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View article: Response to the Letter to the Editor Entitled “From Variant of Unknown Significance to Likely Pathogenic: Adult-Onset - Nephronophthisis Linked to NPHP4 p.T680M”
Response to the Letter to the Editor Entitled “From Variant of Unknown Significance to Likely Pathogenic: Adult-Onset - Nephronophthisis Linked to NPHP4 p.T680M” Open
View article: Systematic review of outcomes reported in clinical research on nephronophthisis: how do they align with SONG Kids priorities?
Systematic review of outcomes reported in clinical research on nephronophthisis: how do they align with SONG Kids priorities? Open
Background Nephronophthisis (NPH) is a rare hereditary cystic kidney disease, characterized by a highly variable clinical and genetic presentation, accounting for up to 10% of kidney failure in children. Despite advances in understanding i…
View article: Association of intrapatient tacrolimus variability and concentration-to-dose ratio with outcomes in pediatric kidney transplantation
Association of intrapatient tacrolimus variability and concentration-to-dose ratio with outcomes in pediatric kidney transplantation Open
View article: Non-invasive screening for liver fibrosis by acoustic radiation force impulse in patients with ciliopathies
Non-invasive screening for liver fibrosis by acoustic radiation force impulse in patients with ciliopathies Open
Primary cilia are antenna-like structures on the surface of epithelial cells involved in multiple signaling pathways. Their malfunction can cause a heterogenous group of diseases called ciliopathies with a broad spectrum of organ involveme…
View article: Insights from ADPedKD, ERKReg and RaDaR registries provide a multi-national perspective on the presentation of childhood autosomal dominant polycystic kidney disease in high- and middle-income countries
Insights from ADPedKD, ERKReg and RaDaR registries provide a multi-national perspective on the presentation of childhood autosomal dominant polycystic kidney disease in high- and middle-income countries Open
View article: A risk score to predict kidney survival in patients with autosomal recessive polycystic kidney disease at the age of two months
A risk score to predict kidney survival in patients with autosomal recessive polycystic kidney disease at the age of two months Open
Autosomal recessive polycystic kidney disease (ARPKD) is a severe hepatorenal fibrocystic disorder. Its rareness and the variability of disease courses have been major obstacles for the establishment of clinical trials on treatment of kidn…
View article: Urinary Dickkopf-related protein 3 as a novel biomarker for kidney function decline in children with Alport syndrome
Urinary Dickkopf-related protein 3 as a novel biomarker for kidney function decline in children with Alport syndrome Open
View article: Urinary Dickkopf-3 Reflects Disease Severity and Predicts Short-Term Kidney Function Decline in Renal Ciliopathies
Urinary Dickkopf-3 Reflects Disease Severity and Predicts Short-Term Kidney Function Decline in Renal Ciliopathies Open
In renal ciliopathies, uDKK3 is a marker to assess disease severity and estimate short-term kidney function decline.
View article: Incidence, risk factors, management strategies, and outcomes of antibody-mediated rejection in pediatric kidney transplant recipients—a multicenter analysis of the Cooperative European Paediatric Renal Transplant Initiative (CERTAIN)
Incidence, risk factors, management strategies, and outcomes of antibody-mediated rejection in pediatric kidney transplant recipients—a multicenter analysis of the Cooperative European Paediatric Renal Transplant Initiative (CERTAIN) Open
View article: Erratum zu: Das Bardet-Biedl-Syndrom
Erratum zu: Das Bardet-Biedl-Syndrom Open
View article: Das Bardet-Biedl-Syndrom
Das Bardet-Biedl-Syndrom Open
Zusammenfassung Hintergrund Das Bardet-Biedl-Syndrom (BBS) ist eine seltene Multisystemerkrankung, die durch Varianten von derzeit 26 bekannten Genen verursacht wird. Abhängig von der Ausprägung der unterschiedlichen Symptome wird die Diag…
View article: Immunofluorescence analyses of respiratory epithelial cells aid the diagnosis of nephronophthisis
Immunofluorescence analyses of respiratory epithelial cells aid the diagnosis of nephronophthisis Open
Background Nephronophthisis (NPH) comprises a heterogeneous group of inherited renal ciliopathies clinically characterized by progressive kidney failure. So far, definite diagnosis is based on molecular testing only. Here, we studied the f…
View article: Renal and Extrarenal Phenotypes in Patients With HNF1B Variants and Chromosome 17q12 Microdeletions
Renal and Extrarenal Phenotypes in Patients With HNF1B Variants and Chromosome 17q12 Microdeletions Open
View article: Incidence, risk factors, management strategies and outcomes of antibody-mediated rejection in pediatric kidney transplant recipients – a multicenter analysis of the Cooperative European Paediatric Renal Transplant Initiative (CERTAIN)
Incidence, risk factors, management strategies and outcomes of antibody-mediated rejection in pediatric kidney transplant recipients – a multicenter analysis of the Cooperative European Paediatric Renal Transplant Initiative (CERTAIN) Open
Background. This study by the Cooperative European Paediatric Renal Transplant Initiative (CERTAIN) was designed to determine the incidence, risk factors, current management strategies and outcomes of antibody-mediated rejection (ABMR) in …
View article: Verbesserte Versorgungs-und Behandlungsoptionen für Patienten mit Hyperphagie-assoziierter Adipositas bei Bardet-Biedl-Syndrom
Verbesserte Versorgungs-und Behandlungsoptionen für Patienten mit Hyperphagie-assoziierter Adipositas bei Bardet-Biedl-Syndrom Open
Zusammenfassung Das Bardet-Biedl-Syndrom (BBS) ist eine seltene, autosomal-rezessiv vererbte Multisystemerkrankung. Pathophysiologisch liegt eine Funktionsstörung des Primärziliums vor. Die Klinik ist heterogen und variabel und zeigt sich …
View article: Development of a tool for predicting HNF1B mutations in children and young adults with congenital anomalies of the kidneys and urinary tract
Development of a tool for predicting HNF1B mutations in children and young adults with congenital anomalies of the kidneys and urinary tract Open
Background We aimed to develop a tool for predicting HNF1B mutations in children with congenital abnormalities of the kidneys and urinary tract (CAKUT). Methods The clinical and laboratory data from 234 children and young adults with known…
View article: Development of a tool for predicting HNF1B mutations in children with congenital anomalies of the kidneys and urinary tract – a retrospective multicenter study
Development of a tool for predicting HNF1B mutations in children with congenital anomalies of the kidneys and urinary tract – a retrospective multicenter study Open
Background The diagnosis of HNF1B disease is a challenge. We aimed to developa tool for predicting HNF1B mutations in children with congenital abnormalities of the kidneys and urinary tract (CAKUT). Methods The clinical and laboratory data…
View article: Corrigendum: Molecular HLA mismatching for prediction of primary humoral alloimmunity and graft function deterioration in paediatric kidney transplantation
Corrigendum: Molecular HLA mismatching for prediction of primary humoral alloimmunity and graft function deterioration in paediatric kidney transplantation Open
[This corrects the article DOI: 10.3389/fimmu.2023.1092335.].
View article: Molecular HLA mismatching for prediction of primary humoral alloimmunity and graft function deterioration in paediatric kidney transplantation
Molecular HLA mismatching for prediction of primary humoral alloimmunity and graft function deterioration in paediatric kidney transplantation Open
Introduction Rejection remains the main cause of allograft failure in paediatric kidney transplantation and is driven by donor-recipient HLA mismatching. Modern computational algorithms enable assessment of HLA mismatch immunogenicity at t…
View article: Review of neurodevelopmental disorders in patients with HNF1B gene variations
Review of neurodevelopmental disorders in patients with HNF1B gene variations Open
This review investigates the association between neurodevelopmental disorders (NDD) and variations of the gene HNF1B. Heterozygous intragenetic mutations or heterozygous gene deletions (17q12 microdeletion syndrome) of HNF1B are the cause …
View article: Emulation of the control cohort of a randomized controlled trial in pediatric kidney transplantation with Real-World Data from the CERTAIN Registry
Emulation of the control cohort of a randomized controlled trial in pediatric kidney transplantation with Real-World Data from the CERTAIN Registry Open
Background Randomized controlled trials in pediatric kidney transplantation are hampered by low incidence and prevalence of kidney failure in children. Real-World Data from patient registries could facilitate the conduct of clinical trials…
View article: Refining Kidney Survival in 383 Genetically Characterized Patients With Nephronophthisis
Refining Kidney Survival in 383 Genetically Characterized Patients With Nephronophthisis Open
The presented data will enable clinicians to better estimate kidney prognosis of distinct patients with NPH and thereby allow personalized counseling.
View article: Development of an Interactive Dashboard for OSSE Rare Disease Registries
Development of an Interactive Dashboard for OSSE Rare Disease Registries Open
Background: The Open Source Registry System for Rare Diseases (OSSE), a web-based tool to create rare disease patient registries, currently offers no possibility to view aggregated registry data within the system. Here, we present the deve…
View article: Erratum zu: Studientagung der Gesellschaft für Pädiatrische Nephrologie – Grundlage innovativer Forschung
Erratum zu: Studientagung der Gesellschaft für Pädiatrische Nephrologie – Grundlage innovativer Forschung Open
View article: Progressive liver, kidney, and heart degeneration in children and adults affected by TULP3 mutations
Progressive liver, kidney, and heart degeneration in children and adults affected by TULP3 mutations Open
View article: Primary URECs: a source to better understand the pathology of renal tubular epithelia in pediatric hereditary cystic kidney diseases
Primary URECs: a source to better understand the pathology of renal tubular epithelia in pediatric hereditary cystic kidney diseases Open
View article: Early childhood height-adjusted total kidney volume as a risk marker of kidney survival in ARPKD
Early childhood height-adjusted total kidney volume as a risk marker of kidney survival in ARPKD Open
View article: mTOR-Activating Mutations in RRAGD Are Causative for Kidney Tubulopathy and Cardiomyopathy
mTOR-Activating Mutations in RRAGD Are Causative for Kidney Tubulopathy and Cardiomyopathy Open
Significance Statement Although advances in genetic techniques have resulted in the identification of rare hereditary disorders of renal magnesium and salt handling, some patients with tubulopathy lack a genetic diagnosis. In a cohort of p…
View article: Correction to: Different approaches to long-term treatment of aHUS due to MCP mutations: a multicenter analysis
Correction to: Different approaches to long-term treatment of aHUS due to MCP mutations: a multicenter analysis Open
View article: Refining genotype–phenotype correlations in 304 patients with autosomal recessive polycystic kidney disease and PKHD1 gene variants
Refining genotype–phenotype correlations in 304 patients with autosomal recessive polycystic kidney disease and PKHD1 gene variants Open