Diffuse alveolar hemorrhage in infants: Report of five cases.

Diffuse alveolar hemorrhage (DAH) is a rare life-threatening condition in children. In this entity, the bleeding originates from the pulmonary microvasculature as a result of microvascular damage leading to blood leakage into the alveolar spaces. DAH can occur as an isolated medical entity or may be associated with other organ system injury or dysfunction. The classic triad of symptoms includes hemoptysis, anemia and diffuse pulmonary infiltrates. Hemoptysis is the usual presenting symptom but is not constant. A variety of diseases is associated with the development of DAH. Current classification organize the etiologies of diffuse alveolar hemorrhage based on the presence of severe immune disorders (such as systemic vasculitis and collagenosis) or non-immunodeficiency disorders (with an identified cardiac or non-cardiac origin, or idiopathic). The five cases of DAH presented in this study were all diagnosed in full-term infants, four males and one female, with normal neonatal adaptation and without family history of notable diseases. In all cases the diagnosis was made between the age of three and eighteen weeks-old. Moreover, all five patients, at the time of diagnosis, presented with hemoptysis, mild or severe dyspnea, anemia and abnormal chest X-rays. Consequently, the diagnosis of DAH was strongly suspected and, eventually, confirmed by bronchoscopy. Additional laboratory tests, as well as selected serologic and radiographic studies were performed in order to identify a specific etiology. The final diagnoses reflect a variety of causes: infections, idiopathic pulmonary hemosiderosis, accidental suffocation and Heiner syndrome. Treatment included oral corticosteroids except from one patient that received antimicrobial therapy.

[Twin pregnancy and polymalformative syndrome by Enterovirus]. / Grossesse gémellaire et syndrome polymalformatif infectieux à Entérovirus.

Prenatal diagnosed congenital infection by Enterovirus is rarely described in the literature. A few casereports describe severe abnormalities observed by ultrasound that have led to spontaneous intrauterine demise or early death of the newborn. We report the case of a dichorionic diamniotic twin pregnancy. At 24 weeks of gestation, the second trimester ultrasound examination shows cardiac, brain and abdominal abnormalities in one of the fetuses. The other fetus has a normal appearance. "Standard" serological tests conducted on the mother are negative and amniocentesis reveals no genetic abnormality. After birth, Reverse Transcription Polymerase Chain Reaction (PCR) on samples of blood, ascites and stool reveals to be positive for Enterovirus in both newborns. Both are viable and exhibit severe brain abnormalities with severe neurological sequelae such as cerebral palsy, visual and hearing impairment. This case report illustrates the difficulty of prenatal diagnosis of congenital Enterovirus infection and informs about its possible neurological sequelae.

L'infection foetale précoce à Entérovirus (EV) est peu décrite dans la littérature. De rares cas rapportent de sévères anomalies vues à l'échographie qui conduisent à la mort foetale in utero ou au décès postnatal précoce. Nous présentons le cas d'une patiente présentant une grossesse gémellaire bichoriale biamniotique. L'échographie morphologique réalisée à 24 semaines d'aménorrhée révèle chez l'un des foetus des anomalies cardiaques, cérébrales et abdominales. Le second foetus présente un développement organique normal. Les sérologies «standards¼ réalisées chez la mère sont négatives et la ponction de liquide amniotique ne met pas en évidence d'anomalie génétique. A la naissance, une recherche d'Entérovirus par «Reverse Transcription Polymerase Chain Reaction¼ (RTPCR) se révèle positive pour les deux enfants. Ces derniers sont viables, mais présentent de sévères anomalies cérébrales causant des lourdes séquelles neurologiques. Ce cas clinique illustre la difficulté du diagnostic de l'infection congénitale à Entérovirus ainsi que ses conséquences potentielles.

[Therapeutic advances in neonatology]. / Actualités thérapeutiques en néonatologie.

In this article, we will review major therapeutic advances in neonatology over the past ten years. We will discuss the antenatal administration of magnesium sulfate, the interest of hypothermia in the context of hypoxic ischaemic encephalopathy, the benefits and modalities of placental transfusion, less invasive techniques for ventilation and administration of the surfactant, possibilities to fortify breast milk and the concept of developmental care. These therapeutic advances are sometimes based on new therapeutics, sometimes on new concepts and, sometimes, on new less invasive techniques. They have made it possible to optimize the care of premature babies but also of term newborns.

Dans cet article, nous allons passer en revue les grandes avancées thérapeutiques dans le domaine de la néonatologie au cours des dix dernières années. Nous traiterons de l'administration anténatale du sulfate de magnésium, de l'intérêt de l'hypothermie contrôlée dans le cadre de l'encéphalopathie d'origine hypoxo-ischémique, des bénéfices et modalités de la transfusion placentaire, des techniques moins invasives pour la ventilation et pour l'administration du surfactant, de la fortification du lait maternel et du concept de soins de développement. Ces avancées thérapeutiques reposent tantôt sur des nouveautés thérapeutiques, tantôt sur de nouveaux concepts et, parfois, sur de nouvelles techniques moins invasives. Elles ont permis d'optimiser la prise en charge des prématurés, mais aussi des nouveau-nés à terme.

[Late preterm : high risk newborns despite appearances]. / La prématurité tardive : des nourrissons fragiles malgré les apparences.

Late preterm infants are born between 34 weeks of amenorrhea and 36 weeks 6 days. Late preterms represent the largest proportion of premature infants (about 75 %). Late prematurity is increasing in recent decades. While studies initially focused on mortality and morbidity related to very preterm birth, the late preterms have been the subject of increased attention over the past 15 years. Late preterm infants have an increased risk of respiratory complications, infections, feeding problems, hypothermia and hypoglycemia. Neonatal, infant and during adulthood mortalities are significantly higher in late preterm than in term infants. In addition, late preterm infants carry an increased risk of long-term morbidities, such as neurodevelopmental delay, cerebral palsy, chronic respiratory or metabolic diseases. This review highlights the evidence that late preterm infants are high risk newborns and require adapted follow-up.

Les enfants nés entre 34 semaines d'aménorrhée et 36 semaines 6 jours sont dans la période de prématurité tardive. Ils sont également appelés «late-preterm¼. Ces enfants représentent près de 75 % des naissances prématurées dans les pays industrialisés. Cette prématurité tardive est en augmentation croissante sur les dernières décennies. Alors qu'initialement les études se concentraient essentiellement sur la mortalité et les morbidités liées à la grande prématurité, la population des prématurés tardifs a fait l'objet d'une attention accrue ces 15 dernières années. Il est ainsi démontré que ces enfants présentent un risque accru de complications respiratoires, d'infections, de problèmes d'alimentation, d'hypothermie et d'hypoglycémie. La mortalité néonatale, infantile et jusqu'à l'âge adulte des late-preterm est significativement plus élevée que chez les nouveau-nés à terme. De plus, les morbidités à long terme, tels que le retard neurodéveloppemental, l'infirmité motrice cérébrale, les pathologies respiratoires chroniques ou métaboliques, sont significativement plus élevées. A travers cette revue de la littérature, nous revoyons ces risques, qui contribuent à faire des «late preterm¼ une population fragile et nécessitant un suivi adapté.

A not so harmless mass: Kaposiform hemangioendothelioma complicated by a Kasabach-Merritt phenomenon.

A vascular mass localized in the face and the neck was displayed by ultrasonography in a 38-week-old male fetus. At birth, the mass was bulky and purplish. The newborn breathed spontaneously but with severe desaturation. During laryngoscopy, we observed an obstruction of the larynx with a left-shift caused by the hemorrhagic mass. Blood analysis revealed anemia, severe thrombocytopenia, and coagulation disorders. The diagnosis of kaposiform hemangioendothelioma (KHE) complicated by a Kasabach-Merritt phenomenon (KMP) was put forward and treatment with propranolol, corticoids, and vincristine was initiated. Platelets were transfused daily for 8 days but did not resolve the thrombocytopenia. At day 8, we added sirolimus to the treatment and noted a rapid response with the normalization of the platelet count within 1 week and a significant regression of the mass. In this paper, we review the clinical and biological features of hemangioendothelioma associated with KMP and discuss its current and future treatment. Sirolimus seems to be very promising.

[Neuroprotection by controlled hypothermia in neonatal hypoxic-ischemic encephalopathy]. / Neuroprotection par hypothermie contrôlée dans l'encéphalopathie anoxo-ischémique du nouveau-né.

Neonatal hypoxic-ischemic encephalopathy is a major cause of death and neurodevelopmental delay. Brain cooling by mild controlled hypothermia is currently the most promising therapy.

[Update in the management of persistent pulmonary hypertension of the newborn]. / Actualités thérapeutiques dans l'hypertension artérielle pulmonaire persistante du nouveau-né.

Persistent pulmonary hypertension of the newborn is a severe disease leading to persistent and refractory hypoxemia with bad outcomes. The introduction of inhaled nitric oxide therapy significantly improved short and long term prognosis of those infants. More recently, sildenafil also appeared promising, but regimen and indications still need to be delineated.