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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.
Pubmed: (View Article at PubMed) PMID:16703326
DOI: Full-text: DOI:10.1007/s00431-006-0143-y

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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CRRD Object Information
CRRD ID: 12743607
Created: 2017-02-09
Species: All species
Last Modified: 2017-02-09
Status: ACTIVE


RGD is funded by grant HL64541 from the National Heart, Lung, and Blood Institute on behalf of the NIH.