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Acute neonatal citrullinemia type I

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Disease definition

A severe form of citrullinemia type 1 characterized biologically by hyperammonemia and clinically by progressive lethargy, poor feeding and vomiting, seizures and possible loss of consciousness, within one to a few days of birth, with variable signs of increased intracranial pressure. The condition can lead to significant neurologic deficits.

ORPHA:247546

Classification level: Subtype of disorder

Synonym(s):
  • Early-onset citrullinemia type I
  • Acute neonatal citrullinemia type 1
  • Early-onset citrullinemia type 1

Source: PubMed ID 31469252

Prevalence: -

Inheritance: Autosomal recessive

Age of onset: Infancy, Neonatal

ICD-10: E72.2

ICD-11: 5C50.A3

OMIM: 215700

UMLS: C5679618

A summary on this disease is available in Français, Español, Deutsch, Italiano, Nederlands Ελληνικά
Detailed information

Logo ERN: produced/endorsed by ERN(s) Logo FSMR: produced/endorsed by FSMR(s)

Guidelines
Emergency guidelines
Français (2023.pdf) - Orphanet Urgences
Clinical practice guidelines
English (2019) - Orphanet J Rare Dis
Disease review articles
Clinical genetics review
English (2022) - GeneReviews
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