Kuloğlu Zarife, Ustündağ Gonca, Kirsaçlioğlu Ceyda Tuna, Kansu Aydan, Bingol-Kologlu Meltem, Vargun Rahsan, Hazinedaroğlu Selcuk, Karayalçin Selim, Girgin Nurten
Department of Pediatric Gastroenterology, School of Medicine, Ankara University, Ankara, Turkey.
Pediatr Transplant. 2008 Dec;12(8):906-9. doi: 10.1111/j.1399-3046.2008.00955.x. Epub 2008 May 23.
An 11-yr-old boy with familial YNS and FHF and who underwent LRLT is presented. LRLT was performed from his father with YNS. The findings of hepatic failure resolved immediately after LRLT, but severe respiratory complications and chylous ascites were observed during the follow-up. At 12 months after successful LT, the patient has good graft function, but findings of YNS including chronic cough, lymphedema and yellow nails are still present. To the best of our knowledge, this is the first case of YNS who underwent LRLT for FHF.
本文报告了一名11岁患有家族性黄甲综合征（YNS）和暴发性肝衰竭（FHF）并接受活体右肝移植（LRLT）的男孩。LRLT供体为其患有YNS的父亲。肝衰竭症状在LRLT后立即得到缓解，但随访期间观察到严重的呼吸系统并发症和乳糜性腹水。肝移植成功后12个月，患者移植物功能良好，但YNS的表现如慢性咳嗽、淋巴水肿和黄甲仍存在。据我们所知，这是首例因FHF接受LRLT的YNS患者。
