Megacystis microcolon intestinal hypoperistalsis syndrome: A report of a nationwide survey in Japan

Soh, Hideki; Fukuzawa, Masahiro; Kubota, Akio; Kawahara, Hisayoshi; Ueno, Takehisa; Taguchi, Tomoaki

doi:https://doi.org/10.1016/j.jpedsurg.2015.08.026

Megacystis microcolon intestinal hypoperistalsis syndrome: A report of a nationwide survey in Japan

Soh, Hideki; Fukuzawa, Masahiro; Kubota, Akio et al.

Journal of Pediatric Surgery, 2015, 50(12), 2048-2050

アクセス数:631件（2025-07-26 12:32 集計）

固定URL: https://hdl.handle.net/11094/99830

閲覧可能ファイル

ファイル	フォーマット	利用条件	サイズ	閲覧回数	利用開始日
JPediatrSurg_50_12_2048_Title	pdf	なし	92.1 KB	14	2025.01.23
JPediatrSurg_50_12_2048_ manuscript	pdf	なし	183 KB	12	2025.01.23
JPediatrSurg_50_12_2048_Table1	pdf	なし	32.4 KB	12	2025.01.23
JPediatrSurg_50_12_2048_Table2	pdf	なし	56.9 KB	12	2025.01.23
JPediatrSurg_50_12_2048_Table3	pdf	なし	26.9 KB	13	2025.01.23
JPediatrSurg_50_12_2048_Fig.1	pdf	なし	10.4 KB	13	2025.01.23
JPediatrSurg_50_12_2048_Figure legend,_References	pdf	なし	108 KB	13	2025.01.23

Creative Commons : 表示 - 非営利 - 改変禁止

論文情報

ファイル出力 EndNote Basic出力 Mendeley出力

タイトル	Megacystis microcolon intestinal hypoperistalsis syndrome: A report of a nationwide survey in Japan
著者	NRID 1000000457011 1000000457011 著者名 Soh, Hideki
	NRID 1000060165272 1000060165272 researchmap permalink read0106298 read0106298 著者名 Fukuzawa, Masahiro
	NRID 1000010161671 1000010161671 著者名 Kubota, Akio
	NRID 1000020224826 1000020224826 著者名 Kawahara, Hisayoshi
	NRID 1000010456957 1000010456957 ORCID 0000-0002-4639-8814 0000-0002-4639-8814 researchmap permalink ueno-pedsurg ueno-pedsurg Scopus Author ID 21743992300 21743992300 著者名 Ueno, Takehisa
	NRID 1000020197247 1000020197247 著者名 Taguchi, Tomoaki
キーワード等	Distended bladder
	Functional bowel obstruction
	Megacystis microcolon intestinal hypoperistalsis syndrome (MMIHS)
	Microcolon
内容	Soh H., Fukuzawa M., Kubota A., et al. Megacystis microcolon intestinal hypoperistalsis syndrome: A report of a nationwide survey in Japan. Journal of Pediatric Surgery 50, 2048 (2015); https://doi.org/10.1016/j.jpedsurg.2015.08.026.
抄録	Background: Megacystis microcolon intestinal hypoperistalsis syndrome (MMIHS) is a rare condition with a poor outcome. A nationwide survey was conducted to identify the clinical features and outcomes of MMIHS in Japan. Methods: Data were collected via a questionnaire, which was sent to 353 pediatric/pediatric surgical departments in Japan. Results: Of the 28 patients registered as having a certain diagnosis of MMIHS, 19 (male/female, 3/16) patients were analyzed. All of the patients developed functional bowel obstruction in the neonatal period and showed a distended bladder and microcolon in the radiological examination. A histopathology assessment of the full-thickness of intestinal specimens showed no pathological abnormalities in all patients. Although various medications were given, the patients did not show significant improvement. Drainage stomas were created in the jejunum (n = 11) and colon (n = 5). Sixteen patients were maintained by parenteral nutrition (PN). Nine patients died of sepsis or liver failure. The five- and ten-year survival rates were 63% and 57%, respectively. Conclusions: MMIHS patients developed severe functional intestinal obstruction in the neonatal period and had no specific therapeutic intervention. The majority of MMIHS patients required long-term PN. Small bowel or multivisceral transplantation may be necessary to improve the outcome of this condition.
出版者	Elsevier Inc.
収録物名	Journal of Pediatric Surgery
巻(号)	50(12)
ページ	2048-2050
刊行年月	2015-12-01
言語	英語
ハンドルURL	https://hdl.handle.net/11094/99830
PISSN	00223468
eISSN	15315037
関連情報 (isVersionOf)	DOI (出版社版) https://doi.org/10.1016/j.jpedsurg.2015.08.026
アクセス権	open access
権利情報	© 2015. This manuscript version is made available under the CC-BY-NC-ND 4.0 license https://creativecommons.org/licenses/by-nc-nd/4.0/
出版タイプ	Accepted Manuscript
カテゴリ	会議発表論文 Conference Paper

資源タイプ	学術雑誌論文
ローカル資源タイプ	学術雑誌論文
DCTERMS.bibliographicCitation	Journal of Pediatric Surgery. 2015, 50(12), p. 2048-2050
DC.title	Megacystis microcolon intestinal hypoperistalsis syndrome: A report of a nationwide survey in Japan
DC.creator	Soh, Hideki
	Fukuzawa, Masahiro
	Kubota, Akio
	Kawahara, Hisayoshi
	Ueno, Takehisa
	Taguchi, Tomoaki
DC.publisher	Elsevier Inc.
DC.language' scheme='DCTERMS.RFC1766	英語
DCTERMS.issued' scheme='DCTERMS.W3CDTF	2015-12-01
DC.identifier	https://doi.org/10.1016/j.jpedsurg.2015.08.026
DC.subject	Distended bladder
	Functional bowel obstruction
	Megacystis microcolon intestinal hypoperistalsis syndrome (MMIHS)
	Microcolon
DC.description	Soh H., Fukuzawa M., Kubota A., et al. Megacystis microcolon intestinal hypoperistalsis syndrome: A report of a nationwide survey in Japan. Journal of Pediatric Surgery 50, 2048 (2015); https://doi.org/10.1016/j.jpedsurg.2015.08.026.
DCTERMS.abstract	Background: Megacystis microcolon intestinal hypoperistalsis syndrome (MMIHS) is a rare condition with a poor outcome. A nationwide survey was conducted to identify the clinical features and outcomes of MMIHS in Japan. Methods: Data were collected via a questionnaire, which was sent to 353 pediatric/pediatric surgical departments in Japan. Results: Of the 28 patients registered as having a certain diagnosis of MMIHS, 19 (male/female, 3/16) patients were analyzed. All of the patients developed functional bowel obstruction in the neonatal period and showed a distended bladder and microcolon in the radiological examination. A histopathology assessment of the full-thickness of intestinal specimens showed no pathological abnormalities in all patients. Although various medications were given, the patients did not show significant improvement. Drainage stomas were created in the jejunum (n = 11) and colon (n = 5). Sixteen patients were maintained by parenteral nutrition (PN). Nine patients died of sepsis or liver failure. The five- and ten-year survival rates were 63% and 57%, respectively. Conclusions: MMIHS patients developed severe functional intestinal obstruction in the neonatal period and had no specific therapeutic intervention. The majority of MMIHS patients required long-term PN. Small bowel or multivisceral transplantation may be necessary to improve the outcome of this condition.
DC.rights	© 2015. This manuscript version is made available under the CC-BY-NC-ND 4.0 license https://creativecommons.org/licenses/by-nc-nd/4.0/
DC.format	99830
citation_title	Megacystis microcolon intestinal hypoperistalsis syndrome: A report of a nationwide survey in Japan
citation_author	Soh, Hideki
	Fukuzawa, Masahiro
	Kubota, Akio
	Kawahara, Hisayoshi
	Ueno, Takehisa
	Taguchi, Tomoaki
citation_publisher	Elsevier Inc.
citation_language	英語
citation_date	2015-12-01
citation_journal_title	Journal of Pediatric Surgery
citation_volume	50
citation_issue	12
citation_firstpage	2048
citation_lastpage	2050
citation_doi	https://doi.org/10.1016/j.jpedsurg.2015.08.026
citation_public_url	https://hdl.handle.net/11094/99830

Megacystis microcolon intestinal hypoperistalsis syndrome: A report of a nationwide survey in Japan

閲覧可能ファイル

論文情報

論文詳細を表示