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Proteus syndrome: Clinical profile of six patients and review of literature.
[proteus syndrome]
Proteus
syndrome
(
PS
)
is
characterized
by
patchy
or
segmental
overgrowth
and
hyperplasia
of
multiple
tissues
and
organs
,
along
with
susceptibility
to
development
of
tumors
.
Very
few
cases
are
reported
in
literature
from
developing
countries
.
Due
to
certain
overlapping
features
with
other
overgrowth
syndromes
,
diagnosis
is
usually
delayed
.
Our
aim
was
to
describe
clinical
profile
of
this
rare
condition
in
six
patients
.
Retrospective
case
sheet
review
of
patients
followed
in
a
Pediatric
Genetic
and
Metabolic
clinic
at
a
tertiary
care
institute
of
North
India
with
a
diagnosis
of
hemihypertrophy
/
overgrowth
syndrome
.
Six
cases
presented
with
asymmetric
overgrowth
and
peculiar
features
suggestive
of
PS
were
included
in
this
study
.
Age
at
presentation
was
2
months
to
10
years
;
two
were
males
and
four
were
females
.
Hemihypertrophy
was
noticed
in
only
one
case
at
birth
,
and
focal
overgrowths
in
rest
of
other
patients
were
seen
later
during
childhood
.
Due
to
certain
overlapping
features
with
other
overgrowth
syndromes
,
diagnosis
of
PS
is
usually
delayed
.
Pediatricians
are
the
first
persons
who
come
across
such
patients
and
they
should
be
aware
about
this
rare
condition
.
