ReviewPediatric research2014
Pathophysiology of childhood polycystic kidney diseases: new insights into disease-specific therapy.
Review in Pediatric research, 2014. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Cited by 25 papers, 1 of them a synthesis that pooled it.
What it found
Each row is one number read from the abstract, on the scale the paper reported it, with its interval. Left of the dashed line favours the treatment, right favours the comparator. Under each row is the sentence it came from. New to these charts? A ten-minute tutorial.
The abstract states no effect estimate the extractor could read, or names no intervention and outcome on the map, so this paper lights no cell and moves no belief. It is still indexed, cited and linked below.
The trial behind it
Trials whose registry record cites this paper, or whose number appears in the abstract. A trial that started after this paper was published is citing it as background, not reporting it.
Neither the registry nor the abstract names a trial number. If this is a trial report, that itself is worth knowing.
Who cites it
25 citing papers in PubMed, 1 synthesis or guideline pooled it, 56 citations in OpenAlex.
- Pooled it
- Assessment of Interleukin-15 (IL-15) Concentration in Children with Cystic Kidney Disease.Biomedicines · 2026Article
- Next-Generation Sequencing Defines a Molecularly Confirmed ARPKD Core Within the BroaderGenes · 2026Article
- Atypical presentations of fetal polycystic kidney disease demonstrates the utility of a genomic autopsy for accurate post-mortem diagnoses.Human genomics · 2025Article
- A case of pregnancy complicated by caroli disease.BMC pregnancy and childbirth · 2025Article
- Activation of Notch3 in Renal Tubular Cells Leads to Progressive Cystic Kidney Disease.International journal of molecular sciences · 2022Article
- Predictors of progression in autosomal dominant and autosomal recessive polycystic kidney disease.Pediatric nephrology (Berlin, Germany) · 2021Review
- Article
- Molecular Pathophysiology of Autosomal Recessive Polycystic Kidney Disease.International journal of molecular sciences · 2021Review
- Autosomal Dominant Polycystic Kidney Disease does not significantly alter major COVID-19 outcomes among veterans.medRxiv : the preprint server for health sciences · 2020Article
- Proliferative signaling by ERBB proteins and RAF/MEK/ERK effectors in polycystic kidney disease.Cellular signalling · 2020Review
- Targeting and therapeutic peptide-based strategies for polycystic kidney disease.Advanced drug delivery reviews · 2020Review
- Management of delivery of a fetus with autosomal recessive polycystic kidney disease: a case report of abdominal dystocia and review of the literature.Journal of medical case reports · 2019Review
- Aberrant Regulation of Notch3 Signaling Pathway in Polycystic Kidney Disease.Scientific reports · 2018Article
- Tesevatinib ameliorates progression of polycystic kidney disease in rodent models of autosomal recessive polycystic kidney disease.World journal of nephrology · 2017Article
- Genetic analysis of the PKHD1 gene with long-rang PCR sequencing.Journal of Huazhong University of Science and Technology. Medical sciences = Hua zhong ke ji da xue xue bao. Yi xue Ying De wen ban = Huazhong keji daxue xuebao. Yixue Yingdewen ban · 2016Article
- Heterotrimeric G protein signaling in polycystic kidney disease.Physiological genomics · 2016Review
- Kidney Injury Molecule-1 Is Specifically Expressed in Cystically-Transformed Proximal Tubules of the PKD/Mhm (cy/+) Rat Model of Polycystic Kidney Disease.International journal of molecular sciences · 2016Article
- Rationale for early treatment of polycystic kidney disease.Pediatric nephrology (Berlin, Germany) · 2015Review
- Rationale, design and objectives of ARegPKD, a European ARPKD registry study.BMC nephrology · 2015Observational
Corrections and comments
PubMed lists nothing against this paper. Absence here is not a guarantee, only a check that was made.
Authors and funding
2 authors at 1 institution in 1 country.
Funding
Abstract
Autosomal dominant polycystic kidney disease (ADPKD) and autosomal recessive polycystic kidney disease (ARPKD) are significant causes of morbidity and mortality in children and young adults. ADPKD, with an incidence of 1:400 to 1:1,000, affects more than 13 million individuals worldwide and is a major cause of end-stage renal disease in adults. However, symptomatic disease is increasingly recognized in children. ARPKD is a dual-organ hepatorenal disease with an incidence of 1:20,000 to 1:40,000 and a heterozygote carrier rate of 1 in 70. Currently, no clinically significant disease-specific therapy exists for ADPKD or ARPKD. The genetic basis of both ADPKD and ARPKD have been identified, and delineation of the basic molecular and cellular pathophysiology has led to the discovery that abnormal ADPKD and ARPKD gene products interact to create "polycystin complexes" located at multiple sites within affected cells. The extracellular matrix and vessels produce a variety of soluble factors that affect the biology of adjacent cells in many dynamic ways. This review will focus on the molecular and cellular bases of the abnormal cystic phenotype and discuss the clinical translation of such basic data into new therapies that promise to alter the natural history of disease for children with genetic PKDs.
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What OpenQuestion holds
Registered trials
Read under generation 80e0d062 · epoch 390. Bibliography from PubMed, PubMed Central and OpenAlex; grants from NIH RePORTER; trial links from ClinicalTrials.gov; estimates, votes and beliefs from the OpenQuestion graph.