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Renal autotransplantation and modified pyelovesicostomy for intractable metabolic stone disease.
[cystinuria]
Some
patients
with
intractable
metabolic
stone
disease
experience
narcotic
dependence
,
which
can
not
be
managed
with
standard
treatments
.
We
offered
these
patients
renal
autotransplantation
with
a
modified
pyelovesicostomy
as
an
alternative
solution
.
Renal
autotransplantation
with
pyelovesicostomy
was
performed
for
15
kidneys
in
12
patients
(
3
bilateral
,
2
solitary
)
,
9
female
and
3
male
,
with
a
mean
age
of
33
.
8
years
(
range
16
to
55
)
.
The
etiology
of
metabolic
stone
disease
was
calcium
oxalate
(
40
%
)
,
cystinuria
(
33
%
)
,
type
1
renal
tubular
acidosis
(
14
%
)
,
calcium
oxalate
/
urate
(
7
%
)
and
medullary
sponge
kidney
(
7
%
)
.
Patients
reported
that
lifetime
stone
events
ranged
from
10
to
more
than
70
,
that
underwent
an
average
of
3
to
4
surgical
interventions
per
year
in
the
previous
2
years
and
that
they
were
dependent
on
daily
oral
narcotics
for
stone
related
pain
.
All
15
kidneys
were
successfully
autotransplanted
with
a
mean
followup
of
41
.
8
months
(
range
3
to
74
)
.
We
used
a
modified
pyelovesicostomy
with
ureteral
strip
in
13
and
standard
Boari
tube
in
2
cases
.
All
patients
continued
to
pass
small
stone
debris
per
urethra
with
minimal
symptoms
.
Of
12
patients
11
(
92
%
)
were
weaned
off
daily
narcotics
.
There
have
been
17
stone
episodes
in
4
patients
(
3
cystinuria
)
for
which
medical
intervention
and
pain
medication
was
required
.
The
number
of
urological
procedures
/
patients
before
(
155
/
12
[
12
.
9
]
)
and
after
(
8
/
12
[
0
.
66
]
)
autotransplantation
was
dramatically
reduced
(
paired
t
test
p
=
0
.
0001
)
.
The
preoperative
mean
estimated
glomerular
filtration
rate
was
77
.
2
cc
/
minute
,
and
73
.
5
,
71
.
9
,
79
.
2
cc
/
minute
at
12
,
36
and
60
months
,
respectively
.
Renal
autotransplantation
and
pyelovesicostomy
offer
patients
with
intractable
metabolic
stone
disease
the
opportunity
to
improve
quality
of
life
and
to
decrease
daily
narcotic
use
.
Diseases
Validation
Diseases presenting
"pain"
symptom
achondroplasia
acute rheumatic fever
adrenal incidentaloma
adrenomyeloneuropathy
aniridia
aromatase deficiency
carcinoma of the gallbladder
cholangiocarcinoma
coats disease
congenital diaphragmatic hernia
congenital toxoplasmosis
cushing syndrome
cutaneous mastocytosis
cystinuria
dedifferentiated liposarcoma
dentin dysplasia
dracunculiasis
dystrophic epidermolysis bullosa
epidermolysis bullosa simplex
erdheim-chester disease
erythropoietic protoporphyria
esophageal adenocarcinoma
esophageal carcinoma
esophageal squamous cell carcinoma
fabry disease
familial mediterranean fever
focal myositis
hirschsprung disease
hodgkin lymphoma, classical
holt-oram syndrome
junctional epidermolysis bullosa
kabuki syndrome
kindler syndrome
lamellar ichthyosis
liposarcoma
locked-in syndrome
lymphangioleiomyomatosis
malignant atrophic papulosis
neuralgic amyotrophy
oligodontia
oral submucous fibrosis
papillon-lefèvre syndrome
phenylketonuria
pleomorphic liposarcoma
primary hyperoxaluria type 1
proteus syndrome
pyomyositis
scrub typhus
sneddon syndrome
systemic capillary leak syndrome
thoracic outlet syndrome
trochlear dysplasia
typhoid
von hippel-lindau disease
waldenström macroglobulinemia
well-differentiated liposarcoma
wolf-hirschhorn syndrome
This symptom has already been validated