[This corrects the article DOI: 10.1016/j.ekir.2025.01.014.].

Background: In spring 2019, an outbreak of Shiga toxin-producing Escherichia coli-associated hemolytic ure-mic syndrome (STEC HUS) occurred in France. Epidemiological investigations made by Sante publique France in connection with microbiological investigations at the national reference center for STEC promptly identi-fied a common exposure to consumption of raw cow's milk cheese, and confirmed a cluster affiliation of the E. coli O26:H11 outbreak strain. Here, we report the clinical characteristics of the patients, the treatment used, as well as the outcome at 1 month. Method: Patients with STEC HUS linked to the E. coli O26:H11 outbreak strain were identified from the national surveillance network of pediatric STEC HUS cases coordinated by Sante publique France. Clinical data were analyzed from the patients' hospital records obtained from the treating physicians. Results: Overall, 20 pediatric cases of STEC HUS linked to the outbreak strain were identified. Their median age of the patients was 16 months (range: 5-60). Most of them presented with diarrhea but none had received prior antibiotherapy. A total of 13 patients required dialysis; 10 patients and four patients had central nervous system (CNS) and cardiac involvement, respectively. No deaths occurred. At the 1-month follow-up, only two patients had a decreased glomerular filtration rate, below 80 mL /min/ 1.73m(2) and four had hypertension. One patient had neurological sequelae. Conclusion: The E. coli O26:H11 strain identified as the cause of an STEC HUS outbreak in France in spring 2019 is notable for the initial severe clinical presentation of the patients, with a particularly high frequency of CNS and cardiac involvement similar to the German E. coli O104:H4 outbreak described in 2011. However, despite the initial severity, the 1-month outcome was favorable in most cases. The patients' young age in this outbreak highlights the need to improve information and caregiver awareness regarding consumption of at -risk foods by young children as key preventive measures against STEC infections. (C) 2022 French Society of Pediatrics. Published by Elsevier Masson SAS. All rights reserved.

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 studied a cohort of 170 children with biopsy-proven IgAV-N, diagnosed between 2007 and 2017. One-quarter of the cohort (27%) presented with initial nephrotic syndrome (NS). Kidney biopsy revealed International Study of Kidney Disease (ISKDC) grade II or grade III in 83% of cases. Endocapillary proliferation was observed in 73% of patients, and chronic lesions were observed in 25%. Data analysis showed a significant association between NS at onset and endocapillary proliferation and cellular crescents. After a median follow-up of 21 months (IQR 12–39), 30% of patients had persistent proteinuria or decreased eGFR. At the end of follow-up, kidney impairment was more often observed in patients with NS at onset and those with cellular crescents and chronic lesions on initial kidney biopsy. Conclusion: This study highlights the relationship between the clinical and histological presentation of IgAV-N and the factors that affect kidney outcome. The ISKDC classification may be improved by including lesions that are more discriminating for disease severity and prognosis.

INTRODUCTION:Tubulointerstitial nephritis (TIN) and uveitis (TINU) syndrome is a rare disease. The renal prognosis is generally thought to be better in children with TINU syndrome than in adults. However, data are scarce. We aimed to investigate the long-term renal prognosis in a French cohort of children with TINU syndrome. METHODS:We performed a national retrospective study including 23 French pediatric nephrology centers enrolling patients with TINU syndrome diagnosed between January 2000 and December 2018. RESULTS:A total of 46 patients were included (52% female, median age 13.8 years). At diagnosis of TIN, the median estimated glomerular filtration rate (eGFR) was 30.6 ml/min per 1.73 m2 (4.9-62.8). The median time between diagnosis of uveitis and TIN was 0.4 months (-4.1; +17.1). All patients had anterior uveitis, but 12 (29%) were asymptomatic. Nearly all patients (44 of 46) received steroid treatment, and 12 patients (26%) received a second-line therapy. At last follow-up (median 2.8 years), the median eGFR was 87.5 ml/min per 1.73 m2 (60.3-152.7) and <90 ml/min per 1.73 m2 in 20 patients. CONCLUSION:In our study, nearly half of the patients had renal sequelae at last follow-up. Given the possible progression to chronic kidney disease, long-term monitoring of children with TINU syndrome is mandatory. Approximately a quarter of the children had asymptomatic uveitis suggesting all children presenting with TIN should undergo systematic ophthalmologic screening even in the absence of ocular signs.

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 rituximab is effective in preventing early relapses in SDNS/FRNS; however, the long-term relapse rate remains high (~70% at 2 years). This trial will assess the association of intravenous immunoglobulins (IVIgs) to rituximab in patients with SDNS/FRNS and inform clinicians on whether IVIg's immunomodulatory properties can alter the course of the disease and reduce the use of immunosuppressive drugs and their side effects. METHODS AND ANALYSIS:We conduct an open-label multicentre, randomised, parallel group in a 1:1 ratio, controlled, superiority trial to assess the safety and efficacy of a single infusion of rituximab followed by IVIg compared with rituximab alone in childhood-onset FRNS/SDNS. The primary outcome is the occurrence of first relapse within 24 months. Patients are allocated to receive either rituximab alone (375 mg/m²) or rituximab followed by IVIg, which includes an initial Ig dose of 2 g/kg, followed by 1.5 g/kg injections once a month for the following 5 months (maximum dose: 100 g). ETHICS AND DISSEMINATION:The study has been approved by the ethics committee (Comité de Protection des Personnes) of Ouest I and authorised by the French drug regulatory agency (Agence Nationale de Sécurité du Médicament et des Produits de Santé). Results of the primary study and the secondary aims will be disseminated through peer-reviewed publications. TRIAL REGISTRATION NUMBER:NCT03560011.

Los trastornos miccionales del niño son patologías frecuentes (hasta el 15% de los niños de 7 años) e invalidantes. En muchos casos, una anamnesis orientada y completa permite, junto con una exploración física simple, hacer el diagnóstico, buscar las enfermedades asociadas y proponer un tratamiento adecuado. El proceso diagnóstico descarta de entrada las fugas urinarias sin trastorno miccional (desembocadura ectópica de la uretra, micción vaginal, incontinencia de esfuerzo, polaquiuria comportamental, vejiga neurógena, etc.) y las disfunciones secundarias de la vejiga (infección urinaria, estreñimiento, hidratación insuficiente, irritación de los órganos genitales externos, cristaluria, etc.). A menudo, es necesario imponer hábitos de bebida y micciones regulares, así como regularizar el tránsito intestinal para clasificar bien la enfermedad. Después, el enfoque fisiopatológico debe distinguir la enuresis primaria aislada (EnPA) y el trastorno primario de la eliminación de orina, que a su vez se divide en disfunción de la fase de llenado (contracciones desinhibidas del detrusor e inestabilidad uretral) y disfunción de la fase de vaciado vesical (hipertonía esfinteriana y vejiga hipoactiva [lazy bladder]), pues algunas de estas patologías con frecuencia están intrincadas. El tratamiento de la enuresis debe iniciarse con una hidratación suficiente y bien distribuida a lo largo del día, y micciones diurnas en número suficiente. Los tratamientos que se utilizan como segunda línea son, según los casos, la desmopresina, las alarmas nocturnas o una combinación de las dos. Las disfunciones vesicales primarias responden, según su causa, a técnicas de rehabilitación con biorretroalimentación o a tratamientos médicos. En los casos difíciles, hay que recurrir a exploraciones urodinámicas realizadas en un centro especializado.

Objectif Valider 4 grilles d’évaluation : ventilation (V), intubation (I), catheter intra-ossseux (O), massage cardiaque externe (M) Matériel et méthode Des items techniques en cotation binaire ont été conçus par un groupe d’experts à partir de l’ERC, puis validés en multicentrique par 13 autres séniors experts via la méthode DELPHI. La validation des grilles a consisté en la mise en situation sur mannequins low-fi, en présence de 2 évaluateurs et relecture vidéo, de 24 sujets répartis en 4 groupes de niveau : 6 externes, 6 internes, 6 séniors non experts, 6 sénior experts. Résultats Les scores moyens des groupes augmentent en fonction du niveau d’expertise pour les grilles V, I, O (avec respectivement p= 0,0006, p=0,0003 et p=0,0026). Les scores moyens ne différent pas en fonction des groupes pour la grille M (p=0,25) Conclusion Nos grilles sont globalement performantes et permettent leur réutilisation pour des formations aux gestes techniques comme lors de scénarios plus complexes hi-fi. Les résultats concernant la grille M peuvent s’expliquer, d’une part, par des formations au massage cardiaque plus récentes des externes et internes via la simulation, et d’autre part, par la rareté de la mise en situation des séniors induisant un biais dans le niveau d’expertise des groupes.

La coqueluche maligne est la première cause de mortalité infectieuse du nourrisson de moins de deux mois avec une évolution fatale dans 75% des cas. Nous rapportons le cas de M, 5 semaines, hospitalisé en réanimation pour une coqueluche confirmée par PCR. Le diagnostic de coqueluche maligne était évoqué devant une altération de l'état général, une tachycardie sine materia, une dyspnée marquée associées à une hyperlymphocytose majeure et une hyponatrémie sévère. Rapidement, la dégradation respiratoire et hémodynamique imposait la mise en place d'une ventilation mécanique, d'un soutien tensionnel par catécholamines et l'administration de monoxyde d'azote inhalé. Devant la majoration de l'hyperleucocytose (>100 000/mm3), une exsanguino-transfusion était réalisée. A 24 heures de réanimation, l'échec de toutes les thérapeutiques faisait prendre la décision de mise en place d'une oxygénation par membrane extracorporelle (ECMO) dont M a pu être sevré au bout de 7 jours. L'évolution a été favorable sur le plan pulmonaire et neurologique. Malgré l'absence de recommandation, nous discutons l'interêt de la mise en place précoce d'une exsanguino-transfusion et d'une assistance par ECMO dans les formes malignes de coqueluche.