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Objective: To describe the neurodevelopmental outcome and perinatal factors associated with favorable outcome among extremely preterm children at 3 years of age., Methods: All infants born before 26 weeks of gestation between 2007 and 2011, admitted to intensive care units participating in a French regional network (western PACA-southern Corsica) were included. Perinatal data were collected to assess the main neonatal morbidities. At 3 years of age, the children's neurodevelopment was assessed by trained physicians participating in the follow-up network. Children were classified according to their disability: none, moderate, or severe. Using logistic regression, we determined the perinatal factors associated with the absence of disability at 3 years of age., Results: One hundred and sixty-two very preterm newborns were admitted to neonatal intensive care units. At discharge the survival rate was 62% (101). Rates of survival increased with gestational age (33% at 23 weeks, 57% at 24 weeks and 68% at 25 weeks). Among the 101 surviving extremely preterm children, 66 were evaluated at 3 years. The perinatal characteristics were not significantly different from those of the children lost to follow-up. Overall, 56% of extremely preterm children had no disability and 6% had severe disability. Cerebral palsy was diagnosed in 13% of children. At 3 years of age, the main perinatal factors associated with no disability were short duration of mechanical ventilation (OR=0.96 [0.93-0.99]; P=0.03) and complete course of prenatal corticosteroids (OR=4.7 [1.2-17.7]; P=0.02)., Conclusion: As mortality rates continue to decrease for very preterm infants, concerns are rising about their long-term outcome. In this high-risk population, improving perinatal care remains a challenge to improve long-term outcome., (Copyright © 2016 Elsevier Masson SAS. All rights reserved.)

Monitoring of blood glucose is usually reported to reduce the risk of hypoglycemia in term newborns with high risk factors and for prematurity in neonatal intensive care unit patients. Differential diagnosis has rarely been discussed. In the eutrophic term newborn, hypoglycemia remains rare and an etiological diagnosis must be made. Intensive management of neonatal hypoglycemia is required to prevent neurodevelopmental defects. Without evident cause or if hypoglycemia persists, a systematic review of possible causes should be made. We report isolated glucocorticoid deficiency diagnosed in an infant at 10 months of age. This boy had neonatal hypoglycemia and mild jaundice that had not been investigated. During his first 9 months of life, he presented frequent infections. At 10 months of age, febrile seizures occurred associated with shock, hypoglycemia, hyponatremia, mild hyperpigmentation, and coma. He was diagnosed with hypocortisolemia and elevated ACTH levels. Brain injury was revealed by MRI after resuscitation, with hypoxic-ischemic and hypoglycemic encephalopathy. The molecular studies demonstrated the presence of p.Asp107Asn and previously unreported frameshift p.Pro281GlnfsX9 MC2R gene mutations. A substitutive hormone therapy was provided and during a follow-up of 12 months no adrenal crisis was noted. We report an unusual case of familial glucocorticoid deficiency with severe neurological injury. This case demonstrates the importance of an appropriate etiological diagnosis in neonatal hypoglycemia., (Copyright © 2014 Elsevier Masson SAS. All rights reserved.)

Some mammalian rhabdoviruses may infect humans, and also infect invertebrates, dogs, and bats, which may act as vectors transmitting viruses among different host species. The VIZIER programme, an EU-funded FP6 program, has characterized viruses that belong to the Vesiculovirus, Ephemerovirus and Lyssavirus genera of the Rhabdoviridae family to perform ground-breaking research on the identification of potential new drug targets against these RNA viruses through comprehensive structural characterization of the replicative machinery. The contribution of VIZIER programme was of several orders. First, it contributed substantially to research aimed at understanding the origin, evolution and diversity of rhabdoviruses. This diversity was then used to obtain further structural information on the proteins involved in replication. Two strategies were used to produce recombinant proteins by expression of both full length or domain constructs in either E. coli or insect cells, using the baculovirus system. In both cases, parallel cloning and expression screening at small-scale of multiple constructs based on different viruses including the addition of fusion tags, was key to the rapid generation of expression data. As a result, some progress has been made in the VIZIER programme towards dissecting the multi-functional L protein into components suitable for structural and functional studies. However, the phosphoprotein polymerase co-factor and the structural matrix protein, which play a number of roles during viral replication and drives viral assembly, have both proved much more amenable to structural biology. Applying the multi-construct/multi-virus approach central to protein production processes in VIZIER has yielded new structural information which may ultimately be exploitable in the derivation of novel ways of intervening in viral replication., (Copyright 2010 Elsevier B.V. All rights reserved.)