[Congenital myasthenic syndromes in childhood: Drug therapeutic strategies].

Congenital myasthenia syndromes (CMS) are a group of genetic disorders responsible for neuromuscular junction dysfunction. Usually beginning before 2 years of age, they are revealed by fatigability and muscle weakness, especially after stress, and often prevent the child's normal development. Over recent years, major advances in therapeutic strategies have been made following the discovery of numerous mutations responsible for CMS and the understanding of their pathogenic role.

[Neuroprotection with hypothermia for hypoxic ischemic encephalopathy in term newborn: review of the learning].

The hypothermia treatment for neonatal hypoxic ischemic encephalopathy is a concept revisited for more than 10 years. With this strategy, animal studies have shown an 80% reduction of brain damage. Conditions for the practice of hypothermia, to obtain neuroprotection, have been described in these studies: rapidity of the onset of cooling after the hypoxic ischemic event, prolonged duration during several hours, ability to obtain neuroprotection with two methods of cooling, selective head cooling or whole body hypothermia.

Lung deposition of HFA beclomethasone dipropionate in an animal model of bronchopulmonary dysplasia.

The best delivery of a drug in ventilated neonates is obtained when using a small particle diameter solution administered via a spacer. Lung deposition of hydrofluoroalkane beclomethasone dipropionate (QVAR, 1.3 microm particles), delivered via an Aerochamber-MV15, was measured in piglets under conditions mimicking ventilated severely ill neonates (uncuffed 2.

[Fetal toxicity of angiotensin-II receptor antagonists].

We report a case of a newborn with an oligohydramnios, acute renal failure and ossification abnormalities. The role of maternal treatment of essential hypertension by angiotensin-II receptor antagonists is discussed in regard to the literature and pathophysiological data.

[Early management of infants born to mothers with meconium-stained amniotic fluid: a survey in French delivery rooms].

Unlabelled: The meconium aspiration syndrome is the first cause of respiratory distress in full term newborns. At birth, management includes systematical oropharyngeal suctioning, before and after shoulders delivery, followed by tracheal suction. During last 10 years, many trials were published which discuss again the value of this strategy.

Maternal exposure to LPS induces hypomyelination in the internal capsule and programmed cell death in the deep gray matter in newborn rats.

Epidemiologic and experimental findings implicate maternal infection in the etiology of injury to brain white matter, which may lead to cerebral palsy in preterm newborns. In the present study, inflammation and brain damage in 1- and 7-d-old rats were investigated after maternal inflammation. Intraperitoneal injection of 300 microg/kg of Escherichia coli lipopolysaccharide was administered to pregnant Wistar rats at d 19 and 20 of gestation (LPS group).

[Metoclopramide and neonatal methaemoglobinemia].

We report the case of a five-day-old newborn with cyanosis. After exclusion of pulmonary and cardiac illness, methaemoglobinemia was diagnosed. Cyanosis is the first symptom of methaemoglobinaemia.

The safety and efficacy of nitric oxide therapy in premature infants.

Objectives: To assess the safety-efficacy balance of low-dose inhaled nitric oxide (iNO) in hypoxemic premature infants because no sustained beneficial effect has been demonstrated clearly and there are concerns about side effects.

Study Design: Eight hundred and sixty infants <32 weeks were randomized at birth to receive 5 ppm iNO or placebo when they presented with hypoxemic respiratory failure (HRF) defined by a requirement for mechanical ventilation, fraction of inspired oxygen (FIO 2 ) >40%, and arterio-alveolar ratio in oxygen (aAO 2 ) <0.22.

Spread of Stenotrophomonas maltophilia colonization in a pediatric intensive care unit detected by monitoring tracheal bacterial carriage and molecular typing.

In our pediatric intensive care unit in Tours (France), intubated and ventilated inpatients are systematically monitored for tracheal bacterial colonization twice a week. This led us to detect five patients colonized with Stenotrophomonas maltophilia over a 4-month period. Molecular typing of the isolates using random amplified polymorphism DNA (RAPD) and pulsed-field gel electrophoresis (PFGE) confirmed that four of the five isolates were genetically related.

Comparison of fecal flora following administration of two antibiotic protocols for suspected maternofetal infection.

Two protocols are used by French neonatologists for the treatment of suspected maternofetal infection (SMFI). Three groups of premature and term neonates were included to study the impact of antibiotics on fecal flora: 10 infants with SMFI treated with amoxicillin and netilmicin (group BI), 10 infants with SMFI treated with amoxicillin, cefotoxime and netilmicin (group TRI) and 10 infants without antibiotic therapy as controls (group C). Group BI samples were colonized with Klebsiella oxytoca and Escherichia coli resistant to amoxicillin and by Eneterococcus faecium and coagulase-negative staphylococci.

[Study of correlations between antibiotics prescriptions and guidelines in a neonatal intensive care unit].

Unlabelled: The increasing use of wide spectrum antibiotics has been reported to be associated with a greater prevalence of multi-resistant bacteria.

Objectives: The aims of this study were to survey the use of antibiotics and to evaluate the correlations between patterns of prescription of antibiotics and prescription guidelines in a neonatal intensive care unit.

Material And Methods: In this 6-month study, all newborns admitted to the NICU and treated with antibiotics were included.

Hypoxic preconditioning reduces apoptosis in a rat model of immature brain hypoxia-ischaemia.

Hypoxic events are common in newborns but their consequences on brain development have not been demonstrated. It has been reported that in newborn animal models of cerebral hypoxic-ischaemic insult, short-term hypoxia before the insult completely prevented brain damage. The mechanisms of this brain tolerance have not been fully elucidated.

Whole-body cooling after perinatal asphyxia: a pilot study in term neonates.

In order to test the practicability and safety of whole-body cooling in term neonates with moderate-to-severe hypoxic-ischaemic encephalopathy (HIE) and to report outcomes, a prospective pilot study was carried out in 25 term infants (median postmenstrual age 38 weeks, range 36 to 41 weeks; 20 males, five females). Whole-body cooling, to a target core temperature of 33 to 34 degrees C, started within 6 hours of birth and was maintained for 72 hours. Of the 25 newborn infants (19 Sarnat II and six Sarnat III, 18 outborn), 18 survived, including 13 (72%) with normal cerebral signal by MRI.

Akinesia, arthrogryposis, craniosynostosis: a presentation of neonatal myasthenia with fetal onset.

Major akinesia with arthrogryposis and craniosynostosis at birth mimics irreversible disorders of the nervous system of pejorative outcome. In this context, the early detection of anti-acetylcholine fetal receptor antibodies in the mother may allow rapid diagnosis of transient neonatal myasthenia of favorable prognosis.

The dopamine mesocorticolimbic pathway is affected by deficiency in n-3 polyunsaturated fatty acids.

Background: Several findings in humans support the hypothesis of links between n-3 polyunsaturated fatty acid (PUFA) status and psychiatric diseases.

Objective: The involvement of PUFAs in central nervous system function can be assessed with the use of dietary manipulation in animal models. We studied the effects of chronic dietary n-3 PUFA deficiency on mesocorticolimbic dopamine neurotransmission in rats.

mRNA D(2) dopaminergic receptor expression after hypoxia-ischemia in rat immature brain.

Previous studies have shown a reduction of dopaminergic D(2) receptors (D(2)R) in the striatum after hypoxia-ischemia in newborn rats. We show here an early and transient reduction of mRNA D(2)R in nonatrophic brains following hypoxia-ischemia. The left carotid artery of P7 rats was ligated followed by hypoxia for 2 h.

Prospective randomized multicenter comparison of high-frequency oscillatory ventilation and conventional ventilation in preterm infants of less than 30 weeks with respiratory distress syndrome.

Background: Early use of high-frequency ventilation and exogenous surfactant is proposed as the optimal mode of ventilatory support in infants with respiratory distress syndrome. In very premature infants, we tested the hypothesis that high-frequency versus conventional ventilation could decrease exogenous surfactant requirements and improve pulmonary outcome, without altering the complication rate, including that of severe intraventricular hemorrhage.

Methods: Preterm infants with a postmenstrual age of 24 to 29 weeks, presenting with respiratory distress syndrome were randomly assigned to high-frequency oscillatory ventilation (lung volume recruitment strategy) or conventional ventilation.

[Mortality in a pediatric hospital. Six-year retrospective study].

Aims: To define the characteristics of patients dying in a pediatric hospital, including causes and modes of death.

Patients And Methods: This retrospective, descriptive, epidemiologic study was performed between 1 January 1990 and 31 December 1995. All patients who died in the hospital between these dates were included.

Consequences of pulmonary inflations (sighs) on cerebral haemodynamics in neonates ventilated by high-frequency oscillation.

High-frequency oscillation (HFO) is a technique frequently used in neonatal resuscitation, but which has yet to be evaluated. The use of intrathoracic pressures may have an effect on the cerebral circulation of immature neonates. The aim of this study was to examine the variations in cerebral blood velocity and oxygenation during brief pulmonary inflations (sighs), by focusing on alveolar recruitment.

Congenital neonatal myotonic dystrophy with persistent pulmonary hypertension and coma: a difficult diagnosis.

The fulminant forms of congenital myotonic dystrophy, which rapidly lead to death, are difficult to diagnose. The case described illustrates the roles of persistent pulmonary hypertension in such a fatal form.