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Cytokine production by leukocytes of Papillon-Lefèvre syndrome patients in whole blood cultures.
[papillon-lefèvre syndrome]
Papillon-
Lefèvre
syndrome
(
PLS
)
is
characterised
by
aggressively
progressive
periodontitis
combined
with
palmo-
plantar
hyperkeratosis
.
It
is
caused
by
"
loss
of
function
"
mutations
in
the
cathepsin
C
gene
.
The
hypothesis
behind
this
study
is
that
PLS
patients
'
polymorphonuclear
leukocytes
(
PMNs
)
produce
more
proinflammatory
cytokines
to
compensate
for
their
reduced
capacity
to
neutralize
leukotoxin
and
to
eliminate
Aggregatibacter
actinomycetemcomitans
.
Production
of
more
interleukin
(
IL
)
-
8
would
result
in
the
attraction
of
more
PMNs
.
The
aim
of
this
study
was
to
evaluate
the
cytokine
profile
in
PLS
patients
'
blood
cultures
.
Blood
was
sampled
from
eight
PLS
patients
(
one
female
)
from
six
families
(
antiinfective
therapy
completed
:
six
;
edentulous
:
two
)
with
confirmed
cathepsin
C
mutations
and
deficient
enzyme
activity
.
Nine
healthy
males
served
as
controls
.
Whole
blood
cultures
were
stimulated
with
highly
pure
lipopolysaccharide
(
LPS
)
from
Escherichia
coli
R
515
and
IL
-
1
β
plus
tumor
necrosis
factor
(
TNF
)
-
α
.
Thereafter
,
release
of
IL
-
1
β
(
stimulation
:
LPS
and
LPS
plus
adenosine
triphosphate
)
,
IL
-
6
,
IL
-
8
,
interferon-inducible
protein
(
IP
)
-
10
,
and
interferon
(
IFN
)
-
γ
(
stimulation
:
LPS
,
IL
-
1
β
/
TNF
α
)
were
detected
by
ELISA
.
Medians
of
cytokine
release
were
,
with
the
exception
of
IP-
10
,
slightly
higher
for
PLS
than
for
controls
'
cultures
.
None
of
these
differences
reached
statistical
significance
.
Increased
production
of
IL
-
1
β
,
IL
-
6
,
IL
-
8
,
IP-
10
,
or
IFN
γ
as
a
significant
means
to
compensate
for
diminished
activity
and
stability
of
polymorphonuclear
leukocyte-derived
proteases
could
not
be
confirmed
in
this
study
.
Cytokine
profiles
in
blood
cultures
may
not
be
used
to
identify
PLS
patients
.
Diseases
Validation
Diseases presenting
"more proinflammatory cytokines to compensate for their reduced capacity to neutralize leukotoxin"
symptom
papillon-lefèvre syndrome
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