Ceftriaxone-associated nephrolithiasis.
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Biomedical subjects
Publications and source records attributed to D Floret.
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Four children with persistent neuroblastoma after marrow ablative chemoradiotherapy and autologous bone marrow transplantation received continuous infusion of recombinant interleukin 2, 75 to 120 days after the graft. Recombinant interleukin 2 therapy did not induce any major or nonreversible toxicity, hematological toxicity in particular. One patient entered complete remission for 9 months and a second patient had a long-lasting normalization of urinary catecholamine metabolites with more than 50% regression of bone marrow metastases (8 months). In three children, recombinant interleukin 2 and a second patient entered complete remission for 9 months therapy was followed by major increase and activation of circulating natural killer cells which amounted to 80% of the circulating mononuclear cells.
After having been widely used in biological research, nucleic probes are now becoming available for the purposes of diagnosis in the clinical microbiology laboratories. Due to various technical improvements, an increasing number of kits will be provided and will allow a rapid and specific diagnosis to be carried out for bacterial agents (ie diarrhea, respiratory infections, sexually transmitted diseases...).
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In France, Caillavet's law sets the conditions required for autopsy and organ removal. Despite the liberal conditions of this law, the autopsy rate is becoming rare. Other obstacles exist, in particular, religious beliefs in the case of islamic and jewish people. The final decision rests with the family although this decision may be influenced by the physician, if the family can be convinced of the importance of such an examination.
Six children with Bartter's syndrome aged 6 years 4 months to 13 years 11 months were treated with indomethacin (1.7 to 4.3 mg/kg/day) during 7 to 27 months. A catch up growth was first observed, then growth curve was parallel to the normal. A catch up weight was also observed. The osseous maturation was the faster it was more delayed. These changes were observed despite a partial correction of potassium and plasma renine activity.
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A case of hypomelanosis of Ito in a 22-month-old North African girl is reported. The family history is negative for skin disease. Whorls of depigmentation are present on the trunk and abdomen. Associated defects include: growth retardation, microcephaly, strabismus, bilateral epicanthus, myopia, hypertelorism and abnormalities of external ear. Associated non-cutaneous abnormalities documented in patients with hypomelanosis of Ito are reviewed.
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153 endoscopical measures of the length of the intra-mural ureter in 81 children over 2 years have enabled us to ascertain that this segment of vesical ureter is significantly shorter in most patients with vesico-ureteral reflux. By contrast with other previous studies, we have not been able to use this examination for establishing the prognosis of the reflux, perhaps by lack of sufficiently prolonged follow-up However these data have been helpful to us in two circumstances and were complementary of the determination of aspect and position of ureteral meatus: a - When the child is operated for au unilateral reflux, a controlateral short intra-mural ureter suggests the value of a bilateral reimplantation to avoid the appearance of a controlateral reflux. b - When the child has an urinary infection without demonstrated reflux, a short intra-mural ureter suggests the value of a repeated cystography.
A 4 year old girl with mild mental retardation presented with convulsions, coma and hepatomegaly. She died rapidly. The main biochemical findings were hypoglycaemia, metabolic acidosis, generalised aminoaciduria, elevation of the plasma and urine alpha-amino adipic acid, massive urine excretion of glutaric and glutaconic acids with traces of alpha-hydroxyglutaric acid. The diagnosis of glutaric aciduria was confirmed by the low activity of glutaryl CoA dehydrogenase in liver tissue. This diagnosis should be considered in children with progressive neurological disorders (dystonia, choreoathetosis) and in children with an illness similar to Reye's syndrome.
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The case report concerns a 2 years old girl with hypoproteinemia associated with gastritis. The child recovered completely 8 weeks later. Cytomegalovirus was excreted in urine. The authors suggest that such a clinical picture should not be called Menetrier's disease. The role of cytomegalovirus is discussed.
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A 3 year old portuguese boy with the Sturge-Weber syndrome also had oculocutaneous melanosis. The cutaneous melanosis extended to more than 50 p. 100 of the body surface. Hands, feet and the face were spared. The abnormal pigmentation of the eyes involved the sclerae bilaterally. Clinically, the cutaneous hypermelanoses most likely represented a widespread aberrant mongolian spot. Histological study of the hyperpigmented skin revealed dermal melanocytes in the superficial and mid-dermis. Ultrastructural study showed that most of the dermal melanocytes were loaded with mature melanosomes. All development stages of melanosomes were observed in some of them. Average melanosomal size in the dermal melanocytes was slightly increased when compared to that of melanosomes in epidermal keratinocytes. From this case and from a review of the literature, the authors suggest that the association between the Sturge-Weber syndrome and disturbances of the melanin pigmentation of the skin and eye is probably not coincidental. An embryologic abnormality, affecting both the neural crest (from where melanoblasts originate) and the primary vascular plexus may explain such an association. Various disorders, including nevus of Ota, nevus Flammeus, the Sturge-Weber syndrome associated with ocular and cutaneous pigmentary abnormalities and the Sturge-Weber syndrome in the complete or incomplete forms, may represent the clinical picture of the same embryologic disturbance. The clinical picture might be related to the time at which it acts, the receptivity of the various structures and their level of differentiation.
The report is dealing with a 10 year-old girl. The diagnosis of Albright's type II osteodystrophy relied on clinical, radiological and biological symptoms: evocative dysmorphic syndrome and absence of disturbances in the phosphocalcic metabolism. Hypothyroidism was secondary to an isolated defect in TSH, and a TRH stimulation test did not induce an increase in plasma TSH.
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Five patients with Bartter's syndrome were investigated. Sodium restriction (less than 10 mEq/day for at least 5 days) showed a renal sodium wastage in only two patients (I and II) in spite of increased aldosterone secretion rate (from 151-427 to 680-842 mug/day). The effect of angiotensin II (A II) 80ng/kg/min for 30-180 min, on plasma renin activity (PRA), plasma aldosterone, and urinary sodium excretion was compared with the effect of a previous infusion of 5% dextrose given at the same rate, 0.5 ml/min for 1 hr. A II infusion resulted in increased plasma aldosterone levels: from 236-330 pg/ml to 800-881 pg/ml in 30 min. This increase was also observed in patient II (from 139 to 600 pg/ml). PRA was decreased by A II infusion (from 1,142-2,462 to 121-1,625 ng/liter/min). In patient IV, this decrease in PRA was also observed when he was on a salt-restricted diet (from 1,934 to 370 ng/liter/min); but the minimal PRA was still higher (370 ng/liter/min) than with a normal diet (121 ng/liter/min). In no case could normal PRA level be obtained. A II infusion induced an increase in urinary sodium excretion only in the two patients with renal sodium wastage (from 80-90 to 265-230 muEq/min in 30 min). Urinary sodium excretion decreased in the other patients from (37.5-213 to 4.30-46 muEq/min) and fractional sodium excretion was reduced in patient V (from 0.56% to 0.45% at 30 min and to 0.29% at 120 min). No significant change with A II infusion was observed in patient IV when he was on a sodium-restricted diet (from 1 to 2.5 muEq/min in 30 min). Urinary potassium excretion was similar to sodium excretion. No change was observed in plasma potassium and sodium.