ABSTRACT
Cartilage hair hypoplasia is a rare autosomal recessive form of short-limbed dwarfism associated with a cellular immunodeficiency. In eight patients, the authors studied the presence of T cell subsets and in vitro T cell function in order to address the basis for the immunological disorder. Both the proliferative response to phytohaemagglutinin (PHA) and the PHA-induced IL2 production were 60% lower compared with controls (P = 0.007 and 0.005, respectively). The impaired proliferative response could not be restored by addition of IL-2. This result is in accordance with a decrease in the percentage of activated T cells expressing the p55 subunit of the IL-2 receptor complex (CD25). The results define more precisely that T cells from cartilage hair hypoplasia patients are defective in the transition from the G0 to the G1 phase of the cell cycle. Furthermore, the data demonstrate that several CHH patients show a reduced proportion of CD45RA+ 'naive' T cells. However, the in vitro impairment of T cell function cannot solely be explained by imbalance between 'naive' and 'memory' T cells. Although CHH patients with a history of recurrent respiratory tract infections showed the most aberrant in vitro immune parameters, a clear relationship between clinical data and in vitro parameters could not be established for the whole patient group.
Subject(s)
CD4-Positive T-Lymphocytes/immunology , CD8-Positive T-Lymphocytes/immunology , Osteochondrodysplasias/immunology , Adult , Child , DNA/biosynthesis , DNA Replication , Female , Humans , Interleukin-2/metabolism , Lectins , Leukocyte Common Antigens/metabolism , Lymphocyte Activation/immunology , Male , Middle Aged , Receptors, Interleukin-2/metabolism , Receptors, Transferrin/metabolism , T-Lymphocyte Subsets/immunologyABSTRACT
Immunologic studies of 14 girls with Turner syndrome were done before and during treatment with biosynthetic growth hormone (GH). Compared with control subjects, the patients before treatment had a decreased CD4/CD8 ratio and an increased number of cells bearing the natural killer cell marker CD16; serum immunoglobulin levels were within the normal range. During GH treatment some of the girls had a slight reduction in the percentage of CD20+ B cells, but we observed no impairment of B lymphocyte function as demonstrated by the normal in vivo antibody response to the primary antigen Helix Pomatia hemocyanin, administered 6 months after the start of GH treatment. The number of CD16+ natural killer cells returned to normal. Although the number of children with thyroid antibodies increased from two before treatment to five after 1 year, no conclusion about an adverse effect of GH is warranted, because the phenomenon might be part of the natural course of the disease. We conclude that girls with Turner syndrome have minor changes in some immunologic measurements and that GH treatment resulted in some alterations that have no effect on immune function.
Subject(s)
Growth Hormone/therapeutic use , Turner Syndrome/immunology , Adolescent , Antibody Formation/drug effects , Antibody Formation/immunology , Child , Female , Growth Hormone/adverse effects , Humans , Immunity, Cellular/drug effects , Immunity, Cellular/immunology , Leukocyte Count/drug effects , Lymphocyte Subsets/drug effects , Lymphocyte Subsets/immunology , Time Factors , Turner Syndrome/drug therapyABSTRACT
The levels of pertussis-specific IgA antibodies in sera from vaccinees and from children with Bordetella pertussis infection were compared by an enzyme-linked immunosorbent assay (ELISA). Serum IgA antibodies were produced only after natural contact with the pathogen and, therefore, their presence can be used as an indicator of infection. However, in view of the relatively long interval between infection and the appearance of antibodies, and the prolonged antibody response, their presence cannot be used as proof of recent infection. The finding of these antibodies in a high percentage of the normal adult population may indicate a constant circulation of B. pertussis without symptoms of disease.
Subject(s)
Antibodies, Bacterial/analysis , Bordetella pertussis/immunology , Immunoglobulin A/analysis , Whooping Cough/immunology , Adolescent , Adult , Aged , Antibody Formation , Child , Child, Preschool , Enzyme-Linked Immunosorbent Assay , Female , Humans , Infant , Male , Middle Aged , Netherlands , Species Specificity , VaccinationABSTRACT
The hepatic uptake and biliary excretion of 3H-taurocholate and 3H-ouabain was studied in the rat during saline (control) and dehydrocholate infusions. Dehydrocholate (140 mumol/hr) did not influence the plasma disappearance nor the biliary excretion of taurocholate after a single iv injection (37 mumol/kg). Bile production in the dehydrocholate experiment was increased 2- to 3-fold compared with controls. The biliary transport maximum for exogenously administered taurocholate was determined by constant infusion to be 135.0 +/- 3.0 mumol/hr (22 mumol/min/g of liver). Concomitant infusions of 140 mumol of dehydrocholate per hr did not alter the maximal taurocholate output. The effects of the two bile salts on bile flow were additive. Dehydrocholate (140 mumol/hr) reduced the biliary excretion of 3H-ouabain (0.8 mumol/kg) and elevated the secondary slow component of the plasma disappearance of the cardiac glycoside. The hepatic levels of ouabain were increased compared with controls. It is concluded that dehydrocholate interferes with ouabain transport at the canalicular level but not with primary hepatic uptake. Taurocholate (140 mumol/hr) failed to influence the total biliary output of ouabain. These differences and the lack of interaction between dehydrocholate and taurocholate suggest a hepatic transporting pathway for taurocholate which differs from that for taurocholate which differs from that for dehydrocholate and/or its metabolites.