Near-total intestinal aganglionosis in the Waardenburg-Shah syndrome
| Autor: | Marta Derieg, Berkley R Powell, Y. Edward Hsia, Walton K.T. Shim |
|---|---|
| Rok vydání: | 1999 |
| Předmět: |
medicine.medical_specialty
Pathology Waardenburg syndrome business.industry Infant Newborn Neural crest General Medicine medicine.disease Long segment Dermatology Fatal Outcome Recien nacido Near-total intestinal aganglionosis Pediatrics Perinatology and Child Health medicine Waardenburg Shah syndrome Humans Female Waardenburg Syndrome Surgery Hirschsprung Disease Congenital disease business Colonic disease |
| Zdroj: | Journal of Pediatric Surgery. 34:1853-1855 |
| ISSN: | 0022-3468 |
| DOI: | 10.1016/s0022-3468(99)90330-5 |
| Popis: | Both pigmentation and otic defects of Waardenburg Syndrome and Hirschsprung's disease have a common origin in neural crest cells and were described in 1951 and 1887, respectively. The clinical manifestations of both in the same patient were described in 1981 in 12 infants so afflicted. The authors present such a case of long segment aganglionosis in a 15-day-old Marshallese girl with Waardenburg-Shah syndrome and discuss diagnosis, treatment, and prognosis. |
| Databáze: | OpenAIRE |
| Externí odkaz: |
|
Full Text from ScienceDirect