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Effect of dietary Lorenzo's oil and docosahexaenoic acid treatment for Zellweger syndrome.
[zellweger syndrome]
We
investigated
the
possible
therapeutic
effect
of
decreasing
plasma
levels
of
very
-
long
-chain
fatty
acids
(
C
2
6
:
0
)
with
a
synthetic
oil
containing
trioleate
and
trielucate
(
Lorenzo
's
oil
)
as
well
as
increasing
docosahexaenoic
acid
(
DHA
)
in
red
blood
cells
(
RBC
)
with
DHA
ethyl
ester
in
four
patients
with
Zellweger
syndrome
.
We
investigated
serial
changes
of
plasma
C
2
6
:
0
levels
and
DHA
levels
in
RBC
membranes
by
gas-liquid
chromatography
/
mass
spectrometry
(
GC
/
MS
)
.
After
death
,
the
fatty
acid
composition
of
each
patient
's
cerebrum
and
liver
was
studied
.
Dietary
administration
of
Lorenzo
's
oil
diminished
plasma
C
2
6
:
0
levels
.
Earlier
administration
of
Lorenzo
's
oil
was
more
effective
and
the
response
did
not
depend
on
the
duration
of
administration
.
DHA
was
incorporated
into
RBC
membrane
lipids
when
administrated
orally
,
and
its
level
increased
for
several
months
.
The
final
DHA
level
was
correlated
with
the
duration
of
administration
and
was
not
related
to
the
timing
of
initiation
of
treatment
.
DHA
levels
in
the
brains
and
livers
of
treated
patients
were
higher
than
in
untreated
patients
.
Early
initiation
of
Lorenzo
's
oil
and
the
long
-term
administration
of
DHA
may
be
useful
for
patients
with
Zellweger
syndrome
.
Diseases
Validation
Diseases presenting
"long-chain fatty acids"
symptom
adrenomyeloneuropathy
aniridia
neonatal adrenoleukodystrophy
x-linked adrenoleukodystrophy
zellweger syndrome
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