11/62. 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, infection (Clic here for more details about this article) |
12/62. iga deficiency with membranous glomerulonephritis: a case report and review.Selective immunoglobulin a (IgA) deficiency may result in a predisposition to recurrent sinopulmonary infection and allergic diseases. iga deficiency may also play a role in the development of autoimmune disorders. Selective iga deficiency associated with glomerulonephritis was rare, while the clinical presentation in iga deficiency-associated glomerulonephritis was variable. We report an 83 year-old male with selective iga deficiency associated with membranous glomerulonephritis. He presented with nephrotic syndrome. Percutaneous renal needle biopsy showed diffuse global thickening and rigidity of glomerular capillary walls, mildly diffuse segmental expansion of mesangial matrix, focal and cortical scar with segmental obsolescence of glomeruli. Heavy IgG and moderate C3 deposits were found on immunofluorescence. We also review the previous cases of iga deficiency with glomerulonephritis. Several clues were rendered to establish the association between iga deficiency and membranous glomerulonephritis.- - - - - - - - - - ranking = 0.017078035804561keywords = infection (Clic here for more details about this article) |
13/62. Antibody deficiency with hyper IgM--a case report.A 20-month-old Indian boy presented with recurrent pyogenic infections and failure to thrive. His IgG and IgA levels were low, but his IgM was elevated. He also had undetectable isohaemagglutinin titre and neutropenia, both parameters being poor prognostic indicators in this very rare primary immunodeficiency state--antibody deficiency with hyper IgM. Our patient subsequently succumbed to pseudomonas aeruginosa septicaemia and meningitis inspite of aggressive antibiotic and intravenous gammaglobulin therapy. To the best of our knowledge, this is the first such case to be documented in malaysia.- - - - - - - - - - ranking = 0.017078035804561keywords = infection (Clic here for more details about this article) |
14/62. Isolated iga deficiency after chemotherapy for acute myelogenous leukemia in an infant.iga deficiency is a relatively common congenital immunodeficiency in children. It can either be asymptomatic or lead to frequent infections, most often of the sinuses and lungs. Intensive chemotherapy for acute leukemia is also profoundly immunosuppressive and can be complicated with life-threatening infections, usually associated with neutropenia and prolonged lymphopenia in the post-bone marrow transplant setting. Isolated, acquired immunoglobulin deficiency that occurs during treatment has been described but is usually transient. In this report, the authors describe a patient with infant acute myelogenous leukemia with acquired, persistent iga deficiency.- - - - - - - - - - ranking = 0.034156071609122keywords = infection (Clic here for more details about this article) |
15/62. Recurrent parotitis in selective iga deficiency.Recurrent parotitis is an uncommon condition in children. In most cases the etiology is unknown, although the disease is occasionally associated with viral infections, autoimmune disorders and immunodeficiency. We describe, for the first time, a child with recurrent parotitis and isolated immunoglobulin a (IgA) deficiency, without autoimmune disease. As IgA is the main immunoglobulin secreted into the mucosal surfaces, including that of the respiratory and gastrointestinal tracts, and into the saliva, the lack of IgA may be involved in the pathogenesis of recurrent parotitis. We recommend that IgA and other immunoglobulins be tested in all cases of recurrent parotitis.- - - - - - - - - - ranking = 0.017078035804561keywords = infection (Clic here for more details about this article) |
16/62. 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 = 4.8292196419544keywords = bacterial infection, infection (Clic here for more details about this article) |
17/62. Unusual absence of neurologic symptoms in a six-year old girl with ataxia-telangiectasia.Ataxia-telangiectasia (A-T) is a rare multisystem, neurodegenerative genetic disorder. We present a case of a 6-year-old girl who had a history of frequent respiratory infections and also had ocular and immunological features of this syndrome. The absence of neurological symptoms, which is very unusual for a patient of this age, raised many difficulties in the diagnosis of the disease. It is concluded that a normal neurological assessment must not exclude the diagnosis of A-T and delay the proper interventional measures.- - - - - - - - - - ranking = 0.017078035804561keywords = infection (Clic here for more details about this article) |
18/62. Different clinical and laboratory evolutions in ataxia-telangiectasia syndrome: report of four cases.We report four patients with ataxia-telangiectasia syndrome that presented varied neurologic evolution. Three patients initially presented neurologic alterations of slow progression, evolving to late immunocompromised conditions. The fourth patient presented, from symptom onset, immune and neurologic debilitation, that were both severe and of fast progression. The chronological sequence of the most commonly observed immunocompromised conditions were in our patients, in ascending order, iga deficiency, IgG2 deficiency and the neutrophil phagocytosis stage and common variable immunodeficiency. The first two reports are of sisters in whom the diagnosis was done between the ages of three and six years, having ocular apraxia, cerebellar ataxia and telangiectasia. Slow progression of neurologic debilitation was observed, without presentation of intermittent infections. The patients began presenting accentuated immunocompromised conditions at the ages of 14 and 17 years, dying at the ages of 16 and 20 years, respectively, due to severe infections that were resistant to treatment. The diagnosis of the third case was established when the patient was two years old, presenting ataxia and telangiectasia. Syndrome progression was slow, presenting at the age of eight years more accentuated neurologic disorders and iga deficiency. The fourth case presented significant neurologic compromise at the age of five, simultaneous to IgA and IgG2 deficiency, and repeating pneumonias and sinusitis. At this time, intravenous gammaglobulin reposition was done. The neurologic and immune disorders progressed rapidly, and at the age of eight presented the inability to walk. At this time inversion of the CD4/CD8 ration was verified through laboratory tests.- - - - - - - - - - ranking = 0.034156071609122keywords = infection (Clic here for more details about this article) |
19/62. Recurrent acute postinfectious glomerulonephritis.Recurrent acute postinfectious glomerulonephritis is infrequent in childhood and exceptional in adults. The factors that determine recurrence are poorly understood. Selective iga deficiency is characterized by an increased incidence of gastrointestinal and respiratory infections. The case of a 33-year-old man with a history of repetitive sinopulmonary infections and diagnosed with selective iga deficiency is described. He suffered 2 episodes of postinfectious glomerulonephritis within a 15-year period. Selective iga deficiency may have predisposed to the development of recurrent postinfectious glomerulonephritis- - - - - - - - - - ranking = 0.034156071609122keywords = infection (Clic here for more details about this article) |
20/62. Protracted enteric cryptosporidial infection in selective immunoglobulin a and saccharomyces opsonin deficiencies.Chronic cryptosporidial infection in man usually occurs in those who are immunocompromised. We report a patient with a one year history of bowel symptoms resulting from persistent cryptosporidial infection of the colon. Investigations showed underlying selective IgA and saccharomyces opsonin deficiencies but no evidence of cell mediated immune dysfunction. Both selective immunoglobulin a and opsonin deficiencies are relatively common in the general population and may be a cause of susceptibility to persistent cryptosporidial infection.- - - - - - - - - - ranking = 0.11954625063193keywords = infection (Clic here for more details about this article) |
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