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Hypohidrotic ectodermal dysplasia: a multidisciplinary approach.
[oligodontia]
Hypohidrotic
ectodermal
dysplasia
(
HED
;
Christ-
Siemens
-
Touraine
syndrome
)
is
a
genetic
disorder
characterized
by
sparse
hair
,
oligodontia
with
peg-shaped
teeth
,
reduced
sweating
,
and
defects
in
a
number
of
other
ectodermal
organs
.
A
partial
or
complete
absence
of
eccrine
glands
can
lead
to
recurrent
severe
overheating
that
may
cause
seizures
and
neurological
deficits
.
This
clinical
report
presents
a
14
-
year
-old
male
patient
with
hypohidrotic
ectodermal
dysplasia
,
including
the
clinical
and
radiographic
findings
,
and
multidisciplinary
treatment
.
The
Schedule
for
Affective
Disorders
and
Schizophrenia
for
School
Aged
Children-
Present
and
Lifetime
Version
(
K-SADS-PL
)
was
administered
to
patient
along
with
mother
to
assess
for
any
psychiatric
disorders
.
The
screening
and
rating
scales
completed
by
mother
and
two
teachers
to
evaluate
the
severity
of
attention
deficit
hyperactivity
disorder
(
ADHD
)
symptoms
,
and
other
behavioral
problems
.
Patient
's
academic
performance
,
adaptive
functioning
,
and
problem
behavior
was
evaluated
using
.
The
Teacher
Report
Form
.
Mental
capacity
was
assessed
with
the
Wechsler
Intelligence
Scale
for
Children
(
WISC-R
)
.
Illness
Perception
Questionnaire
Revised
was
used
to
systematically
assess
illness
representation
attributes
and
emotional
representations
of
illness
.
On
the
psychiatric
diagnosis
assessment
using
K-SADS-PL
sub-threshold
attention
deficits
and
anxiety
symptoms
were
determined
.
In
this
case
we
established
a
multidisciplinary
approach
in
his
treatment
with
pediatric
,
dermatological
,
and
dental
examinations
,
beside
his
psychiatric
evaluation
.
The
prosthetic
rehabilitation
included
restoring
upper
teeth
with
copings
and
fabrication
of
upper
and
lower
complete
dentures
.
Metal
framework
was
not
incorporated
in
the
partial
denture
design
allowing
modifications
as
the
oral
and
maxillofacial
development
continued
.
Removable
complete
or
partial
dentures
without
metal
framework
is
a
treatment
of
choice
until
the
completion
of
facial
growth
at
which
definitive
treatment
is
considered
.
Diseases
Validation
Diseases presenting
"absence of eccrine glands"
symptom
oligodontia
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