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Ichthyosis prematurity syndrome with separation of fetal membranes and neonatal asphyxia.
[aniridia]
Ichthyosis
prematurity
syndrome
(
IPS
)
is
a
rare
inherited
skin
disorder
.
Children
are
born
prematurely
with
thick
skin
and
have
been
found
to
develop
neonatal
asphyxia
due
to
occlusions
in
the
bronchial
tree
from
debris
in
the
amniotic
fluid
.
At
31
weeks
of
gestation
,
separation
of
amniotic
and
chorionic
membranes
was
identified
as
well
as
polyhydramnion
.
The
child
was
born
2
weeks
later
,
with
thickened
skin
with
a
granular
appearance
and
required
immediate
ventilation
and
intensive
care
.
At
2
years
of
age
,
the
patient
has
developed
an
atopic
skin
condition
with
severe
itching
,
recurrent
skin
infections
,
food
intolerance
and
periods
of
wheezing
.
Prenatal
observation
of
separation
of
foetal
membranes
or
dense
amniotic
fluid
may
be
signs
of
IPS
and
severe
complication
immediately
after
birth
.
Diseases
Validation
Diseases presenting
"severe complication"
symptom
allergic bronchopulmonary aspergillosis
alpha-thalassemia
aniridia
dystrophic epidermolysis bullosa
erythropoietic protoporphyria
familial mediterranean fever
heparin-induced thrombocytopenia
junctional epidermolysis bullosa
primary hyperoxaluria type 1
severe combined immunodeficiency
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