Zellers T M, Brown K
Department of Pediatrics, University of Texas Southwestern Medical Center at Dallas 75235, USA.
Pediatr Cardiol. 1996 Mar-Apr;17(2):115-7. doi: 10.1007/BF02505095.
Protein-losing enteropathy occurs in up to 10% of patients following the modified Fontan procedure. Treatment is still controversial. We describe a female adolescent who developed protein-losing enteropathy 4 years after a modified Fontan procedure. Treatment with oral prednisone attenuated the protein loss with subsequent normalization of her serum total protein and albumin levels. Discontinuation of prednisone therapy was associated with relapse, which was again treated successfully with low-dose oral prednisone. Small bowel biopsy-proved diagnosis with improvement, relapse, and improvement again are documented, as are other useful laboratory findings.
在改良Fontan手术后，高达10%的患者会发生蛋白丢失性肠病。治疗方法仍存在争议。我们描述了一名女性青少年，她在改良Fontan手术后4年出现了蛋白丢失性肠病。口服泼尼松治疗减轻了蛋白丢失，随后她的血清总蛋白和白蛋白水平恢复正常。停用泼尼松治疗后病情复发，再次使用小剂量口服泼尼松成功治疗。记录了小肠活检确诊、病情改善、复发和再次改善的情况，以及其他有用的实验室检查结果。
