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A national survey of patients with intestinal motility disorders who are potential candidates for intestinal transplantation in Japan.
MedLine Citation:
PMID:  23769101     Owner:  NLM     Status:  In-Data-Review    
Abstract/OtherAbstract:
Intestinal motility disorders are a major cause of intestinal failure. Severe cases such as idiopathic pseudo-obstruction represent life-threatening illnessed. Intestinal transplantation is a treatment for severe motility disorders with irreversible intestinal failure. However, the prevalence of severe motility disorders is unknown. We performed a national survey to identify patients with intestinal motility disorders who require an intestinal transplant. The national survey of 302 institutions treating intestinal motility disorders identified 147 patients treated from 2006 to 2011 at 46 institutions. The mean patient age was 12.1 years (range, 0.3-77.5). The mean age of onset was 3.0 years (range, 0.0-68.8). Diagnoses included chronic idiopathic intestinal pseudo-obstruction (n = 96), Hirschsprung disease (n = 29), megacystis microcolon intestinal hypoperistalsis syndrome (n = 18), and other (n = 6). There were 126 survivors and 21 patients who died during the last 5 years. The mortality rate was 14.3%. Eighty-five percent of patients required parenteral nutrition for more than 6 months, which was defined as irreversible intestinal failure. Among surviving patients with irreversible intestinal failure, 8 (9.4 %) developed hepatic failure with jaundice and 27 (31.8%) 2 or more central vein thromboses. In all, at least 35 patients (41%) with irreversible failure due to intestinal motility disorders may be candidates for transplantation. The prevalence of severe intestinal motility disorders was elucidated in Japan. Severe cases should be referred to transplant centers.
Authors:
T Ueno; M Wada; K Hoshino; S Sakamoto; H Furukawa; M Fukuzawa
Publication Detail:
Type:  Journal Article    
Journal Detail:
Title:  Transplantation proceedings     Volume:  45     ISSN:  1873-2623     ISO Abbreviation:  Transplant. Proc.     Publication Date:  2013 Jun 
Date Detail:
Created Date:  2013-06-17     Completed Date:  -     Revised Date:  -    
Medline Journal Info:
Nlm Unique ID:  0243532     Medline TA:  Transplant Proc     Country:  United States    
Other Details:
Languages:  eng     Pagination:  2029-31     Citation Subset:  IM    
Copyright Information:
Copyright © 2013 Elsevier Inc. All rights reserved.
Affiliation:
Pediatric Surgery, Osaka University, Suita, Japan. Electronic address: ueno@pedsurg.med.osaka-u.ac.jp.
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