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The substantial improvement in survival in France for newborns born at 25 through 31 weeks' gestation was accompanied by an important reduction in severe morbidity, but survival remained rare before 25 weeks. Although improvement in survival at extremely low gestational age may be possible, its effect on long-term outcomes requires further studies. The long-term results of the EPIPAGE-2 study will be informative in this regard.

Patients with biallelic loss-of-function variants of AIRE suffer from autoimmune polyendocrine syndrome type-1 (APS-1) and produce a broad range of autoantibodies (auto-Abs), including circulating auto-Abs neutralizing most type I interferons (IFNs). These auto-Abs were recently reported to account for at least 10% of cases of life-threatening COVID-19 pneumonia in the general population. We report 22 APS-1 patients from 21 kindreds in seven countries, aged between 8 and 48 yr and infected with SARS-CoV-2 since February 2020. The 21 patients tested had auto-Abs neutralizing IFN-α subtypes and/or IFN-ω; one had anti–IFN-β and another anti–IFN-ε, but none had anti–IFN-κ. Strikingly, 19 patients (86%) were hospitalized for COVID-19 pneumonia, including 15 (68%) admitted to an intensive care unit, 11 (50%) who required mechanical ventilation, and four (18%) who died. Ambulatory disease in three patients (14%) was possibly accounted for by prior or early specific interventions. Preexisting auto-Abs neutralizing type I IFNs in APS-1 patients confer a very high risk of life-threatening COVID-19 pneumonia at any age.

Despite recent improvements in survival rates, cerebral palsy remains highly prevalent among very preterm children. Severe cranial ultrasound abnormalities predict motor disability strongly, but one third of infants with cerebral palsy had no ultrasound abnormalities.

WHAT'S KNOWN ON THIS SUBJECT: Neonatal infections are frequent complications in very preterm infants, already at high risk of neurologic and cognitive disabilities. Few studies have linked neonatal infections and neurodevelopmental outcomes. Those that did evaluated children only to the age of 22 months. WHAT THIS STUDY ADDS:This study assessed the respective effects of early-and late-onset sepsis and their association with 5-year neurodevelopmental outcomes. We identified a significant and cumulative risk of cerebral palsy when episodes of early-and late-onset sepsis were associated. abstract OBJECTIVE: To determine whether neonatal infections are associated with a higher risk of adverse neurodevelopment at 5 years of age in a population-based cohort of very preterm children. METHODS:We included all live births between 22 and 32 weeks of gestation, from 9 regions in France, in 1997 (EPIPAGE study). Of the 2665 live births, 2277 were eligible for a follow-up evaluation at 5 years of age: 1769 had a medical examination and 1495 underwent cognitive assessment. Cerebral palsy and cognitive impairment were studied as a function of early-onset sepsis (EOS) and late-onset sepsis (LOS), after adjustment for potential confounding factors, in multivariate logistic regression models. RESULTS:A total of 139 (5%) of the 2665 live births included in the study presented with EOS alone (without associated LOS), 752 (28%) had LOS alone (without associated EOS), and 64 (2%) displayed both EOS and LOS. At 5 years of age, the frequency of cerebral palsy was 9% (157 of 1769) and that of cognitive impairment was 12% (177 of 1495). The frequency of cerebral palsy was higher in infants with isolated EOS (odds ratio [OR] CONCLUSIONS: Neonatal infections in these very preterm infants were associated with a higher risk of cerebral palsy at the age of 5 years, particularly in infants presenting with both EOS and LOS. Pediatrics 2013;132:e372-e380 AUTHORS:

Very preterm children have a higher risk of behavioral problems at 3 years of age compared with term-born children. Health and neurodevelopmental status of the child were significantly associated with behavioral difficulties.

IPHIntraparenchymal haemorrhage IVH Intraventricular haemorrhage PPROM Preterm premature rupture of membranes PTL Preterm labour PVL Periventricular leucomalacia AIM The aim of this study was to assess the independent role of cerebral lesions on ultrasound scan, and several other neonatal and obstetric factors, as potential predictors of cerebral palsy (CP) in a large population-based cohort of very preterm infants.METHOD As part of EPIPAGE, a population-based prospective cohort study, perinatal data and outcome at 5 years of age were recorded for 1812 infants born before 33 weeks of gestation in nine regions of France in 1997. RESULTSThe study group comprised 942 males (52%) and 870 females with a mean gestational age of 30 weeks (SD 2wks; range 24-32wks) and a mean birthweight of 1367g (SD 393g; range 450-2645g). CP was diagnosed at 5 years of age in 159 infants (prevalence 9%; 95% confidence interval [CI] 7-10%), 97 males and 62 females, with a mean gestational age of 29 weeks (SD 2wks; range 24-32wks) and a mean birthweight of 1305g (SD 386g; range 500-2480g). Among this group, 67% walked without aid, 14% walked with aid, and 19% were unable to walk. Spastic, ataxic, and dyskinetic CP accounted for 89%, 7%, and 4% of cases respectively. The prevalence of CP was 61% among infants with cystic periventricular leukomalacia, 50% in infants with intraparenchymal haemorrhage, 8% in infants with grade I intraventricular haemorrhage, and 4% in infants without a detectable cerebral lesion. After controlling for cerebral lesions and obstetric and neonatal factors, only male sex (odds ratio [OR] 1.52; 95% CI 1.03-2.25) and preterm premature rupture of membranes or preterm labour (OR 1.72; were predictors of the development of CP in very preterm infants.INTERPRETATION Cerebral lesions were the most important predictor of CP in very preterm infants. In addition, infant sex and preterm premature rupture of membranes or preterm labour were also independent predictors of CP.Very preterm infants (born before 33wks of gestation) are at high risk of developing motor deficiencies during childhood, particularly cerebral palsy (CP). 1 Cerebral lesions and several other neonatal and obstetric risk factors for CP have been described in the literature.2-4 These factors include, in particular, gestational age, low birthweight, multiple gestation, intrauterine infection, placental abruption, preterm labour or pre-labour rupture of membranes, respiratory distress syndrome, and neonatal sepsis.Since the early 1990s, advances in perinatal and neonatal care have improved the survival of very preterm infants. 5Although the incidence of severe cerebral lesions (i.e. white matter disease) has decreased significantly, 6,7 that of CP has not. 1,8 Indeed, in half of very preterm children who develop CP, no severe cerebral lesion is apparent on neonatal cranial ultrasound scans.9 Nevertheless, it is still strongly recommended that ultrasonography be performed in all infants born at a gestational age of less than 30 weeks and that it be used to p...

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