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[Anesthetic management of a patient with erythropoietic protoporphyria for laparoscopic appendectomy].
[erythropoietic protoporphyria]
Porphyria
is
a
hereditary
disorder
due
to
reduction
in
enzyme
activity
of
heme
synthesis
system
,
resulting
in
accumulation
of
heme
precursors
.
Erythropoietic
protoporphyria
(
EPP
)
is
a
type
of
porphyria
.
There
are
varieties
of
clinical
expressions
of
EPP
such
as
hepatic
damage
and
photosensitivity
,
caused
by
the
accumulation
of
protoporphyrin
in
the
liver
and
the
skin
.
Therefore
it
is
important
to
prevent
development
of
these
clinical
expressions
.
A
36
-
year
-old
woman
with
EPP
was
scheduled
for
a
laparoscopic
appendectomy
.
Anesthesia
was
induced
with
remifentanil
,
sevoflurane
and
rocuronium
,
and
maintained
with
remifentanil
,
fentanyl
and
sevoflurane
.
She
had
performed
normal
daily
activities
without
taking
countermeasures
against
photosensitivity
,
so
we
did
not
change
the
light
in
the
operating
room
,
and
allowed
using
endoscopy
.
The
surgery
was
performed
without
any
complications
.
No
skin
symptom
was
observed
perioperatively
.
The
level
of
aminotransferase
was
elevated
temporally
after
the
operation
,
while
the
level
of
protoporphyrin
was
unchanged
.
Therefore
,
we
considered
there
was
no
manifestation
of
EPP
perioperatively
.
In
general
,
patients
with
EPP
do
not
develop
acute
attacks
induced
by
drugs
like
barbiturates
.
Since
there
was
a
case
report
of
severe
liver
dysfunction
of
EPP
,
we
did
not
use
any
contraindicated
drugs
in
patients
with
acute
intermittent
porphyria
in
this
patient
.
Considering
a
possibility
of
motor
nerve
damage
in
severe
EPP
,
we
carried
out
anesthetic
management
without
epidural
anesthesia
.
Diseases
Validation
Diseases presenting
"normal daily activities"
symptom
erythropoietic protoporphyria
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