BONE DISORDERS IN SICKLE-CELL DISEASE

Sickle cell disease involves many organs but musculo-skeletal problems present most often with bone pain, the most common reason for admissi on to hospital. Two separate pathological abnormalities cause these le sions. Sickling of the red cells produces thromboembolic infarcts in b one leading to pain, crises and sometimes osteomyelitis; increased des truction of sickle red cells produces haemolysis, an increase in eryth roblastic activity and expansion of the bone marrow cavity. Dactylitis , avascular necrosis of the head of the femur particularly, osteomyeli tis, retardation of growth and leg ulcers are commonly encountered Man agement is by standard orthopaedic principals. At operation care must be taken in the use of a tourniquet, adequate oxygenation is required and the possibility of acute renal failure must be recognised.