Gene/Protein Disease Symptom Drug Enzyme Compound
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Query: UNIPROT:P17174 (aspartate aminotransferase)
 14,872 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

Liver dysfunction is a well-recognized complication of intestinal failure in children. Advances in total parenteral nutrition (TPN) have allowed these children to survive while their intestinal tract gradually adapts. Unfortunately TPN may lead to cholestatic liver disease particularly in the young children. Progression of liver disease is associated with a poor prognosis and is an indication for small bowel transplantation. We report our experience of orthotopic liver transplantation in four children with short gut and sequential liver and small bowel transplantation in one child. All children had TPN-related liver failure. Causes of intestinal failure included necrotising enterocolitis (n=2), gastroschisis (n=1), intestinal atresia (n=1), and megacystic, microcolon syndrome (n=1). At the time of liver transplantation the children's mean age was 10.9 months (2.5-24) and weight 6.7 kg (4.8-10.1). The mean serum bilirubin was 522 micromol/liter (299-823), aspartate transaminase 423 IU/liter (49-1024) and international normalized ratio 2.8 (2-3.9). There were two deaths both from respiratory failure secondary to adenovirus pneumonia including the child who received a sequential small bowel transplant. Three children with isolated liver grafts are alive and off TPN at 20 months (mean) follow up (range 6-35). Isolated orthotopic liver transplantation has a role in selected children with intestinal failure, particularly those with short but normally functioning gut and progressing with satisfactory intestinal adaptation but developing liver disease. Those children with TPN-related liver disease and unadapted gut or irreversible intestinal disease need combined liver and small bowel transplantation. Sequential small bowel transplantation is feasible after orthotopic liver transplantation and may provide an option for the child with terminal liver and small bowel failure.
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PMID:Isolated liver transplant and sequential small bowel transplantation for intestinal failure and related liver disease in children. 1086 33

Prediction of outcomes in pediatric intestinal failure is challenging but essential to guide intestinal rehabilitation and transplantation decisions. This review of intestinal failure patients spanning 10 years examines clinical details in relation to outcome to identify factors that may refine predictive accuracy. A search was conducted to identify all children with intestinal failure managed at Stollery Children's Hospital between January 1994 and December 2003. They were divided into 3 groups: early death occurring <or=30 days of age, parenteral nutrition dependence for 30-100 days, and parenteral nutrition dependence for >100 days. The long-term group was divided according to outcome: death or adaptation. Demographics, diagnosis, nutrition requirements, laboratory parameters, and clinical data were recorded. Groups were compared to identify factors associated with outcome. Necrotizing enterocolitis, gastroschisis, and intestinal atresias were the most common causes for intestinal failure; outcome was not related to diagnosis. Although withdrawal of therapy was common in the early death group, most babies had one or more additional significant comorbidity. Among the 29 babies requiring parenteral nutrition for >100 days with known outcomes, 12 died, 16 adapted fully, and 1 received a multivisceral transplant. Intestinal length >40 cm was associated with a significantly increased risk of mortality (P< .001). Abnormal laboratory values (bilirubin, aspartate aminotransferase, alanine aminotransferase, albumin, and platelet count) after 5 months of age were also significantly different between groups. This data, together with data from previous reviews, should be used to investigate potential predictive factors in prospective studies, particularly in the context of expert multidisciplinary care.
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PMID:10-year review of pediatric intestinal failure: clinical factors associated with outcome. 1868 97