[Trial of androgen therapy in the treatment of acute non-lymphoblastic leukemia in children. Initial results].
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Biomedical subjects
Publications and source records attributed to M Bost.
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Thirty three patients with Henoch-Schoenlein purpura were studied at various developmental stages of this disease: specially platelet counts and factors XIII and VIII. During the development phases: 40,6% of the patients have a slight but regressive thrombocytosis (greater than 400 G/l); and 75% a reduced factor XIII, well correlated with the severity of the clinical status (level as low as 60% can be considered as a "gravity threshold"), and corrected during the improvement of the disease. This reduced factor XIII is probably linked to the local inflammation in the vessels. Factor VIII studies (specially VIII A: Ag) were normal.
Lipoprotein-lipase deficiency is an uncommon disease, inherited as an autosomal recessive pattern. The authors report two cases: the first one is a fourteen years old girl. It is revealed by abdominal pain; the diagnosis is detected by a milky plasma and confirmed by the enzyme activity which is dramatically decreased; the other one is a seven year old boy, who shows several pancreatitis but in whom the enzyme activity is not so low, perhaps because of genotypical difference.
We report one case of Digoxin intoxication in a child treated with Fab Fragments of Digoxin-Specific antibodies (Fabad), although there was no evidence of early life threatening complications. The efficacy of this treatment, which prevents further complications as well as its safety, represent strong arguments to treat children at the early stage of the intoxication in order to avoid temporary cardiac pacing.
A case of acute hemorrhagic oedema of the skin is reported. About it we discuss: Anatomo-clinical data, similar to immune complex type III hypersensitivity vasculitis. Antigen has never been defined. In our case adenovirus can be discussed. Evolution is usually benign, but lethal intestinal complications have been described. Nosologic relation with anaphylactoid purpura.
A child with buphthalmos and glaucoma, facial hemihypertrophy and plexiform neuroma of eyelid secondary to neurofibromatosis is described. Diagnostic, pathogeny, treatment are discussed.
McCune Albright Syndrome is characterized by the triad of polyostostic fibrous dysplasia of bones, skin pigmentation (café au lait-spots) and sexual precocity. The authors report a case of a 7 years girl with this syndrome in whom the sexual precocity is due to an autonomous hyperfunctioning of luteinized follicular cysts of ovary. The pathogenic mechanisms of this autonomy are debate.
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.
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