Intravenous immunoglobulins in thrombocytopenia with absent radii.
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Biomedical subjects
Publications and source records attributed to A Stabile.
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Since immunological disorders have been demonstrated in patients with Diamond-Blackfan anaemia (DBA), intravenous immunoglobulins (IVIG) were administered to a 14-year-old girl with DBA and congenital malformations, previously treated with corticosteroids and blood transfusions. No therapeutic effect was observed.
In the present work the Authors report one case of Wilson's disease showing peculiar characteristics diagnosed in pediatric-age patients. In this case, a deep electrophysiological visual analysis was carried out six years after diagnosis. The evoked visual potential alterations might be connected to a retina dysfunction. Further studies should be carried out in order to support such hypothesis.
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We report our prospective experience with sensitivity, specificity, predictive values and efficiency of echocardiography in diagnosing AD involving the ascending aorta (type A). We studied two groups of patients with both echocardiography and aortography. Group 1 was made up by 46 consecutive patients with clinical suspicion of AD. Type A AD was confirmed in 23 cases. Group 2 was comprised of 509 adult patients who had been studied during the same period with both aortography and echocardiography (including 46 patients from group 1); prevalence of type A AD in this group was 4.9 percent. We conclude that the diagnostic usefulness of echocardiography in patients with suspected type A AD is limited by its moderate sensitivity and predictive positive value. Aortography remains the major step in diagnosis. Within these limitations, echocardiography is useful in confirmation of clinical suspicion if all three main echocardiographic signs are present (predictive positive value: 100 percent).
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The incidence of sepsis, mortality due to sepsis, total mortality, and minor infections was evaluated in a group of 46 premature newborn infants who were treated with intravenous immunoglobulins. They were compared with an untreated control group. No significant differences were observed between the two groups.
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The second child of a mother with idiopathic thrombocytopenic purpura was given 400 mg/kg per day polyethylenglycol-treated gammaglobulins during the first 5 days of life. Thrombocytes increased from 15 X 10(9)/l to 200 X 10(9)/l within 10 days and remained at this level afterwards.
A rare case of varicella with bilateral acute arthritis of the knee is described. Clinical features and laboratory results of the patient are briefly compared with those of other sixteen cases previously published. The study of immune response shows a decrease of helper T cells (OKT4+) and an increase of suppressor/cytotoxic T cells (OKT8+) with a normal response of lymphocytes to PHA.
We evaluated sensitivity, specificity and predictive values of echocardiography in detecting aortic dissection. We studied in the same period of time two groups of consecutive patients with good quality echocardiographic examination. Group I, with high prevalence of the disease (76%), was composed of 25 patients; 19 patients with aortic dissection (11 of the type A and 8 of the type B) and 6 patients with clinical and echocardiographic suggestion of aortic dissection which was not confirmed by angiography. Group II, with lower prevalence of the disease (4%), was composed of 382 adult patients who underwent aortic angiography for different reasons. In this second group there were 16 out of the 19 patients of the first group, with aortic dissection, who underwent aortic angiography and the 6 patients with suspicion of aortic dissection which was not confirmed by angiography. Type A dissection: The finding of intimal flap on echocardiography was highly specific (98%) but relatively insensitive (45%); its positive predictive value was low (50% in the first and 34% in the second group). The specificity of increased thickness of aortic wall was lower (89%) and the sensitivity higher (81%); its positive predictive value was satisfactory in the first group (81%) and very low in the second (13%). Aortic root dilatation was fairly specific (76%); the sensitivity of this finding was high (87%) but its positive predictive value was still low (66% in the first and 7% in the second group). To conclude: the positive predictive value of the 3 echocardiographic signs of aortic dissection varied, in relation to the different prevalence of the disease, from 50, 81 and 66% in the first group to 34, 13 and 7% in the second group; the diagnostic utility of the echocardiographic examination appeared limited, in these patients, by the low values of sensitivity and positive predictive values; aortography is still the most valuable technique in the diagnosis of aortic dissection; echocardiography was useful in the early evaluation of some emergency cases (chest pain, shock, collapse...) suggesting sometimes the correct diagnostic hypothesis of aortic dissection; in the presence of a typical clinical picture, the contemporary presence of the 3 echocardiographic signs, though having the lowest sensitivity (36%), was highly predictive of the type A dissection. Type B dissection: In these patients the clinical picture, in contrast with type A dissection, Type A, was not indicating careful and complete echocardiographic aortic scan. Then the echocardiographic examination was even more disappointing: sensitivity 25%.
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