Transition des patients atteints d’une maladie respiratoire chronique depuis la pédiatrie vers les services pour adultes

Du fait de l’augmentation de l’espérance de vie des individus atteints de mucoviscidose, la transition de la pédiatrie vers les services de médecine pour adultes est devenue une étape essentielle pour les patients, les aidants et les soignants. Cette transition doit être progressive et longuement préparée. Avoir un processus établi et formalisé, aborder tôt le thème de la transition avec le patient et sa famille, autonomiser le patient avant le transfert et établir des liens étroits entre les structures médicales pédiatriques et pour adultes sont des éléments importants pour la réussite de cette étape. L’éducation thérapeutique ainsi que l’utilisation de questionnaires validés, d’un plan d’action personnalisé ou d’outils connectés peuvent aider. Le transfert se fera au moment le mieux adapté pour le patient, idéalement en période de stabilité de la maladie, de manière progressive, avec des consultations conjointes ou alternées entre les équipes de pédiatrie et de médecine pour adultes. D’autres maladies respiratoires chroniques débutant dans l’enfance ou à l’adolescence pourront bénéficier d’un processus de transition similaire

COBRAPed cohort: Do sensitization patterns differentiate children with severe asthma from those with a milder disease?

International audienceAbstract Background It is unclear whether sensitization patterns differentiate children with severe recurrent wheeze (SRW)/severe asthma (SA) from those with non‐severe recurrent wheeze (NSRW)/non‐severe asthma (NSA). Our objective was to determine whether sensitization patterns can discriminate between children from the French COBRAPed cohort with NSRW/NSA and those with SRW/SA. Methods IgE to 112 components (c‐sIgE) (ImmunoCAP® ISAC) were analyzed in 125 preschools (3–6 years) and 170 school‐age children (7–12 years). Supervised analyses and clustering methods were applied to identify patterns of sensitization among children with positive c‐sIgE. Results We observed c‐sIgE sensitization in 51% of preschool and 75% of school‐age children. Sensitization to house dust mite (HDM) components was more frequent among NSRW than SRW (53% vs. 24%, p < .01). Sensitization to non‐specific lipid transfer protein (nsLTP) components was more frequent among SA than NSA (16% vs. 4%, p < .01) and associated with an FEV1/FVC < −1.64 z ‐score. Among sensitized children, seven clusters with varying patterns were identified. The two broader clusters identified in each age group were characterized by “few sensitizations, mainly to HDM.” One cluster ( n = 4) with “multiple sensitizations, mainly to grass pollen, HDM, PR‐10, and nsLTP” was associated with SA in school‐age children. Conclusions Although children with wheeze/asthma display frequent occurrences and high levels of sensitization, sensitization patterns did not provide strong signals to discriminate children with severe disease from those with milder disease. These results suggest that the severity of wheeze/asthma may depend on both IgE‐ and non‐IgE‐mediated mechanisms

Predicting the risk of respiratory distress in newborns with congenital pulmonary malformations

International audienceObjectives Most children with prenatally diagnosed congenital pulmonary malformations (CPMs) are asymptomatic at birth. We aimed to develop a parsimonious prognostic model for predicting the risk of neonatal respiratory distress (NRD) in preterm and term infants with CPM, based on the prenatal attributes of the malformation. Methods MALFPULM is a prospective population-based nationally representative cohort including 436 pregnant women. The main predictive variable was the CPM volume ratio (CVR) measured at diagnosis (CVR first) and the highest CVR measured (CVR max). Separate models were estimated for preterm and term infants and were validated by bootstrapping. Results In total, 67 of the 383 neonates studied (17%) had NRD. For infants born at term (>37 weeks, n=351), the most parsimonious model included CVR max as the only predictive variable (receiver operating characteristic (ROC) curve area: 0.70±0.04, negative predictive value: 0.91). The probability of NRD increased linearly with increasing CVR max and remained below 10% for CVR max <0.4. In preterm infants (n=32), both CVR max and gestational age were important predictors of the risk of NRD (ROC: 0.85±0.07). Models based on CVR first had a similar predictive ability. Conclusions Predictive models based exclusively on CVR measurements had a high negative predictive value in infants born at term. Our study results could contribute to the individualised general risk assessment to guide decisions about the need for newborns with prenatally diagnosed CPM to be delivered at specialised centres

Phenotype-genotype correlations of pediatric patients with biallelic mutations in ABCA3 and SFTPB surfactant-related genes

International audienc

Phenotype-genotype correlations of pediatric patients with biallelic mutations in ABCA3 and SFTPB surfactant-related genes

International audienc

Phenotype-genotype correlations of pediatric patients with biallelic mutations in ABCA3 and SFTPB surfactant-related genes

International audienc

Phenotype-genotype correlations of pediatric patients with biallelic mutations in ABCA3 and SFTPB surfactant-related genes

International audienc