[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.

[Early onset neonatal infections in the South of the Reunion Island: Incidence and use of 2002 ANAES risk criteria]. / Infection bactérienne néonatale précoce dans le sud de la Réunion: incidence et application des critères de risque Anaes 2002.

OBJECTIVES: The aim of this study was to describe the incidence of early onset neonatal infections (EONI) in the southern part of the Reunion Island, and to study the application of ANAES criteria. PATIENTS AND METHODS: A cross-sectional study was made of data collected for all live births having occurred between 1st January 2001 and 31st December 2004. RESULTS: Four hundred and thirty-seven in 16,071 neonates (out of 21,231 live births) presented with a certain or probable EONI, accounting for a regional rate of 20 per thousand (CI95 % 18-23 per thousand). Among 437 EONIs, group B streptococcus (GBS) was reported in 70.5% of the cases (n=308), Gram negative bacteria in 19.9% (n=87), of which nearly two thirds of Escherichia coli (n=56). Applying ANAES criteria led to identify 380 EONIs among 437 proven infections (sensitivity: 87%, specificity: 26%). A logistic regression analysis identified eight EONI predictors for the 7015 neonates for whom the mother GBS screening was documented: GBS positive vaginal culture (OR 4.2; CI95% 3.3-5.4), unexplained preterm birth less than 35 weeks (OR 5.7; CI95% 3.7-8.7), prolonged rupture of membranes greater than or equal to 18 hours (OR 2.1; CI95% 1.4-3.0), maternal fever greater than or equal to 37.8 degrees C (OR 3.2; CI95% 2.3-4.5), fetal tachycardia greater than or equal to 160 ppm (OR 2.7; CI95% 1.8-4.0), and thin (OR 1.6; CI95% 1.2-2.1) or thick meconium-stained amniotic fluid (OR 3.0; CI95% 2.1-4.5) or fetid fluid (OR 14.8; CI95% 4.2-51.8). CONCLUSION: The incidence of EONIS far exceeded that observed in metropolitan France, and the ANAES criteria lack sensitivity and specificity.

[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.

Diagnosis of acute renal failure in very preterm infants.

This study was designed to improve the definition of acute renal failure (ARF) in very preterm infants. Twenty-eight newborn infants with gestational age < or =32 weeks were prospectively studied in the first 5 days of life and made up a control group as they did not present risk factors for vasomotor renal insufficiency. Renal insufficiency was defined as an increase in daily serum creatinine concentration above the 99th interval limit obtained in this control group, i.e., 43 micromol/l on day 1 and/or 21 micromol/l on day 2 and/or 14 micromol/l/day on day 3 and/or 22 micromol/l/day on day 4. According to this definition, 20 very preterm infants with ARF were identified. As compared with the control group, the ARF group showed more prolonged oliguric episodes, lower diuresis, insufficient weight loss (in spite of a reduction in water intake) and also more episodes with natremia <130 mEq/l (35 vs. 0%; p <0.05) and/or kalemia >6 mEq/l (40 vs. 11%; p <0.05). Therefore, assessment of daily changes in serum creatinine concentration in very preterm infants allows the diagnosis of clinically significant reduction in glomerular filtration rate.