Results 141 to 150 of about 5,824 (184)

Genetics of Hirschsprung disease

Current Opinion in Pediatrics, 2000
Hirschsprung disease (HSCR), or congenital intestinal aganglionosis, is a relatively common disorder of neural crest migration. It has a strong genetic basis, although simple Mendelian inheritance is rarely observed. Hirschsprung disease is associated with several other anomalies and syndromes, and animal models for these conditions exist. Mutations in
M A, Parisi, R P, Kapur
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Hirschsprung's disease in Oman

Journal of Pediatric Surgery, 1997
The incidence of Hirschsprung's disease (HD) was studied retrospectively in Oman using hospital-based data. In Oman there is a single pediatric surgery unit where a register has been kept from 1989 to 1994, and because all cases are referred to this unit, a national survey could be carried out. There were 85 children with HD born between 1989 and 1994,
A, Rajab, N V, Freeman, M A, Patton
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Reoperation for Hirschsprung's disease

Journal of Pediatric Surgery, 1999
Reoperation for Hirschsprung's disease traditionally has been used for patients with anastomotic leaks or stricture or with severe constipation from retained aganglionic segment or neuronal dysplasia, but there is little information regarding its use for other complications and the long-term outcome in these patients.In a 23-year period, 107 infants ...
T R, Weber, R S, Fortuna, M L, Silen, P A, Dillon   +3 more
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The genetics of Hirschsprung disease

Gastroenterology Clinics of North America, 2003
Understanding the genetics of Hirschsprung disease will naturally expand our understanding of other neurocristopathies, the enteric nervous system, and autonomic system biology. As other disorders of gastrointestinal motility are investigated, genetics may resolve certain clinical questions.
Douglas R, Stewart, Daniel, von Allmen
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Hirschsprung's Disease in the Newborn

Archives of Surgery, 1959
Hirschsprung's disease is one of the more frequent causes of intestinal obstruction in the newborn. Improvements in the methods of diagnosis and management of patients in this group have increased the survival rate. The 3-day-old infant in Figure 1 has the signs of acute intestinal obstruction.
O, SWENSON, J H, FISHER
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Hirschsprung?s disease as a neurochristopathy

Pediatric Surgery International, 1996
Recent molecular-genetic and histochemical studies of intestinal aganglionosis have confirmed the initial classification established by Bolande, who considered Hirschsprung's disease (HD) a neurocristopathy. This paper is a critical review of the results of molecular-genetic studies carried out from 1992 to date.
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Variant Hirschsprung's disease

Journal of Pediatric Surgery, 1997
There are many clinical conditions that resemble Hirschsprung's disease despite the presence of ganglia cells on rectal biopsy. This group has focused its research interest into delineating variant Hirschsprung's disease based on specific histochemical, immunohistochemical, silver staining and electron microscopic studies.
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Hirschsprung Disease

Pediatrics In Review, 2021
Mark, Mahon, Julie, Khlevner
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Hirschsprung's Disease in Infants

Archives of Surgery, 1959
Introduction Until recently, the clinical course of aganglionic megacolon was considered to be of a chronic and protracted nature with obstipation as the presenting symptom. In the past few years, however, several excellent papers have appeared by Dorman 1 and Sieber and Girdany 2 in which the severe and fulminating course of symptomatic Hirschsprung ...
T C, JEWETT, L J, LEAHY, J, LANIGAN
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Hirschsprung's disease?a review

International Journal of Colorectal Disease, 1991
Hirschsprung's disease is not the easiest of diseases to diagnose and there is a range of similar diseases which merge into the problem. The lack of knowledge as to its aetiology means that prevention is not possible. The wide range of treatments suggests that none of them give ideal results every time, although they each have their adherents.
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