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Literature DB >> 12811477

Long segment Hirschsprung's disease in the Waardenburg-Shah syndrome.

E J Gnananayagam¹, R Solomon, A Chandran, S Anbarasi, S Sen, P D Moses.

Abstract

Presentation with signs of bowel obstruction in an infant with Waardenburg syndrome should raise the suspicion of aganglionosis. We report such a case of long segment Hirschsprung's disease associated with Waardenburg syndrome. Long term care of such children is fraught with high morbidity and mortality.

Entities: Disease Species

Mesh：

Year: 2003 PMID： 12811477 DOI： 10.1007/s00383-002-0944-7

Source DB: PubMed Journal: Pediatr Surg Int ISSN： 0179-0358 Impact factor: 1.827

Keyword Cloud
References

12 in total

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Authors: L M Ambani
Journal: J Pediatr Date: 1983-05 Impact factor: 4.406

6. White forelock, pigmentary disorder of irides, and long segment Hirschsprung disease: possible variant of Waardenburg syndrome.

Authors: K N Shah; S J Dalal; M P Desai; P N Sheth; N C Joshi; L M Ambani
Journal: J Pediatr Date: 1981-09 Impact factor: 4.406

Long segment Hirschsprung's disease in the Waardenburg-Shah syndrome.

1. A new syndrome combining developmental anomalies of the eyelids, eyebrows and nose root with pigmentary defects of the iris and head hair and with congenital deafness.

2. Near-total intestinal aganglionosis in the Waardenburg-Shah syndrome.

3. Picture of the month. White forelock of hair in a newborn.

4. Waardenburg's syndrome associated with total aganglionosis.

5. Waardenburg and Hirschsprung syndromes.

6. White forelock, pigmentary disorder of irides, and long segment Hirschsprung disease: possible variant of Waardenburg syndrome.

7. Extensive aganglionosis: further experience with the colonic patch graft procedure and long-term results.

8. Total intestinal aganglionosis: a new technique for prolonged survival.

9. Sox10 mutation disrupts neural crest development in Dom Hirschsprung mouse model.

10. The mutational spectrum in Waardenburg syndrome.