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Biomedical subjects

H Strawczynski

Publications and source records attributed to H Strawczynski.

11 recordsLinked to original sources
PubMed1998

Effect of splenectomy on slowing human immunodeficiency virus disease progression.

BACKGROUND: Lymphoreticular tissue is the most important site for human immunodeficiency virus (HIV) replication in HIV-infected individuals. OBJECTIVE: To compare the long-term effect of splenectomy on survival and time to development of acquired immunodeficiency syndrome in subjects who had undergone splenectomy with subjects who had not undergone splenectomy. DESIGN: A cohort study with a follow-up of up to 13.4 years. SETTING: Subjects were recruited from a hospital outpatient clinic population and a multicenter study of patients with hemophilia. PARTICIPANTS: Forty-five HIV-infected individuals were observed prospectively for up to 13.4 years (17 had undergone splenectomy and 28 had not undergone splenectomy). Five subjects underwent splenectomy before acquiring HIV infection and 12 underwent splenectomy during the asymptomatic phase of HIV infection. The group who did not undergo splenectomy consisted of HIV-infected individuals who were asymptomatic at study enrollment. MAIN OUTCOME MEASURES: A Cox proportional hazards model was used to test the effects of splenectomy on survival and time to development of acquired immunodeficiency syndrome when adjusting for potential confounders (age, initial CD4+ cell count, and treatment with antiretroviral drugs). Splenectomy was treated as a time-dependent covariate to account for the variation in its timing. RESULTS: During the average follow-up of 8.6 years, 9 (53%) of the 17 subjects who underwent splenectomy and 23 (82%) of the 28 subjects who did not undergo splenectomy died; acquired immunodeficiency syndrome developed in 6 (35%) of the subjects who underwent splenectomy and 23 (82%) of the subjects who did not undergo splenectomy. Splenectomy was associated with a significant reduction of risk of developing acquired immunodeficiency syndrome (adjusted relative risk [RR] <0.4, P<.05), whereas the effect on risk of mortality approached, although it did not reach, significance (adjusted RR approximately 0.5, P approximately .10). CONCLUSION: The absence of a spleen during the asymptomatic phase of HIV infection seems to have a beneficial effect on HIV disease progression.

Acquired Immunodeficiency Syndrome↗
PubMed1998

Laboratory and clinical markers of HIV infection in a national haemophilia cohort treated with recombinant factor VIII concentrate. The Association of Hemophilia Clinic Directors of Canada.

Over an interval of approximately six months beginning in October 1993, most haemophilia A patients in Canada were switched from a plasma-derived intermediate-purity factor VIII concentrate (i.p. VIII) to a recombinant factor VIII (rVIII). In order to determine the consequence of this change in therapy on progression of HIV infection, we gathered surveillance data on clinical status and CD4 and CD8 cell counts in those patients who were HIV seropositive at the time of switching concentrates. Data were recorded at the time of switchover, annually for 2 years thereafter, and retrospectively at a point 1 year prior to the switch. CD4 cells fell significantly over the study period. Multiple direct comparisons revealed that this decline was restricted to the time intervals which included the final year in which patients received intermediate-purity factor VIII concentrate (i.p. VIII). In the 2 year interval in which rVIII was used exclusively, there was a nonsignificant fall in CD4 cells. Changes in CD4 cells did not correlate with the intensity of exposure to either i.p.VIII or rVIII. CD8 cells did not fall significantly over the study period. There was no obvious reduction in the incidence of death or clinical progression over the 2 years in which rVIII was used. However, we are hopeful that the stabilizing trend in CD4 cell counts which followed the introduction of rVIII will be predictive of corresponding clinical stabilization over the coming years.

Biomarkers↗
PubMed1997

The Juvenile Arthritis Quality of Life Questionnaire--development of a new responsive index for juvenile rheumatoid arthritis and juvenile spondyloarthritides.

OBJECTIVE: To develop a disease specific measure of quality of life for application in children with juvenile rheumatoid arthritis and juvenile spondyloarthritides-the Juvenile Arthritis Quality of Life Questionnaire (JAQQ). METHODS: Patients and their parents were interviewed by a trained interviewer using a questionnaire focusing on physical function, psychosocial function, and general symptoms to determine the most appropriate items to include in the JAQQ. Respondents volunteered items and scored them for frequency of occurrence and importance. Items so generated were scored by a panel of experts for potential responsiveness and categorized into dimensions. Item number was reduced using this scoring system. The product was then pretested to confirm its construct validity and responsiveness. Thereafter, it was distributed to clinical experts to establish face and content validity. RESULTS: 91 patients, mean age 10.35 years (range 1.25-18.0), mean disease duration 3.99 years, and their parents were included in the interview process. 220 items generated were ultimately reduced to 85. Pretesting this version of the instrument in a further 30 patients showed it to have construct validity and responsiveness and led to a further reduction in items to 74, distributed in 4 dimensions: gross motor function (17 items), fine motor function (16 items), psychosocial function (22 items), and general symptoms (19 items). Face and content validity were established in 20 clinicians. Scaling was by 7 point Likert scale to enhance responsiveness. English and French versions were developed. CONCLUSION: The JAQQ measures physical and psychosocial function and an array of general symptoms. Preliminary data suggest it is valid and responsive and thus might have potential in clinical trials.

Adolescent↗
PubMed1987

Monocyte-macrophage (M-M) functions in asymptomatic hemophiliacs and supertransfused thalassemics.

Patients suffering from acquired immune deficiency syndrome (AIDS) succumb to opportunistic infections due to a generalized failure of their cell-mediated immune defenses. The monocyte-macrophage (M-M) system plays an important role in host defense against viruses, protozoa, mycobacteria, and tumours, all potentially involved in the terminal stages of AIDS. We studied M-M functions in 55 asymptomatic hemophiliacs, 20 supertransfused thalassemics, and 9 von Willebrand's syndrome patients over a period of 17 months to establish the part played by chronic repeated blood component transfusions on the macrophage defense system. We found a significant impairment of chemotaxis in 24 out of 55 hemophiliacs, 13 out of 20 thalassemics, and 4 out of 9 von Willebrand's patients. In contrast, Candida pseudotropicalis killing was markedly increased in all 3 patient groups, while Candida phagocytosis was most significantly diminished in thalassemics. Fc-receptor-dependent erythrophagocytosis showed wide variations in all patients, with a significant decrease only in thalassemics. Phorbol ester-activated nitroblue tetrazolium (NBT) reduction to formazan was normal in most cases, but background spontaneous NBT was often much higher than in the controls. Monocyte adhesion to plastic appeared impaired in hemophiliacs and von Willebrand's Syndrome patients, yet was of no statistical significance. It thus appears that continuous, repeated blood component transfusions cause an alteration of M-M functions. This may be either due to chronic, recurrent antigenic stimulation by foreign proteins transfused, or oncogenic and known immune suppressive viruses, like cytomegalovirus, Epstein-Barr virus, hepatitis, and human T cell lymphotropic virus III. This in turn may increase susceptibility to AIDS.

Adolescent↗
PubMed1983

Immunologic dysfunction in patients with classic hemophilia receiving lyophilized factor VIII concentrates and cryoprecipitate.

The occurrence of the acquired immune deficiency syndrome (AIDS) in patients with hemophilia has suggested that an infectious agent transmitted through the frequent use of pooled blood products could be responsible. To determine if the amount or type of factor VIII preparation alters the risk of acquiring immune defects, three groups of asymptomatic heterosexual men were studied: 34 with severe classic hemophilia who were receiving lyophilized factor VIII concentrate, 10 with either mild classic hemophilia or moderately severe von Willebrand's disease who were receiving cryoprecipitate and 22 normal men who served as controls. Anergy was noted in 68%, 57% and 5% respectively of the three groups. In comparison with the control group, the group treated with lyophilized factor VIII concentrate had a significantly decreased mean ratio of helper to suppressor T lymphocytes, poor responses of the lymphocytes to mitogens, high unstimulated background activity of these cells and significantly elevated serum IgG levels. Although some of the patients with classic hemophilia who were treated with cryoprecipitate were also anergic, they did not manifest these in-vitro abnormalities. The data indicate that a majority of apparently immunocompetent individuals with classic hemophilia show in-vivo and in-vitro evidence of impaired cellular immunity and may be at risk for the development of opportunistic infections and neoplasms.

Acquired Immunodeficiency Syndrome↗
PubMed1983

Lymphocyte transformation to connective tissue antigens in adult and juvenile rheumatoid arthritis, osteoarthritis, ankylosing spondylitis, systemic lupus erythematosus, and a nonarthritic control population.

Transformation of peripheral blood lymphocytes after exposure to connective tissue antigens was measured in patients with adult (n = 35) and juvenile rheumatoid arthritis (n = 34), osteoarthritis (n = 21), ankylosing spondylitis (n = 15), and systemic lupus erythematosus (n = 26) and in control subjects (n = 36). The connective tissue antigens included homologous cartilage-type proteoglycan, cyanogen bromide-derived peptides of type I, II, and III collagens, and type I and II helical collagens. Lymphocyte transformation was not detected in the osteoarthritic and control groups, with one exception. Sensitization to at least one connective tissue antigen was detected in approximately one-third of the rheumatoid arthritic and lupus patients and in one-quarter of the juvenile rheumatoid patients. In ankylosing spondylitis, positive responses occurred to proteoglycan in 20% of patients tested but never to collagens or peptides. Sensitivity to proteoglycan was detected only in ankylosing spondylitis except for one patient with juvenile rheumatoid arthritis. In patients with systemic lupus erythematosus and both forms of rheumatoid arthritis, lymphocyte transformation was usually more frequently detected to peptides than to the helical collagens. In adult rheumatoid arthritis, type II peptides elicited an elevated number of responses (14%) as did type I (9%) and III (8%) peptides to lesser degrees. Responses to type I (4%) and II (4%) helical collagens were infrequent. Rheumatoid arthritic patients usually exhibited sensitivity to only one antigen and lymphocyte transformation was often detected when the arthritis was improving. In juvenile rheumatoid arthritis, lymphocyte transformation was detected to peptides of type I (16%), II (9%), and III (29%) collagens and to helical type I (12%) and II (8%) collagens. In systemic lupus erythematosus, sensitization was detected to peptides of type I (13%), II (20%), and III (14%) collagens and to helical type I collagen (18%) but not type II collagen. Simultaneous sensitivity to several antigens often occurred in both systemic lupus erythematosus and juvenile rheumatoid arthritis. Examination of individual patients in all three rheumatic disease groups revealed that immune sensitivity developed to collagen peptides rather than to the helical molecules, particularly in the case of type II collagen. Thus, some patients with inflammatory arthritis exhibit immune responses to connective tissue components which are, as a group, characteristic for each type of arthritis. These responses, which were not obviously associated with disease activity, may develop as a result of inflammation or trauma which destroys connective tissue and exposes molecules, in either a native or degraded state, to cells of the immune system. Expression of sensitivity to these tissue antigens may contribute to the chronicity of the inflammatory arthritides.

Adult↗
PubMed1980

Evaluation of cardiac function in patients with thalassemia major.

The application of noninvasive techniques to the evaluation of cardiac function in iron overload has identified a high incidence of abnormalities in asymptomatic patients prior to the onset of overt cardiac deterioration. Of the tests we have used, radionuclide cineangiography appears to be the most sensitive because it can be conveniently applied during the physiological stress of exercise. Other tests of cardiac function that include stress are also likely to be more sensitive than resting measurements of cardiac function. Systematic application of these techniques to the study of patients on iron chelation therapy should results in an early determination of the efficacy of such treatment.

Adolescent↗