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Liver cell transplantation in severe infantile oxalosis--a potential bridging procedure to orthotopic liver transplantation?
[primary hyperoxaluria type 1]
The
infantile
form
of
primary
hyperoxaluria
type
I
(
PHI
)
is
the
most
devastating
PH
subtype
leading
to
early
end-
stage
renal
failure
and
severe
systemic
oxalosis
.
Combined
or
sequential
liver
-kidney
transplantation
(
LKTx
)
is
the
only
curative
option
but
it
involves
substantial
risks
,
especially
in
critically
ill
infants
.
The
procedure
also
requires
resources
that
are
simply
not
available
to
many
children
suffering
from
PHI
worldwide
.
Less
invasive
and
less
complex
therapeutic
interventions
allowing
a
better
timing
are
clearly
needed
.
Liver
cell
transplantation
(
LCT
)
may
expand
the
narrow
spectrum
of
auxiliary
measures
to
buy
time
until
LKTx
for
infants
can
be
performed
more
safely
.
We
performed
LCT
(
male
neonate
donor
)
in
a
15
-
month
-old
female
in
reduced
general
condition
suffering
from
systemic
oxalosis
.
Renal
replacement
therapy
,
initiated
at
the
age
of
3
months
,
was
complicated
by
continuous
haemodialysis
access
problems
.
Living
donor
liver
transplantation
was
not
available
for
this
patient
.
Plasma
oxalate
(
Pox
)
was
used
as
the
primary
outcome
measure
.
Pox
decreased
from
104
.
3
±
8
.
4
prior
to
70
.
0
±
15
.
0
μmol
/
L
from
Day
14
to
Day
56
after
LCT
.
A
significant
persistent
Pox
reduction
(
P
<
0
.
001
)
comparing
mean
levels
prior
to
(
103
.
8
μmol
/
L
)
and
after
Day
14
of
LCT
until
LKTx
(
77
.
3
μmol
/
L
)
was
seen
,
although
a
secondary
increase
and
wider
range
of
Pox
was
also
observed
.
In
parallel
,
the
patient
's
clinical
situation
markedly
improved
and
the
girl
received
a
cadaveric
LKTx
12
months
after
LCT
.
However
,
biopsy
specimens
taken
from
the
explanted
liver
did
not
show
male
donor
cells
by
amelogenin
polymerase
chain
reaction
.
With
due
caution
,
our
pilot
data
indicate
that
LCT
in
infantile
oxalosis
warrants
further
investigation
.
Improvement
of
protocol
and
methodology
is
clearly
needed
in
order
to
develop
a
procedure
that
could
assist
in
the
cure
of
PHI
.
Diseases
Validation
Diseases presenting
"liver-kidney transplantation"
symptom
primary hyperoxaluria type 1
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