[EBV infection revealing a long-chain 3-hydroxyacyl-CoA dehydrogenase (LCHAD) deficiency in a 3-year-old boy]. / Infection à EBV révélatrice à l'âge de 3 ans d'un déficit en 3-hydroxyacyl-CoA déshydrogénase des acides gras à chaîne longue (LCHAD).

OBSERVATION: We report on the case of a 3-year-old child from La Réunion island, who presented with hypoglycemic hypoketotic coma secondary to a primary Epstein-Barr virus (EBV) infection. The discovery of the G1528C homozygote mutation provided the diagnosis of long-chain-3-hydroxyacyl-CoA-dehydrogenase (LCHAD); an adapted dietary plan with prevention of fasting and L-carnitine supplementation was initiated. After 2 years, a pigmentary retinopathy appeared and muscle weakness increased. COMMENTS: Isolated LCHAD deficiency is an autosomal recessive disorder of fatty acid metabolism. Prevalence is about 1-9/100,000 and diagnosis is often made before the age of 2 years. The late age of revelation in our case is related to a spontaneous diet without animal fats (disgust for meat, diet based on white rice and skimmed milk) and nighttime breastfeeding until the age of 3 years. In an affected fetus, heterozygous mothers are susceptible to developing a hemolysis, elevated liver enzymes, low platelets (HELLP) syndrome or an acute fatty liver pregnancy (AFLP) syndrome during the 3rd trimester of pregnancy, which motivated us to set up a systematic neonatal screening program and a specific monitoring of these newborns.

[Obstetrical risk factors of 365 primiparous adolescent pregnancies in Reunion Island]. / Etude des facteurs de risques obstétricaux dans le suivi de 365 grossesses primipares adolescentes à l'île de la Réunion.

OBJECTIVES: Analysis of obstetrical risk factors in teenage primiparous pregnancies in Reunion Island (4% of total births). MATERIALS AND METHODS: Retrospective study, between 2001 and 2002, comparing primiparous adolescents (13-17 years, n = 365), with primiparous controls (18-29 years, n = 2050). The analysis included demographical factors, maternal medical histories, prenatal follow-up, obstetrical risk factors, delivery modes and neonatal characteristics. RESULTS: Adolescents attended on average 8 prenatal consultations, however 4% had poor prenatal care (less than 3 visits, OR 4.2, P < 0.001 vs controls). They presented less gestational diabetes, but there were no differences concerning pre-existing hypertension, hypertensive disorders of pregnancy, medical reasons of hospitalisation between the two groups. Mode of delivery was more favorable in adolescents (half rate of caesarean sections, shorter duration of membrane ruptures). Nevertheless, adolescents presented a higher risk of severe prematurity (<32 Weeks gestation, incidence 3.6% vs 1.6%, OR 2.3, p = 0.008). CONCLUSION: With optimal prenatal care (more than 90% of our cohort), primiparous adolescents present globally a favorable course of their pregnancies and have better deliveries than their young (18-29 years) counterparts. However, there is a significant risk of severe prematurity requiring special care for these pregnancies.

[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.