Cystic fibrosis and renal disease: a case report.
Cystic fibrosis and renal disease: a case report.
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DOI：
10.1186/1752-1947-1-24
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发表时间：
2007-06-04
影响因子：
1
通讯作者：
Rao, Aparna R
中科院分区：
文献类型：
--
作者：
Al-Shawwa, Baha A;Rao, Aparna R
背景技术背景：囊性纤维化（CF）是一种常染色体隐性遗传疾病，主要见于高加索人群，涉及多个器官。传统上，它一直被认为是肾脏是唯一的器官，似乎并不普遍受到疾病的影响，虽然囊性纤维化跨膜传导调节（CFTR）基因在kidney.Case介绍：我们报告的情况下，一个11岁的男孩囊性纤维化和肾病综合征，并回顾文献，描述肾病综合征和肾脏参与囊性纤维化。随着囊性纤维化管理的持续进步和预期寿命的提高，预计将出现几种未被识别的合并症。对患者进行可能的合并症筛查很重要。尿液分析可能对这组患者有帮助，任何蛋白尿都应引起囊性纤维化相关肾病的怀疑。
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.