Summary. More precise analysis of causes of death is needed to focus research efforts and improve morbidity and mortality in sickle cell disease. In this study, the morphological evidence of the cause of death was studied in 306 autopsies of sickle cell disease, which were accrued between 1929 and 1996. The most common cause of death for all sickle variants and for all age groups was infection (33-48%). The terminal infection was heralded by upper respiratory tract syndromes in 72AE6% and by gastroenteritis in 13AE7%. The most frequent portal of entry in children was the respiratory tract but, in adults, a site of severe chronic organ injury. Other causes of death included stroke 9AE8%, therapy complications 7AE0%, splenic sequestration 6AE6%, pulmonary emboli/thrombi 4AE9%, renal failure 4AE1%, pulmonary hypertension 2AE9%, hepatic failure 0AE8%, massive haemolysis/red cell aplasia 0AE4% and left ventricular failure 0AE4%. Death was frequently sudden and unexpected (40AE8%) or occurred within 24 h after presentation (28AE4%), and was usually associated with acute events (63AE3%). This study shows that the first 24 h after presentation for medical care is an especially perilous time for patients with sickle cell disease and an acute event. Close monitoring and prompt aggressive treatment are warranted.
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