Systemic primary carnitine deficiency

Orpha code: 158OMIM code: 212140

Definition

A disorder of carnitine cycle and carnitine transport that is characterized classically by early childhood onset cardiomyopathy often with weakness and hypotonia, failure to thrive and recurrent hypoglycemic hypoketotic seizures and/or coma.

Disease data
Classification

Disease

Synonyms
CDSP
CDSP
CUD
Defekt transportera karnityny
Deficyt wychwytu karnityny
Niedobór osoczowo-błonowego transportera karnityny
SPCD
CUD
Carnitine transporter defect
Carnitine uptake deficiency
Deficiency of plasma-membrane carnitine transporter
SPCD
ORPHA code
158
OMIM code
212140
ICD10 code
E71.3
ICD11 code
5C52.00

No additional description.

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