is the diagnosis? Malignant tumor of nasopharyngeal epithelium; Association with EBV; Classicallt seen in African children and Chinese adults; Pleomorphic keratin + epithelial cells in a background of lymphcytes
Nasopharyngeal carcinoma
983
Gastrinoma of the endocrine pancreas is aka:
Zollinger-Ellison syndrome
984
Name the dx: Congenital anomaly in which the urethra opens on the underside of the penis so that the meatus is proximal to its normal glandular location
Hypospadias
Incomplete closure of the urethral folds of the urogenital sinus
985
Genetic mutation associated with Celiac disease:
HLA DQ2 and DQ8
986
What is the likely offending organism producing these symptoms: Immunocomprimised individual (ie chemo); Halo sign: chest CT scan shows multiple 1- to 4-cm nodular densities w/ surrounding areas of ground-glass infiltrate; branching septate hyphae found in sputum from broncheolar lavage
Aspergillus fumigatus Immunocompromised patients with neutropenia may develop invasive aspergillosis. Other patterns include allergic bronchopulmonary aspergillosis in persons with asthma and an aspergilloma, or fungus ball, colonizing a cavitary lesion of tuberculosis or bronchiectasis.
987
What is the diagnosis? Sinus pressure; Firm lesion in nasal wall; Epithelial nests protruding into the submucosa; no cellular atypia
Sinonasal inverted papilloma HPV 6 and 11
988
Anticardiolipin antibody often leads to a false-positive serologic test result for:
syphilis (Treponema pallidum infection) Antiphospholipid syndrome
989
Name the diagnosis corresponding to this autoantibody:
Anticentromere
CREST Syndrome (limited scleroderma)
990
Name the diagnosis: Oral leukoplakia and erythroplakia are precursor lesions; Floor of mouth is common location;
Squamous cell carcinoma - malignant neoplasm of squamous cells lining oral mucosa
991
Wiskott-Aldrich syndrome
Wiskott-Aldrich syndrome is an X-linked recessive congenital disorder characterized by **thrombocytopenia, frequent infections, and eczema (“TIE”).**
992
Osteomyelitis
infection can be in children bacterial like staph lytic focus = sequestrum sclerosis around it = involucrum bone pain and fever etc.
993
When melanoma is seen in the eye, it most commonly arises from what structure?
The uvea
994
the most common subtype of Hodgkin lymphoma is:
nodular sclerosis
995
Leiomyoma
uterine fibroid whirled masses, come in sets usually without encapsulation benign can cause bleeding (--\> anemia)
996
Coxsackievirus B and Trypanosoma cruzi are causes of what kind of cardiac complication?
Coxsackievirus B and Trypanosoma cruzi are causes of myocarditis.
997
What is an atrial myxoma?
An atrial myxoma is the most common primary cardiac neoplasm, but it is rare and is not related to ischemic heart disease.
998
What is the diagnosis? Malignant tumor derived from granular cells of the cerebellum; Usually seen in children; Small round blue cells on histology; Homer-Wright rosettes may be present; Poor prognosis
Medulloblastoma Metastasis to the cauda equina = "drop metastasis"
999
Dysgerminoma
tumor of large cells with clear cytoplasm and central nucleii malignant germ cell tumor LDH may be elevated female counterpart to testicular seminoma in males glycogen-filled cells with septa containing lymphocytes
1000
Small cell carcinoma of the lung is often associated with what paraneoplastic syndrome?
SIADH
1001
Erythematous maculopapular rash pinpoint clear vesicles on the skin newborns
Miliaria
1002
Name the disorder characterized by: 1. acquired antibody against a coagulation factor resulting in impaired factor, ie factor VIII 2. Clinical findings similar to Hemophilia A 3. PTT does not correct when mixed with normal plasma
Coagulation Factor Inhibitor
1003
What is the diagnosis? Degeneration of GABAergic neurons in the caudate nucleus of the basal ganglia
Huntington Disease
1004
What is the most common vasculature to be invaded by a malignancy for hematogenous spread?
Veins and lymphatics If in the head, however, veins bc there are no lymphatics in the cranium.
1005
The direct antiglobulin (Coombs) test detects:
antibody bound to RBCs
1006
Most susceptible brain regions (and cells) to damage from ischemia
perkinje neurons of cerebellum and pryimidal neurons of Sommner's sector in hippocampus
1007
What is the diagnosis? Accumulation of ganglioside GM1 in the CNS neurons; Progressive muscle weakness; Blindness; Death in early childhoo
Tay-Sachs AR disorder Inborn deficiency of hexaminidase A
1008
A lens-shaped lesion on head CT suggests?
Epidural hematoma
1009
Name the disorder characterized by: 1. Genetic Factor VIII deficiency (X-linked recessive, predominantly affects males) 2. Postsurgical, deep tissue and joint bleeding 3. Labs: Increased PTT normal PT Low factor VIII Normal platelet count Normal bleeding time 4. Treat with recombinant factor VIII
Hemophilia A \*PTT does correct when mixed with normal plasma in Hemophilia A
1010
Symmetrically smaller than normal kidneys can be caused by:
diabetes mellitus; systemic hypertension (hyaline arteriosclerosis) Hyaline arteriolosclerosis is seen with long-standing essential hypertension of moderate severity. These lesions give rise to benign nephrosclerosis. The affected kidneys become symmetrically shrunken and granular because of progressive loss of renal parenchyma and consequent fine scarring.
1011
A 45-year-old man receives a cardiac allograft for dilated cardiomyopathy. He has no problems with rejection, but 5 years later he has worsening exercise tolerance with increasing dyspnea and peripheral edema. Echocardiography shows a reduced ejection fraction of 35%. What pathologic abnormalities has he most likely developed in the allograft?
Coronary arteriopathy Nearly every allograft develops some degree of arteriopathy within 10 years, and half of patients have significant arteriopathy by 5 years following transplantation. Unlike atherosclerosis, the smaller coronary artery branches are preferentially affected, but the result is the same: ischemic damage.
1012
A chest CT scan shows a 10-cm mass in the anterior mediastinum; biopsy shows effacement by lymphoid cells with lobulated nuclei having delicate, finely stippled, nuclear chromatin; scant cytoplasm, many mitotic figures are seen; cells express deoxynucleotidyl transferase negative (TdT−), CD2, and CD7 antigens; point mutation in the NOTCH1 gene; seen in children
lymphoblastic lymphoma mediastinal location typical; chemotherapy is curative in most cases
1013
CAH ie 21-hydroxylase deficiency may result in impaired synthesis of what hormone?
Aldosterone --\> salt-wasting CAH --\> hypotension and dehydration and hyponatremia/hyperkalemia, increased renin secretion
1014
An autoimmune disease, most likely systemic lupus erythematosus (SLE), can be accompanied by what cell proliferation?
monocytosis. Cytopenias also can occur in SLE because of autoantibodies against blood elements, a form of type II hypersensitivity.
1015
Large cell carcinoma is usually well-defined and appears as:
large and pleomorphic and form sheets tends to be peripheral
1016
What is Mockeberg arteriosclerosis?
a benign process that is a form of arteriosclerosis, often with no serious sequelae. The distal extremities, pelvis, thyroid, and breast regions are the most common locations.
1017
Endometrial sinus tumor
malignant tumor that mimics the yolk sac
most common germ cell tumor in children
AFP elevated
**Schiller-Duval bodies** (like glomeruli) histology
1018
Chronic Endometritis
lymphocytes and plasma cells
IUD, TB, chronic PID, retained products of conception
abnormal bleeding, pain, infertility
1019
Dx: High serum bilirubin, unconjugated; Mild jaundice Symptoms resolve with corticosteroids
Autoimmune hemolytic anemia Antibody-mediated erythrocyte destruction. Intravascular hemolysis leads to increased serum levels of unconjugated bilirubin which exceed the capacity of hepatocytes to conjugate it.
1020
What is the diagnosis? Most common benign tumor in the oral cavity; Exophytic tumor with a fibrovascular core May occur on the tongue, gingiva, palate, or lips
Squamous papilloma
1021
CSF findings from bacterial meningitis?
neutrophils with decreased CSF glucose
1022
What is the effect of the following teratogen?
Warfarin
Fetal bleeding
1023
What is erythroplakia?
Vascularized leukoplakia Red plaque
1024
What is the diagnosis? An infection by two symbiotic organisms, one a fusiform bacillus and one a spirochete; Inadequate nutrition or immunodeficiency precipitate the invasion by this normal flora; Punched out erosions of the interdental papillae; All gingival margins can become covered by a pseudomembrane
Vincent angina aka Acute Necrotizing Ulcerative Gingivitis
1025
red Forcheimer spots on soft and hard palate
Rubella
1026
Hereditary hemochromatosis may result in what cardiac pathology?
Hereditary hemochromatosis may result in an infiltrative (dilated) cardiomyopathy with iron overload, more typically by the fifth decade.
1027
Name the diagnosis: Vasculitis, with IgA1-dominant immune deposits, affecting small vessels (predominantly capillaries, venules, or arterioles). Often involves skin and gastrointestinal tract, and frequently causes arthritis. Glomerulonephritis indistinguishable from IgA nephropathy may occur.
IgA vasculitis (Henoch-Scho ̈nlein) (IgAV)
1028
What is the diagnosis? Inherited degeneration of the anterior motor horn; Autosomal recessive; Presents as "floppy baby;" Death occurs within a few years of birth
Werdnig-Hoffman Disease
1029
The most common site for intracerebral hemorrhage is ehre?
Basal ganglia Presents as severe HA, nausea, vomiting and eventual coma
1030
What is the diagnosis? One pupil blown; Follows trauma; Death; Duret hemorrhages in the midbrain
Transtentorial herniation
1031
Iron deficiency does not occur in hemolytic anemias because:
the iron that is released from hemolyzed cells is reused
1032
Dx: Acquired malformation of mucosal and submucosal capillary beds; Usually arise in the cecum and right colon (high wall tension); Hematochezia in older adults
Angiodysplasia
1033
Krabbe disease is associated with what histological findings?
White matter disease, associated with perivascular accumulation of mononuclear and and multinucleated "globoid cells" containing undigested galactosylcerimide
1034
How does nasopharyngeal carcinoma often present?
Involvement of cervical lymph nodes
1035
microthrombocytopenia; propensity to develop non-Hodgkin's lymphoma; X-linked; many types of white blood cells, which are part of the immune system, to be abnormal or nonfunctional, leading to an increased risk of several immune and inflammatory disorders; eczema
Wiscott-Aldrich syndrome
1036
Name the disorder characterized by: 1. DVTs at a young age 2. Excessive or defective procoagulant proteins
Hypercoagulable state
1037
What is the action of Reed-Sternberg cells, for example in Hodgkin lymphoma?
Reed-Sternberg cells elaborate cytokines that promote an accompanying reactive cellular proliferation that forms the bulk of a neoplastic mass. Reed Sternberg cells are of B-cell origin, and in many cases, Epstein-Barr virus (EBV) infection can be demonstrated in these cells.
1038
Extramammary Paget Disease
indicates carcinoma in situ
burning sensation
malignant epithelial cells in epidermis
erythamatous, pruritic, ulcerated vulvar skin
**PAS +, keratin -, S100-, CEA+**
1039
What is the diagnosis? Begign neoplasm of the parotid gland; Cystic, granular spaces embedded in dense lymphoid tissue; Most common monomorphic adenoma; Most arise after age 50, more common in men than women
Warthin tumor
1040
Most lymphomas in patients who have AIDS are:
high grade B-cell lymphomas large grade B-cell lymhomas is Burkitts bcl2 gene usually EBV+
1041
High stress in R colon --\> High stress in L colon --\>
High stress in R colon --\> angiodysplasia High stress in L colon --\> diverticulitis Both result in hematochezia
1042
Lymph nodes draining from a cancer often show a reactive pattern, with dilated sinusoids that have endothelial hypertrophy and are filled with histiocytes (i.e., macrophages). Sinus histiocytosis represents:
an immunologic response to cancer antigens. Thus not all enlarged nodes are caused by metastatic disease in cancer patients.
1043
Eventually, glomeruli in the diabetic kidney exhibit a unique lesion called:
Kimmelstiel-Wilson disease or nodular glomerulosclerosis
1044
What cells are seen in the inflammatory infiltrate of rhinitis?
Eosinophils
1045
Dermatomyositis
heliotrope rash (upper eyelids) anti-Jo antibody, ANA+ inflammatory, involves skin and skeletal muscle
1046
Hep D what kind of virus?
ss RNA (defective RNA virus) expresses core antigen named HDAg (delta antigen) co-infection OR superinfection with Hep B
1047
Dx: Mild DM syndrome; Foul-smelling stool; Steatorrhea; Gallstones; Hypochlorhydia
Delta cell tumor - pancreas
1048
What is the diagnosis? Muscle pain after prolonged exercise; Possible myoglobinuria (dark color)
Carnitine Palmityl Transferase Deficiency
1049
T/F: Duodenal ulcers are almost never malignant.
True
1050
What is commonly seen on CXR of a patient with asbestos?
Diaphragmatic pleural calcified plaques The inhaled asbestos fibers become encrusted with iron and appear as the characteristic ferruginous bodies with iron stain. The firm, tan mass encasing the pleura is most likely a malignant mesothelioma. Asbestosis more commonly gives rise to pleural fibrosis and interstitial lung disease, similar to other pneumoconioses. This is seen grossly as a dense pleural plaque, which often is calcified. Asbestosis can give rise to bronchogenic carcinoma, especially in smokers.
1051
Name the dx: Finding in adults with fragile X syndrome
Macroorchidism
1052
Hep C what kind of virus?
RNA virus enveloped 6 main genotypes
1053
The circulating atypical lymphocytes seen in smears with patients with EBV are:
Activated T cells
1054
Defective GFAP Rosenthal fibers
Alexanders disease
1055
Hypersensitivity reaction with lymphocytic infiltrates at the dermal-epidermal junction; results in hyperkeratosis; multiple, flat-topped, violaceous, polygonal papules
Lichen planus
1056
Cherry-red spot describes a bright central foveola that occurs in the setting of what diseases?
Lysosomal storage diseases; Retinal artery occlusion
1057
Does dilated cardiomyopathy feature hypertension as a symptom?
No
1058
Where does the pain from acute cholecystitis radiate to?
R scapula
1059
most common cause of decline in mental status following TBI - produces "spheroids", parasagital white matter damage accompanied by multiple infarcts - due to sheer forces from anchoring to arachnoid villi
diffuse axonal injury
1060
What is the prognosis for small cell lung carcinoma?
Small cell carcinomas are aggressive neuroendocrine tumors that tend to metastasize early. Even when they appear to be small and localized, they are not or will not remain so. Surgery is not an option for these patients. They are treated as if they have systemic disease; some chemotherapy protocols afford benefit for 1 year or more, but cure is uncommon.
1061
Rhabdomyoma
seen in tuberous sclerosis
benign tumor of skeletal muscle
1062
Where does gastric cancer commonly spread?
Left supraclavicular node (Virchow node)
1063
Rheumatoid Arthritis
HLA-DR4 pannus Baker cyst, vasculitis, pleural effusions morning stiffness that improves with movement synovitis progressive IgM autoantibody against Fc portion of IgG (rheumatoid factor)
1064
What is the effect of the following teratogen?
Cigarette smoke
Intrauterine growth retardation
1065
Name the dx: Inflammatory disorder; uncommon; Accumulation of macrophages; Often associated with UTIs caused by E. Coli; Soft yellow plaques on the mucosal surface of the bladder
Malakoplakia
1066
Primary biliary cirrhosis features what positive antibody test?
Anti-mitochondrial antibody
1067
What is the most common genetic mutation associated with Primary Hemochromatosis?
HFE gene - C282Y
1068
What is the diagnosis? Baby born with gross deformity of lower back; Subcutaneous lesion has disorganized neural tissue and entrapment of nerve roots;
Meningomylocele Neural tube defect; Exposes the spinal canal; Causes entrapment of nerve roots
1069
Caruncle
postmenopausal women near urethra ulcerated, bleeding
1070
Destruction of respiratory bronchioles is a feature of \_\_\_\_\_\_\_\_\_.
Destruction of respiratory bronchioles is a feature of centrilobular emphysema.
1071
Tinea versicolor is a common condition caused by a superficial fungal infection of:
Malassezia furfur The lesions can be lighter or darker than the surrounding skin.
1072
Name the diagnosis: Long-standing suprapubic pain; Frequency and urgency; Can be with or withoug hematuria
Chronic interstitial cystitis
1073
Malignancy of the oral cavity typically metastasizes to what lymph node?
Tonsillar node (superior jugular node)
1074
White papules - superficial epidermal inclusion cysts newborns
Milia
1075
Which type of glaucoma is considered an ocular emergency?
Low tension glaucoma; Must start hypotensive tx within 24-48 hours if vision is to be maintained
1076
Name the diagnosis: Vasculitis accompanied by urticaria and hypocomplementemia affecting small vessels (i.e., capillaries, venules, or arterioles), and associated with anti-C1q antibodies. Glomerulonephritis, arthritis, obstructive pulmonary disease, and ocular inflammation are common.
Hypocomplementemic urticarial vasculitis (HUV) (anti-C1q vasculitis)
1077
Embryonal Rhabdomyosarcoma (Sarcoma Botryoides)
Bleeding Grape like mass protruding from vagina children
1078
Myelodysplasia is characterized by:
a cellular marrow in which there are maturation defects in multiple lineages;
presence of ringed sideroblasts, megaloblasts, abnormal megakaryocytes, and myeloblasts in the marrow.
Because these abnormal hematopoietic cells fail to mature normally, they are not released into the peripheral blood, leading to pancytopenia and susceptibility to infections.
Myelodysplasias are clonal stem cell disorders that develop either de novo or after chemotherapy with alkylating agents
1079
WT-1 tumor suppressor protein
loss of fx associated with Wilm's tumor
1080
Name the dx: Born 46, XY; External genital organs appear female or with signs of virilization
Testicular feminization syndrome aka male pseudohermaphroditism
1081
How do children and teenagers get meningitis from n minigititis?
enters through nasopharynx and then gets to blood then from blood gets to the meninges
1082
Name the dx: C-cell thyroid cancer; C-cells secrete calcitonin which lowers serum calcium; Polygonal, granular cells separated by distinct vascular stroma; Stromal amyloid is a distinct feature of this cancer (deposition of procalcitonin); Patients suffer multiple endocrine paraneoplastic syndromes including carcinoid syndrome and Cushing syndrome; Water diarrhea common
Medullary thyroid carcinoma (MTC) Can occur in patients with MEN-2
1083
What is the cause of death with a saddle embolus?
Sudden death occurs from hypoxemia or from acute cor pulmonale with right-sided heart failure.
1084
Another term for scleroderma is:
Progressive systemis sclerosis
1085
Syringomelia
Cavitation of spinal cord (usually cervical)
**Bilateral loss of pain and temperature at the level of the lesion** from damage to the spinothalamic tracts
Associated with **Chiari malformation**
Syringomyelia may result from the obstruction of CSF circulation from the posterior fossa.
Trauma (e.g. whiplash) and prior meningitis can also cause syringomyelia.
1086
What is the diagnosis? Massively dilated 4th ventricle with an absent cerebellum; Often accompanied by hydrocephalus
Dandy-Walker Malformation Congenital failure of the cerebellar vermis to develop
1087
Duodenal Atresia is commonly associated with:
Down Syndrome
1088
What is a Sister Mary Joseph nodule?
Distant metastasis of intestinal type of gastric carcinoma at the periumbilical region
1089
Prothrombin time (PT) measures:
extrinsic factor (factor VII) and common pathways (factors II, V, X and fibrinogen) of the coagulation cascade
1090
Name the dx:
umbilical hernia at birth;
mental insufficiency secondary to congenital hypothyroidism;
Can be due to lack of iodination of salt OR agenesis of the thyroid;
Sluggishness
Low body temperature;
Anemia
Cretinism
1091
Petechiae; Low platelets; bone marrow shows increased number of megakaryocytes; spontaneous recovery usually
Idiopathic thrombocytopenic purpura (ITP); acute ITP in children usually arises after a viral illness;
1092
What is the diagnosis? Triad of asthma, aspirin-induced bronchospasms, nasal polyps
Aspirin-intolerant asthma
1093
Dx: Fulminant liver failure and encephalopathy; Children; Previous viral infection treated with aspirin
Reye Syndrome
1094
What is the diagnosis? Recurrent vesicular lesions on the lips, reactivated by stress, sunlight, menses
Herpes labialis HSV-1 (usually)
1095
Postductal coarctation of the aorta
Postductal coarctation occurs distal to the ductus arteriosus (DA), which usually closes and obliterates. This results in the intercostal arteries providing reverse collateral flow between the internal thoracic artery and the thoracic aorta to provide blood supply to the lower parts of the body
**These patients will be hypertensive in the upper body and hypotensive with weak pulses in the lower limbs.**
Enlargement of the intercostal arteries results in **rib notching** on the lower border of the ribs.
Postductal coarctation can present early with tachypnea and hypertension.
The hypertension seen is a result of decreased blood flow to the kidneys, which in turn will activate the renin-angiotensin-aldosterone system.
1096
T/F: Nephritogenic strains of Strep pyogenes pose no risk for acute rheumatic fever
True
1097
What is the histologic hallmark of meningiomas?
Whorled pattern of "meningothelial" cells Meningiomas grow very slowly
1098
P. vivax
Plasmodium vivax and P. ovale are both capable of causing relapses following recovery from the primary infection because both organisms leave dormant forms (hypnozoites) in the liver.
**Schüffner dots** are only found in RBCs infected by Plasmodium vivax or P. ovale
1099
Hyditaform Mole - Partial Mole
normal ovum, 2 sperm 69 chromosomes fetal tissue present snowstorm on ultrasound, fetal HR neg bHCG much higher than expected
1100
What is the diagnosis? Nodule that arises on the true vocal cord; Often due to excessive use of vocal cords, usually bilateral
Singer's nodule aka Vocal Cord Nodule
1101
What are some causes of type III crescentic GN?
Type III - absense of anti-GBM antibodies or immune complexes
Causes of type III crescentic GN include granulomatosis with polyangiits (ANCA-associated vasculitis) and microscopic polyangiitis.
1102
Thrombotic microangiopathies are most likely to be damaged with:
endothelial cells
1103
Direct erythrocyte trauma due to an abnormal vascular surface ie graft or artificial valve; reticulocytes and schistocytes in peripheral blood smear Anemia is mild to moderate
Macroangiopathic (large vessels) hemolytic anemia
1104
Pustules and papules over erythematous base
Acne rosacea
1105
Cystic Teratoma
fetal tissue most common germ cell tumor\*\* often bilateral struma ovarii if composed of thyroid tissue
1106
Embryonal Carcinoma
malignant, large primitive cells aggressive with early metastases
1107
What is the most common cause of Ischemic colitis?
Atherosclerosis of the SMA --\> ischemic damage to the colon, usually at the splenic flexure
1108
most common cause of laryngotracheobronchitis in children
parainfluenza virus ==\> causes a type of laryngotracheobronchitis called "Croup"
1109
sign of Leser-Trelat
sudden appearance of numerous seborrheic keratosis; sign of internal malignancy ie gastric adenocarcinoma
1110
easily scraped away white deposit on tongue seen in immunocompromised states
Oral candidiasis aka thrush
1111
Gout
MSU crystals negative biferingence hyperuricemia podagra chronic gout --\> renal failure, tophi development
1112
What is the diagnosis? Painful, superficial ulceration of the oral mucosa Stress-related Grayish blue base surrounded by erythema
Aphthous ulcer
1113
Esophageal varices are most often seen in the setting of:
Portal HTN seen in lower third of esophagus
1114
Ectopic pregnancy
PID is risk for surgical emergency major bleed if bursts
1115
T/F: Hodgkin lymphomas have no consistent peripheral blood findings and are not likely to produce solitary lung nodules.
True Hodgkin lymphoma is not characterized by an increased WBC count.
1116
Give an example of a type I hypersensitivity rxn in the kidney:
Allergic nephritis; drug-induced acute interstitial nephritis
1117
Ingestion of strong bases/alkalines produces what kind of reaction?
Liquefactive necrosis with saponification of membrane lipids
1118
Name the dx: Polyploid inflammatory lesions near the urethral meatus that produce pain and bleeding; Occur exclusively in women;
Urethral caruncle
1119
Name the diagnosis corresponding to this autoantibody:
Antimicrosomal, antithyroglobulin
Hashimoto thyroiditis
1120
Name the disorder characterized by: 1. Pathologic activation of the coagulation cascade 2. Widespread ischemia and infarcts 3. Usually secondary to another disease process ie obstetric problems, sepsis etc. 4. Can be caused by a rattlesnake bite 5. Labs: Thrombocytopenia Increased PT/PTT Low fibrinogen microangiopathic hemolytic anemia elevated D-dimer 6. Treat by addressing underlying cause and transfusing blood products
Disseminated Intravascular Coagulation (DIC)
1121
A berry aneurysm is also known as a:
saccular aneurysm congenital most often found in the circle of Willis
1122
What lab values are commonly seen in alcoholic hepatitis?
AST \> ALT
1123
Is Hep A ever chronic?
No
1124
What is xerostomia? keratoconjunctivitis sicca? What disease are both of these found in?
dry mouth, dry eyes Sjogren syndrome
1125
A CXR of a patient with RSV would show:
Interstitial infiltrates
1126
\_\_\_\_\_\_\_\_\_\_ are the most frequent drugs implicated in drug-induced hemolytic anemias.
Cephalosporins
1127
Diffuse alveolar damage is an acute lung injury seen in:
Diffuse alveolar damage is an acute lung injury seen in acute respiratory distress syndrome.
1128
HIV infection can lead to a nephropathy that resembles FSGS, with what deposition?
IgM and C3 are deposited in the mesangial areas of affected glomeruli
1129
What is hemoglobin A1C?
A specific fraction of glycosylated hemoglobin in circulating RBCs
1130
Annular lesions with erythematous margins
tinea corporis
1131
Renal calculi is a complication of:
gout
1132
What is the most common inherited coagulation disorder?
Von Willebrand Disease
1133
Diabetes insipidus reflects a deficiency in what hormone?
ADH
1134
Nodular lesions on the face giving a patient leonine facies characterizes:
Lepromatous leprosy mycobacterium leprae no granulomas present negative lepromin test
1135
Fibromuscular dysplasia typically involves what arteries?
Fibromuscular dysplasia (FMD) typically involves renal and carotid arteries. Renal arterial stenosis activates the renin-angiotensin system, leading to hypertension. On angiography, FMD appears as a “string of beads” caused by focal medial hyperplasia with thickened fibromuscular ridges adjacent to less involved areas of the arterial wall. This is a surgically correctible cause for hypertension.
1136
Name the diagnosis: Eosinophil-rich and necrotizing granulomatous inflammation often involving the respiratory tract, and necrotizing vasculitis predominantly affecting small to medium vessels, and associated with asthma and eosinophilia. ANCA is more frequent when glomerulonephritis is present.
Eosinophilic granulomatosis with polyangiitis (Churg- Strauss) (EGPA)
1137
Raised and pigmented lesion with papillomatous appearing surface.
Intradermal nevus
1138
"opportunistic" virus that causes progressive multifocal leukoencephalopathy in immunocompromised states (e.g. AIDS or organ transplant).
JC virus
1139
Name the diagnosis: Necrotizing granulomatous inflammation usually involving the upper and lower respiratory tract, and necrotizing vasculitis affecting predominantly small to medium vessels (e.g., capillaries, venules, arterioles, arteries and veins). Necrotizing glomerulonephritis is common.
Granulomatosis with polyangiitis (Wegener’s) (GPA)
1140
Give the dx:
Uncommon but aggressive form of glomerulonephritis;
When combined with pulmonary hemorrhage, the dx is Goodpasture syndrome;
Autoimmune attack on GBM component of collagen type IV;
Diffuse linear staining of GBMs for IgG, which indicates autoantibodies bound to the BM;
Anti-GBM antibody glomerulonephritis
Over 90% have glomerular crescents (crescentic glomerulonephritis);
Presents as rapidly progressive renal failure with nephritis
1141
Full-thickness inflammation of the bowel wall is seen in:
Crohn's disease
1142
Durret hemorrhage
result of uncle transtentorial herniation
1143
Certain verocytotoxin-producing Escherichia coli strains can cause hemolytic uremic syndrome by:
Injury to the capillary endothelium
1144
Injury to what cells results in acute pancreatitis?
Acinar cells
1145
Sequestrum
necrotic bone
1146
increased numbers of circulating small, round, mature lymphocytes with scant cytoplasm in the peripheral blood smear; cells express the CD5 marker and the pan B-cell markers CD19 and CD20; Most patients have a disease course of 4 to 6 years before death; symptoms appear as the leukemic cells begin to fill the marrow (thrombocytopenia)
Chronic lymphocytic leukemia (CLL)
1147
Where is the most common site for Crohn disease?
Terminal ileum
1148
Acantholysis means:
loss of intercellular connections
1149
What are the most common risks for developing urothelial cell carinoma of the bladder?
Smoking; Exposure to azo dyes; Infection with Schistosoma haematobium; Cyclophosphamide; Use of certain analgesics; Radiation therapy
1150
Most common cause of post-natal unilateral deafness from viral infection?
Mumps
1151
Prenatal viral infection that causes bilateral deafness and permanant damage to the choclea and vestibular system
rubella
1152
The NOTCH1 gene encodes a transmembrane receptor required for:
T-cell development, and more than half of pre–T-cell tumors have activating point mutations.
1153
mass of accumulated keratin and squamous mucosa that can cause hearing loss and results from growth of squamous epithelium from the external ear through the ruptured ear drum into the middle ear
cholesteatoma
1154
Biopsy shows spindle-shaped cells and extravastated red cells. This indicates what neoplasm?
HHV8 - Kaposi's sarcoma nodules can occur anywhere, not just skin
1155
\_\_\_\_\_\_\_\_\_\_ is a form of self-limited infectious lymphadenitis that most often is seen in children, typically “downstream” of lymphatic drainage from the site of an injury on a distal extremity.
cat-scratch disease from Bartonella henselae infection \*stellate necrosis\*
1156
Hx of B-cell chronic lymphocytic leukemia; Hepatosplenomegaly; rapid onset of fever, abdominal pain, lymphadenopathy; Aggressive and refractory to therapy; High grade, large-cell lymphoma
Richter Syndrome
1157
What is the diagnosis? Antibodies against presynaptic Ca channels of the NMJ; Paraneoplastic syndrome, most often of a small cell carcinoma of the lung; Imparied ACh release; Proximal muscle weakness that improves with use
Lambert-Eaton Syndrome
Eyes are usually spared;
Acetylcholinesterase inhibitors do not improve symptoms;
Resolves with resection of cancer
1158
t(15;17) translocation seen in:
Acute promyelocytic leukemia (APL) it results in the fusion of the retinoic acid receptor gene on chromosome 17 with the promyelocytic leukemia gene on chromosome 15. The fusion gene results in elaboration of an abnormal retinoic acid receptor that blocks myeloid differentiation. Therapy with retinoic acid (vitamin A) can alleviate the block and induce remission in many patients.
1159
What differentiates ALS from Syringomyelia?
lack of sensory impairment in ALS
1160
Paget Disease of Bone
\>60 years imbalance of osteoclast and osteoblast function mosaic pattern (puzzle pieces) of lamellar bone bone pain hearing loss lion-like facies elevated alk phos complication can include high output cardiac failure due to formation of AV shunts in bone increased risk of osteogenic sarcoma more common in people of English descent
1161
Dx:
Late complication of acute pancreatitis;
Necrotic pancreatic tissue is liquefied through the action of pancreatic enzymes;
Tissue then becomes encapsulated by granulation tissue
Pancreatic pseudocyst
Persistent abdominal pain
Anorexia
Abdominal distention
1162
Osteogenesis Imperfecta
blue sclera hearing loss congenital structurally weak bone mutations in the gene for type I collagen (COL1A1 and COL1A2)
1163
Embolic stroke is due to:
Thromboemboli Most common source of emboli is the left side of the heart (ie a-fib); usually involves MCA; results in hemorrhagic infarct at the periphery of the cortex
1164
Protrusion of the meninges AND spinal cord is what form of spina bifida?
Meningomyelocele
1165
Numerous raised, pigmented lesions with a verrucoid surface.
Seborrheic keratosis
1166
\_\_\_\_\_\_\_\_\_ may cause myocarditis with mixed inflammatory cell infiltrates in immunocompromised patients.
Toxoplasma gondii may cause myocarditis with mixed inflammatory cell infiltrates in immunocompromised patients.
1167
Give the dx: Frequent cause of nephrotic syndrome in adults; Accumulation of immune complexes in the subepithelial zone of glomerular capillaries (IgG deposits on immunofluorescence);
Membranous glomerulopathy
1168
The most important histologic feature of mycosis fungoides (a variant of cutaneous T-cell lymphoma) is:
lymphocytes in the epidermis (epidermotropism) CD4+ marker for T-lymphocytes
1169
The most common metastasis for colorectal cancer is the:
liver
1170
A 15-year-old boy complains of pain in his legs when he runs more than 300 m. Physical examination shows temperature, 36.8° C; pulse, 76/min; respirations, 22/min; and blood pressure, 165/90 mm Hg. The radial pulses are 4+, and the dorsalis pedis pulses are 1+. Arterial blood gas measurement shows a normal oxygen saturation level. What congenital cardiovascular anomaly is most likely to be present in this patient?
Coarctation of the aorta In children and adults, the coarctation is typically postductal, and collateral branches from the proximal aorta supply the lower extremities, leading to the large pulse differential between upper and lower extremities. Collaterals often involve intercostal arteries whose enlargement produces “rib notching” on chest radiographs. Diminished renal blood flow below the coarctation increases renin production and promotes hypertension.
1171
Rule of two's with Meckel's Diverticulum
Meckel’s diverticulum can present at any age, but young children are more often symptomatic (think **two-year-olds**). Around 2-4% of patients present with complications. There are two main complications: **bleeding or obstruction**. The diverticula are around **two inches in length**, but vary in size, and usually located **within 2 feet of the ileocecal valve**. There are two main heterotopic tissue types (**gastric, pancreatic**). This combination of “twos” is called the “rule of twos.”
1172
Give the associated cancer(s) for this tumor marker:
beta-HCG
trophoblastic tumors;
choriocarcinoma;
testicular carcinoma
1173
Give the associated cancer(s) for this tumor marker:
CEA
Carcinoma of the lung, breast, pancreas, colon, stomach
1174
Give the associated cancer(s) for this tumor marker:
CA-125
Ovarian cancer
1175
Give the associated cancer(s) for this tumor marker:
CA 19-9
Pancreatic cancer
1176
Give the associated cancer(s) for this tumor marker:
Placental alkaline phosphatase
Seminoma
1177
Give the associated cancer(s) for this tumor marker:
Prostatic acid phosphatase
Prostate cancer
1178
Give the associated cancer(s) for this tumor marker:
S-100
Melanoma
Neural-derived tumors
Astrocytoma
1179
Give the associated cancer(s) for this tumor marker:
Tartrate-resistant acid phosphatatase (TRAP)
Hairy cell leukemia
