T-cell dependent and T-cell independent antibody response to bacterial polysaccharides in patients with IgG2 deficiency.
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Antibodies, Bacterial↗
Biomedical subjects
V Wahn
Publications and source records attributed to V Wahn.
[Partial tracheal rupture in a newborn infant. A rare traumatic birth complication or intubation sequela?].
The neonate was born with the help of vacuum extraction because of difficult delivery of the shoulders. After birth respiratory distress developed and endotracheal intubation and artificial ventilation became necessary. When the tube was changed in the intensive care unit the infant's clinical status suddenly deteriorated. A rupture of the trachea was diagnosed immediately followed by surgery. The child survived without neurological sequelae. The chronological sequence of symptoms after birth suggests that the rupture of the trachea primarily developed during delivery and was secondarily enlarged by repeated endotracheal intubation.
Asphyxia Neonatorum↗
[Homozygous C7 defect in a German family].
An 11 year old boy with recurrent meningitis/sepsis (once without positive bacterial culture, once with demonstration of Neisseria meningitidis in blood) was evaluated for suspected immunodeficiency. Absent activity of both the classical and alternative pathway of complement suggested a defect of the membrane attack complex. Immunochemical and functional analyses together with family studies revealed a homozygous defect of the seventh component of complement in the boy. This is the first description of C7 deficiency in a German family.
Child↗
[Adolescents and AIDS: sex behavior, knowledge and attitudes. Results of a survey of students in a large West German city].
OBJECTIVES: It was the goal of this study to describe sexual and contraceptive behaviour of adolescents as well as their knowledge about and attitudes towards AIDS and AIDS prevention, in order to assess their risk of HIV infection and to offer improved health education. METHODS: Using a questionnaire a survey was carried out among 740 pupils of several Düsseldorf schools during the summer of 1991. RESULTS: The majority of respondents neither reported frequent change of sex partners nor intercourse with unknown partners. About 50% reported occasional use of condoms. Although adolescents in this study were well informed about AIDS, there was a wish for better information on HIV infection and AIDS. CONCLUSIONS: Adolescents in this sample cannot be considered to be at high risk for HIV infection. More promiscuous subgroups should be urged to increase condom use. It should be explored why adolescents although well informed about AIDS are not satisfied with the AIDS education they received, perhaps there is a desire for more direct instructions.
Acquired Immunodeficiency Syndrome↗
[Treatment of secondary infections in symptomatic HIV-infections in childhood].
Treatment of secondary infections in HIV infected children represents a problem of increasing importance in several children's hospitals. As it is unlikely that the AIDS-problem will be solved by itself within the next years it seems reasonable to summarize our current knowledge about opportunistic infections in order to establish standards for therapy. We will mainly focus on microorganisms endemic in Germany.
AIDS-Related Opportunistic Infections↗
[Intermediate results of a multicenter study in long-term management of HIV exposed and infected children].
AIM OF THE STUDY: In most cases (about 80%) the HIV-infection of children is acquired during pregnancy or birth. Therefore peculiarities for diagnostic procedures and in the natural course of the disease can be expected if compared to AIDS acquired at adult age. Further interesting questions are the frequency of the (vertical) transmission from the infected mother to the child, the influence of mother- and/or child-related factors as well as the method of delivery on this transmission frequency. METHODS: In order to answer these questions the children of HIV-infected mothers since April 1, 1988 have been examined virologically, immunologically and clinically since birth by 6 study centers according to a standard protocol. The pregnancy data of the mothers have been documented, according to uniform criteria. Pre- or perinatally infected children not known to be antibody-positive at birth have been separately analysed. RESULTS: The frequency of pre/perinatal transmission from the mother to the child is 15.3%, the elective caesarean section seems to lower the transmission rate. Mother and/or infant related cofactors of the transmission could not be defined. In comparison to the control groups of the intrauterine HIV-exposed but not infected children, at birth the HIV-infected children were clinically and immunologically not ill. During their first months some of the infected children had a significant increase of serum IgG. Oral candidiasis, chronic recurrent diarrhoea and bacterial infections are indicator symptoms of beginning HIV-disease. The Minimum-AIDS-Prevalence in the child's first year is 24%. The relatively late AIDS manifestation (3.7 years) and the average value of 28 months of survival in retrospective observed children were a remarkable result. CONCLUSION: In general, the course of an HIV-infection in children of HIV-positive women in Germany seems to be comparable to results in other countries. The frequency of transmission from mother to child is relatively low (15.3%) and is an important result for maternity care. The caesarean section should be considered. The course of pregnancy must be examined more subtly in order to find out the still unclear causes of transmission. The Minimum-AIDS-Prevalence of 24% and the frequency of HIV-related signs and symptoms of 29% in the child's first year make it necessary to observe these children rather closely during this time.
AIDS Serodiagnosis↗
[High-dose immunoglobulin therapy of hyperbilirubinemia in rhesus incompatibility].
We conducted a multicenter controlled trial to test the hypothesis that high intravenous doses of immunoglobulin (HDivIg) can modulate the bilirubin production and reduce the frequency of exchange transfusions in newborn infants with rhesus incompatibility. Thirty-four patients with rhesus incompatibility proven by positive direct antiglobulin test (Coombs test) were randomly assigned to conventional treatment including phototherapy, with or without additional HDivIg at 500 mg/kg given over 2 h, as soon as the diagnosis was established. Exchange transfusions were performed if serum bilirubin concentrations exceeded the modified curves of Polácek by more than 2 mg/dl. The results in 32 infants were analyzed. In the HdivIg-treated group, 2 of 16 (12.5%) children required exchange transfusions, whereas it became necessary in 11 of 16 (69%) in the control group (p < 0.005). Bilirubin levels in the HDivIg-treated group were lower despite a reduced frequency of exchange transfusions. We conclude that HDivIg by a yet unknown mechanism reduces bilirubin serum levels in children with rhesus incompatibility and the need for exchange transfusions.
Bilirubin↗
[Immunomodulating therapy of systemic juvenile rheumatoid arthritis: administration of high-dose intravenous gamma globulin].
Two children of 9 and 10 years suffering from severe systemic juvenile rheumatoid arthritis were treated intravenously with high-dose human immunoglobulin. Treatment was performed every 4 weeks for 7 and 18 months, respectively. Improvement of arthritic symptoms was demonstrable by significant decreases of Ritchie index and number of swollen joints and the disappearance of heated joints in one patient. The other patient was free of arthritic symptoms since the introduction of immunoglobulin therapy. Clinical symptoms of systemic illness were markedly improved and no relapse was seen. Laboratory parameters also improved, including erythrocyte sedimentation rate, C-reactive protein, hemoglobin, and serum iron levels. Parallel investigations of immunological parameters revealed a decrease of serum Il-1 beta and Il-6 levels and a diminished in vitro production of Il-1 beta, Il-6, and tumor necrosis factor-alpha. Therefore, we suggest a decreased activation status of the monocyte-macrophage system as one possible mode of action.
Arthritis, Juvenile↗
[Intravenous immunoglobulins in bronchial asthma: a therapeutic alternative?].
Treatment of severe bronchial asthma usually requires the use of steroids. Given the known side effects of steroid treatment, potential alternative therapeutical strategies are currently evaluated; among others, intravenously administered immunoglobulins (ivIg) may be considered. In one study of 5 children with bronchial asthma and IgG subclass deficiency, an improvement of asthma was demonstrated in 4 out of the 5 patients under ivIg treatment over several months. In another study on ivIg treatment in 8 immunocompetent children with steroid-dependent asthma, there also was an improvement of asthma, leading to a reduction in the required steroid dose; furthermore, there was a diminution in skin prick test reactivity. At present, only speculations can be made about the possible mechanisms of action.
Adolescent↗
[Mechanisms of immune modulation in immune thrombocytopenia].
Since Imbach's publication in 1981 on the effect of high-dose intravenous immunoglobulins in idiopathic thrombocytopenia of childhood, many studies have been performed in order to elucidate their mechanism of action. In this review, the author tries to illustrate the mechanisms which are currently discussed and which are sufficiently supported by experimental data.
Autoimmune Diseases↗
Severe adverse reaction to carbamazepine: significance of humoral and cellular reactions to the drug.
Hypersensitivity to carbamazepine is a well-known phenomenon. The involvement of several organ systems including liver, kidney, bone marrow and other organs have been described. We have observed a 7-year-old boy who had been treated with carbamazepine for seizures. After 10 days of treatment he developed a severe illness with skin rash, high fever, lymphadenopathy, hepatosplenomegaly and lymphopenia. Only slightly decreased complement components and increased complement split products but no circulating immune complexes were demonstrable on admission. Anti-carbamazepine antibodies, T-cell-activation and a significant T-cell reactivity against carbamazepine were found, indicating specific hypersensitivity. Complete recovery was observed after discontinuation of the drug and steroid treatment.
Carbamazepine↗
Inhibition of adhesion of S-fimbriated Escherichia coli to epithelial cells by meconium and feces of breast-fed and formula-fed newborns: mucins are the major inhibitory component.
We investigated the ability of meconium, feces from human milk-fed (HMF) newborns, and feces from formula-fed (FF) newborns to inhibit adhesion of S-fimbriated E. coli to human buccal epithelial cells. S-fimbriae are a common property of E. coli strains causing sepsis and meningitis in neonates. Meconium had the highest content of neuraminic acid and the strongest inhibitory effect on bacterial adhesion. HMF also exerted high inhibitory activity while FF was markedly less active: To achieve inhibitory effects comparable to HMF a sixfold amount of FF was required. Glycoproteins from excretions were separated by gel chromatography. Fractions obtained were analyzed for adhesion-inhibiting activity. In all excretions analyzed, the mucin-containing fraction could be identified as the major inhibitory component. Inhibition was probably mediated by specific interaction of this fraction with S-fimbriae, as shown by binding of isolated fimbriae on Western blots after electrophoretic separation of glycoproteins. In conclusion, our data support the view that the mucin-containing fraction from meconium and human milk exerts antibacterial functions by preventing adhesin-mediated binding of pathogenic bacteria to mucosal epithelia.
Adult↗
Treatment of patients with chronic granulomatous disease with recombinant human interferon-gamma does not improve neutrophil oxidative metabolism, cytochrome b558 content or levels of four anti-microbial proteins.
Recombinant interferon-gamma (rIFN-gamma) has been described to enhance phagocyte functions in vitro and in vivo in several patients with chronic granulomatous disease (CGD). To demonstrate the clinical usefulness of this treatment, 128 patients were treated in a randomized, double-blind multi-centre study with a placebo preparation or with rIFN-gamma. We analysed parameters of neutrophil oxidative and non-oxidative metabolism in 16 patients enrolled in this study. No enhanced superoxide-release was observed in patients treated with rIFN-gamma compared to placebo-treated patients. Phagocyte cytochrome b558 content also remained unchanged. Levels of four non-oxidative antimicrobial proteins (cathepsin G, azurocidine, p29b, lactoferrin) rose, fell, or remained unchanged, irrespective of treatment with rIFN-gamma or placebo.
Blood Proteins↗
Inhibition of adhesion of S-fimbriated Escherichia coli to buccal epithelial cells by human milk fat globule membrane components: a novel aspect of the protective function of mucins in the nonimmunoglobulin fraction.
We investigated the presence of factors in human milk that inhibit invasion of pathogenic bacteria. The effect of human milk fat globule membrane (HMFGM) components on adhesion of cloned S-fimbriated Escherichia coli to human buccal epithelial cells was analyzed. S fimbriae are a common feature of E. coli strains causing sepsis and meningitis in newborns and are bound to epithelia via sialyl-(alpha-2-3)galactoside structures. Human milk fat globules (HMFG) could be agglutinated by the above-mentioned bacteria. Agglutination could be inhibited by fetuin, human glycophorin, and alpha 1-acid glycoprotein. In addition, pretreatment of HMFG with Vibrio cholerae neuraminidase markedly reduced bacterium-induced agglutinations, indicating the involvement of neuraminic acid-containing glycoproteins. In contrast, lipid droplets of infant formula or artificial lipid emulsions (Intralipid) could not be agglutinated. HMFG were present in stools of breast-fed neonates as shown by indirect immunofluorescence staining with a monoclonal antibody directed against carbohydrate residues present on HMFGM. These HMFG could be agglutinated by bacteria. HMFG inhibited E. coli adhesion to buccal epithelial cells. To further characterize relevant E. coli binding structures, HMFGM components were separated by gel chromatography. The mucin fraction showed the most pronounced inhibitory effect on adhesion of S-fimbriated E. coli to human buccal epithelial cells. Our data suggest that HMFG inhibit bacterial adhesion in the entire intestine and thereby may provide protection against bacterial infection.
Bacterial Adhesion↗
[Farquhar disease. Reduced phagocyte function in a 9-year-old girl with erythrophagocytic lymphohistiocytosis].
We report phagocyte function tests in a female infant with familial hemophagozytotic lymphohistiocytosis. Chemiluminescence and killing of Saccharomyces cerevisiae by monocytes and granulocytes before chemotherapy and in remission were examined. Patients' monocytes and granulocytes showed a markedly reduced chemiluminescence and killing capacity irrespective of the stage of disease.
Antineoplastic Combined Chemotherapy Protocols↗
Expansion of a maternally derived monoclonal T cell population with CD3+/CD8+/T cell receptor-gamma/delta+ phenotype in a child with severe combined immunodeficiency.
The diagnosis of severe combined immunodeficiency complicated by chronic graft-vs-host disease affecting liver and skin in association with engraftment of maternal T cells was established in a 5-mo-old boy. Detailed immunologic and molecular genetic studies were performed because a unique T cell phenotype was identified on initial evaluation. A major proportion of the patient's peripheral T cells expressed a CD8+ and TCR-gamma/delta+ phenotype while CD4+ T cells were virtually absent. Southern blot analysis of cell subpopulations isolated by fluorescence activated cell sorting indicated that approximately 50% of CD8+/TCR-gamma/delta+ cells were clonally related. Immunophenotyping and -genotyping also identified a clonal TCR-gamma/delta+ cell population in the child's mother. Clonal identity of these T cell populations in mother and child was demonstrated by studies using a clonspecific TCR-delta probe generated by polymerase chain reaction as well DNA sequence analysis. HLA typing and DNA fingerprinting confirmed that the child had acquired this clone diaplacentally from the mother. According to immunohistology and DNA analysis the clone was found to be virtually absent in the liver tissue suggesting that this clonal T cell population plays a minor role, if any, in the pathogenesis of the liver abnormalities in the patient. In the mother the CD8+/TCR-gamma/delta+ clone spontaneously declined to a level around 1% of PBMC several months later and has remained at this level since. We conclude that 1) a clonal expansion of TCR-gamma/delta T cells, triggered by yet unknown stimuli, may occur in otherwise healthy individuals, 2) respective T cells are able to cross the placental barrier, and 3) in an microenvironment precluding rejection, i.e., in severely immunocompromised patients, these cells may persist and even represent a significant proportion of circulating T cells.
Alleles↗
High-dose intravenous gammaglobulin in rhesus-haemolytic disease.
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Coombs Test↗
IgG2 deficiency in children with human immunodeficiency virus infection.
Infection with the human immunodeficiency virus (HIV) induces a polyclonal B-cell activation. Despite elevated serum immunoglobulin levels, a significant deterioration of the antigen specific humoral immune response exists in most cases. We studied the influence of HIV infection on the serum levels of IgG subclasses in children. We investigated 76 children (aged 15 months to 18 years) with HIV-1-infection. Most children (88%) showed elevated serum immunoglobulin levels. IgA (87%) and IgM (74%) were more often above normal levels for age than IgG (60%). IgG subclass serum levels were significantly altered. The increase in total IgG was mainly due to a marked augmentation of the IgG1 fraction. In most cases IgG3 was simultaneously elevated. Ten children (13%) had very low IgG4 levels (less than 0.03 g/l). Out of 61 patients older than 2 years 8 (13%) had a profound IgG2 deficiency with normal or elevated total IgG. Four of them also had low IgG4 levels (less than 0.03 g/l). A correlation between IgG2 deficiency and HIV infection according to the Centres for Disease Control classification for acquired immunodeficiency syndrome could not be demonstrated (three patients with symptomatic and five with asymptomatic infection).
Acquired Immunodeficiency Syndrome↗