Renal Focal Tubulointerstitial Fibrosis with Short Bowel Syndrome: Report of a Case

Department of Pediatric Surgery, Akita University, Akita, Akita, Japan
Surgery Today (Impact Factor: 1.53). 02/2002; 32(7):646-50. DOI: 10.1007/s005950200118
Source: PubMed


We report a male patient with short bowel syndrome (SBS) and renal focal tubulointerstitial fibrosis (FTIF). Seven years after surgery, he was introduced to us due to severe undernutrition, an impairment of growth hormone (GH) secretion, and abnormally low levels of plasma citrulline and arginine at 11 years 7 months of age, just before nutritional support using total parenteral nutrition (TPN) was begun. Thereafter, the support was changed to home TPN with GH supplementation. After an improvement of the disorders, GH was stopped at 17 years 3 months of age. However, hyperuricemia appeared and a renal biopsy revealed FTIF at 20 years of age. Home TPN was continued twice a week because the plasma arginine level was still low. His follow-up biopsy at 23 years of age showed morphometric amelioration. Arginine deficiency following SBS may be associated with FTIF. The cause of hyperuricemia after SBS therefore needs to be investigated in detail.

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Available from: Hirokazu Imai, Jun 26, 2014
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    ABSTRACT: Amino acid utilization and, therefore, demand differ between the healthy state and various disease states. In the healthy state most circulating amino acids are derived from dietary proteins that are stored and broken down in the gut and released gradually into the portal circulation, and from continuous turnover of body protein. In disease states, the amino acid composition of amino acids derived from periferal protein breakdown and released in the circulation, is different, for example because a substantial part of the branched-chain amino acids is broken down to yield glutamine and alanine, which are released in the circulation. It appears to be advantageous to mimic this continuous autoinfusion in patients, dependent of parenteral of enteral tube feeding. In disease, different endpoints should be used to assess the adequacy of the administered amino acid mix. Maintenance of a positive nitrogen balance and growth is less important than support of wound healing and immune function. Several amino acids such as glutamine, cysteine, and taurine are shown or suggested to be conditionally essential in disease, and to form substrate in the stressed patient for anabolic processes in liver, immune system, and injured sites. Amino acid toxicity is rare, and protein restriction for patients with renal or liver failure is obsolete because this only aggravated malnutrition. A true example of protein toxicity consists of gastrointestinal hemorrhage that precipitates hepatic encephalopathy in liver insufficiency, most likely because hemoglobin is an unbalanced protein because it lacks the essential amino acid isoleucine.
    No preview · Article · Jul 2004 · Journal of Nutrition