Respiratory syncytial virus-associated mortality in a healthy 3-year-old child: a case report.

BACKGROUND: Respiratory syncytial virus (RSV) is the most frequently identified pathogen in children with acute lower respiratory tract infection. Fatal cases have mainly been reported during the first 6 months of life or in the presence of comorbidity. CASE PRESENTATION: A 47-month-old girl was admitted to the pediatric intensive care unit following sudden cardiopulmonary arrest occurring at home. The electrocardiogram showed cardiac asystole, which was refractory to prolonged resuscitation efforts. Postmortem analyses detected RSV by polymerase chain reaction in an abundant, exudative pericardial effusion. Histopathological examination was consistent with viral myoepicarditis, including an inflammatory process affecting cardiac nerves and ganglia. Molecular analysis of sudden unexplained death genes identified a heterozygous mutation in myosin light chain 2, which was also found in two other healthy members of the family. Additional expert interpretation of the cardiac histology confirmed the absence of arrhythmogenic right ventricular dysplasia or hypertrophic cardiomyopathy. CONCLUSIONS: RSV-related sudden death in a normally developing child of this age is exceptional. This case highlights the risk of extrapulmonary manifestations associated with this infection, particularly arrhythmia induced by inflammatory phenomena affecting the cardiac autonomic nervous system. The role of the mutation in this context is uncertain, and it is therefore necessary to continue to assess how this pathogenic variant contributes to unexpected sudden death in childhood.

Partial acute transverse myelitis is a predictor of multiple sclerosis in children.

BACKGROUND: Acute transverse myelitis (ATM) in children is a rare and often severe disease for which there are few known prognostic factors, particularly the subsequent risk of multiple sclerosis (MS) diagnosis. OBJECTIVES: To determine the clinical course and prognostic factors after a first episode of ATM in children. METHODS: Thirty children below 16 years of age diagnosed with a first neurological episode of ATM were included retrospectively. Clinical evaluation, treatment, laboratory, and MRI data were collected. RESULTS: Median age at onset was 11 years (range 3-15 years). Follow-up data were available for a median of 4 years (range 0.5-16.7 years). Five patients subsequently had a diagnosis of MS (17%), which was associated with acute partial transverse myelitis (odds ratio 5; 95% confidence interval 2.3-11), with a 60% probability of having a relapse at five years (p < 0.01). The 2011 Verhey criteria correctly identified MS in children with the highest specificity (96%) and sensitivity (80%). CONCLUSION: Acute partial transverse myelitis and brain MRI abnormalities at initial presentation are significantly predictive of a subsequent diagnosis of MS in children with ATM. These findings suggest that closer brain MRI monitoring after acute partial transverse myelitis might make the earlier introduction of disease-modifying therapies possible.

Neonatal mortality and morbidity in preterm infants born from assisted reproductive technologies.

AIM: Premature birth is frequent in infants conceived with assisted reproductive technologies (ART). We sought to determine whether neonatal outcome in ART preterm infants differs from that of spontaneously conceived (SC) preterm infants. METHODS: Data were prospectively collected in infants born ≤ 32 weeks after ART or SC. We calculated a composite index of severe morbidity (based on occurrences of severe necrotizing enterocolitis, severe intraventricular haemorrhage, periventricular leukomalacia or bronchopulmonary dysplasia). Survival rate without severe morbidity was compared between the two groups. RESULTS: Six hundred and twelve preterm infants were hospitalized in our tertiary care centre: 81 in ART group and 521 in SC group. In the ART group, twin pregnancy (69.1% vs. 15.9%, p < 0.001) and inborn delivery (98.8% vs. 90.0%, p < 0.01) were more frequent. Gestational age (29 vs. 28 weeks, p < 0.05) and birth weight (1100 vs. 1020 g, p < 0.001) were also higher. Survival without severe morbidity was significantly higher in ART infants (76.5% vs. 55.2%, p < 0.001), with the difference mainly observed in infants born ≤ 28 weeks (22.9% vs. 55.7%, p < 0.001). CONCLUSION: Assisted reproductive technologies was not associated with adverse neonatal outcome. Differences in pregnancy and neonatal characteristics probably explain the increased survival without severe morbidity in ART infants.

[A moderate intrauterine growth delay with lethal outcome: neonatal Menkes disease]. / Un retard de croissance intra-utérin d'allure banale mais de pronostic sévère: la maladie de Menkes à révélation périnatale.

We report a case of moderate intrauterine growth delay with a congenital skull fracture and subdural hematoma, related to Menkes disease. The diagnosis was established in the neonatal period and absorptiometry showed global osteopenia. This disorder has an X-linked recessive inheritance pattern. It results from an abnormality in copper transport with a reduction in the ability to incorporate copper into certain enzymes that need it as a cofactor. The clinical phenotype stems from a deficiency of these enzymes, which explains the diversity of the symptoms. It begins in the first months of life with neurological disorders (hypotonia, seizures) and bone and vascular abnormalities. Usually, death occurs before the age of 5.

[Effect of magnesium sulphate on mortality and neurologic morbidity of the very-preterm newborn (of less than 33 weeks) with two-year neurological outcome: results of the prospective PREMAG trial]. / Effet du sulfate de magnésium sur la mortalité et la morbidité neurologique chez le prématuré de moins de 33 semaines, avec recul à deux ans: résultats de l'essai prospectif multicentrique contre placebo PREMAG.

OBJECTIVE: To evaluate whether magnesium sulphate (MgSO(4)) given to women at risk of very-preterm birth would be neuroprotective in preterm newborns. PATIENTS AND METHODS: In 18 French centres, women with fetuses of gestational age less than 33 weeks whose birth was expected within 24 hours were randomised from 1993 to 2003 with follow-up of infants until two years of age after discharge. They received a single injection of 0.1 mg/l de MgSO(4) (4g) or isotonic 0.9% saline over 30 minutes. This study is registered as an International Standard Randomised Controlled Trial, number 00120588. Analyses were based on intention to treat. RESULTS: Data from 688 infants were analysed of which 606 were followed up and 10 were lost to follow-up. Comparing infants who received MgSO(4) or placebo, respectively, has shown a decrease of all primary endpoints (total mortality, severe white matter injury and their combined outcome) and of all secondary endpoints (motor dysfunction, cerebral palsy, cognitive dysfunction and their combined outcomes at two years of age) in the MgSO(4) group. The decrease was nearly significant or significant for gross motor dysfunction (OR: 0.65 [0.41-1.02]) and combined criteria: death and cerebral palsy (OR: 0.65 [0.42-1.03]); death and gross motor dysfunction (OR: 0.62 [0.41-0.93]); death, cerebral palsy and cognitive dysfunction (OR: 0.68 [0.47-1.00]). No major maternal adverse effects were observed in the MgSO(4) group. DISCUSSION AND CONCLUSION: Given its beneficial effects and safety, the use of prenatal low-dose MgSO(4) for preventing neurodisabilities of very-preterm infants should be discussed either as a stand-alone treatment or as part of a combination treatment, at least in the context of clinical trials.

[Pneumococcal hemolytic uremic syndrome: about 2 cases]. / Syndrome hémolytique et urémique à pneumocoque : à propos de 2 cas.

UNLABELLED: Haemolytic and uremic syndrome (HUS) is the most frequent cause of pediatric acute renal failure. It occurs classically after a diarrhea due to Escherichia coli, seldom in the context of pneumococcus infection. HUS due to pneumococcus has epidemiologic, therapeutic and prognostic characteristics. OBSERVATIONS: We report on the cases of 2 young girls who contracted pneumococcal HUS, one with meningitis and the other with pneumonia. Both were less than 2-year-old. Transfusions of washed blood cells were performed, and dialysis therapy was necessary for 6 days in one and 35 days in the other case. The 1st patient was hospitalised for 15 days and recovered completely in 8 months, the 2nd was hospitalised for 39 days and after 3 months still had renal insufficiency. DISCUSSION: Pneumococcal HUS usually affects healthy children of under 24 months, and often requires dialysis therapy. All usually described serotypes of pneumococci are not included in Prevenar vaccine. The serotypes found in the 2 vaccinated young patients reported here were included in Pneumo23 but not in Prevenar vaccine. The use of washed blood products is preferable in case of blood transfusion, as the presence of plasma may prolong hemolysis through the action of a neuraminidase. The evolution of pneumococcal HUS, usually considered worse than that of the typical HUS, is similar if the last 30 cases described are considered. CONCLUSION: Pneumococcal HUS is a disease that should be better known, whose incidence may be increasing. Prognosis improves if dialysis and antibiotics are started early. Antipneumococcal vaccination reduces the incidence of this disease.

Pediatric angiostrongyliasis.

Angiostrongyliasis, the leading cause worldwide of eosinophilic meningitis, is an emergent disease due to Angiostrongylus cantonensis larvae, transmitted accidentally to humans. Contamination of children usually occurs by direct contact with an infected mollusk. Eosinophilic meningoencephalitis is the major clinical feature of this parasitic infection in humans. It is usually benign for adults, but more severe for children. Clinical symptoms usually combine fever, meningitis, and neurological signs (somnolence, moaning, hypotonia, convulsions, and increased intracranial pressure). Presumptive diagnosis of human angiostrongyliasis is based on epidemiologic characteristics, clinical symptoms, medical history, and laboratory findings, in particular, hypereosinophilia in blood and cerebrospinal fluid. Treatment is based on corticosteroids associated with anthelmintics. This work reviews the diagnosis and treatment of this life-threatening (especially in children) parasitic disease and the need for preventive action.

[Anemia, a new severity factor in young infants with acute viral bronchiolitis?] / L'anémie, un nouveau facteur de sévérité de la bronchiolite aiguë du nourrisson ?

INTRODUCTION: The role of anemia is raised as a risk of low respiratory infection of the child, but there are no data on anemia as a severity factor in acute viral bronchiolitis (AVB) in infants. METHODS: All infants less than 16 weeks old admitted to Montpellier University Hospital from 2015/10/01 to 2016/04/01 for AVB were included in a retrospective observational study. The primary objective was to determine whether the hemoglobin (Hb) concentration on admission was an independent factor of clinical severity, judged by the modified Wood's clinical asthma score (m-WCAS). The secondary objective was to assess the impact of Hb level on the characteristics of hospitalization, including the type and duration of respiratory support. RESULTS: The m-WCAS was used at least once during hospitalization in 180 out of 220 patients (82%), making it possible to distinguish patients with mild AVB (maximum m-WCAS<2, n=81) from patients with severe AVB (maximum m-WCAS>2, n=99). A logistic regression model indicated that the Hb concentration, for every 1g/dL decrement, was an independent factor of AVB severity (OR 1.16 [1.03-1.29], P=0.026). A level under 10g/dL on admission was associated with a higher use of continuous positive airway pressure (P<0.001), as well as a longer duration of respiratory support (P=0.01). CONCLUSION: This study suggested that anemia may influence the clinical expression of AVB in young infants.

[Fusobacterium necrophorum meningitis: forgotten complication of a gingivitis]. / Méningite à Fusobacterium necrophorum: complication oubliée d'une gingivite.

UNLABELLED: By now Lemierre's syndrome is a seldom-described disease whose prognosis depends on the precocity of treatment. CASE PRESENTATION: We report the case of an 11-month-old child, with a fulminant Fusobacterium necrophorum meningitis, which derived from a gingival infection, with fatal outcome. CONCLUSION: This atypical case of Lemierre's syndrome (young age occurrence and localisation) underlines the potential severity of F. necrophorum sepsis.

[Revelation of an acute lymphoblastic leukemia in the delivery room]. / Leucémie aiguë lymphoblastique néonatale: une affection rare à révélation immédiate en salle de naissance.

Acute leukemia is uncommon in neonates and has a much poorer prognosis than in older children. We report on a case of acute lymphoblastic leukemia observed in a neonate who had bleeding and hepatosplenomegaly at birth, which justified intensive care during the first postnatal week. Despite early appropriate treatment, the patient died at 7 months of age. We present here physical and laboratory findings, which indicate a grim prognosis. These criteria should be considered carefully in order to ensure a realistic information for the parents and appropriate decisions.

[Digoxin-Josamycin: a dangerous drug interaction in children]. / Digoxine-Josamycine: une interaction dangereuse chez l'enfant.

Digitalis intoxication is usually accidental in children. We report the case of a young infant with congenital heart disease in whom the coadministration of digoxin and josamycin led to a 50% increase in the digoxin concentration, generating sinoatrial block and cardiac failure. Clinical and electrocardiographic symptoms very quickly resolved following immunotherapy with antidigitalis Fab fragments. Digoxin concentrations must be carefully monitored in patients concomitantly receiving macrolides to ensure that the digoxin dose can be readjusted if necessary.

Prophylactic ibuprofen versus placebo in very premature infants: a randomised, double-blind, placebo-controlled trial.

BACKGROUND: Patent ductus arteriosus is a common complication of prematurity that frequently requires surgical or medical treatment. The benefit of prophylactic treatment by indometacin, a cyclo-oxygenase inhibitor, remains uncertain compared with curative treatment. This benefit could be improved with ibuprofen, another cyclo-oxygenase inhibitor with fewer adverse effects than indometacin on renal, mesenteric, and cerebral perfusion. We aimed to compare prophylactic and curative ibuprofen in the treatment of this abnormality in very premature infants. METHODS: We did a randomised controlled trial in infants younger than 28 weeks of gestation, who were randomly assigned to receive either three doses of ibuprofen or placebo within 6 h of birth. After day 3, symptomatic patent ductus arteriosus was treated first by open curative ibuprofen, then back-up indometacin, surgery, or both. The primary endpoint was need for surgical ligation. Analysis was per protocol. FINDINGS: The study was stopped prematurely after 135 enrollments because of three cases of severe pulmonary hypertension in the prophylactic group. 65 infants received prophylactic ibuprofen, and 66 received placebo. Prophylaxis reduced the need for surgical ligation from six (9%) to zero (p=0.03), and decreased the rate of severe intraventricular haemorrhage from 15 (23%) to seven (11%) (p=0.10). However, survival was not improved (47 [71%] placebo vs 47 [72%] treatment, p=1.00), because of high frequency of adverse respiratory, renal, and digestive events. INTERPRETATION: In premature infants, prophylactic ibuprofen reduces the need for surgical ligation of patent ductus arteriosus, but does not reduce mortality or morbidity. Therefore, it should not be preferred to early curative ibuprofen.

Does smoking in pregnancy modify the impact of antenatal steroids on neonatal respiratory distress syndrome? Results of the Epipage study.

OBJECTIVES: To assess the relation between cigarette smoking during pregnancy and neonatal respiratory distress syndrome (RDS) in very preterm birth, and to analyse the differential effect of antenatal steroids on RDS among smokers and non-smokers. DESIGN: A population based cohort study (the French Epipage study). SETTING: Regionally defined births in France. METHODS: A total of 858 very preterm liveborn singletons (27-32 completed weeks of gestation) of the French Epipage study were included in this analysis. The odds ratio for RDS in relation to smoking in pregnancy was estimated using a logistic regression to control for gestational age. The odds ratio for RDS in relation to antenatal steroids was estimated taking into account an interaction between antenatal steroids and cigarette smoking, using multiple logistic regression to control for gestational age, birthweight ratio, main causes of preterm birth, mode of delivery, and sex. RESULTS: The odds ratio for RDS in relation to smoking in pregnancy adjusted for gestational age (aOR) was 0.59 (95% confidence interval (CI) 0.44 to 0.79). The aOR for RDS in relation to antenatal steroids was 0.31 (95% CI 0.19 to 0.49) in babies born to non-smokers and 0.63 (95% CI 0.38 to 1.05) in those born to smokers; the difference was significant (p = 0.04). CONCLUSIONS: Cigarette smoking during pregnancy is associated with a decrease in the risk of RDS in very preterm babies. Although antenatal steroids reduce the risk of RDS in babies born to both smokers and non-smokers, the reduction is smaller in those born to smokers.

[Congenital myotonic dystrophy type I in a very premature neonate: ethical concerns]. / Dystrophie myotonique de Steinert congénitale chez un grand prématuré : questionnements éthiques.

UNLABELLED: The congenital form of myotonic dystrophy type I (CDM1) corresponds to a>1500 expansion of an unstable trinucleotide (CTG) repeat. Two prognostic factors predict the risk of death in early infancy: maturity of less than 35 weeks of gestation and neonatal invasive ventilation for more than 30 days. OBSERVATION: The case of a 29-week-old premature female infant, conceived by in vitro fertilization, is reported. Generalized hypotonia led to the diagnosis of the disease. Ethical concertation about withdrawal or maintenance of intensive care was engaged, taking into account the prolonged ventilation, the degree of prematurity, and the parental wishes for maximum care. The infant was extubated after 2 months. At 17 months, motor development and precursors of language were delayed, and difficulties in feeding had required a gastrostomy. DISCUSSION: Technical advances in neonatal intensive care now allow CDM1 children to survive prolonged ventilation. The signification of such ventilatory needs on patient outcome, particularly for motor handicaps, speech and language delay, and mental deficiency, remains uncertain. The potential impact of in vitro fertilization on disease expression may also be considered. CONCLUSION: CDM1 is a severe condition, but variability in clinical manifestations and absence of genotype-phenotype correlation result in problems predicting prognosis at the individual level. Ethical issues about the level of care, notably for tracheostomy and gastrostomy, should be adapted to each case, in partnership with parents.

Glutamate inhibition of NMDA-induced hydroxyl radical release: an ontogenic study in rat.

Hydroxyl radicals (.OH) are frequently associated with glutamate excitotoxicity and may be critical in the occurrence of perinatal brain damage. We thus investigated the mechanisms regulating the glutamate-induced release of toxic.OH during development, using microdialysis and salicylate as an.OH trap. Glutamate inhibited.OH release until post-natal day 14, but stimulated this release from day 21 onwards. DHPG [(RS)-3,5-dihydroxyphenylglycine], a group-I metabotropic glutamate receptor agonist, similarly reduced the.OH release at day 14, but was ineffective afterwards. DHPG also completely blunted the tremendous NMDA-induced.OH release at day 14 but not at day 21. Glutamate itself therefore tonically inhibited a possible free radical release through NMDA channel activation during early development.

Infantile bilateral striatal necrosis following measles.

A previously healthy 4-year-old boy presented with typical measles and demonstrated lesions confined to basal ganglia. The clinical symptoms were an abrupt onset, impaired consciousness and mutism, extrapyramidal signs and severe neurovegetative dysfunction. No modification of the cerebrospinal fluid was observed; laboratory tests were all normal with the exception of a positive serologic test for measles. Cranial magnetic resonance imaging showed abnormal signals in the striatum, affecting the putamen and the caudate nuclei bilaterally. Neurologic improvement occurred within 2 months, with regression of lesions on cranial imaging, suggesting that edema played an important role in the initial stage of the disease.

[Digitalis intoxication during the neonatal period: role of dehydration]. / Intoxication digitalique en période néonatale: rôle de la déshydratation.

BACKGROUND: Despite the great progress which has been made in the treatment of acute digitalis intoxication by digoxin-immune Fab, it still remains a severe complication of cardiotonic therapy. CASE REPORT: A neonate with ventricular septal defect and large left-to-right shunt was treated with digitalis and diuretics at the usual starting doses. An intensive phototherapy was also required because of a hyperbilirubinemia due to glucose-6-phosphate dehydrogenase deficiency. Toxic digoxin accumulation (plasma level 14 ng/mL) was diagnosed three days after the initiation of treatment by the presence of sinus bradycardia and bursts of ventricular fibrillation. Intravenous administration of digoxin-specific antibody Fab fragments (Digidot) was effective, with a rapid improvement of the digitalis poisoning. CONCLUSION: Because of the particularities concerning drug distribution, metabolism and elimination of drugs in the neonatal period, the digoxin therapeutic index is narrow. This case report suggests the involvement of phototherapy and diuretics, which might induce a significant decrease in extracellular water and drug distribution volumes, ultimately promoting the occurrence of an intoxication.

[Mycoplasma pneumoniae meningoencephalitis]. / Méningoencéphalite à Mycoplasma pneumoniae.

BACKGROUND: Severe central nervous system diseases, such as encephalitis, have been reported in association with Mycoplasma pneumoniae infections. CASE REPORT: After an ENT infection, a 9-year-old boy with Down's syndrome developed encephalitis revealed by an acute alteration in consciousness. Head computed tomography showed, after 2 weeks, an infiltration in the basal ganglia region. The diagnosis of Mycoplasma pneumoniae encephalitis was made; recovery was complete in a few weeks. CONCLUSION: Mycoplasma pneumoniae infection should be considered in all cases of acute encephalopathy; yet the pathogenesis of the disorder is unknown and the treatment uncertain.

[Treatment of early-onset generalized dystonia by chronic bilateral stimulation of the internal globus pallidus. Apropos of a case]. / Traitement de la dystonie généralisée à début précoce par stimulation chronique bilatérale des globus pallidus internes. A propos d'un cas.

Dystonia musculorum deformans is an inherited severe disease, with a wide clinical polymorphism. The most severe clinical forms with early onset carry a high risk of life-threatening complications. In the absence of any efficient medical treatment, bilateral pallidotomy has previously been reported to be of value in the management of this disease. We report the first clinical case of a severe early-onset generalized dystonia dramatically improved by a bilateral stimulation of the internal globus pallidus. In November 1996, we proposed this neurosurgical procedure for a 8-year-old girl, who had suffered since the age of 3 from severe generalized dystonia, and who progressively became totally dependent and bedridden. She had been under sedation and permanent controlled respiratory assistance for the last two months. The etiology of the disease remained unknown (the DYT1 mutation was absent). Under general anesthesia, we bilaterally implanted a four-contacts electrode in the internal globus pallidus, using the Leksell's stereotactic frame and a 1.5 tesla MRI control. A dramatic improvement was noted 6 weeks later and led us to connect the two electrodes to neurostimulators inserted under the abdominal skin.