1/6. IgG2 immunodeficiency: association to pediatric patients with bacterial meningoencephalitis.An IgG subclass deficiency is often associated with bacterial infections. We studied four pediatric patients suffering from meningoencephalitis, two of them due to streptococcus pneumoniae and two due to haemophilus influenzae type b. Simultaneous diagnostic serum and cerebrospinal fluid samples were taken during income. The four subclasses of IgG and albumin were quantified in both biologic fluids by radial immunodiffusion. Very low levels of seric IgG2 with non detectable cerebrospinal fluid IgG2 were found in the patients. No intrathecal IgG subclass synthesis was found in two patients. One patient with S. pneumoniae had IgG3 intrathecal synthesis. Intrathecal IgG1, IgG3 and IgG4 synthesis was found in one patient suffering from H. influenzae according with reibergrams. Substitutive therapy with intravenous gammaglobulin was given to the patients as part of the treatment.- - - - - - - - - - ranking = 1keywords = bacterial infection (Clic here for more details about this article) |
2/6. The syndrome of chronic mucocutaneous candidiasis with selective antibody deficiency.BACKGROUND: Most patients with chronic mucocutaneous candidiasis (CMC) have a selective defect of cell-mediated immunity against candida albicans (as demonstrated by cutaneous anergy and decreased lymphoproliferative responses to Candida antigen) and intact antibody responses. Many CMC patients also develop infections with other organisms, suggesting a more extensive immunologic defect. OBJECTIVES: The aim of this study was to describe a patient with CMC and selective antibody deficiency and identify eight similar previously reported patients. DATA SOURCES: Relevant articles in the English language derived from searching the medline database were used. RESULTS: We describe an 18-year-old male patient who was identified with CMC as an infant and later developed immunoglobulin (Ig)G2, IgG4, and iga deficiency at age 12 associated with poor antibody responses to vaccine antigens. We have identified eight other previously reported CMC patients with selective antibody deficiencies and bacterial infections. IgG2 deficiency was present in all nine patients, and was associated with IgG4 deficiency in 8 patients and iga deficiency in 3 patients. Six patients had poor or absent antibody responses to pneumococcal polysaccharide vaccine, and all nine patients developed severe recurrent lung infections. CONCLUSIONS: We suggest that these cases represent a distinct phenotype of CMC and should be studied for common histocompatibility leukocyte antigen types and molecular defects.- - - - - - - - - - ranking = 1keywords = bacterial infection (Clic here for more details about this article) |
3/6. Susceptibility to infections in children with selective IgA- and IgA-IgG subclass deficiency.This study included 36 children with IgA-deficiency, increased susceptibility to infections and/or other disorders. Recurrent, usually bacterial infections were noticed in 23 out of 26 patients (88%) with complete and in 7 out of 10 patients (70%) with partial IgA-deficiency. All patients with severe infections had complete IgA-deficiency. Complete IgA-deficiency was also present in the six children who had autoimmune disorders associated with recurrent infections. In 22 out of the 36 patients studied the serum could be analysed for concomitant IgG subclass deficiencies: one patient had marked decrease of IgG2. In a second patient IgG4 was not detectable. Two patients had combined IgG2-IgG4-deficiency. In a girl with severe acute and chronic infections and relapsing idiopathic thrombocytopenic purpura, IgA-IgG2-IgG4-deficiency was found to be the prodromal stage of common variable immunodeficiency with panhypogammaglobulinaemia.- - - - - - - - - - ranking = 1keywords = bacterial infection (Clic here for more details about this article) |
4/6. Lack of IgG in a healthy adult: a rare case of dysgammaglobulinemia with undetectable serum IgG, IgA2, and IgE.No IgG of any subclass could be detected in the serum of a normal healthy male adult (E.E.) who had no recent history of repeated infections. Markedly increased concentrations of both IgM and IgA were present although no IgA2 nor IgE could be demonstrated. No anti-immunoglobulin antibodies were present in the serum; when used as supplement in in vitro cultures, the serum supported differentiation of IgG-producing cells to an equal extent as that of normal serum. No cells with surface or intracellular IgG were found in the peripheral blood although low numbers of IgG-secreting cells could be induced in vitro after mitogen stimulation. No increase in suppressor cell activity was found in cocultivation experiments. T-Cell-enriched populations from E.E. were poor inducers of immunoglobulin synthesis in allogeneic B cells, and B-cell-enriched populations from E.E. could not be triggered to synthesize IgG with the aid of allogeneic T cells. Thus, it seems that lack of IgG does not necessarily lead to a highly increased susceptibility to bacterial infections.- - - - - - - - - - ranking = 1keywords = bacterial infection (Clic here for more details about this article) |
5/6. Inherited deficiency of the third component of complement associated with recurrent pyogenic infections, circulating immune complexes, and vasculitis in a Dutch family.A family is described in which 3/11 children showed a homozygous deficiency of C3, and both parents and six other children had subnormal levels of C3. The three children with selective C3 deficiency suffered repeatedly from bacterial infections, whereas the parents and the other siblings were clinically healthy. During infectious episodes the patients showed a maculopapular skin rash, and at such times immune complexes were present in the serum. biopsy specimens of the skin lesions showed the picture of leukocytoclastic vasculitis.- - - - - - - - - - ranking = 1keywords = bacterial infection (Clic here for more details about this article) |
6/6. An infant with both autoimmune neutropenia and idiopathic thrombocytopenia with IgG2/iga deficiency.We report an infant with autoimmune neutropenia (AIN), idiopathic thrombocytopenia (ITP), and IgG2/iga deficiency. The patient was referred to our hospital at 5 months of age because of epistaxis and generalized petechiae. physical examination revealed moderate hepatosplenomegaly. A complete blood count revealed a platelet count of 2.0 x 10(3) cells/microliters, and a white cell count of 3,600 cells/microliters, with severe neutropenia (less than 1% bands and segmented cells). neutrophils and platelets adhering to megakaryocytes were decreased in the bone marrow. Tests for serum neutrophil-binding IgG (NB-IgG) and platelet-associated IgG (PA-IgG) were positive. A diagnosis of both AIN and ITP was made and therapy with intact-type gamma-globulin and prednisolone was initiated. Improvement occurred, but was temporary. A lack of serum IgA and IgG2 was noted during the clinical course. The patient has not been susceptible to bacterial infections but has had a severe clinical course with rubella and chickenpox.- - - - - - - - - - ranking = 1keywords = bacterial infection (Clic here for more details about this article) |