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Combined split liver and kidney transplantation in a three-year-old child with primary hyperoxaluria type 1 and complete thrombosis of the inferior vena cava.
[primary hyperoxaluria type 1]
PH
1
is
an
inborn
error
of
the
metabolism
in
which
a
functional
deficiency
of
the
liver
-
specific
peroxisomal
enzyme
,
AGT
,
causes
hyperoxaluria
and
hyperglycolic
aciduria
.
Infantile
PH
1
is
the
most
aggressive
form
of
this
disease
,
leading
to
early
nephrocalcinosis
,
systemic
oxalosis
,
and
end-
stage
renal
failure
.
Infantile
PH
1
is
rapidly
fatal
in
children
unless
timely
liver
-kidney
transplantation
is
performed
to
correct
both
the
hepatic
enzyme
defect
and
the
renal
end-organ
damage
.
The
surgical
procedure
can
be
further
complicated
in
infants
and
young
children
,
who
are
at
higher
risk
for
vascular
anomalies
,
such
as
IVC
thrombosis
.
Although
recently
a
limited
number
of
children
with
IVC
thrombosis
have
underwent
successful
kidney
transplantation
,
successful
multi-organ
transplantation
in
a
child
with
complete
IVC
thrombosis
is
quite
rare
.
We
report
here
the
interesting
and
technically
difficult
case
of
a
three
-
yr
-old
girl
with
a
complete
thrombosis
of
the
IVC
,
who
was
the
recipient
of
combined
split
liver
and
kidney
transplantation
for
infantile
PH
1
.
Although
initial
delayed
renal
graft
function
with
mild
-
to
-
moderate
acute
rejection
was
observed
,
the
patient
rapidly
regained
renal
function
after
steroid
boluses
,
and
was
soon
hemodialysis-independent
,
with
good
diuresis
.
Serum
and
plasma
oxalate
levels
progressively
decreased
;
although
,
to
date
they
are
still
above
normal
.
Hepatic
and
renal
function
indices
were
at
,
or
approaching
,
normal
values
when
the
patient
was
discharged
15
-
wk
post-transplant
,
and
the
patient
continues
to
do
well
,
with
close
and
frequent
follow-up
.
This
is
the
first
report
of
a
successful
double
-organ
transplant
in
a
pediatric
patient
presenting
with
infantile
PH
1
complicated
by
complete
IVC
thrombosis
.
Diseases
Validation
Diseases presenting
"split liver"
symptom
primary hyperoxaluria type 1
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