Abstract
The one boy and two of three girls in a consanguineous kindred have exhibited undue susceptibility to invasive infections with Hemophilus influenzae and pneumococci. One of the affected siblings died of overwhelming H. influenzae type B sepsis and was found at autopsy to have a minute spleen. No other anatomic abnormalities were present. The two affected live siblings were each shown to have no demonstrable splenic tissue by scintillation scanning of the abdomen following intravenous injection of colloidal Au198. Normal splenic tissue was demonstrable in both parents by this tecnique. Examination of the peripheral blood of affected offspring revealed the presence of Heinz and Howell-Jolly bodies. The antibody response to subcutaneous injection of tetanus and diphtheria toxoids and typhoid bacilli was normal. Their red cell survival and response to intravenous particulate antigens are under investigation and the results will be reported. (SPR)
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