[On a case of peripheral dysostosis].
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Biomedical subjects
Publications and source records attributed to M Bardare.
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Intradermal skin test antigen (Varidase, Mumps, Candidine) and contact sensitization with 2,4-dinitrochlorobenzene (DNCB) were employed in 35 previously untreated children with neuroblastoma and malignant lymphoma, to evaluate delayed cutaneous hypersensitivity. Cutaneous responses were correlated to stage of disease and survival. The limited number of patients prevents any definitive conclusion. It is apparent, however, that in childhood non-Hodgkin's lymphoma anergy to DNCB is correlated to advanced stages with rapid progressive disease. In neuroblastoma and Hodgkin's disease patients skin test sensitization seems to be of questionable clinical value.
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An immunological imbalance is probably one of the major pathogenetic causes of rheumatoid arthritis in adults as well as in children. This aspect is the rationale for the use of immuno-modulating drugs. In our study we evaluated the effects of intravenously-administered thymopentin on systemic and local features in 10 children affected with systemic onset juvenile chronic arthritis (JCA). We also considered the effects of intra-articular thymopentin in 3 children affected with pauciarticular onset JCA.
Calciotropic hormone assays and bone mineral density (BMD) evaluations were carried out on a group of 36 children affected with juvenile chronic arthritis (JCA) (13 with pauciarticular, 8 with polyarticular and 15 with systemic onset subtype) and in a matched control group. The plasma levels of calcium, phosphate and alkaline phosphatase were normal in all the groups. Basal 25-OH D plasma levels were significantly decreased both in the polyarticular and in the systemic groups; the latter showed a further decrease after 1 year. There was a significant decrease in both the basal and the 1-year plasma levels of 1-25-OH D only in the systemic group. The BMD was decreased in the systemic group alone and the percentage increase after 1 year was significantly inferior to that in the other groups and in the controls. There was a clear relationship between the decrease in hormones and the cumulative dose of steroids administered, as well as between the BMD decrease and the physical activity of the patients (according to Steinbrocker's classification).
The main clinical features as well as the most important laboratory test in systemic lupus erythematosus (SLE) are reviewed. The peculiar aspects both in clinical presentation and in natural history of this disease in childhood are stressed. Personal experience is reported: 32 cases, 8 males and 24 females, mean age of onset 10.9 + 2.1 yrs, are evaluated. The most frequent clinical symptoms at diagnosis were fever, skin involvement and joint involvement, while anemia, nephropathy and hepatosplenomegaly were frequently present at onset. ANA were detected in all the subjects, anti dsDNA in 84% of cases; in only one patient SS-A/SS-B assayed positive. C4 was decreased in 17/32 cases at onset, in the others during the course of disease. Three patients died, 2 for infections, 1 for a non-Hodgkin lymphoma. Two cases present a chronic renal failure (1 is dialyzed).
Classification criteria and pathogenetic mechanisms of vasculitis in childhood are discussed. The paper is focused on the role played by immunocomplexes, cell-mediated immunity and granulomatous reactions in vascular injury.
The outcome of lupus nephritis was studied in 12 children. Their age at onset was 5-13 years (mean 9.8 +/- 2.1 years), less than that reported in recent years of children with lupus nephritis. All patients had clinical symptoms and signs and/or laboratory findings of renal involvement. Renal biopsy, performed in all patients, showed: minimal change in 3 patients, mesangial glomerulonephritis in 5, focal proliferative glomerulonephritis in 3 and membranous glomerulonephritis in one patient. There was no correlation between age at onset and histological categories, while clinical data were well correlated with the degree of histological involvement. 11 patients were followed for 5.16 +/- 3.2 years (range 1-13 years); 7 patients are in complete remission, 3 patients have only microscopic hematuria and/or mild proteinuria, one patient died because of infection complicating pulmonary involvement. Our report confirms a more favorable evolution of lupus nephritis in children than in adults. In particular none of our patients whose disease started before the age of 10 years had poor outcome, in contrast to previous report describing a higher mortality and morbidity in younger children. The more favorable prognosis of our patients could depend not only on the absence of the more severe form of lupus nephritis that is diffuse proliferative glomerulonephritis, but also on the earlier diagnosis and subsequent therapy.
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Here is referred the case history of a boy - age 6 yrs - who was hospitalised for a persistent high temperature with generalised limphonodes hypertrophy. The limphonode biopsy together with decreased NK activity and increased antiEAD and antiVCA antibodies allowed the diagnosis of chronic mononucleosis.
Twenty-one children, age range 5 mo - 14 yrs, affected by candidosis, were treated with ketoconazole (tablets or suspension). Patients had alimentary tract involvement (12), oesophagitis (1), urinary tract candidosis (3), vaginitis (2), septicaemia (1), endophtalmitis (1) and chronic pulmonary illness with persistence of Candida albicans in sputum (3). Daily drug doses ranged from 3 to 13 mg/Kg and treatment period from 7 days to 14 months. Pharmacokinetic study in 15 children showed large individual variability of drug serum levels. Pharmacokinetic parametres, related to different schedules of the two ketoconazole formulations (tablets and suspension) are reported; drug levels after chronic administration are also evaluated. A daily dose of 3 mg/Kg of ketoconazole suspension, given in 3 administrations, did not result in sufficiently high levels, which indeed were obtained with a daily dose of 10 mg/Kg (3.3 X 3). The effect of treatment is proven by negativization of cultures in 90% of patients, by disappearance of clinical signs in 67% by improvement in 9%. The therapeutic effect on the remaining 24% (5 patients) is not evaluable. Adverse effects were only nausea and pyrosis in four cases; no laboratory abnormalities were found. A daily dosage from 7 to 10 mg/Kg, in two or three administrations, is suggested to obtain therapeutic levels in children.
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