Background: Hirschsprung's disease (HD) is widely quoted to have an incidence of approximately 1 in 5000 live births. However, it is also known that regional variation in incidence can occur. The aim of this paper was to study the incidence of Hirschsprung's disease in Tasmania and to document a regional experience of HD.
Methods: A retrospective review of all patients with newly diagnosed HD between January 1998 and December 2005 was carried out. Data were extracted from medical records and reported. Tasmanian birth statistics from 1998 to 2005 were obtained from the Australian Bureau of Statistics website and the incidence was calculated.
Results: Over the 8-year study period, 14 new cases of HD were identified in Tasmania. The estimated incidence of HD in Tasmania based on this study is 1 in 3429. This incidence is higher than the widely quoted incidence of 1 in 5000, but the difference did not reach statistical significance. Between 2003 and 2005, there was a surge in the number of cases with 11 of 14 cases being derived from these 3 years. No obvious reasons were found to explain this surge in the number of cases. Abdominal distension and vomiting were most common modes of presentation (100 and 71%, respectively). There was delayed passage of meconium in 43%.
Conclusion: The incidence of Hirschsprung's disease seems higher in Tasmania. The surge in the number of cases is also of interest, although the reasons behind this remain unknown. Delayed passage of meconium was also less common than previously cited.
Download full-text PDF |
Source |
|---|---|
| http://dx.doi.org/10.1111/j.1445-2197.2008.04724.x | DOI Listing |
Publication Analysis
Top Keywords
Similar Publications
Colonic Derotation Revisited: The "Deloyers Procedure" for Long-Segment Hirschsprung Disease.
Cureus
December 2024
Comprehensive Colorectal Center, Department of Surgery, Children's Mercy Kansas City, Kansas City, USA.
Long-segment Hirschsprung disease (HSCR) presents significant challenges in surgical management, often requiring extensive bowel mobilization and creative techniques to achieve tension-free anastomosis. Colonic derotation offers a viable solution for preserving bowel length and maintaining the ileocecal valve, which is crucial for postoperative bowel function. The procedure involves extensive colonic mobilization and strategic vascular divisions of the right and middle colic vessels while preserving the ileocolic and marginal arteries, followed by a 180° counterclockwise rotation of the colon around the ileocolic vascular axis.
View Article and Find Full Text PDFUtility of Monitoring Fasting Phase During Pediatric Colonic Manometry Studies.
Neurogastroenterol Motil
January 2025
Gastroenterology, Hepatology and Nutrition, Cincinnati Children's Hospital Medical Center, Cincinnati, Ohio, USA.
Introduction: Colonic manometry (CM) is a diagnostic procedure used to evaluate pediatric patients with refractory constipation, fecal incontinence, Hirschsprung disease, and pediatric intestinal pseudo-obstruction. Pan-colonic high-amplitude propagated contractions (HAPCs), measured by CM, reflect an intact neuromuscular function of the colon. Current guidelines recommend starting CM with fasting recording for 1-2 h, but no prior evaluation has determined the diagnostic yield of the fasting phase.
View Article and Find Full Text PDFFrozen Section Doughnuts Obtained with a 5 mm Stapling Device Improve Outcomes in Laparoscopic Endorectal Pull-Throughs for Hirschsprung's Disease.
J Laparoendosc Adv Surg Tech A
January 2025
The Department of Paediatric Surgery, The Royal Hospital for Children Glasgow, Glasgow, United Kingdom.
A primary pull-through for Hirschsprung's disease (HD) requires confirmation of normal ganglionic bowel by intraoperative biopsies to determine the level of resection. Despite this, aganglionic bowel that is not fully resected (so-called "transition zone pull-throughs") is reported in 15%-19% of patients. We hypothesize that this may result from insufficient biopsies sent for intraoperative diagnosis.
View Article and Find Full Text PDFTwo Different Brain Injury Patterns Associated with Compound Heterozygosis of the PIGO Gene in a Term Newborn: A Case Report.
Biomedicines
December 2024
Diagnostic and Interventional Neuroradiology Unit, Bambino Gesù Children's Hospital, IRCCS, 00165 Rome, Italy.
The glycosylphosphatidylinositol (GPI) is a glycol-lipid that anchors several proteins to the cell surface. The GPI-anchor pathway is crucial for the correct function of proteins involved in cell function, and it is fundamental in early neurogenesis and neural development. The PIG gene family is a group of genes involved in this pathway with six genes identified so far, and defects in these genes are associated with a rare inborn metabolic disorder manifesting with a spectrum of clinical phenotypes in newborns and children.
View Article and Find Full Text PDFWhole-genome sequencing identified ALK as a novel susceptible gene of Hirschsprung disease.
Arab J Gastroenterol
January 2025
Department of Pediatric Surgery, Tongji Medical College, Union Hospital, Huazhong University of Science and Technology, Wuhan 430015, China.
Background And Study Aims: Hirschsprung disease (HD) is a complex developmental disease that resulted from impaired proliferation and migration of neural crest cells. Despite the genetic causation of enteric nervous system have been found to be responsible for part of HD cases, the genetic aetiology of most HD patients still needs to be explored.
Patients And Methods: Whole-genome sequencing and subsequent Sanger sequencing validation analysis were performed in 13 HD children and their unaffected parents.
Want AI Summaries of new PubMed Abstracts delivered to your In-box?
Enter search terms and have AI summaries delivered each week - change queries or unsubscribe any time!