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Review / Wrong Answers > 6/21 UWorld > Flashcards

6/21 UWorld Flashcards

1
Q

Treatment and presentation of osteopetrosis

A
  • Treatment:
    • Bone marrow transplant (osteoclasts derived from monocytes)
  • Clinical features:
    • Fractures
    • Bone fills marrow space
      • Extramedullary hematopoiesis
      • Pancytopenia
      • “Bone-in-bone” x-ray
    • Vision and hearing loss
      • Impingement of cranial nerves
    • Hydrocephalus
      • Narrowing of foramen magnum
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2
Q

What are the lab values in rickets/osteomalacia (calcium, phosphate, PTH, ALP)

A
  • Decreased vitamin D
  • Decreased Ca2+
  • Increased PTH
  • Decreased phosphate
  • Increased alkaline phosphatase (hyperactivity of osteoblasts)
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3
Q

What disease presents with increasing hat size

A

Paget

  • Clinical features:
    • Mosaic pattern of woven and lamellar bone
    • Increased hat size due to skull thickening
    • Hearing loss due to impingement of cranial nerve
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4
Q

Complications of Pagets

A
  • High output cardiac failure – due to increased blood flow from formation of arteriovenous shunts in bone
  • Osteosarcoma – end-stage osteoblasts at risk for mutation
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5
Q

Normal function of FGFR3

A
  • Normally responsible for inhibition of cartilage proliferation
  • Achondroplasia = Constitutive activation of FGFR3 = inhibited chondrocyte proliferation
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6
Q

What is Polyostic fibrous dysplasia

A
  • Bone is replaced by collagen and fibroblasts
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7
Q

Classic presentation of McCne Albright syndrome

A

Cafe-au-lait spots (unilateral)

Polyostotic fibrous dysplasia (Bone is replaced by collagen and fibroblasts)

Precocious puberty

Multiple endocrine abnormalities

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8
Q

Heberden vs. Bouchard node

A

Heberden = DIP

Bouchard = PIP

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9
Q

What is pannus, and which arthritis is it associated with?

A

Pannus = inflamed granulation tissue in RA

RA is a systemic autoimmune disease that causes synovitis which leads to the creation of pannus (inflamed granulation tissue)

Recall that myofibroblasts within granulation tissue contract, which is what causes a lot of symptoms of RA

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10
Q

What is the MOA of colchicine

A

Colchicine – stabilizes tubulin to impair leukocyte chemotaxis

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11
Q

Why must Cidofovir and Probenicid be administered together

A

Cidofovir used to treat CMV

Is nephrotoxic so must be administerd with probenicid which will block tubular secretion of Cidofovir

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12
Q

What seronegative spondyloarthropathy is associated with “pencil-in-cup” x-ray?

A

Psoriatic arthritis

Deformity of the DIP

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13
Q

Arthritis associated with osteophytes (bone spurs)

A

OA

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14
Q

Disease associated with Anti-SSA (Anti-Ro) and Anti-SSB (Anti-La)

A

Sjogren

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15
Q

Most common cause of death in Duchenne

A

Dilated cardiomyopathy

  • Clinical features:
    • Weakness begins in pelvic girdle and progresses superiorly
    • Pseudohypertrophy of calf muscles due to fibrofatty replacement of muscle
    • Waddling gait
    • Gower sign
    • Onset before 5 y/o
    • Dilated cardiomyopathy is common cause of death
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16
Q

What other disorder is associated with polymyalgia rheumatica

A

Giant cell arteritis

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17
Q

Treatment of fibromyalgia

A

Exercise, antidepressants (SNRI, TCA), anticonvulsants (Gabapentin)

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18
Q

What junction allows signalling between adjacent cells, and what is the protein component?

A

Gap junctions

Made up of connexons

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19
Q

What is the pathophysiology behind freckles?

A

Increased number of melanosomes (melanocytes NOT increased)

20
Q

Drugs that cause drug induced lupus

A
  • SHIPP-E
    • S – sulfa drugs
    • H – Hydralazine
    • I – Isoniazid
    • P – Procainamide
    • P – Phenytoin
    • E – Etanercept
21
Q

What is the disease classically associated with heliotrope rash

A

Dermatomyositis - rash of uppr eyelids

22
Q

Describe histologic features of psoriasis

A

Acanthosis (epidermal hyperplasia)

Increase in stratum spinosum and decrease in stratum granulosum

Parakeratosis (hyperkeratosis with retention of keratinocyte nuclei in the stratum corneum)

Munro microabscesses (neutrophils within the stratum corneum)

Auspitz sign (pinpoint bleeding due to thinning of epidermis above elongated dermal papilla)

23
Q

Describe defect behind albinism

A

Congenital lack of pigment due to enzyme defect (e.g. tyrosinase) that impairs melanin production

Normal melanocyte number with decreased melanin production

Increased risk of skin cancer

24
Q

Describe defect behind vitiligo

A

Localized loss of skin pigment due to autoimmune destruction of melanocytes

25
Q

In what skin layer does epidermolysis occur in staph scalded skin syndrome

A

Results in epidermolysis of stratum granulosum

Leads to sloughing of skin (+Nikolsky sign)

26
Q

Describe presentation of Lichen planus

A
  • 6 P’s = pruritic, purple, polygonal, planar, papules and plaques
  • Wickham striae (reticular white lines) = mucosal involvement
    • Think of a witch (idk why Lichen makes me think of witches)
      • Witches wear purple and have a bunch of warts – 6 Ps
      • Wicken = Wickham striae
27
Q

Histology of Lichen planus

A
  • Inflammation of dermal-epidermal junction
  • Saw-tooth appearance
    • Witches torture people = sawtooth
28
Q

What epidermal layer is especially hyperplastic in acanthosis nigracans?

A

statrum spinosum (definition of acanthosis is increased stratum spinosum)

29
Q

What is pityriasis rosea

A

Initial lesion (“herald patch”) followed by scaly erythematous plaques in a “Christmas tree” distribution on trunk

Self-resolving in 6-8 weeks

30
Q

Mutation commonly associated with Melanoma

A

BRAF = remember Benjamin Franklin

31
Q

Histology of seborrheic keratosis

A

keratin pseudocysts

32
Q

Describe pathogenesis of CHF

A
  • Decrease cardiac output leads to:
    • Increased volue (stimulated by RAAS and ADH)
    • Vasoconstriction (stimulated by RAAS and sympathetic)
    • Increased HR/contractility (stimulated by sypathetic)
  • These all lead to increased hemodynamic stress on the heart, which further worsens CHF
33
Q

Effects of ANP and BNP

A
  • Causes vasodilation and decreased Na+ reabsorption at the renal collecting tubule
  • Dilates afferent arteriole and constricts efferent arterial, promoting diuresis
34
Q

What are the 3 substances that are chemotactic for neutrophils

A

Leukotriene B4, C5a, IL-8

35
Q

MOA, uses, and special characteristics of Buspirone

A
  • MOA:
    • 5-HT1A agonist
  • Uses:
    • Generalized anxiety disorder
  • Characteristics:
    • Slow onset of action (takes 1-2 weeks to take effect)
    • Does not cause sedation, addiction, or tolerance
    • Does not interact with alcohol (vs. barbs and benzos)
36
Q

MOA and uses of Nesiritide

A
  • Nesiritide = “turn the tide” elephant sign
    • Mechanism of action
      • Synthetic form of BNP, which increased cGMP in smooth muscles, leading to venous and arteriolar dilation (reducing afterload and preload)
        • = BuMP
        • = dilated red ears and blue legs
      • Also causes natriuresis
        • = elephant trunk spewing out salty peanut stream
    • Uses:
      • Acute heart failure
        • = acute issues sign
37
Q

Presentation and cause of asterixis

A
  • Extension of wrists causes “flapping” motion
  • Associated with hepatic encephalopathy, Wilson disease, and other metabolic derangements
38
Q

Presentaitino and causes of chorea

A
  • Sudden, jerky, purposeless movements
  • Causes:
    • Due to lesion of basal ganglia (e.g. Huntington’s)
    • Sydenham chorea seen in acute rheumatic fever
39
Q

What is the embryologic defect that causes tetralogy of fallor

A

Anterosuperior displacement of the infundibular septum

40
Q

Presentation following blood transfusion in a patient with selective IgA deficiency

A

Anaphylactic (IgE-mediated) reaction to foreign IgA

41
Q

What is it called when a patient has sustained, involuntary muscle contractions

A

Dystonia

  • Due to Writer’s cramp, blepharospasm (forced closure of eyelids), torticollis (cervical dystonia)
42
Q

Presentation of diseases of biliary tract

A
  • Jaundice, pruritis, dark urine, light colored (acholic) stools, increased alkaline phosphatase, increased direct bilirubin
43
Q

Cause and presentation of primary biliary cirrhosis

A
  • Autoimmune destruction of intra-hepatic bile ducts
    • Associated with other autoimmune conditions
  • Lymphocytic infiltrate + granulomas
  • Mostly occurs in women around age 40
  • Cirrhosis is a late complication
44
Q

Presentation of Primary sclerosing cholangitis

A
  • Unknown cause
  • Concentric fibrosis of the intra- and extra-hepatic bile ducts
  • Contrast imaging will show “beaded” appearance due to alternating fibrotic and dilated regions
  • Mostly occurs in men around age 40
  • Associated with (+) pANCA and Ulcerative colitis
  • Increased risk for cholangiocarcinoma
45
Q

What disease is associated with anti-mitochondrial antibodies

A

Primary biliary cholangitis

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