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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
"first year"
symptom
22q11.2 deletion syndrome
achondroplasia
alpha-thalassemia
aniridia
benign recurrent intrahepatic cholestasis
child syndrome
cohen syndrome
congenital adrenal hyperplasia
congenital diaphragmatic hernia
congenital toxoplasmosis
cowden syndrome
dystrophic epidermolysis bullosa
epidermolysis bullosa simplex
erythropoietic protoporphyria
gm1 gangliosidosis
hirschsprung disease
homocystinuria without methylmalonic aciduria
junctional epidermolysis bullosa
kabuki syndrome
kindler syndrome
krabbe disease
lymphangioleiomyomatosis
megacystis-microcolon-intestinal hypoperistalsis syndrome
neonatal adrenoleukodystrophy
phenylketonuria
pleomorphic liposarcoma
pyruvate dehydrogenase deficiency
severe combined immunodeficiency
sneddon syndrome
waldenström macroglobulinemia
wolf-hirschhorn syndrome
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