Immunoglobulin (Ig) A deficiency has long been recognized in patients with chromosome 18 abnormalities. We present the case of a young girl in whom a chromosome 18p deletion syndrome (46,XX,del[18][p11.1]) was associated not only with IgA deficiency, but also with an inability to make antibody to the unconjugated pneumococcal polysaccharide vaccine, Pneumovax II, indicating a concomitant specif...

PURPOSE/OBJECTIVES To review major clinical applications of immune globulin IV (IGIV) therapy, properties of currently available IGIV preparations, procedures for dosing and administration, management of infusion-related adverse effects, and strategies for effective patient education. DATA SOURCES Published articles, abstracts, and textbook chapters. DATA SYNTHESIS IGIV therapy supports imm...

RecQ family helicases play important roles at G-rich domains of the genome, including the telomeres, rDNA, and immunoglobulin switch regions. This appears to reflect the unusual ability of enzymes in this family to unwind G4 DNA. How RecQ family helicases recognize this substrate has not been established. Here, we show that G4 DNA is a preferred target for BLM helicase within the context of lon...

Three generations of relatives of 58-year-old nonidentical twins with chronic bronchitis and fibrotic lung disease were evaluated. Sera of 23 family members, 14 with a history of excessive sinopulmonary infections, were examined for deficiencies of immunoglobulin classes, IgG subclasses, and specific antibody to tetanus toxoid and Hemophilus influenzae type b. Of 14 symptomatic family members, ...

patients  with  primary  antibody  deficiencies  (pad)  are  susceptible  to  recurrent  and chronic infections and a variety of complications. this study was performed to assess quality of life (qol) of pad patients who were under long term treatment and regular follow-up.thirty six adults with proved diagnosis of pad, who had received regular intravenous immunoglobulin replacement therapy, we...