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Biomedical subjects

E Agosti

Publications and source records attributed to E Agosti.

8 recordsLinked to original sources

Treatment of sphingomyelinase deficiency by repeated implantations of amniotic epithelial cells.

Five young patients with Niemann-Pick disease type B were treated with repeated implantations of amniotic epithelial cells, as a source of exogenous sphingomyelinase. This treatment abolished the recurrent infections, mainly of the respiratory tract, and led to other improvements of the general conditions of the patients. In particular, we noticed a disappearance of vomiting, a recovery from muscular hypotrophy, and significantly reduced pulmonary distress. In four subjects, who were in a prepuberal state, there was a puberal spurt with a concomitant burst of growth. In two cases, characterized by a greater than normal content of sphingomyelin in urinary sediments, a single implantation caused a sustained normalization of sphingomyelin and total phospholipids in the urine. Finally, sphingomyelinase activity of peripheral leukocytes, when assayed 0.5 to 4 months after some of the implantations, showed a rise to heterozygous values in 30-40% of the assays.

Adolescent

[Transfer factor].

Explore the source record for details and available documents.

Chromatography, Gel

[Comparison of high-dose immunoglobulin and cyclosporine in newly diagnosed diabetic children].

A controlled trial was carried out on type I diabetic children to evaluate and to compare the clinical effects of two different kinds of immunotherapy: high doses intravenous gammaglobulin (IVIgG) and cyclosporine A (CyA). 30 newly diagnosed patients were admitted to the trial, 10 of whom served as controls (group A), 10 received 400 mg/kg b.w. of IVIgG on 5 consecutive alternate days and subsequently after 15 days and monthly thereafter for up to six months (group B), 10 patients received CyA 5-10/kg b.w. by mouth in two daily doses for a period comprised between 6 and 18 months (group C). Serum post-prandial C-peptide level was significantly higher after 6 months in group B and C than in group A; after 12 months, only group C showed significantly higher values. This difference was no longer significative at 18 and 24 months. Insulin requirement in the treated groups was significantly lower than in control group at 6 months, this difference was no longer significative at 12 months. We didn't find any difference concerning insulin requirement during the study comparing the two groups treated with the two different immunosuppressive therapies. In 3 patients in group B and in 3 patients in group C we didn't observe any appreciable response to immunosuppressive therapy (defined as insulin requirement greater than 0.5 UI/kg b.w. at 6 months and/or greater than 0.8 UI/kg b.w. at 12 months). We couldn't find any significant difference between responders and not responders to the immunosuppressive therapies regarding age, symptoms lasting before the diagnosis, weight loss, ketoacidosis intensity and serum post-prandial C-peptide level at the onset.(ABSTRACT TRUNCATED AT 250 WORDS)

Adolescent

Intestinal permeability, atopic eczema and oral disodium cromoglycate.

A dual sugar (lactulose-mannitol) absorption test was performed in 19 patients with atopic eczema before and after a 21 day elimination-diet. Moreover L/M test was carried out in 20 controls. The mean value of lactulose-mannitol urinary ratio (L/M) was 0.015 (+/- 0.018 SD) in the group of patients and 0.012 (+/- 0.011 SD) in the control group (p = 0.49). The mean clinical score improved significantly after elimination diet (41,6 +/- 12.9 SD before the diet, 21.7 +/- 10.4 SD after the diet, p less than 0.001) but no significant modification of intestinal permeability was recorded (L/M = 0.015 +/- 0.018 SD before the diet and 0.21 +/- 0.022 SD after the diet, p = 0.38). Using a double blind approach we were not able to demonstrate any significant effect of disodium cromoglycate on the clinical score and intestinal permeability. The connections between food allergy, intestinal permeability and atopic dermatitis have not been understood, but disodium cromoglycate doesn't seem to play a significant role in the treatment of atopic dermatitis nor in the modification of intestinal permeability.

Administration, Oral

[Bone marrow transplantation in pediatrics: its current status, the problems and prospects].

Notwithstanding the high proportion that achieve a cure after chemotherapy, there are still a case in which only a BMT can offer a chance of cure. This minority of patients can undergo an allogeneic BMT if a HLA matched donor is available or an autologous BMT if a good remission is achieved before the BMT. Not all the patients comply with these criteria. Therefore we need to widen the availability of the donors searching for unrelated matched donors or facing the problems of an aplohidendical BMT. The efforts to treat even children with advanced disease are based on the possibility of overcoming the blasts resistance or of stimulating the non-HLA restricted cytotoxicity with IL2.

Bone Marrow Transplantation