1/10. 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) |
2/10. Chron's disease, rare association with selective IgA immunodeficiency, and development of life-threatening bacterial infections.life-threatening necrotizing fasciitis and relapsing lemierre syndrome associated with fusobacterium necrophorum septicaemia occurred in young adults with a moderate Chron's disease and a missed profound iga deficiency. This unexpected association of a chronic bowel inflammatory syndrome with prominent IgA abnormalities and severe bacterial infection deserves careful attention by physicians faced with young patients with Chron's disease.- - - - - - - - - - ranking = 5keywords = bacterial infection (Clic here for more details about this article) |
3/10. Transient hypogammaglobulinemia of infancy with severe bacterial infections and persistent iga deficiency.A 1-year-old boy who had had recurrent episodes of sepsis was found to have transient hypogammaglobulinemia of infancy and was treated with gammaglobulin supplements. He subsequently remained IgA deficient and a regulatory T cell imbalance was found.- - - - - - - - - - ranking = 4keywords = bacterial infection (Clic here for more details about this article) |
4/10. 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) |
5/10. malacoplakia associated with vesicoureteral reflux and selective immunoglobulin a deficiency.A case of malacoplakia involving the lower urinary tract of a young black boy, with associated bilateral vesicoureteral reflux, hydronephrosis and selective immunoglobulin a deficiency is reported. Reflux was caused by the malacoplakia. Reflux and hydronephrosis persisted despite elimination of bacterial infection and malacoplakia by drug therapy. These abnormalities were corrected by a conventional antireflux operation. malacoplakia appears to be related to immunologic incompetence and diminished levels of intracellular cyclic 3',5' guanine monophosphate. cholinergic agonists reverse or prevent the pathological changes of malacoplakia.- - - - - - - - - - ranking = 1keywords = bacterial infection (Clic here for more details about this article) |
6/10. 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) |
7/10. A case of protein-losing enteropathy in idiopathic thrombocytopenic purpura with decreased IgA.A young woman presented with high fever and edema in January, 1984, and was diagnosed as having systemic lupus erythematosus. prednisolone administration failed to improve her symptoms. In May she was admitted to hospital because of elevated erythrocyte sedimentation rate (ESR), hypoproteinemia, hypogammaglobulinemia, hypocomplementemia, positive antinuclear antibody, elevated immune complex level, and diarrhea. edema disappeared following administration of diuretics and albumin, although the pathogenesis was still undetermined. In September, she was referred to our institution because of severe watery diarrhea and hypoproteinemia. Endoscopic examination showed a diffuse inflammatory lesion in the duodenum and the colon. Radioisotopic 51Cr-albumin study results were compatible with protein-losing enteropathy. hypoproteinemia and inflammatory changes of the intestine were improved by antibiotics, suggesting that the inflammatory lesion was caused by bacterial infection. Despite the improvements in clinical symptoms and laboratory findings, the serum IgA level was still low and the thrombocytopenia remained. The morphological characteristics of the megakaryocytes were consistent with idiopathic thrombocytopenic purpura. In May, 1986, the thrombocytopenia deteriorated, causing purpura. prednisolone was administered again, and this resulted in normalization of the platelet count, although the IgA level remained low. Finally the prednisolone was stopped, and the IgA level gradually recovered, with the improvement of the enterocolitis. The exact pathogenesis of the whole picture in this case is unclear, but an 8-year-long clinical course suggests that the protein-losing was caused by an infectious enterocolitis superimposed on iga deficiency.- - - - - - - - - - ranking = 1keywords = bacterial infection (Clic here for more details about this article) |
8/10. 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) |
9/10. Abdominal T-cell non-Hodgkin's lymphoma of the gamma/delta type in a patient with selective immunoglobulin a deficiency.A 28-year-old man presented with selective immunoglobulin a deficiency and severe diarrhea responding to a gliadin-free diet. biopsy samples of the small intestine showed dense T-cell infiltrations in the lamina propria and a slight increase of intraepithelial t-lymphocytes. No clonal rearrangement of the T-cell receptor c-beta chain genes was detectable by Southern blotting. Four years later, at the age of 32, the patient was hospitalized again with liver failure, abdominal lymphadenopathy, pancytopenia, and recurrent bacterial infections. Retrospective polymerase chain reaction analysis of formalin-fixed tissues of the intestinal biopsy samples obtained 4 years earlier showed monoclonal T-cell receptor gamma-chain gene rearrangement. Lymphoid cells of the peripheral blood showed an immunophenotype of CD3-positive gamma/delta T cells with a negativity for CD4 and CD8. A clonally rearranged T-cell receptor delta chain gene and a germline configuration of the c-beta chain genes was found by Southern blotting. cytogenetics showed an abnormal karyotype with unbalanced translocations t(1;5) and t(9;13). The patient died of extensive lung infiltrations by gamma/delta T cells; autopsy showed a peripheral T-cell lymphoma of the gamma/delta type in the enlarged abdominal lymph nodes. This is the first report of an abdominal T-cell lymphoma of the gamma/delta type in a patient with selective immunoglobulin a deficiency.- - - - - - - - - - ranking = 1keywords = bacterial infection (Clic here for more details about this article) |
10/10. Type A immunoglobulin deficiency presenting as a mixed polymicrobial brain abscess: case report.OBJECTIVE AND IMPORTANCE: We present a case report of a patient with a left frontal brain abscess. Cultures obtained from the abscess at the time of surgery were identified as dental flora known to establish a synergistic relationship in polymicrobial infections. This type of synergistic relationship makes the clearance of an infection more difficult for an intact immune system. A serum immunoglobulin (Ig) Type A deficiency was identified postoperatively. This immunodeficiency may have contributed to the development of the abscess. CLINICAL PRESENTATION: The patient presented with headaches and photophobia. Computed tomography of the head performed with intravenously administered contrast demonstrated a left frontal brain abscess. INTERVENTION: The patient was operated on through a left frontal approach, carefully avoiding the frontal sinus. The abscess was aspirated, and the patient was treated with intravenous antibiotics for several weeks. Postoperatively, the patient did well. There were no signs of enhancement on follow-up computed tomographic scans at 7 and 12 months postoperatively. CONCLUSION: Through a comprehensive immunological workup, an iga deficiency was identified postoperatively. Although the deficiency of a single type of Ig may be asymptomatic, complications from recurrent or chronic bacterial infections may occur. The deficiency of IgA, combined with a synergistic polymicrobial infection, contributed to the development of an intracranial abscess. A patient presenting with a brain abscess without any predisposing medical history should be evaluated for an underlying immune deficiency.- - - - - - - - - - ranking = 1keywords = bacterial infection (Clic here for more details about this article) |
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