ABSTRACT
BACKGROUND: It has been suggested that the cause of Perthes disease may be intravascular thrombosis induced by a potential congenital hemostatic disorder leading to conditions of thrombophilia or hyperfibrinolysis. METHOD: Complete study of hemostasis with coagulation and antithrombin factors as well as study of thrombophilia and fibrinolysis in these patients could determine the prevalence of hemostasis and fibrinolysis in this group of patients. PATIENTS: Twenty-five patients between the ages of 5 and 25 years during the study period and previously diagnosed with Perthes' disease underwent hemostasis and thrombophilia analysis. Results were compared with those of a control group. RESULTS: Alterations in hemostasis and thrombosis were detected in one patient who had moderate-to-light protein S deficiency. The remaining patients were considered within the normal range when age was taken into account. CONCLUSIONS: Epidemiological and laboratory data from this group of pediatric patients and from the control group do not support the hypothesis that a thrombogenesis defect could be the underlying cause of avascular necrosis of the hip joint.
Subject(s)
Legg-Calve-Perthes Disease/etiology , Thrombophilia/complications , Adolescent , Adult , Child , Child, Preschool , Female , Humans , MaleABSTRACT
A cross-sectional study was designed to determine whether plasma concentrations of glutathione and cysteine in HIV-infected hemophiliacs vary according to the progression of the disease and to compare them with those obtained in HIV negative hemophiliacs. Cysteine, total glutathione and glutathione disulphide were measured in plasma of HIV-infected hemophiliacs at different stages of HIV infection and in plasma of HIV-negative hemophiliacs. CD4 and CD8 T-cell counts, leukocyte and lymphocyte counts, beta 2-microglobulin and p24 antigen values were recorded for HIV positive hemophiliacs at the time of the study. The hemophiliac HIV-positive group showed a decrease in total glutathione levels (-18%) and an increase of glutathione disulphide (8.18 vs. 14.90%) compared to the HIV-negative group. The cysteine levels found in HIV-positive hemophiliacs were not different from those found in the HIV-negative group. There were no differences with statistical significance in total glutathione, glutathione disulphide and cysteine among HIV-infected hemophiliacs according to the different clinical stage of the disease (AIDS vs. non-AIDS). The interest of evaluating plasma concentrations of glutathione and cysteine in HIV-infected patients is limited from the point of view of considering them as markers of progression of the disease. Interest in a therapeutic strategy designed to replenish or normalize glutathione plasma levels is also limited.
Subject(s)
Cysteine/blood , Glutathione/blood , HIV Seropositivity/blood , Hemophilia A/blood , Acquired Immunodeficiency Syndrome/blood , Adolescent , Adult , Disease Progression , Female , Glutathione/analogs & derivatives , Glutathione Disulfide , HIV Seropositivity/complications , Hemophilia A/complications , Humans , Male , Middle AgedABSTRACT
BACKGROUND: The progression of HIV infection in a cohort of hemophiliacs was evaluated taking into consideration the particularities of the natural history of HIV in this population and comparing the same with that described for other series of hemophiliacs and other risk groups for HIV infection. METHODS: A cohort of 141 hemophiliac patients with HIV infection controlled in a Hemophilia Unit since January 1983 was studied. The accumulated incidence of AIDS and the mortality at 11 years of follow up were evaluated. Likewise, the association of prognostic factors such as age, type of hemophilia or previous treatment with antihemophilic factors were evaluated. RESULTS: The accumulated incidence of AIDS at the end of follow up was 56% with a mortality rate of 33%. The evolution showed statistically significant differences with regard to age (p = 0.00048) and previous treatment (p = 0.00239). No differences were observed concerning the type of hemophilia or its severity. CONCLUSIONS: HIV infection in the cohort of hemophiliacs studied presented similar accumulated AIDS incidence and mortality to those described in other series of hemophiliacs. These values are apparently more favorable than those referred for other risk groups, possibly due to the particular epidemiologic characteristics.
Subject(s)
HIV Infections/epidemiology , Hemophilia A/complications , Acquired Immunodeficiency Syndrome/epidemiology , Acquired Immunodeficiency Syndrome/mortality , Adolescent , Adult , Age Factors , Child , Child, Preschool , Cohort Studies , Female , Follow-Up Studies , HIV Infections/mortality , Hemophilia A/mortality , Humans , Incidence , Infant , Male , Middle Aged , Prevalence , Prognosis , Risk Factors , Spain/epidemiology , Time FactorsABSTRACT
BACKGROUND: The administration of factor VIII concentrates of intermediate purity in HIV+ hemophiliac patients has been related with disorders of the immunologic system of these patients which may be observed in a more acute decrease in CD4 lymphocyte count in relation to that described in patients treated with products of greater purity. The present study evaluates the impact of these treatments (concentrates of intermediate or high purity) on the immune system of HIV+ hemophiliac patients by exhaustive analysis of the literature. METHODS: Meta-analysis of 8 clinical studies published between 1983 and 1993 was performed by multivariate analysis which considers the decrease in the number of CD4 lymphocytes as an indicator of the evolution of the disease. Likewise, the size of the effect and combined significance were also evaluated. RESULTS: The absolute decrease in the number of CD4 lymphocytes is significantly lower in patients treated with products of greater purity (354 +/- 52 vs 448 +/- 54 cells/microliters p = 0.037) as was the size of the effect and the different tests of combined significance. The speed of decrease was between 1.25 and 16.55 cells/microliters/year lower in the treated group than in the control (p = 0.029). CONCLUSIONS: According to meta-analysis, the factor VIII concentrates of intermediate purity influence the immune system of HIV+ hemophiliac patients to a greater extent leading to a more acute decrease in the number of CD4 lymphocytes over time. Further studies are required to confirm whether this facts leads to differences in clinical manifestations of the infection or in survival.
