囊性纤维化与肾脏疾病：一例病例报告。
Cystic fibrosis and renal disease: a case report.
作者信息
Al-Shawwa Baha A, Rao Aparna R
机构信息
Department of Pediatrics, Medical College of Wisconsin (Pulmonary Section), Children's Hospital of Wisconsin, 9000 West Wisconsin Avenue, MS # B620, Milwaukee, WI 53226, USA.
出版信息
J Med Case Rep. 2007 Jun 4;1:24. doi: 10.1186/1752-1947-1-24.
BACKGROUND
Cystic fibrosis (CF) is an autosomal recessive disease that is predominantly seen in the Caucasian population and involves multiple organs. Traditionally it has been thought that the kidney is the only organ which does not seem to be generally affected by the disease although the cystic fibrosis transmembrane conductance regulator (CFTR) gene is expressed in the kidney.
CASE PRESENTATION
We report the case of an 11 year old boy with cystic fibrosis and nephrotic syndrome and review the literature that describes nephrotic syndrome and renal involvement in cystic fibrosis.
CONCLUSION
With continued advances in the management of cystic fibrosis and improvement in life expectancy, several unrecognized co-morbidities are expected to emerge. It is important to screen patients for possible co-morbidities. Urine analysis may be helpful in this group of patients and any proteinuria should raise the suspicion of cystic fibrosis-related renal disease.
背景
囊性纤维化（CF）是一种常染色体隐性疾病，主要见于白种人群体，累及多个器官。传统上认为，尽管囊性纤维化跨膜传导调节因子（CFTR）基因在肾脏中表达，但肾脏似乎是唯一一般不受该疾病影响的器官。
病例报告
我们报告了一名患有囊性纤维化和肾病综合征的11岁男孩的病例，并回顾了描述囊性纤维化中肾病综合征和肾脏受累情况的文献。
结论
随着囊性纤维化治疗的不断进步以及预期寿命的提高，预计会出现一些未被认识到的合并症。对患者进行可能合并症的筛查很重要。尿液分析可能对这组患者有帮助，任何蛋白尿都应引起对囊性纤维化相关肾脏疾病的怀疑。
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