Bernd Höppe
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View article: Normative urinary excretion parameters for early identification of primary hyperoxaluria or other kidney stone associated diseases in infants < 8 weeks of life
Normative urinary excretion parameters for early identification of primary hyperoxaluria or other kidney stone associated diseases in infants < 8 weeks of life Open
Background A thorough set of reference values for lithogenic and stone inhibitory substances in urine is of great relevance for diagnostic purposes in patients with kidney stones or nephrocalcinosis. This is especially important for the ea…
View article: Crystal deposition triggers tubule dilation that accelerates cystogenesis in polycystic kidney disease
Crystal deposition triggers tubule dilation that accelerates cystogenesis in polycystic kidney disease Open
View article: Results of a multicenter, randomized trial examining a new transition model for post-kidney transplant adolescents
Results of a multicenter, randomized trial examining a new transition model for post-kidney transplant adolescents Open
View article: PHYOX3: Nedosiran Long-Term Safety and Efficacy in Patients With Primary Hyperoxaluria Type 1
PHYOX3: Nedosiran Long-Term Safety and Efficacy in Patients With Primary Hyperoxaluria Type 1 Open
View article: Effective Newborn Screening for Type 1 and 3 Primary Hyperoxaluria
Effective Newborn Screening for Type 1 and 3 Primary Hyperoxaluria Open
View article: The TRANSNephro-study examining a new transition model for post-kidney transplant adolescents - Results of a multicenter, randomized-controlled trial
The TRANSNephro-study examining a new transition model for post-kidney transplant adolescents - Results of a multicenter, randomized-controlled trial Open
Allograft loss after pediatric kidney transplantation (KTx) is highest in adolescents and young adults. Non-adherence and Health Care Transition (HCT) are important factors, but others do also contribute. Patients were randomized 1:1. The …
View article: Genetic kidney stones disease in adults
Genetic kidney stones disease in adults Open
View article: #1323 Kidney health in children with X-linked hypophosphatemia: lessons from the prospective multicenter study in Germany and Switzerland
#1323 Kidney health in children with X-linked hypophosphatemia: lessons from the prospective multicenter study in Germany and Switzerland Open
Background and Aims X-linked hypophosphatemia (XLH) is the most common genetic cause of hypophosphatemia. Mutations in the PHEX gene cause elevated circulating levels of fibroblast growth factor 23 (FGF23), phosphaturia, rickets and osteom…
View article: Nedosiran Safety and Efficacy in PH1: Interim Analysis of PHYOX3
Nedosiran Safety and Efficacy in PH1: Interim Analysis of PHYOX3 Open
View article: An update on clinical presentation and responses to therapy of patients with hereditary hypophosphatemic rickets with hypercalciuria (HHRH)
An update on clinical presentation and responses to therapy of patients with hereditary hypophosphatemic rickets with hypercalciuria (HHRH) Open
View article: The Importance of Detecting the Signs of Primary Hyperoxaluria—Cardiac Oxalosis in Primary Hyperoxaluria Type 1
The Importance of Detecting the Signs of Primary Hyperoxaluria—Cardiac Oxalosis in Primary Hyperoxaluria Type 1 Open
A 27-year-old man presented with influenza-like symptoms and rapidly progressing respiratory failure requiring mechanical ventilation and venovenous extracorporeal membrane oxygenation on the day of admission. With a medical history consis…
View article: Multicenter Long-Term Real World Data on Treatment With Lumasiran in Patients With Primary Hyperoxaluria Type 1
Multicenter Long-Term Real World Data on Treatment With Lumasiran in Patients With Primary Hyperoxaluria Type 1 Open
Lumasiran treatment is safe and efficient. Dosage (interval) adjustment necessities need clarification. In dialysis, lack of Pox reduction may relate to dissolving systemic oxalate deposits. Pglyc increment may be a considerable acid load …
View article: Is Genotype the Major Outcome Parameter of Kidney Failure in Patients With Primary Hyperoxaluria Type 1?
Is Genotype the Major Outcome Parameter of Kidney Failure in Patients With Primary Hyperoxaluria Type 1? Open
See Clinical Research on Page 2029 See Clinical Research on Page 2029 The primary hyperoxalurias are a group of autosomal recessive inherited rare inborn errors of glyoxylate metabolism. Three different deficient enzymes result in endogeno…
View article: Ratio of Urinary Proteins to Albumin Excretion Shifts Substantially during Progression of the Podocytopathy Alport Syndrome, and Spot Urine Is a Reliable Method to Detect These Pathologic Changes
Ratio of Urinary Proteins to Albumin Excretion Shifts Substantially during Progression of the Podocytopathy Alport Syndrome, and Spot Urine Is a Reliable Method to Detect These Pathologic Changes Open
The urinary albumin- and protein-to-creatinine ratios (UACR and UPCR, respectively) are key endpoints in most clinical trials assessing risk of progression of chronic kidney disease (CKD). For the first time, the current study compares the…
View article: Long-term outcome after combined or sequential liver and kidney transplantation in children with infantile and juvenile primary hyperoxaluria type 1
Long-term outcome after combined or sequential liver and kidney transplantation in children with infantile and juvenile primary hyperoxaluria type 1 Open
Introduction Combined or sequential liver and kidney transplantation (CLKT/SLKT) restores kidney function and corrects the underlying metabolic defect in children with end-stage kidney disease in primary hyperoxaluria type 1 (PH1). However…
View article: Simple, fast and inexpensive quantification of glycolate in the urine of patients with primary hyperoxaluria type 1
Simple, fast and inexpensive quantification of glycolate in the urine of patients with primary hyperoxaluria type 1 Open
In primary hyperoxaluria type 1 excessive endogenous production of oxalate and glycolate leads to increased urinary excretion of these metabolites. Although genetic testing is the most definitive and preferred diagnostic method, quantifica…
View article: Correction to: Three Tesla magnetic resonance imaging detects oxalate osteopathy in patients with primary hyperoxaluria type I
Correction to: Three Tesla magnetic resonance imaging detects oxalate osteopathy in patients with primary hyperoxaluria type I Open
View article: The retinal phenotype in primary hyperoxaluria type 2 and 3
The retinal phenotype in primary hyperoxaluria type 2 and 3 Open
Background The primary hyperoxalurias (PH1-3) are rare inherited disorders of the glyoxylate metabolism characterized by endogenous overproduction of oxalate. As oxalate cannot be metabolized by humans, oxalate deposits may affect various …
View article: Chronic liver disease and hepatic calcium-oxalate deposition in patients with primary hyperoxaluria type I
Chronic liver disease and hepatic calcium-oxalate deposition in patients with primary hyperoxaluria type I Open
View article: PHYOX2: a pivotal randomized study of nedosiran in primary hyperoxaluria type 1 or 2
PHYOX2: a pivotal randomized study of nedosiran in primary hyperoxaluria type 1 or 2 Open
Nedosiran is an investigational RNA interference agent designed to inhibit expression of hepatic lactate dehydrogenase, the enzyme thought responsible for the terminal step of oxalate synthesis. Oxalate overproduction is the hallmark of al…
View article: Improving Treatment Options for Primary Hyperoxaluria
Improving Treatment Options for Primary Hyperoxaluria Open
The primary hyperoxalurias are three rare inborn errors of the glyoxylate metabolism in the liver, which lead to massively increased endogenous oxalate production, thus elevating urinary oxalate excretion and, based on that, recurrent urol…
View article: Relationship between age at initiation of cysteamine treatment, adherence with therapy, and glomerular kidney function in infantile nephropathic cystinosis
Relationship between age at initiation of cysteamine treatment, adherence with therapy, and glomerular kidney function in infantile nephropathic cystinosis Open
Infantile nephropathic cystinosis, due to impaired transport of cystine out of lysosomes, occurs with an incidence of 1 in 100-200,000 live births. It is characterized by renal Fanconi syndrome in the first year of life and glomerular dysf…
View article: Improved Outcome of Infantile Oxalosis Over Time in Europe: Data From the OxalEurope Registry
Improved Outcome of Infantile Oxalosis Over Time in Europe: Data From the OxalEurope Registry Open
View article: Chronic liver disease and hepatic calcium-oxalate deposition in patients with primary hyperoxaluria type I
Chronic liver disease and hepatic calcium-oxalate deposition in patients with primary hyperoxaluria type I Open
Patients with primary hyperoxaluria type I (PHI) are prone to develop early kidney failure. Systemic deposition of calcium-oxalate (CaOx) crystals starts, when renal function declines and plasma oxalate (Pox) increases. All tissue, but esp…
View article: Clinical and economic impact of primary hyperoxaluria: a retrospective claims analysis
Clinical and economic impact of primary hyperoxaluria: a retrospective claims analysis Open
BACKGROUND: Primary hyperoxaluria (PH) is a family of rare, life-threatening genetic liver disorders characterized by elevated production and excretion of oxalate. To date, the clinical and economic burden associated with PH has not…
View article: New Aspects of Kidney Fibrosis–From Mechanisms of Injury to Modulation of Disease
New Aspects of Kidney Fibrosis–From Mechanisms of Injury to Modulation of Disease Open
Organ fibrogenesis is characterized by a common pathophysiological final pathway independent of the underlying progressive disease of the respective organ. This makes it particularly suitable as a therapeutic target. The Transregional Coll…
View article: Long-Term Transplantation Outcomes in Patients With Primary Hyperoxaluria Type 1 Included in the European Hyperoxaluria Consortium (OxalEurope) Registry
Long-Term Transplantation Outcomes in Patients With Primary Hyperoxaluria Type 1 Included in the European Hyperoxaluria Consortium (OxalEurope) Registry Open
View article: Diet-related urine collections: assistance in categorization of hyperoxaluria
Diet-related urine collections: assistance in categorization of hyperoxaluria Open
View article: Correction to: Endurance-oriented training program with children and adolescents on maintenance hemodialysis to enhance dialysis efficacy—DiaSport
Correction to: Endurance-oriented training program with children and adolescents on maintenance hemodialysis to enhance dialysis efficacy—DiaSport Open
View article: Safety, pharmacodynamics, and exposure-response modeling results from a first-in-human phase 1 study of nedosiran (PHYOX1) in primary hyperoxaluria
Safety, pharmacodynamics, and exposure-response modeling results from a first-in-human phase 1 study of nedosiran (PHYOX1) in primary hyperoxaluria Open