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J Litzman

Publications and source records attributed to J Litzman.

35 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↗
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↗
PubMed1992

[Plasma viscosity. The effect of plasma proteins].

The authors assessed the plasma viscosity by means of a capillary viscosimeter of their own design and assessed at the same time the concentration of 22 plasma proteins or lipids. Based on the results of these examinations it proved possible to elaborate an original equation which describes the plasma viscosity as a function of the concentration of fibrinogen, alpha-2-globulins (and among them most probably haptoglobin), gammaglobulins, IgA and IgM. The authors discuss in detail the importance of this finding for clinical haemorheology.

Blood Proteins↗
PubMed1991

[Current therapeutic use of gamma globulin preparations].

Non-specific intramuscular and especially intravenous immunoglobulins became an important part of the treatment of two different immunopathological states. They are used as substitution therapy in absolute or relative shortage of specific antibodies in primary humoral immunodeficiencies--especially various types of hypogammaglobulinemia, and secondary humoral immunodeficiencies--lymphatic system malignancies, serious septic states etc. The second indication area of intravenous immunoglobulins is based on the proved abilities of intravenous immunoglobulins to suppress the production of antibodies (and autoantibodies), and to block phagocytic cells. Intravenous gammaglobulin treatment is generally used in idiopathic thrombocytopenic purpura and Kawasaki disease. The results of the treatment of autoimmune diseases of connective tissue, hematological and nervous system diseases are also promising. Side-effects of treatment are not frequent, but anaphylactic reactions may be fatal.

Humans↗
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↗
PubMedDate not supplied

Early manifestation and recognition of C2 complement deficiency in the form of pyogenic infection in infancy.

OBJECTIVE: Although frequently asymptomatic, C2 complement component deficiency may lead to severe pyogenic infections or lupus-like illness. In the present report, we describe infectious manifestations in infancy and childhood in our C2-deficient patients. METHOD: A retrospective study of clinical manifestation in three patients was carried out. C2 deficiency was proved both by undetectable serum C2 level and typical homozygous 28 bp deletion of the C2 gene. RESULTS: All patients were hospitalized at least once by the age of 12 months, each had one episode of meningitis in infancy, one also had arthritis with septicaemia. Infections of the respiratory tract were the causes of other hospitalizations. Two patients also suffered from frequent mild respiratory tract infections; in both patients, decreased immunoglobulin IgA and immunoglobulin IgG2 or immunoglobulin IgG3 levels were recorded. CONCLUSION: Our observations point to an early manifestation of C2 deficiency within the first year of life, with meningitis as the most severe complication. The severity of immunodeficiency may be influenced by concomitant deficiencies of immunoglobulin isotypes.

Bacterial Infections↗
PubMedDate not supplied

The mechanism of erythrocyte sedimentation in Westergren's examination.

The authors deduced the equation that describes the sedimentation of erythrocytes as the function of time, hematocrit, hemoglobin and some plasma protein concentrations and the citrate viscosity and density. This values served to describe plasma and erythrocyte density, plasma viscosity, erythrocyte aggregation and the influence of suspension concentration on the erythrocyte sedimentation rate. The influence of citrate on blood dilution (the reduction of hematocrit and plasma protein concentrations) was also considered. A good agreement between the observed and predicted values was obtained.

Blood Sedimentation↗
PubMedDate not supplied

Analysis of zinc, iron and copper serum levels in patients with common variable immunodeficiency.

Serum zinc, copper and iron levels together with content of zinc in hair were investigated in 13 patients with common variable immunodeficiency (CVID) and in 13 controls. A significant decrease in serum zinc (mean = 11.3 mumol/l, SD = 2.9 in CVID patients compared to mean 14.3 mumol/l, SD = 2.4 in controls) and iron levels (mean = 11.8 mumol/l, SD = 3.1 in CVID patients, mean = 18.3 mumol/l, SD = 4.7 in controls) in CVID patients were observed. Hair zinc content of CVID patients was significantly decreased compared to healthy persons (1.41 mumol/g, SD = 0.64 in CVID patients, and mean = 2.23 mumol/g, SD = 0.83 in controls). Serum copper level in CVID patients was significantly increased compared to controls (mean = 24.4 mumol/l, SD = 4.8 in CVID Patients, mean = 14.6 mumol/l, SD = 2.8 in controls). The decreased serum zinc and iron levels may be caused by disturbed absorption in the intestines of patients with CVID, but redistribution due to chronic inflammatory processes is a second possible explanation of hypozincemia in CVID patients.

Adolescent↗
PubMedDate not supplied

Progression of selective IgA deficiency to common variable immunodeficiency in a 16 year old boy.

A case report of a 16 year old boy in whom selective IgA deficiency progressed to typical common variable immunodeficiency (CVID) is described. This boy with a history of frequent but not severe respiratory tract infections was referred to hospital because of severe pleuropneumonia and decreased levels of IgA (0.23 g/L), but normal IgG and IgM levels. Lymphocyte subpopulation determination revealed a decreased proportion of CD4+ lymphocytes (30%) and an increased proportion of CD8+ lymphocytes (32%), while CD3+, CD19+ and CD16+/56+ subpopulations were normal. During the subsequent 17 months a gradual decrease in IgG (ultimate level 2.23 g/L), IgA (< 0.05 g/L) and IgM (< 0.05 g/L) levels was observed, the decrease in IgM being the slowest reflecting a constant heavy chain gene order on chromosome 14. The observation supports the thesis of a close relation of selective IgA deficiency and common variable immunodeficiency.

Adolescent↗