Hôpital Robert-Debré

Hypoxic Burden Is an Important Determinant of Height in Nonobese Children With Sleep‐Disordered Breathing

ABSTRACT The relationship between linear growth and obstructive sleep‐disordered breathing (SDB) has not been extensively studied. Hypoxic burden (HB) is a new metric that may better capture end‐organ damage in paediatric SDB compared to the traditional apnea‐hypopnea index (AHI)...

Escherichia coli ST131 isolates are rare in human meningitis but belong to a distinct O25b-fimH22 clade B lineage shared with avian pathogenic Escherichia coli

Escherichia coli ST131 is a globally disseminated extraintestinal lineage best known for its resistance-driven expansion, yet it is rarely reported as a cause of neonatal meningitis. Whether specific ST131 sublineages retain neuroinvasive potential remains poorly understood. We a...

CGM-derived disposition index estimates beta cell function in individuals with islet autoimmunity who do not require insulin treatment; changes in the disposition index precede changes identified by CGM metrics 1 year later

Abstract Aims/hypothesis Monitoring of beta cell function in individuals with islet autoimmunity who do not require insulin treatment (preclinical type 1 diabetes) is normally done via OGTTs, posing major challenges given the invasiveness of the test. Here, we validated a disposi...

End-of-induction circulating tumor DNA outperforms conventional cellular MRD for predicting early progression in follicular lymphoma: an ancillary study of the RELEVANCE randomized trial

In this ancillary study of the RELEVANCE phase III trial (NCT01650701), we performed the first direct intra-patient comparison of circulating tumor DNA (ctDNA)-based versus cellular t( BCL2 / JH ) translocation-based MRD at end of induction (EOI) in 278 patients with advanced FL,...

Semaglutide for Treating Paediatric Craniopharyngioma‐Related Obesity: A Multicentre Case Series

ABSTRACT Aims Obesity is a complication of childhood craniopharyngioma that remains difficult to treat. We investigated whether semaglutide was associated with a decrease in BMI among adolescents with craniopharyngioma‐related obesity. Material and Methods This multicentre retros...

Daratumumab in Combination With High‐Dose Cytarabine and Amsacrine as an Effective Salvage Therapy and Bridge to Allogeneic Hematopoietic Stem Cell Transplantation for Refractory T‐Cell Acute Lymphoblastic Leukemia: A Case Report

ABSTRACT Patients with refractory or relapsed T‐cell acute lymphoblastic leukemia (R/R T‐ALL) have dismal outcomes. While immunotherapies are growing opportunities for B‐cell ALL, targeted therapies for T‐ALL remain limited. The CD38 antigen, steadily expressed on T‐ALL blasts, r...

Anesthesia TeleConsultation for Pediatric preoperative assessment: a large national prospective multicentric study in children (TeleCCCAP)

Background: While interest in pediatric anesthesia teleconsultation is growing, published data remain scarce. This study evaluated the feasibility of anesthesia teleconsultation in children, providing a comprehensive analysis of failure, quality, dedicated time and satisfaction....

Identifying methodological guidance used for systematic reviews, clinical practice guidelines, and health technology assessment for rare diseases: a scoping review protocol.

OBJECTIVE: This scoping review will identify methodological guidance relevant to systematic reviews (SRs), clinical practice guidelines (CPGs), and health technology assessments (HTAs) for rare diseases, and examine methodological challenges associated with their development. INT...

Efficacy and safety of fenfluramine in Dravet syndrome: The impact of patient clinical characteristics

Abstract Objective To assess the efficacy and safety of fenfluramine in patients with Dravet syndrome (DS) stratified by age, number of previously attempted antiseizure medications (ASMs), and SCN1A pathogenic variant status. Methods In this post hoc analysis, data from three ran...

The clinical and molecular spectrum of AGO2-associated Lessel-Kreienkamp neurodevelopmental syndrome

Abstract Background Pathogenic variants in AGO2 ,encoding a central component of the RNA-induced silencing complex (RISC), cause the neurodevelopmental disorder Lessel-Kreienkamp syndrome (LESKRES). The variant spectrum and associated molecular mechanisms underlying phenotypic va...

Surgical Morbidity and Short-Term Oncologic Status after Prophylactic-Intent Thyroidectomy in a Cohort of Children with MEN2 from the Multicenter GTE ENDOCAN-RENATEN Study

BACKGROUND: Children carrying germline RET mutations associated with multiple endocrine neoplasia type 2 (MEN2) are at high risk of developing medullary thyroid carcinoma (MTC). Prophylactic-intent thyroidectomy is recommended during childhood to prevent progression to advanced d...