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Sickle cell disease in children: chronic complications and search of predictive factors for adverse outcomes.
[alpha-thalassemia]
Sickle
cell
disease
(
SCD
)
has
extremely
variable
phenotypes
,
and
several
factors
have
been
associated
with
the
severity
of
the
disease
.
To
analyze
the
chronic
complications
of
SCD
and
look
for
predictive
risk
factors
for
increased
severity
and
number
of
complications
.
Retrospective
study
including
all
children
followed
for
SCD
in
the
Paediatric
Haematology
Unit
of
a
tertiary
hospital
in
Portugal
,
who
completed
17
Â
yr
old
between
the
years
2004
and
2013
.
We
identified
44
patients
,
55
%
female
and
98
%
black
.
Chronic
complications
occurred
in
80
%
of
cases
.
Slight
dilatation
of
the
left
ventricle
was
the
most
frequent
complication
(
47
.
7
%
)
,
followed
by
respiratory
function
disturbs
(
43
.
2
%
)
,
microlithiasis
or
cholelithiasis
(
40
.
9
%
)
,
increased
flow
velocity
of
cerebral
arteries
(
31
.
8
%
)
,
enuresis
,
delayed
puberty
and
bone
abnormalities
(
6
.
8
%
each
)
,
sickle
cell
retinopathy
and
leg
ulcer
(
4
.
6
%
each
)
and
recurrent
priapism
(
2
.
3
%
)
.
We
identified
a
statistically
significant
association
between
leukocytes
>
15
Â
000
/
μL
and
a
higher
number
of
hospitalizations
(
P
Â
<
Â
0
.
001
)
and
chronic
complications
of
the
disease
(
P
Â
=
Â
0
.
035
)
.
The
occurrence
of
dactylitis
in
first
year
of
life
was
also
significantly
associated
with
a
higher
number
of
hospitalizations
(
P
Â
=
Â
0
.
004
)
and
chronic
complications
(
P
Â
=
Â
0
.
018
)
.
The
presence
of
α-thalassemia
was
associated
with
a
lower
number
of
chronic
complications
(
P
Â
=
Â
0
.
036
)
.
Leucocytosis
and
dactylitis
in
the
first
year
of
life
can
be
predictors
of
SCD
severity
,
while
the
presence
of
α-thalassemia
can
be
protective
.
The
determination
of
early
predictors
of
chronic
complications
of
SCD
may
improve
the
comprehensive
care
of
these
patients
.
Diseases
Validation
Diseases presenting
"priapism"
symptom
alpha-thalassemia
This symptom has already been validated
