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A systematic review of clinical trials of treatments for the congenital ichthyoses, excluding ichthyosis vulgaris.
[lamellar ichthyosis]
The
ichthyoses
comprise
a
group
of
inherited
disorders
of
keratinization
.
Because
of
the
need
for
lifelong
treatment
,
it
is
important
that
therapies
are
beneficial
,
safe
,
and
well
tolerated
.
We
sought
to
review
the
evidence
on
existing
treatments
for
the
congenital
ichthyoses
,
excluding
ichthyosis
vulgaris
.
We
undertook
a
systematic
review
using
the
methodology
of
the
Cochrane
Collaboration
.
Articles
published
in
MEDLINE
,
EMBASE
,
and
CENTRAL
and
registered
clinical
trials
were
screened
.
Randomized
controlled
trials
involving
patients
with
the
inherited
ichthyoses
,
either
syndromic
or
nonsyndromic
but
excluding
ichthyosis
vulgaris
,
were
considered
.
Six
trials
met
the
inclusion
criteria
.
Topical
treatments
including
5
%
urea
,
20
%
propylene
glycol
alone
or
in
combination
with
5
%
lactic
acid
,
calcipotriol
ointment
,
and
liarozole
5
%
cream
showed
therapeutic
benefit
.
Oral
liarozole
,
a
retinoic
acid
metabolism
blocking
agent
,
showed
no
advantage
over
oral
acitretin
.
Most
studies
were
performed
on
a
small
sample
of
patients
and
lacked
methodological
and
reporting
quality
.
The
small
number
of
trials
and
the
nearly
constant
positive
results
make
publication
bias
likely
.
The
absence
of
standardization
of
outcome
measures
precluded
the
comparison
of
studies
.
Topical
treatments
including
emollients
,
calcipotriol
ointment
,
and
liarozole
cream
seem
to
have
therapeutic
benefit
and
a
good
safety
profile
,
although
the
use
of
topical
calcipotriol
is
limited
by
a
maximum
weekly
dose
of
100
g
.
The
advantage
of
oral
liarozole
over
acitretin
is
uncertain
.
Multicenter
trials
comparing
oral
and
topical
interventions
and
evaluation
of
long
-term
outcomes
are
needed
.
Diseases
Validation
Diseases presenting
"long-term outcomes"
symptom
acute rheumatic fever
alpha-thalassemia
aromatase deficiency
cholangiocarcinoma
classical phenylketonuria
congenital adrenal hyperplasia
congenital diaphragmatic hernia
cushing syndrome
dystrophic epidermolysis bullosa
esophageal squamous cell carcinoma
hirschsprung disease
homocystinuria without methylmalonic aciduria
kabuki syndrome
lamellar ichthyosis
omenn syndrome
phenylketonuria
proteus syndrome
trochlear dysplasia
von hippel-lindau disease
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