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

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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.
Authors:	Nagasaka, Hironori Yorifuji, Tohru Murayama, Kei Kubota, Mitsuru Kurokawa, Keiji Murakami, Tomoko Kanazawa, Masaki Takatani, Tomozumi Ogawa, Atsushi Ogawa, Emi Yamamoto, Shigenori Adachi, Masanori Kobayashi, Kunihiko Takayanagi, Masaki
Citation:	Nagasaka H, etal., Eur J Pediatr. 2006 Sep;165(9):618-24. Epub 2006 May 16.
RGD ID:	12743607
Pubmed:	PMID:16703326 (View Abstract at PubMed)
DOI:	DOI:10.1007/s00431-006-0143-y (Journal Full-text)
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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Object Symbol	Species	Term	Qualifier	Evidence	With	Notes	Source	Original Reference(s)
IGFBP3	Human	ornithine carbamoyltransferase deficiency	treatment	IEP			RGD
Igfbp3	Rat	ornithine carbamoyltransferase deficiency	treatment	ISO	IGFBP3 (Homo sapiens)		RGD
Igfbp3	Mouse	ornithine carbamoyltransferase deficiency	treatment	ISO	IGFBP3 (Homo sapiens)		RGD

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Genes (Rattus norvegicus)

Igfbp3 (insulin-like growth factor binding protein 3)

Genes (Mus musculus)

Igfbp3 (insulin-like growth factor binding protein 3)

Genes (Homo sapiens)

IGFBP3 (insulin like growth factor binding protein 3)

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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.