Amirou M, Bourdat-Michel G, Pinel N, Huet G, Gaultier J, Cochat P
Service de Néphrologie, Centre Hospitalier Général, Gap, France.
Pediatr Nephrol. 1998 May;12(4):293-4. doi: 10.1007/s004670050456.
Jeune syndrome (asphyxiating thoracic dystrophy) is a rare inherited disease which is fatal in early childhood in 70% of cases. Severe renal involvement may occur and lead to chronic renal insufficiency in patients who survive respiratory failure. Therefore the opportunity to perform kidney transplantation is quite rare. We report a successful cadaver renal transplantation in a 10-year-old boy with Jeune syndrome type 2.
儒内综合征（窒息性胸廓发育不良）是一种罕见的遗传性疾病，70%的病例在儿童早期会致命。严重的肾脏受累可能发生，并导致呼吸衰竭存活患者出现慢性肾功能不全。因此，进行肾脏移植的机会相当罕见。我们报告了一例成功的尸体肾移植病例，受者是一名患有2型儒内综合征的10岁男孩。
