ArticleClinical journal of the American Society of Nephrology : CJASN2009
Renal involvement in cystic fibrosis: diseases spectrum and clinical relevance.
Article in Clinical journal of the American Society of Nephrology : CJASN, 2009. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Cited by 29 papers, 2 of them syntheses that pooled it.
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Who cites it
29 citing papers in PubMed, 2 syntheses or guidelines pooled it, 61 citations in OpenAlex.
- Altered intravenous drug disposition in people living with cystic fibrosis: A meta-analysis integrating top-down and bottom-up data.CPT: pharmacometrics & systems pharmacology · 2022Pooled it
- Pharmacokinetics in Patients with Cystic Fibrosis: A Systematic Review of Data Published Between 1999 and 2019.Clinical pharmacokinetics · 2020Pooled it
- Ano1 Antisense Therapy Improves Enamel Properties in Molars but not Amelogenesis in a Murine Model of Cystic Fibrosis.Calcified tissue international · 2026Article
- Cystic fibrosis-related kidney disease-emerging morbidity and disease modifier.Pediatric nephrology (Berlin, Germany) · 2025Review
- Article
- Hyperfiltration and Elevated Fractional Excretion of Magnesium in Children with Cystic Fibrosis.ACS pharmacology & translational science · 2025Article
- Patients With Cystic Fibrosis Undergoing Posterior Lumbar Fusion Are at an Increased Odds of Perioperative Complications.Journal of the American Academy of Orthopaedic Surgeons. Global research & reviews · 2025Article
- The ageing of people living with cystic fibrosis: what to expect now?European respiratory review : an official journal of the European Respiratory Society · 2024Review
- Clinicopathologic Features of Kidney Disease in Patients With Cystic Fibrosis.Kidney international reports · 2024Article
- Gene Polymorphism of Biotransformation Enzymes and Ciprofloxacin Pharmacokinetics in Pediatric Patients with Cystic Fibrosis.Biomedicines · 2022Article
- Renal Function in Patients with Cystic Fibrosis: A Single-Center Study.International journal of environmental research and public health · 2022Article
- Urinary tract infections in cystic fibrosis patients.Journal of cystic fibrosis : official journal of the European Cystic Fibrosis Society · 2022Article
- Kidney and combined kidney and pancreas transplantation may be under-utilized in cystic fibrosis.Frontiers in transplantation · 2022Article
- The Potential Use of Cannabis in Tissue Fibrosis.Frontiers in cell and developmental biology · 2021Review
- The future of cystic fibrosis care: a global perspective.The Lancet. Respiratory medicine · 2020Review
- Comparable Bioavailability and Disposition of Pefloxacin in Patients with Cystic Fibrosis and Healthy Volunteers Assessed via Population Pharmacokinetics.Pharmaceutics · 2019Article
- Secondary IgA nephropathy.Kidney international · 2018Review
- Case report on pathogenetic link between gluten and IgA nephropathy.BMC gastroenterology · 2018Article
- C3 glomerulopathy in cystic fibrosis: a case report.BMC nephrology · 2018Article
- Diagnosis and management of treatment-refractory hypothyroidism: an expert consensus report.Journal of endocrinological investigation · 2017Review
Corrections and comments
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Authors and funding
10 authors at 1 institution in 1 country.
Funding
No grant is acknowledged in the PubMed record.
Abstract
BACKGROUND AND
objectivesClinically relevant kidney involvement is uncommonly described in adult patients with cystic fibrosis (CF). We sought to report on a series of patients with CF and kidney biopsy-documented renal involvement. DESIGN, SETTING, PARTICIPANTS, & MEASUREMENTS: A retrospective study was undertaken in two referral centers for adult patients with CF in Paris, France. Patients who had undergone a biopsy of native kidneys between 1992 and 2008 were identified, and their medical records were reviewed.
resultsWe identified 13 adult patients with CF and renal disease. Proteinuria was present in all but two cases and was associated with progressive renal impairment in four patients (median serum creatinine 85 micromol/L; range 53 to 144 micromol/L). Renal biopsy disclosed a heterogeneous spectrum of nephropathies including AA amyloidosis (n = 3), diabetic glomerulopathy (n = 3), FSGS (n = 2), minimal-change disease (n = 1), postinfectious glomerulonephritis (n = 1), IgA nephropathy related to Henoch-Schönlein purpura (n = 1), membranous nephropathy (n = 1), and chronic interstitial nephropathy (n = 1). Chronic renal failure occurred in five patients, and one patient reached ESRD.
conclusionsAlthough rare, clinically significant renal disease may arise in young adult patients with CF. Given the wide spectrum of diseases that may be encountered, definite diagnosis by kidney biopsy is mandatory to optimize clinical treatment of these complex patients, particularly in the perspective of organ transplantation.
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