Isolated intestinal neuronal dysplasia Type B (IND-B) in Japan: Results from a nationwide survey

T. Taguchi, H. Kobayashi, Y. Kanamori, O. Segawa, A. Yamataka, M. Sugiyama, T. Iwanaka, N. Shimojima, T. Kuroda, A. Nakazawa, Y. Oda, K. Miyoshi, S. Ieiri

研究成果: Contribution to journalArticle査読

8 被引用数 (Scopus)

抄録

Purpose: Intestinal neuronal dysplasia Type B (IND-B) has been proposed to be an allied disorder of Hirschsprung's disease (ADHD). The original histological criteria included hyperganglionosis, giant ganglia, ectopic ganglion cells and an increased AChE activity in the lamina propria. The criteria for IND-B have been gradually revised. The present diagnostic criteria are [1] more than 20 % of the submucosal ganglia contain nine or more ganglion cells and [2] the patient is older than 1 year. To clarify the current status of IND-B in Japan, a nationwide retrospective cohort study was performed. Methods: Questionnaires were sent to 161 major institutes of pediatric surgery and gastroenterology in Japan. Results: A total of 355 cases of ADHD were collected, including 18 cases of IND-B (5 %). Based on original criteria, 13 out of 18 cases were diagnosed as IND-B. However, only four cases met the current criteria. Three of the four patients (75 %) required pull-through operation. All of the patients exhibited giant ganglia and ganglioneuromatosis-like hyperplasia of the myenteric plexus. Conclusions: IND-B cases matching the current criteria are thought to be quite rare and they are associated with marked hyperplasia of the myenteric plexus. "True" IND-B is a rare and intractable disease.

本文言語英語
ページ(範囲)815-822
ページ数8
ジャーナルPediatric surgery international
30
8
DOI
出版ステータス出版済み - 8 2014

All Science Journal Classification (ASJC) codes

  • Pediatrics, Perinatology, and Child Health
  • Surgery

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