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Первинна гіпероксалурія

ORPHA:416· ICD-10 E74.8· Primary hyperoxaluria

Визначення(English summary)

A disorder of glyoxylate metabolism characterized by an excess of oxalate resulting in kidney stones, nephrocalcinosis and ultimately renal failure and systemic oxalosis. There are 3 types of PH, types 1-3, all caused by liver-specific enzyme defects.

Поширеність
1-9 / 1 000 000
Успадкування
Autosomal recessive
Вік початку
All ages