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Pulmonary manifestations and management of proteus syndrome.
[proteus syndrome]
Proteus
syndrome
is
a
very
rare
,
sporadic
and
congenital
condition
that
is
characterized
by
postnatal
mosaic
overgrowth
.
This
disorder
is
thought
to
be
caused
by
a
somatic
gene
mutation
,
but
the
exact
etiology
is
unknown
.
Commonly
involved
tissues
include
connective
tissue
,
bone
,
skin
and
the
central
nervous
system
.
Another
less
common
symptom
involves
pulmonary
emphysematous
changes
.
This
report
documents
a
25
-
year
-old
man
with
Proteus
syndrome
who
presented
with
progressive
exertional
dyspnea
and
asymmetric
overgrowth
of
his
extremities
.
He
underwent
left
pneumonectomy
and
his
postoperative
course
was
uneventful
.
Lung
tissue
showed
emphysematous
changes
with
multiple
bulla
formation
and
scattered
calcification
.
We
also
review
recent
literature
related
to
pulmonary
manifestations
and
management
of
Proteus
syndrome
.
Diseases
Validation
Diseases presenting
"pulmonary emphysematous changes"
symptom
proteus syndrome
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