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A syndrome of immunoglobulin A deficiency, diabetes mellitus, malabsorption, a common HLA haplotype. Immunologic and genetic studies of forty-three family members.

Three persons in a kindred of 43 had variable expression of a syndrome consisting of immunoglobulin A deficiency, diabetes mellitus, malabsorption, and a common HLA haplotype. Findings from the proband included life-threatening malabsorption; idiopathic intestinal mucosal atrophy with infalmmation; IgA deficiency and antibodies to multiple endocrine organs; insulin-dependent diabetes mellitus; and the major histocomptability antigens HLA-A2, B8, and DW3. In addition to the described syndrome other conditions present in the family include Graves' disease, vitiligo, hypocomplementemia, rheumatic fever, multiple sclerosis, and a high frequency of antibodies to endocrine tissue. Since Graves' disease, diabetes mellitus, and idiopathic Addison's disease have all been described in association with HLS-B8 and DW3, we believe that the occurrence of these diseases in this family suggests that a single immune response gene or gene complex is linked with HLA-B8 and DW3.

Addison Disease↗

Suppression by dietary alcohol of resistance to Cryptosporidium during murine acquired immune deficiency syndrome.

Significant immunological changes occur following LP-BM5 murine leukemia retrovirus infection as well as chronic alcohol consumption. Retrovirus infection which has proceeded to murine AIDS permitted persistent Cryptosporidium infection, while non-retrovirus infected mice were resistant. Dietary alcohol provided until the day before parasite challenge did not affect resistance in controls, but increased the numbers of oocysts in the feces of retrovirus suppressed mice. Mortality was significant in retrovirus infected mice, and exacerbated slightly by dietary ethanol, while all controls survived parasite challenge. The retrovirus infected mice had greatly reduced numbers of intestinal CD4+ T helper cells and IgA+ B cells, which may explain their loss of intestinal resistance. Clearly, the severely immunosuppressed animals with murine AIDS were more sensitive to alcohol consumption than uninfected controls. This suggests that alcohol can synergize with murine retrovirus infection to exacerbate loss of resistance to an opportunistic pathogen common in human AIDS patients.

Animals↗

[General variable immunologic deficiency with malabsorption syndrome].

Twenty-two patients with general variable immunodeficiency (GVI) and malabsorption syndrome (MS) were followed up for 2-12 years. III degree MS was found in 17 cases. Serum immunoglobulins concentration and T-lymphocyte count were reduced, the latter at the expense of theophylline-resistant and active E-RFC. With casein and milk albumin as the antigens, lymphokine-producing capacity of the mononuclear cells appeared elevated. MS treatment with adjuvant gamma-globulin produced a positive trend in clinical manifestations of the disease, content of T lymphocytes and relevant subpopulations. Long-term results were less favourable: partial compensation with recurrences persisted in 15 patients only. Seven patients died: two of pneumonia, five of cardiac failure and visceral dystrophy. All MS patients are recommended to undergo serum immunoglobulins diagnosis of GVI and in case of its verification to receive life-time gamma-globulin replacement therapy.

Adolescent↗

The potential role of vitamin E in the treatment of immunologic abnormalities during acquired immune deficiency syndrome.

The literature is briefly summarized as to immunologic modifications caused by the human acquired immune deficiency syndrome (AIDS), immunocompetence at various nutritional states of vitamin E, and the immunoenhancing properties of vitamin E. The abnormalities of immune components present in AIDS are similar to those that are stimulated or restored by intake of high doses of vitamin E. Dietary supplementation of vitamin E with an adequate nutrition support or concomitant use of this vitamin with current drug therapies [For example, Zidovudine (AZT)] may increase the therapeutic efficiency of drugs and enhance immune resistance to opportunistic infections associated with AIDS. Supplementation with vitamin E may also decrease the progression of the disease to AIDS. Unlike many pharmacological agents which are toxic at low levels, vitamin E is non-toxic over a wide range of intakes. A moderately high dose may be used to target and stimulate some specific immune cells destroyed by HIV infection. However, further interdisciplinary studies are much needed to relate various levels of intake of this vitamin as a supplement to clinical outcomes during HIV infection and establish the role for this vitamin in human immunity during AIDS.

Acquired Immunodeficiency Syndrome↗

Stress deficiency of the T-lymphocyte system exemplified by Down syndrome.

A comparison of immune competence in 26 patients with Down syndrome in an institution and 26 matched healthy controls revealed an atypical pattern of T-cell immunodeficiency in the Down-syndrome patients. The patients with Down syndrome had a lymphocytosis in blood with high counts of T (and B) cells, but with impaired effector function of T cells as judged by anergy to dinitrochlorobenzene, low responsiveness to ubiquitous antigens which elicit delayed-type hypersensitivity reactions, and low mitogenic activity of non-stimulated and phytohaemagglutinin-stimulated lymphocytes in culture. Helper-T-cell function measured by the humoral immune response to flagellin was intact, and there were minor abnormalities of the B-cell system. Attempted restoration of T-cell function with levamisole was unsuccessful. This pattern of T-lymphocytosis with impaired effector function could be explained by "stress-deficiency" of the immune system consequent upon a heavy load of infection in early life.

Adolescent↗

Thymic hormone deficiency in normal ageing and Down's syndrome: is there a primary failure of the thymus?

Normal individuals aged over 50 and most young Down's syndrome (DS) subjects had markedly reduced concentrations of circulating thymic hormone (facteur thymique sérique, FTS). Plasma from these two groups contained factors capable of inhibiting biological activity of FTS in vitro. Addition of zinc sulphate to plasma samples from DS subjects or the older individuals induced concentrations of FTS comparable to those observed in young healthy people and completely prevented FTS-inhibitory activity. These findings suggest that biologically active circulating thymic hormone is bound to zinc. The decline in thymic hormone activity in older individuals and DS subjects may be the result of changes in the mechanism of zinc-dependent activation of FTS molecules, which are probably associated with marginal zinc deficiency rather than with a primary failure of the thymus. Addition of zinc salt to plasma samples unmasks the presence of inactive FTS molecules.

Adolescent↗

[Dynamics of immune status indices of liquidators in the remote period after the accident at the Chernobyl NPP].

In the report there are present the results of monitoring of the state of immune status in the remote period after the accident at the Chernobyl Nuclear Power Plant among three groups of liquidators, which were from Moscow and the Moscow district, the Northwestern region of Leningrad district and from Krasnoyarsk territory. During the monitoring of the liquidators from different regions there was found phenotype of immune status with regional characteristics. In the Moscow district the clinical displays of immune insufficiency prevail and in the North western region the modifications of immune status prevail among liquidators. In all the groups we could see quantitative and immunoregulatory disbalance with the increase of parameters of cellular activity such as CD95+, HLA-DR+, T-activated lymphocytes, fluctuation cytotoxic cells (CD8+, CD16+), persevering disimmunoglobulinemia with differently directed modification serum of immunoglobulins at decrease B-lymphocytes. By the end of the twenty-year period after the accident we can see a similar phenotype of immune status in all the groups with increasing of the marker of late activation (HLA-DR+) and apoptotic activity (CD95+) and it is potentially dangerous regarding cancerogenesis. According to the basis of long-term monitoring there has been discovered the immune characteristic of proliferative syndrome for the first time. The monitoring of 2004 has revealed a huge similarity immune status at non-malignant growth at significant decrease CD25+ in the Northwestern region and some differences in common IgE.

Chernobyl Nuclear Accident↗