The presentation and management of the acute abdomen in the patient with sickle-cell anemia.

Sickle crises frequently manifest as abdominal pain that may simulate intra-abdominal infection. To establish parameters to distinguish these, we retrospectively studied 53 patients with sickle-cell anemia who had abdominal pain (genotype SS 62%, SC 15%, SA 11%, S-other 11%; 30% men and 70% women; mean age 23). A vaso-occlusive crises was responsible for the pain in 57 per cent; 23 per cent had a surgical entity and 20 per cent had a nonsurgical genitourinary disorder. Of the surgical conditions, 9 of 12 patients (95%) had cholecystitis and 4 of 12 patients (33%) had acute appendicitis (one patient had both). Vaso-occlusive crises were diffuse in 15 of 30 patients (50%), compared with proven surgical conditions, and was more often associated with remote pain such as limbs and chest (23 of 30 [77%] P less than 0.005). The pain of vaso-occlusive crises simulated prior crises in 21 of 30 patients (70%) compared with 1 of 12 patients (8%) who had surgical abdominal pain (P less than 0.005). A precipitating event (especially upper respiratory infection) was found in 50 per cent of abdominal vaso-occlusive crises versus 0 per cent of surgical abdomens (P less than 0.010). The pain was relieved with hydration and oxygen in 97 per cent of sickle crises within 48 hours versus 0 per cent of surgical abdomens (P less than 0.01).(ABSTRACT TRUNCATED AT 250 WORDS)