Infantile cholestatic jaundice associated with adult-onset type II citrullinemia.

Tazawa, Y; Kobayashi, K; Ohura, T; Abukawa, D; Nishinomiya, F; Hosoda, Y; Yamashita, M; Nagata, I et al. · J Pediatr · 2001

case_report · Level V

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Abstract

Adult-onset type II citrullinemia, characterized by a liver-specific argininosuccinate synthetase deficiency, is caused by a deficiency of citrin that is encoded by the SLC25A13 gene. Three patients with infantile cholestatic jaundice were found to have mutations of the SLC25A13 gene. Adult-onset type II citrullinemia may be associated with infantile cholestatic disease.

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