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Journal of the Korean Society of Neonatology 2001;8(1):156-160.
Published online May 1, 2001.
A Case of Transient Hyperammonemia of the Newborn Infant.
Ji Youn Choi, Sang Hee Lee, Seong Sook Jun, Son Sang Seo
Department of Pediatrics, Ilsin Christian Hospital, Pusan, Korea.
신생아 일과성 고암모니아혈증 1례
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Transient hyperammonemia of the newborn is an overwhelming disease manifestated by hyperammonemic coma in ill premature infant. This recognized metabolic disorder is chiefly characterized by severe hyperammonemia in the postnatal period, a comatous state, absence of abnormal organic aciduria, normal activity of urea cycle enzymes and, usually, complete recovery. The etiology is unknown. Infant had mild respiratory distress that progressed within 48 hours to deep coma requiring ventilatory assistance and had marked hyperammonemia. The degree of neurologic impairment and developmental delay in this disorder depends on the duration of the hyperammonemic coma. So, treatment of hyperammonemia should be initiated promptly and continued vigorously. We report of a preterm infant (34+5 weeks of gestation) presenting with respiratory distress, seizure, coma, and marked elevated plasma ammonia level.
Key Words: Transient hyperammonemia; Newborn infant


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