[Severe forms of chikungunya virus infection in a pediatric intensive care unit on Reunion Island]. / Infections graves a virus chikungunya en réanimation pédiatrique a l'ile de La Réunion.

UNLABELLED: In 2005-2006, an unexpected, massive outbreak of chikungunya occurred on Reunion Island, a French overseas territory in the Indian Ocean. This arboviral infection transmitted by a mosquito of the Aedes genus is usually benign. A surprising feature of the Reunion Island epidemic was the occurrence of rare severe forms involving adults as well as children. OBJECTIVES: The purpose of this report is to describe severe forms of chikungunya observed in children hospitalized in a pediatric intensive care unit. PATIENTS AND METHODS: This retrospective single-center study was conducted from January 1st to April 30th, 2006. Children between 1 month and 15 years admitted to the pediatric intensive care unit with proven chikungunya infection were included. RESULTS: A total of 9 children were included. The main manifestations were extensive skin blisters in 5 cases, neurological symptoms (encephalopathy) in 4, cardiac complications (myocarditis, hemodynamic disorders) in 5 and bleeding in 1. Two children died. The causes of death were circulatory failure associated with coma and massive hemorrhage in one case and post-infectious encephalitis in the other. Three survivors present long-term neurologic or dermatologic sequels. DISCUSSION: Severe cases of chikungunya in children provide a stark reminder of the cardiac and neurological tropism of the virus and its hemorrhagic forms with high potential mortality and morbidity. These cases underline the need for personal protection measures and for research to develop specific antiviral therapy and vaccines to prevent potentially lethal forms of the disease.

Management of neonatal spontaneous intestinal perforation by peritoneal needle aspiration.

OBJECTIVE: To describe conservative management of spontaneous intestinal perforation (SIP) in preterm infants using peritoneal needle aspiration (PNA). STUDY DESIGN: Monocentric retrospective review of SIP cases treated primarily by PNA between 1999 and 2015 (n=31). RESULTS: Mean gestational age was 29.2±2.4 weeks and birthweight 1149±428 g. SIP occurred at 3.7±2.2 days of life. PNA achieved definitive treatment in 18 patients (60%) with a mean of 1.8 (±0.8) procedures. All patients requiring more than three PNAs had secondary laparotomy. Two patients died and five presented severe cerebral lesions. Full enteral feeding was achieved 42±18 days after SIP. Intestinal morbidity included cholestasis (n=6), intestinal stricture (n=1) and growth restriction (n=22). On follow-up (n=25, median=4 years), no severe impairment was noted. Seventeen children (68%) had a normal development. CONCLUSION: PNA as primary therapy for SIP is a viable option, resulting in definitive treatment in 60% of cases, with limited mortality and morbidity.

[Fryns syndrome. Report on 3 new cases]. / Syndrome de Fryns. Présentation de 3 nouvelles observations.

BACKGROUND: Fryns syndrome is a lethal autosomal recessive syndrome which includes congenital diaphragmatic hernia (CDH), pulmonary hypoplasia, cranio-facial manifestations including a coarse face, a cleft palate / lip and cloudy corneae, distal limb hypoplasia and internal malformations including central nervous system, cardiovascular, gastrointestinal, urogenital and skeletal anomalies. CASES REPORT: We report on 3 patients with Fryns syndrome in the french Indian Ocean islands (La Réunion and Mayotte islands). Pregnancies were complicated by polyhydramnios. All patients had coarse face, cloudy corneae, cleft lip / palate or high-arched palate, hypoplastic nails and brachytelephalangy. They died in the neonatal period. Two had CDH and 1 did not have a diaphragmatic involvement but a severe respiratory distress syndrome with refractory hypoxemia. Cytogenetic studies of blood lymphocytes and skin fibroblasts were normal. CONCLUSIONS: Fryns syndrome is the most common multiple congenital anomaly associated with CDH. The diagnosis is strongly suggested when CHD and brachytelephalangy are associated. Phenotypes with CDH similar to Fryns syndrome have been described with cytogenetic aberrations with G-banded chromosome analysis and submicroscopic chromosome deletions detected by high resolution karyotyping or array comparative genomic hybridization (CGH). Exclusion of chromosome aberrations (including isochromosome 12p in skin fibroblasts) is needed prior to making the diagnosis of Fryns syndrome. In sporadic cases, high resolution karyotyping or array CGH should be performed for correct diagnosis and genetic counselling.

Fryns syndrome without diaphragmatic hernia. Report on a new case and review of the literature.

Fryns syndrome is an autosomal recessive multiple congenital anomaly syndrome characterized by coarse facies, diaphragmatic hernia, distal limb hypoplasia and malformations of the cardiovascular, gastrointestinal, genitourinary and central nervous systems. Diaphragmatic hernia is a leading diagnostic feature in Fryns syndrome, recorded in more than 80% of cases. We report a newborn with clinical features of Fryns syndrome except the diaphragmatic hernia. Cases of Fryns syndrome without diaphragmatic hernia are reviewed. Even in the absence of diaphragmatic hernia, pulmonary anomalies are described in Fryns syndrome, especially pulmonary hypoplasia. Fetal mice, exposed to nitrofen, have a high incidence of congenital diaphragmatic hernia and other malformations similar to that seen in Fryns syndrome. Nitrofen might target molecular mechanisms similar to those involved in Fryns syndrome.

[Primary amebic meningoencephalitis: 1st case observed in Madagascar]. / Méningo-encéphalite primitive à amibes libres: 1er cas observé à Madagascar.

The primary amebic meningoencephalitis is an acute suppurative infection that involves both the brain and the meninges. It is caused by Naegleria fowleri and is a very rare and fulminating condition, so far nearly always fatal. We report the first case in the area of Southern part of Indian Ocean that occured in a 7-year old French boy living in Madagascar. It is assumed that the disease was contracted by swimming in warm fresh water in a lake of the Madagascar east coast. Clinical signs began 10-12 days after exposure, associating headache, vomiting and pyrexia (39-40 degrees C). Upon admission in a Madagascar hospital, the patient was started on antibiotics, that did not control the disease and soon presented with a loss of consciousness and a delirium. He was transferred to Reunion island (Centre Hospitalier Départemental Félix-Guyon), where the diagnosis of primary amebic meningoencephalitis was confirmed. Therefore, he was started on high-dose of intraspinal amphotericin B, IV amphotericin B lipid complex and tetracycline. He developed myocarditis, diabetes insipidus, deep coma and subsequently died a week later. The diagnosis of amebic meningoencephalitis was based on: -- the cerebrospinal fluid examination that confirmed the diagnosis of purulent meningitis: 420 leucocytes (76% polynuclears, 14% lymphocytes), 90 red blood cells, and showed 50 ameboid trophozoites per 100 leucocytes, approximately 20 microm in size. -- the flagellate transformation test in distilled water showed two anterior flagellas that confirmed the genus Naegleria.

Peritoneal needle suction for intestinal perforation in the preterm neonate.

UNLABELLED: A few years ago, most intestinal perforations in the premature newborn appeared within the clinical context of necrotising enterocolitis (NEC). Since then, we have observed an increase in the number of isolated perforations appearing outside typical NEC. The fact that the perforations are more often isolated, and the healing capabilities of the premature intestine, led us to propose peritoneal needle suction (PNS) alone as first treatment for intestinal perforations in the premature neonate. MATERIALS AND METHOD: The charts of 6 consecutive premature infants presenting with intestinal perforations treated initially by PNS alone were reviewed. RESULTS: The patients' median birth weight was 1030 g, with a median gestational age of 27 weeks. In 5 out of 6 infants (83 %), PNS achieved complete exsufflation without recurrence of the pneumoperitoneum and complete intestinal healing, allowing complete enteral feeding 30 to 71 days after perforation. One infant with recurrent pneumoperitoneum after 3 PNS and peritoneal drainage was operated. All infants survived. CONCLUSION: We believe that for early perforations of the premature neonate, the poor diffusion of the infection and the frequent capacity of the perforation to close and subsequently heal without scars, favour a minimally invasive management using PNS.

[Diagnostic value of granulocyte elastase determination in cord blood of newborn infants at risk for maternofetal infection]. / Valeur diagnostique du dosage de l'élastase granulocytaire au sang du cordon ombilical chez des nouveau-nés en situation de risque d'infection maternofoetale.

BACKGROUND: Polymorphonuclear elastase is an early and sensitive indicator of neonatal infection when performed at the beginning of clinical symptoms. PATIENTS AND METHODS: To investigate the diagnostic value of elastase measurement in cord blood immediately after birth, 211 neonates (103 boys vs 108 girls, 154 vaginal delivery vs 57 cesarean section). Mean gestational age 38.9 weeks (range: 30-42), mean birth weight 3,260 g (range: 1,430-4,920 g). After clinical, bacterial and biological screening, the infants were classified in three groups. Group A (n = 118): none infectious risk factor neither clinical signs of infection; group B (n = 79): one or more risk factors but no evidence of infection; group C (n = 14): proved or probable infection. Polymorphonuclear elastase was measured in cord blood of all infants using an heterogeneous enzyme-linked-immunosorbent assay. RESULTS: We observed higher elastase values in group C (176 +/- 67 micrograms/L) than in group A (91 +/- 64 micrograms/L) and B (67 +/- 61 micrograms/L) (mean +/- SD, P = 0.0001). With a cutoff value fixed at 80 micrograms/L, the sensitivity of this test applicated to neonates presenting materno-fetal infectious risk factor(s) was 85% (12/14), specificity 74% (59/79), positive predictive value 37%, and negative predictive value 96%. CONCLUSION: Because two of the 14 infected infants (15%) were not detected by elastase dosage in cord blood, this test cannot be used as an early indicator of materno-fetal infection.

[Bronchopulmonary dysplasia and cytomegalovirus pneumonia]. / Dysplasie bronchopulmonaire et pneumopathie à cytomégalovirus.

Hyaline membrane disease (HMD) of premature newborn can lead to bronchopulmonary dysplasia (BPD). We report the observation of a 33 weeks premature newborn with HMD, treated with exogenous surfactant and mechanical ventilation. The patient developed respiratory distress with oxygen dependency initially related to BPD. Because of worsening of respiratory condition after 2 months, despite corticosteroid therapy, further investigation was performed. Cytomegalovirus (CMV) was found in urine and in the pharynx and CMV-pneumonia was diagnosed. Treatment with gancyclovir allowed a rapid regression of symptoms. Association between BPD and CMV-pneumonia has been previously reported but the causal relationship is controversial. In premature newborn, CMV-pneumonia can appear clinically and radiologically like a BPD. When evolution is atypical, with persistence of respiratory distress despite BPD treatment, CMV-pneumonia must be considered as a specific antiviral therapy may be discussed.

[Neonatal accidental burn by isopropyl alcohol]. / Brûlure néonatale accidentelle à l'isopropanol.

Antiseptic solutions are commonly used for skin care but are not always safe. In preterm infants, application of antiseptic solutions can lead to serious burns. We report the case of a premature newborn who developed severe burns at 35 weeks post-conceptional age, after his mother used disposable antiseptic towels containing isopropyl alcohol for his skin care. Burns outcome led to diffuse skin burn and death of the baby. Several cases of isopropyl alcohol poisoning through skin absorption have been reported in neonate and infants. Because of its neonatal toxicity, isopropyl alcohol has been excluded from composition of antiseptic solutions commonly used in neonatology. However, isopropyl alcohol is still available in many housecleaning and cosmetic products, while its toxic effects in children are not clearly mentioned. A specific mention "toxic for infants and children" should appear on mass consumption products containing isopropyl alcohol. Moreover, health workers may individually inform parents about possible hazards of poisoning through skin absorption.

[Value of toxicological research in newborn infants of addicted mothers by the study of several samples (urine, meconium, hair)]. / Intérêt des recherches toxicologiques chez les nouveau-nés de mères toxicomanes par l'étude de plusieurs échantillons (urines, méconium, cheveux).

BACKGROUND: Urinary detection of prenatal drug exposure in the neonate may give false-negative results. We report our experience on meconium and hair testing, in addition to urine testing in order to improve diagnosis of fetal drug exposure. POPULATION AND METHODS: Thirty-one infants (aged 1-45 days) whose mothers were confirmed (n = 12) or suspected (n = 19) to be drug-addicted were included in the study. One or more specimens of urine, meconium or hair were collected in the 31 infants, two of the specimens in 17 and three in six. Drugs and their metabolites were detected by immunoenzymologic techniques and positive results were confirmed by gas-exchange chromatography. All the mothers and families were interviewed during admission and the information was compared to those provided by medical and social services; the results of laboratory analysis were not known by the investigators at this time of the study. RESULTS: The maternal drug addiction was confirmed after clinical investigation in 18 cases including the 12 cases detected by prenatal interview (group 1), and recused in 13 other cases (group 2). In group 1, nine infants of 12 had a positive urine test (seven opiate, one cocaine, one cannabis), 11 of 11 a positive meconium test (nine opiate, one cocaine, one cannabis), ten of 19 a positive hair test (eight opiate, one cocaine, one cannabis); all infants in this group had at least one positive result. In group 2, all tests were negative except one urine test positive for opiate after cesarean delivery performed under anesthesia including opiate analgesia. CONCLUSIONS: Urine, meconium and hair testing versus urine testing alone increase the sensitivity of laboratory analysis for detection of prenatal drug exposure.

[27-gauge percutaneous catheters: use in nutritional management of very-low-birth-weight and severely premature newborns in a neonatal intensive care unit]. / Cathéters épicutanéocaves 27 gauge: utilisation chez les nouveau-nés de très faible poids de naissance et les grands prématurés dans un service de réanimation néonatale.

UNLABELLED: Percutaneous central venous catheterization for parenteral feeding is a permanent problem in the management of very low birth weight neonates. Usually, 23-gauge catheters (diameter: 0.6 mm) are used. In our unit, we have started to use 27-gauge catheters (diameter 0.35 mm) since 1995. The aim of this study was to report our experience with this materiel. SUBJECT AND METHODS: Between September 1, 1997 and January 30, 2000, 352 catheter implantations were performed using 27-gauge infusing sets in 200 premature infants (gestational age less than or equal to 33 weeks [mean 29.2], weight less than or equal to 1,500 g [mean: 1, 152.5 g]). Data were reviewed retrospectively with the view to determine the modality of use and complications. Peculiar insertion modalities were prospectively evaluated in the 50 last included infants (92 catheters). RESULTS: In 97% of cases, the physician needed no help to insert the catheter. In 99.5% of cases (199 neonates), the insertion was successful (the procedure failed in one case). The mean duration procedure was 26 min (range 10 to 85 min). The mean age at insertion of the first catheter was 29 hours (range 0 to 216). Mean catheter maintenance duration was 15 days (range 1 to 53). In 31 cases, documented sepsis related to the catheter were noted (8.8% or 5.8 infections for 1,000 catheter-days). Endocarditis was observed in one case. A pericardial effusion was diagnosed in two cases. No death related to the catheter was noted. CONCLUSION: In our experience, 27-gauge catheter implantation is an easy and safe procedure. We noted no major maintenance problems. Complications were not observed more frequently than those usually encountered with 23-gauge catheters. We conclude that 27-gauge catheter utilization is an attractive alternative for percutaneous central venous catheterization, in very low birth weight neonates.

[Early continuous positive pressure in the labor room]. / Pression positive continue précoce en salle de travail.

BACKGROUND: Indication for intubation and mechanical ventilation in premature infants may be reduced by initiating continuous positive airway pressure (CPAP) in delivery room. POPULATION AND METHODS: Immediately after birth, respiratory support with CPAP was given to all infants with gestational age less than 32 weeks. In case of apnea or progressing symptoms with hypoxemia or carbonic acidosis, with PCO2 increasing to more than 60 mmHg, infants were treated with nasotracheal intubation and ventilation. RESULTS: One hundred and fifty one infants, with mean gestational age 29.6 +/- 1.9 weeks and mean birth weight 1,326 +/- 378 g were delivered in the obstetrical department of Marseille. In delivery room, 63% were treated with CPAP, and only 13% with nasotracheal intubation. The need for subsequent mechanical ventilation was reduced to 40% of the population. Surfactant therapy was used in 17% of this cohort. Two infants were given surfactant and extubated. Three of 14 deaths (9.2%) were caused by respiratory disease. CONCLUSIONS: Early CPAP reduces the indication of mechanical ventilation in premature infants. Incidence of pulmonary complications such as pneumothorax or bronchopulmonary dysplasia is low among those infants who require mechanical ventilation later. Early CPAP takes place in a general policy to decrease neonatal morbidity.

[Feingold syndrome]. / Syndrome de Feingold.

BACKGROUND: Feingold syndrome is a combination of hand and foot anomalies, microcephaly, tracheo-esophageal fistula, esophageal/duodenal atresia, short palpebral fissures and developmental delay. The most frequent physical findings are clinodactyly of the second and fifth fingers with absent or hypoplastic middle phalanges, and syndactyly of the toes. Inheritance is autosomal dominant (MIM number 164280) with full penetrance concerning hand anomalies, variable expressivity and great intrafamilial variability. CASE REPORT: We report the case of an African boy with duodenal atresia, microcephaly, brachymesophalangy of the second and fifth fingers, unilateral thumb hypoplasia, bilateral syndactyly of toes 2-3 and amesophalangy of the toes. Karyotype was normal. No other member of the family was affected. A fresh mutation is possible. CONCLUSION: Brachymesophalangy affecting mainly the second and fifth fingers and amesophalangy of the lateral toes are cardinal clinical findings in Feingold syndrome. These clinical findings define the syndrome of brachydactyly A4-Temtamy type (MIM 112800), suggesting that brachydactyly A4 would be a partially expressed Feingold syndrome. The incidence of esophageal/duodenal atresia is 29%, including all the known cases. All karyotypes of reported patients were normal.

[Malignant pertussis in the young infant]. / La coqueluche maligne du petit nourrisson.

UNLABELLED: Pertussis remains in France the first cause of bacterial, infectious death in infant aged 10 days to 2 months. It is especially in this age group that malignant pertussis occurs. CASE REPORT: A 40-day-old infant was admitted in the intensive care unit with symptoms of bronchiolitis along with a 200 bpm permanent tachycardia. He presented a marked leukocytosis with lymphocytosis. On the second day, convulsions and coma occurred, followed rapidly by respiratory failure, with a subsequent deterioration due to the development of severe pulmonary hypertension. Circulatory failure caused the infant's death on the beginning of the 5th day. Pertussis was confirmed by PCR on nasopharyngeal swab. Intra-familial contamination was most likely. COMMENTS: Malignant pertussis is characterized by the very young age of patients, permanent tachycardia sine materia, dyspnea with early respiratory failure, frequent neurological symptoms, severe hyperleukocytosis and hyperlymphocytosis, and deep hyponatremia with oliguria and edema. Mortality remains superior to 75% despite the various treatments and life support measures that have been attempted. Adult pertussis, which represents one third of the cases of prolonged cough in this age group, is the main source of contamination of non-immunized young infants. This mode of transmission stresses the importance of the generalization of pertussis vaccine booster in early adolescence, which is recommended in France since 1998. Its extension towards the adult age is under study.

[Validation trial of a neonatal prophylactic antibiotic protocol in the delivery room]. / Essai de validation d'un protocole d'antibioprophylaxie néonatale en salle de travail.

Maternofetal infection occurs in 1 to 10 of every 1,000 newborns. Prognosis is poor and an extremely rapid the clinical course is sometimes observed. The situation suggests that widespread use of antibiotics and more than 90% of the newborns receiving antibiotics are not infected. A prospective study based on simple, recognized criteria predictive of maternal-fetal infection was conducted in 3,392 deliveries to evaluate the effect of an antibiotic prophylaxy protocol. Specificity, sensitivity, positive and negative predictive value were evaluated for each of the eleven criteria retained with the goal of increasing sensitivity and decrease the use of unnecessary antibiotics. Among the 3,392 infants delivered from January 1989 to December 1990, 286 (8.4%) newborns entered the study and were given mezlocillin (150 mg/kg/12 h). This treatment was stopped at 48 hours of life if the infant was not infected. RESULTS. Infection was confirmed in 48 of 3,392 infants (1.4%). All were in the risk group: 48/286 (16.7%). The germs the most often found were group B Streptococcus (n = 16), Escherichia coli (n = 8) and Listeria monocytogenes (n = 3). Nine criteria were well correlated with maternal-fetal infection. The two most important criteria were maternal pyrexia above 39 degrees C and Apgar score below 7 to 5 min (poor neonatal adaptation), with a 99% and 90% and a positive predictive value of 80% and 37% respectively. Inversely, Two criteria were poorly correlated with maternal-fetal infection: labour duration above 12 hours and instrumental extraction (positive predictive value from 10% to 17%).(ABSTRACT TRUNCATED AT 250 WORDS)

[Prenatal diagnosis at 25 weeks gestation and neonatal management of a vallecular cyst]. / Découverte anténatale à 25 semaines d'aménorrhée et prise en charge néonatale d'un kyste de la vallécule.

Due to the anatomical location, vallecular cyst is a rare but well-recognized cause of upper airway obstruction and death in newborn. This cyst can be accurately diagnosed by echography in utero and by MR imaging. Prenatal diagnosis allows for early consultation with surgical specialist, so that the time and place of the delivery can be addressed for neonatal preoperative planning. We report the first prenatal diagnosis of a vallecular cyst at 25 weeks of gestation. At birth, the cyst was drained and then marsupialized. We believed that, in cases of oropharyngeal tumors discovered in utero, elective delivery should be realised in a tertiary referral center in which emergency ventilation and tracheostomy are possible.

[Prevalence, severity, and predictors of bronchopulmonary dysplasia in a cohort of very preterm infants]. / Prévalence, gravité et facteurs prédictifs de la dysplasie bronchopulmonaire dans une cohorte ultramarine de grands prématurés.

OBJECTIVES: The aim of this retrospective cohort study was to determine the prevalence and severity of BPD and its predictors in a regional cohort of very preterm (VP) infants in Reunion Island. METHODS: All autochthonous VP infants, live-born before the 33rd week of gestation (WG) between 1st January 2008 and 31st December 2009, were eligible for the study. Only VP infants surviving at least 28 days, for whom the parameters were known from birth, were included in the case-control study of predictors of moderate to severe BPD (BPDmo/s). RESULTS: In VP infants less than 33 WG, the rate of overall BPD (3 grades of severity) was 30.7%. Among those who survived 28 days or more, the rate of BPDmo/s was 13.1% (95%CI: 10.2-15.9%). In VP infants less than 32 WG that survived at 36 WG, the prevalence of BPDmo/s was 18.2% (95% CI: 14.2-22.1%). In a fixed-effect logistic model, adjusted for gestational age, postnatal growth, and the mode of ventilation at 24h, 4 key factors were predictive of BDPmo/s: small for gestational age, surfactant, delayed energy intake, and late-onset neonatal infection. In a mixed-effect logistic model adjusted for these same cofactors, the site was associated with BPDmo/s, in line with a center-effect. CONCLUSION: The prevalence of BPDmo/s in the mixed-race population of Reunion Island is consistent with those observed in Europe but were site-specific. In our setting, predictors of individual BPDmo/s are similar to those already identified.