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Transplantation in adults with primary hyperoxaluria: single unit experience and treatment algorithm.
[primary hyperoxaluria type 1]
Kidney
transplantation
alone
in
Primary
Hyperoxaluria
is
associated
with
a
high
rate
of
recurrence
and
in
many
cases
early
graft
loss
.
Liver
transplantation
offers
the
possibility
of
correcting
the
metabolic
defect
.
A
retrospective
review
of
five
cases
of
Primary
Hyperoxaluria
managed
at
a
major
transplant
unit
was
performed
.
The
5
patients
had
a
mean
age
of
32
.
2
years
(
range
28
-
40
)
at
time
of
first
transplantation
.
3
patients
had
kidney
only
transplants
(
one
live
donor
,
2
deceased
donor
)
and
2
had
segmental
liver
followed
by
delayed
kidney
transplantation
.
All
3
kidney
alone
failed
and
one
is
now
awaiting
a
live
donor
transplant
,
one
underwent
kidney
alone
retransplantation
(
failed
5
years
later
)
and
one
had
a
combined
deceased
donor
liver
and
kidney
transplantation
(
remains
well
at
4
years
)
.
The
2
segmental
liver
sequential
kidney
transplant
recipients
remain
well
at
1
year
and
3
years
.
Combined
liver
-kidney
transplantation
may
be
a
better
choice
as
the
primary
transplant
procedure
.
The
indication
and
timing
for
pre-emptive
liver
or
liver
followed
by
delayed
kidney
transplantation
remains
a
matter
of
debate
.
Diseases
Validation
Diseases presenting
"combined deceased donor liver and kidney transplantation"
symptom
primary hyperoxaluria type 1
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