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Biomedical subjects

J Lokaj

Publications and source records attributed to J Lokaj.

At least 37 records · Page 2Linked to original sources
PubMed1996

Orally administered bacterial lysate Broncho-Vaxom for the treatment of common variable immunodeficiency.

Broncho-Vaxom (B-V) is a lysate of eight bacterial pathogens of the respiratory tract with immunomodulatory properties. It is used in prophylaxis of respiratory tract infections. We conducted an open, placebo controlled, cross-over trial of B-V treatment in patients with common variable immunodeficiency (CVID). B-V or placebo were given for the period of four months either in winter or spring period. No significant improvement of clinical state of patients during the period of B-V treatment compared with the period when placebo was administered was observed. When subjective comparison of the health state of patients with the same period of the previous year was assessed, significant improvement after B-V treatment compared to placebo was recorded. Evaluating serum immunoglobulin levels a significant increase in serum IgA level was observed after B-V treatment. The results warrant further studies of B-V treatment in CVID patients.

Adjuvants, Immunologic↗
PubMed1996

[Laboratory markers in hepatitis C virus infection in patients with antibody formation disorders treated with immunoglobulin preparations].

The presence of hepatitis C virus (HCV) was investigated in 20 agammaglobulinemic patients using polymerase chain reaction (PCR) and by specific anti-HCV antibodies detection. Fourteen patients suffered from common variable immunodeficiency (CVID), 5 patients from X-linked agammaglobulinemia and one from specific antibody-formation deficiency. All patients were treated by the replacement immunoglobulin therapy (6-30 years), in 19 of them also intravenous immunoglobulin was used. Although 2 patients suffered from chronic hepatic disease (chronic active hepatitis and granulomatous hepatitis of unknown origin), in none of the investigated patients any laboratory marker of HCV infection was proved. Although no HCV infection was observed in our group of patients, PCR for HCV-RNA should be performed in all patients previously treated by the intravenous immunoglobulin.

Adult↗
PubMed1995

[Personal experience with replacement therapy with intravenous gamma globulin].

Replacement therapy with intravenous immunoglobulin (IVIG) is currently the therapy of choice in patients with antibody-formation deficiency. Our 30-month experience with IVIG treatment in 8 patients with common variable immunodeficiency and in 4 patients with x-linked agammaglobulinemia previously treated by intramuscular immunoglobulin or low-dose IVIG is presented. Long-term dosage was 400 mg/kg once in 3--4 weeks. Ten patients reported an improvement of their health state, especially the signs of their chronic bronchitis improved. Compared to 2 cases of pneumonia which occurred within 30 months before IVIG therapy had started, no case of pneumonia occurred during IVIG treatment. Infusion rate of 4 mg/kg/min was safe enough in 11 of our patients. Serum trough IgG level is the most important for laboratory monitoring of the patients under IVIG therapy.

Adult↗
PubMed1994

A defect in the early phase of T-cell receptor-mediated T-cell activation in patients with common variable immunodeficiency.

Common variable immunodeficiency (CVID) is characterized by an impairment of specific antibody production and a decrease in all or selected Ig isotypes. Abnormalities at the level of the B cells, T cells, and antigen-presenting cells have been described. In the present study, we have focused our attention on T-cell activation in CVID. T cells from 15 of 24 patients failed to respond to recall antigens (eg, tetanus toxoid, Escherichia coli). Of these 15 patients, 11 were studied in detail and showed significantly decreased T-cell proliferative responses and/or decreased interleukin-2 and interferon-gamma production on T-cell receptor-mediated stimulation with recall antigens and superantigens (staphylococcal enterotoxins [SE]); however, T-cell response to mitogens (anti-CD3 monoclonal antibody, phytohemagglutinin) was normal. The defect in interleukin-2 and interferon-gamma release on tetanus toxoid stimulation could also be documented in purified CD4 T cells of the patients and was present in patients with high and normal CD8 counts alike. Furthermore, patients' T cells failed to mount a significant elevation in free intracellular calcium (Ca++ flux) in response to superantigen, whereas the response to phorbol myristate acetate and ionomycin, bypassing receptor-mediated signaling, was unimpaired. These results indicate a defect in the early phase of T-cell activation after triggering of the T-cell receptor in a significant subgroup of CVID patients.

Adolescent↗
PubMed1994

The costimulatory signal CD28 is fully functional but cannot correct the impaired antigen response in T cells of patients with common variable immunodeficiency.

A wide spectrum of different immunologic abnormalities have been postulated as being responsible for the impairment of specific antibody production and the decrease in all or selected immunoglobulin isotypes present in common variable immunodeficiency (CVID). These abnormalities include impaired B cell differentiation and/or function, defective macrophage function, and significant T cell defects. The aim of the present study was to delineate whether the accessory molecule CD28 is involved in the impaired antigen response of T cells from patients with CVID. Our results demonstrate that CD28 costimulation was functional in T cells stimulated with anti-CD3 or anti-TCR MoAb, but could not correct the impaired response of patients' peripheral blood T cells to tetanus toxoid. Analysis of patients' long-term cultured T cells further confirmed these results. Exogenous rIL-2, another costimulus, augmented but did not correct the defective proliferation and lymphokine production in patients' antigen-driven peripheral blood T lymphocytes or in long-term cultured T cells. These findings indicate that the CD28 signalling pathway in these patients' T cells is unimpaired, and that costimulation via CD28 cannot correct the defect occurring in the course of TCR-mediated T cell activation.

Adolescent↗
PubMed1993

[Immunologic laboratory findings in relatives of patients with common variable immunodeficiency].

A statistically significant increase of selective IgA deficiency was found evaluating 52 first degree relatives of 15 patients with common variable immunodeficiency, 4/5 of these cases were observed in the children of our patients. In addition to this decreased mean serum IgM level and increase of frequency of antithyroid autoantibodies compared to control subjects were observed. An excess of rheumatoid factor or antinuclear antibodies as not observed.

Adolescent↗
PubMed1993

Activation of CVID patients' T cells with conventional antigens and superantigens.

Defects in T cell function are known to be present in a subset of patients with CVID, but the true nature of these defects still has to be revealed. In prior studies we described that T cells from these patients show an impaired proliferative response following activation with recall antigens (E. coli, Tet. Tox., TBE and PPD). Gene expression of IL2 and IFN-gamma in patients' T cells following antigenic stimulation was significantly reduced compared to controls, while IL-2R transcripts were normal. To further characterize the defect we examined T cell responses to bacterial enterotoxins, collectively termed superantigens. Following stimulation with optimal (10 ng/ml p < 0.05) as well as suboptimal (1 ng/ml p < 0.0025) concentrations of staphylococcal enterotoxin A (SEA), proliferative response and cytokine release (IL-2 and IFNg) were significantly decreased in patients' T cells as compared to controls'. When patients' T cells were stimulated with staph. enterotox. C3 (SEC3) an even more pronounced difference between patients' and controls' T cells could be observed (10 ng/ml p < 0.002, 1 ng/ml p < 0.0005). Our data indicate that, in addition to the defect in antigen-induced T cell activation, T cells of CVID patients express a broader impairment in the interaction between the antigen presenting cell and the TCR.

Antigens↗
PubMed1992

[Side effects of administration of gamma globulin preparations in patients with primary agammaglobulinemia].

Eight patients with adverse reactions were observed in a group of 20 primary agammaglobulinaemic patients treated with gammaglobulin derivatives. In four cases only sporadic reactions were observed, but in 2 cases repeated reactions made gammaglobulin therapy impossible. In the remaining two patients a change of gammaglobulin derivatives made adequate therapy possible. Possible causes of adverse reactions during gammaglobulin treatment and their prevention are discussed.

Adolescent↗
PubMed1991

Family studies in common variable immunodeficiency.

The occurrence of cancer, immunodeficiency, and diseases with possible autoimmune aetiology were studied in 355 blood relatives of 12 patients with common variable immunodeficiency (CVID). The family members were identified through the patients and interviewed after completing a questionnaire, their diseases were medically confirmed by local general practitioners. In two families consanguineous marriages were identified with the coefficients of inbreeding of 0.03125 and 0.01563, respectively: one patient, a dizygotic twin of an unaffected sister, was a granddaughter of first cousins, the second patient was the third daughter of second cousins. These cases of CVID strongly support the autosomal recessivity of the underlying genes. One male patient with CVID was shown to be related to a patient with X-linked hypogammaglobulinaemia, both sharing a common carrier. The different clinical courses of their diseases suggest two genetically determined immunodeficiencies and genetic heterogeneity. No family had an unusual clustering of cancer. The occurrence of tumours in the blood relatives of CVID patients was not significantly higher than in the relatives of spouse controls. Immunological examination of 30 first degree relatives of the CVID patients revealed three children (2 males and 1 female) with selective IgA deficiency, in one boy combined with elevated serum IgE level. Four relatives with rheumatoid heart disease, 12 cases of gastric or duodenal ulcer, and 14 relatives with thyroid disease represented the most often encountered diagnoses with a possible autoimmune component in their aetiology.

Autoimmune Diseases↗
PubMed1990

Common variable immunodeficiency and malignancy: a report of two cases and possible explanation for the association.

Two patients with common variable immunodeficiency (CVID) and malignant tumours are reported. The first patient developed myelogenous leukaemia soon after the myelodysplastic syndrome has been diagnosed. The undifferentiated gastric lymphoma found in the second patient suggests that an increased risk of gastrointestinal malignancies in CVID could partly be due to lymphomas. We hypothesize that the tissue- or site-specific risk of lymphomas and gastrointestinal cancer can be explained by an increased chromosomal or genomic instability with a higher mutation rate and genomic disorganization, and that this instability could be related to viral carcinogenesis. The primary immunodeficiency per se may not be responsible for the cancer susceptibility in CVID patients.

Adult↗
PubMed1989

Increased bleomycin-induced chromosome damage in lymphocytes of patients with common variable immunodeficiency indicates an involvement of chromosomal instability in their cancer predisposition.

To study mutagen-induced chromosome instability in cancer disposition, late S and G2 lymphocytes of 15 patients with common variable immunodeficiency and 14 healthy controls were exposed to bleomycin in vitro. The groups did not differ in the frequency of spontaneous chromosome aberrations. In bleomycin-treated samples we found higher numbers of break events per cell and increased frequency of cells with aberrations compared to the control group. A slightly reduced breakage of chromosome group D was noted in patients. These results support the hypothesis that a higher incidence of cancer in patients with genetically determined immunodeficiencies may be explained by an increased mutagen-induced chromosome instability in at least some of them.

Bleomycin↗
PubMed1988

Hereditary angioedema in Czechoslovakia. Clinical, immunological, genetic and therapeutic studies of 16 families.

The analysis of the findings in 16 families with hereditary angioedema detected in Czechoslovakia over the years 1975-1986 is being presented. In 14 families C1-inhibitor deficiency and in two families afunction of C1-inhibitor was established. Of the total number of 175 examined family members C1-inhibitor defect was registered in 66 subjects (60 with deficiency and 6 with afunction), and of these 48 suffered from clinical symptoms of edematous attacks affecting the skin, larynx, intestine and urinary tract, whereas 18 subjects were asymptomatic. Genealogical studies confirmed that the defect is inherited as an autosomal dominant trait. In 16 patients long-term prophylactic treatment with Danol was introduced. The effective minimum dosage was tested individually for each patient.

Angioedema↗
PubMed1987

Immunorestoration in children with recurrent respiratory infections treated with isoprinosine.

In 27 children, 4-8 years old, with recurrent respiratory infections of the upper and lower respiratory tracts Isoprinosine (ISO) tablets were administered for 7-10 days at daily doses of 50-100 mg/kg. Clinical signs of acute respiratory disease, including temperature abnormalities and subjective complaints, subsided in a short time and the children showed no symptoms for periods ranging from several weeks to several months following the therapy. The children were selected for immunotherapy with ISO on the basis of their low levels of E-rosette forming cells in peripheral blood. Several immune function parameters assessed immediately after treatment with ISO and compared with those obtained before illness and ISO administration. Low levels of T-lymphocytes returned to normal after ISO therapy, B-lymphocyte relative and absolute numbers, however, were not affected by the treatment. Nor were any changes due to ISO found in immunoglobulins, complement components, beta 2-microglobulin and C-reactive protein. Moreover, ISO had no stimulative effect on spontaneous tetrazolium reductase activity of granulocytes but it showed a slight inhibition of their phagocytosis-associated metabolic activity.

Child↗