ArticleScientific reports2019
Clinical courses and complications of young adults with Autosomal Recessive Polycystic Kidney Disease (ARPKD).
Article in Scientific reports, 2019. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Cited by 45 papers, 1 of them a synthesis that pooled it.
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Who cites it
45 citing papers in PubMed, 1 synthesis or guideline pooled it, 78 citations in OpenAlex.
- Systematic review on outcomes used in clinical research on autosomal recessive polycystic kidney disease-are patient-centered outcomes our blind spot?Pediatric nephrology (Berlin, Germany) · 2021Pooled it
- Health-related quality of life, mental health and caregiver burden in children with autosomal recessive polycystic kidney disease.Pediatric nephrology (Berlin, Germany) · 2026Article
- Clinical spectrum, genetic variants, and outcomes of autosomal recessive polycystic kidney disease in Omani children: two-center experience.Pediatric nephrology (Berlin, Germany) · 2026Article
- Modeling Complex Developmental Disease: The Case of Polycystic Kidney Disease.Journal of developmental biology · 2026Review
- Advanced MRI methods for children and young adults with autosomal recessive polycystic kidney disease (ARPKD) to support future multi-center clinical trials.Pediatric radiology · 2026Review
- Deciphering the Impact of RAC1-SPTAN1 in ARPKD Cystogenesis Using Multifaceted Models.Advanced science (Weinheim, Baden-Wurttemberg, Germany) · 2026Article
- Obstructive jaundice induced by postoperative intra-cystic infection with polycystic liver disease: a rare case report and literature review.International journal of surgery case reports · 2026Article
- Predictors of kidney survival in children with autosomal recessive polycystic kidney disease.Clinical and experimental nephrology · 2026Article
- ARPKD With Shrinking Kidneys and Progressive Loss of Kidney Function.Kidney international reports · 2026Article
- Next-Generation Sequencing Defines a Molecularly Confirmed ARPKD Core Within the BroaderGenes · 2026Article
- A Rare PKHD1 Frameshift Variant Identified in a Prenatally Diagnosed ARPKD Case From a Consanguineous Jordanian Family.Case reports in obstetrics and gynecology · 2026Article
- Determinants of left ventricular mass in children with autosomal recessive polycystic kidney disease.Journal of nephrology · 2025Article
- 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 · 2025Review
- Genes, kidneys, and the future: transforming chronic kidney disease management through genomic insights.Childhood kidney diseases · 2025Review
- Non-invasive screening for liver fibrosis by acoustic radiation force impulse in patients with ciliopathies.Scientific reports · 2025Article
- Case Report: An atypical case of ARPKD highlights the utility and challenges of implementing genetic testing in cystic kidney disease.Frontiers in pediatrics · 2025Article
- Multimodal Magnetic Resonance Imaging Assessments of Kidney Disease Severity in Autosomal Recessive Polycystic Kidney Disease.Kidney international reports · 2024Article
- Ultrasound evaluation of kidney and liver involvement in Bardet-Biedl syndrome.Orphanet journal of rare diseases · 2024Article
- Protocol for the nationwide registry of patients with polycystic kidney disease: japanese national registry of PKD (JRP).Clinical and experimental nephrology · 2024Article
- Genetic landscape and clinical outcomes of autosomal recessive polycystic kidney disease in Kuwait.Heliyon · 2024Article
Corrections and comments
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Authors and funding
24 authors at 13 institutions in 6 countries.
Funding
No grant is acknowledged in the PubMed record.
Abstract
Autosomal recessive polycystic kidney disease (ARPKD) is a severe pediatric hepatorenal disorder with pronounced phenotypic variability. A substantial number of patients with early diagnosis reaches adulthood and some patients are not diagnosed until adulthood. Yet, clinical knowledge about adult ARPKD patients is scarce. Here, we describe forty-nine patients with longitudinal follow-up into young adulthood that were identified in the international ARPKD cohort study ARegPKD. Forty-five patients were evaluated in a cross-sectional analysis at a mean age of 21.4 (±3.3) years describing hepatorenal findings. Renal function of native kidneys was within CKD stages 1 to 3 in more than 50% of the patients. Symptoms of hepatic involvement were frequently detected. Fourteen (31%) patients had undergone kidney transplantation and six patients (13%) had undergone liver transplantation or combined liver and kidney transplantation prior to the visit revealing a wide variability of clinical courses. Hepatorenal involvement and preceding complications in other organs were also evaluated in a time-to-event analysis. In summary, we characterize the broad clinical spectrum of young adult ARPKD patients. Importantly, many patients have a stable renal and hepatic situation in young adulthood. ARPKD should also be considered as a differential diagnosis in young adults with fibrocystic hepatorenal disease.
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