Introduction: Phenotypic heterogeneity and unpredictability of individual disease progression present enormous challenges in ultrarare renal ciliopathies. The tubular-derived glycoprotein, Dickkopf-related protein 3 (DKK3) is a promising biomarker for kidney fibrosis and prediction of kidney function decline. Here, we measured urinary DKK3 (uDKK3) levels in 195 pediatric patients with renal ciliopathy to assess its potential as a discriminative and prediction marker.
View Article and Find Full Text PDFBackground And Purpose: With the increasing amount of in-house created deep learning models in radiotherapy, it is important to know how to minimise the risks associated with the local clinical implementation prior to clinical use. The goal of this study is to give an example of how to identify the risks and find mitigation strategies to reduce these risks in an implemented workflow containing a deep learning based planning tool for breast Volumetric Modulated Arc Therapy.
Materials And Methods: The deep learning model ran on a private Google Cloud environment for adequate computational capacity and was integrated into a workflow that could be initiated within the clinical Treatment Planning System (TPS).
Background: Pediatric organ transplantation is the primary treatment for end-organ failure. Improving medication adherence and healthcare compliance can decrease healthcare burdens, graft rejection, morbidity, and mortality. Adolescents commonly struggle with non-adherence, necessitating a smooth transition to adult care.
View Article and Find Full Text PDFIntroduction: Data on age-related differences in rejection rates, infectious episodes, and tacrolimus exposure in pediatric kidney transplant recipients (pKTRs) on a tacrolimus-based immunosuppressive regimen are scarce.
Methods: We performed a large-scale analysis of 802 pKTRs from the Cooperative European Paediatric Renal Transplant Initiative (CERTAIN) registry from 40 centers in 14 countries. The inclusion criteria were a tacrolimus-based immunosuppressive regimen and at least 2 years of follow-up.
Orphanet J Rare Dis
November 2024
Background: Bardet-Biedl syndrome (BBS) is a rare autosomal-recessive ciliopathy with pathogenic variants in 26 BBS genes. It affects multiple organs, including the kidney and liver, with varying degrees regarding extent and time of first manifestation. Structural renal anomalies are an early feature and end-stage kidney disease (ESKD) cumulates to 25% in adulthood.
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