[Cardiac sinus node dysfunction due to a new mutation of the SCN5A gene]. / Une dysfonction sinusale en rapport avec une nouvelle mutation faux-sens du gène SCN5A.

A 10-year-old child was hospitalized for bradycardia during a viral infection with chikungunya. His history showed unexplored episodes of bradycardia. Cardiologic explorations revealed cardiac sinus node dysfunction (SD). Mutational screening of the SCN5A gene showed that this case was a compound heterozygote for p.Ala735Val and p.Asp1792Asn missense mutants. Five years later, the child underwent a pacemaker insertion after an electrophysiological study performed during an atrial flutter access.

[Five-year outcome of mother-to-child transmission of chikungunya virus]. / Devenir a 5 ans des infections materno-foetales a virus chikungunya.

Chikungunya virus is an arbovirus (alphavirus) transmitted by Aedes albopictus in Reunion Island. A huge chikungunya outbreak swept Reunion Island in 2005- 2006. We report the first case of chikungunya neonatal infection and the 5-years outcome of the 18 neonates hospitalized in neonatal intensive care unit between June 2005 and March 2006.

[Collodion baby and respiratory distress syndrome]. / Bébé collodion et syndrome de détresse respiratoire.

The congenital ichthyosis appears mainly in the form of collodion baby. This one can be suspected in case of polyhydramnios associated with a snowy aspect of the amniotic liquid in the antenatal echography. The reported four cases of collodion baby were premature children having presented a respiratory distress syndrome of variable gravity. The pulmonary disease was of multifactorial origin. The outcome was favorable for four children.

[Maternal-fetal chikungunya infection associated with Bernard-Soulier syndrome]. / Infection materno-fœtale à chikungunya associée à un syndrome de Bernard-Soulier.

Neonatal thrombocytopenia may stem from several origins. A newborn was hospitalized on the 3rd day of life with thrombocytopenia likely due to maternofetal autoimmune thrombocytopenia. Two days later, he displayed a chikungunya virus infection, during a severe epidemic on Reunion Island. The characterization of human platelet antigen (HPA) in the parents showed incompatibility in the HPA-3 system; however, no detectable antiHPA-3a antibody was found in the mother's serum. The persistence of moderate thrombocytopenia led to the diagnosis, using flow cytometry, of Bernard-Soulier syndrome (BSS), with no detectable GPIbIX. This case shows that neonatal inherited thrombocytopenia may be difficult to characterize, especially when it is associated with an acquired cause.

[Subgaleal hematoma in 2 neonates]. / Hématome du scalp chez 2 nouveau-nés.

Subgaleal hematoma in the newborn infant is rare, occurs early, and often bears serious consequences. We report on 2 subacute cases of bruising of the scalp that occurred following the use of a suction cup. Emergency treatment consisted of a transfusion of packed red blood cells and fresh frozen plasma. Children born by use of vacuum extractor or forceps require careful monitoring by the nursing staff throughout their stay in the maternity unit.

[Malignant pertussis: 3 case reports]. / La coqueluche maligne : à propos de 3 observations.

Three infants aged less than 2 months were hospitalized for malignant pertussis. Echocardiography showed pulmonary hypertension. High-frequency oscillations and nitric oxide were ineffective. Respiratory and hemodynamic conditions deteriorated secondarily. The third case received an exchange transfusion without success. All three infants died following multiorgan failure. Malignant pertussis is the leading cause of infectious death in infants less than 2 months of age, treatment is often ineffective, and prevention, targeting the population of young adults, is particularly important.

[Twin pregnancies in southern Reunion Island: a three-year cross-sectional study of risk factors and complications]. / Grossesses gémellaires dans le sud de l'île de la Réunion: étude transversale sur 3 ans des facteurs de risque et des complications.

OBJECTIVES: To describe characteristics of multiples pregnancies in southern Reunion Island. MATERIAL AND METHODS: A three-year [corrected] cross-sectional observational study aimed at describing risk factors, events and [corrcected] complications and associated with twin pregnancies from 22 weeks gestation onward, within a tertiary care hospital centre, the Groupe Hospitalier Sud-Réunion. RESULTS: The study included 241 multiple pregnancies out of 15 837 pregnant women. The regional incidence of multiple pregnancies was 1.5%. Indicators of eligible twin pregnancies (n=234) were mother age up or equal to 35 years (63% of women, OR 8.4; CI95% 6.3-11.1), infertility treatments (15%): ovarian stimulations (OR: 50.2; CI95% 24.7-102) and in vitro-fertilisations (OR: 44.3; CI95% 22.6-86.3). A low maternal corpulence before pregnancy (BMI<20) and celibacy were negatively associated with twin pregnancies. Prenatal cares for twin pregnancies were globally adequate. The need for hospitalisation was 50% (30% of whom for preterm delivery threats). Twin pregnancies increased risks for pre eclampsia (OR: 3.0; CI95% 1.6-5.7) and gestational diabetes (OR: 1.9; CI95% 1.2-2.8). Caesareans and instrumental delivery rates were 50% and 12% (vs 16% and 7%), respectively. Twin infants were preterm for 62% and very preterm for 18%. Last, they were more likely to die than singletons (perinatal mortality: 78 per thousand for monochorionic twins, 57 per thousand for bichorionic twins vs 17 per thousand, p<0.0001, respectively). CONCLUSION: In Reunion Island, the incidence of multiple pregnancies is similar to that seen in Europe, but seems less due to assisted reproduction techniques. In return, they are associated with higher morbidity and mortality rates.

[Full term abdominal pregnancy]. / Grossesse abdominale menée à terme.

Abdominal pregnancy is a rare localization of ectopic pregnancy, more frequently observed in underdeveloped countries. We report a case of abdominal pregnancy carried to full term delivery, discovered at a time of a cesarean for low site of placenta attachment. Discovery of an abdominal pregnancy at the time of C-section seems exceptional with clinical and ulrasonographic surveillance of pregnancy. The objective of our article is to emphasize the importance of localizing the appendix at the first quarter echography and the utility of the endovaginal ulrasound.

[Chikungunya infection in pregnancy: Evidence for intrauterine infection in pregnant women and vertical transmission in the parturient. Survey of the Reunion Island outbreak]. / Infection à Chikungunya chez la femme enceinte et risque de transmission materno-foetale.

PURPOSE: Since February 2005, an outbreak of Chikungunya virus (CHIKV) infections occurred in Reunion Island. It is transmitted by the Aedes albopictus mosquito. Neonatal cases observations suggest possible fetal transmission during pregnancy. MATERIAL [corrected] AND METHODS. Observations made in 160 pregnant mothers infected by CHIKV between June 1, 2005 and February 28, 2006, in the south of Reunion island were recorded. RESULTS: Three of nine miscarriages before 22 weeks of gestation could be attributed to the virus. 3,829 births took place during this time. Among the 151 infected women, 118 were viremia negative at delivery, and none of the newborns showed any damage. Among the 33 with positive viremia at delivery, 16 newborns (48.5%) presented neonatal Chikungunya. DISCUSSION: Though fetal contamination risks appear to be rare before 22 weeks of gestation, they are potentially dangerous. After 22 weeks gestation, newborns infection occurs if the mother is viremia positive at delivery. Transplacental transmission is suspected, but the pathogenic mechanism remains unknown.

[Herpetic neonatal hepatitis]. / Les hépatites herpétiques néonatales.

Herpes simplex virus (HSV) infection can affect various organs-systems in the neonatal period. Herpetic hepatitis was seldom reported in the literature. We report on 2 cases. Firstly, a 16 day-old newborn infant was admitted because of haemorrhagic syndrome and shock. Biological assessment showed a severe hepatic insufficiency. Antibiotic and aciclovir therapy was started as HSV infection was suspected. Five days later, the herpetic attack was confirmed by polymerase chain reaction (PCR) in blood and cerebrospinal fluid (CSF). The genotye of the virus in the CSF was HSV1. Treatment included aciclovir for 21 days intravenously and 2 months orally. At 10 months, the clinical and biological examinations were normal. Secondly, a 4 day-old newborn was hospitalised because of fever and polypnea. Pulmonary X rays showed heterogeneous opacities of the right base. Serum C reactive protein was 30 mg/l. Antibiotic therapy was started. Two days later, the fever persisted while a severe hepatic insufficiency developed. The diagnosis of herpetic hepatitis was evoked and the child was given aciclovir. Forty-eight hours later, the PCR confirmed a HSV in blood, while viral culture of a mouth swab found HSV 2. Evolution was favourable after 21 days of specific and symptomatic treatment. Aciclovir treatment was continued orally for six months. Herpetic hepatitis is rare in the neonatal period. Diagnosis must be evoked early when facing severe neonatal hepatic insufficiency. Provided specific treatment, prognosis is good.

[Congenital varicella: limits of prenatal diagnosis]. / La varicelle congénitale: les limites du diagnostic prénatal.

INTRODUCTION: Primary varicella infection during pregnancy is uncommon. Fetal varicella syndrome is unusual when varicella occurs after 20 weeks of gestation. CASE REPORT: A mother contracted chicken pox at 21 weeks and 3 days of gestation. Monthly monitoring was assured by the center for prenatal diagnosis, starting from 23 weeks. At 36 weeks, foetal echography detected liver calcifications, without other lesions. At 38 weeks, the patient went into spontaneous labour and delivered a male baby. The baby presented cicatricial skin lesions all over the body and scalp. The cerebral scan detected calcifications and a bilateral chorioretinitis was noticed. At 12 months, the infant had delayed psychomotor acquisitions, a cerebral cortical atrophy and blindness. CONCLUSION: The presence of fetal liver calcifications after chicken pox in the mother is a seldom reported sign. In our observation, liver calcifications were the single sign of a severe fetal damage.

[Post mortem brain MRI: an alternative for pathology examination in Bourneville tuberous sclerosis of the fetus?]. / IRM cérébrale post mortem: une substitution à l'examen anatomo-pathologique dans la sclérose tubéreuse de Bourneville du foetus?

Antenatal discovery of cardiac rhabdomyomes evokes the diagnosis of Bourneville's disease. Antenatal brain exploration with ultrasonography and magnetic resonance imaging (MRI) can highlight cerebral localizations. In the event of termination of pregnancy, confirmation of the cerebral lesions can be achieved with post mortem MRI as well as pathology examination. MRI can be usefully employed in the event pathology examination is not feasible.

[Maternal toxoplasmosis before conception and chorioretinitis in twin sisters]. / Toxoplasmose maternelle anté-conceptionnelle et choriorétinite chez des jumelles.

A maternal toxoplasmosis before conception is exceptionally transmitted to the fetus. We report an observation of twin sisters who presented congenital toxoplasmosis with chorioretinitis detected at nine months of age. The anamnesis revealed that the mother had had toxoplasmosis one month before conception. In the event of preconceptual infections, we propose fetal ultrasonography, histological examination of the placenta at delivery, as well as a pediatric follow-up of the infants (serological samples every month, cranial ultrasonography, fundus oculi).

[Severe cytomegalic inclusions disease in the fetus of a woman immunized before pregnancy]. / Maladie des inclusions cytomégaliques sévères chez le foetus d'une femme immunisée avant la grossesse.

After detection of a fetal microcephaly at 24 weeks gestation, we performed an amniocentesis at 29 weeks with chromosomal and polymerase chain reaction (PCR) search for viral contamination. Cytomegalovirus (CMV) infection was confirmed by PCR although the mother had previously been tested as immunized for CMV prior to conception. Abortion was induced; the fetus presented clinical CMV injuries confirmed by positive tissue culture (liver, brain and lungs). Recent publications have reported similar observations with variable viral strains. These findings point out the importance attentive search for ultrasonographic signs suggestive of fetal CMV infection.