CONGENITAL ABSENCE OF THE SPLEEN

[摘要] A case is reported of congenital absence of the spleen in a 15-month-old Negro female infant. Her condition was uncomplicated by other congenital abnormalities, and represents the second such case in a child to appear in the literature. Most of the other reported instances of splenic agenesis are associated with congenital cardiac anomalies, partial situs inversus, accessory lobe of the left lung, and abnormal mesenteric attachments, forming a syndrome of anomalies. These probably arise from factors affecting the fetus at the 5-week stage of development. Some patients have hematologic findings similar to patients post splenectomy.This child had a course marked by lack of resistance to infection, and leukocyte counts so high as to simulate leukemia. She finally expired, with pneumonia and Waterhouse-Friderichsen syndrome.

[学科分类] 儿科学