JOSEPH A. CHAZAN, M.D., F.A.C.P.; MARY AMBLER, M.D.; ALBERT KALDERON, M.D.; JORDAN J. COHEN, M.D., F.A.C.P.; JESSICA ZACKS, M.D.
Two brothers with hereditary nephritis developed a severe, painful, proximal myopathy in association with the onset of the uremic syndrome. The myopathic symptoms were aggravated by exercise and relieved by rest and appeared to be ischemic in nature. Pathologic examination of the skeletal muscle showed an unusual deposition vasculopathy. These deposits were distributed throughout the blood vessel wall, significantly reducing the lumina of many of the vessels supplying skeletal muscle, and were therefore considered to be the cause of the ischemic myopathy. Histochemical studies indicated that these deposits were composed either of acid mucopolysaccharides and phospholipids or of glycolipids. Electron microscopic evaluation of the affected vessels demonstrated membranous bodies confirming the presence of a phospholipid. This vasculopathy may represent a previously undescribed extrarenal manifestation of this inherited disorder, or, alternatively, the vasculopathy may represent a previously unrecognized complication of the uremic syndrome per se.
CHAZAN JA, AMBLER M, KALDERON A, et al. Vascular Deposits Causing Ischemic Myopathy in Uremia: Two Brothers with Hereditary Nephritis. Ann Intern Med. 1970;73:73–79. doi: https://doi.org/10.7326/0003-4819-73-1-73
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Published: Ann Intern Med. 1970;73(1):73-79.
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