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Pericardial effusion as a crucial presentation of Erdheim-Chester disease in a hemodialysis patient: an overlooked diagnosis.
[erdheim-chester disease]
Erdheim-
Chester
disease
(
ECD
)
is
a
rare
non-
Langerhans
cell
histiocytosis
whose
hallmark
is
tissue
infiltration
by
CD
68
-
positive
,
CD
1
a-negative
and
usually
S-
100
protein-
positive
foamy
non-
Langerhans
histiocytes
and
mononuclear
cells
.
Here
,
we
report
a
hemodialysis
(
HD
)
patient
who
presented
with
fever
and
pericardial
effusion
.
We
performed
pericardiocentesis
with
pericardial
biopsy
and
the
histological
findings
indicated
ECD
.
We
administered
intravenous
methylprednisolone
pulse
therapy
(
250
mg
/
d
)
followed
by
oral
prednisolone
(
50
mg
/
d
)
.
The
patient
's
fever
gradually
subsided
and
there
was
no
recurrence
of
pericardial
effusion
.
This
is
the
first
report
of
an
HD
patient
with
ECD
.
We
suggest
that
ECD
be
considered
in
the
differential
diagnosis
of
new
HD
patients
who
present
with
pericardial
effusion
,
especially
when
this
did
not
improve
following
increased
dose
of
HD
.
Diseases
Validation
Diseases presenting
"first report"
symptom
achondroplasia
alexander disease
aniridia
cadasil
canavan disease
child syndrome
cohen syndrome
congenital toxoplasmosis
cowden syndrome
cushing syndrome
cutaneous mastocytosis
cystinuria
dedifferentiated liposarcoma
dentinogenesis imperfecta
dracunculiasis
dystrophic epidermolysis bullosa
epidermolysis bullosa simplex
erdheim-chester disease
esophageal squamous cell carcinoma
fabry disease
familial mediterranean fever
focal myositis
harlequin ichthyosis
hirschsprung disease
hodgkin lymphoma, classical
holt-oram syndrome
homocystinuria without methylmalonic aciduria
inclusion body myositis
junctional epidermolysis bullosa
kabuki syndrome
kindler syndrome
krabbe disease
lamellar ichthyosis
liposarcoma
lymphangioleiomyomatosis
monosomy 21
neonatal adrenoleukodystrophy
neuralgic amyotrophy
oculocutaneous albinism
oligodontia
omenn syndrome
pendred syndrome
pleomorphic liposarcoma
primary hyperoxaluria type 1
pyomyositis
pyruvate dehydrogenase deficiency
scrub typhus
severe combined immunodeficiency
sneddon syndrome
triple a syndrome
typhoid
waldenström macroglobulinemia
werner syndrome
wiskott-aldrich syndrome
x-linked adrenoleukodystrophy
zellweger syndrome
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