Clinical Spectrum and Prognosis of Atypical Autosomal Dominant Polycystic Kidney Disease Caused by Monoallelic Pathogenic Variants of IFT140.

Rationale & Objective: Monoallelic predicted Loss-of-Function (pLoF) variants in IFT140 have recently been associated with an autosomal dominant polycystic kidney disease (ADPKD)-like phenotype. This study sought to enhance the characterization of this phenotype.

Study Design: Case series.

Integrated use of Autosomal Dominant Polycystic Kidney Disease Prediction Tools and Risk Prognostication.

Background: Autosomal dominant polycystic kidney disease (ADPKD) is the most common genetic cause of kidney failure. Specific treatment is indicated upon observed or predicted rapid progression. For the latter, risk stratification tools have been developed independently based on either total kidney volume or genotyping as well as clinical variables.

SGLT2-Inhibition in Patients With Alport Syndrome.

Introduction: Large-scale trials showed positive outcomes of sodium-glucose cotransporter-2 inhibitors (SGLT2i) in adults with chronic kidney disease (CKD). Whether the use of SGLT2i is safe and effective in patients with the common hereditary CKD Alport syndrome (AS) has not yet been investigated specifically in larger cohorts.

Methods: This observational, multicenter, international study (NCT02378805) assessed 112 patients with AS after start of SGLT2i.

Self-efficacy, social support and oral health-related quality of life in patients with kidney transplantation and under hemodialysis.

Background: Aim of this questionnaire-based cross-sectional study was to compare self-efficacy, social support, oral hygiene-related self-efficacy (OHRSE) and oral health-related quality of life (OHRQoL) between patients under chronic hemodialysis (HD) and patients after kidney transplantation (KTx) as well as a healthy comparison group (HC).

Methods: Patients under HD were recruited during their routine outpatient dialysis therapy, KTx patients during their maintenance appointment and HC patients during their regular dental check-up in the dental clinic. General self-efficacy, the OHRSE, social support (F-SozU-K14) and the OHRQoL (OHIP-G5) were assessed by specific validated questionnaires.

Deceased donor urinary Dickkopf-3 associates with future allograft function following kidney transplantation.

Predicting future kidney allograft function is challenging. Novel biomarkers, such as urinary Dickkopf-3 (uDKK3), may help guide donor selection and improve allograft outcomes. In this prospective multicenter pilot trial, we investigated whether donor uDKK3 reflects organ quality and is associated with future allograft function.