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Гомоцистинурія без метилмалонової ацидурії

ORPHA:622· ICD-10 E72.1· Homocystinuria without methylmalonic aciduria

Визначення(English summary)

Homocystinuria without methylmalonic aciduria is an inborn error of vitamin B12 (cobalamin) metabolism characterized by megaloblastic anemia, encephalopathy and, sometimes, developmental delay, and associated with homocystinuria and hyperhomocysteinemia. There are three types of homocystinuria without methylmalonic aciduria; cblE, cblG and cblD-variant 1 (cblDv1).

Поширеність
<1 / 1 000 000
Успадкування
Autosomal recessive
Вік початку
All ages