Saccharopinuria

Orpha code: 3124OMIM code: 268700

Definicja

A rare autosomal recessive disorder of the lysine catabolism characterized by elevated levels of lysine in the cerebrospinal fluid and blood with variable degrees of saccharopinuria. Some patients present with neurological symptoms such as seizures, spastic diplegia, mild psychomotor delay, intellectual deficit, and behavioral difficulties. However, current findings suggest no causal association between isolated hyperlysinemia and neurological symptoms.

Disease data
Klasyfikacja

Disease

Synonimy
Hyperlysinemia type II
Hiperlizynemia typu II
Niedobór dehydrogenazy sacharopiny
Saccharopine dehydrogenase deficiency
Kod ORPHA
3124
Kod OMIM
268700
Kod ICD10
E72.3
Kod ICD11
5C50.4

No additional description.

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