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CPS1 deficiency

Pathology: hyperammonemia Genetics: - autosomal recessive - associated with defects in CPS1 Clinical manifestations: - vomiting in infancy - protein intolerance - intermittent ataxia - seizures - lethargy - mental retardation Laboratory: - increased plasma NH3

General

amino acid inborn error of metabolism

Database Correlations

OMIM 237300

References

OMIM :accession 237300