Jérôme Harambat
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View article: #3416 Development and validation of a prediction model for progression of CKD in European children
#3416 Development and validation of a prediction model for progression of CKD in European children Open
Background and Aims Families and children with chronic kidney disease (CKD) often request prognostic information regarding their child's condition in order to understand its implications and plan for the future. However, prediction of when…
View article: #413 Effects of long-term GH treatment on FGF23/Klotho axis in pediatric patients with CKD
#413 Effects of long-term GH treatment on FGF23/Klotho axis in pediatric patients with CKD Open
Background and Aims Chronic kidney disease (CKD) is characterized by low levels of the anti-aging protein α-Klotho. Growth hormone (GH) is thought to stimulate calcitriol production and the phosphaturic and vascular actions of FGF23 by upr…
View article: #412 Reduced arterial stiffness in children with CKD under long-term growth hormone treatment
#412 Reduced arterial stiffness in children with CKD under long-term growth hormone treatment Open
Background and Aims Chronic kidney disease (CKD) is characterized by low levels of the anti-aging protein α-Klotho and accelerated cardiovascular (CV) morbidity. Reduced levels of sKlotho are associated with premature aging and CV disease.…
View article: Long-term lumasiran therapy final results from a Phase 2 open-label extension study in primary hyperoxaluria
Long-term lumasiran therapy final results from a Phase 2 open-label extension study in primary hyperoxaluria Open
Background Primary hyperoxaluria type 1 (PH1) is a rare genetic disorder of hepatic oxalate overproduction leading to kidney failure and systemic oxalosis. Lumasiran is an RNA interference therapeutic approved for lowering urinary oxalate …
View article: Regional variation of underlying kidney diseases in children undergoing chronic kidney replacement therapy around the globe
Regional variation of underlying kidney diseases in children undergoing chronic kidney replacement therapy around the globe Open
Background There is a scarcity of demographic information regarding the distribution of the diseases leading to kidney failure (KF) in children living in the emerging world. We used data from international, regional and national registries…
View article: Health-related quality of life in French pediatric patients with X-linked hypophosphatemia: real-world data from the International XLH Registry
Health-related quality of life in French pediatric patients with X-linked hypophosphatemia: real-world data from the International XLH Registry Open
X-linked hypophosphatemia (XLH) is a phosphate-wasting disorder caused by increased fibroblast growth factor 23 (FGF23); it leads to skeletal deformities, muscle weakness, and pain. In a pediatric phase 3 trial, the FGF23 inhibitor burosum…
View article: Optimizing ADAMTS13 prophylaxis to reduce relapse and organ failure in congenital thrombotic thrombocytopenic purpura
Optimizing ADAMTS13 prophylaxis to reduce relapse and organ failure in congenital thrombotic thrombocytopenic purpura Open
Congenital thrombotic thrombocytopenic purpura (cTTP) is caused by a severe inherited ADAMTS13 deficiency. While acute episodes are life-threatening, long-term burden of ischemic complications and effectiveness of prophylactic strategies r…
View article: Impact of adverse childhood experiences on kidney health
Impact of adverse childhood experiences on kidney health Open
View article: 6-year treatment follow-up with an extended-release alkaline formulation (Sibnayal®) in primary distal renal tubular acidosis
6-year treatment follow-up with an extended-release alkaline formulation (Sibnayal®) in primary distal renal tubular acidosis Open
Background Distal renal tubular acidosis (dRTA) is a rare disease characterized by hyperchloremic metabolic acidosis affecting growth, bone and kidney health. Methods The aim of B22CS study was to evaluate long-term safety and efficacy (an…
View article: Growth hormone treatment associates with improved circulating anti-aging protein Klotho and reduced arterial stiffness in children with CKD
Growth hormone treatment associates with improved circulating anti-aging protein Klotho and reduced arterial stiffness in children with CKD Open
Background Chronic kidney disease (CKD) is characterized by low levels of the anti-aging protein α-Klotho and accelerated cardiovascular (CV) morbidity. Short-term treatment with growth hormone (GH) was shown to enhance soluble Klotho (sKl…
View article: GDF-15 and uEGF Independently Associate With CKD Progression in Children
GDF-15 and uEGF Independently Associate With CKD Progression in Children Open
View article: Population pharmacokinetic modelling of prednisolone in systemic lupus erythematosus patients: Analysis of exposure and disease activity
Population pharmacokinetic modelling of prednisolone in systemic lupus erythematosus patients: Analysis of exposure and disease activity Open
Aims Prednisone is a widely used glucocorticoid in the treatment of lupus, although its dosing is often determined empirically. Prednisolone, the active metabolite of prednisone, is found in its free form in the serum. The goal of this stu…
View article: Corrigendum to “Genotype-Phenotype Correlations in Denys-Drash Syndrome in Children” [Kidney International Reports Volume 10, Issue 4, April 2025, Pages 1205-1212]
Corrigendum to “Genotype-Phenotype Correlations in Denys-Drash Syndrome in Children” [Kidney International Reports Volume 10, Issue 4, April 2025, Pages 1205-1212] Open
View article: Progression of Carotid Intima‐Media Thickness in Children of the Cardiovascular Comorbidity in Children With Chronic Kidney Disease Study: Risk Factors and Impact of Blood Pressure Dynamics
Progression of Carotid Intima‐Media Thickness in Children of the Cardiovascular Comorbidity in Children With Chronic Kidney Disease Study: Risk Factors and Impact of Blood Pressure Dynamics Open
Background Carotid intima‐media thickness (cIMT) may identify early alterations in the vascular phenotype in children with chronic kidney disease (CKD). Methods and Results Investigation of longitudinal changes in cIMT SD scores (SDS) in 6…
View article: Longitudinal Lipid Trajectories and Progression of CKD in Children
Longitudinal Lipid Trajectories and Progression of CKD in Children Open
View article: Access to kidney transplantation and re-transplantation from childhood to adulthood: long-term data from the ERA Registry
Access to kidney transplantation and re-transplantation from childhood to adulthood: long-term data from the ERA Registry Open
Background and hypothesis Knowledge regarding access to first kidney transplantation (KT) and subsequent KT in patients commencing kidney replacement therapy (KRT) in childhood is limited. Methods Using European Renal Association (ERA) Reg…
View article: International validation of a pre-transplant risk assessment tool for graft survival in pediatric kidney transplant recipients
International validation of a pre-transplant risk assessment tool for graft survival in pediatric kidney transplant recipients Open
Background A pre-transplant prediction model using commonly available factors is valuable for optimizing donor selection, communication, and counseling for pediatric kidney transplant (PKT) recipients. This study aims to externally validat…
View article: Genotype-Phenotype Correlations in Denys-Drash Syndrome in Children
Genotype-Phenotype Correlations in Denys-Drash Syndrome in Children Open
In our cohort, children's DDS clinical trajectory was associated with exon localization. In the era of genomic newborn screening, depicting genetic risk is of utmost importance for personalized patient care.
View article: Long-term clinical outcomes with an average of 6 years follow-up in dRTA patients treated with ADV7103 an oral twice-daily fixed and prolonged-release combination of potassium bicarbonate and potassium citrate (Sibnayal®).
Long-term clinical outcomes with an average of 6 years follow-up in dRTA patients treated with ADV7103 an oral twice-daily fixed and prolonged-release combination of potassium bicarbonate and potassium citrate (Sibnayal®). Open
Background: Distal renal tubular acidosis (dRTA) is a rare disease characterized by hyperchloremic metabolic acidosis affecting growth, bone and kidney health. Methods: The aim of B22CS study was to evaluate long-term safety and efficacy o…
View article: Cystinosis metabolic bone disease: inflammatory profile in human peripheral blood mononuclear cells and derived osteoclasts
Cystinosis metabolic bone disease: inflammatory profile in human peripheral blood mononuclear cells and derived osteoclasts Open
Cystinosis metabolic bone disease (CMBD) is an emerging concept in infantile nephropathic cystinosis, patients presenting with bone pains, fractures, and deformations during teenage or early adulthood. The underlying mechanisms remain uncl…
View article: Clinical outcomes of pediatric kidney replacement therapy after childhood cancer—An ESPN/ERA Registry study
Clinical outcomes of pediatric kidney replacement therapy after childhood cancer—An ESPN/ERA Registry study Open
View article: #1122 Anaemia in CKD and cardiovascular risk; all cut from the same cloth?
#1122 Anaemia in CKD and cardiovascular risk; all cut from the same cloth? Open
Background and Aims Anaemia in chronic kidney disease (CKD) has been repeatedly associated with increased cardiovascular morbidity and mortality, but most studies have not investigated its longitudinal association by sex and age. Our aim w…
View article: #190 Long-term treatment with lumasiran: final results from the phase 2 open-label extension study
#190 Long-term treatment with lumasiran: final results from the phase 2 open-label extension study Open
Background and Aims Primary hyperoxaluria type 1 (PH1) is a rare genetic disorder in which hepatic oxalate overproduction can lead to kidney failure and life-threatening systemic disease. Lumasiran, an RNA interference (RNAi) therapeutic i…
View article: Social Deprivation and Incidence of Pediatric Kidney Failure in France
Social Deprivation and Incidence of Pediatric Kidney Failure in France Open
View article: Metabolic Acidosis Is Associated With an Accelerated Decline of Allograft Function in Pediatric Kidney Transplantation
Metabolic Acidosis Is Associated With an Accelerated Decline of Allograft Function in Pediatric Kidney Transplantation Open
The degree of metabolic acidosis is associated with allograft dysfunction.
View article: High Amino Acid Intake in Early Life Is Associated With Systolic but Not Diastolic Arterial Hypertension at 5 Years of Age in Children Born Very Preterm
High Amino Acid Intake in Early Life Is Associated With Systolic but Not Diastolic Arterial Hypertension at 5 Years of Age in Children Born Very Preterm Open
Background The life course of individuals born very premature is a topic of increasing concern. The association between high early amino acid intake and later high blood pressure (HBP) in preterm neonates is debated. Methods and Results In…
View article: Decline in Left Ventricular Early Systolic Function with Worsening Kidney Function in Children with Chronic Kidney Disease: Insights from the 4C and HOT-KID Studies
Decline in Left Ventricular Early Systolic Function with Worsening Kidney Function in Children with Chronic Kidney Disease: Insights from the 4C and HOT-KID Studies Open
View article: Determinants of Kidney Failure in Primary Hyperoxaluria Type 1: Findings of the European Hyperoxaluria Consortium
Determinants of Kidney Failure in Primary Hyperoxaluria Type 1: Findings of the European Hyperoxaluria Consortium Open
View article: Stricter Blood Pressure Control Is Associated With Lower Left Ventricular Mass in Children After Kidney Transplantation: A Longitudinal Analysis of the 4C-T Study
Stricter Blood Pressure Control Is Associated With Lower Left Ventricular Mass in Children After Kidney Transplantation: A Longitudinal Analysis of the 4C-T Study Open
BACKGROUND: We assessed the effect of blood pressure (BP) control on left ventricular mass index (LVMI) and left ventricular hypertrophy (LVH). METHODS: Ninety-six patients (64 males) ≥9 months post–kidney transplantation from the 4C-T (Ca…
View article: Efficacy and Safety of Eculizumab in Pediatric Patients Affected by Shiga Toxin–Related Hemolytic and Uremic Syndrome: A Randomized, Placebo-Controlled Trial
Efficacy and Safety of Eculizumab in Pediatric Patients Affected by Shiga Toxin–Related Hemolytic and Uremic Syndrome: A Randomized, Placebo-Controlled Trial Open
Significance Statement Shiga toxin–related hemolytic uremic syndrome (STEC-HUS) is a serious condition, characterized by multiorgan thrombotic microangiopathy, mainly affecting children. Renal involvement is severe, with approximately half…