Hirschsprung disease is the most common neurocristopathy in children, resulting in the congenital loss of enteric ganglia. Rare reports of skip lesions have previously been reported in the literature. We present a case of skip lesions known prior to surgery and managed by pull-through of the right colon that allowed the preservation of the colon.
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Impact of a New Preoperative Immune-Nutrition Protocol Using Zinc on Hospital Outcomes of Children with Hirschsprung's Disease: A Novel Randomized Controlled Trial.
Pediatr Gastroenterol Hepatol Nutr
January 2025
Department of Pediatric Surgery, Faculty of Medicine, Cairo University, Cairo, Egypt.
Purpose: Research proved the beneficial effect of Zinc on human health and Gastrointestinal tract inflammatory diseases. We propose that zinc would be of value in children with Hirschsprung's disease (HD) undergoing elective pull-through surgery. This study was carried out to determine the influence of preoperative zinc intake on postoperative outcomes, especially the hospital length of stay in patients diagnosed with HD as a primary outcome.
View Article and Find Full Text PDFNonylphenol exposure increases the risk of Hirschsprung's disease by inducing macrophage M1 polarization.
Ecotoxicol Environ Saf
January 2025
Department of Pediatric Surgery, Affiliated Hospital of Zunyi Medical University, Zunyi, Guizhou 563000, China; Department of Pediatric Surgery, Guizhou Children Hospital, Zunyi, Guizhou 563000, China. Electronic address:
Nonylphenol (NP), a ubiquitous environmental contaminant used as a surfactant in industrial production and classified as an endocrine disruptor, could interfere hormone secretion and exhibit neurotoxicity in organisms. Hirschsprung's disease (HSCR), one of the most frequently observed congenital malformations of the digestive system, arises mainly due to the failure of enteric neural crest cells to migrate to the distal colon during embryonic development. However, the effects of NP exposure on HSCR are largely unknown.
View Article and Find Full Text PDFBackground: Hirschsprung disease (HSCR) is a rare neurodevelopmental disorder caused by disrupted migration and proliferation of enteric neural crest cells during enteric nervous system development. Genetic studies suggest a complex etiology involving both rare and common variants, but the contribution of ultra-rare pathogenic variants (PAs) remains poorly understood.
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J Pediatr Surg
January 2025
Division of Pediatric Surgery, Department of Surgery, Stanford University, Palo Alto CA, USA. Electronic address:
Background: We aimed to analyze the effect of age and weight on 30-day outcomes of single-stage endorectal pull tthrough (ERPT) procedures for Hirschsprung's Disease (HD) using the National Surgical Quality Improvement Program-Pediatric (NSQIP-P) database to identify an optimal time for surgery.
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Rare and common genetic variants underlying the risk of Hirschsprung's disease.
Hum Mol Genet
January 2025
Department of Pediatric Surgery, Tongji Hospital, Tongji Medical College, Huazhong University of Science and Technology, No. 1095 Jiefang Avenue, Qiaokou District, Wuhan, Hubei 430030, China.
Hirschsprung's disease (HSCR) is a congenital enteric neuropathic disorder characterized by high heritability (>80%) and polygenic inheritance (>20 genes). The previous genome-wide association studies (GWAS) identified several common variants associated with HSCR and demonstrated increased predictive performance for HSCR risk in Europeans using a genetic risk score, there remains a notable gap in knowledge regarding Chinese populations. We conducted whole exome sequencing in a HSCR case cohort in Chinese.
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