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[Cystinuria in children: A report of 4 cases].
[cystinuria]
Cystinuria
is
an
inherited
autosomal-recessive
disorder
of
renal
reabsorption
of
the
dibasic
amino
acids
.
It
is
the
cause
of
about
6
%
of
all
kidney
stones
observed
in
children
.
Cystine
is
relatively
insoluble
at
the
physiological
pH
of
urine
.
Cystine
stones
are
characteristic
and
frequent
recurrences
are
observed
.
We
report
on
4
cases
and
describe
the
initial
presentation
(
obstructive
renal
failure
,
urinary
sepsis
,
familial
screening
)
and
the
medical
and
surgical
management
.
Medical
management
is
mainly
based
on
hyperhydration
and
urine
alkalinization
.
Long
-term
therapy
with
sulfhydryl
agents
to
prevent
formation
of
renal
stones
seems
to
be
effective
but
adverse
side
effects
are
frequent
,
requiring
the
withdrawal
of
treatment
.
Urological
management
has
evolved
from
surgical
stone
removal
to
minimally
invasive
procedures
(
extracorporeal
shock
wave
lithotripsy
,
ureteroscopy
)
.
