Cholycystectomy in a child with sickle cell disease

Introduction Sickle cell disease (SCD) describes a group of disorders associated with chronic haemolytic anaemia, acute vaso-occlusive crisis (leading to progressive end-organ damage) and infection. It is due to the inheritance of the sickle β haemoglobin gene which results in a single point mutation on the β globin chain. When deoxygenated, sickle haemoglobin (HbS) becomes insoluble and if present in sufficient quantity, will polymerise to form rigid fibres. The red cells become distorted and less deformable (typically sickle-shaped cells). Sickle cells have reduced survival and are responsible for the vaso-occlusion which is the hallmark of the disease.