[Adolescents and health care].
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Biomedical subjects
Publications and source records attributed to V Courtecuisse.
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A 3-year experiment in adolescent hospitalization in a department of pediatrics is described. Details are given concerning patient referral to this specialized sub-unit and staff behavior.
Of 33 children with membranous nephropathy screened for HBs Ag, 14 were found to be HBs Ag carriers, whereas HBs Ag was detected in 3 of 170 and 4 of 100 children with glomerular and nonglomerular kidney diseases, respectively. HBs Ag was often associated with acute hepatitis at onset (five patients) or with elevated transminases values. This high incidence and the prevalence of an unusual subtype (ayw2) suggest a relationship between HBs Ag and the glomerular lesions. Using immunofluorescence, however, HBs Ag could not be detected within the deposits, so that the nature of the relationship cannot be considered as established. The clinical outcome (50% remission), the plasma complement component disturbances, and findings by immunofluorescence did not differ from those observed in children with MGN without detectable HBs Ag.
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A study of 72 children, aged between 6 and 24 months who came from socio-economic background has shown: plasma ferritin levels decrease gradually between 3 weeks and 6 months of age. Between 6 months and two years, levels reach a plateau but the values are lower than those of adults. The mean ferritin level is lower at any given age in children with iron deficiency but plasma ferritin levels may be in the normal range although there is obvious iron deficiency. By contrast the saturation of siderophillin can be used to determine inadequate supplies of iron to the marrow. The frequency of iron dificiency in infants under 1 year was similar in the two socio-economic groups. However between 1 and 2 years no iron deficiency was detected in the children under regular supervision but all the infants who were socially disadvantaged became iron-deficient demonstrating the need for regular iron supplements.
Three siblings, respectively 20, 16 and 12 years old, presented with hypocalcemic vitamin D resistant rickets (Prader's type). Their clinical history included several periods of spontaneous cessation of therapy, with severe relapses. Since 1973, treatment was strictly observed, allowing to test the therapeutic effects of 25 OH D3, 1-25 (OH) 2 D3 and 1-alpha (OH) D3. The clinical effects are reported as well as biochemical data. Among them, an inactive form of hyperparathyroidism is emphasized, which may resemble some cases of pseudohypoparathyroidism. Simultaneous resistance to exogenous PTE was also demonstraded.
Three children with congenital heart disease presented with more or less severe renal dysfunction. Histologic studies showed signs of membranoproliferative pseudoglomerulonephritis without deposits on immunofluorescence. Surgical correction of the heart defect induced a spectacular functional and histologic improvement.
A 18 year old patient presented with a female phenotype and no signs of sexual maturation. The leukocyte karyotype was XY. At laparotomy, there was no uterus and the gonadal streaks had a XY/XO mosaicism. There were rudiments of fallopian tubes and epididymis. Histologic examination of the gonads showed foci of Leydig and Sertoli cells.
Two cases of delayed unilateral lymphedema are reported in children presenting with Turner's syndrome. On this occasion, the frequency of lymphedema in Turner's syndrome and the more general problem of the lymphatic changes in this syndrome are discussed. The importance of isotopic lymphography is emphasized, because of its simplicity, its harmlessness and the good quality of the morphological and cinetic data obtained.
This investigation confirms that 1alpha-hydroxyvitamin D3 (1alpha-OHD3) is a potent drug for the treatment of patients with pseudo-deficiency rickets (Balsan et al., 1975a; Reade et al., 1975; Prader et al., 1976). 1alpha-OHD3 corrects their intestinal malabsorption of calcium and phosphorus, normalizes their serum calcium and phosphate concentrations and promotes healing of skeletal lesions. This study also shows differences in the needs for 1alpha-OHD3 of children with PDR. Three factors appear to be of importance: familial sensitivity, severity of chronic secondary hyperparathyroidism, and periods of increased growth velocity. Tolerance to long-term 1alpha-OHD3 therapy, at doses varying from 0.5 to 2 microgram/d is excellent. Surveillance of patients should include regular measurements of 24 h urinary excretion of calcium, since hypercalciuria is the first signal of overdosage.
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Five children from 3 different families, presented several attacks of hemolytic-uremic syndrome with peculiar features:--in 4/5 children the disease was familial; 4 presented 2 attacks, the 5th 3 attacks, separated by several months or years of time-interval;--the "bouts" were constantly marked by the initial high level of reticulocytes, rapid recovery of hematuria, renal failure and thrombocytopenia;--overall, repeated biopsies of the kidney never showed any lesion of the basement membrane of the glomerular capillaries or of the arteriolar walls leading to the diagnosis of thrombotic microangiopathy;--all etiological enquiries to precise the cause of chronic hemolysis were negative.
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