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198 9: Muscles and Joints<br />

76. (D) Deficient or defective 1,25(OH)2 vitamin D<br />

receptors will result in vitamin D resistance.<br />

Parathyroid hormone levels increase secondary<br />

to decreased vitamin D effect. The 1(OH) vitamin<br />

D is an intermediate metabolite. (Kasper, p. 2247)<br />

77. (C) Aging decreases the responsiveness of the<br />

renal 25(OH) D-1-hydroxylase to parathyroid<br />

hormone (PTH), thus decreasing circulatory<br />

levels of the active metabolite 1,25(OH)2 vitamin<br />

D. This results in decreased calcium<br />

absorption from the gut. There is not a close<br />

relationship of 1(OH) vitamin D levels and<br />

osteopenia. (Kasper, pp. 2246–2247)<br />

78. (B) Muscle weakness is common in both rickets<br />

and osteomalacia, and proximal leg muscles are<br />

particularly involved. The combination of leg<br />

deformity and muscle weakness in rickets can<br />

result in an inability to walk. The presentation<br />

of osteomalacia is more insidious in the elderly,<br />

but proximal myopathy may be severe enough<br />

to cause a waddling gait and mimic a primary<br />

muscle disorder. The other clinical findings<br />

listed are found only in rickets. (Kasper, p. 2247)<br />

79. (D) The term pseudogout refers to crystal deposition<br />

disease not due to uric acid (gout). By far<br />

the most common cause of pseudogout is calcium<br />

pyrophosphate deposition (CPPD). The<br />

major predisposing factors are advanced age<br />

and preexisting joint disease. The knee is the<br />

most common joint involved, and presentation<br />

can mimic acute gout. However, in the majority<br />

of cases, the deposition of calcium pyrophosphate<br />

seems to be asymptomatic. (Kasper, p. 2047)<br />

80. (E) Unfortunately, there is no medical means to<br />

remove the deposits of CPPD. Colchicine does<br />

seem to decrease the rate of recurrence.<br />

NSAIDs, steroids (systemic or intra-articular),<br />

and colchicine are all helpful in acute attacks.<br />

(Kasper, pp. 2048–2049)<br />

81. (C) Although gout and CPPD disease are found<br />

in chronic renal failure, hydroxyapatite deposition<br />

is characteristic of end-stage renal failure.<br />

The crystals are very small, nonbirefringent, and<br />

only seen on election microscopy. Treatment is<br />

symptomatic, and similar to the treatment of<br />

gout or CPPD disease. (Kasper, p. 2049)<br />

82. (E) “Milwaukee shoulder” represents an<br />

unusual manifestation of calcium hydroxyapatite<br />

deposition disease. Once destruction<br />

changes start occurring, medical management<br />

is relatively unsuccessful. The exact reason why<br />

destructive arthritis occurs is not understood.<br />

The knee is the other joint affected in this<br />

manner. (Kasper, p. 2048)<br />

83. (C) The high white cell count suggests infection.<br />

In RA and crystal-induced arthritis, the<br />

white cell count is usually less than 50,000/µL.<br />

Staphylococcus aureus infection is the most<br />

common type in RA. (Kasper, p. 2050)<br />

84. (B) Churg-Strauss is a granulomatous vasculitis.<br />

Pulmonary involvement often dominates<br />

the clinical presentation with severe asthma<br />

attacks and pulmonary infiltrates. Peripheral<br />

eosinophilia is present in virtually all cases.<br />

(Kasper, p. 2007)<br />

85. (A) PAN is a multisystem, necrotizing vasculitis<br />

of small- and medium-sized muscular arteries.<br />

Aneurysmal dilations of the arteries are<br />

characteristic. Nonspecific signs and symptoms<br />

are the usual method of presentation. Renal<br />

involvement is clinically present in 60% of cases<br />

and is the most common cause of death in<br />

untreated cases. (Kasper, pp. 2007–2008)<br />

86. (F) A high percentage of patients with<br />

Wegener’s develop ANCAs. In particular, cytoplasmic<br />

or c-ANCAs are both sensitive and<br />

specific for Wegener’s. However, tissue diagnosis<br />

is still required. (Kasper, pp. 2004–2006)<br />

87. (C) Henoch-Schönlein purpura, characterized<br />

by palpable purpura, arthralgias, GI symptoms,<br />

and glomerulonephritis, can be seen in<br />

any age-group but is most common in children.<br />

It can resolve and recur several times over a<br />

period of weeks or months and can resolve<br />

spontaneously. (Kasper, p. 2010)<br />

88. (I) Behçet’s syndrome is a leukocytoclastic<br />

venulitis characterized by episodes of oral<br />

ulcers, genital ulcers, iritis, and cutaneous<br />

lesions. Eye involvement can rapidly progress<br />

to blindness. (Kasper, pp. 2012, 2014)

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