Cystic fibrosis of pancreas and nephrotic syndrome: a rare association

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.

Renal involvement and metabolic alterations in adults patients affected by cystic fibrosis

“…Due to the increase in patient lifespans, previously unobserved disease associations have been detected. Renal involvement in CF patients has always been considered rare, but recently, renal pathologies such as glomerulosclerosis, mesangial proliferation, membranoproliferative and postinfectious glomerulonephritis, nephrocalcinosis and hematuria, tubular damage, fibrillary glomerulonephritis, and amyloidosis, in particular amyloid protein A (AA) in children, have been reported [23]. There has been a dramatic increase in median survival of CF patients over the last two decades, in fact median survival has improved to 45 years, therefore it becomes necessary to deal with long-term complications such as renal and cardiovascular diseases.…”