Historical Cohort Study of Congenital Isolated Hypoganglionosis of the Intestine: Determining the Best Surgical Interventions
Rattachement africain : jp. Niveau de preuve : code pays fourni par la source.
Le résumé fourni par la source
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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Le contrôle bibliographique ouvert
DOI retrouvé dans Crossref DOI retrouvé ; titre concordant.
- Titre Crossref
- Historical Cohort Study of Congenital Isolated Hypoganglionosis of the Intestine: Determining the Best Surgical Interventions
- Date Crossref
- 23/10/2023
- Éditeur
- MDPI AG
- Type
- journal-article
Ce recoupement confirme des métadonnées liées au DOI. Il ne confirme ni la méthode ni les conclusions de l’étude, et il ne compte pas comme une seconde source scientifique indépendante.
Où se fait cette recherche
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Keio University Department of Pediatric Surgery pays non établi dans la noticeUniversité ou école supérieure
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National Center For Child Health and Development pays non établi dans la noticeOrganisme public
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National Hospital Organization Department of Pediatric Surgery pays non établi dans la noticeOrganisme public
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Tokyo Metropolitan Children's Medical Center Department of Surgery pays non établi dans la noticeÉtablissement de santé
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Tokai University Department of Pediatric Surgery pays non établi dans la noticeUniversité ou école supérieure
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Kanagawa Children's Medical Center pays non établi dans la noticeÉtablissement de santé
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Department of Pediatric Surgery pays non établi dans la noticeInstitution
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Department of Pathology pays non établi dans la noticeInstitution
Department of Pediatric Surgery — Keio University, National Center For Child Health and Development et Department of Pediatric Surgery — National Hospital Organization, avec 5 autres affiliations.
Une affiliation ne permet pas de déduire la nationalité d’un auteur.