concanavalin-a and Hypoparathyroidism

Five Persian Jews were detected with the polyglandular deficiency syndrome (PDS). Primary hypoparathyroidism and hypogonadism were present in each, adrenal insufficiency in two, and insulin-dependent diabetes mellitus and latent hypothyroidism in single subjects. The percentage of T and B cells, and the mononuclear cell response to phytohemagglutinin and Concanavalin A were normal in all five. IgG and IgA levels and the OKT4+/OKT8+ cell ratio were low in one subject. Antinuclear and antithyroid antibodies were present in one subject. HLA-DR5 was present in 4/4, HLA-24 and B5 (B51) in 3/4 subjects. A single case of isolated hypoparathyroidism (IHP) was detected among 12 first degree relatives. HLA antigens B8, DR3, were absent in all of these subjects. Seven non-Iranian Jews with IHP were also examined. HLA A26 or A25 were present in all seven. Persian Jews appear to have a unique variant of PDS.

Topics: Adolescent; Adrenal Insufficiency; Adult; Antibodies, Antinuclear; B-Lymphocytes; Concanavalin A; Diabetes Mellitus, Type 1; Female; HLA Antigens; Humans; Hypogonadism; Hypoparathyroidism; Hypothyroidism; Immunoglobulin A; Immunoglobulin G; Iran; Jews; Lymphocyte Activation; Male; Monocytes; Phytohemagglutinins; Syndrome; T-Lymphocytes

Four paediatrics patients affected by Chronic Mucocutaneous Candidiasis associated with hypoparathyroidism have been studied. In vivo and in vitro responses to Candida albicans antigens and to other common antigens (Dermatophytin T, Varidase), the percentage of T and B cells and the response to the selective polyclonal phytomitogens (PHA, ConA, PWM) have been investigated in order to quantitate and functionally characterize the B and T lymphocyte classes. The HLA A an B typing has been also performed. Our results show a remarkable and most likely primitive impairment of the T cells, unresponsive either to the C. albicans and the two other skin test antigens or to the selective polyclonal mitogens in vitro. The infectious disease (Mucocandidiasis) is most likely secondary to the T-cell failure. The association between the Mucocandidiasis, hypoparathyroidism and the T-cell deficiency is probably due to a common primitive disembryogenetic defect.

Topics: Antigens, Fungal; B-Lymphocytes; Candida albicans; Candidiasis; Candidiasis, Chronic Mucocutaneous; Child; Concanavalin A; Female; HLA Antigens; HLA-A Antigens; HLA-B Antigens; Humans; Hypoparathyroidism; Lymphocyte Activation; Male; Phytohemagglutinins; Pokeweed Mitogens; T-Lymphocytes