Acute chest syndrome and pulmonary hypertension in sickle cell diseases

Abstract

Background: Sickle cell diseases (SCD) are severe inflammatory processes on vascular endothelium, particularly at the capillary level since the capillary system is the main distributor of hardened red blood cells (RBC) into tissues. Methods: All patients with the SCD were included. Results: The study included 222 males and 212 females with similar mean ages (30.8 versus 30.3 years, p>0.05, respectively). Smoking (23.8% versus 6.1%, p<0.001), alcohol (4.9% versus 0.4%, p<0.001), disseminated teeth losses (5.4% versus 1.4%, p<0.001), ileus (7.2% versus 1.4%, p<0.001), cirrhosis (8.1% versus 1.8%, p<0.001), leg ulcers (19.8% versus 7.0%, p<0.001), digital clubbing (14.8% versus 6.6%, p<0.001), coronary heart disease (CHD) (18.0% versus 13.2%, p<0.05), chronic renal disease (CRD) (9.9% versus 6.1%, p<0.05), chronic obstructive pulmonary disease (COPD) (25.2% versus 7.0%, p<0.001), and stroke (12.1% versus 7.5%, p<0.05) were higher in males but not acute chest syndrome (ACS) (2.7% versus 3.7%), pulmonary hypertension (PHT) (12.6% versus 11.7), and deep venous thrombosis and/or varices and/or telangiectasias (9.0% versus 6.6%), significantly (p>0.05 for all). Mean ages of ACS and PHT were 30.3 and 34.0 years (p<0.05), respectively. Conclusion: Although smoking, alcohol, disseminated teeth losses, ileus, cirrhosis, leg ulcers, digital clubbing, CHD, CRD, COPD, and stroke-like atherosclerotic events were higher in males, and the male sex alone is a risk factor for atherosclerosis, ACS and PHT were similar in both genders, and mean age of PHT is much higher than ACS, significantly. In other words, PHT may have a hardened RBC-induced chronic, whereas ACS an acute thromboembolic background, in the SCD. Key words: Sickle cell diseases, acute chest syndrome, pulmonary hypertension, thromboembolism, chronic endothelial damage, atherosclerosis, metabolic syndrome