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.

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.

[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.

[Takotsubo cardiomyopathy in an adolescent girl]. / Cardiomyopathie de type tako-tsubo chez une adolescente.

We report the case of a 14-year-old girl who presented with symptoms of left ventricular systolic failure following the rupture of a cerebellar arteriovenous malformation. Takotsubo cardiomyopathy needs to be recognized by pediatricians because the triggering factors, such as physical or emotional stress, are common during childhood. Echocardiography showed a typical dyskinesia of the left ventricular apical or midventricular segments with a hyperkinetic basal region. Symptomatic treatment may be necessary in cases of hypotension, arrhythmias, or acute heart failure. As the prognosis is generally good, reassuring information can be given and cardiologic investigations limited.

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.

Group B streptococci in milk and late neonatal infections: an analysis of cases in the literature.

BACKGROUND: The source for late-onset neonatal infections (LONI) due to group B Streptococcus (GBS) has not been fully explored. We reviewed GBS LONI cases associated with contaminated breast milk to determine whether breast milk was a possible route for neonatal infection. DATA SOURCES: A PubMed search from January 1977 to March 2013 was performed with MeSH words "Streptococcus agalactiae", "group B Streptococcus", "infection", "milk", "human", "late-onset infection" and/or "neonate"; relevant cross references were also reviewed. RESULTS: Forty-eight documented cases of GBS LONI matched our search criteria and were retrieved from the literature. When performed, molecular typing identified clonal isolates in the neonate and milk samples taken after LONI in all cases, with the hypervirulent sequence type 17 (ST-17) clone identified in two of these cases. Caesarean delivery combined with the absence of GBS recovery from maternal samples other than milk was noted for four cases. The rate of recurrent infections was high (35%) and, together with the data reviewed, points to a potential role of breast milk in GBS LONI. CONCLUSIONS: The cases reviewed here, together with the evidence of breast milk transmission for other pathogens, suggest that breast milk, which would account for repeated GBS transmission to the neonate, may favour gut translocation and subsequent LONI. Further investigations are nevertheless needed to study the relative importance of this contamination route compared with persistent postnatal gut colonisation and the dynamics of milk and neonatal gut colonisation.

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.

[Anuric acute renal failure after suprapubic catheterization]. / Insuffisance rénale aiguë anurique après mise en place d'un cathéter sus-pubien.

Percutaneous suprapubic catheterization is an alternative when placement of a urethral catheter is contraindicated or unsuccessful. We report the case of a 3-day-old newborn, who presented anuric acute renal failure secondary to placement of a suprapubic catheter. An excessive length of catheter had been inserted into the bladder and was obstructing the vesicoureteral junctions. Several measures can be taken to prevent this complication, such as systematic measurement of the length inserted into the bladder, use of a catheter with 1cm gradations or recourse to radiopaque material.

[Neonatal seizures management]. / Prise en charge des crises du nouveau-né

For several decades, experimental studies have sought to explain the biological causes of newborn seizures and to assess the anatomical and functional consequences. Laboratory studies have shown that prolonged or repeated seizures disturb central nervous system development and may predispose to later epilepsy or cognitive deficits. Although these findings have not been clinically demonstrated in humans, several observations suggest that neonatal seizures have a deleterious effect on the immature brain and generate long-term sequelae. No therapeutic trial, however, has directly demonstrated the benefits of treatment, underlining the need for controlled studies that integrate the advances in electroencephalographic monitoring and pharmacology of anticonvulsant drugs.

[Acute intussusception, a rare cause of small bowel obstruction in premature neonates: the advantages of early diagnosis]. / L'invagination intestinale aiguë, cause rare de syndrome occlusif chez un nouveau-né prématuré : intérêt du diagnostic précoce.

We report a case of intussusception in a premature neonate who presented with early signs of upper gastrointestinal tract obstruction. Diagnosing acute intussusception in premature infants is difficult because of its infrequency relative to other neonatal abdominal problems and because the clinical symptomatology shows similarities with that of necrotizing enterocolitis. In the reported case, the diagnosis was made by sonography. Unlike full-term neonates, the presence of a pathological lead point is very infrequent. The intussusception is predominantly located in the small bowel and is most often associated with bowel compromise. This disease should be considered in all neonates with signs of intestinal obstruction to permit a better prognosis via prompt surgical intervention.

Sudden infant death syndrome (SIDS): characteristics of deaths since the fall in SIDS in the French region of Languedoc-Roussillon.

The characteristics of sudden infant death syndrome (SIDS) in the French region of Languedoc-Roussillon from 2003-2008 were examined in a retrospective study of two groups classified as unexplained sudden unexpected death in infancy (SUDI) (SIDS, n = 27) or explained SUDI (n = 22). The interval between the time the baby was last observed alive (time of last feed) and the discovery of death was evaluated. In SIDS, 67% (18/27) of deaths were discovered during the day (09:00-21:00 h) mostly within 4 h after feeding (66%). In explained SUDI, 68.2% of deaths were discovered at night and time intervals between the last feed and discovery of death were longer (mean 7.1 h, p<0.01). Most SIDS deaths were discovered during the day and explained SUDI discovered at night. Variations in time between the last feed and discovery of death in the two groups may result from differences in parental attentiveness during the day and at night.

Anaesthesia mode for caesarean section and mortality in very preterm infants: an epidemiologic study in the EPIPAGE cohort.

BACKGROUND: Little is known about the influence of anaesthesia for caesarean section on outcome in very preterm infants. METHODS: A prospective, population-based, cohort study (the EPIPAGE cohort) included all births before 33 weeks in nine French regions in 1997. Of 2360 infants live-born between 27 and 32 weeks, 1338 were delivered by caesarean section with general anaesthesia (n=711, 53.1%), spinal anaesthesia (n=419, 31.3%), or epidural anaesthesia (n=208, 15.6%). Neonatal mortality was compared among these three groups using bi- (according to gestational age and to anaesthetic technique) and multivariate analyses. RESULTS: Neonatal mortality was 10.1% with general anaesthesia, 12.2% with spinal anaesthesia and 7.7% with epidural anaesthesia. After adjustment for gestational age and characteristics of pregnancy, delivery and neonate, spinal anaesthesia was associated with a higher risk of neonatal death than general anaesthesia (adjusted odds ratio, 1.7; 95% confidence interval 1.1 to 2.6). CONCLUSION: In this population-based study, spinal anaesthesia was associated with an increased risk of neonatal mortality in very preterm infants compared to general anaesthesia (and epidural anaesthesia), independently from gestational age and characteristics of the pregnancies, deliveries and neonates. Although this multivariate analysis does not prove a causal relationship, the results suggest it could exist, particularly if maternal haemodynamics are poorly controlled. With recent significant change in the conduct of spinal anaesthesia, further studies are needed to investigate potential harmful effects of anaesthesia on very preterm infants delivered by caesarean section.

[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.

Comparison of chlorpromazine versus morphine hydrochloride for treatment of neonatal abstinence syndrome.

AIM: To compare the duration of treatment for neonatal abstinence syndrome (NAS) using chlorpromazine versus morphine hydrochloride. METHODS: We compared two case series of term infants with NAS treated with either morphine hydrochloride (MH) or chlorpromazine (CP). Seventeen infants were treated with MH from 1998 to 1999, and 20 infants were managed with CP from 2000 to 2001. The duration of treatment was compared, and multivariate analysis was used to identify independent risk factors related to the duration of treatment. RESULTS: Characteristics of the mothers (duration of drug addiction, abuse of other substances) and infants (birth weight, proportion breastfed) were similar in the two groups. The mean duration of CP treatment was 6 days (range 3.5-9 days), significantly fewer days than with MH treatment, which was 16 days (range 10-21 days; p < 0.001). There were fewer hospitalization days (11 days; range 9-14 days) for CP treatment compared with MH-treated infants (18 days; range 16-25 days). Treatment with CP was independently associated with shorter hospitalization time. CONCLUSION: CP appears to shorten the duration of NAS compared with MH. Larger prospective randomized trials are needed to confirm our findings.