- General Drug Summary
- Description
- Citrulline is an amino acid. It is made from ornithine and carbamoyl phosphate in one of the central reactions in the urea cycle. It is also produced from arginine as a by-product of the reaction catalyzed by NOS family. Its name is derived from citrullus, the Latin word for watermelon, from which it was first isolated.
- Also Known As
- 2-Amino-5-uredovaleric acid; Citrulline; delta-Ureidonorvaline; N5-(Aminocarbonyl)ornithine; N5-Carbamoyl-L-ornithine; N5-carbamoylornithine; N5-carbamylornithine; Sitrulline
- Categories
- Non-Essential Amino
- Groups
- approved; nutraceutical
- Structure
- Summary In Neonatal Jaundice
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1 record(s) for L-Citrulline Not Effective to Patients in Neonatal Jaundice.
- PMID
- Drug Name
- Efficacy
- Evidence
- 20614727
- L-Citrulline
- Not Effective to Patients
- Case Report
- Summary
- Elevated plasma citrulline was an important biochemical marker. NICCD should be considered in the differential diagnosis of cholestatic jaundice in Malysian infants regardless of ethnic origin.
- Neonatal intrahepatic cholestasis caused by citrin deficiency (NICCD) in three Malay children. The Malaysian journal of pathology, 2010 Jun [Go to PubMed]
- Citrin deficiency is an autosomal recessive disorder caused by mutation in the SLC25AJ3 gene. It has two major phenotypes: adult-onset type II citrullinemia (CTLN2) and neonatal intrahepatic cholestatic caused by citrin deficiency (NICCD). NICCD is characterized by neonatal/infantile-onset cholestatic hepatitis syndrome associated with multiple amino acidemia and hypergalactosemia. NICCD is self-limiting in most patients. However, some patients may develop CTLN2 years later, which manifests as fatal hyperammonemia coma. We report three unrelated Malay children with genetically confirmed NICCD characterised by an insertion mutation IVS16ins3kb in SLC25A13 gene. All 3 patients presented with prolonged neonatal jaundice which resolved without specific treatment between 5 to 10 months. Of note was the manifestation of a peculiar dislike of sweet foods and drinks. Elevated plasma citrulline was an important biochemical marker. NICCD should be considered in the differential diagnosis of cholestatic jaundice in Malysian infants regardless of ethnic origin.