Clinical and Experimental Pediatrics

The Korean Pediatric Society (대한소아청소년과학회)
권호 표지
DOI QR코드

DOI QR Code

Cystic fibrosis of pancreas and nephrotic syndrome: a rare association

  • Kelekci, Selvi (Department of Pediatric Pulmonology, Dicle University Faculty of Medicine) ;
  • Karabel, Musemma (Department of Pediatric Pulmonology, Dicle University Faculty of Medicine) ;
  • Ece, Aydin (Department of Pediatric Nephrology, Dicle University Faculty of Medicine) ;
  • Sen, Velat (Department of Pediatric Pulmonology, Dicle University Faculty of Medicine) ;
  • Gunes, Ali (Department of Pediatrics, Dicle University Faculty of Medicine) ;
  • Yolbas, Ilyas (Department of Pediatrics, Dicle University Faculty of Medicine) ;
  • Sahin, Cahit (Department of Pediatrics, Dicle University Faculty of Medicine)
  • Received : 2013.02.13
  • Accepted : 2013.05.09
  • Published : 2013.10.15
Download PDF
⟨ Previous Next ⟩

Abstract

Cystic fibrosis (CF) is a genetic disease with autosomal recessive inheritance and is common in Caucasian people. The prevalence of this disease is between 1/2,000 and 1/3,500 live births, and the incidence varies between populations. Although the CF transmembrane conductance regulator gene is expressed in the kidneys, renal involvement is rare. With advances in the treatment of CF, life expectancy has increased, and some previously unobserved disease associations are now seen in patients with CF. It is important to follow patients with CF for possible abnormalities that may accompany CF. In this paper, we present two rare cases of CF accompanied by nephrotic syndrome.

Keywords

References

  1. Gadsby DC, Vergani P, Csanady L. The ABC protein turned chloride channel whose failure causes cystic fibrosis. Nature 2006;440:477-83. https://doi.org/10.1038/nature04712
  2. Wallis C. Diagnosis of cystic fibrosis. In: Hodson M, Geddes D, Bush A, editors. Cystic fibrosis. London: Hodder Arnold, 2007:99-108.
  3. Ratjen F, Doring G. Cystic fibrosis. Lancet 2003;361:681-9. https://doi.org/10.1016/S0140-6736(03)12567-6
  4. O'Sullivan BP, Freedman SD. Cystic fibrosis. Lancet 2009;373: 1891-904. https://doi.org/10.1016/S0140-6736(09)60327-5
  5. McCormick J, Green MW, Mehta G, Culross F, Mehta A. Demographics of the UK cystic fibrosis population: implications for neonatal screening. Eur J Hum Genet 2002;10:583-90. https://doi.org/10.1038/sj.ejhg.5200850
  6. Jouret F, Devuyst O. CFTR and defective endocytosis: new insights in the renal phenotype of cystic fibrosis. Pflugers Arch 2009;457: 1227-36. https://doi.org/10.1007/s00424-008-0594-2
  7. Perez-Brayfield MR, Caplan D, Gatti JM, Smith EA, Kirsch AJ. Metabolic risk factors for stone formation in patients with cystic fibrosis. J Urol 2002;167(2 Pt 1):480-4. https://doi.org/10.1016/S0022-5347(01)69068-2
  8. Orr A, McVean RJ, Webb AK, Dodd ME. Questionnaire survey of urinary incontinence in women with cystic fibrosis. BMJ 2001; 322:1521. https://doi.org/10.1136/bmj.322.7301.1521
  9. Mc Laughlin AM, Crotty TB, Egan JJ, Watson AJ, Gallagher CG. Amyloidosis in cystic fibrosis: a case series. J Cyst Fibros 2006;5: 59-61. https://doi.org/10.1016/j.jcf.2005.09.010
  10. Melzi ML, Costantini D, Giani M, Appiani AC, Giunta AM. Severe nephropathy in three adolescents with cystic fibrosis. Arch Dis Child 1991;66:1444-7. https://doi.org/10.1136/adc.66.12.1444
  11. Soriano E, Fischman D, Cheriyath P. Membranoproliferative glomerulonephritis in patients with cystic fibrosis: coincidence or comorbidity? A case series. South Med J 2008;101:641-5. https://doi.org/10.1097/SMJ.0b013e318172f697
  12. Al-Shawwa BA, Rao AR. Cystic fibrosis and renal disease: a case report. J Med Case Rep 2007;1:24. https://doi.org/10.1186/1752-1947-1-24
  13. Laufer J, Augarten A, Szeinberg A, Rapoport J, Katzenelson D, Yahav Y. Nephrotic syndrome and fibrillary glomerulonephritis. J Intern Med 1997;242:83-6. https://doi.org/10.1046/j.1365-2796.1997.00164.x
  14. Canciani M, Pederzini F, Mastella G, Boccato P. Systemic amyloidosis in cystic fibrosis. Acta Paediatr Scand 1985;74:613-4. https://doi.org/10.1111/j.1651-2227.1985.tb11043.x
  15. Yahiaoui Y, Jablonski M, Hubert D, Mosnier-Pudar H, Noel LH, Stern M, et al. Renal involvement in cystic fibrosis: diseases spectrum and clinical relevance. Clin J Am Soc Nephrol 2009;4:921-8. https://doi.org/10.2215/CJN.00750209

Cited by

  1. Renal involvement and metabolic alterations in adults patients affected by cystic fibrosis vol.17, pp.1, 2013, https://doi.org/10.1186/s12967-019-02139-4