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Neuropediatrics 1991; 22(4): 198-202
DOI: 10.1055/s-2008-1071441
Original article

© Georg Thieme Verlag KG Stuttgart · New York

A Nationwide Survey on Transient Hyperammonemia in Newborn Infants in Japan: Prognosis of Life and Neurological Outcome

M.  Yoshino1 , Y.  Sakaguchi1,2 , N.  Kuriya1 , Y.  Ohtani1 , F.  Yamashita1 , T.  Hashimoto3 , K.  Oyanagi4 , K.  Tada5 , K.  Narisawa6 , T.  Kitagawa7 , T.  Sakiyama7 , N.  Nagata8 , I.  Matsuda8
  • 1Department of Pediatrics & Child Health, Kurume University School of Medicine, Kurume, Japan
  • 2present affiliation; Sakaguchi Children's Clinic, Japan
  • 3High-risk Newborn Center, St. Mary's Hospital, Kurume, Japan
  • 4Department of Pediatrics, Sapporo Medical College, Sapporo, Japan
  • 5Department of Pediatrics, Tohoku University School of Medicine, Sendai, Japan
  • 6Department of Biochemical Genetics,Tohoku University School of Medicine, Sendai, Japan
  • 7Departments of Pediatrics, Nihon University School of Medicine, Tokyo, Japan
  • 8Departments of Pediatrics, Kumamoto University School of Medicine, Kumamoto, Japan
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Publication History

Publication Date:
19 March 2008 (online)

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Abstract

A nationwide survey of transient hyperammonemia in newborns was carried out in Japan. A total of 18 patients, consisting of 12 male and 6 female infants, were reported from 11 facilities. These neonates exhibited hyperammonemia with plasma ammonia levels in the range from 124 to 6256 µg/dl. Four newborn infants of the 18 died in the neonatal period, and an additional one died in the early infancy. Among the 13 infants who were alive at the time of this survey, 6 had neurological sequelae, including mental retardation, spastic quadriplegia and epilepsy. The multivariate analysis revealed that the Apgar score at 1 minute, peak plasma ammonia concentration, birth weight and sex were significant factors affecting the prognosis of life.

Key words

Transient hyperammonemia - Newborn infant - Prognosis - Neurological sequelae - Nationwide survey

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