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Step 2 CK > Syndromes > Flashcards

Syndromes Flashcards

1
Q

Name the three granulomatous vasculitides

A

Takayasu, Wegeners, and eosinophilic granulomatosis with polyangiitis (Churg-Strauss)

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2
Q

Beckwith-Wiedmann pathogenesis

A

Deregulation of imprinted gene expression in chromosome 11p15

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3
Q

Beckwith-Wiedmann presentation

A

Fetal macrosomia, rapid growth until late childhood, omphalocele or umbilical hernia, macroglossia, hemihyperplasia. Wilms tumor and hepatoblastoma are complications.

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4
Q

Reye syndrome pathogenesis

A

Peds aspirin with influenza or VZV

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5
Q

Reye syndrome presentation

A

Acute liver failure and encephalopathy

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6
Q

Serum sickness-like syndrome pathogenesis and presentation.

A

Antibiotic reaction; Fever, urticaria, and polyarthralgia 1-2wks after exposure

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7
Q

Wiskott-Aldrich pathogenesis

A

X-linked recessive, T cell dysfunction

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8
Q

Wiskott-Aldrich presentation

A

Early in infancy with eczema and bleeding due to thrombocytopenia and hypogammaglobulinemia. Bacterial, viral, and opportunistic infections.

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9
Q

CHARGE syndrome pathogenesis

A

Failure of the post nasal passage to canalize completely

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10
Q

CHARGE syndrome presentation

A

Colobama, Heart defects, Atresia choanae, Retardation of growth/development, Genito-urinary anomalies, and Ear abnormalities/deafness

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11
Q

HUS pathogenesis

A

Vascular damage and microthrombi

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12
Q

HUS presentation

A

Fatigue, pallor, schistocytes, thrombocytopenia, AKI

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13
Q

Osgood-Schlatters pathogenesis

A

Traction apophysitis

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14
Q

Osgood-Schlatters presentation

A

Adolescent males or females with knee pain and proximal tibial swelling

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15
Q

Kawasaki presentation

A

Fever >4d plus >3 of following: bilateral nonexudative conjunctivitis, mucositis, cervical lymphadenopathy, erythematous polymorphous rash, extremity changes

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16
Q

Kawasaki complications

A

Coronary artery aneurysms, MI and ischemia

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17
Q

Kawasaki tx

A

Aspirin & IVIG

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18
Q

Henoch-Schonlein purpura pathogenesis

A

IgA-mediated leukocytoclastic vasculitis

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19
Q

Henoch-Schonlein purpura presentation

A

Palpable purpura, arthritis/arthralgia, abdominal pain, intussusceptions, renal disease similar to IgA nephropathy

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20
Q

Henoch-Schonlein purpura lab findings

A

Normal platelet count and coag studies, normal to increased SCr, hmaturia +/- RBC casts +/- proteinuria, normal complement

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21
Q

Cryoglobulinemia syndrome pathogenesis & presentation

A

A manifestation of chronic HepC. Palpable purpura, arthralgias, GN, low complement.

22
Q

Sarcoidosis presentation

A

Hilar adenopathy, interstitial pulm infiltrates, ant/post uveitis, hepatosplenomegaly, acute polyarthritis, chronic arthritis, AV block, dilated or restrictive cardiomyopathy, Ccentral DI

23
Q

Felty syndrome presentation

A

RA + leukopenia + splenomegaly

24
Q

Riedel thyroiditis pathogenesis

A

Progressive fibrosis of thyroid and surrounding tissues.

25
Q

Bacillary angiomatosis

A

Bartonela henseliae; Vascular cutaneous lesions and systemic symptoms - diagnose with lesions biopsy

26
Q

Trousseau’s

A

Migratory superficial thrombophlebitis. Hypercoagulability. Look for occult malignancy!

27
Q

Conn’s syndrome

A

Primary hyperaldosteronism. HTN, mild hypernatremia, hypokalemia, and metabolic alkalosis.

28
Q

Cryoglobulinemia pathogenesis

A

Immune complex deposition in small to medium-sized vessesl, leading to endothelial injury and end-organ damage.

29
Q

Cryoglobulinemia presentation

A

Fatigue, palpable purpura, arthralgias, renal disease, and peripheral neuropathies

30
Q

Werdnig-Hoffman syndrome pathogenesis

A

Aut rec. Degeneration of anterior horns cells and cranial nerve motor nuclei. “Floppy baby” syndrome.

31
Q

Myotonic congenital myopathy

A

Aut dom. Presents with muscle weakness and atrophy (distal muscles), moyotonia, testicula atrophy, and baldness. Cataracts, conduction abnormalities.

32
Q

Wegener’s granulomatosis pathogenesis and presentation

A

Necrotizing pulm vasculitis. Fever, weight loss, rhinosinusitis

33
Q

Waldenstrom macroglobulinemia presentation

A

Hyperviscosity syndrome, neuroapathy, bleeding, hepatosplenomegaly

34
Q

Waldenstrom macroglobulinemia lab findings

A

sharp IgM spike, >10% clonal B cells on bone marow bx

35
Q

Myxedema coma (severe hypothyroidism) presentation

A

Decreased mental status, hypothermia, bradycardia,

36
Q

Does HCTZ increase or decrease urinary Ca excretion?

A

Decreases - used to treat recurrent Ca stones

37
Q

Does furosemide increase or decrease urinary Ca excretion?

A

Increases - increased risk of Ca nephrolithiasis

38
Q

What is Cushing’s reflex

A

HTN, bradycardia, and respiratory depression - indicates elevated ICP

39
Q

Presentation of DiGeorge

A

Congenital heart disease, facial dysmorphia, thymic hypoplasia, cleft palate, and hypocalcemia

40
Q

Presentation of polyarteritis nodosa

A

Systemic symptoms, skin findings (livedo reticularis, purpura), kidney dz, abd pain, and muscle aches or weakness; elevated CRP

41
Q

Presentation of Sturge-Weber

A

Focal/generalized seizures, mental retardation, port wine stain or nevus flammeus on trigeminal n.

42
Q

Presentation of Whipple’s disease

A

White middle-aged man with abd pain, diarrhea, malabsorption w/ distention flatulence and steatorrhea. Extraintestinal manifestations include polyarthropathy, chornic cough, and myocardial or valvular involvement leading to CHF or valvular regurgitation.

43
Q

Ludwig angina

A

Cellulitis of submandibular space - usu from dental infections

44
Q

Waterhouse-Friderichsen syndrome

A

Adrenal hemorrhage in meningococcemia. Sudden vasomotor collapse and skin rash.

45
Q

Presentation of leukocytoclastic vasculitis

A

dd

46
Q

Adult Still disease

A

Recurrent high fevers, arthritis/arthralgias, and salmon-colored macular or maculopapular rash

47
Q

Lynch syndrome

A

Colorectal, endometrial, ovarian

48
Q

FAP

A

Colorectal, desmoids and osteomas, brain tumors

49
Q

VHL

A

Hemangioblastomas, clear cell renal carcinoma, pheochromocytoma

50
Q

MEN type 1

A

Pituitary adenomas, parathyroid hyperplasia, pancreatic adenomas (diamond)

51
Q

MEN type 2A

A

Parathyroid hyperplasia, pheochromocytoma, medullary thyroid cancer (square)

52
Q

MEN type 2B

A

Medullary thyroid cancer (calcitonin), pheochromocytoma, mucosal neuromas/marfanoid habitus (triangle)

Step 2 CK (8 decks)

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