Carnosinase deficiency

Orpha code: 1361OMIM code: 212200

Definition

A rare inborn error of metabolism characterized by low serum carnosinase activity, persistent carnosinuria, and carnosinemia. The clinical phenotype is highly variable, with some patients remaining asymptomatic, while others have been reported to show severe developmental delay, intellectual disability, hypotonia, seizures, and other neurological signs and symptoms.

Disease data
Classification

Biological anomaly

Synonyms
Deficyt karnozydazy
ORPHA code
1361
OMIM code
212200
ICD10 code
E70.8
ICD11 code
5C50.F1

No additional description.

Orphanet - interntowa baza danych dotyczących rzadkich chorób i sierochych leków. ©INSERM 1999 - Dostępna na stronie www.orphanet.pl