Intestinal transplantation for total intestinal aganglionosis: a series of 12 consecutive children
| Autor: | Laurent Dupic, Yves Aigrain, Nathalie Bourdaud, Dominique Jan, Chiara Grimaldi, Jean Pierre Cezard, Olivier Goulet, Fabio Fusaro, Frédérique Sauvat, Virginie Colomb, Franck F. Ruemmele, Florence Lacaille, Yann Revillon |
|---|---|
| Rok vydání: | 2007 |
| Předmět: |
Adult
Diarrhea Graft Rejection Liver Cirrhosis Male Reoperation medicine.medical_specialty Cirrhosis medicine.medical_treatment Gastric Bypass Liver transplantation Gastroenterology Total Intestinal Aganglionosis Sepsis Feeding Methods Postoperative Complications Internal medicine medicine Humans Hirschsprung Disease Child Hirschsprung's disease Survival rate Postoperative Care business.industry Enterostomy Infant General Medicine medicine.disease Tissue Donors Surgery Liver Transplantation Transplantation Intestines Survival Rate Jaundice Obstructive Parenteral nutrition Child Preschool Pediatrics Perinatology and Child Health Female business |
| Zdroj: | Journal of pediatric surgery. 43(10) |
| ISSN: | 1531-5037 |
| Popis: | Background Management of patients with total intestinal aganglionosis (TIA) is a medical challenge because of their dependency on parenteral nutrition (PN). Intestinal transplantation (ITx) represents the only alternative treatment for patients with irreversible intestinal failure for achieving intestinal autonomy. Methods Among 66 patients who underwent ITx in our center, 12 had TIA. They received either isolated ITx (n = 4) or liver-ITx (LITx, n=8) after 10 to 144 months of total PN. All grafts included the right colon. Results After a median follow-up of 57 months, the survival rate was 62.5% in the LITx group and 100% in the ITx patients. The graft survival rate was 62.5% in the LITx group and 75% in the ITx group. All the surviving patients were fully weaned from total PN, after a median of 57 days. Pull through of the colon allograft was carried out in all patients. Fecal continence is normal in all but one of the surviving children. Conclusion These results suggest that ITx with colon grafting should be the preferred therapeutic option in TIA. Early referral to a transplantation center after diagnosis of TIA is critical to prevent PN-related cirrhosis and thereby to permit ITx, which is associated with a good survival rate. |
| Databáze: | OpenAIRE |
| Externí odkaz: |
|
Full Text from ScienceDirect