Regarding sickle cell disease,
1. The most common cause of death is stroke
2. Intracranial anuesysms associated with sickle cell disease are usually single
3. Moya moya is characterictic of sickle cell disease
4. "hair-on-end"appearance on the skull radiograph is characteristic feature of sickle cell disease
5. The most common cause of osteomyelitis is salmonella
Answers: F, F, F, F, T
Notes:
The most common cause of death in sickle cell disease (SCD) is pulmonary complications. Children with SCD are 100 times more risk for pneumonias. Acute chest syndrome is more lethal in adults than children. The most common complication in brain are stroke, ischemia and atrophy. 11% have stroke by the age of 20 years. Infarcts are common in children and haemorrhage in adults. Moyamoya is secondary to large vessel occlusion with telangiectactic colateral circulation and seen in SCD, tuberous sclerosis, neurofibromatosis, vasculitides, infection. Intracranial anuerysms associated with SCD are usually multiple (57%) and 30% are seen in posterior circulation.
"Hair-on-end" appearance is seen in any anaemia. SCD patients are prone for bone infarcts and osteomyelitis, especially from salmonella. Osteomyelitis most commonly affects diaphysis of long bones.
Reference:
Madani G et al. The radiological manifestations of sickle cell disease. Clinical Radiology. Volume 62, Pages 528-538
