[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.

[Chikungunya virus infections in children]. / Infections à virus Chikungunya chez l'enfant.

Chikungunya fever is an arbovirosis caused by an alphavirus (CHIKV) belonging to the Togaviridae family. Its main vectors are Aedes mosquitoes. In its classic form, Chikungunya consists in a flu-like illness that can be very disabling, especially by incapacitating arthralgia. In children, the arthropathy is well known to be better tolerated than in adulthood but severe manifestations and complications can occur owing to neurologic, cardiac, hematologic or cutaneous dysfunctions, all carrying a fatality risk in the absence of appropriate intensive care. Out of these, the most singular is a severe encephalopathy, even in some cases genuine encephalitis. More rare, but quite specific of small infants, skin blisters have been reported, sometimes complicated by extensive detachments. Mother-to-child infections were demonstrated on La Réunion island with a fifty-percent probability of vertical transmission when the mother was highly viremic around the term of pregnancy. The diagnosis can be made by detecting CHIKV RNA using RT-PCR or specific IgM antibodies using MAC-Elisa serology. Chikungunya is a notifiable disease. The epidemic that emerged in Indian Ocean islands during 2005-2006, its progressive extension to Asia and even to Italy in July 2007, highlighted a very important capacity of CHIKV to cause huge outbreaks wherever Aedes sp. can proliferate. In France, Aedes albopictus is definitively endemic in the departments of Alpes-Maritimes since 2004, Corsica since 2005, and Var since 2007. Therefore, the risk of introduction of CHIKV from an epidemic area to Europe and especially in France is real.

[Lymphocytis meningitis: Listeria monocytogenes is a potential risk in a immunocompetent child]. / Méningite lymphocytaire: Listeria monocytogenes, une étiologie à ne pas négliger chez l'enfant immunocompétent.

UNLABELLED: Meningoencephalitis due to Listeria monocytogenes is a rare and serious form of brainstem infection in childhood. OBSERVATION: We report the case of a 7 year-old girl presenting lymphocytic meningitis with a high CRP level. Parenteral antibiotics combining ceftriaxone and vancomycine led initially to clinical improvement. Ten days later, secondary brainstem inflammation with hydrocephalus appeared and led to the detection of L. monocytogenes during external ventricular bypass. CONCLUSION: This observation of paediatric lymphocytic meningoencephalitis suggests a prescription of amoxicillin in association with first line antibiotics, particularly when an important inflammatory syndrome exists, immunocompetent children included.

Urinary excretion of free cysteine in critically ill neonates.

UNLABELLED: In preliminary observations, significant amounts of free cysteine, a neurotoxic amino acid, were noted in the urine of asphyxiated or septic-shocked neonates. The present study was conducted to determine whether free urinary cysteine was elevated in these critically ill neonates compared with a control group, and to assess the clinical significance of this generation. Free cysteine was measured in the urine of newborn infants with perinatal asphyxia (n = 16) or neonatal sepsis (n = 14) and the urine of a control group (n = 10) by ion-exchange chromatography. Relationships between cysteine levels and the clinical severity, sulfite supply and neurological outcome of the patients were then studied. Urinary cysteine was 27.6 (15-49) mmol mol(-1) creatinine for the patients but was not detectable in the control group. Cysteine levels were correlated with the severity of neonatal septic shock but not with the grade of perinatal asphyxia and did not have a specific influence on the neurological outcome of these patients. The correlation between cysteine level and the severity of neonatal septic shock was indirect and probably linked to higher sulfite administration in this population. CONCLUSION: The mean daily supply of sulfites is high in critically ill neonates, mainly originating from dopamine and generating significant amounts of cysteine. Although a worsening effect attributable to cysteine on the neurological outcome of the patients could not be demonstrated, the appropriateness of cryptic administration of sulfites by way of drug excipients is called into question.

Infantile bilateral striatal necrosis following measles.

A previously healthy 4-year-old boy presented with typical measles and demonstrated lesions confined to basal ganglia. The clinical symptoms were an abrupt onset, impaired consciousness and mutism, extrapyramidal signs and severe neurovegetative dysfunction. No modification of the cerebrospinal fluid was observed; laboratory tests were all normal with the exception of a positive serologic test for measles. Cranial magnetic resonance imaging showed abnormal signals in the striatum, affecting the putamen and the caudate nuclei bilaterally. Neurologic improvement occurred within 2 months, with regression of lesions on cranial imaging, suggesting that edema played an important role in the initial stage of the disease.