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01.09.2006 | Original Paper

Effects of arginine treatment on nutrition, growth and urea cycle function in seven Japanese boys with late-onset ornithine transcarbamylase deficiency

verfasst von: Hironori Nagasaka, Tohru Yorifuji, Kei Murayama, Mitsuru Kubota, Keiji Kurokawa, Tomoko Murakami, Masaki Kanazawa, Tomozumi Takatani, Atsushi Ogawa, Emi Ogawa, Shigenori Yamamoto, Masanori Adachi, Kunihiko Kobayashi, Masaki Takayanagi

Erschienen in: European Journal of Pediatrics | Ausgabe 9/2006

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Abstract

Background

The aim of this study was to investigate the effects of arginine on nutrition, growth and urea cycle function in boys with late-onset ornithine transcarbamylase deficiency (OTCD). Seven Japanese boys with late-onset OTCD enrolled in this study resumed arginine treatment after the cessation of this therapy for a few years. Clinical presentations such as vomiting and unconsciousness, plasma amino acids and urinary orotate excretion were followed chronologically to evaluate urea cycle function and protein synthesis with and without this therapy. In addition to height and body weight, blood levels of proteins, lipids, growth hormone (GH), insulin-like growth factor-I (IGF-I) and IGF-binding protein -3 (IGFBP-3) were monitored.

Results

The frequency of hyperammonemic attacks and urinary orotate excretion decreased significantly following the resumption of arginine treatment. Despite showing no marked change in body weight, height increased gradually. Extremely low plasma arginine increased to normal levels, while plasma glutamine and alanine levels decreased considerably. Except for a slight increase in high-density lipoprotein cholesterol level, blood levels of markers for nutrition did not change. In contrast, low serum IGF-I and IGFBP-3 levels increased to age-matched control levels, and normal urinary GH secretion became greater than the level observed in the controls.

Conclusion

Arginine treatment is able to reduces attacks of hyperammonemia in boys with late-onset OTCD and to increase their growth.

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Titel: Effects of arginine treatment on nutrition, growth and urea cycle function in seven Japanese boys with late-onset ornithine transcarbamylase deficiency
verfasst von: Hironori Nagasaka
Tohru Yorifuji
Kei Murayama
Mitsuru Kubota
Keiji Kurokawa
Tomoko Murakami
Masaki Kanazawa
Tomozumi Takatani
Atsushi Ogawa
Emi Ogawa
Shigenori Yamamoto
Masanori Adachi
Kunihiko Kobayashi
Masaki Takayanagi
Publikationsdatum: 01.09.2006
Verlag: Springer-Verlag
Erschienen in: European Journal of Pediatrics / Ausgabe 9/2006
Print ISSN: 0340-6199
Elektronische ISSN: 1432-1076
DOI: https://doi.org/10.1007/s00431-006-0143-y

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Effects of arginine treatment on nutrition, growth and urea cycle function in seven Japanese boys with late-onset ornithine transcarbamylase deficiency