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Abstract
Background: Sickle
cell diseases (SCDs) are inborn
and destructive processes
on vascular endothelium, particularly
at the capillaries.
Methods:
All patients with the SCDs
were included.
Results:
We studied 222 males and 212
females with similar 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), 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 higher in
males but not acute chest
syndrome (2.7% vs 3.7%), pulmonary
hypertension (12.6% vs 11.7),
deep venous thrombosis and/or
varices and/or telangiectasias
(9.0% vs 6.6%), or mean age
of mortality (30.2 vs 33.3
years) (p>0.05 for all).
Conclusion:
The sickled or just hardened
red blood cells (RBCs)-induced
capillary endothelial damage
initiates at birth, and terminates
with multiorgan failures and
sudden deaths even at childhood.
Although RBCs suspensions
and corticosteroids in acute,
and aspirin plus hydroxyurea
both in acute and chronic
phases decrease severity,
survivals are still shortened
in both genders, dramatically.
Infections, medical or surgical
emergencies, or emotional
stress-induced increased basal
metabolic rate accelerates
sickling, and an exaggerated
capillary endothelial edema-induced
myocardial infarction or stroke
may cause sudden deaths. Lifelong
aspirin with an anti-inflammatory
dose plus low-dose warfarin
may be life-saving even at
childhood both to decrease
severity of capillary endothelial
inflammation and to prevent
thromboembolic complications
in the SCDs.
Key
words: Sickle cell diseases,
sickled or just hardened red
blood cells, capillary endothelial
edema, myocardial infarction,
stroke, sudden deaths, low-dose
warfarin
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