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Cystinuria: mechanisms and management.
[cystinuria]
Cystinuria
is
a
relatively
uncommon
cause
of
pediatric
stone
disease
,
but
has
significant
morbidity
if
not
properly
controlled
because
of
its
significant
stone
recurrence
rate
.
Cystinuria
is
caused
by
the
inability
of
the
renal
tubules
to
reabsorb
filtered
cystine
,
which
is
poorly
soluble
at
a
typical
urine
pH
<
7
.
Although
many
advances
have
been
made
in
the
understanding
of
the
genetic
and
physiological
basis
of
cystinuria
,
the
cornerstones
of
treatment
still
involve
stone
prevention
with
dietary
measures
and
pharmacological
therapy
,
coupled
with
surgical
interventions
for
stone
removal
.
Pharmacological
treatments
can
carry
significant
side
effects
that
must
be
monitored
and
can
limit
therapy
as
well
as
impede
compliance
.
Most
patients
will
require
surgical
intervention
for
stone
removal
,
although
compliance
with
prevention
strategies
reduces
the
need
for
intervention
.