Studies in sickle-cell anemia. XXXIV. Observations on enuresis in childhood and nocturia in adults.

Abstract

T I-I I s R E P o R T is a clinical analysis of 10 children who died suddenly during severe crisis. The clinical manifestations and postmortem findings will be cited and the mechanism of sudden death in uncomplicated sickle cell disease will be discussed. Tomlinson 1 in 1945 called attention to a striking clinical picture of abdominal crises terminating fatally in patients with sickle cell anemia. After reviewing a seri~s of 11 such patients, 10 of whom were children, he attributed the deaths to sudden massive hemolysis of sickled red blood cells, clinical shock with constricted peripheral vasculature, congested viscera, and cerebral edema. Four years later Raper ~ described sudden death resulting from multiple capillary blockage by sickled cells in 4 East African children and 1 adult. We feel that it is timely to re-emphasize the vulnerability of the very young child with sickle cell anemia to severe crises which may terminate in early death. Six of the in1 fants in this report were under 20 months of age at the time of their las t hospitalization. The deaths of 6 children in this series were revealed during a comprehensive analy-