Hirschsprung disease

L Montalva, LS Cheng, R Kapur, JC Langer… - Nature Reviews …, 2023 - nature.com
Hirschsprung disease (HSCR) is a rare congenital intestinal disease that occurs in 1 in
5,000 live births. HSCR is characterized by the absence of ganglion cells in the myenteric …

ERNICA guidelines for the management of rectosigmoid Hirschsprung's disease

K Kyrklund, CEJ Sloots, I de Blaauw… - Orphanet journal of rare …, 2020 - Springer
Background Hirschsprung's disease (HSCR) is a serious congenital bowel disorder with a
prevalence of 1/5000. Currently, there is a lack of systematically developed guidelines to …

Enteric nervous system development: what could possibly go wrong?

M Rao, MD Gershon - Nature Reviews Neuroscience, 2018 - nature.com
The gastrointestinal tract contains its own set of intrinsic neuroglial circuits—the enteric
nervous system (ENS)—which detects and responds to diverse signals from the …

Diagnosis of Hirschsprung disease

L Ambartsumyan, C Smith… - Pediatric and …, 2020 - journals.sagepub.com
Diagnosis or exclusion of Hirschsprung disease (HSCR) is a frequent exercise in any
pediatric hospital. Although HSCR may present at different ages and with varied clinical …

Interstitial cells of Cajal and human colon motility in health and disease

JD Huizinga, A Hussain… - American Journal of …, 2021 - journals.physiology.org
Our understanding of human colonic motility, and autonomic reflexes that generate motor
patterns, has increased markedly through high-resolution manometry. Details of the motor …

Current understanding of Hirschsprung-associated enterocolitis: Pathogenesis, diagnosis and treatment

RA Lewit, KP Kuruvilla, M Fu, A Gosain - Seminars in pediatric surgery, 2022 - Elsevier
Hirschsprung-associated enterocolitis (HAEC) was described in 1886 by Harald
Hirschsprung and is a potentially deadly complication of Hirschsprung Disease. HAEC is …

Evaluation and management of postsurgical patient with Hirschsprung disease Neurogastroenterology & Motility Committee: position paper of North American Society …

L Ambartsumyan, D Patel, P Kapavarapu… - Journal of Pediatric …, 2023 - journals.lww.com
Children with Hirschsprung disease have postoperative long-term sequelae in defecation
that contribute to morbidity and mortality and significantly impact their quality of life. Pediatric …

Surgery, surgical pathology, and postoperative management of patients with Hirschsprung disease

C Smith, L Ambartsumyan… - Pediatric and …, 2020 - journals.sagepub.com
Endorectal pullthrough surgery is integral in the treatment of patients with Hirschsprung
disease. Several different surgical procedures exist, which share as common goals to excise …

Evaluation and treatment of the post pull-through Hirschsprung patient who is not doing well; update for 2022

H Ahmad, D Yacob, DR Halleran, AC Gasior… - Seminars in pediatric …, 2022 - Elsevier
After operative intervention for Hirschsprung disease (HD) a child should thrive, be fecally
continent, and avoid recurrent episodes of abdominal distention and enterocolitis. This is …

Guidelines for the management of postoperative soiling in children with Hirschsprung disease

P Saadai, AF Trappey, AM Goldstein… - Pediatric Surgery …, 2019 - Springer
Although most children with Hirschsprung disease ultimately achieve functional and
comfortable stooling, some will experience a variety of problems after pull-through surgery …