Fabry's disease: a multidisciplinary disorder.

Peters, F P; Sommer, A; Vermeulen, A; Cheriex, E C; Kho, T L · Postgrad Med J · 1997

case_report · Level V

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Abstract

Fabry's disease is an X-linked hereditary disorder resulting in accumulation of a glycolipid (galactosylgalactosyl glucosylceramide) due to deficiency of alpha-galactosidase A. The diagnosis can be made by histopathologic examination of skin biopsy, low activity of alpha-galactosidase in leucocytes and genetic examination. Treatment is symptomatic. We want to stress the multidisciplinary collaboration necessary to deal with this condition, in order to prevent acceleration of symptoms.

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