Acute splenic sequestration is a potential source of morbidity and mortality early in life for children with sickle cell anemia and at any age for those with Hb SC disease and sickle thalassemia. These episodes are characterized by rapid increase in splenic size and decrease in hemoglobin. Treatment includes support and aggressive transfusion with splenectomy for repeated or resistant cases. Strokes also occur in early childhood. Transcranial Doppler is being explored as a way of identifying infants at high risk for stroke so that primary prevention may be accomplished.
Gallstones occur in children and adults with increasing frequency with age.
Symptomatic gall stones are an indication for cholecystectomy. There is controversy about the management of asymptomatic gall stones. Acute chest episodes characterized by chest pain, fever, and infiltrates occur at any age. Recent studies suggest that infection, fat embolization, and primary infarction may all lead to this clinical problem. Episodes may be severe, life-threatening events and recurrent episodes may result in chronic lung disease and pulmonary hypertension.