Implementation of a hepatitis A prevention policy in haemophiliacs: results from the French cohort.

In the early nineties, the occurrence of hepatitis A outbreaks in some patients with haemophilia in some countries led French health authorities to recommend hepatitis A virus (HAV) vaccination in HAV-seronegative haemophiliacs. The French 'Suivi thérapeutique National des Hémophiles' cohort permitted to assess the implementation of this recommendation by the analysis of the vaccinal process, i.e. HAV seropositivity assessment and vaccination of HAV-seronegative patients, in a survival approach. In a subgroup of 812 patients diagnosed earlier than 1990 (prevalent cohort), the implementation of vaccinal process increased quickly from 0% in 1993 to 41.8% in 1994 and to 71.2% in 1996, suggesting a 'notification effect'. The vaccinal process was associated to three cofactors in a Cox model analysis (age, severity of haemophilia, centre of treatment). No infection was observed during the survey in this group. In another subgroup of 201 boys born since 1993 (incident cohort), 27.5% and 15.4% patients remained exposed to the risk at 3 and 5 years from diagnosis respectively, again with a 'centre effect', which might be linked to various factors such as regain in confidence for products or economic reasons. Only five infectious seroconversions were assessed over the 7-year survey, which represents 14.5 cases per 1000 person-year incidence without any relationship with products. Our data combined with the contemporary hepatitis A epidemiology and the current safety of anti-haemophilic concentrates, should lead to a new assessment of the risk of hepatitis A in haemophiliacs. We suggest that among patients with bleeding disorder, as well as in other populations, HAV prevention policy might be stressed on those who already suffer from chronic liver disease and/or travel in endemic countries.

Benign childhood epilepsy with centrotemporal spikes and hippocampal atrophy.

A boy without significant family or personal history had three consecutive nocturnal seizures at 1-month intervals at age 10 years, all simple focal seizures with motor and sensory symptoms, the last with secondary generalization. Waking and napping EEG showed focal sharp changes typical of benign epilepsy with centrotemporal spikes (BECTS). A magnetic resonance imaging documented a marked right hippocampal atrophy (HA). After valproic acid (VPA) therapy, there were no more seizures, and there were fewer EEG changes. An EEG performed in the younger, fully asymptomatic 8-year-old sister documented unilateral right focal sharp waves. This case shows that HA, as well as other central nervous system lesions, can be found fortuitously in patients with BECTS.

[Precocious puberty secondary to an intramedullary germinoma]. / Puberté précoce secondaire à un germinome intramédullaire.

BACKGROUND: hCG secreting tumors are responsible for 21% of precocious puberties in boys. Usual localizations are hepatic, cerebral, mediastinal and gonadic. CASE REPORT: A 4-year-old boy developed precocious puberty with rapid evolution. Serum beta hCG suggested germinal etiology, but radiological procedures failed to find any usual localization. Further occurrence of pain in the legs led to carry out a lumbar puncture. The high cerebrospinal fluid/blood gradient of beta hCG suggested the presence of an intramedullar tumor. Medullar magnetic resonance imaging found a large tumor facing L1 and L2. CONCLUSION: To our knowledge, this localization is described for only the second time.

[A fatal respiratory form of type C Niemann-Pick disease]. / Forme respiratoire mortelle de maladie de Niemann-Pick type C.

An unusual case of Niemann-Pick disease type C is reported. The disease was first manifested in utero with hepatomegaly and ascitis. At the age of 3 months, respiratory signs were noted due to diffuse alveolar and interstitial pneumonia. Both bronchoalveolar lavage and blood serologic studies revealed respiratory infection by respiratory syncitial virus and Chlamydia trachomatis. These concomitant infections delayed the diagnosis of Niemann-Pick disease which was finally made by the electronic microscopic studies of liver biopsy and bone marrow specimens. Type C was identified by biochemical characterization of lipid accumulation in hepatocytes and by lipid enzyme profiles obtained from cutaneous fibroblast cultures. The child died at the age of 6 months from respiratory failure. Post mortem examination of the lung showed the presence of numerous overloaded alveolar macrophages in the alveolar spaces and walls. The severity of the lung issue disease is unusual in type C Niemann-Pick disease, in which neurologic involvement is usually the main prognosis factor.

[Meningo-encephalomyelitis in Lyme disease]. / Méningo-encéphalomyélite de la maladie de Lyme.

A case of isolated central nervous system involvement in Lyme disease is described. A 13 year-old boy developed progressive spastic quadraparesis, chronic lymphocytic meningitis with a low CSF glucose concentration and demyelinating lesions of the white matter on MRI. The diagnosis was proved serologically by high antibody titers against Borrelia burgdorferi (BB) in the serum (1:5, 120) and CSF (1:1,280). There was evidence of specific intrathecal immune response against the BB antigen. The patient was treated with penicillin G and then ceftriaxone. The CSF abnormalities quickly improved but improvement of the neurologic symptoms was gradual and to date still incomplete.

[Fusobacterium necrophorum septicemia in an infant]. / Septicémie à Fusobacterium necrophorum chez un nourrisson.

In a 9 month-old infant admitted to hospital for a fever with chilles, anaerobic blood cultures isolated Fusobacterium necrophorum. On the 5th day of intravenous treatment with amoxicillin and metronidazole clinical signs of mastoiditis, the likely source of the sepsis, became apparent. Septicemias with Fusobacterium necrophorum are usually observed in teenagers and young adults during an acute bout of tonsilitis. This type of infection is exceptional in infants and requires a careful search for a primary focus in facial cavities and in the base of the skull.

[Pulmonary and cerebral aspergillosis in Burkitt's lymphoma]. / Aspergillose pulmonaire et cérébrale au cours d'un lymphome de Burkitt.

Multiple intracerebral aspergillus abscesses in a 5 year old boy with a Burkitt's lymphoma are described. The disease was fatal despite antifungal treatment. The diagnostic and therapeutic problems, the risk factors and preventive care are discussed.

[Thrombosis and antiprothrombinase in children. Apropos of 2 cases]. / Thrombose et antiprothrombinase chez l'enfant. A propos de deux observations.

Two cases of arterial and venous thrombosis associated with lupus anticoagulant are reported. The first case was observed in the context of a systemic lupus erythematosus. In the second case, no underlying disease was found. From these 2 cases and a review of the literature, the particularities of this association in children is discussed.

[Treatment of asthma crises in children with nebulized salbutamol]. / Traitement de la crise d'asthme de l'enfant par nébulisations de salbutamol.

Thirty-two children were treated with nebulized salbutamol for acute asthma. Seventy-five per cent of the treatments were efficient, either after a first nebulization at 0.15 mg/kg (47% = group I), or after a second nebulization 45 min later, at 0.05 mg/kg (27% = group II). Twenty-five per cent of the treatments (group III) were inefficient or only partly efficient. The clinical tolerance was good except in two children. Group I and II presented differences only for the auscultation score. Children from group I and II were older and had less severe asthma than those from group III. On the basis of this study, nebulized salbutamol appears to be an affective and safe treatment for acute asthma. The repeated administration of low doses, shortly after the first nebulization increases the quality of the response.

[Pneumoperitoneum in a newborn infant after assisted ventilation]. / Pneumopéritoine chez le nouveau-né ventilé.

The authors report a case of massive pneumoperitoneum in a newborn who underwent assisted-ventilation for a severe aspiration of meconial amniotic fluid. Clinical and roentgengraphic findings that are helpful in differentiating a pneumoperitoneum of gastrointestinal origin from an extrathoracic air leak are reviewed. In such a situation, laparotomy should be avoided.

[Somatostatin in a case of neonatal hyperinsulinism caused by focal adenomatosis of the head of pancreas]. / La somatostatine dans un cas d'hyperinsulinisme néonatal par adénomatose focale de la tête du pancréas.

The use of somatostatin in the treatment of a newborn infant with hyperinsulinism is reported. When administered alone, somatostatin was only able to prevent hypoglycemia for a short period of time, whereas the addition of a constant infusion of glucagon allowed successful control of the patient's hypoglycemia. As hyperinsulinism relapsed after a subtotal pancreatectomy, a trial was carried out with a somatostatin analog, which has an expected longer duration of action. It led to a significant rise in the blood glucose level but failed to prevent safely hypoglycemia even when 4 injections were performed daily.

[Acute chorea, systemic lupus erythematosus and antiphospholipid antibodies. Apropos of a case]. / Chorée aiguë, lupus érythémateux disséminé et anticorps antiphospholipides. A propos d'une observation.

A case of acute chorea in a 10 years old girl complicating a systemic lupus erythematosus associated with antiphospholipid antibodies is reported. The lupus anticoagulant was detected with a coagulation assay and the false serological reaction for syphilis by the RPR test. The child recovered with Prednisone therapy. The place of chorea in the context of neurological complications of SLE and the particularity of its association with anti-phospholipid antibodies are discussed.

[Severe hyperthermia syndrome in the infant. Apropos of 11 cases]. / Syndrome d'hyperthermie majeure du nourrisson. A propos de 11 observations.

The authors report a retrospective study of 11 cases of malignant hyperthermia. The mean age of the patients was 5 months and 3 weeks. Clinical features included severe hyperthermia (greater than 41 degrees C), seizures, coma, collapse, rhabdomyolysis, acute renal failure and functional renal failure. Three infants died. Four patients presented neurological damages. Four recovered fully. The authors discuss the difficulties of diagnosis, the nosological position and the pathophysiology of this syndrome.

[Intracranial arachnoid cysts in children. Apropos of 8 cases]. / Les kystes arachnoidiens intracrâniens de l'enfant. A propos de 8 observations.

Eight cases of intracranial arachnoid cysts are reported. They are benign congenital lesions who result from an abnormal development of the earliest arachnoid. A subsequent development is possible. The macrocephaly is the first sign and other symptoms give evidence to the localization. Thanks to the C.T. scan one can make the diagnosis, appraise the repercussion and put the therapeutic indications. The surgical treatment is reserved to symptomatic forms. The peritoneal shunt takes the ablation's place. This method is easier and less liable to complications. The results depend on the localization, the pattern of operation and the precocity of the diagnosis.