ReviewPediatric nephrology (Berlin, Germany)2017
Proteinuria in Dent disease: a review of the literature.
Review in Pediatric nephrology (Berlin, Germany), 2017. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Cited by 37 papers, 1 of them a synthesis that pooled it.
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Who cites it
37 citing papers in PubMed, 1 synthesis or guideline pooled it, 64 citations in OpenAlex.
- Dent disease: clinical practice recommendations.Nephrology, dialysis, transplantation : official publication of the European Dialysis and Transplant Association - European Renal Association · 2025Guideline
- Proteinuria in adolescence.Pediatric nephrology (Berlin, Germany) · 2026Review
- Molecular Basis of Rare Inherited Tubulopathies of the Kidney: A Primer for Clinicians.International journal of molecular sciences · 2026Review
- Phenotype and genotype analyses of 21 Chinese patients with Dent disease.Journal of biomedical research · 2025Article
- Cell cycle disorders in podocytes: an emerging and increasingly recognized phenomenon.Cell death discovery · 2025Review
- The renal histopathology of nonproteinuric kidney impairment: a three center experience.Clinical and experimental medicine · 2024Article
- Prevalence of kidney failure in adults diagnosed with hereditary tubulopathies.Journal of nephrology · 2024Observational
- Pediatric Dent disease presenting with rickets and end-stage renal disease: case report and literature review.The Journal of international medical research · 2024Review
- 4-Phenylbutyric Acid Treatment Reduces Low-Molecular-Weight Proteinuria in aInternational journal of molecular sciences · 2024Article
- Renal antiporter ClC-5 regulates collagen I/IV through the β-catenin pathway and lysosomal degradation.Life science alliance · 2024Article
- Prenatal diagnosis of dent disease type I with a nonsense pathogenic variant in CLCN5: a case study.BMC medical genomics · 2024Article
- Case Report: Early acute kidney failure in an 11-year-old boy with Dent disease type 1.Frontiers in pediatrics · 2024Article
- A missense mutant of ocrl1 promotes apoptosis of tubular epithelial cells and disrupts endocytosis and the cell cycle of podocytes in Dent-2 Disease.Cell communication and signaling : CCS · 2023Article
- Increased Renal Medullary NOX-4 in Female but Not Male Mice during the Early Phase of Type 1 Diabetes: Potential Role of ROS in Upregulation of TGF-β1 and Fibronectin in Collecting Duct Cells.Antioxidants (Basel, Switzerland) · 2023Article
- Dent disease manifesting as nephrotic syndrome.Intractable & rare diseases research · 2023Article
- Dent's disease: An unusual cause of kidney failure.Clinical nephrology. Case studies · 2023Article
- Emerging roles of proximal tubular endocytosis in renal fibrosis.Frontiers in cell and developmental biology · 2023Review
- Fanconi syndrome, nephrotic-range proteinuria, and hypoalbuminemia in a newborn-Occam's razor or Hickam's dictum? Answers.Pediatric nephrology (Berlin, Germany) · 2022Article
- A novel CLCN5 frame shift mutation responsible for Dent disease 1: Case report.Frontiers in pediatrics · 2022Article
- Whole-Exome Sequencing in Idiopathic Short Stature: Rare Mutations Affecting Growth.Journal of pediatric genetics · 2021Article
Corrections and comments
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Authors and funding
4 authors at 2 institutions in 2 countries.
Funding
No grant is acknowledged in the PubMed record.
Abstract
backgroundDent disease is a rare X-linked recessive proximal tubulopathy caused by mutations in CLCN5 (Dent-1) or OCRL (Dent-2). As a rule, total protein excretion (TPE) is low in tubular proteinuria compared with glomerular disease. Several authors have reported nephrotic-range proteinuria (NP) and glomerulosclerosis in Dent disease. Therefore, we aimed to analyze protein excretion in patients with documented CLCN5 or OCRL mutations in a systematic literature review.
designPubMed and Embase were searched for cases with documented CLCN5 or OCRL mutations and (semi-)quantitative data on protein excretion. The most reliable data (i.e., TPE > protein-creatinine ratio > Albustix) was used for NP classification.
resultsData were available on 148 patients from 47 reports: 126 had a CLCN5 and 22 an OCRLmutation. TPE was not significantly different between both forms (p = 0.11). Fifty-five of 126 (43.7 %) Dent-1 vs 13/22 (59.1 %) Dent-2 patients met the definition of NP (p = 0.25). Serum albumin was normal in all reported cases (24/148). Glomerulosclerosis was noted in 20/32 kidney biopsies and was strongly related to tubulointerstitial fibrosis, but not to kidney function or proteinuria.
conclusionMore than half of the patients with both forms of Dent disease have NP, and the presence of low molecular weight proteinuria in a patient with NP in the absence of edema and hypoalbuminemia should prompt genetic testing. Even with normal renal function, glomerulosclerosis and tubulointerstitial fibrosis are present in Dent disease. The role of proteinuria in the course of the disease needs to be examined further in longitudinal studies.
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