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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 of social deprivation. Methods: All patients < 20 years who initiated KRT from 2010 to 2015 in metropolitan France were included. Data were collected from the comprehensive French registry of KRT French Renal Epidemiology and Information network (REIN). We used a validated ecological index to assess social deprivation, the 2011 French version of the European Deprivation Index (EDI). We estimated the age standardized incidence rates according to the quintiles of EDI using direct standardization and incidence rate ratio using Poisson regression. Results: We included 672 children with kidney failure (58.6% males, 30.7% with glomerular or vascular disease, 43.3% starting KRT between 11 and 17 years). 38.8% were from the most deprived areas (quintile 5 of EDI). The age standardized incidence rate increased with quintile of EDI, from 5.45 (95% confidence interval [CI] = 4.25-6.64) per million children per year in the least deprived quintile to 8.46 (95% CI = 7.41-9.51) in the most deprived quintile of EDI (incidence rates ratio Q5 vs. Q1 1.53-fold; 95% CI = 1.18-2.01). Conclusion: This study showed that even in a country with a universal health care system, there is a strong association between the incidence of pediatric KRT and social deprivation showing that social health inequalities appear from KRT initiation. This study highlights the need to look further into social inequalities in the earliest stage of chronic kidney disease (CKD).
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On the occasion of the 20th anniversary of the REIN (French Renal Epidemiology and Information Network), a summary work on the contributions of the national French ESKD register was carried out. On the issue of paediatric CKD patients, the following key messages were retained. Paediatric stage 5 chronic kidney disease (CKD) has particularities that require to be analysed and taken into account because the mortality of these patients remains 30 times higher than that of children of the same age. The REIN registry enables illustrating the specificities of stage 5 CKD in the paediatric age-group in France and providing a set of indicators making it possible to describe the future of these patients as well as the choices made concerning the modalities of replacement therapy. As compared to other European countries, the incidence and prevalence of stage 5 CKD in France is in the middle range for children under 15 and 20 years old. Renal transplant is by far the leading treatment for stage 5 CKD in children and adolescents under 18 years of age in France, allowing to offer these patients the best possible life expectancy. Owing to the small volume of patients, only a nationwide registry can provide an unbiased view and enables analysing this population requiring a hyperspecialised treatment. The participation of French paediatric nephrologists in the REIN French registry also enables providing input to the European registry (ESPN/ERA www.espn-reg.org/index.jsp) and the international registry (IPNA https://ipna-registry.org) (Consulted on September 15th 2022) and thus the possibility of international studies, which are vital to be in line with an approach to improving practices.
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 et prises en compte car la mortalité de ces patients reste 30 fois supérieure à celle des enfants du même âge. Le registre REIN permet d'illustrer les spécificités de la MRC stade 5 à l'âge pédiatrique en France et de fournir un ensemble d'indicateurs permettant de décrire le devenir de ces patients ainsi que les choix faits concernant les modalités de traitement de suppléance. En comparaison à d'autres pays européens, l'incidence et la prévalence de la MRC stade 5 en France se situent dans les valeurs moyennes chez les enfants de moins de 15 et 20 ans. La transplantation rénale est de loin le premier traitement de la MRC stade 5 parmi les enfants et les adolescents de moins de 18 ans en France, permettant d'offrir à ces patients la meilleure espérance de vie possible. En raison du petit volume de patients, seul un registre à l'échelon national permet d'avoir une vision non biaisée et d'analyser cette population nécessitant une prise en charge hyperspécialisée. La participation des néphropédiatres français au registre français REIN permet aussi d'alimenter le registre européen (ESPN/ ERA www.espn-reg.org/index.jsp) et le registre international (IPNA https://ipna-registry.org) (Consulté le 15 septembre 2022) et ainsi la possibilité d'études internationales, indispensables pour s'inscrire dans une démarche d'amélioration des pratiques.
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Falência Renal Crônica , Transplante de Rim , Adolescente , Humanos , Criança , Adulto Jovem , Adulto , França/epidemiologia , Rim , Falência Renal Crônica/epidemiologia , Falência Renal Crônica/terapia , PacientesRESUMO
BACKGROUND: 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. METHODS: All children with IgAVN diagnosed 2000-2019 in 14 French pediatric nephrology units with minimal follow-up of 6 months were retrospectively included. Outcomes of patients treated with steroids were compared with those of a control group of untreated patients matched for age, sex, proteinuria, eGFR, and histological features. The primary endpoint was IgAVN remission defined as urine protein-to-creatinine ratio < 20 mg/mmol without impaired eGFR one year after disease onset. RESULTS: A total of 359 patients with IgAVN were included with a median follow-up time of 249 days (range 43-809). One hundred eight (30%) patients received oral steroids alone, 207 (51%) patients received three methylprednisolone pulses followed by oral steroids, and 44 patients (12.5%) did not receive steroids. Thirty-two children treated with oral steroids alone were compared with 32 matched control patients who did not receive steroids. One year after disease onset, IgAVN remission proportion was not different between these two groups: 62% versus 68%, respectively. Ninety-three children treated with oral steroids alone were compared with 93 matched patients treated with three methylprednisolone pulses followed by oral corticosteroids. IgAVN remission proportion was not different between these two groups: 77% versus 73%, respectively. CONCLUSION: The benefit of oral steroids alone and methylprednisolone pulses could not be established based on this observational study. Randomized controlled trials are thus required to determine the efficacy of steroids in IgAVN. A higher resolution version of the Graphical abstract is available as Supplementary information.
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Vasculite por IgA , Nefrite , Humanos , Criança , Vasculite por IgA/complicações , Vasculite por IgA/tratamento farmacológico , Vasculite por IgA/patologia , Estudos Retrospectivos , Nefrite/patologia , Rim/patologia , Metilprednisolona , Imunoglobulina ARESUMO
Background: ROHHAD syndrome (Rapid-onset Obesity with Hypothalamic dysfunction, Hypoventilation and Autonomic Dysregulation) is rare. Rapid-onset morbid obesity is usually the first recognizable sign of this syndrome, however a subset of patients develop ROHHAD syndrome without obesity. The prevalence of this entity is currently unknown. Alteration of respiratory control as well as dysautonomic disorders often have a fatal outcome, thus early recognition of this syndrome is essential. Material and methods: A retrospective, observational, multicenter study including all cases of ROHHAD without rapid-onset obesity diagnosed in France from 2000 to 2020. Results: Four patients were identified. Median age at diagnosis was 8 years 10 months. Median body mass index was 17.4 kg/m2. Signs of autonomic dysfunction presented first, followed by hypothalamic disorders. All four patients had sleep apnea syndrome. Hypoventilation led to the diagnosis. Three of the four children received ventilatory support, all four received hormone replacement therapy, and two received psychotropic treatment. One child in our cohort died at 2 years 10 months old. For the three surviving patients, median duration of follow-up was 7.4 years. Conclusion: ROHHAD syndrome without rapid-onset obesity is a particular entity, appearing later than ROHHAD with obesity. This entity should be considered in the presence of dysautonomia disorders without brain damage. Likewise, the occurrence of a hypothalamic syndrome with no identified etiology requires a sleep study to search for apnea and hypoventilation. The identification of ROHHAD syndrome without rapid-onset obesity is a clinical challenge, with major implications for patient prognosis.
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Introduction: Socioeconomic status (SES) is recognized as an important determinant of kidney health. We aimed to evaluate the association of social deprivation with different indicators at kidney replacement therapy (KRT) initiation in the French pediatric metropolitan population. Methods: All patients with end-stage kidney disease (ESKD) who started KRT before 20 years old in France between 2002 and 2015 were included. We investigated different indicators at KRT initiation, which are as follows: KRT modality (dialysis vs. pre-emptive transplantation), late referral to a nephrologist, and dialysis modality (hemodialysis [HD] vs. peritoneal dialysis [PD], urgent vs. planned start of dialysis, use of catheter vs. use of fistula for HD vascular access). An ecological index (European Deprivation Index [EDI]) was used as a proxy for social deprivation. Results: A total of 1115 patients were included (males 59%, median age at dialysis 14.4 years, glomerular/vascular diseases 36.8%). The most deprived group represented 38.7% of the patients, suggesting pediatric patients with ESKD come from a more socially deprived background. The most deprived group was more likely to initiate KRT with dialysis versus kidney transplantation. Among patients on HD, the odds of starting treatment in emergency with a catheter was >2-fold higher for the most deprived compared with the least deprived children (adjusted odds ratio [aOR] 2.35, 95% CI 1.16-4.78). Conclusion: Children from the most deprived area have lower access to pre-emptive transplantation, have lower access to PD, tend to be late referred to a nephrologist, and have more urgent initiation of HD with a catheter.
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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 emergency dialysis or PKT. We identified four pre-CKD stage 5 nephrology care trajectories before KRT that were extracted from the national exhaustive medical-administrative database and used logistic regression to explore associations between patient characteristics, care trajectories, and KRT initiation. RESULTS: Six hundred forty-three pediatric patients started KRT in France; 406 started dialysis and 30.5% emergency dialysis. The "optimal" care trajectory encompassed 179 patients, 82.7% with at least 18 months nephrology follow-up. Conversely, the "no care" trajectory encompassed 118 patients with no nephrology follow-up before KRT. The "severe" trajectory encompassed 128 patients; 93% hospitalized more than once a year and 18% in an intensive care unit. Finally, the "irregular" trajectory encompassed 127 patients, 77% and 46% with irregular laboratory monitoring and CKD drug delivery, respectively. With the "optimal" trajectory as the reference, probability of emergency dialysis was higher with the "irregular" and "no care" trajectories (odds ratio 3.02 [95% confidence interval 1.18-7.66] and 26.5 [10.8-64.8], respectively), and PKT was reduced with the "severe" trajectory (0.43 [0.23-0.82]). CONCLUSION: We identified a group of patients with irregular follow-up who may benefit the most from interventions aiming at improving adherence to treatment and earlier diagnosis of their CKD to improve access to PKT. A higher resolution version of the Graphical abstract is available as Supplementary information.
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Falência Renal Crônica , Nefrologia , Insuficiência Renal Crônica , Criança , Humanos , Falência Renal Crônica/complicações , Diálise Renal , Insuficiência Renal Crônica/complicações , Insuficiência Renal Crônica/diagnóstico , Insuficiência Renal Crônica/terapia , Terapia de Substituição RenalRESUMO
BACKGROUND: Kidney transplantation (KT) is the optimal treatment for children with end-stage kidney disease. The aim of this study was to evaluate the impact of preemptive kidney transplantation (PKT) and of pretransplant dialysis duration on graft survival among French pediatric kidney transplant recipients. METHODS: We analyzed all first pediatric kidney-only transplantations performed in France between 1993 and 2012. A Cox multivariable model was used to investigate the association of PKT and pretransplant dialysis time with the hazard of graft failure defined as death, return to dialysis, or retransplant, whichever occurred first. RESULTS: Patients (n = 1911) were included, of which 380 (19.8%) received a PKT. Median time of follow-up was 7.0 y. PKT was associated with a 55% reduction of the hazard of graft failure at any time after KT compared with patients transplanted after dialysis (hazard ratio, 0.45; 95% confidence interval, 0.33-0.62), after adjustment for recipient sex and age, primary kidney disease, donor age and type (living or deceased donor), number of HLA mismatches, cold ischemia time, and year of transplantation. A reduction of the hazard of graft failure was found in PKT whatever the compared duration of dialysis, even when <6 mo and whatever the dialysis modality. Results were similar in multiple sensitivity analyses. CONCLUSIONS: In France, PKT among pediatric patients is associated with a better graft survival when compared with KT after dialysis, even when <6 mo. Based on these findings, we suggest that PKT should be considered as the treatment of choice for children with end-stage kidney disease.
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Falência Renal Crônica , Transplante de Rim , Criança , Sobrevivência de Enxerto , Humanos , Falência Renal Crônica/diagnóstico , Falência Renal Crônica/cirurgia , Transplante de Rim/métodos , Sistema de Registros , Diálise Renal/métodos , Terapia de Substituição Renal , Resultado do TratamentoRESUMO
INTRODUCTION: Urinary tract infections occur in around 1%-4% of boys and 3%-8% of girls under 2 years old. Diagnosis is difficult because of non-specific symptoms and the risk of urine analysis contamination depending on the sampling method used for precontinent infants. The American Academy of Pediatrics recommend transurethral catheterisation and suprapubic aspiration because of a low contamination rate but these techniques are invasive. On the other hand, while the National Institute for Health and Care Excellence advocate clean catch urine for its minimal invasiveness and acceptable contamination rate, it is difficult to accomplish in precontinent infants. Two recent methods have been described: the Quick-Wee method by Kaufman et al (suprapubic stimulation with cold saline-soaked gauze); and bladder stimulation by Herreros et al then by Tran et al (pubic tapping alternating with lumbar massage). This study aims to compare the effectiveness in collecting midstream urine by bladder stimulation vs the Quick-Wee method in infants under 1 year, before walking. METHODS AND ANALYSIS: This study is a multicentre randomised controlled trial of 230 infants under 1 year and before walking who need urine analysis, conducted in four paediatric emergency departments in France. Patients will be randomised into two groups: bladder stimulation and Quick-Wee method.The primary endpoint will be the success rate of voiding at least 2 mL of urine in less than 5 min.Secondary outcomes are the time to collect at least 2 mL of urine, comfort, quality of urine and the risk factors associated with failure of the two techniques. ETHICS AND DISSEMINATION: The study protocol was approved by the French national ethic committee (consultative committee of the protection of persons). The results of the study will be published in a peer-reviewed journal. TRIAL REGISTRATION NUMBER: Clinical Trials Registry - NCT04587999. DATE AND PROTOCOL VERSION IDENTIFIER: October 2020, V.1.
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Bexiga Urinária , Infecções Urinárias , Criança , Pré-Escolar , Feminino , Humanos , Lactente , Masculino , Estudos Multicêntricos como Assunto , Ensaios Clínicos Controlados Aleatórios como Assunto , Urinálise , Micção , Coleta de UrinaRESUMO
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 (KRT) in France, between 2010 and 2016 and transplanted before December 31, 2017, were included. A propensity score (PS) of receiving PKT was estimated by multivariate logistic regression based on recipient medical characteristics. Healthcare use during the 24 months prior to KRT initiation was extracted from the French National Health Insurance database, and a pre-KRT follow-up of more than 18 months was considered sufficient to allow preemptive transplantation. RESULTS: Among 643 patients who started KRT, 149 (23.2%) were preemptively transplanted. Using PS stratification, among 391 NPKT patients, we identified 145 patients (37%) suitable for PKT, according to clinical characteristics. Mean age was 12.3 years, 67% were males, and 56% had urological abnormalities. Among those 145 patients, we identified 79 NPKT patients who started on dialysis despite early referral to a nephrologist (more than 18 months prior to KRT initiation). CONCLUSIONS: This method estimates a potential of 228 (149 + 79) PKT (35%) among pediatric patients in France. A similar method could be used in adults or in other countries. Estimation of the rate of patients with CKD stage 5 medically suitable for PKT will be of interest for health policy makers when setting up objectives for improvement in preemptive kidney transplant access.
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Transplante de Rim/estatística & dados numéricos , Terapia de Substituição Renal/estatística & dados numéricos , Adolescente , Estudos de Casos e Controles , Criança , Pré-Escolar , Bases de Dados Factuais , França , Humanos , Lactente , Recém-Nascido , Falência Renal Crônica/terapia , Transplante de Rim/tendências , Pontuação de Propensão , Sistema de Registros , Terapia de Substituição Renal/efeitos adversosRESUMO
The specific treatment of idiopathic nephrotic syndrome is based on corticosteroid therapy and/or steroid-sparing immunosuppressive agents in children who are steroid-dependant or frequent relapsers (60-70 %). Patients have an increased infectious risk not only related to the disease during relapses (hypogammaglobulinemia and urinary leakage of opsonins) but also to treatments (corticosteroids or immunosuppressive agents) in period of remission. Vaccination is therefore particularly recommended in these patients. Potential vaccine risks are ineffectiveness, induction of vaccine disease and relapse of idiopathic nephrotic syndrome. Only live vaccines expose to the risk of vaccine disease: they are in general contra-indicated under immunosuppressive treatment. The immunogenicity of inactivated vaccines is reduced but persists. The immunogenic stimulus of vaccination may in theory trigger a relapse of the nephrotic syndrome. Nevertheless, this risk is low in the literature, and even absent in some studies. The benefit-risk ratio is therefore in favor of vaccination with respect to the vaccination schedule for inactivated vaccines, with wide vaccination against pneumococcus and influenza annually. Depending on the context and after expert advice, immunization with live vaccines could be discussed if residual doses/levels of immunosuppressive treatments are moderate and immunity preserved.
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Nefrose Lipoide , Síndrome Nefrótica , Vacinas , Criança , Humanos , Imunossupressores/uso terapêutico , Síndrome Nefrótica/tratamento farmacológico , VacinaçãoAssuntos
Cardiomiopatia Dilatada/diagnóstico , Surdez/diagnóstico , Doenças Mitocondriais/diagnóstico , Síndrome Nefrótica/diagnóstico , Insuficiência Renal Crônica/terapia , Adolescente , Biópsia , Cardiomiopatia Dilatada/etiologia , Cardiomiopatia Dilatada/terapia , Criança , Pré-Escolar , Surdez/etiologia , Testes Genéticos , Humanos , Lactente , Recém-Nascido , Rim/patologia , Rim/cirurgia , Transplante de Rim , Estudos Longitudinais , Masculino , Proteínas de Membrana/genética , Doenças Mitocondriais/complicações , Doenças Mitocondriais/congênito , Doenças Mitocondriais/terapia , Nefrectomia , Síndrome Nefrótica/complicações , Síndrome Nefrótica/congênito , Síndrome Nefrótica/terapia , Insuficiência Renal Crônica/etiologia , Adulto JovemAssuntos
Testes Genéticos/métodos , Proteínas de Membrana/genética , Doenças Mitocondriais/diagnóstico , Síndrome Nefrótica/diagnóstico , Adolescente , Biópsia , Cardiomiopatia Dilatada/diagnóstico , Cardiomiopatia Dilatada/genética , Cardiomiopatia Dilatada/terapia , Criança , Pré-Escolar , Surdez/diagnóstico , Surdez/genética , Sequenciamento de Nucleotídeos em Larga Escala , Humanos , Lactente , Recém-Nascido , Íntrons/genética , Rim/patologia , Estudos Longitudinais , Masculino , Doenças Mitocondriais/complicações , Doenças Mitocondriais/congênito , Doenças Mitocondriais/genética , Mutação , Síndrome Nefrótica/complicações , Síndrome Nefrótica/genética , Síndrome Nefrótica/terapia , Insuficiência Renal Crônica/etiologia , Insuficiência Renal Crônica/terapia , Adulto JovemRESUMO
BACKGROUND: Chromosome 8p deletions are associated with a variety of conditions, including cardiac abnormalities, mental, behavioral problems with variable morphotype and genitourinary anomalies in boys. METHODS: We describe the follow-up over almost 15 years of a boy who initially presented with perineal hypospadias with a micropenis and cryptorchidism with 46,XY DSD. RESULTS: Imaging, pathology, and hormonal exploration suggested gonadal dysgenesis. Further genetic studies were deemed necessary during follow-up. The child's further development recommended further genetic analyses. High-resolution analysis showed an interstitial deletion on the short arm of a chromosome 8: 46,XY,del(8)(p23.1p23.1). We reviewed the literature and found 102 cases including 54 boys: 62.7% had mental problems, 50.9% a dysmorphic disorder, 55.9% cardiac anomalies, and 46.3% of the boys had genitourinary anomalies. Our patient's genital abnormalities can be explained by the haploinsufficiency of the genes, such as GATA4 (OMIM 600576) that are included in the deleted area. CONCLUSION: This case of severe 46,XY DSD raises the question of the role played by 8p23 microdeletion in gonadal dysgenesis. Clinicians are encouraged to look for this anomaly on chromosome 8 in cases of unexplained gonadal dysgenesis even when few signs suggestive of this anomaly are present.
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Disgenesia Gonadal 46 XY/genética , Adolescente , Deleção Cromossômica , Cromossomos Humanos Par 8/genética , Disgenesia Gonadal 46 XY/patologia , Disgenesia Gonadal 46 XY/terapia , Humanos , Cariótipo , MasculinoRESUMO
Around 1/1000 people have a solitary kidney. Congenital conditions mainly include multicystic dysplastic kidney and unilateral renal aplasia/agenesis; acquired conditions are secondary to nephrectomy performed because of urologic structural abnormalities, severe parenchymal infection, renal trauma, and renal or pararenal tumors. Children born with congenital solitary kidney have a better long-term glomerular filtration rate than those with solitary kidney secondary to nephrectomy later in life. Acute and chronic adaptation processes lead to hyperfiltration followed by fibrosis in the remnant kidney, with further risk of albuminuria, arterial hypertension, and impaired renal function. Protective measures rely on non-pharmacological renoprotection (controlled protein and sodium intake, avoidance/limitation of nephrotoxic agents, keeping normal body mass index, and limitation of tobacco exposure). Lifelong monitoring should include blood pressure and albuminuria assessment, completed by glomerular filtration rate (GFR) estimation in case of abnormal values. In the absence of additional risk factors to solitary kidney, such assessment can be proposed every 5 years. There is no current consensus for indication and timing of pharmacological intervention.
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Adaptação Fisiológica , Taxa de Filtração Glomerular/fisiologia , Neoplasias Renais/cirurgia , Rim/fisiopatologia , Rim Único/fisiopatologia , Adulto , Animais , Criança , Ensaios Clínicos como Assunto , Modelos Animais de Doenças , Humanos , Hipertrofia/etiologia , Hipertrofia/fisiopatologia , Hipertrofia/terapia , Rim/anormalidades , Rim/lesões , Rim/cirurgia , Nefrectomia/efeitos adversos , Rim Único/etiologia , Rim Único/terapiaRESUMO
BACKGROUND AND OBJECTIVES: Data on recovery of kidney function in pediatric patients with presumed ESKD are scarce. We examined the occurrence of recovery of kidney function and its determinants in a large cohort of pediatric patients on maintenance dialysis in Europe. DESIGN, SETTING, PARTICIPANTS, & MEASUREMENTS: Data for 6574 patients from 36 European countries commencing dialysis at an age below 15 years, between 1990 and 2014 were extracted from the European Society for Pediatric Nephrology/European Renal Association-European Dialysis and Transplant Association Registry. Recovery of kidney function was defined as discontinuation of dialysis for at least 30 days. Time to recovery was studied using a cumulative incidence competing risk approach and adjusted Cox proportional hazard models. RESULTS: Two years after dialysis initiation, 130 patients (2%) experienced recovery of their kidney function after a median of 5.0 (interquartile range, 2.0-9.6) months on dialysis. Compared with patients with congenital anomalies of the kidney and urinary tract, recovery more often occurred in patients with vasculitis (11% at 2 years; adjusted hazard ratio [HR], 20.4; 95% confidence interval [95% CI], 9.7 to 42.8), ischemic kidney failure (12%; adjusted HR, 11.4; 95% CI, 5.6 to 23.1), and hemolytic uremic syndrome (13%; adjusted HR, 15.6; 95% CI, 8.9 to 27.3). Younger age and initiation on hemodialysis instead of peritoneal dialysis were also associated with recovery. For 42 patients (32%), recovery was transient as they returned to kidney replacement therapy after a median recovery period of 19.7 (interquartile range, 9.0-41.3) months. CONCLUSIONS: We demonstrate a recovery rate of 2% within 2 years after dialysis initiation in a large cohort of pediatric patients on maintenance dialysis. There is a clinically important chance of recovery in patients on dialysis with vasculitis, ischemic kidney failure, and hemolytic uremic syndrome, which should be considered when planning kidney transplantation in these children.
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Nefropatias/terapia , Rim/fisiologia , Recuperação de Função Fisiológica , Diálise Renal , Adolescente , Criança , Pré-Escolar , Europa (Continente) , Feminino , Humanos , Lactente , Masculino , Sistema de RegistrosRESUMO
Background: Despite major technical improvements in the care of children requiring renal replacement therapy (RRT) before 2 years of age, the management of those patients remains challenging and transplantation is generally delayed until the child weighs 10 kg or is 2 years old. In this national cohort study, we studied patient and graft survival in children starting RRT before 2 years of age to help clinicians and parents when deciding on RRT initiation and transplantation management. Methods: All children starting RRT before 24 months of age between 1992 and 2012 in France were included through the national Renal Epidemiology and Information Network (REIN) registry. The primary endpoints were patient survival on dialysis and 10-year graft survival. Results: A total of 224 patients were included {62% boys, median age 10.5 months [interquartile range (IQR) 5.8-15.6]}. The 10-year survival rate was 84% (IQR 77-89). Suffering from extrarenal comorbidities was the only factor significantly associated with both an increased risk of death on dialysis [hazard ratio 5.9 (95% confidence interval 1.8-19.3)] and a decreased probability of being transplanted. During follow-up, 174 renal transplantations were performed in 171 patients [median age at first transplantation 30.2 (IQR 21.8-40.7) months]. The 10-year graft survival was 74% (IQR 67-81). Factors associated with graft loss in multivariate analysis were the time spent on dialysis before transplantation, donor/recipient height ratio with an increased risk for both small and tall donors and presenting two human leucocyte antigen-antigen D-related mismatches. Conclusions: This study confirms the good outcome of children starting RRT before 2 years of age. The main question remains when and how to transplant those children. Our study provides data on the optimal morphological and immunological matching in order to help clinicians in their decisions.
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Falência Renal Crônica/terapia , Transplante de Rim/métodos , Sistema de Registros , Diálise Renal/métodos , Feminino , Seguimentos , França/epidemiologia , Sobrevivência de Enxerto , Humanos , Lactente , Falência Renal Crônica/mortalidade , Masculino , Taxa de Sobrevida/tendênciasRESUMO
BACKGROUND: Hemolytic uremic syndrome related to Shiga-toxin-secreting Escherichia coli infection (STEC-HUS) remains a common cause of acute kidney injury in young children. No specific treatment has been validated for this severe disease. Recently, experimental studies highlight the potential role of complement in STEC-HUS pathophysiology. Eculizumab (EC), a monoclonal antibody against terminal complement complex, has been used in severe STEC-HUS patients, mostly during the 2011 German outbreak, with conflicting results. METHODS: On behalf of the French Society of Pediatric Nephrology, we retrospectively studied 33 children from 15 centers treated with EC for severe STEC-HUS. Indication for EC was neurologic involvement in 20 patients, cardiac and neurologic involvement in 8, cardiac involvement in 2, and digestive involvement in 3. Based on medical status at last follow-up, patients were divided into two groups: favorable (n = 15) and unfavorable outcomes (n = 18). RESULTS: Among patients with favorable outcome, 11/14 patients (79%) displayed persistent blockade of complement activity before each EC reinjection. Conversely, in patients with unfavorable outcome, only 9/15 (53%) had persistent blockade (p = n.s.). Among 28 patients presenting neurological symptoms, 19 had favorable neurological outcome including 17 with prompt recovery following first EC injection. Only two adverse effects potentially related to EC treatment were reported. CONCLUSIONS: Taken together, these results may support EC use in severe STEC-HUS patients, especially those presenting severe neurological symptoms. The study, however, is limited by absence of a control group and use of multiple therapeutic interventions in treatment groups. Thus, prospective, controlled trials should be undertaken.
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Anticorpos Monoclonais Humanizados/uso terapêutico , Inativadores do Complemento/uso terapêutico , Infecções por Escherichia coli/tratamento farmacológico , Síndrome Hemolítico-Urêmica/tratamento farmacológico , Escherichia coli Shiga Toxigênica/isolamento & purificação , Injúria Renal Aguda/etiologia , Injúria Renal Aguda/prevenção & controle , Anticorpos Monoclonais Humanizados/farmacologia , Criança , Pré-Escolar , Ativação do Complemento/efeitos dos fármacos , Ativação do Complemento/imunologia , Complemento C5/antagonistas & inibidores , Complemento C5/imunologia , Inativadores do Complemento/farmacologia , Infecções por Escherichia coli/complicações , Infecções por Escherichia coli/imunologia , Infecções por Escherichia coli/microbiologia , Feminino , Seguimentos , Síndrome Hemolítico-Urêmica/complicações , Síndrome Hemolítico-Urêmica/imunologia , Síndrome Hemolítico-Urêmica/microbiologia , Humanos , Lactente , Masculino , Estudos Retrospectivos , Índice de Gravidade de Doença , Resultado do TratamentoRESUMO
BACKGROUND: Infection is the leading cause of death and hospitalization in renal transplant recipients. We describe posttransplant infections requiring hospitalization, their risk factors and cost in a national pediatric kidney transplantation cohort. METHODS: Data on renal transplant recipients <20 years were extracted from the French National Medicoadministrative Hospital Discharge database between 2008 and 2013 and matched with the Renal Transplant Database. We used Cox regression to study risk factors of hospitalization and calculated the instantaneous risk of hospitalization per month for all infections and by infection type. RESULTS: Five hundred and ninety-three patients were included, and 660 infection-related hospitalizations were identified in 260 patients. The leading cause of hospitalization was urinary tract infection (UTI), followed by viral infection (16.6 and 15.6 per 100 person-years, respectively). Risk factors were younger age at transplantation, high number of HLA mismatches, and use cyclosporine rather than tacrolimus as first anticalcineurin treatment. Risk factors varied by infection type. Female gender, uropathy, cold ischemia time, and cyclosporine were associated with increased risk of UTI, while only age at transplantation inversely correlated with virus-related hospitalizations. Instantaneous risk of all infections decreased with time, except for cytomegalovirus (CMV) infection that displayed a peak at 6 months posttransplantation after prophylaxis withdrawal. Total cost of infection-related hospitalizations was 1600 kilo-euro (k) (933 /person-years). CONCLUSIONS: This study highlights the high burden of infection in transplanted pediatric patients, especially the youngest. This should be considered both for pretransplantation information and designing procedures aiming to decrease hospitalization rate and duration.
Assuntos
Custos de Cuidados de Saúde/estatística & dados numéricos , Hospitalização/estatística & dados numéricos , Infecções/epidemiologia , Transplante de Rim/efeitos adversos , Adolescente , Criança , Pré-Escolar , Estudos de Coortes , Bases de Dados Factuais , Feminino , França , Hospitalização/economia , Humanos , Incidência , Infecções/economia , Masculino , Fatores de RiscoRESUMO
OBJECTIVES: To develop a reliable and validated tool to evaluate technical resuscitation skills in a pediatric simulation setting. STUDY DESIGN: Four Resuscitation and Emergency Simulation Checklist for Assessment in Pediatrics (RESCAPE) evaluation tools were created, following international guidelines: intraosseous needle insertion, bag mask ventilation, endotracheal intubation, and cardiac massage. We applied a modified Delphi methodology evaluation to binary rating items. Reliability was assessed comparing the ratings of 2 observers (1 in real time and 1 after a video-recorded review). The tools were assessed for content, construct, and criterion validity, and for sensitivity to change. RESULTS: Inter-rater reliability, evaluated with Cohen kappa coefficients, was perfect or near-perfect (>0.8) for 92.5% of items and each Cronbach alpha coefficient was ≥0.91. Principal component analyses showed that all 4 tools were unidimensional. Significant increases in median scores with increasing levels of medical expertise were demonstrated for RESCAPE-intraosseous needle insertion (P = .0002), RESCAPE-bag mask ventilation (P = .0002), RESCAPE-endotracheal intubation (P = .0001), and RESCAPE-cardiac massage (P = .0037). Significantly increased median scores over time were also demonstrated during a simulation-based educational program. CONCLUSIONS: RESCAPE tools are reliable and validated tools for the evaluation of technical resuscitation skills in pediatric settings during simulation-based educational programs. They might also be used for medical practice performance evaluations.