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Kindler syndrome and periodontal disease: review of the literature and a 12-year follow-up case.
[kindler syndrome]
The
association
of
aggressive
periodontitis
with
Kindler
syndrome
was
based
on
a
single
case
in
1996
and
later
confirmed
with
a
larger
population
.
Since
then
,
significant
research
has
greatly
increased
our
understanding
of
the
molecular
pathology
of
this
disorder
.
We
review
recent
advances
in
the
molecular
mechanisms
of
the
syndrome
and
present
a
maintenance
case
report
of
a
patient
who
has
been
followed
in
our
clinic
.
A
female
patient
who
was
diagnosed
with
Kindler
syndrome
and
aggressive
periodontitis
at
the
age
of
16
years
has
been
followed
and
treated
in
our
clinic
for
12
years
.
Her
main
treatment
has
been
maintenance
therapy
following
her
initial
treatment
and
restorative
work
previously
documented
.
Gingival
biopsies
obtained
during
the
recent
extraction
of
hopeless
maxillary
molars
were
used
for
histologic
assessment
of
gingival
tissue
attachment
apparatus
and
to
isolate
gingival
fibroblasts
.
Reverse
transcription-polymerase
chain
reaction
(
RT-PCR
)
was
performed
using
these
cells
to
confirm
the
lack
of
expression
of
kindlin-
1
.
RT-PCR
showed
the
total
loss
of
kindlin-
1
mRNA
in
cultured
gingival
fibroblasts
,
supporting
the
clinical
diagnosis
of
Kindler
syndrome
.
Tissue
biopsies
revealed
atypical
pocket
epithelium
.
Maintenance
therapy
has
been
moderately
successful
.
Teeth
that
were
recently
lost
had
a
poor
prognosis
at
the
initial
assessment
.
The
patient
's
gingiva
and
oral
mucosa
continue
to
be
fragile
with
episodes
of
sloughing
and
inflammation
.
Periodontitis
in
Kindler
syndrome
responds
to
maintenance
therapy
,
but
the
gingiva
and
oral
mucosa
continue
to
display
an
abnormal
appearance
with
white
patches
.
Histologic
findings
suggest
that
the
junctional
epithelium
in
Kindler
syndrome
may
be
abnormal
and
could
explain
why
these
patients
have
periodontal
disease
.
Attachment
loss
progressed
around
teeth
with
an
initial
guarded
or
poor
prognosis
.
Teeth
that
started
with
a
good
or
fair
prognosis
continue
to
have
a
fair
prognosis
.
Limited
dental
implant
treatment
is
being
considered
.
Diseases
Validation
Diseases presenting
"total loss"
symptom
canavan disease
dystrophic epidermolysis bullosa
kindler syndrome
oculocutaneous albinism
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