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Burgmaier, Kathrin; Kilian, Samuel; Bammens, Bert; Benzing, Thomas; Billing, Heiko; Büscher, Anja; Galiano, Matthias; Grundmann, Franziska; Klaus, Günter; Mekahli, Djalila; Michel-Calemard, Laurence; Milosevski-Lomic, Gordana; Ranchin, Bruno; Sauerstein, Katja; Schaefer, Susanne; Shroff, Rukshana; Sterenborg, Rosalie; Verbeeck, Sarah; Weber, Lutz T.; Wicher, Dorota; Wühl, Elke; Dötsch, Jörg; Schaefer, Franz; Liebau, Max C.

Clinical courses and complications of young adults with Autosomal Recessive Polycystic Kidney Disease (ARPKD)

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  • Medientyp: E-Artikel
  • Titel: Clinical courses and complications of young adults with Autosomal Recessive Polycystic Kidney Disease (ARPKD)
  • Beteiligte: Burgmaier, Kathrin; Kilian, Samuel; Bammens, Bert; Benzing, Thomas; Billing, Heiko; Büscher, Anja; Galiano, Matthias; Grundmann, Franziska; Klaus, Günter; Mekahli, Djalila; Michel-Calemard, Laurence; Milosevski-Lomic, Gordana; Ranchin, Bruno; Sauerstein, Katja; Schaefer, Susanne; Shroff, Rukshana; Sterenborg, Rosalie; Verbeeck, Sarah; Weber, Lutz T.; Wicher, Dorota; Wühl, Elke; Dötsch, Jörg; Schaefer, Franz; Liebau, Max C.
  • Erschienen: Springer Science and Business Media LLC, 2019
  • Erschienen in: Scientific Reports
  • Sprache: Englisch
  • DOI: 10.1038/s41598-019-43488-w
  • ISSN: 2045-2322
  • Schlagwörter: Multidisciplinary
  • Entstehung:
  • Anmerkungen:
  • Beschreibung: <jats:title>Abstract</jats:title><jats:p>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.</jats:p>
  • Zugangsstatus: Freier Zugang