FABRI DISEASE

From Big Medical Encyclopedia

FABRI the DISEASE (J. Fabry, is mute. dermatologist, 1860 — 1930; synonym: An -

a giokeratoma of a trunk diffusion, a lipoidosis of distopicheskiya hereditary, Fabri an angiokeratoma, Fabri a syndrome) — the hereditary disease caused by disturbance of exchange of glycolipids, the reason to-rogo is genetic defect of enzyme of ceramide-ss-galactoside-zy A; it is characterized by damage of skin of a trunk in the form of multiple high-quality vascular educations like angiomas, and also kidneys, eyes, intestines, a myocardium and other bodies — see Glikozidoza (t. 10, additional materials), Ruitera — Pompena a symptom complex.

Яндекс.Метрика