Rare Diseases Symptoms Automatic Extraction
Home
A random Abstract
Our Project
Our Team
Clinical experience with an L-proline–stabilized 10 %intravenous immunoglobulin (Privigen®): real-life effectiveness and tolerability.
[severe combined immunodeficiency]
This
retrospective
study
evaluated
the
effectiveness
and
tolerability
in
clinical
practice
of
an
L-
proline-stabilized
10
%
intravenous
immunoglobulin
(
IVIG
;
Privigen
®
)
in
patients
with
primary
(
PID
)
or
secondary
immunodeficiency
(
SID
)
.
Patients
from
6
centers
in
Europe
and
the
US
were
treated
with
individually
determined
regimens
of
Privigen
®
for
≥
3
months
.
Serum
immunoglobulin
G
(
IgG
)
trough
levels
,
annualized
rates
of
infection
,
hospitalization
and
antibiotics
use
,
and
the
incidence
of
adverse
events
(
AEs
)
were
analyzed
.
Of
72
patients
,
three
infants
with
severe
combined
immunodeficiency
(
SCID
)
were
analyzed
separately
.
The
remaining
69
patients
(
52
.
2
%
male
;
median
age
38
years
[
range
:
0
.
1
-
90
.
0
]
)
with
PID
(
82
.
6
%
)
or
SID
(
17
.
4
%
)
received
a
mean
(
±
standard
deviation
)
Privigen
®
dose
of
532
±
250
mg
/
kg
/
month
resulting
in
trough
serum
IgG
levels
of
407
-
1
,
581
mg
/
dL
(
median
:
954
mg
/
dL
)
.
Ten
patients
(
14
.
5
%
)
experienced
11
serious
bacterial
infections
over
22
.
0
±
15
.
0
months
of
treatment
(
0
.
087
events
/
patient
/
year
,
upper
one
-sided
99
%
confidence
interval
:
0
.
170
)
,
the
most
common
being
pneumonia
(
11
.
6
%
)
.
The
rates
for
any
infection
and
hospitalization
were
1
.
082
events
/
patient
/
year
and
3
.
63
days
/
patient
/
year
,
respectively
.
Two
patients
with
severe
disease
accounted
for
303
of
460
hospital
days
.
Across
all
72
patients
,
13
(
18
.
1
%
)
patients
experienced
AEs
,
including
10
(
13
.
9
%
)
patients
with
AEs
at
least
possibly
related
to
Privigen
®
,
including
headache
(
8
.
3
%
)
,
fever
,
and
chills
(
2
.
8
%
each
)
.
No
related
serious
AEs
were
reported
.
One
infant
with
SCID
died
due
to
severe
viral
infection
.
Despite
the
heterogeneous
population
,
effectiveness
and
tolerability
of
Privigen
®
in
clinical
practice
closely
matched
those
reported
in
clinical
studies
.
Diseases
Validation
Diseases presenting
"pneumonia"
symptom
22q11.2 deletion syndrome
acute rheumatic fever
allergic bronchopulmonary aspergillosis
alpha-thalassemia
classical phenylketonuria
cohen syndrome
congenital diaphragmatic hernia
heparin-induced thrombocytopenia
hydrocephalus with stenosis of the aqueduct of sylvius
junctional epidermolysis bullosa
lamellar ichthyosis
legionellosis
liposarcoma
lymphangioleiomyomatosis
monosomy 21
oculocutaneous albinism
omenn syndrome
pleomorphic liposarcoma
primary effusion lymphoma
proteus syndrome
pyomyositis
scrub typhus
severe combined immunodeficiency
triple a syndrome
waldenström macroglobulinemia
wiskott-aldrich syndrome
x-linked adrenoleukodystrophy
zellweger syndrome
This symptom has already been validated