Results 11 to 20 of about 5,922 (184)
Clinical Case ReportsWith reported occurrences ranging from 1% to 20%, total colonic Hirschsprung disease (TCHD) accounts for about 8% of Hirschsprung disease cases. The selection and sequencing of the surgical approach, access to parenteral nutrition, and collaborative care
Ali Khaksour +2 more
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Seminars in Pediatric Surgery, 2010 Hirschsprung's disease (HSCR) is characterized by absence of the enteric nervous system in a variable portion of the distal gut. Affected infants usually present in the days after birth with bowel obstruction. Despite surgical advances, long-term outcomes remain variable.
GarciaBarcelo, M, Tam, PKH, Kenny, SE
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Multiple Roles of Ret Signalling During Enteric Neurogenesis
Frontiers in Molecular Neuroscience, 2022 The majority of the enteric nervous system is formed by vagal neural crest cells which enter the foregut and migrate rostrocaudally to colonise the entire length of the gastrointestinal tract.
Dipa Natarajan +9 more
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Hirschsprung Disease in an Infant with L1 syndrome: Report of a New Case and a novel L1CAM variant
Clinical Case Reports, 2021 L1syndrome is an X‐linked disorder manifesting with congenital hydrocephalus, adducted thumbs and spasticity. There are rare cases of L1 syndrome and coincident Hirschsprung disease, with mutations in the L1CAM gene thought to underlie both. We present a
Timothy D. Gauntner +7 more
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Radiology Perspective One-Year Study of Hirschsprung Disease
Folia Medica Indonesiana, 2021 Hirschsprung disease is a rare developmental disorder of the enteric nervous system. This researched purpose to give an epidemiological database and current trend analysis from radiological perspective of Hirschsprung disease at our center. A prospective
Anastasia Tjan
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Clinics, 2012 Hirschsprung disease is a congenital form of aganglionic megacolon that results from cristopathy. Hirschsprung disease usually occurs as a sporadic disease, although it may be associated with several inherited conditions, such as multiple endocrine ...
Elisangela P. S. Quedas +6 more
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Journal of Pediatric Surgery Case Reports, 2022 Hirschsprung disease is associated with significant bacterial proliferation and colonic dysbiosis leading to life threating hyperammonemia. In this report, we highlight the use of carglumic acid; a synthetic ammonia scavenger in hyperammonemia secondary ...
Tala Shalakhti +6 more
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National Journal of Physiology, Pharmacy and Pharmacology, 2017 Background: Hirschsprungs disease and other gut malformations commonly present with obstructive features of gut in pediatric age group. Problem of obstruction persists even after resection and anastomosis operation.
Anil Kumar Tiwari +6 more
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Ileal atresia and total colonic hirschsprung disease in a 36‐week neonate: A case report
Clinical Case Reports, 2023 Intestinal atresia and hirschsprung disease are two common causes of bowel obstruction in neonates; simultaneous occurrence is rare. This report delineates a 36‐week newborn with ileal atresia and total colonic hirschsprung who was referred to our unit ...
Khashahyar Atqiaee +3 more
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Fatal Toxic Megacolon in a Child of Hirschsprung Disease [PDF]
Journal of Clinical and Diagnostic Research, 2016 Hirschsprung disease (HD) in late childhood is uncommon and often undiagnosed or misdiagnosed. However, in a patient with Hirschsprung disease, of greater significance is the occurrence of life threatening enterocolitis. In its more severe form, this is
Shiwani R Garg +3 more
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