MULTIVISCERAL TRANSPLANTATION FOR MEGACYSTIS MICROCOLON INTESTINAL HYPOPERISTALSIS SYNDROME
| Autor: | Werviston DeFaria, Jose Nery, Maria M. Rodriguez, John F. Thompson, Andreas G. Tzakis, Rita L. Romaguera, Antonio Daniele Pinna, M.F. Khan, Phillip Ruiz, Michele Masetti, Tomoaki Kato, Roberto Verzaro |
|---|---|
| Rok vydání: | 1999 |
| Předmět: |
medicine.medical_specialty
Colon medicine.medical_treatment Urinary Bladder Kidney Enteral administration Humans Medicine Abnormalities Multiple Postoperative Period Transplantation business.industry Intestinal Pseudo-Obstruction Infant Syndrome Megacystis Microcolon medicine.disease Gastrostomy Surgery Viscera Parenteral nutrition Liver Child Preschool Female Peristalsis Autopsy business Complication Neonatal bowel obstruction |
| Zdroj: | Transplantation. 68:228-232 |
| ISSN: | 0041-1337 |
| DOI: | 10.1097/00007890-199907270-00011 |
| Popis: | Background Megacystis microcolon intestinal hypoperistalsis syndrome (MMIHS) is a rare autosomal recessive disorder causing a functional neonatal bowel obstruction. Its etiopathogenesis is not fully understood. The prognosis is poor in the majority of cases; most patients die before the age of 6 months. In this report, we describe our experience with three patients with MMIHS in whom multivisceral transplantation was performed. Methods Three patients with MMIHS underwent multivisceral transplantation. All patients were females with a history of long-term total parenteral nutrition (TPN) with TPN-related cholestatic liver disease. Results Patient 1 died 17 months after transplantation because of aspiration after revision of her feeding gastrostomy. At the time of death, the graft was functioning and the patient was completely off TPN. Patient 2 is alive 17 months after transplant. She is a fully functional, active 2-year-old and has also recently begun oral feeding after intensive rehabilitation. Patient 3 died on day 44 of multisystem failure. Conclusions This is the first report in the literature of multivisceral transplantation for MMIHS. Although one of the three patients died 44 days after surgery from multiorgan system failure, the other two patients had long-term survival after transplant and both grew well on enteral feeding alone. One patient died 17 months from a non-transplant-related complication, while the other is living at home off of TPN, with almost complete dietary rehabilitation 17 months after transplant. Our case reports suggest that multivisceral transplantation is a valuable therapeutic option for patients affected by MMIHS with TPN-induced liver failure. |
| Databáze: | OpenAIRE |
| Externí odkaz: |
|