title: A CF patient with progressive proteinuric renal disease: a CF-specific nodular glomerulosclerosis?
abstract: Cystic fibrosis (CF) is a multisystemic disease but without a classical disease-specific renal phenotype. A 32-year-old male patient with CF (ΔF508/ΔF508) presented with a nephrotic syndrome. Renal biopsy revealed nodular glomerulosclerosis (NGS) occurring in the absence of diabetes mellitus, amyloidosis and any other known common cause of NGS. He had a progressive decline in estimated glomerular filtration rate (eGFR) to chronic kidney disease stage V (eGFR <15 mL/min/1.73 m(2)) over a 3-year period despite optimal medical management. This is the fourth reported case of NGS in a patient with CF without diabetes and is the first to originate from a European country. This case supports the concept of a CF-related NGS.
authors.affiliation: Cork Adult Cystic Fibrosis Centre , Cork University Hospital , University College Cork , Ireland.
authors.forename: Oisin
authors.initials: O
authors.lastname: O'Connell
authors.affiliation: Department of Renal Medicine , Cork University Hospital , University College Cork , Ireland.
authors.forename: Ciara N
authors.initials: CN
authors.lastname: Magee
authors.affiliation: Department of Pathology , Cork University Hospital, University College Cork , Ireland.
authors.forename: Brendan
authors.initials: B
authors.lastname: Fitzgerald
authors.affiliation: Department of Pathology , Cork University Hospital, University College Cork , Ireland.
authors.forename: Louise
authors.initials: L
authors.lastname: Burke
authors.affiliation: Department of Renal Medicine , Cork University Hospital , University College Cork , Ireland.
authors.forename: William D
authors.initials: WD
authors.lastname: Plant
authors.affiliation: Cork Adult Cystic Fibrosis Centre , Cork University Hospital , University College Cork , Ireland.
authors.forename: Barry J
authors.initials: BJ
authors.lastname: Plant
keywords: CF renal disease; cystic fibrosis; nebulized tobramycin toxicity; nodular glomerulosclerosis