Rare Diseases Symptoms Automatic Extraction
Home
A random Abstract
Our Project
Our Team
Idiopathic pulmonary arterial hypertension in a young patient with the Cohen syndrome.
[cohen syndrome]
We
admitted
a
16
-
year
-old
boy
with
the
Cohen
syndrome
to
our
institution
for
increasing
dyspnoea
.
Investigations
revealed
idiopathic
pulmonary
hypertension
.
He
was
commenced
on
bosentan
and
oral
anticoagulation
and
was
followed
up
for
nearly
7
years
,
during
which
he
was
readmitted
for
dynamic
measurements
of
pulmonary
artery
pressure
.
Despite
initial
improvement
,
the
right
heart
pressures
increased
again
and
sildenafil
was
added
.
His
final
hospitalisation
was
due
to
increasing
breathlessness
and
episodes
of
syncope
.
The
addition
of
prostacyclin
conferred
no
reduction
in
pulmonary
artery
pressure
.
The
patient
suffered
a
cardiac
arrest
and
remained
intubated
for
2
weeks
,
during
which
Klebsiella
pneumonia
and
superinfection
with
the
H
1
N
1
swine
flu
virus
occurred
.
The
patient
died
due
to
multi-organ
failure
,
nearly
7
years
after
his
initial
diagnosis
.
The
Cohen
syndrome
,
its
phenotype
and
clinical
findings
,
and
the
incidence
and
treatment
of
pulmonary
hypertension
are
discussed
.