1/13. Autoimmune hemolytic anemia in a patient with autosomal dominant chronic mucocutaneous candidiasis.Chronic mucocutaneous candidiasis is a heterogeneous immunodeficiency syndrome characterized by recurrent candidal infections of the skin, nails, and mucous membranes. The syndrome can be associated with autoimmune conditions, especially endocrine disorders. Typically, inheritance is autosomal recessive, and abnormal T-cell-mediated immunity is thought to be the underlying deficit. We describe a 27-year-old man with chronic mucocutaneous candidiasis inherited in an autosomal dominant fashion, in whom both lymphocyte blastogenesis and delayed-type skin reactivity to Candida antigens were normal. Notable features of the case include autoimmune hemolytic anemia, probable hypoparathyroidism, and hypogonadal hypogonadism.- - - - - - - - - - ranking = 1keywords = antigen (Clic here for more details about this article) |
2/13. Distinct clinical phenotype and immunoreactivity in Japanese siblings with autoimmune polyglandular syndrome type 1 (APS-1) associated with compound heterozygous novel AIRE gene mutations.We herein report on two Japanese siblings with autoimmune polyglandular syndrome type 1 (APS-1). The brother, who expressed a characteristic phenotype of APS-1, had developed severe mucocutaneous candidiasis in early infancy and thereafter developed hypoparathyroidism and Addison's disease, along with a severe deterioration of his immunologic function. In contrast, the 44-year-old sister, who showed a noncharacteristic phenotype of APS-1, developed insulin-dependent diabetes with high anti-glutamic acid decarboxylase antibody, mild nail candidiasis, and autoimmune hepatitis with intact immunoreactivity. She had three susceptible human leukocyte antigen (HLA) loci for type 1 autoimmune diabetes. The expression of T cell receptor (TCR)V beta 5.1 increased in both patients, while the brother showed a widely suppressed expression of many TCRV beta families. Both individuals possessed compound heterozygous novel autoimmune regulator (AIRE) gene mutations (L29P and IVS9-1G > C). The same AIRE gene mutations can thus be associated with characteristic and noncharacteristic phenotypes of APS-1, and HLA may possibly influence the phenotype of APS-1.- - - - - - - - - - ranking = 1keywords = antigen (Clic here for more details about this article) |
3/13. 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 = 3keywords = antigen (Clic here for more details about this article) |
4/13. Chronic mucocutaneous candidiasis in a 6-year-old boy.Chronic mucocutaneous candidiasis (CMC) is a complex disorder characterized by persistent or recurrent candidal infections of the skin, nails and/or mucous membranes. A familial occurrence has been reported in some instances, suggesting a genetic predisposition. CMC has also been suggested to be associated with a selective defect in T cell-mediated immunity to Candida antigens. Reports of cases in Asians are rare. We report a case of CMC in a 6-year-old boy with chronic candidal infection since 7 months of age. The patient presented with deficient cell-mediated immunity and decreased natural killer cells. This case highlights the need for detailed studies for evaluating the T-cell immunity in patients with chronic candial infection.- - - - - - - - - - ranking = 1keywords = antigen (Clic here for more details about this article) |
5/13. Transient abrogation of immunosuppression in a patient with chronic mucocutaneous candidiasis following vaccination with candida albicans.A patient with long-standing chronic mucocutaneous candidiasis had reversed T helper/suppressor (TH/TS) cell ratios, hypergammaglobulinaemia E and serum inhibitors of lymphocyte transformation to mitogens and candida antigens. Following vaccination with whole heat-killed yeasts of candida albicans, temporary clinical improvement coincided with the return of the TH/TS cell ratio to normal, reduction in concentration of IgE and reduced serum inhibitory activity to concanavalin a and candida antigens. These changes were not permanent and 6 months after vaccination all indices had reverted to their pretreatment values. The production of antibody to a 47 kDa antigen of C. albicans has been shown to coincide with recovery from systemic disease. High concentrations of this antibody were demonstrated initially in the patient's serum and were unaffected by vaccination. If the 47 kDa antibody is protective, the results of this study suggest that separate immune responses may protect against mucocutaneous and systemic candidiasis and that defective immune responses against mucocutaneous disease need not imply lack of protection against systemic spread.- - - - - - - - - - ranking = 3keywords = antigen (Clic here for more details about this article) |
6/13. Suppressor T cells role in the unresponsiveness to candida albicans in chronic mucocutaneous candidiasis.We investigated the immune function of two members of the same family, mother and son, affected by chronic mucocutaneous candidiasis (CMC). In these cases CMC was not associated with hypofunction of endocrine organs, while the son showed histological feature of chronic active hepatitis. We observed a decrease in OKT4/OKT8 ratio and an increased suppressor cell function in both patients. Elimination of cytotoxic/suppressor T cells by lysis of these cells with OKT8 plus complement markedly improved lymphocyte proliferative responses to Candida antigens but not to unrelated antigens. These data suggest that specific suppression is probably responsible for failure of candida albicans clearance in CMC patients.- - - - - - - - - - ranking = 2keywords = antigen (Clic here for more details about this article) |
7/13. Severe aplastic anemia associated with chronic mucocutaneous candidiasis. Immunologic and hematologic reconstitution after allogeneic bone marrow transplantation.Chronic mucocutaneous candidiasis (CMC) is typically associated with the inability of T lymphocytes to proliferate and produce lymphokines in response to Candida antigen. A 7-year-old girl with CMC developed severe aplastic anemia and, after conditioning with cyclophosphamide, 200 mg/kg, underwent bone marrow transplantation from her HLA-identical sister. Engraftment was prompt and complete. The patient is surviving more than 3 years after transplantation with normal donor-derived hemopoiesis and immune function. Manifestations of CMC have resolved completely and she has not received antifungal therapy for more than 2 years.- - - - - - - - - - ranking = 1keywords = antigen (Clic here for more details about this article) |
8/13. Immunological observations before and after successful treatment of chronic mucocutaneous candidiasis with ketoconazole and transfer factor.A girl, 13 months of age, presented with generalised granulomatous skin, hair and mucosal candidiasis. Her lymphocytes failed to respond in vitro to Candida antigen (CA); the intradermal test with CA was also negative. serum immunoglobulins, complement components, granulocyte functions (phagocytic and fungicidal), T-cell subsets, mitogenic and allogenic lymphocyte stimulation, natural killer cell activity and immune interferon production were all found to be normal. No circulating immune complexes were detected. ketoconazole, an antimycotic drug, 5 mg/kg twice daily for 1 month and 2.5 mg/kg twice daily for another month spectacularly cleared all lesions. Afterwards, 4-monthly injections with transfer factor (TF) were given. Intradermal reactivity to CA was observed after the second TF injection. The lymphocyte responsiveness to CA in vitro became strongly positive 3 months after the last TF injection. The level of CA precipitins in serum, which was very high (11 lines) before ketoconazole treatment, decreased to 4 lines. No serum inhibitor of lymphocyte proliferation to CA could be demonstrated in the patient's serum before or after treatment. This specific CA unresponsiveness was not due to an excess of OKT8 (suppressor) cells; macrophage migration inhibiting factor (MIF) production was normal. The nonresponsiveness might be due to antigenic overload or to suppressor cell induction not demonstrable in the present studies. The child has remained free of lesions during 3 years of follow-up without any further treatment.- - - - - - - - - - ranking = 2keywords = antigen (Clic here for more details about this article) |
9/13. Chronic mucocutaneous candidiasis with iga deficiency in a two-year-old African girl who responded well to ketoconazole.The rare disorder of chronic mucocutaneous candidiasis is described in a two-year-old African girl who had a moderate lymphopenia due to fewer T and B cells, impaired lymphocyte transformation to PHA and candida antigen, decreased leucocyte migration inhibition to candida antigen and a defective cutaneous delayed hypersensitivity reaction to candida antigen and dinitrochlorobenzene. The patient's serum had a marginal inhibitory effect on the transformation of normal lymphocytes to candida antigen and no effect on PHA stimulation of these cells, as measured by stimulation index. In addition, IgA was deficient in her serum. No associated endocrinopathy was detected and she had a mild iron deficiency anaemia. There was a rapid and excellent response to ketoconazole with all lesions clearing within two months of treatment.- - - - - - - - - - ranking = 4keywords = antigen (Clic here for more details about this article) |
10/13. Chronic mucocutaneous candidosis and other superficial and systemic mycoses successfully treated with ketoconazole.Four patients with chronic mucocutaneous candidosis from early infancy were treated successfully with ketoconazole given orally. All thrush lesions were clinically and mycologically cured within a few days of treatment with 100-400 micrograms of ketoconazole daily; skin lesions were cured within a few weeks, and nails were cured after about three months of treatment. Delayed cutaneous hypersensitivity to candidin was acquired by the third month. Cellular and humoral immunologic responses were related to the suppression of candida albicans antigen by ketoconazole. A fifth patient with chronic lingual granuloma due to C. albicans improved considerably. Favorable results also were seen in individual patients with oral and disseminated histoplasmosis due to histoplasma capsulatum; laryngeal, pulmonary, and hepatic disease with continuous fever also due to H. capsulatum; pulmonary histoplasmosis due to histoplasma duboisii; cutaneous sporotrichosis; and cutaneous blastomycosis due to blastomyces dermatitidis and in three patients with favus due to trichophyton schoenleinii; six of seven patients with tinea capitis due to trichophyton violaceum (after one month of treatment); and four patients with infections due to Petriellidium boydii, phialophora pedrosoi, or beauveria species. All patients responded rapidly to 400 mg of ketoconazole per day given orally. Only the patient with hepatic histoplasmosis required 800 mg per day. Measurements of ketoconazole in the serum during treatment were useful in the evaluation of therapy.- - - - - - - - - - ranking = 1keywords = antigen (Clic here for more details about this article) |
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