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Anti-IL-1 treatment in familial Mediterranean fever and related amyloidosis.
[familial mediterranean fever]
Colchicine
is
the
standard
treatment
in
familial
Mediterranean
fever
(
FMF
)
patients
.
New
treatment
strategies
are
needed
in
FMF
patients
who
were
unresponsive
to
colchicine
therapy
or
who
had
developed
amyloidosis
.
The
aim
of
this
study
was
to
present
clinical-laboratory
features
and
treatment
responses
of
pediatric
FMF
patients
that
were
treated
with
anti-
IL
-
1
therapies
.
Files
of
patients
who
had
been
followed
in
our
department
with
diagnosis
of
FMF
were
retrospectively
evaluated
.
Patients
that
have
been
receiving
anti-
IL
-
1
therapies
(
anakinra
or
canakinumab
)
were
included
to
the
study
.
All
patients
were
interpreted
with
respect
to
the
demographic
data
,
clinical
and
laboratory
features
of
the
disease
,
genetic
analysis
of
MEFV
mutations
and
treatment
responses
.
Among
330
currently
registered
FMF
patients
,
13
patients
were
included
to
the
study
.
Seven
of
them
received
anti-
IL
-
1
therapy
due
to
colchicine
resistance
and
6
due
to
FMF
-related
amyloidosis
(
1
of
them
with
nephrotic
syndrome
,
2
with
chronic
kidney
disease
,
3
with
renal
transplantation
)
.
In
all
treated
patients
,
attacks
completely
disappeared
or
decreased
in
frequency
;
partial
remission
occured
in
nephrotic
syndrome
patient
;
and
their
life
quality
improved
.
Anti-
IL
-
1
therapies
can
be
successfully
used
in
colchicine-resistant
FMF
patients
and
patients
with
amyloidosis
during
childhood
and
adolescent
period
without
major
side
effects
.
Diseases
Validation
Diseases presenting
"fever"
symptom
22q11.2 deletion syndrome
acute rheumatic fever
alexander disease
allergic bronchopulmonary aspergillosis
canavan disease
carcinoma of the gallbladder
child syndrome
congenital toxoplasmosis
cushing syndrome
cystinuria
dracunculiasis
erdheim-chester disease
esophageal adenocarcinoma
esophageal carcinoma
familial mediterranean fever
focal myositis
hodgkin lymphoma, classical
lamellar ichthyosis
legionellosis
locked-in syndrome
malignant atrophic papulosis
neonatal adrenoleukodystrophy
neuralgic amyotrophy
oculocutaneous albinism
papillon-lefèvre syndrome
pyomyositis
pyruvate dehydrogenase deficiency
scrub typhus
severe combined immunodeficiency
sneddon syndrome
systemic capillary leak syndrome
triple a syndrome
typhoid
waldenström macroglobulinemia
wolf-hirschhorn syndrome
This symptom has already been validated