[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.

[Clinical aspects and genetic specificities of cystic fibrosis in Reunion Island]. / Aspects cliniques et spécificités génétiques de la mucoviscidose à l'île de la Réunion.

OBJECTIVES: Evaluation of the phenotype-genotype correlation of a specific mucoviscidosis mutation, "Y122X", in Reunion Island. This mutation represents 25% of our cases. PATIENTS AND METHODS: Retrospective study of a cohort of 84 children presenting cystic fibrosis (CF) during a 5-year period (1994-1998). Diagnosis was based on one or two identified genetic mutations and/or minimum two abnormal chloride sweat tests (Cl > 70 mmol/l). Follow-up of this cohort was performed in the two referral centers of the Island following the French national guidelines (INSERM U 155). RESULTS: In our population, we identified 10 mutations, of which three of them represented more than 80% of the cases: Delta F508 (51.8%), Y122X (24.4%) and 3120 + 1G --> A (4.8%). The authors report clinical significant differences in children with the homozygote mutation Y122X as compared with children presenting the Delta F508 CF-mutation: failure to thrive affecting mainly the height with, paradoxically, a relatively normal weight development, and a better pulmonary function. CONCLUSION: The frequent Y122X CF-mutation reported in "la Reunion" seems to affect mainly height in children with a relatively good nutritional outcome. This failure to thrive does not seem to be of digestive origin. These results suggest that growth gene(s) located nearby the cystic fibrosis transmembrane conductance regulator (CFTR) may have suffered the same segregation than the Y122X mutation or that clusters of this specific Caucasian population known as "petits blancs" in la Reunion are smallest for ethnic reasons.

[Tracheal neurinoma simulating status asthmaticus in a child]. / Neurinome de la trachée simulant un état de mal asthmatique chez l'enfant.

BACKGROUND: Neurilemmoma is a benign tumor that is rarely located in the trachea. A neurilemmoma in the intrathoracic part of the trachea can mimic severe status asthmaticus. CASE REPORT: A 14 year-old girl was admitted because of persistent signs of status asthmaticus, despite bronchodilator therapy. She had no history of asthma. A worsening of the wheezing while she was in the intensive care unit led to intubation and respiratory support. X-rays showed pneumomediastinum. A dramatic improvement only followed replacement of the intratracheal tube. On the 5th day of the disease, tracheoscopy showed a sessile tumor obstructing two-thirds of the lumen, 3 cm above carena. Biopsy showed the tumor to be a neurilemmoma; it was excised. Scar tissue developed and was responsible for stenosis; it required laser therapy and an endotracheal prosthesis. Neither the girl nor her parents showed signs of neurofibromatosis. CONCLUSIONS: An intrathoracic tracheal tumor can produce asthmatoid wheezing. A definitive diagnosis can be made only by tracheoscopy.