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Neurodevelopmental disorder with epilepsy and hemochromatosis(NEDEPH)

MedGen UID:
989059
Concept ID:
CN307964
Disease or Syndrome
Synonym: NEDEPH
 
Gene (location): PIGA (Xp22.2)
 
OMIM®: 301072

Definition

Neurodevelopmental disorder with epilepsy and hemochromatosis (NEDEPH) is an X-linked recessive disorder characterized by global developmental delay, early-onset seizures, and progressive systemic iron deposition particularly affecting the liver and resulting in juvenile-onset hemochromatosis. Variable additional features may include joint contractures, visual or hearing impairment, and skin abnormalities (summary by Swoboda et al., 2014 and Muckenthaler et al., 2022). [from OMIM]

Recent clinical studies

Etiology

Hui J, Kirby DM, Thorburn DR, Boneh A
Dev Med Child Neurol 2006 Feb;48(2):132-6. doi: 10.1017/S0012162206000284. PMID: 16417669

Diagnosis

Hui J, Kirby DM, Thorburn DR, Boneh A
Dev Med Child Neurol 2006 Feb;48(2):132-6. doi: 10.1017/S0012162206000284. PMID: 16417669

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