ArticleNephrology, dialysis, transplantation : official publication of the European Dialysis and Transplant Association - European Renal Association2016
Effect of genotype on the severity and volume progression of polycystic liver disease in autosomal dominant polycystic kidney disease.
Article in Nephrology, dialysis, transplantation : official publication of the European Dialysis and Transplant Association - European Renal Association, 2016. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Cited by 41 papers.
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41 citing papers in PubMed, 75 citations in OpenAlex.
- Effects of the GnRH agonist leuprorelin on liver volume in premenopausal women with severe polycystic liver disease.Hepatology communications · 2026Trial
- Modeling Complex Developmental Disease: The Case of Polycystic Kidney Disease.Journal of developmental biology · 2026Review
- Dual Monogenic Cystic Disease Case Report: Autosomal Dominant Polycystic Kidney Disease and Autosomal Dominant Polycystic Liver Disease.Clinical case reports · 2026Article
- Unravelling sex-specific differences in autosomal dominant polycystic kidney disease: a multiorgan perspective.Clinical kidney journal · 2026Review
- KDIGO 2025 ADPKD guideline: a commentary on diagnosis and management of hepatopancreatic manifestations by the ERA Working Group Genes & Kidney.Nephrology, dialysis, transplantation : official publication of the European Dialysis and Transplant Association - European Renal Association · 2025Article
- Autosomal dominant polycystic kidney disease: an overview of recent genetic and clinical advances.Renal failure · 2025Review
- A Case Study of Liver After Kidney Transplantation: Addressing Polycystic Diseases With Tailored Surgical and Postoperative Approaches.Clinical case reports · 2025Article
- Characterization of the Cystic Phenotype Associated with Monoallelic ALG8 and ALG9 Pathogenic Variants.Journal of the American Society of Nephrology : JASN · 2025Article
- Effects of Pregnancy on Liver and Kidney Cyst Growth Rates in Autosomal Dominant Polycystic Kidney Disease: A Pilot Study.Journal of clinical medicine · 2025Article
- Novel PKD1 Mutation (c.G10086T) Drives High Intracranial Aneurysm Risk in Autosomal Dominant Polycystic Kidney Disease.European journal of neurology · 2025Article
- Liver manifestations in autosomal dominant polycystic kidney disease (ADPKD) and their impact on quality of life.Clinical kidney journal · 2025Article
- Brachial-ankle pulse wave velocity predicts liver volume in patients with autosomal dominant polycystic kidney disease.PloS one · 2025Article
- Autosomal Dominant Polycystic Kidney Disease: Extrarenal Involvement.International journal of molecular sciences · 2024Review
- Factors Associated With the Development and Severity of Polycystic Liver in Patients With Autosomal Dominant Polycystic Kidney Disease.Journal of Korean medical science · 2023Article
- Pkhd1Journal of molecular medicine (Berlin, Germany) · 2023Article
- Genetic Spectrum of Polycystic Kidney and Liver Diseases and the Resulting Phenotypes.Advances in kidney disease and health · 2023Review
- Questionnaire PLD-complaint-specific assessment identifies need for therapy in polycystic liver disease: A multi-centric prospective study.United European gastroenterology journal · 2023Observational
- Bone health in autosomal dominant polycystic kidney disease (ADPKD) patients after kidney transplantation.Bone reports · 2023Article
- Modelling polycystic liver disease progression using age-adjusted liver volumes and targeted mutational analysis.JHEP reports : innovation in hepatology · 2022Article
- Protocol for a randomized controlled multicenter trial assessing the efficacy of leuprorelin for severe polycystic liver disease: the AGAINST-PLD study.BMC gastroenterology · 2022Article
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8 authors at 2 institutions in 2 countries.
Funding
Abstract
backgroundThe autosomal dominant polycystic kidney disease (APDKD) genotype influences renal phenotype severity but its effect on polycystic liver disease (PLD) is unknown. Here we analyzed the influence of genotype on liver phenotype severity.
methodsClinical data were retrieved from electronic records of patients who were mutation screened with the available liver imaging (n = 434). Liver volumes were measured by stereology (axial or coronal images) and adjusted to height (HtLV).
resultsAmong the patients included, 221 (50.9%) had truncating PKD1 (PKD1-T), 141 (32.5%) nontruncating PKD1 (PKD1-NT) and 72 (16.6%) PKD2 mutations. Compared with PKD1-NT and PKD2, patients with PKD1-T had greater height-adjusted total kidney volumes (799 versus 610 and 549 mL/m; P < 0.001). HtLV was not different (1042, 1095 and 1058 mL/m; P = 0.64) between the three groups, but females had greater HtLVs compared with males (1114 versus 1015 mL/m; P < 0.001). Annualized median liver growth rates were 1.68, 1.5 and 1.24% for PKD1-T, PKD1-NT and PKD2 mutations, respectively (P = 0.49), and remained unaffected by the ADPKD genotype when adjusted for age, gender and baseline HtLV. Females <48 years of age had higher annualized growth rates compared with those who were older (2.65 versus 0.09%; P < 0.001). After age 48 years, 58% of females with severe PLD had regression of HtLV, while HtLV continued to increase in males.
conclusionsIn contrast to the renal phenotype, the ADPKD genotype was not associated with the severity or growth rate of PLD in ADKPD patients. This finding, along with gender influence, indicates that modifiers beyond the disease gene significantly influence the liver phenotype.
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