Jérôme Harambat

• MD, PhD
• Consultant at Centre Hospitalier Universitaire de Bordeaux

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 670 patients from the 4C Study (Cardiovascular Comorbidity in Children With CKD Study), aged 6 to 17 years, w...

Autosomal dominant polycystic kidney disease (ADPKD) is the most common genetic kidney disorder. Pathogenic variants in PKD1 and PKD2 genes are the main causes of ADPKD. Biallelic inheritance of pathogenic variants leading to very early-onset manifestations have been described in the literature. A female fetus was prenatally diagnosed with oligohyd...

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) Registry data, we investigated European patients who started KRT below 20 years of age between 1978 and 2019. Acc...

Background A pre-transplant prediction model using commonly available factors is valuable for optimizing donor selection, communication, and counselling for pediatric kidney transplant (PKT) recipients. This study aims to externally validate a Dutch PKT prediction model and assess its international applicability. Methods Data from the Dutch-, CERT...

Introduction Denys-Drash syndrome (DDS) is a rare disease typically associated with a triad of early onset nephrotic syndromes (NS), susceptibility to Wilms tumor (WT), and genitourinary structural defects. DDS is caused by Wilms’ tumor suppression gene (WT1) variants, with the most frequent variants in exons 8 and 9. This study aimed to evaluate t...

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 unclear. Our aim was to explore the pro-inflammatory profile of osteoclastic lineage in cystinotic patients. We obt...

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 of ADV7103, a prolonged-release alkalizing formulation with twice daily dosing, in children and adults with dRTA...

Rationale: The progression of cardiovascular disease (CVD) in children with chronic kidney disease (CKD) is not well understood. Objective: To investigate carotid intima-media thickness (cIMT) as a surrogate marker for CVD in 670 patients of the 4C Study (The Cardiovascular Comorbidity in Children with CKD Study), aged 6 - 17 years, with CKD stage...

Introduction The choice and timing of kidney replacement therapy (KRT) is influenced by clinical factors, laboratory features, feasibility issues, family preferences, and clinicians' attitudes. We analyzed the factors associated with KRT modality and timing in a multicenter, multinational prospective pediatric cohort study. Methods A total of 695...

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 was to study the association between haemoglobin (Hb) current value and the hazard of major adverse cardiovascul...

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 indicated for treatment of PH1 to lower urinary oxalate (UOx) and plasma oxalate (POx) levels, reduces hepatic o...

Introduction Approximately 8 per million children and young adults aged < 20 years initiate kidney replacement therapy (KRT) per year in France. We hypothesize that social deprivation could be a determinant of childhood-onset kidney failure. The objective of this study was to estimate the incidence of pediatric KRT in France according to the level...

Background Children requiring kidney replacement therapy experience high burden of cardiovascular (CV) disease leading to increased mortality. Intima–media thickness (IMT) indicating atherosclerosis is a validated surrogate marker for future CV events. Methods We investigated the effect of different treatment modalities (dialysis, preemptive kidne...

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 a national, prospective, population‐based birth cohort, EPIPAGE‐2 (Etude Epidémiologique sur Petits Ages Gest...

Background Recent evidence suggests overestimation of benefits associated with arteriovenous (AV) fistula versus graft in certain populations. We assessed hazards of all-cause and cause-specific hospitalization and death associated with AV access type in patients who started hemodialysis with a catheter in France, overall and by subgroups of age, s...

Background Trajectories of haemoglobin in patients with chronic kidney disease (CKD) have been poorly described. In such patients, we aimed to identify typical haemoglobin trajectory profiles and estimate their risks of major adverse cardiovascular events (MACE). Methods We used 5-year longitudinal data from the CKD-REIN cohort patients with moder...

BACKGROUND. The life course of individuals born very premature is a topic of increasing concern as their neonatal survival has dramatically increased. In a national, prospective, population-based birth cohort, EPIPAGE-2, we observed that amino-acid intakes greater than 3.5 g/kg/day at day 7 after birth were independently associated with higher inte...

Introduction Primary hyperoxaluria type 1 (PH1) has a highly heterogeneous disease course. Apart from the c.508G>A (p.Gly170Arg) AGXT variant, which imparts a relatively favorable outcome, little is known about determinants of kidney failure. Identifying these is crucial for disease management, especially in this era of new therapies. Methods In t...

Background: We assessed the effect of blood pressure 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 (Cardiovascular Comorbidity in Children with Chronic Kidney Disease-Transplantation) study were analyzed longitudina...

Background and Aims Anemia in chronic kidney disease (CKD) has been repeatedly associated with increased cardiovascular and all-cause morbidity and mortality. Most observational studies have focused on one single measurement of hemoglobin, but very few have investigated the trajectory of hemoglobin over time. Our aim was to identify, in moderate to...

Background and Aims Knowledge regarding graft survival after transplantation in European children with multiple kidney transplants followed from childhood into adulthood including factors affecting these outcomes is lacking. Method Using ERA Registry data, we investigated all patients on kidney replacement therapy (KRT) who received their first ki...

Background and Aims Distal renal tubular acidosis (dRTA) is a rare disease, inherited or acquired, characterized by hyperchloremic metabolic acidosis leading to negative effects on growth, bone and kidney, with growth retardation, rickets in children and osteomalacia in adults, nephrolithiasis and chronic kidney disease. No alkalizing treatment, ne...

Background and Aims Iron deficiency is a frequent condition in moderate to severe chronic kidney disease (CKD), often left under-screened, and infrequently supplemented. Iron deficiency is a major contributor to anemia, which is known to negatively impact outcomes and quality of life in CKD. However, evidence of an anemia-independent effect of iron...

Background: Shiga toxin-related hemolytic uremic syndrome (STEC-HUS) in children is a severe condition, resulting in approximately 50% of patients requiring renal replacement therapy (RRT). Furthermore, at least 30% of survivors experience kidney sequelae. Recently, activation of the complement alternative pathway has been postulated as a factor i...

Background: Associations between anthropometric measures and patient outcomes in children are inconsistent and mainly based on data at kidney replacement therapy (KRT) initiation. We studied associations of height and body mass index (BMI) with access to kidney transplantation, graft failure, and death during childhood KRT. Methods: We included...

IgA vasculitis (IgAV) is the most common vasculitis in children. IgAV long-term prognosis depends on kidney involvement or IgA vasculitis with nephritis (IgAVN). To date, steroid treatment (oral steroids or methylprednisolone pulses) has not proven to be formally efficient. This study aimed to assess the role of steroids on IgAVN outcome. All child...

Nephronophthisis (NPH) is an autosomal-recessive ciliopathy representing one of the most frequent causes of kidney failure in childhood characterized by a broad clinical and genetic heterogeneity. Applied to one of the worldwide largest cohorts of patients with NPH, genetic analysis encompassing targeted and whole exome sequencing identified diseas...

Children with CKD and their family or caregivers will experience complex decisions on timing of kidney replacement therapy initiation and treatment modality choice that may significantly impact kidney health and psychosocial adjustment. Timely discussions regarding planning of kidney replacement therapy should be multidisciplinary including patient...

Background: Childhood-onset chronic kidney disease is a progressive condition that can have a major effect on life expectancy and quality. We evaluated the usefulness of the kidney tubular cell stress marker urinary Dickkopf-related protein 3 (DKK3) in determining the short-term risk of chronic kidney disease progression in children and identifyin...

Major advances have been made in the management of children with chronic kidney disease (CKD) over the past 30 years. However, existing epidemiological data mainly relies on registries of chronic kidney replacement therapy. The incidence and prevalence of earlier stages of CKD remain largely unknown, but rare population-based studies suggest that t...

We investigated the shape of the relationship between longitudinal uric acid (UA) and the hazard of kidney failure and death in chronic kidney disease (CKD) patients, and attempted to identify thresholds associated with increased hazards. We included CKD stage 3–5 patients from the CKD-REIN cohort with one serum UA measurement at cohort entry. We u...

The recommended equation to estimate the value of glomerular filtration rate (eGFR) among children is Schwartz equation updated in 2009. However, it is few frequently used because involves height, a factor rarely documented for laboratories, especially hospital laboratories. The FAS (Full Aged Spectrum) formula developed by a European group, allows...

Background COVID-19 is a complex multisystem disease, frequently associated with kidney injury. Since the beginning of the COVID-19 pandemic, we observed a striking increase in the incidence of acute tubulointerstitial nephritis (aTIN) without or with uveitis (TINUs) among children. This prompted us to examine whether SARS-CoV-2 might be the underl...

Background Data on comorbidities in children on kidney replacement therapy (KRT) is scarce. Considering their high relevance for prognosis and treatment, this study aims to analyse the prevalence and implications of comorbidities in European children on KRT. Methods We included data from patients aged < 20 years when commencing KRT from 2007 to 20...

Introduction Primary infection or reactivation of Epstein-Barr Virus (EBV) is a significant cause of morbidity and mortality in pediatric kidney transplantation. Valganciclovir (VGC) treatment is recommended for prophylaxis of cytomegalovirus infection, but its role for the prevention of EBV infection remains controversial. Patients and methods Al...

Background: Pediatric ANCA vasculitis is a rare group of diseases with a scarcity of data in children. Annual incidence appeared to increase in the last several years, placing higher interest in the clinical and therapeutical outcomes of the disorder. Also, the growing use of rituximab questions the latest outcomes in these diseases. We therefore...

Arteriovenous (AV) access choice has sparked controversy with recent evidence suggesting overestimation of benefits associated with AV fistula versus graft in certain populations. We assessed outcomes associated with first-line AV access type in patients who started hemodialysis with a catheter in France, overall and by subgroups of age, sex, and c...

Background Cytomegalovirus (CMV) is one of the most frequent opportunistic infections in kidney transplant (KT) recipients and is a risk factor for patient and graft survival after KT. Center-to-center variation, optimal prevention and treatment strategies in pediatric KT are currently unknown. This survey aimed to assess current CMV prevention and...

Introduction: Vitamin D dependent rickets type 1A (VDDR1A) is a rare genetic disease associated with loss-of-function variations in the gene encoding the vitamin D activating enzyme 1α-hydroxylase (CYP27B1). Phenotype-genotype correlation is unclear. Long-term outcome data are lacking. The objective of this study was to describe characteristics an...

The global burden of chronic kidney disease remains high, and the cost of caring for patients with CKD is substantial, especially if end-stage kidney disease occurs. The incidence and prevalence of chronic kidney disease is understudied in children, and most of our data and current understanding are derived from large national registries of childre...

Introduction Dans la population des patients malades rénaux chroniques (MRC), la carence martiale est une complication fréquente, sous-diagnostiquée et associée à une augmentation de la morbi-mortalité notamment cardiovasculaire. La prise en charge de la carence martiale dans cette population MRC non suppléée est controversée. Notre objectif était...

Introduction L’évolution du taux d’hémoglobine au cours du temps est encore peu étudiée chez les patients atteints de maladie rénale chronique (MRC). Notre objectif était d’identifier, au sein de la population MRC non suppléée, des classes distinctes d’évolution d’hémoglobine et d’estimer pour chaque classe, le risque de présenter un évènement card...

RÉSUMÉ À l’occasion des 20 ans du REIN (Réseau Epidémiologie et Information en Néphrologie), un travail de synthèse sur les apports du registre a été mené. Sur la question des patients pédiatriques, les messages clés suivants ont été retenus. La maladie rénale chronique (MRC) stade 5 pédiatrique a des particularités qui nécessitent d’être analysées...

BACKGROUND AND AIMS Vascular access choice for patients with high risk of arteriovenous (AV) access failure has been sparking growing controversy as recent studies show similar survival and morbidity across patients receiving arteriovenous (AV) fistula or graft. We assessed hospitalization and mortality risks associated with access type in patients...

Introduction Les fistules artérioveineuses sont la voie d'abord de prédilection chez les patients en hémodialyse (HD). Toutefois, l’échec de maturation des fistules peut être fréquent selon l'âge, le sexe et la présence de comorbidités, et associé à une morbidité élevée. L'objectif de cette étude a été de comparer le risque d'hospitalisation et de...

Introduction Infantile oxalosis is the most severe form of primary hyperoxaluria type 1 (PH1), with onset of end-stage kidney disease (ESKD) during infancy. We aimed to analyze the outcome of these patients as our current understanding is limited due to a paucity of reports. Methods A retrospective registry study was conducted using data from the...

Objectives Nutritional vitamin D supplements are often used in general pediatrics. Here, the aim is to address vitamin D supplementation and calcium nutritional intakes in newborns, infants, children, and adolescents to prevent vitamin D deficiency and rickets in general populations. Study design We formulated clinical questions relating to the fo...

Background: To improve pre-emptive kidney transplantation (PKT) in children and limit starting dialysis in an emergency, we aimed to describe nephrology care trajectories pre-CKD stage 5. Methods: We included all children in France who, between 2010 and 2016, started kidney replacement therapy (KRT): standard dialysis (reference group) and emerg...

Background Lumasiran, a sub-cutaneous RNA-interference therapy, has been recently approved for primary hyperoxaluria type 1 (PH1), with doses and intervals according to body weight. Little is known as to its use in infants; the aim of this study was to describe treatment outcome in 3 infants who received lumasiran therapy before 2 years of age.Case...

Background There seems to be a possible link between nephrotic syndrome (NS) and lymphoproliferative syndrome, but it remains poorly understood.Methods This multicentric and retrospective study focuses on children, who developed idiopathic NS and malignant or benign proliferation between 2000 and 2021.ResultsEleven patients were included, with a me...

Introduction Socioeconomic status is recognized as an important determinant of kidney health. We aimed to assess the association of social deprivation with different indicators at kidney replacement therapy (KRT) initiation in the French pediatric metropolitan population. Methods All end-stage kidney disease (ESKD) patients who started KRT before...

Mortality in children with kidney failure is higher in girls than boys with cardiovascular complications representing the most common causes of death. Pulse wave velocity (PWV), a measure of vascular stiffness, predicts cardiovascular mortality in adults. Here, PWV in children with kidney failure undergoing kidney replacement therapy was investigat...

Autosomal recessive polycystic kidney disease (ARPKD) is characterized by bilateral fibrocystic changes resulting in pronounced kidney enlargement. Impairment of kidney function is highly variable and widely available prognostic markers are urgently needed as a base for clinical decision-making and future clinical trials. In this observational stud...

Rationale and objective There is a dearth of data characterizing patients requiring kidney replacement therapy (KRT) for kidney failure due to systemic lupus erythematosus (SLE) and their clinical outcomes. The aim of this study was to describe trends in incidence and prevalence of KRT among these patients as well as to compare their outcomes to pa...

Assessing the initial severity of immunoglobulin A vasculitis nephritis (IgAV-N) is important due to its determining effect on kidney management and outcomes. This paper describes a multicentre paediatric cohort of IgAV-N patients and discusses relationships among clinical presentation, histological features, and kidney outcome. We retrospectively...

Résumé Des progrès majeurs ont été réalisés dans la prise en charge des enfants avec une maladie rénale chronique au cours des 30 dernières années. Cependant, les données épidémiologiques pédiatriques existantes proviennent essentiellement des registres de traitement de suppléance de l’insuffisance rénale terminale, et les informations disponibles...

Introduction L’évolution de l’hémoglobinémie et les seuils associés à un risque accru de morbi-mortalité sont mal connus chez les sujets présentant une maladie rénale chronique (MRC) non suppléée. Notre objectif était d’identifier des sous-populations de MRC partageant un profil d’évolution similaire de l’hémoglobinémie et d’estimer leur risque d’i...

Introduction Socioeconomic status is recognized as an important determinant of kidney health. We aimed to assess the association of social deprivation with different indicators at kidney replacement therapy (KRT) initiation in the French pediatric population. Description Data from the REIN registry. Methods All end-stage kidney disease (ESKD) pat...

Growth retardation is a major complication in children with chronic kidney disease (CKD) and on kidney replacement therapy (KRT). Conversely, better growth in childhood CKD is associated with an improvement in several hard morbidity–mortality endpoints. Data from pediatric international registries has demonstrated that improvements in the overall c...

Background and objectives In the rare disease primary hyperoxaluria type 1, overproduction of oxalate by the liver causes kidney stones, nephrocalcinosis, kidney failure, and systemic oxalosis. Lumasiran, an RNA interference therapeutic, suppresses glycolate oxidase, reducing hepatic oxalate production. The objective of this first-in-human, randomi...

Background: Many centers accept a minimum body weight of 10 kg as threshold for kidney transplantation (Tx) in children. As solid evidence for clinical outcomes in multinational studies is lacking, we evaluated practices and outcomes in European children weighing below 10 kg at Tx. Methods: Data were obtained from the ESPN/ERA-EDTA Registry on a...

Background: Kidney transplantation (KT) is the optimal treatment for children with end-stage kidney disease (ESKD). The aim of this study was to evaluate the impact of PKT and of pretransplant dialysis duration on graft survival among French pediatric kidney transplant recipients. Methods: We analyzed all first pediatric kidney-only transplantat...

Assessing the initial severity of immunoglobulin A vasculitis nephritis (IgAV-N) is challenging important due to its determining effect on kidney management and outcomes. This study paper aims to describe describes a multicentre paediatric multicenter pediatric cohort of IgAV-N patients and discusses whilst investigating the relationships among bet...

Chronic kidney disease (CKD) is a major health problem because of its high prevalence, associated complications and high treatment costs. Several aspects of CKD differ significantly in the Eastern European nephrology community compared with Western Europe because of different geographic, socio-economic, infrastructure, cultural and educational feat...

Background A new prolonged-release formulation of potassium citrate and potassium bicarbonate, ADV7103, has been shown to improve metabolic control, palatability, and gastrointestinal safety in patients with distal renal tubular acidosis (dRTA) when compared to standard of care (SoC) treatments. The present work evaluates safety and efficacy of ADV...

La néphrocalcinose est définie par des dépôts de phosphate de calcium ou d’oxalate de calcium dans le parenchyme rénal, en particulier dans les cellules épithéliales des tubules rénaux et dans le tissu interstitiel. Il faut la différencier des néphrolithiases où les dépôts calciques se situent dans les cavités excrétrices rénales. La néphrocalcinos...

Background In patients with primary hyperoxaluria (PH), endogenous oxalate overproduction increases urinary oxalate excretion, leading to compromised kidney function and often kidney failure. Highly elevated plasma oxalate (Pox) is associated with systemic oxalate deposition in patients with PH and severe chronic kidney disease (CKD). The relations...

Background For 10 consecutive years, the ESPN/ERA-EDTA Registry has included data on children with stage 5 chronic kidney disease (CKD 5) receiving kidney replacement therapy (KRT) in Europe. We examined trends in incidence and prevalence of KRT and patient survival. Methods We included all children aged <15 years starting KRT 2007–2016 in 22 Euro...

Résumé La néphrocalcinose est définie par des dépôts de phosphate de calcium ou d’oxalate de calcium dans le parenchyme rénal, en particulier dans les cellules épithéliales des tubules rénaux et dans le tissu interstitiel. Il faut la différencier des néphrolithiases où les dépôts calciques se situent dans les cavités excrétrices rénales. La néphroc...

Background: Several models have been proposed to predict kidney graft failure in adult recipients but none in younger recipients. Our objective was to propose a dynamic prediction model for graft failure in young kidney transplant recipients. Methods: We included 793 kidney transplant recipients waitlisted before the age of 18 years who received...

The published version of the article unfortunately contained a mistake.

Introduction: Guidelines for the treatment of steroid-dependent nephrotic syndrome (SDNS) and frequently relapsing nephrotic syndrome (FRNS) are lacking. Given the substantial impact of SDNS/FRNS on quality of life, strategies aiming to provide long-term remission while minimising treatment side effects are needed. Several studies confirm that rit...

Introduction La transplantation rénale pédiatrique présente des spécificités, notamment chez les receveurs les plus jeunes. À ce jour, aucun modèle prédictif de survie du greffon n’existe chez les jeunes receveurs pour aider à la sélection du donneur. Description Notre objectif était de développer et valider un modèle prédictif de survie du greffo...

Introduction L’identification de facteurs de progression de la maladie rénale chronique (MRC) est primordiale. L’association entre la bicarbonatémie et l’insuffisance rénale chronique terminale traitée (IRTT) et la mortalité chez les patients présentant une MRC n’a pas été étudiée à l’aide de données longitudinales. Description Nous avons inclus l...

Background Mycophenolic acid (MPA), the active compound of mycophenolate mofetil (MMF), is widely used in lupus nephritis treatment. Therapeutic drug monitoring of adults suggests that area under the concentration-time curve (AUC) of MPA (MPA-AUC) is associated with clinical outcomes, but childhood data are scarce.Methods Retrospective study of 27...

Background Distal renal tubular acidosis (dRTA), due to impaired acid secretion in the urine, can lead to severe long-term consequences. Standard of care (SoC) oral alkalizers, requiring several daily intakes, are currently used to restore normal plasma bicarbonate levels. A new prolonged-release formulation, ADV7103, has been developed to achieve...

Background and Aims The association between hyperuricemia and CKD progression is not well established in Europe and, to our knowledge, has not yet been investigated using longitudinal measurements. Method We used data from CKD-REIN, a prospective cohort study conducted in 40 representative French nephrology clinics, which included 3033 patients wi...

Background and Aims The ESPN/ERA-EDTA Registry includes data on children with end-stage kidney disease receiving renal replacement therapy (RRT) in Europe for 10 consecutive years. We examined time trends in incidence, prevalence and patient survival for paediatric RRT in Europe. Method We included all children aged < 15 years starting RRT in 22 E...

Background and Aims Few studies have focused on trends of hypertension (HTN) and status of blood pressure (BP) control over time in children on renal replacement therapy (RRT). In this study, we aimed to evaluate the 5-year trend in BP antihypertensive medications (AHs) use, as well as the association between HTN and mortality among RRT patients en...

Background and Aims Metabolic acidosis is a common complication of CKD and may contribute to CKD progression. However, the association between serum bicarbonate and CKD progression has not yet been investigated using longitudinal measurements. Method We used data from CKD-REIN, a prospective cohort study conducted in 40 representative French nephr...

Background This study aims to develop a method to estimate the potential of preemptive kidney transplantation (PKT) by identifying patients who were transplanted after a dialysis period (non-preemptive kidney transplantation (NPKT)) despite being medically suitable for PKT.Methods All children (< 18 years old) starting kidney replacement therapy (K...