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Assessment of renal involvement in patients with familial Mediterranean fever: a clinical study from Ardabil, Iran.
[familial mediterranean fever]
Familial
Mediterranean
fever
(
FMF
)
is
an
autosomal
recessive
disease
characterised
by
recurrent
episodes
of
painful
inflammation
in
the
abdomen
,
chest
or
joints
.
The
association
between
FMF
and
non-amyloid
glomerulopathies
are
unusual
.
In
this
study
,
we
describe
our
experiences
and
observations
about
renal
involvement
in
patients
with
FMF
.
A
total
of
108
patients
with
FMF
was
enrolled
in
the
study
.
Twelve
patients
with
FMF
were
referred
to
the
Nephrology
Service
,
for
evaluation
and
assessment
of
the
degree
of
renal
involvement
.
All
the
12
patients
underwent
percutaneous
ultrasound-guided
renal
biopsies
and
genetic
analysis
.
On
microscopic
examination
of
the
kidney
specimens
,
six
patients
were
found
to
have
amyloidosis
,
five
focal
segmental
glomerulosclerosis
and
one
patient
membranoproliferative
glomerulonephritis
.
It
seems
that
in
patients
with
FMF
and
renal
amyloidosis
,
the
response
to
treatment
with
colchicine
is
excellent
,
but
in
patients
with
FMF
and
focal
segmental
glomerulosclerosis
,
the
response
to
treatment
with
colchicine
is
poor
.
We
present
an
evidence-based
algorithm
,
constructed
based
on
literature
review
,
to
aid
decision
making
in
management
of
renal
involvement
in
patients
with
FMF
.
T
he
results
of
our
study
suggest
that
in
patients
with
FMF
and
renal
involvement
,
non-amyloid
renal
lesions
should
be
considered
in
the
differential
diagnosis
in
addition
to
amyloidosis
.
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