The ANTENATAL multicentre study to predict postnatal renal outcome in fetuses with posterior urethral valves: objectives and design

Abstract Background Posterior urethral valves (PUV) account for 17% of paediatric end-stage renal disease. A major issue in the management of PUV is prenatal prediction of postnatal renal function. Fetal ultrasound and fetal urine biochemistry are currently employed for this prediction, but clearly lack precision. We previously developed a fetal urine peptide signature that predicted in utero with high precision postnatal renal function in fetuses with PUV. We describe here the objectives and design of the prospective international multicentre ANTENATAL (multicentre validation of a fetal urine peptidome-based classifier to predict postnatal renal function in posterior urethral valves) study, set up to validate this fetal urine peptide signature. Methods Participants will be PUV pregnancies enrolled from 2017 to 2021 and followed up until 2023 in >30 European centres endorsed and supported by European reference networks for rare urological disorders (ERN eUROGEN) and rare kidney diseases (ERN ERKNet). The endpoint will be renal/patient survival at 2 years postnatally. Assuming α = 0.05, 1–β = 0.8 and a mean prevalence of severe renal outcome in PUV individuals of 0.35, 400 patients need to be enrolled to validate the previously reported sensitivity and specificity of the peptide signature. Results In this largest multicentre study of antenatally detected PUV, we anticipate bringing a novel tool to the clinic. Based on urinary peptides and potentially amended in the future with additional omics traits, this tool will be able to precisely quantify postnatal renal survival in PUV pregnancies. The main limitation of the employed approach is the need for specialized equipment. Conclusions Accurate risk assessment in the prenatal period should strongly improve the management of fetuses with PUV

Mégavessies anténatales (évaluation de la prise en charge(2004/2011))

AIX-MARSEILLE2-BU Méd/Odontol. (130552103) / SudocSudocFranceF

Néphrostomie percutanée chez l'enfant (indications diagnostiques et thérapeutiques à propos de 43 patients)

AIX-MARSEILLE2-BU Méd/Odontol. (130552103) / SudocPARIS-BIUM (751062103) / SudocSudocFranceF

Functional Ectopic Ureter in Case of Ureteric Duplication in Children: Initial Experience with Laparoscopic Low Transperitoneal Ureteroureterostomy

info:eu-repo/semantics/publishe

Evaluation de la croissance de la ceinture pelvienne foetale

Le but de ce travail est d'analyser la morphogenèse de la ceinture pelvienne foetale, de définir les repères radiographiques normaux en fonction de l'âge gestationnel et du sexe pour établir un référentiel.<br />Chaque foetus mort-né de la région marseillaise (26 maternités) est adressé au Registre des Malformations des Bouches du Rhône avec un questionnaire complet précisant l'âge gestationnel, le sexe, le poids, le déroulement de la grossesse. Un examen anatomo-pathologique, un caryotype sont pratiqués ainsi qu'une radiographie du squelette. Un échantillon de 500 radiographies de bassin de face de foetus normaux a été étudié. Des mesures radiographiques (largeur de bassin, distance inter-épines iliaques postéro-inférieures, distance inter-épines sciatiques, distance inter-ischiatique et largeur symphysaire) corrélées à l'âge gestationnel, validées par deux observateurs, ont été réalisées sur un bassin de face strict.<br />Résultats : la population étudiée présentait une distribution homogène entre 18 et 41 semaines d'aménorrhée. La relation entre les mesures radio-anthropométriques du bassin en fonction de l'âge gestationnel a été modélisée par cinq courbes de régression cubique. Une différence significative (p=0,0062) de distance inter-ischiatique entre la fille et le garçon a été observée et ce, à partir de 26-27 semaines d'aménorrhée. Conclusion : l'établissement d'un référentiel de croissance de la ceinture pelvienne chez le foetus déterminant les critères de normalité morphométrique en fonction de l'âge gestationnel ainsi que ses variations par rapport à la normale est utile en pratique foeto-pathologique

Predicting postnatal renal function of prenatally detected posterior urethral valves using fetal diffusion-weighted magnetic resonance imaging with apparent diffusion coefficient determination

International audienc

Hemidiaphragmatic paralysis in preterm neonates: a rare complication of peripherally inserted central catheter extravasation

Unilateral diaphragmatic paralysis was diagnosed in 2 preterm neonates born at 29 and 25 weeks of gestation, respectively. In both instances, the pathophysiology was phrenic nerve injury after extravasation of parenteral nutrition fluid. Misplacement and infection were predisposing factors. Diaphragmatic movement analysis by time-motion-mode ultrasonography was helpful in achieving a diagnosis. The first neonate required a diaphragmatic placation, whereas the other infant was managed nonoperatively. These cases confirm a rare etiology of diaphragmatic paralysis and possible spontaneous recovery. In neonates with very low birth weight, general anesthesia and thoracic surgery may be associated with a high morbidity, suggesting that nonoperative medical treatment, when possible, is preferable if the neonate does not require supplemental oxygen

Quality of life and neuropsychological development at school age in Hirschsprung's disease

International audienc

Neurostimulation-Guided Anal Intrasphincteric Botulinum Toxin Injection in Children with Hirschsprungʼs Disease

International audienceIn Hirschsprung disease (HD), despite successful surgical treatment, 50% of children experience long-term functional gastrointestinal problems, particularly chronic functional obstructive symptoms. We report our experience regarding clinical effects of neurostimulation-guided anal intrasphincteric botulinum toxin (BT) injections on postoperative obstructive symptoms attributed to a nonrelaxing anal sphincter complex in HD patients

Neurodevelopment and Health-Related Quality of Life in Infants Born with Gastroschisis: A 6-Year Retrospective French Study

International audienceIntroduction Quantify quality of life (QoL) outcomes in gastroschisis children is little assessed. The primary objective was to describe the long-term outcome of newborns with gastroschisis treated in three tertiary care hospitals of France in terms of neurodevelopment and QoL. Materials and Methods The study reported was a cross-sectional, descriptive multicentric retrospective study assessing the outcome of newborns with gastroschisis, born between January 1, 2009, and December 31, 2014, treated at two large and French level III neonatal intensive care units. Long-term outcome data were assessed by questionnaires sent to the infants' parents. Questionnaires explored global health, neurological development, and quality of life (overall assessment including socio-economic and medical), Age & Stages Questionnaire, infants' quality of life (KIDSCREEN), and quality of parents' life (General Questionnaire Short Form-36). Results In this study, 50% of the survivor's families answered the assessment form (n = 33). The average follow-up age was 40 months, ranging from 8 months to 6 years. Cases of simple gastroschisis more often had a normal score for "communication" (p = 0.033), while patients who received morphine for a longer duration had significantly lower scores for the items "communication" and "problem resolving" (p = 0.024 and p = 0.011, respectively). Children's QoL was significantly lower for patients with gestational age younger than 36 weeks (p = 0.023) and for patients born following "fetal cause delivery" (p = 0.022). Parents had a significantly higher physical composite score if their child underwent primary closure (p = 0.012). Conclusion Our analyses confirm the idea that cases of complex gastroschisis and preterm delivery may lead to poorer outcome. Such hindsight (40 months in mean) allowed for an interesting assessment of development long after the patient's initial hospitalization and to confirm these results, a standardized neuropsychological evaluation of patients should be done when at least 6 years old. An accurate assessment of the social environment and its impact on the development and QoL of children will be fundamental to avoid selection bias