Research Article
Risk Factors for Hirschsprung Disease
Author(s): Melanie Kapapaa* MD, MA; Stella Freinb, Alexandre Serraa MD, PhD
aDivision of Pediatric Surgery, Department of Surgery, University Medical Centre Ulm, Eythstrasse 24, 89075 Ulm, Germany
bClinical Centre Worms, Centre for Trauma Surgery, Orthopedics and Hand Surgery, Gabriel-von-Seidl-Straße 81, 67550 Worms
*Corresponding author: Melanie Kapapa, MD, MA, Division of Pediatric Surgery, Department of Surgery, University Medical Centre Ulm, Eythstrasse 24, 89075 Ulm, Germany; Tel: +49 731 500-53519, FAX: +49 731 500-53703. E-Mail: melanie.kapapa@uniklinik-ulm.de;Co-authors e-mails: Stella_Racz@hotmail.com (SF); alexandre.serra@uniklinik-ulm.de (AS).
Received Date: 12th July, 2022
Accepted Date: 18th July, 2022
Published Date: 25th July, 2022
Citation: Kapapa M, Frein S, Serra A (2022) Risk Factors for Hirschsprung Disease. Eur J Pedia Neon: EJPN-101. DOI: 10.47378/EJPN/2022.1.101.
Abstract
Background: Hirschsprung’s disease (HSCR) is a congenital disorder with an approximate incidence of 1:5.000 live births, usually also associated to trisomy 21. Clinically, HSCR manifests with ileus due to a functional intestinal stenosis caused by the lack of intestinal innervation in the affected segments of the gut, usually beginning between the 5th and 12th week of embryonic development. The aim of the current research was to assess familial and environmental risk factors which could be associated to the development of HSCR, particularly during the first trimester of pregnancy.
Methods: The families from HSCR patients were interviewed through specialized questionnaires aiming to evaluate data on medical and pregnancy history, including changing of habits (nutrition, diet, medications), drug consumption (alcohol, smoking, illicit drugs), maternal physiological background and preexisting diseases or medical/surgical therapies. The study consisted thus of a retrospective, observational control group-supported examination.
Results: HSCR mothers had a significantly higher baseline weight before the start of pregnancy (p=0.045) with a higher number of live-born children (p=0.036), while HSCR fathers were significantly younger than control group (CG) fathers (p=0.03). HSCR mothers had unhealthier eating habits and a higher self- (p=0.012) or partner-nicotine consumption measured as number of cigarettes/day (p=0.042), which were also not reduced during pregnancy. Interestingly, HSCR mothers had a significantly lower medication intake when compared to CG mothers (p=0.04).
Conclusion: The key to reducing maternal risk factors for HSCR is in our opinion a consistent counselling of expectant mothers before and during pregnancy. Parental lifestyle parameters which may predispose to the occurrence of HSCR such as maternal obesity, smoking and unhealthy eating habits as well as vitamin deficiencies can be influenced and need to be optimised during pregnancy, since other known risk-factors cannot be altered (such as paternal age).
Keywords: Hirschsprung disease, risk factors, pregnancy, age, nutrition.
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