Hirschsprung's disease in Japan: analysis of 3852 patients based on a nationwide survey in 30 years
Section snippets
Patients and methods
Questionnaires consisting of an individual patient form relating to the general incidence, diagnosis, and treatment of Hirschsprung's disease were sent to 196 departments of pediatric surgery at major hospitals throughout Japan. The patients studied in this survey were limited to cases appearing during the 5-year period from 1998 to 2002. The completed forms of 1103 patients were sent back from 159 hospitals (81.1%), including almost all major services for pediatric surgery in Japan. These data
Results
The estimated incidence of Hirschsprung's disease based on the annual number of cases divided by the annual number of newborns 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 groups 1, 2, and 3, respectively. The percentage of patients with a birth weight less than 2500 g significantly increased to 10.4% in group 3 as compared with 6.5% in group 2 and 5.5% in group 1. The patients with a family history also significantly increased to 6.0% in group 3 as
Discussion
There have so far been very few reports on multi-institutional surveys of Hirschsprung's disease. The members of a surgical section of the American Academy of Pediatrics collected 1196 cases and showed an estimated incidence of 1/5257 [7]. A recent report from Oman showed a higher incidence of 1/3070 [8], whereas a report from Australia showed a lower incidence of 1/7165 [9]. The incidence in Japan is almost the same as that in the United States and it is also similar to the generally accepted
Acknowledgment
The authors thank Mr Brian Quinn for reading the manuscript and Dr Naoko Kinukawa for helping with statistical analysis.
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