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Elevated soluble transferrin receptor levels reflect increased erythropoietic drive rather than iron deficiency in pediatric sickle cell disease.
[erythropoietic protoporphyria]
The
prevalence
of
iron
deficiency
in
pediatric
sickle
cell
disease
(
SCD
)
is
difficult
to
describe
because
standard
markers
of
iron
metabolism
are
altered
in
this
inflammatory
state
.
Soluble
transferrin
receptor
(
sTfR
)
is
an
alternative
marker
of
iron
utilization
.
Our
primary
objective
was
to
evaluate
the
utility
of
sTfR
as
a
biomarker
of
hemolysis
and
erythropoietic
drive
versus
iron
metabolism
in
SCD
.
In
a
prospective
cohort
study
,
we
screened
51
children
with
SCD
at
steady
state
with
markers
of
iron
status
and
sTfR
.
Iron
deficient
patients
were
treated
with
ferrous
sulfate
for
6
weeks
,
followed
by
repeat
testing
.
At
baseline
,
there
was
stronger
correlation
between
sTfR
and
markers
of
hemolysis
and
erythropoietic
drive
,
including
hemoglobin
(
Spearman
's
r
=
-
0
.
67
,
P
<
or
=
0
.
001
)
and
reticulocyte
count
(
Spearman
's
r
=
0
.
59
,
P
<
or
=
0
.
001
)
,
than
with
markers
of
iron
metabolism
,
such
as
transferrin
saturation
(
TS
;
Spearman
's
r
=
0
.
33
,
P
=
0
.
02
)
and
ferritin
(
Spearman
's
r
=
0
.
17
,
P
=
0
.
26
)
.
Eleven
(
21
%
)
of
the
51
children
were
identified
as
iron
deficient
.
For
treated
patients
,
response
to
iron
therapy
was
variable
,
although
all
patients
had
a
significant
increase
(
mean
11
+
/
-
3
.
9
%
,
P
=
0
.
001
)
in
TS
.
sTfR
activity
is
a
stronger
biomarker
of
hemolysis
and
erythropoietic
drive
than
of
iron
-
restricted
erythropoiesis
in
pediatric
SCD
.
Low
TS
was
most
suggestive
of
iron
deficiency
and
an
increase
in
TS
represented
the
most
consistent
indicator
of
response
to
iron
supplementation
.
Diseases
Validation
Diseases presenting
"an increase in ts represented the most consistent indicator of response to iron supplementation"
symptom
erythropoietic protoporphyria
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