天美传媒

Abstract

Background: The hardened red blood cells (RBC)-induced capillary endothelial damage, inflammation, edema, and fibrosis are initiated at birth, and terminate with disseminated tissue hypoxia, acute painful crises, multiorgan failures, and sudden death even at childhood in the sickle cell diseases (SCD).

Methods: All cases with the SCD were included into the study.

Results: We studied 222 males and 212 females with similar mean ages (30.8 vs 30.3 years, p>0.05, respectively). Disseminated teeth losses (5.4% vs 1.4%, p<0.001), ileus (7.2% vs 1.4%, p<0.001), cirrhosis (8.1% vs 1.8%, p<0.001), leg ulcers (19.8% vs 7.0%, p<0.001), digital clubbing (14.8% vs 6.6%, p<0.001), coronary heart disease (18.0% vs 13.2%, p<0.05), chronic renal disease (9.9% vs 6.1%, p<0.05), chronic obstructive pulmonary disease (25.2% vs 7.0%, p<0.001), and stroke (12.1% vs 7.5%, p<0.05) were all higher but not acute chest syndrome (2.7% vs 3.7%, p>0.05) or pulmonary hypertension (12.6% vs 11.7%, p>0.05) or deep venous thrombosis and/or varices and/or telangiectasias (9.0% vs 6.6%, p>0.05) in males, significantly.

Conclusion: Infections, medical or surgical emergencies, or emotional stress-induced increased basal metabolic rate aggravates the sickling and capillary endothelial inflammation and edema, and may terminate with disseminated tissue hypoxia, acute painful crises, multiorgan failures, and sudden deaths in the SCD. RBC support may have the stongest analgesic effect, and decrease the risk of multiorgan failures and sudden death by decreasing the density of causative hardened cells from the circulation during such severe crises.