[The pediatricians and the French system of protection against child abuse since the law n. 89-487 of 10 July 1989].
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Biomedical subjects
Publications and source records attributed to M Bost.
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Dilated cardiomyopathies occur mostly in infants and affect their vital and functional prognosis. They may be primary or secondary to various pathologies such as congenital cardiac malformation, toxic myocardial drugs or mucoviscidosis. Medical treatment may only be transiently effective and brutal or progressive aggravation may lead to cardiac transplantation being considered in those cardiomyopathies which are primary (without metabolic causes) or chronic secondary. Echocardiographic survey is the most efficient way to follow the evolution and to detect complications (cardiac thrombosis).
Pharmacokinetic studies of 13 children treated with cyclosporine A (CyA) were retrospectively analysed (9 renal transplants, 1 combined liver-kidney transplant, 1 heart transplant; 2 were treated for a nephrotic syndrome). The patients were separated into 2 groups: patients 0-15 years (group 1), patients 15-18 years (group 2). In comparison with adults (group 3), children of the 2 groups required higher CyA doses, related either to body weight or to body surface area. CyA dosage was performed by high performance liquid chromatography on whole blood samples. Despite higher doses, trough CyA levels were lower in groups 1 and 2 compared with group 3. Nephrotoxicity occurred in the only 2 children treated with CyA doses higher than 10 mg/kg/d.
In 2 boys, aged 3 and 13 years, suffering from severe growth failure, endocrine evaluation showed growth hormone deficiency and central hypothyroidism without diabetes insipidus. Magnetic resonance imaging demonstrated a transection of the pituitary stalk, and the presence of an ectopic neurohypophyseal nodule.
In France the epidemiology of child abuse is badly known, because of the lack of connexion between different institutions. We present a child abuse observatory set up in Grenoble in May 1987. The social, educational, judicial and medical department's services are working together. In the first 20 months, 87 cases were recorded: 57 physical abuse, 26 sexual abuse and 5 cases of abuses due to negligence. Precise information was collected concerning the victims, their siblings, the family's risk factor and the offenders, the method by which the information was obtained and the prosecution undertaken. A 10 July 1989 law enforced each Department's governor to set up a service for collecting information about child abuse. Our observatory will serve as a model for this law application.
In 19 children with familial hypercholesterolemia type IIa measures to reduce the amount of saturated fat and cholesterol in the diet lead to a mean decrease of cholesterolemia of 15%, thus confirming the favorable effect of the diet previously reported in the literature. However, there were large variations in the efficacy of the diet from no effect up to a 30% decrease. "Non-responders", in whom the diet was not effective, require treatment with drugs. The diet must be individualised and should involve the whole family. Children must be followed in order to encourage compliance to the dietetic measures and to detect eventual side effects. Furthermore, the dietetic measures must be associated with steps to control the other risk factors of arteriosclerosis.
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.
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.
We report on a case of congenital stomatocytosis in a French boy presenting with a haemolytic anaemia requiring splenectomy at the age of 6. The red cells included 15-20% stomatocytes and displayed a marked increase of volume. Their osmotic resistance and density were reduced; however, their deformability was unaltered in isotonicity. Erythrocyte Na+ was high (27 mEq/l) and K+ low (65 mEq/l). The newly described (K+, Cl-)-cotransporter normally triggered by hypo-osmotic stress, was activated to maximal capacity. Membrane band 7 was reduced by 72%. From anamnestic data, the condition appears to have been transmitted by the father. The mother proved to be strictly normal on clinical, morphological, osmotic and biochemical bases. We suggest that the partly missing band 7 may play an important role in the genesis of stomatocytosis.
The pulmonary artery sling is a congenital malformation where the left pulmonary artery forms a vascular sling that bends around the right border of the lower trachea. Its clinical manifestations are signs of tracheal compression in the first months of life. The diagnosis of this rare anomaly is suspected on radiological and echocardiographic grounds and confirmed by oesophagography, tracheoscopy and angiocardiography. Diagnostic problems may be encountered when the condition is associated with tracheobronchial (50% of the cases) or cardiovascular malformations. In the case reported here respiratory symptoms and heart failure were present in a 1-month hypotrophic infant who also had ventricular septal defect and dextrocardia due to right lung hypoplasia. The pulmonary artery sling was diagnosed by angiography. The severity of the clinical signs precluded all attempts at surgical repair of the cardiovascular anomalies. The child died at the age of 2 1/2 months.
The authors list the principal normolipidemic drugs. Only cholestyramine (Questran) and some fibrates (fenofibrate: Lipanthyl for instance) are registered for pediatrics. Cholestyramine acts by sequestering biliary acids, and thereby inhibiting intestinal cholesterol resorption. It is the primary drug used for children, when diet has been unable to restore normal cholesterol values.
Nailfold capillaries may be visualized in vivo using a simple non invasive microscopic technic. The assessment of capillary flow disturbances and organic microangiopathies is therefore rendered possible through capillary microscopy. Three kinds of clinically significant morphological abnormalities can be easily recognized: megacapillaries are huge capillaries with a diameter greater than 50 microns. They are mainly found in progressive systemic sclerosis and dermatomyositis, together with avascular areas and pericapillary edema; ramified capillaries are neovessels, most frequently related to subclinical cutaneous vasculitis; capillary hemorrhages may be induced either by active microangiopathies, or by deep vasomotor changes. The semiological consistency of these abnormalities makes capillary microscopy a valuable procedure for the early recognition of connective tissue diseases and the evaluation of vasomotor disturbancies in children as well as in adults. Further investigation of other microangiopathies like purpura rheumatica and diabetes are needed.
A psycho-social survey by questionnaire was conducted among parents accompanying their children to the Pediatric Emergency Ward of the Medical Center of Grenoble, in order to identify the motivations of those who do not seek a medical advice prior to their coming to the hospital. From this survey, several motivating factors were identified: an economical factor, the difficulty to rapidly call a physician, the place of residence, an insufficient knowledge of the medical services, the familiarity of parents with the hospital, the advice of relatives, the anxiety caused by the child's illness and the comforting image of the hospital. In a synthetic analysis, several types of parents are distinguished according to their behavior.
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.
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.
In the Grenoble Medical Centre, pediatric emergency admissions have been drastically influenced by the opening of an Emergency Unit in May 1983. Our work was carried out one year after the opening in order to study the characteristics of the admitted children. The enquiry was done over 4 months, one month for each season of that year. A total of 1,382 children were included in the study. Thirty-four percent were seen for a medical advice (66% hospitalized). Children seen for medical advice are younger and mostly migrants. They live close to the hospital and are brought most often for fever. Fifty one per cent directly brought to the hospital by their parents for a first pediatric advice (49% were sent by their personal physician). Children directly brought by their parents are in majority infants and migrants. They are mostly admitted at night and morning and during the week-end. They are more frequently followed within the public mother-child health protection system ("Service de Protection Maternelle et Infantile", PMI).
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.
Two siblings with chronic adrenal insufficiency, diagnosed at 12 years of age, failed to show a spontaneous onset of puberty in spite of adequate adrenocortical therapy. Testosterone plasma levels were low, without concomitant increase in gonadotropin levels. This association strongly suggest a sex-linked cytomegalic adrenocortical hypoplasia.