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Severe α-thalassemia intermedia due to a compound heterozygosity for the highly unstable Hb Adana (HBA2: c.179G>A) and a novel codon 24 (HBA2: c.75T>A) mutation.
[alpha-thalassemia]
We
report
a
novel
mutation
at
codon
24
of
the
α
2
-
globin
gene
(
HBA
2
:
c
.
75
T
 
>
 
A
)
found
in
a
Sundanese
family
.
This
novel
mutation
was
detected
during
prenatal
diagnosis
.
The
couple
already
had
a
7
-
year
-old
boy
who
exhibited
clinically
severe
α-thalassemia
intermedia
(
α-
TI
)
,
and
he
was
found
to
be
a
compound
heterozygote
for
the
novel
mutation
at
codon
24
and
the
previously
described
Hb
Adana
(
HBA
2
:
c
.
179
G
 
>
 
A
)
at
codon
59
of
the
α
2
-
globin
gene
.
The
father
was
a
carrier
of
the
novel
point
mutation
and
showed
normal
hemoglobin
(
Hb
)
and
a
low
mean
corpuscular
volume
(
MCV
)
and
mean
corpuscular
Hb
(
MCH
)
value
.
Diseases
Validation
Diseases presenting
"clinically severe α-thalassemia intermedia"
symptom
alpha-thalassemia
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