Rare Diseases Symptoms Automatic Extraction
Home
A random Abstract
Our Project
Our Team
Igf-I stimulates in vivo thymopoiesis after stem cell transplantation in a child with Omenn syndrome.
[omenn syndrome]
Children
with
severe
combined
immunodeficiency
who
receive
a
T
cell-depleted
hematopoietic
stem
cell
transplantation
(
HSCT
)
have
delayed
immune
reconstitution
.
To
examine
the
use
of
recombinant
human
insulin
-like
growth
factor
-
I
(
rhIGF-
I
)
therapy
to
stimulate
thymopoiesis
after
HSCT
.
A
child
with
Omenn
syndrome
failed
T
cell
reconstitution
6
months
after
HSCT
.
She
started
rhIGF-
I
therapy
just
before
age
18
months
.
The
initial
dose
(
40
microg
/
kg
twice
daily
)
was
increased
every
2
weeks
to
a
maximum
dose
of
120
microg
/
kg
twice
daily
.
The
patient
's
absolute
T
cells
increased
from
7
to
132
/
mm
(
3
)
and
662
/
mm
(
3
)
after
3
and
5
months
of
rhIGF-
I
therapy
,
respectively
,
and
her
blastogenic
response
to
phytohemagglutinin
normalized
.
Three
months
after
discontinuation
of
rhIGF-
I
therapy
,
the
T
cells
continued
to
increase
(
to
2
,
427
/
mm
(
3
)
)
although
the
blastogenic
response
to
phytohemagglutinin
decreased
.
This
is
the
first
known
use
of
rhIGF-
I
therapy
to
help
restore
thymopoiesis
in
a
child
.
Diseases
Validation
Diseases presenting
"cell-depleted hematopoietic stem cell transplantation"
symptom
omenn syndrome
You can validate or delete this automatically detected symptom
Validate the Symptom
Delete the Symptom