This disorder is caused by an accumulation of phospholipid in RES cells. It is most common in children of European Jewish descent. It is characterised by marrow expansion and cortical thinning in the long bones plus coxa valga. It is most common in children of Mediterranean descent. Symptoms are similar to some in Sickle Cell Disease including severe bone pain.
Leg ulceration is common.
Radiographs are needed to pick up developing osteoporosis and distorted trabeculae. The incidence of children born with AIDS has increased dramatically. Afflicted children require ongoing medical support. Protection for surgeons is essential.