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Historical Cohort Study of Congenital Isolated Hypoganglionosis of the Intestine: Determining the Best Surgical Interventions

No standard diagnostic method or surgical treatment for congenital isolated hypoganglionosis (CIHG) has been established. This study aimed to analyze the clinical outcomes of patients with CIHG and identify the best surgical interventions provided thus far. Data on surgical interventions in 19 patie...

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Autores principales: Yamada, Yohei, Mori, Teizaburo, Takahashi, Nobuhiro, Fujimura, Takumi, Kano, Motohiro, Kato, Mototoshi, Takahashi, Masataka, Shimojima, Naoki, Watanabe, Toshihiko, Yoshioka, Takako, Kanamori, Yutaka, Kuroda, Tatsuo, Fujino, Akihiro
Formato: Online Artículo Texto
Lenguaje:English
Publicado: MDPI 2023
Materias:
Acceso en línea:https://www.ncbi.nlm.nih.gov/pmc/articles/PMC10605557/
https://www.ncbi.nlm.nih.gov/pubmed/37892242
http://dx.doi.org/10.3390/biom13101560
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author Yamada, Yohei
Mori, Teizaburo
Takahashi, Nobuhiro
Fujimura, Takumi
Kano, Motohiro
Kato, Mototoshi
Takahashi, Masataka
Shimojima, Naoki
Watanabe, Toshihiko
Yoshioka, Takako
Kanamori, Yutaka
Kuroda, Tatsuo
Fujino, Akihiro
author_facet Yamada, Yohei
Mori, Teizaburo
Takahashi, Nobuhiro
Fujimura, Takumi
Kano, Motohiro
Kato, Mototoshi
Takahashi, Masataka
Shimojima, Naoki
Watanabe, Toshihiko
Yoshioka, Takako
Kanamori, Yutaka
Kuroda, Tatsuo
Fujino, Akihiro
author_sort Yamada, Yohei
collection PubMed
description No standard diagnostic method or surgical treatment for congenital isolated hypoganglionosis (CIHG) has been established. This study aimed to analyze the clinical outcomes of patients with CIHG and identify the best surgical interventions provided thus far. Data on surgical interventions in 19 patients were collected between 1992 and 2020, including the type of enterostomy, type of revision, and length of the intestines. Ganglion cells in the myenteric plexus were enumerated using Hu C/D staining. The ratio of the length of the small intestine to its height was defined as the intestinal ratio (IR). The outcomes were assessed using the stoma output, growth parameters including the body mass index (BMI), and parenteral nutrition (PN) dependency. All patients required a diverting enterostomy. The IR ranged from 0.51 to 1.75 after multiple non-transplant surgeries. The stoma types were tube-stoma, end-stoma, Santulli-type, and Bishop–Koop (BK)-type. Patients with Santulli- or BK-type stomas had better BMIs and less PN dependency in terms of volume than those with end-stomas or tube-stomas. Two patients with BK-type stomas were off PN, and three who underwent an intestinal transplantation (Itx) achieved enteral autonomy. The management of CIHG involves a precise diagnosis using Hu C/D staining, neonatal enterostomy, and stoma revision using the adjusted IR and Itx if other treatments do not enable enteral autonomy.
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spelling pubmed-106055572023-10-28 Historical Cohort Study of Congenital Isolated Hypoganglionosis of the Intestine: Determining the Best Surgical Interventions Yamada, Yohei Mori, Teizaburo Takahashi, Nobuhiro Fujimura, Takumi Kano, Motohiro Kato, Mototoshi Takahashi, Masataka Shimojima, Naoki Watanabe, Toshihiko Yoshioka, Takako Kanamori, Yutaka Kuroda, Tatsuo Fujino, Akihiro Biomolecules Article No standard diagnostic method or surgical treatment for congenital isolated hypoganglionosis (CIHG) has been established. This study aimed to analyze the clinical outcomes of patients with CIHG and identify the best surgical interventions provided thus far. Data on surgical interventions in 19 patients were collected between 1992 and 2020, including the type of enterostomy, type of revision, and length of the intestines. Ganglion cells in the myenteric plexus were enumerated using Hu C/D staining. The ratio of the length of the small intestine to its height was defined as the intestinal ratio (IR). The outcomes were assessed using the stoma output, growth parameters including the body mass index (BMI), and parenteral nutrition (PN) dependency. All patients required a diverting enterostomy. The IR ranged from 0.51 to 1.75 after multiple non-transplant surgeries. The stoma types were tube-stoma, end-stoma, Santulli-type, and Bishop–Koop (BK)-type. Patients with Santulli- or BK-type stomas had better BMIs and less PN dependency in terms of volume than those with end-stomas or tube-stomas. Two patients with BK-type stomas were off PN, and three who underwent an intestinal transplantation (Itx) achieved enteral autonomy. The management of CIHG involves a precise diagnosis using Hu C/D staining, neonatal enterostomy, and stoma revision using the adjusted IR and Itx if other treatments do not enable enteral autonomy. MDPI 2023-10-23 /pmc/articles/PMC10605557/ /pubmed/37892242 http://dx.doi.org/10.3390/biom13101560 Text en © 2023 by the authors. https://creativecommons.org/licenses/by/4.0/Licensee MDPI, Basel, Switzerland. This article is an open access article distributed under the terms and conditions of the Creative Commons Attribution (CC BY) license (https://creativecommons.org/licenses/by/4.0/).
spellingShingle Article
Yamada, Yohei
Mori, Teizaburo
Takahashi, Nobuhiro
Fujimura, Takumi
Kano, Motohiro
Kato, Mototoshi
Takahashi, Masataka
Shimojima, Naoki
Watanabe, Toshihiko
Yoshioka, Takako
Kanamori, Yutaka
Kuroda, Tatsuo
Fujino, Akihiro
Historical Cohort Study of Congenital Isolated Hypoganglionosis of the Intestine: Determining the Best Surgical Interventions
title Historical Cohort Study of Congenital Isolated Hypoganglionosis of the Intestine: Determining the Best Surgical Interventions
title_full Historical Cohort Study of Congenital Isolated Hypoganglionosis of the Intestine: Determining the Best Surgical Interventions
title_fullStr Historical Cohort Study of Congenital Isolated Hypoganglionosis of the Intestine: Determining the Best Surgical Interventions
title_full_unstemmed Historical Cohort Study of Congenital Isolated Hypoganglionosis of the Intestine: Determining the Best Surgical Interventions
title_short Historical Cohort Study of Congenital Isolated Hypoganglionosis of the Intestine: Determining the Best Surgical Interventions
title_sort historical cohort study of congenital isolated hypoganglionosis of the intestine: determining the best surgical interventions
topic Article
url https://www.ncbi.nlm.nih.gov/pmc/articles/PMC10605557/
https://www.ncbi.nlm.nih.gov/pubmed/37892242
http://dx.doi.org/10.3390/biom13101560
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