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Хвороба Танжера

ORPHA:31150· ICD-10 E78.6· Tangier disease

Визначення(English summary)

A rare, genetic neurometabolic disease characterized biochemically by an almost complete absence of plasma high-density lipoproteins (HDL), and clinically by liver, spleen, lymph node and tonsil enlargement along with multifocal peripheral neuropathy, corneal, skin and nail and, occasionally, cardiovascular disease.

Поширеність
<1 / 1 000 000
Успадкування
Autosomal recessive
Вік початку
Adolescent, Adult, Childhood, Infancy, Neonatal