Protein energy malnutrition leading to growth failure is an inevitable consequence of chronic liver disease in childhood. Although the precise pathophysiology is not understood considerable progress has been made in understanding the mechanisms of fat malabsorption and protein turnover in liver disease. There are many difficulties with the correct assessment of nutritional parameters in children with liver disease related to their abnormal body composition and energy expenditure and care needs to be taken with the interpretation of results. The effects of malnutrition secondary to chronic liver disease are varied and include fat soluble vitamin deficiencies, generalised growth failure, impairment of gastrointestinal function, immunosuppression and hypotonia. It is now recognised that malnutrition is an important risk factor for liver transplantation and increases both mortality and morbidity. Strategies to prevent or reverse malnutrition are now established and include the use of specific infant formulas based on low salt protein and an increased concentration of medium train triglyceride (50-70%). Careful nutritional support in association with generous fat soluble vitamin supplementation may produce dramatic improvement in catch up weight gain but for those children in whom growth failure persists, the only management is liver transplantation.

Child↗

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Is serum gentamicin level a good predictor of graft injury in intestinal transplantation?

INTRODUCTION: Following intestinal transplant (SBT), the early diagnosis and treatment of rejection is a major management aim. The diagnosis of rejection is based on histology of stomal biopsies. Oral gentamycin (2.5 mg/kg) was used for selective decontamination of the digestive system. Our hypothesis was that gentamycin might be absorbed in the presence of graft dysfunction. AIM: Our goal was to assess the correlation between serum gentamycin level and the health of the intestinal graft. SUBJECTS AND METHODS: Among 33 SBT performed from 1993 to 2005, serum gentamycin levels were performed once weekly or more often when there was a suspicion of rejection. All data were analyzed retrospectively. RESULTS: Adequate trough levels were achieved for only 23 patients, six of whom had histologically proven rejection and only one did not have a raised gentamycin content. Five patients with raised levels but no rejection included two with severe intestinal ischemia and three with bowel obstruction/ileus. Four of the five patients required laparotomies. CONCLUSION: We concluded that in our study raised serum gentamycin levels were a good predictor of rejection or significant injury to the graft.

Biomarkers↗

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New approaches to paediatric liver transplantation.

Explore the source record for details and available documents.

Child↗

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Yersinia enterocolitis in iron overload.

Yersinia enterocolitis with peritonitis and septicaemia developed in a 4-year-old child with thalassemia intermedia and iron overload. It is likely that the illness was exacerbated by continued administration of desferrioxamine, a bacterial siderophore, which facilitated systemic spread of Yersinia. It is recommended that Yersinia enterocolitis be considered in children with iron overload and that desferrioxamine be discontinued and prophylactic antibiotics be administered while Yersinia is sought.

Child, Preschool↗

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Symptomatic selenium deficiency in a child on home parenteral nutrition.

A 27-month-old girl with microvillous atrophy who was maintained on long-term home parenteral nutrition presented with regression of walking skills. She was found to have a tender skeletal myopathy affecting both legs only. Plasma selenium level was less than 0.05 mumol/L (0.4 microgram/dl) (normal 0.8-1.6 mumol/L; 6.3-12.6 micrograms/dl), red blood cell glutathione peroxidase was undetectable, and plasma glutathione peroxidase was 6 U/L (normal 90-350 U/L). Electromyography was abnormal, muscle biopsy showed a nonspecific myopathy, and muscle selenium was 0.23 micrograms/g dry weight (normal 0.45-1.05 micrograms/g dry weight). Following replacement with intravenous sodium selenite, there was a rapid improvement with complete disappearance of muscle pain and tenderness within 1 week. Crawling and walking skills were regained within 6 weeks, indicating that selenium deficiency was the cause of her symptoms. It is suggested that selenium be monitored and supplemented in children on long-term total parenteral nutrition particularly if enteral absorption is deficient.

Electromyography↗

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Liver transplantation therapy for children: Part 2.

End-stage liver disease affects nearly 300 children annually in the United States. Based upon the annual birth rate in the United States of 3.1 million, the incidence of the most prominent causes of liver disease in children (biliary atresia, alpha 1-antitrypsin deficiency, Wilson's disease, and neonatal hepatitis), and the frequency of end-stage liver disease in each disorder. Liver transplantation now offers these children at least a 65%, and in some series as high as 85%, chance of surviving at least 2 years postoperatively. Much of this improvement is attributable to advances in immunosuppression. However, one must not underestimate the importance of the tremendous knowledge that has been gained in the care of a large number of these patients over the last decade. The major barriers standing in the way of wider application of liver transplantation include the reluctance of primary care physicians to refer patients for early consideration, the growing crisis in the availability of donor organs, and the lack of adequate numbers of centers involved in performing adequate numbers of procedures. The major hurdles to overcome in order to affect further improvements in results include more specific immunosuppression, more accurate means of diagnosing rejection, better techniques of organ preservation, and further optimization of the care of both candidates preoperatively and recipients after transplantation.

Child↗