Evidence map›Paper›PMID 32919899›Full record

ArticleMolecular genetics and metabolism

Prospective evaluation of kidney and liver disease in autosomal recessive polycystic kidney disease-congenital hepatic fibrosis.

Nehna Abdul Majeed, Esperanza Font-Montgomery, Linda Lukose, Joy Bryant, Peter Veppumthara, Peter L Choyke, Ismail B Turkbey, Theo Heller, William A Gahl, Meral Gunay-Aygun

Open access · greenAbstract read
PubMed Full text at PMC Publisher
In one paragraph

Article in Molecular genetics and metabolism. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Cited by 14 papers.

0numbers the graph read from it
0cells of the map it votes in
14citing papers in PubMed
2.7field-weighted citation impact, top 10% of its field
1 · What the graph read from 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.

2 · The registry

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.

3 · Its place in the literature

Who cites it

14 citing papers in PubMed, 23 citations in OpenAlex.

  1. Review
  2. Review
  3. Review
  4. Review
  5. Article
  6. Polycystic Kidney Disease in Children: The Current Status and the Next Horizon.American journal of kidney diseases : the official journal of the National Kidney Foundation · 2025
    Review
  7. Article
  8. Article
  9. Article
  10. Article
  11. Article
  12. Observational
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4 · The record

Corrections and comments

PubMed lists nothing against this paper. Absence here is not a guarantee, only a check that was made.

5 · Who and what money

Authors and funding

10 authors at 5 institutions in 1 country.

Nehna Abdul MajeedLiver Diseases Branch, National Institute of Diabetes and Digestive and Kidney Diseases, National Institutes of Health, Bethesda, MD 20892, USA; MedStar Health, Internal Medicine, Baltimore, MD, USA.
Esperanza Font-MontgomerySection on Human Biochemical Genetics, Medical Genetics Branch, National Human Genome Research Institute, National Institutes of Health, Bethesda, MD 20892, USA; University of Missouri, Department of Pediatrics and Medical Genetics, Columbia, MO 65212, USA.
Linda LukoseSection on Human Biochemical Genetics, Medical Genetics Branch, National Human Genome Research Institute, National Institutes of Health, Bethesda, MD 20892, USA; RBK Pediatrics, Commack, NY 11725, USA.
Joy BryantSection on Human Biochemical Genetics, Medical Genetics Branch, National Human Genome Research Institute, National Institutes of Health, Bethesda, MD 20892, USA.
Peter VeppumtharaRadiology and Imaging Sciences, Clinical Center, National Institutes of Health, Bethesda, MD 20892, USA.
Peter L ChoykeMolecular Imaging Program, National Cancer Institute, National Institutes of Health, Bethesda, MD 20892, USA.
Ismail B TurkbeyMolecular Imaging Program, National Cancer Institute, National Institutes of Health, Bethesda, MD 20892, USA.
Theo HellerLiver Diseases Branch, National Institute of Diabetes and Digestive and Kidney Diseases, National Institutes of Health, Bethesda, MD 20892, USA.
William A GahlSection on Human Biochemical Genetics, Medical Genetics Branch, National Human Genome Research Institute, National Institutes of Health, Bethesda, MD 20892, USA; NIH Undiagnosed Diseases Program, Common Fund, Office of the Director, National Institutes of Health, Bethesda, MD 20892, USA.
Meral Gunay-AygunSection on Human Biochemical Genetics, Medical Genetics Branch, National Human Genome Research Institute, National Institutes of Health, Bethesda, MD 20892, USA; Johns Hopkins University School of Medicine, McKusick-Nathans Department of Genetic Medicine, Department of Pediatrics, Baltimore, MD 21287, USA. Electronic address: mgaygun@mail.nih.gov.
National Institutes of Health · USNational Institute of Diabetes and Digestive and Kidney Diseases · USNational Cancer Institute · USNational Institutes of Health Clinical Center · USOffice of the Director · US

Funding

Intramural NIH HHS Z99 HG999999
6 · The paper itself

Abstract

BACKGROUND AND

objectivesWe have previously published the characteristics of kidney and liver disease in a cohort of 73 individuals with molecularly confirmed autosomal recessive polycystic kidney disease-congenital hepatic fibrosis, based upon cross-sectional data. Here, we present prospective data on the same cohort. DESIGN, SETTING, PARTICIPANTS, AND MEASUREMENTS: Comprehensive biochemical and imaging data on progression of kidney and liver disease in 60 of the 73 patients were prospectively collected at the NIH Clinical Center on multiple visits between 2003 and 2019. RESULTS AND

conclusionsOf the 73 patients, 23 received a renal allograft at an average age of 17.5 years and 10 underwent liver transplantation at an average age of 20.3 years. Patients who presented perinatally and those who had corticomedullary disease required kidney transplantation significantly earlier. The mean eGFR slope in patients with corticomedullary disease was -1.6 ml/min/1.73 m

Indexed as

AdolescentAdultChildCohort StudiesCross-Sectional StudiesDisease ProgressionFemaleGenetic Diseases, InbornHumansHypertension, PortalKidneyKidney TransplantationLiverLiver CirrhosisLiver TransplantationMalePKHD1 protein, humanReceptors, Cell Surface

Identifiers

PMID32919899
PMCPMC7749036
OpenAlexW3082786579

What OpenQuestion holds

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Read underepoch 390

Registered trials

None linked

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.