Internal-Medicine
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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)