Outcomes in children after intestinal transplant
Child; Female; Humans; Male; Survival Rate; Treatment Outcome; Preschool; infant; retrospective studies; Intestines/transplantation; Intestinal Diseases/surgery
OBJECTIVE: The survival rates after pediatric intestinal transplant according to underlying disease are unknown. The objective of our study was to describe the population of pediatric patients receiving an intestinal transplant and to evaluate survival according to specific disease condition. PATIENTS: Pediatric patients (< or =21 years of age) with intestinal failure meeting criteria for intestinal transplant were included in the study. METHODS: A retrospective review of the United Network for Organ Sharing intestinal transplant database (January 1, 1991, to May 16, 2008), including all pediatric transplant centers participating in the United Network for Organ Sharing, was conducted. The main outcome measures were survival and mortality. RESULTS: Eight hundred fifty-two children received an intestinal transplant (54% male). Median age and weight at the time of transplant were 1 year (interquartile rage: 1-5) and 10.7 kg (interquartile rage: 7.8-21.7). Sixty-nine percent of patients also received a simultaneous liver transplant. The most common diagnoses among patients who received a transplant were gastroschisis (24%), necrotizing enterocolitis (15%), volvulus (14%), other causes of short-gut syndrome (19%), functional bowel syndrome (16%), and Hirschsprung disease (7%). The Kaplan-Meier curves demonstrated variation in patient survival according to diagnosis. Cox regression analysis confirmed a survival difference according to diagnosis (P < .001) and demonstrated a survival advantage for those patients listed with a diagnosis of volvulus (P < .01) compared with the reference gastroschisis. After adjusting for gender, recipient weight, and concomitant liver transplant, children with volvulus had a lower hazard ratio for survival and a lower risk of mortality. CONCLUSIONS: Survival after intestinal transplant was associated with the underlying disease state. The explanation for these findings requires additional investigation into the differences in characteristics of the population of children with intestinal failure.
2010
Lao OB; Healey PJ; Perkins JD; Horslen S; Reyes JD; Goldin AB
Pediatrics
2010
Article information provided for research and reference use only. PedPalASCNET does not hold any rights over the resource listed here. All rights are retained by the journal listed under publisher and/or the creator(s).
Journal Article
<a href="http://doi.org/10.1542/peds.2009-1713" target="_blank" rel="noreferrer">10.1542/peds.2009-1713</a>
Irreversible intestinal failure
Child; Humans; Parenteral Nutrition; Adaptation; infant; Physiological; Gastrointestinal Motility; Intestinal Absorption; Digestive Physiology; Hirschsprung Disease/complications; Intestinal Pseudo-Obstruction/complications/surgery; Intestines/transplantation; Liver Diseases/prevention & control; Malabsorption Syndromes/etiology/therapy; Short Bowel Syndrome/complications/surgery
Intestinal failure (IF) can be defined as the reduction of functional gut mass below the minimal amount necessary for digestion and absorption adequate to satisfy the nutrient and fluid requirements for maintenance in adults or growth in children. In developed countries, IF mainly includes individuals with the congenital or early onset of conditions requiring protracted or indefinite parenteral nutrition (PN). Short bowel syndrome was the first commonly recognized cause of protracted IF. The normal physiologic process of intestinal adaptation after extensive resection usually allows for recovery of sufficient intestinal function within weeks to months. During this time, patients can be sustained on parenteral nutrition. Only a few children have permanent intestinal insufficiency and life-long dependency on PN. Non-transplant surgery including small bowel tapering and lengthening may allow weaning from PN in some cases. Hormonal therapy with recombinant human growth hormone has produced poor results while therapy with glucagon-like peptide-2 holds promise. Congenital diseases of enterocyte development such as microvillus inclusion disease or intestinal epithelial dysplasia cause permanent IF for which no curative medical treatment is currently available. Severe and extensive motility disorders such as total or subtotal intestinal aganglionosis (long segment Hirschsprung disease) or chronic intestinal pseudo-obstruction syndrome may also cause permanent IF. PN and home-PN remain are the mainstays of therapy regardless of the cause of IF. Some patients develop complications while receiving long-term PN for IF especially catheter related complications (thrombosis, sepsis) and liver disease. These patients may be candidates for intestinal transplantation. This review discusses the causes of irreversible IF and emphasizes the specific medico-surgical strategies for prevention and treatment of these conditions at several stages of IF.
2004
Goulet O; Ruemmele F; Lacaille F; Colomb V
Journal Of Pediatric Gastroenterology And Nutrition
2004
Article information provided for research and reference use only. PedPalASCNET does not hold any rights over the resource listed here. All rights are retained by the journal listed under publisher and/or the creator(s).
Journal Article
<a href="http://doi.org/10.1097/00005176-200403000-00006" target="_blank" rel="noreferrer">10.1097/00005176-200403000-00006</a>