Acquired isolated hypoganglionosis as a distinct entity: results from a nationwide survey

Satoshi Obata, Koichiro Yoshimaru, Kosuke Kirino, Tomoko Izaki, Satoshi Ieiri, Atsuyuki Yamataka, Tsugumichi Koshinaga, Jun Iwai, Hitoshi Ikeda, Hiroshi Matsufuji, Yoshinao Oda, Tomoaki Taguchi

研究成果: ジャーナルへの寄稿記事

抄録

Purpose: Acquired isolated hypoganglionosis (A-IH) is a late-onset intestinal pseudo-obstruction disorder and shows different pathophysiological findings from congenital isolated hypoganglionosis (C-IH). In this study, we retrospectively examined five cases of A-IH and investigated the features of A-IH. Methods: Five cases of A-IH were extracted from a nationwide retrospective cohort study in 10 years, from which totally 355 cases of Allied Disorders of Hirschsprung’s Disease (ADHD) were collected. Results: Ages of onset were between 13 and 17 years in three cases, and 4 years and 4 months in ones. Initial symptoms were abdominal distension and/or chronic constipation in 4 cases, whereas one exhibited intestinal perforation. Affected lesions varied from case to case, extending various length of intestinal tracts. All cases underwent multiple operations (average: 4.6 times), such as enterostomy, resection of dilated intestines, and/or pull-through. Pathological findings showed the decreased numbers of ganglion cells and degeneration of ganglion cells, whereas the size of the plexus was normal. Currently, all cases were alive and almost all eat regular food without requiring parenteral feeding. Conclusion: A-IH is rare, but distinct entity characterized by different clinical courses and pathological findings from those of C-IH. The outcome is considered to be favorable after a resection of affected intestine.

元の言語英語
ページ(範囲)215-220
ページ数6
ジャーナルPediatric surgery international
35
発行部数2
DOI
出版物ステータス出版済み - 2 5 2019

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Ganglia
Intestines
Enterostomy
Intestinal Perforation
Intestinal Pseudo-Obstruction
Hirschsprung Disease
Parenteral Nutrition
Constipation
Cell Size
Age of Onset
Cohort Studies
Retrospective Studies
Cell Count
Food
Surveys and Questionnaires

All Science Journal Classification (ASJC) codes

  • Pediatrics, Perinatology, and Child Health
  • Surgery

これを引用

Acquired isolated hypoganglionosis as a distinct entity : results from a nationwide survey. / Obata, Satoshi; Yoshimaru, Koichiro; Kirino, Kosuke; Izaki, Tomoko; Ieiri, Satoshi; Yamataka, Atsuyuki; Koshinaga, Tsugumichi; Iwai, Jun; Ikeda, Hitoshi; Matsufuji, Hiroshi; Oda, Yoshinao; Taguchi, Tomoaki.

:: Pediatric surgery international, 巻 35, 番号 2, 05.02.2019, p. 215-220.

研究成果: ジャーナルへの寄稿記事

Obata, Satoshi ; Yoshimaru, Koichiro ; Kirino, Kosuke ; Izaki, Tomoko ; Ieiri, Satoshi ; Yamataka, Atsuyuki ; Koshinaga, Tsugumichi ; Iwai, Jun ; Ikeda, Hitoshi ; Matsufuji, Hiroshi ; Oda, Yoshinao ; Taguchi, Tomoaki. / Acquired isolated hypoganglionosis as a distinct entity : results from a nationwide survey. :: Pediatric surgery international. 2019 ; 巻 35, 番号 2. pp. 215-220.
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abstract = "Purpose: Acquired isolated hypoganglionosis (A-IH) is a late-onset intestinal pseudo-obstruction disorder and shows different pathophysiological findings from congenital isolated hypoganglionosis (C-IH). In this study, we retrospectively examined five cases of A-IH and investigated the features of A-IH. Methods: Five cases of A-IH were extracted from a nationwide retrospective cohort study in 10 years, from which totally 355 cases of Allied Disorders of Hirschsprung’s Disease (ADHD) were collected. Results: Ages of onset were between 13 and 17 years in three cases, and 4 years and 4 months in ones. Initial symptoms were abdominal distension and/or chronic constipation in 4 cases, whereas one exhibited intestinal perforation. Affected lesions varied from case to case, extending various length of intestinal tracts. All cases underwent multiple operations (average: 4.6 times), such as enterostomy, resection of dilated intestines, and/or pull-through. Pathological findings showed the decreased numbers of ganglion cells and degeneration of ganglion cells, whereas the size of the plexus was normal. Currently, all cases were alive and almost all eat regular food without requiring parenteral feeding. Conclusion: A-IH is rare, but distinct entity characterized by different clinical courses and pathological findings from those of C-IH. The outcome is considered to be favorable after a resection of affected intestine.",
author = "Satoshi Obata and Koichiro Yoshimaru and Kosuke Kirino and Tomoko Izaki and Satoshi Ieiri and Atsuyuki Yamataka and Tsugumichi Koshinaga and Jun Iwai and Hitoshi Ikeda and Hiroshi Matsufuji and Yoshinao Oda and Tomoaki Taguchi",
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T2 - results from a nationwide survey

AU - Obata, Satoshi

AU - Yoshimaru, Koichiro

AU - Kirino, Kosuke

AU - Izaki, Tomoko

AU - Ieiri, Satoshi

AU - Yamataka, Atsuyuki

AU - Koshinaga, Tsugumichi

AU - Iwai, Jun

AU - Ikeda, Hitoshi

AU - Matsufuji, Hiroshi

AU - Oda, Yoshinao

AU - Taguchi, Tomoaki

PY - 2019/2/5

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N2 - Purpose: Acquired isolated hypoganglionosis (A-IH) is a late-onset intestinal pseudo-obstruction disorder and shows different pathophysiological findings from congenital isolated hypoganglionosis (C-IH). In this study, we retrospectively examined five cases of A-IH and investigated the features of A-IH. Methods: Five cases of A-IH were extracted from a nationwide retrospective cohort study in 10 years, from which totally 355 cases of Allied Disorders of Hirschsprung’s Disease (ADHD) were collected. Results: Ages of onset were between 13 and 17 years in three cases, and 4 years and 4 months in ones. Initial symptoms were abdominal distension and/or chronic constipation in 4 cases, whereas one exhibited intestinal perforation. Affected lesions varied from case to case, extending various length of intestinal tracts. All cases underwent multiple operations (average: 4.6 times), such as enterostomy, resection of dilated intestines, and/or pull-through. Pathological findings showed the decreased numbers of ganglion cells and degeneration of ganglion cells, whereas the size of the plexus was normal. Currently, all cases were alive and almost all eat regular food without requiring parenteral feeding. Conclusion: A-IH is rare, but distinct entity characterized by different clinical courses and pathological findings from those of C-IH. The outcome is considered to be favorable after a resection of affected intestine.

AB - Purpose: Acquired isolated hypoganglionosis (A-IH) is a late-onset intestinal pseudo-obstruction disorder and shows different pathophysiological findings from congenital isolated hypoganglionosis (C-IH). In this study, we retrospectively examined five cases of A-IH and investigated the features of A-IH. Methods: Five cases of A-IH were extracted from a nationwide retrospective cohort study in 10 years, from which totally 355 cases of Allied Disorders of Hirschsprung’s Disease (ADHD) were collected. Results: Ages of onset were between 13 and 17 years in three cases, and 4 years and 4 months in ones. Initial symptoms were abdominal distension and/or chronic constipation in 4 cases, whereas one exhibited intestinal perforation. Affected lesions varied from case to case, extending various length of intestinal tracts. All cases underwent multiple operations (average: 4.6 times), such as enterostomy, resection of dilated intestines, and/or pull-through. Pathological findings showed the decreased numbers of ganglion cells and degeneration of ganglion cells, whereas the size of the plexus was normal. Currently, all cases were alive and almost all eat regular food without requiring parenteral feeding. Conclusion: A-IH is rare, but distinct entity characterized by different clinical courses and pathological findings from those of C-IH. The outcome is considered to be favorable after a resection of affected intestine.

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