Document Detail


Hirschsprung's disease in Japan: analysis of 3852 patients based on a nationwide survey in 30 years.
MedLine Citation:
PMID:  15868585     Owner:  NLM     Status:  MEDLINE    
Abstract/OtherAbstract:
BACKGROUND/PURPOSE: The introduction of laparoscope and transanal endorectal pull-through has caused a revolution in the operative procedures for Hirschsprung's disease. To study the changing profile of Hirschsprung's disease in Japan, the authors carried out a national survey. METHOD: Patient data were collected in 3 phases: group 1, 1628 patients between 1978 and 1982; group 2, 1121 patients between 1988 and 1992; and group 3, 1103 patients between 1998 and 2002, respectively. RESULTS: The incidence was 1:4697, 1:5544, and 1:5343 and the male/female ratio was 3.0:1, 3.4:1, and 3.0:1 in each group, respectively. Patients weighing less than 2500 g at birth increased to 10.4% in group 3, whereas they were 6.5% in group 2 and 5.5% in group 1. The patients with a family history also increased to 6.0% in group 3, in comparison with 2.8% in group 2 and 3.0% in group 1. The incidence of associated anomalies increased over time, 11.1% in group 1, 16.3% in group 2, and 21.2% in group 3. Mutations of genes were found in 4 of the 23 patients examined. The extent of aganglionosis was almost the same in each group. Regarding the definitive operation, the procedures without laparotomy, including transanal endorectal pull-through, increased up to 42.6% in group 3 but 0% in groups 1 and 2. The frequency that a primary operation without stoma was performed also increased. The age at definitive operation decreased in group 3. The incidence of preoperative and postoperative enterocolitis also decreased over time. The mortality was decreased over time, 7.1%, 4.9%, and 3.0% for groups 1, 2, and 3, respectively. CONCLUSIONS: The authors analyzed 3852 Japanese patients over 3 decades. The ratio of patients with a low birth weight, associated anomalies, or a family history was increased in the last 10 years. A primary operation without laparotomy has thus become the procedure of choice for a definitive operation.
Authors:
Sachiyo Suita; Tomoaki Taguchi; Satoshi Ieiri; Takanori Nakatsuji
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Publication Detail:
Type:  Journal Article; Multicenter Study; Research Support, Non-U.S. Gov't    
Journal Detail:
Title:  Journal of pediatric surgery     Volume:  40     ISSN:  1531-5037     ISO Abbreviation:  J. Pediatr. Surg.     Publication Date:  2005 Jan 
Date Detail:
Created Date:  2005-05-03     Completed Date:  2006-06-09     Revised Date:  2007-11-15    
Medline Journal Info:
Nlm Unique ID:  0052631     Medline TA:  J Pediatr Surg     Country:  United States    
Other Details:
Languages:  eng     Pagination:  197-201; discussion 201-2     Citation Subset:  IM    
Affiliation:
Department of Pediatric Surgery, Graduate School of Medical Sciences, Kyushu University, Fukuoka 812-8582, Japan. suita@pedsurg.med.kyushu-u.ac.jp
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MeSH Terms
Descriptor/Qualifier:
Anal Canal / abnormalities
Anastomosis, Surgical
Congenital Abnormalities / epidemiology
Digestive System Surgical Procedures*
Female
Genetic Predisposition to Disease
Health Surveys
Hirschsprung Disease / diagnosis,  epidemiology*,  surgery*
Humans
Incidence
Infant
Infant, Low Birth Weight
Infant, Newborn
Japan / epidemiology
Male
Rectum / abnormalities
Surgical Procedures, Minimally Invasive

From MEDLINE®/PubMed®, a database of the U.S. National Library of Medicine


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