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Abstract
Background: Sickle cell
diseases (SCDs) are inborn
and severe inflammatory processes
on vascular endothelium, particularly
at the capillaries which are
the actual distributors of
the sickled or just hardened
red blood cells (RBCs) into
the tissues.
Methods:
All patients of 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), 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 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
RBCs-induced capillary endothelial
damage, inflammation, edema,
and fibrosis are initiated
at birth, and terminate with
disseminated tissue hypoxia,
multiorgan failures, and sudden
deaths even at childhood.
Although RBCs suspensions
and corticosteroids in acute
and aspirin plus hydroxyurea
in acute and chronic phases
decrease severity of the destructive
process, survivals are still
shortened in both genders,
dramatically. Infections,
medical or surgical emergencies,
or emotional stresses-induced
increased basal metabolic
rate aggravates the sickling
and capillary endothelial
edema, and may terminate with
multiorgan failures-induced
sudden deaths in the SCDs.
Key
words: Sickle cell diseases,
sickled or just hardened red
blood cells, capillary endothelial
damage, exaggerated capillary
endothelial edema, sudden
deaths
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