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Biomedical subjects

V Wahn

Publications and source records attributed to V Wahn.

At least 127 records · Page 7Linked to original sources

Adverse reactions in selected patients following intravenous infusions of gamma globulin.

Fifteen patients with hypogammaglobulinemia or agammaglobulinemia were treated with intravenous gamma globulin preparation over a 17-month period. The patients were selected for treatment if they had chronic antibody deficiency syndromes associated with increased susceptibility to infections. Levels of circulating immune complexes, C1q, C3, and C3d were determined in serum samples obtained before treatment and immediately following treatment with the gamma globulin. In every patient studied, circulating immune complexes were detectable in the postinfusion samples. Two patients had adverse reactions to the intravenous gamma globulin therapy. Analysis of the serum samples of both of these patients showed that one patient had an autoantibody to IgA and the other had an autoantibody to beta-lipoprotein. Both IgA and beta-lipoprotein were present in the intravenous gamma globulin preparations. Therefore, reaction with each of the autoantibodies by the antigens activated the complement system in vivo with production of split products of C3.

Adult↗

[Immunomodulation by intravenous gamma globulin?].

The continuous improvement of gammaglobulin preparations for intravenous use has lead to new potential therapeutic applications no longer based on the substitution of lacking specific antibodies. Especially high-dose administration may exert positive influences on pathologically altered immune functions. The theoretical basis and clinical facts are discussed.

Animals↗

Evidence of persistent IgA/IgG circulating immune complexes associated with activation of the complement system in serum of a patient with common variable immune deficiency: anaphylactic reactions to intravenous gammaglobulin.

A 44 year old woman with common variable immunodeficiency developed severe anaphylactic reactions to intravenous gammaglobulin. Analysis of her serum prior to the infusion of gammaglobulin was thus analyzed and the tests revealed a complete absence of free IgA, presence of an IgG autoantibody to IgA, IgA-IgG circulating immune complexes, and depressed levels of hemolytic C3 associated with elevated levels of C3a and C3d. The IgA-IgG complexes did not clear from the circulation even after six months following cessation of gammaglobulin infusions. Analysis of the complexes isolated by sucrose density gradient ultracentrifugation showed that they bind to solid phase F(ab')2 anti-C1q, have a high molecular weight (greater than 19s), activate the complement (C) system via the classical pathway in vitro and are comprised of IgG and IgA. These data suggest that in this patient an autoantibody response to IgA was probably associated with persistent endogenous production of IgA yielding IgG-IgA circulating immune complexes and activation of the complement system. Although the patient has no free IgA and no surface IgA bearing B cells, her peripheral blood lymphocytes were shown to contain cells capable of secreting IgA. Low levels of IgM and IgG were detectable in her serum, and B cells bearing surface IgM, IgG and IgD were present in normal numbers in the blood.

Adult↗

[Immunologic findings in juvenile Mediterranean kala-azar].

The course of immunological parameters in a 2 year old patient with mediterranean kala-azar is reported. Plasma-fibronectin was reduced to 33% of the normal value at the time of diagnosis. When the clinical symptoms of the disease were most severe, a marked activation of complement without demonstrable circulating immune complexes was observed. Therapy with sodium-stibogluconate lead to improvement of the child's condition, normalization of complement reaction and fibronectin levels, and detection of circulating immune complexes. The activity of the disease seems to be well indicated by the level of C-reactive protein and the complement cleavage product C3 d. Incubation of living Leishmania donovani with normal human serum leads to activation of complement in vitro.

Antibodies↗

[The anticomplementary activity of 2 intravenous gamma globulin preparations in children with acute lymphatic leukemia].

Children with acute lymphocytic leukemia received gammaglobulin replacement therapy using either one of two i.v. gammaglobulin preparations in order to reduce the frequency and severity of infections during cytostatic treatment. Sera and EDTA-plasma taken prior to each infusion and following each infusion were analyzed for several complement components and immune complexes in order to assess in vivo anticomplementary activity. Preliminary data published in this paper show only little changes in the complement system. This may be related to fact that infusions were well tolerated. IgG-levels could be raised for more than 200 mg/dl by administration of doses around 150 mg/kg body weight.

Adolescent↗

[Blood platelet function following gamma globulin infusions in children with acute lymphatic leukemia during the remission-maintaining long-term therapy].

Platelet function tests prior to and post intravenous gammaglobulin infusions (i.v. IgG) were performed in 20 children with acute lymphoblastic leukemia under conventional maintainance therapy. Platelet function was not influenced in 8 patients with decreased preinfusion IgG-levels. Severe platelet function defects, resembling an acquired storage pool disease, post i.v. IgG-infusion, however, were observed in 2 of 12 patients with normal preinfusion IgG-levels.

Blood Platelets↗

[Late manifestation of vitamin K deficiency in breast fed infants].

Two breast fed infants had late manifestations of Vitamin K deficiency. No underlying disease was found in the first case. The second patient was found to have alpha 1-Antitrypsin deficiency (Pi type ZZ). The latter patient initially responded well to a single dose of vitamin K administered orally (3 mg). However, three weeks later, he was admitted to our hospital with severe intracranial hemorrhage due to severe vitamin K deficiency. Vitamin K requirements in infants and clinical characteristics of vitamin K deficiency in infants older than 1 week are discussed.

Blood Coagulation Tests↗

Modified pharmacokinetics of I-asparaginase from E coli by formation of specific antibodies to I-Asparaginase of different immunoglobulin classes in children with acute lymphocytic leukemia.

Twenty-four children (2-15 years old) with acute lymphocytic leukemia (ALL) were treated intravenously with 1-Asparaginase (1-Asp) isolated from E coli at a dose of 3,000 U/kg body weight four times every third day as part of a standard chemotherapy protocol. Sera of patients were obtained prior to each infusion, immediately following each infusion, and at defined intervals (2, 4, 12, 24, 36, and 48 hours postinfusion) and assayed for 1-Asp enzymatic activity.1-Asp antigen, and anti-1-Asp antibodies. Results indicate that the in-vivo elimination half-life of 1-Asp activity in patients with no demonstrable specific antibody is approximately 5.5 hours. Half-life of enzymatic activity in patients with a moderately high level of specific antibodies (pre-infusion) was prolonged (approximately 7.0 hours) in comparison to the group with no specific antibodies. In patients with very high levels of specific antibodies several infusions could not be completed because of apparent anaphylactic reactions. In-vitro studies showed that experimental immune complexes made of 1-Asp and the IgG-fraction of a rabbit-anti-1-Asp antibody under conditions of antigen excess still exhibit enzymatic activity. On the basis of this observation we conclude that specific antibodies to 1-Asp in vitro and, most likely, in vivo do not inactivate the drug but may lead to either delayed elimination of enzyme activity or, in the presence of high levels of specific antibodies, anaphylactic reaction.

Adolescent↗

[Hereditary complement defects].

The complement system is one of the most important and best analyzed biological amplification systems in humans. Hereditary deficiency states of one of the components may lead to various autoimmune disorders, recurrent bacterial infections or recurrent cutaneous edema in non-atopic subjects. This review makes an attempt to describe clinics and pathophysiology of the component deficiencies known to date. A procedure for laboratory evaluation of patients with suspected complement deficiency is described.

Autoimmune Diseases↗

[A case of juvenile priapism (author's transl)].

A case of juvenile priapism is presented following cortisone and penicillin treatment for a traumatic lesion of the oesophagus. Increased coagulability from such treatment is discussed as causative factor. The importance of early surgical intervention is stressed and the methods of choice presented.

Blood Coagulation Disorders↗