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Combined liver and kidney transplantation in children.
[primary hyperoxaluria type 1]
Simultaneous
combined
liver
-kidney
transplantation
(
CLKT
)
is
a
rare
operation
in
pediatric
patients
so
that
annually
only
10
-
30
operations
are
performed
worldwide
.
The
main
indications
for
CLKT
are
primary
hyperoxaluria
type
1
and
autosomal
recessive
polycystic
kidney
disease
.
In
addition
,
CLKT
is
indicated
in
individual
patients
with
metabolic
or
cirrhotic
liver
diseases
and
end-
stage
kidney
disease
.
The
surgery
and
immediate
post-operative
management
of
CLKT
remain
challenging
in
infants
and
small
children
.
The
patients
should
be
operated
on
before
they
become
severely
ill
or
develop
major
systemic
manifestations
of
their
metabolic
disorder
.
The
liver
allograft
is
immunologically
protective
of
the
kidney
graft
in
simultaneous
CLKT
,
often
resulting
in
well-preserved
kidney
function
.
The
long
-term
outcome
after
CLKT
is
nowadays
comparable
to
that
of
isolated
liver
and
kidney
transplantations
.
Diseases
Validation
Diseases presenting
"long-term outcome"
symptom
aniridia
cholangiocarcinoma
classical phenylketonuria
cystinuria
erythropoietic protoporphyria
esophageal squamous cell carcinoma
fabry disease
hirschsprung disease
homocystinuria without methylmalonic aciduria
hydrocephalus with stenosis of the aqueduct of sylvius
inclusion body myositis
kabuki syndrome
krabbe disease
oligodontia
papillon-lefèvre syndrome
primary hyperoxaluria type 1
trochlear dysplasia
von hippel-lindau disease
waldenström macroglobulinemia
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