The sickle cell diseases. Clinical manifestations including the "sickle crisis"

Working definitions of "sickle cell disease," "sickle-cell trait," and "the sickle crisis" are given. The pathogenesis of the crisis is explained and the complications of the disease are presented in a quantitative manner. The need to look at the patient as a whole and to assess the natural history of the disease against the background of an often prevalent social and environmental pathologic condition is stressed. A call for effective public health measures, including raising of living standards and providing comprehensive health care facilities for sickle cell disease patients is made.