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Immunodeficiency in familial erythrophagocytic lymphohistiocytosis.

4 children with familial erythrophagocytic lymphohistiocytosis and hyperlipidaemia were found to have a previously unrecognised immunological deficiency syndrome which included defects in both humoral and cellular immunity and a plasma inhibitor of in-vitro lymphocyte blastogenesis. The inhibitory activity was proportional to the increase in the triglyceride concentration in the patients' plasma. Immunological deficiency, to which hyperlipidaemia may be a contributing factor, appears to be a significant feature of familial erythrophagocytic lymphohistiocytosis.

Antibody-Dependent Cell Cytotoxicity↗

Family studies in cases with malignant lymphomas.

A short review of the genetic aspects of tumors of the reticuloendothelial system is given. Of special interest is the observation of a "familial lymphohistiocytosis of the nervous system" published by PRICE et al. in 1971. Important are also our own observations with different types of malignant lymphoma in the same family. In addition to the possibilities of classical mendelian inheritance and the possibility of multifactorial inheritance preexisting immunological deficiency syndromes and chromosomal aberrations have to be considered as causal factors.

Consanguinity↗

The DiGeorge sequence. II. Immunologic findings in partial and complete forms of the disorder.

We present immunological in vitro data on a group of 16 patients with the DiGeorge sequence (DGS). Detailed tests gave evidence that the decrease in number and function of mature T-cells is closely correlated with phenotypic expression and represents a wide immunological spectrum ranging from normal function to severe combined immunodeficiency with B-cells. Careful immunological testing in DGS may help to plan therapeutic procedures (i.e. thymic factor therapy or bone marrow transplantation), may also have prognostic significance and help to gain insight into mechanisms operative in disturbances of T-cell regulation.

Adenosine Deaminase↗

Opportunistic cancers in patients with immunodeficiency syndromes.

Patients with inherited or acquired immunodeficiency disorders are vulnerable to a broad spectrum of opportunistic infectious diseases, but a narrow spectrum of malignancies. These malignancies--B-cell lymphomas, Kaposi's sarcomas, and squamous cell carcinomas--are likely due to failure of immune surveillance to recognize virally transformed target cells. The evidence for this hypothesis is substantial regarding Epstein-Barr virus-induced lymphoproliferative lesions in immunodeficient patients.

Acquired Immunodeficiency Syndrome↗

[Acquired immunodeficiency syndrome and related syndromes. Critical analysis of biological tests of cell-mediated immunity].

In order to assess the actual value of the in vitro tests of cellular immunity performed to provide diagnostic evidence for acquired immune deficiency syndrome (AIDS) or related syndromes, we have evaluated T-lymphocyte subsets and in vitro proliferative responses. The respective profiles of 44 symptom-free homosexual men, 30 patients with AIDS and 42 patients with lymphadenopathy were compared with each other and with profiles of blood bank volunteers, using stepwise discriminant analysis. At first investigation, control homosexuals, AIDS patients and lymphadenopathy patients displayed varying degrees of the same type of immunological abnormalities. The percentage of OKT4+ cells was the most discriminant variable between blood donors and control homosexuals, AIDS or lymphadenopathy patients. The latter differed from homosexuals by a higher percent of OKT8+ cells and from AIDS patients by a lesser reduction in T-lymphocyte counts. OKT4+ cell counts differentiated AIDS patients from control homosexuals. However, none of these major discriminant variables could classify correctly more than 70% of subjects in the homosexual and in the two patient groups. Thus, a single investigation of T-lymphocyte immune parameters does not provide substantial diagnostic and prognostic evidence.

Acquired Immunodeficiency Syndrome↗

[Chronic fatigue syndrome: immune dysfunction, role of pathogens and toxic agents and neurological and cardial changes].

375 patients with chronic fatigue syndrome (CFS) were examined using a standardized questionnaire and subsequent interview on 11 risk factors and 45 symptoms. Additionally immunologic, serologic, toxicologic, neuroradiologic, neurophysiologic and cardiologic investigations were performed. Immunologic tests showed cellular immunodeficiences particularly in functional regard (pathological lymphocyte stimulation in 50% of the patients, disorders of granulocyte function in 44%). Furthermore variable deviations were found in the lymphocyte subpopulations (CD3, CD4, CD8, CD19, DR, Leu 11 + 19). In the humoral part tendencies to low IgG-3- and IgG-1-subclass-levels occurred (59% respectively 11% of the patients) also as decreases in complement system (CH50, C3, C4, C1-esterase-inhibitor). In the group of activation markers and cytokines 42% of the investigated patients had circulating immune complexes (CIC), 47% increases of tumor-necrosis-factor (TNF-a) and 21% increases of soluble interleukin-2-receptor (IL-2-R). The increased occurrence of autoantibodies in the CFS-patients (specially antinuclear anti-bodies [ANA], microsomal thyroid antibodies) suggest, that CFS is associated with or the beginning of manifest autoimmune disease. Under the pathogens 78% of the patients had a striking serological constellation of Epstein-Barr-Virus (EBV-EA positive, low EBNA-titers), in the HHV-6-Virus 47% showed increased antibody-titers. Tests on further herpes viruses and on Borreliae, Chlamydiae, Candida and Amoebae were positive in 8 to 36% of the examined patients. Furthermore there were found variable deficits of vitamins and trace elements also as hormonal disturbances.(ABSTRACT TRUNCATED AT 250 WORDS)

Adult↗

[A community-based acquired immunodeficiency syndrome education intervention study in Kunshan county].

OBJECTIVE: To evaluate the effectiveness of a community-based acquired immunologic deficiency syndrome (AIDS) educational intervention in young adults. METHODS: A quasi-experimental design was used. Two townships in Kunshan county, matched by socioeconomic characteristics, were randomly assigned to intervention or control groups. Eight hundred young adults aged 18-30 were recruited from the two townships as study subjects. A 12-month comprehensive educational intervention on AIDS prevention was conducted in the intervention group through local family planning services. RESULTS: After intervention, subjects in intervention group significantly improved their knowledge on AIDS and attitudes related to AIDS prevention. Reported rate of condom use during the last episode of sexual intercourse increased from 8.5% at baseline to 14.4% after intervention (chi 2 = 4.66, P = 0.030 8). No obvious change was observed among subjects in control group. CONCLUSION: This study demonstrated the feasibility of integration of AIDS educational intervention into the existing family planning services. Integration of AIDS programs into family planning program should be considered as a realistic approach for AIDS control in China.

Acquired Immunodeficiency Syndrome↗

Chorioretinitis with a combined defect in T and B lymphocytes and granulocytes. A new syndrome successfully treated with dialyzable leukocyte extracts (transfer factor).

A patient with immune deficiency, recurrent pyogenic infections and active chorioretinitis is described; in addition to agammaglobulinemia, both quantitative and qualitative T-cell deficiencies were documented. Furthermore, the patient's granulocytes (polymorphonuclear leukocytes), although normal in their bactericidal capacity for Staphylococcus, responded poorly to both leukocyte migration inhibition factor and neutrophil immobilizing factor obtained from normal cells. The immunologic features of this patient appear to comprise a new syndrome. Remarkable diminution of the ocular lesions and increased visual acuity occurred within two months after the initiation of therapy with dialyzable leukocyte extracts (transfer factor). Concurrent testing of the patient's cell-mediated immunity showed increased numbers of circulating T lymphocytes and improved T-cell function following dialyzable leukocyte extract [DLE] therapy. The dramatic clinical results indicate that similar therapy may prove to be beneficial in other patients with chorioretinitis and T-cell deficiency.

Adolescent↗