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FGFR3-related condition: a skeletal dysplasia with similarities to thanatophoric dysplasia and SADDAN due to Lys650Met.
[achondroplasia]
Mutations
in
the
fibroblast
growth
factor
receptor
3
(
FGFR
3
)
gene
account
for
six
related
skeletal
dysplasia
conditions
:
achondroplasia
,
hypochondroplasia
,
thanatophoric
dysplasia
types
1
and
2
,
SADDAN
(
severe
achondroplasia
with
developmental
delay
and
acanthosis
nigricans
)
,
and
platyspondylic
lethal
skeletal
dysplasia
,
San
Diego
type
.
This
group
of
disorders
has
very
characteristic
clinical
and
radiologic
features
,
which
distinguish
them
from
other
skeletal
dysplasias
.
They
display
a
spectrum
of
severity
in
the
skeletal
findings
,
ranging
from
relatively
mild
hypochondroplasia
to
lethal
thanatophoric
dysplasia
.
We
report
a
patient
who
has
the
missense
FGFR
3
mutation
,
Lys
650
M
et
,
previously
reported
in
association
only
with
SADDAN
,
who
exhibits
some
findings
similar
to
both
thanatophoric
dysplasia
(
types
1
and
2
)
in
addition
to
those
findings
characteristic
of
SADDAN
.
Diseases
Validation
Diseases presenting
"ranging from relatively mild hypochondroplasia to lethal thanatophoric dysplasia"
symptom
achondroplasia
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