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Biomedical subjects

F Albertini

Publications and source records attributed to F Albertini.

At least 19 recordsLinked to original sources

Bone marrow transplantation in adult thalassemia.

Early trials of allogeneic marrow transplantation for homozygous thalassemia were disappointing in patients older than 16, with four of six patients dying early of graft-versus-host disease-related complications, one patient dying at 9 months of infection due to graft failure, and one dying at 6 years of recurrent thalassemia. Three classes of risk could be identified in analyses of results of transplantation in younger patients using the criteria of degree of hepatomegaly, the presence or absence of portal fibrosis, and a history of adequate or inadequate chelation therapy. Patients for whom all three criteria were adverse constituted a very high risk group (class 3) for marrow transplantation. On the basis of these analyses, a conditioning regimen was designed that yielded superior results for class 3 patients under 17 years of age. Most patients older than 16 years presenting for transplantation have disease characteristics that place them in class 3 and, because of the improved results with the new class 3 regimen in younger patients, a study was designed to treat patients older than 16 years using treatment regimens assigned on the basis of disease class. Twenty patients were treated using this protocol and, with a minimum follow-up of 9 months, there have been three early deaths, one patient has recurrent thalassemia, and 16 patients are alive disease-free. The actuarial probabilities of survival, disease-free survival, and rejection are 0.85, 0.80, and 0.05, respectively, with a survival plateau extending from 6 months to 3 years. Marrow transplantation is a reasonable option for adults with progressive thalassemia who have suitable donors.

Adolescent

Bone marrow transplantation in thalassemia.

Since 1983, 350 patients aged 1 to 19 years with beta-homozygous thalassemia were given infusions of HLA-identical marrow after high doses of busulphan and cyclophosphamide. Survival and event-free survival leveled off about 1 year after bone marrow transplantation at 82% and 75%, respectively. In 172 consecutive patients who were treated with our current regimen since June 1985, a multivariate analysis demonstrated that portal fibrosis, hepatomegaly, and a history of inadequate chelation therapy were significantly associated with reduced probabilities of survival and event-free survival. The patients were divided into three classes on the basis of the presence of hepatomegaly, portal fibrosis, and inadequate chelation therapy. Class 1 had none of the factors and class 3 had all three factors; class 2 had different associations of two out of the three factors. For class 1 patients, the 3-year probabilities of survival and event-free survival were 97% and 94%, respectively. For class 2 patients, the probabilities were 86% and 83%, and for class 3 patients, 58% and 52%. Bone marrow transplantation from HLA-identical donors is followed by a high probability of event-free survival in thalassemic patients, particularly if they belong to class 1.

Adolescent

Bone marrow transplantation in patients with thalassemia.

We reviewed the results of transplantation of allogeneic marrow from HLA-identical donors in patients with beta-thalassemia who were less than 16 years old. Among the 222 consecutive patients who had received transplants since 1983, survival and event-free-survival curves leveled off about one year after transplantation, at 82 and 75 percent, respectively. Pretransplantation clinical characteristics were examined for their impact on survival, event-free survival, and the recurrence of thalassemia in the 116 consecutive patients who were treated with our current regimen, in use since June 1985. In a multivariate analysis, portal fibrosis and either the presence of hepatomegaly or a history of inadequate chelation therapy were significantly associated with reduced probabilities of survival and event-free survival. The patients were divided into three classes on the basis of the presence of hepatomegaly or portal fibrosis (class 1 had neither factor, class 2 had one, and class 3 had both). For class 1 patients the three-year probabilities of survival, event-free survival, and recurrence were 94, 94, and 0 percent, respectively. For class 2 patients the probabilities were 80, 77, and 9 percent, and for class 3 patients 61, 53, and 16 percent. We conclude that for patients under 16 years of age, transplantation of bone marrow from an HLA-identical donor offers a high probability of complication-free survival, particularly if they do not have hepatomegaly or portal fibrosis.

Adolescent

Longitudinal melanonychia induced by 3'-azidodeoxythymidine. Report of 9 cases.

9 Caucasian patients who developed longitudinal melanonychia during 3'-azidodeoxythymidine (AZT) treatment for HIV infection are reported. The occurrence of nail pigmentation was not related to drug dosage or to the severity of the HIV infection. AZT as well as other drugs acting on DNA synthesis or transcription seem to be a quite frequent cause of longitudinal melanonychia.

Adult

Clinical and immunological assessment in HIV+ subjects receiving inosine-pranobex. A randomised, multicentric study.

Inosine-pranobex (methisoprinol, isoprinosine; INPX) is the p-acetamidobenzoic salt of N,N-dimethylamino-2-propanol and inosine in a 3:1 molar ratio. In early studies, INPX was found to partially inhibit human immunodeficiency virus (HIV) and to increase the immunocompetence of HIV-infected subjects in vitro. We report the results of a randomised, multicentric clinical trial carried out on 553 HIV+ patients. 261 individuals were treated with INPX (two 500 mg tablets every 6 h for 3 months) and the remaining 292 constituted the untreated control group. INPX treatment was associated with a slightly improved clinical condition or with a trend in that direction, as compared to the untreated group. A preservation of the CD4/CD8 cell ratio values, a decrease in the CD8+ cells and an increase in the Leu 2-7+ cell number better than in the untreated individuals was also observed in the patients taking INPX. No serious or adverse effects of INPX have been observed.

Acquired Immunodeficiency Syndrome

[Unilateral Moebius syndrome associated with Poland's abnormality in a 25-year-old woman (author's transl)].

The authors present a case of unilateral Moebius syndrome associated with Poland abnormality in a 25-year-old woman. The patient had a complete left peripheral facial paralysis associated with involvement of both VI. In addition, there was microdactyly, syndactyly of the left hand, absence of the pectoralis major, of the mammary gland and nipple adding up to Poland syndrome. Surgery was envisaged with the aim of performing a hypoglossofacial anastomosis. Operation revealed muscles of normal appearance with a somewhat thin digastric and total absence of the facial nerve both at the point of its emergence through the stylomastoid foramen as well as in the parotid.

Abducens Nerve

[Memorization and headaches (author's transl)].

The pathogenicity of headaches is still, in many cases, a mystery evoking the involvement or modification of basic algogenic processes. These types of headaches are usually resistant to true analgesics, and a medication which acts on the mesolimbic system could be effective. Tiapride, which has the characteristic of acting at this level, is not a true analgesic according to classical tests. Experimentally, however, it has been shown to inhibit the hypertensive algogenic response, which is a test of the overall response of the organism to painful stimuli. This special activity of tiapride probably accounts for its efficacy in many types of headache which are resistant to classical medication, the underlying tension, and emotional and memorization problems being due to the involvement of the mesolimbic system.

Analgesics