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[General anesthesia in Cohen syndrome. Report of a clinical case].
[cohen syndrome]
The
case
of
a
23
years
old
woman
,
affected
by
the
Cohen
syndrome
,
who
underwent
general
anesthesia
for
extensive
dental
surgery
,
is
reported
.
The
Cohen
syndrome
is
an
autosomal
recessive
syndrome
that
causes
mental
retardation
,
obesity
,
short
stature
as
well
as
oral
,
ocular
,
and
limb
anomalies
.
The
problems
the
anesthesiologist
could
deal
with
include
the
capacity
of
the
patient
to
cooperate
;
difficult
intubation
because
of
maxillary
hypoplasia
,
micrognathia
,
narrow
and
high-arched
palate
,
and
prominent
maxillary
central
incisors
;
generalized
muscular
hypotonia
;
moderate
leukopenia
,
that
could
theoretically
increase
the
risk
of
infection
:
and
,
finally
,
possible
associated
mitral
valve
prolapse
or
hiatus
hernia
.
In
the
case
reported
the
presence
of
mitral
valve
prolapse
or
hiatus
hernia
was
ruled
out
echographically
.
The
patient
was
premedicated
with
diazepam
and
atropine
i
.
m
.
;
general
anesthesia
was
carried
out
by
propofol-fentanyl
association
and
myorelaxation
was
obtained
with
atracurium
.
Nasotracheal
intubation
was
performed
easily
in
spite
of
oral
anomalies
so
that
the
usefulness
of
thyromental
distance
,
which
was
7
cm
long
,
as
a
clinical
test
to
evaluate
a
potentially
difficult
intubation
was
confirmed
.
Noteworthy
,
the
thyromental
distance
was
the
only
test
which
was
suitable
for
the
uncooperative
patient
.
At
the
end
of
surgery
muscular
tone
recovered
promptly
and
the
endotracheal
tube
could
be
regularly
removed
.
No
complication
was
registered
postoperatively
.
Diseases
Validation
Diseases presenting
"mental retardation"
symptom
achondroplasia
alexander disease
alpha-thalassemia
aniridia
aromatase deficiency
canavan disease
classical phenylketonuria
coats disease
cohen syndrome
cowden syndrome
cystinuria
dentin dysplasia
familial hypocalciuric hypercalcemia
homocystinuria without methylmalonic aciduria
hydrocephalus with stenosis of the aqueduct of sylvius
kabuki syndrome
kallmann syndrome
lamellar ichthyosis
lymphangioleiomyomatosis
monosomy 21
phenylketonuria
primary hyperoxaluria type 1
proteus syndrome
pyruvate dehydrogenase deficiency
sneddon syndrome
triple a syndrome
wolf-hirschhorn syndrome
zellweger syndrome
This symptom has already been validated