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Atypical Fabry's disease presenting with cholesterol crystal embolization.
- Source :
-
Internal medicine (Tokyo, Japan) [Intern Med] 2000 Aug; Vol. 39 (8), pp. 646-9. - Publication Year :
- 2000
-
Abstract
- We describe a 65-year-old man who presented with pulmonary hemorrhage and progressive renal insufficiency three months after resection surgery for an abdominal aortic aneurysm. Intensive treatment with corticosteroids and hemodialysis were not effective, and the patient died. Postmortem examination of the kidneys revealed widespread cholesterol clefts within the renal arterioles and a number of lamellar inclusion bodies were observed by electron microscopy. The diagnosis of Fabry's disease was made by the absence of plasma alpha-galactosidase A activity. This was a very rare case of subclinical Fabry's disease coexistent with cholesterol crystal embolization, mimicking pulmonary-renal syndrome.
- Subjects :
- Aged
Crystallization
Diagnosis, Differential
Hemorrhage complications
Humans
Kidney pathology
Kidney Failure, Chronic complications
Kidney Failure, Chronic pathology
Male
Cholesterol metabolism
Embolism, Fat complications
Embolism, Fat diagnosis
Fabry Disease complications
Fabry Disease diagnosis
Subjects
Details
- Language :
- English
- ISSN :
- 0918-2918
- Volume :
- 39
- Issue :
- 8
- Database :
- MEDLINE
- Journal :
- Internal medicine (Tokyo, Japan)
- Publication Type :
- Academic Journal
- Accession number :
- 10939539
- Full Text :
- https://doi.org/10.2169/internalmedicine.39.646