Quoi de neuf dans les hémangiomes infantiles ?

Infantile hemangioma is the most common vascular tumor of childhood. This benign tumor appears during the first weeks of life, grows during the first months (endothelial proliferation) and then involutes slowly and spontaneously until resolution (possibly with sequelae) which is observed after several years. Although they are always benign tumors, infantile hemangiomas can cause complications. Regarding their location or size, infantile hemangiomas can be life or function-threatening. They are also painful when ulcerated and can cause permanent disfigurement or long-term adverse psychological consequences. Since 2008 and the serendipitous discovery of the efficacy of propranolol in the treatment of infantile hemangiomas, systemic propranolol therapy has been widely used, with a very interesting benefits/risks balance. It has progressively superseded general corticosteroid therapy. A Compassionate Use Program (CUP) for systemic propranolol therapy has been developed in France and represents nowadays the first-line use for the vast majority of French prescribers.     

Le syndrome de Turner : quoi de neuf dans la prise en charge ?

Turner syndrome is a rare genetic disorder, affecting approximately one in 2500 live-born female, due to total or partial absence of the X chromosome. Typical clinical features are short stature and premature ovarian failure and less constantly phenotypic particularities such as congenital malformations, acquired cardiovascular, otological (hearing impairment), autoimmune and metabolic pathologies. The phenotype is highly variable with slight or even normal phenotype. Several studies have shown that growth hormone treatment improves adult height. The possibility of pregnancies after oocyte donation highlights the high risk of these pregnancies requiring a careful follow-up, especially in terms of cardiovascular issues. Although the quality of life seems similar to the normal population, the presence of cardiovascular and otological diseases, and delayed feminisation are associated with an impaired quality of life. Early diagnosis and regular screening for potentials associated complications are essential in the medical follow-up of these patients. The recent publication of recommendations should lead to an optimization and harmonisation of the medical practices and follow-up from paediatric age to adulthood, a lowering morbidity and self-esteem improvement. The interest of ovarian cryopreservation at an early age in these patients is under investigation.     

Réanimation du nouveau-né en salle de naissance : qu’apportent les recommandations de 2010 ?

For apneic or bradycardic babies born at term, it is best to begin ressuscitation in the delivery room with air rather than 100% oxygen. Administration of supplementary oxygen should be regulated by blending oxygen and air, and the concentration delivered should be guided by oximetry. Preterm babies less than 32 weeks gestation may not reach the same arterial blood oxygen saturations in air as those achieved by term babies. Therefore, blended oxygen and air should be given guided by pulse oximetry. Detection of exhaled carbon dioxide in addition to clinical assessment is recommended as the most reliable method to confirm placement of a tracheal tube in neonates. If presented with a floppy, apnoeic baby born through meconium, it is reasonable to rapidly inspect the oropharynx to remove potential secretions. Tracheal intubation and suction may be useful. Therapeutic hypothermia should be considered for infants born at term or near-term with evolving moderate to severe hypoxic-ischemic encephalopathy, with protocol and follow-up coordinated through a regional perinatal system. For preterm babies of less than 28 weeks gestation delivery room temperatures should be at least 26 °C. They should be completely covered in a food-grade plastic bag up to their necks, without drying, immediately after birth. If the heart rate of a newly born baby is not detectable and remains undetectable for 10 min, it is then appropriate to consider stopping resuscitation. Simulation should be used as a methodology in resuscitation education.