Unusual cardiac, renal and pulmonary involvement in Gaucher's disease. Interstitial glucocerebroside accumulation, pulmonary hypertension and fatal bone marrow embolization

Rober R.L. Smith, Grover M. Hutchins, George H. Sack, Ren L. Ridolfi

Research output: Contribution to journalArticlepeer-review

Abstract

A 25 year old black woman who had manifestations of Gaucher's disease since one year of age is described. This patient had clinically significant cardiac, renal and pulmonary involvement with Gaucher's disease. Interstitial infiltration of the myocardium by Gaucher cells caused decreased left ventricular compliance and decreased cardiac output. In the kidney, Gaucher cells were present in the mesangium of the glomeruli and the interstitium of the cortex. Also, electron dense, intramembranous granular deposits were seen in glomeruli on electron microscopy. The pulmonary findings included pulmonary arterial hypertension, accentuated basilar deposition of glucocerebroside in the interstitium of alveolar septums and fatal bone marrow embolization.

Original languageEnglish (US)
Pages (from-to)352-360
Number of pages9
JournalThe American journal of medicine
Volume65
Issue number2
DOIs
StatePublished - Aug 1978
Externally publishedYes

ASJC Scopus subject areas

  • Medicine(all)

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