From www.bloodjournal.org by guest on June 16, 2017. For personal use only.
Hereditary
Methemoglobinemia
in
and
E. M.
By
H
EREDITARY
by
changes.
been
and,
as
shown
Huennekens
et al.2’
an
of
cells,
suggested
cells.
methylene
Eder,
cleotide,
in
enzyme
Finch
and
a co-enzyme
report
tively
high.
in this
intake
that
state
Ilot
the
red
fully
understood.
rate
from
of reduction
of
methemoglobinernia
of the
in
missing
showed
diapilorases,
in
reductase
the
because
however,
describes
in Alaskan
globin
dietary
is
methemoglobin
known
trait.3
metilemo-
effective-
methemoglohinernic
in methemoglobinemic
that
flavin
I)resent
was
is
marked
adenine
dinu-
in normal
amounts
cells.
present
globinernia
tilat
was
Avail-
to
reduced
hereditary
diaphorase
McKee,8
of the
in methemoglobinemic
The
reducing
have
100.
a recessive
a inethernoglohin
showed
largely
cases
substrate.
The oxidation
which
in turn
reduce
met-
nucleotides
RyOI16
50
than
as
mechanism
Gibson,7
the
the
oxidized
pathologic
oxidation
in
to isolate
in the
more
as
char-
other
than
are
glucose
with
blue
that
is kept
and
condition
no
fewer
there
spontaneous
persons
rare
and
Gibson1
utilizes
pyridine
of
very
is inherited
a slow
which
Sievers
a
of hemoglobin
that
condition
attempted
cells
in normal
ness
cells.
and
by
suggests
transfer
have
in
than
amount
Warburg,4
electron
et al.3
methemoglobin
lower
by
System
reduced
by
cells.
Barcroft
is
review
Dines2
HosKiNs
polycythemia,
that the
umidergoes
enzymatic
generates
hemoglobin
red
D.
DALE
a variable
to a recent
although
able evidence
indicates
In air, hemoglobin
cell by an
of glucose
AND
a compensatory
According
reported,
globin
Scorr
cyanosis,
methemoglobin,
Eskimos
Indians
METHEMOGLOBINEMIA
acterized
form
Alaskan
an epidemiologic
Eskimos
Evidence
will
condition
of ascorbic
and
be
preselltel
varies
acid.
study
Indians,
with
where
to
some
of hereditary
its
show
methemno-
prevaleiice
that
the
environmental
is compara-
level
of
methenlo-
which
may
be
determnineti
by
the
Beckman
spectro-
colorinieter.
Where
factor
MFTHODS
Henoglobin
method
of
photometer
or
mnethemoglobin
Rate
Washed
After
From
was
of
(iet(’rmflined
Evelyn
a
No.
was
as
oxylmemnoglobin.’
Malloy,’#{176}using
and
62
filter
found,
methemoglobin
an
with
the
an excess
repeatedly
the Arctic Health
Research
Center,
Public
and Welfare,
Anchorage,
Alaska.
Oct. 10, 1957; accepted
for publication
authors
wish to thank
Drs. Helen Whaley,
who
in
brought
field
and
several
hospital
of
these
cases
to
their
attention,
investigations.
795
was
with
Ill/i
was
determined
Education
Submitted
The
635
the
photoelectric
correction
was
red cells were treated
with
2 hours, the cells were washed
at
Klett-Sunlnmerson
appropriate
reduction
1etI1tn1ogloi)iI1
absorption
in
applied
both
of sodium
with
to
whole
the
cells
(2
nitrite
hemoglobin
and
ng.
phosphate-buffered
Health
March
Harriet
and
Service,
25,
per
ml.
cells).
( 9 vols.
saline
Department
of
Health,
1958.
C. Jackson
many
levels.
hemolyzates.
other
and
persons
Jean
C.
who
Persons
assisted
From www.bloodjournal.org by guest on June 16, 2017. For personal use only.
796
-
.
0.9%
sodium
cells,
200
chloride
plus
glucose
per
mg.
duction
was
followed
termination.
1 vol.
ml.
at
of
water.
The
cleotide.
Serum
ascorbic
modified
for
methemoglobm,
of
acid
as
of
pH
withdrawal
cells
with
made
by
the
for
by
0.7
freezing
of
15
persons
oxidized
whole
the
of
or
rate
by
per
jsnloles
method
HOSKINS
re-
mnethemoglobin
contained
the
AND
and
samples
hemolyzates
and
determined
To
substrate
of
were
the
7.35).
reducing
glucose-6-phosphate
was
volumes
buffer,
added
oxidized
system
j.tmoles
larger
-1 phosphate
were
periodic
the
reducing
20
0.1
cells
38#{176}by
Hemolyzates
distilled
SCOTT
-
dc.
addition
ml. : 3
of
jtmoies
triphosphopyridine
Bessey,
nu-
Lowry
and
Brock,”
serum.
RESULTS
Occurrence
of
Met heinoglobinemia
Methemoglobin
was
demonstrated
in
in
9
Eskimo
families
as shown
in table
1. In addition,
were
reported
to be cyanotic,
but have
two newborn
not yet been
tested.
persons
in six
families
because
presented
out
other
symptoms.
any
tives of the known
The geographic
Because
tend
of
Alaska.
are
great
fact,
Ill
Definite
shown
is also
child
treatment
pital,
was
He
cent
IV-1
ascorbic
acid
per
of
cent
while
was
acid
only
change
ascorbic
over
during
acid
30 per
Enzymatic
The
case
cases,
families
areas
families;
a distant
in
these
relationship
VII
and
VIII,
of
of her
by
total
total
and
to
week
and
hospital
11.7 per
level
was
hospital,
and
weeks
on
hemoglobin
hos-
given
level
fell
of
no
of
9.7
detectable
ascorbic
of therapy.
After
two weeks
ml. One month
after
release
of therapy,
from
the
again
of
methemoglobin
was
this
was
mg.
to
400
methemoglobin;
hemoglobin
mg.
ml.
her
mg.
75
During
this period
At this
time
her
there
400
was
this
in the
received
diet.
cent.
1.6 mg./100
to the
was
after
methemoglobin.
ml. She was
then
her methemoglobin
VII-2
two
hemlloglobin
another
weeks
hemoglobin
After
total
injection,
After
three
acid
of patient
serum.
of his
reverted
for
1.4 mg./100
a week,
and
of
cent
acid
per
did
not
serum
hospital,
methemoglobin.
Studies
capacity
of
1.
Indians
distinct
families
blue
to that in her
from
19.3 to
four more
weeks
was 1.6 mg./100
cent
in figure
of these
four
between
detected.
had
hospital
ascorbic
cent
cent
be
hemoglobin
tile
serum
per
per
methylene
could
level
was
day for
in her
8.8
rela-
Acid
25 per
given
of admission
37.3
Ascorbic
in addition
level
fell
her
time
ascorbic
day,
per
all
proximity,
V and
available
and
four
between
found
an(1
hospitalized,
ilis
ill
daily,
ascorbic
acid
At the
level
Ill
and
was
was
nIetilemoglobill
serum
ascorbic
he
in
eight
with-
shown
that
occur
15,
cyanosis
Eskimos
unlikely
Indian
families
these
all
are
because
to
Of
testing
Alaska
in
and
of geographic
families
by
or
in these
found.
1-3
8.4 per
found
appears
could
no methemoglobin
Patient
her
it is extremely
condition
Blue
nlethemoglobin.
they
families
area,
2. Because
of Methylene
\Vhen
of these
involved,
between
was
were
distances
the
in figure
none
Effects
otilers
relationships
possible
although
The
in a given
related.
discovered
cases.
locations
the
to stay
are
were
infants
of red
cells
three
methemoglobinemic
the reducing
capacity
hemolyzates,
glucose-6-phosphate
to reduce
methemoglobin
persons
was
below
plus
was
determined
as shown
in table
normal.
As reducing
either
di-
or
2.
In all
substrates
triphosphopyridine
in the
three
in
nu-
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HEREDITARY
797
METHEMOCLOBINEMIA
1.-Cases
TABLE
of Methernoglobinemia
in Alaskan
Eskimos
and
Indians
Present
Family
Siblings
Age
Years
1
10
NI
13.8,14.6
2
3
7
5
4
F
13.9,12.9
M
16.0,15.4
4.0,7.0
Sept.
‘55,
NI
14.0,13.1
1.9,5.2
I)ec.
‘56
3
F
12.0,14.5
1.0,4.7
2
F
11.8,
2.63.6
4
5
6
7
III
Hemoglobin
Gm./100
ml.
Sex
<1
?
9.2
-‘
Methemoglobin
Gm./100
ml.
Race
Remarks
0,0
Ingalik
Nleasured
0,0
In(iian
in
_1
1
10
F
13.7,13.1
0,0
Ingalik
Measured
2
3
4
5
6
8
F
NI
14.1,14.6
12.3,12.9
2.2,1.0
0,0
Indian
in
Sept.
‘55,
M
12.9,13.1
0,0
Dec.
‘56
NI
12.9,12.9
7
5
3
___________
<1
0,0
-,l2.6
-,0
_________________
-ii
7
NI
11.4
0
12
5 normal
2
6
NI
12.5
0
Ingalik
half-siblings;
3
4
NI
12.3
0
Indian,
mneasurc(I
4
5
6
3
NI
12.5
5-16
in
1
F
10.7
Koyoknk
Dec.
<1
NI
3.8
0
‘56
Indian,
_1
3
I
16
Vliite
IV
1
F’
16
15.1,16.1
2.8,3.1
Ingalik
Indian
3 normal
half-siblings;
nleaSure(l
V
VI
VII
VIII
1
7
2
5
3
4
1
6
2
3
4
4
3
NI
NI
F’
13.7
13.5
13.2
F
F
M
<1
0
1.0
1.0
2.8
0
3.5
12.9
12.8
11.2
Oct.
‘55,
Jan.
‘57
7/8
Koyokuk
Indian,
April
Measured
1,’8
ascorbic
White
therapy
acid
Eskimo
Measured
Sept.
‘55
?
1
2
22
15
F
11.6
F
17.2
1
2
17
14
F
NI
16.2,1
14.1
3
12
F
13.8
0
VIII-1
NI
NI
M
1 1.9
1 1.3
1 1.9
1 1.3
0
0
0
measured
4
5
6
7
9
7
5
1
F
0
Eskimo
6.2
1.4
Measured
June
3.6,0.5
0
Eskimo
1
9
F
13.5
2
7
F
14.5
3
3
‘Newborn
child
reported
‘Newborn
child,
not
M
to be
half-sibling;
4. 1
first
in
‘48;
others
measured
cyanotic.
0__
5.4
9.5
cyanotic
0
by
medical
‘57
1 normal
Dec.
Ix
‘56,
after
personnel.
Eskimo
in
‘56
Measured
in
June
May
‘57
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798
SCOTT
AND
HOSKINS
I
I
(4,
-
yf
Fmc.
1.-Geographic
present
residence
location
of
one
globinemic
families,
cleotides,
tide,
and
glucose
plus
the reduced
where
about
equally
effective.
In this hemolyzate
blue,
and
adenine
flavin
than
system,
IV-1
and
that
of 1-3
and
no indication
was
obtained.
a number
D-
of substances
normal
last
lipoic
acid
levels
same
circle
the
must
speed
of
methemo-
the
reduc-
present
phosphate,
in much
higher
glutathione,
ergo-
ineffective.
nucleotides,’2
cells.
The
riboflavin
hemoglobin
mobility
absorption
up
nucleowere
but not in its
These
include
riboflavin
be
Homocystine,
were
electrophoretic
of an abnormal
will
riboflavin,
four
in her
the
represents
parents
reducing
substrate
and
normal
cells.
of pvridine
compounds’4
the
of
to be effective.
A and
of
residence.
or L-cystine,
The
concentration
had
present
in the presence
methemoglobinemic
sulfhydryi
and
the
Each
birthplace
triphosphate
and triphosphopyridine
di- and triphosphopyridine
nucleotides
dinucleotide.
had
indicate
from
adenosine
forms
of
co-enzyme
Patient
tides13
arrows
differs
ferricyanide,
physiologic
thioneine,
families.
methemogiohinemic
The
tllis
tion of methemoglobin
absence,
and
in both
methylene
of
family.
as normal
spectrum
nucleo-
of this
patient
hemoglobin
of the
A,
methemoglobin
DIScuSsION
The
Alaskan
principal
Eskimos
methemoglobin
in figure
2 are
cessive
trait.
conclusion
and
from
Indians
is modified
consistent
by
with
this
is
some
the
study
hereditary
is
in
environmental
inheritance
that
origin,
methemoglobinemia
but
factor.
in
that
the
The
relationships
of methemoglobinemia
level
of
as a re-
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HEREDITARY
799
METHEMOCLOBINEM1A
a
UI
2.-Relationship
Fic.
females;
=
I
of
diamond
hemogiobinemic;
four
families
sex
=
crosse(l
II
with
unknown.
synihols
Open
symilbols
All
(It’c#{128}’ase(i.
=
Squares
nlethenloglohinenlia.
normal;
=
living
symbols
SOli(i
persons
males;
=
shown
cirmet-
=
tested
were
for
fllethemOglobin.
That
neighbors
the
and that
of family
condition
of these
they
had had this
VI knew
and were
distinctly
children.
was
blue,
just
The
as
from
appears
others,
knew
to rule
VI-2
for
and
a toxic
between
to show
looked
like
that
condition
cause.
Finally,
the
family
3.4
LX-2
per
+ G$-P
TPN’
hour
and
of
difference
and
11-2,
I,
methylene
the
blue
in rate
normal
results
Hemolyzates
of
Hemolyzatea
and
Whole
Cells
+ Glucose
percent
Control
+ G-6-P
TPN’
per hour
-
13.4
-
-
3.0(1)
2.5 (10)
2.9 ( 1 )
2.4(1)
Vu-i
6.2(1)
5.3 ( 1)
8.0(1)
-
4.4(1)
IX-1
-
5.8(1)
(4)3
-
‘Glucose-6-phosphate
the
and
‘Control
was
3Figures
in parenthesis
reduced
same
triphosphopyridine
age,
race,
are
number
triphosphopyridine
and
(4)
6.5
nucleotide.
sex
of
of
subjects,
from
the
Hemolyzates
Whole
Cells
+ Glucose
percent
Eskimo
child,
persistence
after
methemoglohinemic
that
methemoglobinemia
other
in
with
its recurrence
the parents
VI-3,
were
their
existed
13 months
parents
and
was unusual
example,
V1-1 and
recognized
this
This,
out
the fact that
appearance
of Reduction
of Methemoglohin
by Cells
and
Methemoglobinernic
071(1 Normal
Subjects
2.-Rate
Iv-1
VII-2
VII-2
jtmoles
child
likewise
in IV-1.
was hospitalized
reduction
2, appears
Methemoglobinemic
Patient
30
that
throughout.
methemoglobin
shown
in table
TABLE
knew
II
suggested
its occurrence
One of these
patients
indicated
by
the children’s
since
birth.
For
that their
children,
of family
the
methemoglobinemia
was
that
appearance
concerned
they
parents
different
treatment,
inborn
knew
was
children
as
patient.
determinations.
nucleotide
used
in
place
of
G-6-P
and
TPN.
(10)
and
From www.bloodjournal.org by guest on June 16, 2017. For personal use only.
800
SCOTT
absence
of
an
hemoglobin,
enzyme
as
or
Otiler
suggested
factor
necessary
Sievers
by
and
Gibson,7
on the basis of a differential
globin
reduction
by glucose
and lactate,
of
methemoglobin
phorase
is mediated
and
that
this
the
in reducing
matic
studies
globin
of both
methemoglobin
in
must
be
by reduced
Our
due
er’5
forms
above
globinemia
observations
cited
with
hemoglobin
and
not
to a slow
a dominant
some
substance
but
missing
that
active
the
enzy-
methemo-
reduction
methemoglobinemia
of methemo-
in
our
subjects
is not
M, which
H#{246}rlein and Webthe direct
evidence
in two sub-
it
the
methemoglobinemia
sensitivity
of hemoglobin
has
been
rate
shown
that
to abnormal
of methemoglobin
hemoglobin
M,
like
reduction.22
other
associated
M to oxidation
Further,
abnormal
it has
hemoglobins,
of family
later.
Only
were
factor
in table
affects
methemoglobin
1. Thus
the amount
I was definitely
two
children
less
were
been
sug-
is inherited
cyanotic
in December.
engaged
markedly
in picking
of all
during
the
dietary
source
patient
IV-1
wild
Eskimos
winter
they
almost
months,
and
as effective
doses
as
and
Eskimos
to
occur
in other
population
in
north
the
from
of
have
that
ascorbic
acid.
were
actively
found
detectable
that
serum
to
see
time.
populations.
of about
what
results
on
of ascorbic
methemoglobin
in
over
ascorbic
from
The
intakes
in lowering
Indians,
of which
Navajo,
and several
are all
different
living
found
Indian
we
persons
found
in December,
in September,
may be
I lives
is difficult
at
moderate
appears
not
no
it
vitamin
that
factor
family
hand,
show
indeed
suggest
as larger
are in Athabaskan
the Apache,
the
Greenlandic
other
the
methemoglobinemia
The
Eskimo
cases
language
distinctly
the
tested
obtain
above
with
a frequency
in a total
Eskimo
Alaskan
On
indians
could
presented
Hereditary
in Indians
including
berries.
and
level
in these
of methemoglobin
in September
than
evidently
cyanotic
evidence
indicates
that this environmental
in September,
persons
in the village
where
This
However,
the
Our
Thus,
Indians
occur
inadequate,
equally
in hereditary
mediates
four
be
is
nearly
hemolyzates.
while
may
are
in
methemoglobin
characteristic.
in members
15 months
acid
dia-
of the abnormal
hemoglobin
in a German
family.
Besides
That
an environmental
is evident
from
the data
a third
methemoreduction
as present
in
explanation
component
that
blue on
normal
methemoglobinemia.
it is inactive
This
cell
of
nucleotides.
M is due
that
the
indicate
above,
gested2
that
the
is postulated
nucleotides
normal
reduction
Barcroft.a
in hereditary
pyridine
HOSKINS
niicleotide-linked
diaphorase
cells,
is added.
show
pyridine
to the presence
first observed
jects
is missing
methemoglobinemic
methylene
blue
reduced
complete
by
effect
of methylene
has inferred
that
nucleotide-linked
both normal
and
reduction
unless
and
a (liphosphopyridme
diaphorase
A triphosphopyridine
since
by
for
Ryan
AND
Alaskan
acid
level.
Eskimos
Thus,
these
20,000.
All the
and
cases
cases
there
are many
subdivisions,
Canadian
and Alaskan
tribes.
southern
group
of Eskimos
who
speak
that
spoken
by other
Eskimos,
including
Norton
Sound
and
Siberian,
Canadian,
a
and
Eskimos.
apparent
be in part
globinemia
high
the result
normally
frequency
of the
depends
of
occurrence
environmental
on observation
in
Indians
and
factor.
Recognition
of cyanosis,
and
Eskimos
of
in
may
methemoour experi-
From www.bloodjournal.org by guest on June 16, 2017. For personal use only.
HEREDITARY
ence,
at least
is evident.
20 per
Ample
globin
be
to
only
intakes.
cent
of the
ascorbic
present
evident
acid
801
METHEMOCLOBINEMIA
hemoglobin
acid
below
this
in persons
can
level.
with
In six other
be
cyanosis
genetic
where
oxidized
before
by reducing
Consequently,
the
reports
must
prevent
cyanosis
the
methemo-
methemoglobinemia
factor
serum
who
ascorbic
also
have
acid
was
might
low
ascorbic
measured,6’5’
low levels
(0.2 to 0.5 mg./100
ml. ) were
found.
All of our cases
are in children,
of which
the oldest
is now 17 years
of age.
520 Eskimo
men from
all parts
of Alaska,
as well as over
1,200 other
Eskimos
16-19
and
Indians
including
all
the known
cases,
have
results.
The
observed
chance,
mortality.
cago
or
it may
However,
Hospital
available
indicate
an
case VIII-1
when
living
been
screened
for
exclusive
occurrence
the
child
association
was first
was
and
near
years
old.
At
and eight
the possibility
that
neighbors
of
with
negative
be a result
of
may
of methemoglobinemia
discovered
at the
eight
methemoglobin,
This
suggests
relatives
methemoglobinemia
in children
with
University
time,
22.2
early
of Chi-
per
cent
years
later
only 4.8 per
that
methemoglobinemia
cent
of the hemoglobin
was
methemoglobin.
was
tends
to disappear
of cases
in family
while
both
parents
with
age. If it does,
this might
explain
the large
number
I. In this family,
five of seven
children
have
the condition
do not. If the character
is recessive,
its occurrence
in five
out of seven
children
would
be expected
to occur
If however
the condition
tends
to disappear
with
and
may
have
rison2#{176}also
zygous
found
the
been
in only one out
age, one parent
methemoglobinemic
condition
ill
five
as a child.
of
children
seven
of 70 families.
may be homo-
Gibson
and
Har-
in a family.
Surmiii
Methemoglobinemia
in whom
pears
to be
duction
probably
due
cases
to the
frequent
absence
amount
of methemoglobin
depending
on some
this
environmental
dc
Alaska.
Iste
anormalitate
Le
personas
suggere
IN
casos
de
es variabile.
Illo
que iste influentia
Indians
known.
It ap-
mediates
This
time
The
the
re-
anomaly
in these
evidence
is
persons
suggests
of ascorbic
is
acid.
INTERLLNGUA
frequente
es
de
un
per
presente
(lepende
de
es le ingestion
and
are
which
intake
in
eschimos
cognoscite
in
e
tin
factor
erythrocytic
reducite
nucleotidos
hereditabile
methemoglohina
cells
any one
influence.
dietary
confirmate
resultar
del absentia
de methemoglohina
Eskimos
20,000
nucleotides.
at
is tile
es probabilemente
quantitate
pridine
inusualmente
Dece-cinque
de 20.000.
JIb pare
mediar
le reduction
in red
present
environmental
SuxIARIo
es
of about
of a factor
influence
Methemoglobinemia
in Alaskan
in a population
of methemoglobin
by reduced
inherited
as a recessive
trait.
The
variable,
that
is unusually
15 confirmed
como
a tin
tin influentia
dietari
de
tin
que
tracto
momento
ilidianos
population
deherea
pyridinic.
recessive.
particular
ab le ambiente.
acido
ascorbic.
in iste
Le
datos
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1958 13: 795-802
Hereditary Methemoglobinemia in Alaskan Eskimos and Indians
E. M. SCOTT and DALE D. HOSKINS
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