Outcome of Children With Intestinal Failure Due to Waardenburg Syndrome From an Intestinal Transplant Center: A Case Series
EXPERIMENTAL AND CLINICAL TRANSPLANTATION, cilt.23, sa.7, ss.506-508, 2025 (SCI-Expanded, Scopus)
- Yayın Türü: Makale / Tam Makale
- Cilt numarası: 23 Sayı: 7
- Basım Tarihi: 2025
- Doi Numarası: 10.6002/ect.2020.0166
- Dergi Adı: EXPERIMENTAL AND CLINICAL TRANSPLANTATION
- Derginin Tarandığı İndeksler: Science Citation Index Expanded (SCI-EXPANDED), Scopus, EMBASE, MEDLINE
- Sayfa Sayıları: ss.506-508
- Anahtar Kelimeler: Intestinal rehabilitation, Shah-Waardenburg syndrome, Small bowel transplant
- Dokuz Eylül Üniversitesi Adresli: Hayır
Özet
Waardenburg syndrome is a genetic disease char-acterized by hearing loss and pigmentation abnor-malities. Waardenburg syndrome type 4 is very rare, and children with Waardenburg syndrome type 4 present with intestinal aganglionosis. The associated findings and severity of Waardenburg syndrome type 4 may also differ significantly between cases. Intestinal insufficiency is probable and creates difficulties in terms of treatment; intestinal transplant may be required. In this case report, we present 4 cases of patients with Waardenburg syndrome who have intestinal issues, 2 of whom underwent small bowel transplant. Appropriate surgical and nutritional management should be provided for patients with Waardenburg syndrome type 4 who have gastro-intestinal manifestations.