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Abstract
Background: Sickle
cell diseases (SCDs) are inborn
and catastrophic processes
on vascular
endothelium, particularly
at the capillaries.
Methods:
All patients were included.
Results:
We studied 222 males and 212
females with similar mean
ages (30.8 vs 30.3 years,
p>0.05, respectively).
Beside cirrhosis (8.1% vs
1.8%, p<0.001), smoking
(23.8% vs 6.1%, p<0.001),
alcohol (4.9% vs 0.4%, p<0.001),
transfused red blood cells
(RBCs) in their lives (48.1
vs 28.5 units, p=0.000), disseminated
teeth losses (5.4% vs 1.4%,
p<0.001), ileus (7.2% vs
1.4%, p<0.001), chronic
obstructive pulmonary disease
(COPD) (25.2% vs 7.0%, 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 (CHD)
(18.0% vs 13.2%, p<0.05),
chronic renal disease (CRD)
(9.9% vs 6.1%, p<0.05),
and stroke (12.1% vs 7.5%,
p<0.05) were all higher,
and autosplenectomy (50.4%
vs 53.3%, p<0.05) and mean
age of mortality were lower
in males, significantly (30.2
vs 33.3 years, p<0.05).
Conclusion:
The hardened RBCs-induced
capillary endothelial damage
initiates at birth, and terminates
with multiorgan failures even
at childhood. Parallel to
cirrhosis, all of the atherosclerotic
risk factors or consequences
including smoking, alcohol,
disseminated teeth losses,
ileus, COPD, leg ulcers, digital
clubbing, CHD, CRD, and stroke
were higher, and autosplenectomy
and mean age of mortality
were lower in males which
can not be explained by effects
of smoking and alcohol alone
at the relatively younger
mean age. So autosplenectomy
may be a good whereas male
gender alone may be a bad
prognostic factor, and cirrhosis
may have an atherosclerotic
background in the SCDs.
Key
words: Sickle cell diseases,
hardened red blood cells,
capillary endothelial damage,
capillary endothelial edema,
sudden deaths, atherosclerosis,
cirrhosis
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