作者: Rober R.L. Smith , Grover M. Hutchins , George H. Sack , Ren L. Ridolfi
DOI: 10.1016/0002-9343(78)90832-X
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摘要: A 25 year old black woman who had manifestations of Gaucher's disease since one age is described. This patient clinically significant cardiac, renal and pulmonary involvement with disease. Interstitial infiltration the myocardium by Gaucher cells caused decreased left ventricular compliance cardiac output. In kidney, were present in mesangium glomeruli interstitium cortex. Also, electron dense, intramembranous granular deposits seen on microscopy. The findings included arterial hypertension, accentuated basilar deposition glucocerebroside alveolar septums fatal bone marrow embolization.