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Liver transplantation for erythropoietic protoporphyria in Europe.
[erythropoietic protoporphyria]
Liver
transplantation
is
an
established
lifesaving
treatment
for
patients
with
severe
protoporphyric
liver
disease
,
but
disease
recurrence
in
the
graft
occurs
for
the
majority
of
recipients
.
Severe
burn
injuries
may
occur
when
protective
light
filters
are
not
used
with
surgical
luminaires
.
Motor
neuropathy
with
an
unclear
pathogenesis
is
a
frequent
complication
.
We
retrospectively
studied
35
transplants
performed
for
protoporphyric
liver
disease
in
31
European
patients
between
1983
and
2008
.
Most
of
the
patients
were
male
(
61
.
3
%
)
,
and
the
mean
age
at
the
time
of
primary
transplantation
was
39
years
(
range
=
9
-
60
years
)
.
The
overall
patient
survival
rates
were
77
%
at
1
year
and
66
%
at
5
and
10
years
.
The
overall
rate
of
disease
recurrence
in
the
graft
was
69
%
.
Forty
-
three
percent
of
the
patients
experienced
recurrence
within
a
year
,
but
this
was
often
a
transient
finding
that
was
associated
with
other
graft
complications
.
Phototoxic
injuries
due
to
surgical
luminaires
were
seen
in
25
.
0
%
of
the
patients
who
were
not
protected
by
filters
,
but
these
injuries
were
not
seen
in
the
9
patients
who
were
protected
by
filters
.
Significant
motor
neuropathies
requiring
prolonged
ventilation
complicated
the
postoperative
course
for
5
of
the
31
patients
(
16
.
1
%
)
.
Hematopoietic
stem
cell
transplantation
was
performed
for
3
patients
to
prevent
graft
loss
due
to
disease
recurrence
.
Prognostic
markers
are
needed
to
identify
patients
prone
to
severe
protoporphyric
liver
disease
so
that
curative
stem
cell
transplantation
can
be
offered
to
select
patients
instead
of
liver
transplantation
.
Diseases
Validation
Diseases presenting
"liver disease"
symptom
benign recurrent intrahepatic cholestasis
carcinoma of the gallbladder
cholangiocarcinoma
cutaneous mastocytosis
erythropoietic protoporphyria
legionellosis
megacystis-microcolon-intestinal hypoperistalsis syndrome
neonatal adrenoleukodystrophy
papillon-lefèvre syndrome
primary effusion lymphoma
primary hyperoxaluria type 1
pyomyositis
typhoid
zellweger syndrome
This symptom has already been validated