Cystic fibrosis and renal disease: a case report

Baha A Al-Shawwa and Aparna R Rao

Department of Pediatrics, Medical College of Wisconsin (Pulmonary Section), Children's Hospital of Wisconsin, 9000 West Wisconsin Avenue, MS # B620, Milwaukee, WI 53226, USA

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Journal of Medical Case Reports 2007, 1:24doi:10.1186/1752-1947-1-24


Published:	4 June 2007

Abstract

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.