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Systemic capillary leak syndrome.
[systemic capillary leak syndrome]
Systemic
capillary
leak
syndrome
is
a
rare
condition
characterized
by
unexplained
episodic
capillary
hyperpermeability
.
A
shift
of
fluid
and
protein
from
the
intravascular
to
the
interstitial
space
results
in
hypovolaemia
.
Attacks
vary
in
frequency
,
severity
and
duration
and
can
be
fatal
,
although
they
often
remit
spontaneously
.
During
acute
episodes
there
is
a
pathognomonic
elevated
haematocrit
and
reduced
serum
albumin
,
frequently
associated
with
a
monoclonal
gammopathy
.
Treatment
has
been
largely
empirical
but
there
are
anecdotal
reports
of
beneficial
therapy
.
We
describe
a
further
case
which
highlights
the
typical
clinical
presentation
,
course
and
investigate
findings
and
review
the
other
cases
described
.