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Elsahy-Waters syndrome: Evidence for autosomal recessive inheritance.
[dentin dysplasia]
Elsahy-
Waters
or
branchioskeletogenital
syndrome
is
a
rare
MCA
/
MR
syndrome
characterized
by
moderate
mental
retardation
,
hypospadias
and
characteristic
craniofacial
morphology
,
which
includes
brachycephaly
,
facial
asymmetry
,
exotropia
,
hypertelorism
/
telechantus
,
broad
nose
,
concave
nasal
ridge
,
underdeveloped
midface
,
prognathism
,
and
radicular
dentin
dysplasia
.
Here
we
report
on
a
44
-
year
-old
woman
and
her
45
-
year
-old
brother
,
born
to
consanguineous
parents
,
who
show
a
striking
resemblance
to
the
earlier
described
patients
.
The
hitherto
reported
patients
were
male
and
in
one
pedigree
parents
were
consanguineous
.
The
present
report
of
an
affected
woman
and
her
brother
,
born
to
consanguineous
parents
,
supports
autosomal
recessive
inheritance
of
this
condition
.
We
provide
a
short
review
of
all
previously
reported
patients
with
Elsahy-
Waters
syndrome
and
related
entities
.
Diseases
Validation
Diseases presenting
"related entities"
symptom
dentin dysplasia
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