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Late-onset citrullinemia type I

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Disease definition

A form of citrullinemia type I characterized clinically by adult onset of symptoms including variable hyperammonemia and less striking neurological findings which may include intense headache, scotomas, migraine-like episodes, ataxia, slurred speech, lethargy and drowsiness. Serious increased intracranial pressure may occur.

ORPHA:247573

Classification level: Subtype of disorder

Synonym(s):
  • Late-onset citrullinemia type 1

Source: PubMed ID 31469252

Prevalence: -

Inheritance: Autosomal recessive

Age of onset: Adult

ICD-10: E72.2

OMIM: 215700

UMLS: C0268546

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
English (2012.pdf) - Brit Inher Metab Dis Group
Clinical practice guidelines
English (2019) - Orphanet J Rare Dis
Disease review articles
Clinical genetics review
English (2022) - GeneReviews
The documents contained in this website are presented for information purposes only. The material is in no way intended to replace professional medical care by a qualified specialist and should not be used as a basis for diagnosis or treatment.