From www.bloodjournal.org by guest on June 14, 2017. For personal use only.
Toxic
By
T
HE
EXCESSIVE
porphyrin
occurs
in
various
the
It is well
intake
following
that
compounds
was
in
mides,
man
other
sulfa
methyichioride
of
porphyrinogenic
sists
in an increase
the
Later
Fischer
phyrins
in
peated
these
During
This
produced
and
of
by
trional
last
with
few
based
years,
on
rats.2
may
has
in
and
fed
porwas
sulfanilafollowing
after
and
admin-
thiosinamine.
animals
usually
con-
excretion.
sulfonal,
Ellinger
which
of
sulfonal.6
the
with
observed
and
1895
related
noted
occurs
been
man
traces
in
and
Porphyrinuria
rats
coproporphyrin
negative
a
the
Stokvis
of sulfonal
in
porphyrins
fed
and
porphyrinuria.
by
cinchophen
drugs
fecal
detected
experiments
the
it
these
rabbits
and
Brownlee
in
choral,
uro-type
metabolism.
Coproporphyrinuria
and
Duesberg
urine
proposed
which
of
urine
1891.1
and
barbiturates,
in the
the
in
groups
anilin.3’4
that
diseases
produce
1849
action
Occasionally
of urinary
toxemia
can
in
sulfanilamide
and
action
found
been
drugs
arsphenamine,
The
In
with
intoxication.5
istration
(1916)
treated
porphyrin
various
poisonS
aromatic
of
porphyrinuria
poisons.
Garrod
Salkowsky
certain
disturbances
the
in
of
porphyrinogenic
by
of
idiopathic
from
alterations
and
by
in
course
and
drugs
The
described
action
observed
with
observed
intoxication.
phyrinogenic
the
drugs
certain
was
lead
in
of various
known
seen
differentiated
observed
A. OZEN
MUHLIS
be
associated
he
Coproporphyrinuria
AND
excretion
must
diseases
can
from
CETINGIL
porphyrin
metabolism
Porphyrinuria
result
I.
ARIF
Porphyria
are
similar
and
Riesser
insoluble
in
ether.
etherinsoluble
Waldenstr#{246}m
and
porWendt
re-
results.7
new
classification
work
done
in
of
the
human
porphyria
laboratories
of
has
Watson,8’9
is as follows:
was
1.
Erythropoietic
2.
Hepatic
in
a.
intermittent
I).
cutanea
c.
mixed
part
same
laboratories.101
type
III
in
together
type
duced
the
with
From
(Director:
the
by
Schmid
bone
et
marrow
of
al.’1
by
experimental
found
and
porphyria
an
increase
erythrocytes
They
marrow.11”2
et al.’3
tha 2nd
Internal
Clinic,
Prof. A. I. Cetingil).
Sept.
studies
porphyrins.
bone
Schwartz
We are greatly
Dr. M. Bassipahi,
Submitted
acute
tarda
supported
uro-type
I from
by
porphyria
porphyria
were
of
able
Medical
porphyria”
animals
School
lead
to crystallize
“Experimental
intoxicating
of
of
with
the
made
in
intoxited
rabbits
uroporphyrin
was
first
1959;
accepted
for
publication
1002
Feb.
Istanbul
12,
pro-
phenylhydrazine
University,
indebted
to Fikret Biyal, who made the fluorometric
readings,
Dr. E. Ozkan and Mrs. Civas for their technical
assistance.
13,
the
coproporphyrin
1960.
and
Turkey
and
to
From www.bloodjournal.org by guest on June 14, 2017. For personal use only.
‘l OXIC
10()3
PORi’IIYRIA
lead and exposing
rabbits
resembled
it exhibited
Stan(i1)oimit
hepatic
cutamiea
and
Schmid
in rabbits
“experimental
porphyria,
all three
of
imiterniittent
and
resembling
This
agent.
the
features
tarda
in character.
iii
them
to light.
This
humami
erythropoietic
Schwartztm
also
I
human
hepatic
is
associated
disturbance
treated
with
Sedormid
were
exhibited
fOun(l
to
of
StIm(Iy
man
experimental
toxic
An
cases
1956
Niore
bakir,
of
by
sent
Cihat
Cam,
He
has
commonly
affected
and
and
6 females).
noted
that
the
treated
with
Sedor-
of
aninials
most
other
likely
fungicide
to
to study
the
The
following
toxic
clinic.
Similar
the
In
the
Of
the
were
appeared
the
earliest
consummliption
of
cases
“porphyria
idiopathic
the
mast
extremiiities,
on
the
after
admission
children
i.e.,
chest.
on
They
mechanical
to
the
the
disease
one
the
the
the
who
varied
of
eaten
all
not
(31
have
fungicide-
disease
including
that
the
was
used
the
in more
Cam
disease
as
a
opportunity
detail.
clinic.
an
attributem!
6)
and
at
was
error
from
parts
District
the
of
the
to
latest
children
presemice
metabolism,”
defect,
sign
was
sunlight.
a
of
tile
of
acquired
to
size
in the
be
Diyarbakir,
poisoned!
4 were
wheat,
3 years
(case
of
signs
and
as
Garrod
had
produced
by
toxic
2)
after
1).
exposed!
fouir
the
cereals,
concluded
our
Bismiiil
first
of
frequently
The
other
did
found
Cihat
eaten
which
in
to
the
in
irritation
had
Diyar-
frequently
Government.
Camil
feature
1-4)
parts
clinic
involved.
he
(table
as
most
rarely
had
(case
here
being
around
arouiid
was
disease
4 were
the
and
REPORTS
mnomith
(cases
boys
Six
observed
porphyria.
villages
less
by
was
“inborn
in
toxic
The
metabolism
clinical
seen
h-
to us when
been
occurred
studied
The
already
and
15,
seed.
town
cereal
tarda.”
was
affected
Ironi
of
Turkey.
of
hexachlorobenzene,
been
remarkable
cutanea
porphyria,
the
If
made
it
Unfortumiately
have
caine
males.
at
continuous
all
who
6 cases
All
of
people
treated
CASE
children.
age
in porphyrin
6 cases
a (Icin the
situation
working
Bingo!.
adults
who
by
disturbance
of
in
itself
had
in town
distributed
wheat.
por
“heme”
south-eastern
outbreak
and
among
individtmals
treat
advances
the
was
the
imnder
the
green
as
resulted
presented
in
who
girls).
in
than
the
cases
to
l)elo\\
was
produced
These
Sedormid
porphyria
discovered
which
was
liver
well
porphyria,
were
occurred
observed
wheat,
miative
On
injected
The
discussed.
Children
wheat
never
48
disease
seed
was
found
toxic
metabolism
as
Despite
human
have
been
reported
Elazig,
Siirt,
Ius
boys
miiales
livers.
attention
in children
(169
that
a dermatologist
called
than
400 cases
Urfa,
Wlardin,
most
appeared
the
as
catalase
discoloration.
showedi
human
to our
chicks
light.
also
being
porphyria
were
Diyarhakir.
and
transitory
porphyria
Sedormid
in
of
porphyrins
idiopathic
study
to
cases
tissues
ultraviolet
other
imi chick
is still
opportunity
)f these
In
was
experimental
a defect
fromri
al.’7
and
porphyria
ntmmerous
In
an
using
with
( erythropoietic,
of porphyria
(liSturbance
produced
a greemiish
(lifter
hiemoglobin’M
Talman
et
crease
in catalase
activity
of
This
.
1)rOduced
in
a chemical
liver.’5”#{176}
)hyrins
signs
)
porphyria
Talmami
et a!. ‘ observed
in embryonic
mni(I a brilliant
red fluorescence
un(ler
in
types
acute
)orphyria
but
from
probably
the
had
of
Occasionally
pinhead
skin
formation
not
to
exposed
extensive
a
bullae
some
pigeon’s
to
synlptomiis
termed!
a
on
bullae
egg.
toxin.
face
were
Buliae
also
sunlight.
pyodermic
lesions
on
the
From www.bloodjournal.org by guest on June 14, 2017. For personal use only.
1004
CETINGTL
Table
Ca.se
no.
IniSials
i
Age
H. K.
-
6
of
Prevous
diseases
poisoned
wheat
for
Typhoid
1 Year
First
Builae
Fever
:2
A. A.
11
OZEN
______ _____-
1 ____________
Intake
AND
symptoms
on
face
and
scalp,
pyodermatitis
3 Years
-
Buiiae
on
face,
pyoder-
nose
and
miiatitis
3
N. C.
11
1 Year
Bullae
on
pollex,
4
A. K.
-
8
1 Year
right
pyodermatitis
Btmilae
on
fingers,
pyoder-
niatitis
5
C. K.
6
28
NI. I).
face,
scaip,
h(ak’(l
amid!
)
1
neck,
The
.
iml 7 to
liyperpigmiicmmtcdl
All
of
the
child!reml
4 ) lost
its
had
the
into
a
Gonorrhea
Intervals
iiand!s,
legs
bullae
which
without
In
had
2
atrophic
plicae,
of
feet
which
formed!
and
the
scalp
which
fell
thin
amu!
ad!herent
Omie
patient
a scierodlermila.
were
Infected!
4
due
to
lesions
left
resulted
easily.
to
secondary
hospital
lesion
off
and
Orbital
hypertrichosis
hyperpignientation
in the
while
nails
iieconiimig
imiipressiomi
and
hypertrichosis
hyperpigmentation
1 Nlonth
scarrimig.
eases
Orbital
were
white
( case
scars
skin
)
was
of
aseptically
surroumnded
by
permanent
of
the
underlying
2
infections
treated
in
The
the
and
( case
hands
tissue
umable
so
to
a
alopecia.
that
flex
his
2
one
fingers
fist.
All
eases
iresented!
6 ) , there
l)tmllad,
haul
with
-
10 d!ays
area.
imi(l
and!
new
4 Years
Fever,
19
arms,
( fig.
limillac
Rheumatic
(hiring
l)(illae
amid
over
the
the
eyes.
In
cheeks.
amid
(lark
fl
color.
Except
for
cachectic
ease
legs
5
sumnkcmi
and
were
the
all
appearance
of
had
a
was
covered
the
patients
3).
This
hair
skimi
like
that
in
entire
appearance
The
noted
in
d!ume to
the
than
( fig.
of
chest,
adults.
patient
an
whose
excessive
around
in
had
2,
5
eyes
patients
children
case
case
2 ). This
both
few
the
anterior
children
bo!y
malnutrition.
especially
scar,
and
tibias,
in aim aduilt
the
a
5
a
around
forehead
forearms,
pronoumnced
exhibited
face
the
( cases
adults
6
leaving
the
over
cheeks,
over
showed
was
monkey-like
the
moore
with
The
in
particimlarly
over
two
case
withoumt
prominent
present,
also
forehead!.
(fig.
who
found
most
the
In
Althoumgh
disappeared
was
Hypertrichosis
on
hypertrichosis.
d!istumrl)ance.
which
was
it was
back.
hair
the
hospital
Hypertrieliosis
armmms and
case
of
pigmemitation
eases
1 had!
amid
in
The
and!
signs
clays
all.
severe
2, the
first
first
at
am-nis
In ease
the
his
no
abd!omcn,
hyperpigmentation
were
was
almost
eyes
were
hypertrichosis
and!
I mvpdrpigmemitation.
Except
was
for
within
the
enlarged!
normal
limits.
Laboratory
\Vith
amid
the
exception
5 the
treatmemit
slight
fever
In
all
case
test
negative
sedhimmiemitatiomi
of
the
in
the
urine
urobilin
the
1 andl
in
in
zinc
two
patients.
all
patients
2,
the
rate
accelerated;
was
process
remainder
of
the
dark
presemit
in
color
and!
in
reaction
sulfate
and
gave
a normochromic
serologic
tests
this
resulted!
d!isappeared
patients
was
adults
had
and!
porphobilinogen
the
all
5,
normiial,
infectious
these
was
eadmiiium,
domie
ease
were
prescmit
eases
3.
mmrimie, limit
turbid!itv,
of
eoumits
since
In
in cases
physical
examination
Findings
d!ifkrential
bmmt ease
liver
amid
cases
was
was
in
as
and
normal
almost
2,
dime
tests
Takata-Ara
to
the
all
In
pyodermia,
rate.
The
subsided.
black
when
was
of
some
were
leukocyte
negative.
sedlimilentation
uirobilinogen
In
The
were
infection
negative.
resumits.
anemia.
syphilis
probably
a
the
became
1
Kumnkel-phenol
normal
for
the
positive.
and
to
air.
present
in
the
cases
The
formol
exposed
the
hippuric
gel
tests
thymol
acid
were
From www.bloodjournal.org by guest on June 14, 2017. For personal use only.
TOXIC
Fig.
1005
PORPHYRIA
1.-Photosensitive
skin
It is noteworthy
ratio
was
were
norniah,
that
reversed.
Liver
parenchyma,
imicreased!
yellow
pigmiient
within
amid
There
was
were
nornial.
tions
of the
Tests
peatedlly
was
chest,
was
for
enipioyed
determined
by
in
skull
the
chloride,
adhequuate
adrenal
tendency.
Serum
a slight
miormohiast
and
in
by
urine
for
estiniatiomi
the
fohlowing
in
showed
revealed!
decrease
porphobihinogen
miegative
th(
amid
sod!iumm,
mmiarrow
heart,
no
cells
although
( .lui(Ose
iii
hvdropic
muomionuclear
of hemorrhagic
bone
norumual
Were
of legs.
time
t(ml(rIlice
A
U
tests
hi)i(i0grtmu1.
granumlar
capillaries
of
degeneration
the
(11(1
the
of
prsmtiu
a
cells.
mcluding
The
There
normal.
of
hypertrichosis
ehectroplmoreis.
abnormalities
significant
feet;
proliins
paper
advanced
17-ketosteroids
evidence
no
110
and
hands
serum
omi
revealed
number
parenchymah
electrolytes,
Serum
Eosinophils
conlirnied
were
biopsy
aspiration
total
patients
was
there
aui(l
all
iii
This
on face,
lesions
long
the
of
method:
the
were
ioiies
hihid!o of the
time sinlpld
of all
potassiumumi
patients.
urinary
The
iumethod
The
and
ii
noriuial.
mmurmnal.
Were
mjectmn
of
A( ‘l’H.
of
huyperpIasia.
Fhie
X-ra
(I )lmlit
exam ninawas
ehectrocard!iogralmi
a(lumlts.
Watson
miii.”
eoproporphiyriii.
combined
ii
l)ihirmmhin amid ret icu ul()eyte
two
“5
phosphorus.
respom use to
aqueous
antI
miiethod
Urinary
extracts
Sehmvartz”#{176}
of
Schwartz
was
et
uiroporphyriii
obtained
during
real.
was
the
I
From www.bloodjournal.org by guest on June 14, 2017. For personal use only.
1006
CETINGIL
,
)
1
Fig.
determination
of
washing,
the
Tissue
2.-Case
coproporphyrin
were
estimated
porphyrins
determined
was
extraction
of umroporphyrin
of methanol
and
extractions.23
urine
calcium
with
acid
was
case
the
copro-
with
Urinary
fecal
porphyrin
I.
the
fecal
In
cases
exammed
The
melting
144
by
fresh
6
above.
obtained
tube.
The
by
purified
esters
previous
feces
by
from
were
As
mixture
the
from
extracted
had
to
the
and
means
the
of
column
crystallized!
bone
liver
was
a melting
the
was
points
miiarrow
from
cellular
miiarrow
no
was
insufficient
in
placed
C.,
and
that
III.
to
porphyrin
when
bone
in
was
foumnd
for
uro-
fluorescence
material
was
C.,
of
coproporphyrin
porphyrins
marrow
289
of
points
153
of
exhibited
of
point
melting
crystals
melting
the
of
the
method!
al.#{176}:1 miil. of
centrifugation,
of
19:1
following
acid! an(!
the
above.
a
obtained
were
After
Uroporphyrin
diescribed
incomplete,
fractions
determination
et
content
biopsy
bone
of
The
After
porphyrin
needle
to
Schmid
\Vikoff.22
the tissue residue
coproporphyrin
smears
of
is
oxide.
fluorometrically.’3
as
corresponds
corresponds
unstained
method
this
which
urinary
aluminum
2.
by
microscopy.
the
centrifuge
d!escnhed
of
which
fluorescence
by
containing
point
C.,
and
acid
chloroform,
in table
C.
of
quantitated
Schwartz
Porphyrins
the
buttocks.
chromatography
uroporphyrin
obtained
282
and
alcohmol’sumlfuric
of
given
of
group
1 and
and!
methyl
crystals
of
oxide
and
a coiummn
HC1
ethylacetate’acetic
evaporation
resuuhts are
N
mnethod
chromatography.
uroporphyrin
performed
tissue
The
uroporphyrin
the
was
After
alcohol.
the
1.5
uused to extract
19:1
colummn
chloroform.
and
1,
esterifled
carbonate
methyl
of
sulfuric
In
were
by
back
tiiroimgh
with
aluminummii
I with
over
filtered
eluted
by
in tissues
____
were
was
ZEN
_w
2: hypertrichosis
uroporphyrin
AND
marrow
a heparin-
extracted
be
normal.
as
Tue
determination.
DIscussIoN
All
the
cases
disease.
of this
Heredity
series
came
could
from
be
ruled
an
area
out
in
as
an
which
etiologic
many
inhabitants
factor
because
have
the
From www.bloodjournal.org by guest on June 14, 2017. For personal use only.
FOXI(:
the
1007
P()HPIIYI1E
Fig. 3.-Cachexia,
hands
and feet
disease
occurred
cases
was
family
vidual
hypertrichosis
over face
and extremities.
due to liniments
for the pyodermatitis.
in
hundreds
of Yugoslav
People
of
extraction
that
ingested
susceptibility
severely
and
are
(case
the toxic
wheat
played
a certain
affected
than
adults,
at
3).
one
Since
were
role.
time,
not
affected,
Children
particularly
with
The
white
and
areas
one
of
all
of the
members
it
is possible
appeared
to
regard
to
over
our
of a
that
be
indimore
photosensitivity
hypertrichosis.
None
of
the
manifestations.
Table
patients
had
Splenomegalv
2.-Amount
evidence
was
of Porphyrin
A’
Plasma
B’
C’
A’
Erythrocytes
B’
The
Urine,
Plasma
Feces,
A’
marrow
B*
18.0
0
0.2
0
0.6
36.0
18.0
0
0
‘A,
potoporphyrin,
g./100
tr.,
trace.
nil.;
B,
uroporphyrin,
cells
Bone
or
showed
Marrow,
psychic
no
signif-
Erythrocytes
0
Feces
C’
Cs
tr.’
1.0
0.8
27.0
porphobilinogen;
neurologic
red
of Patients
Bone
C’
abdominal,
present.
in the
and
Case
no.
of
not
0
1.4
pg./l00
nil.;
C,
B
Urine
C
D’
867:1
1550
0
10,148
1432
0
1426
432:3
6681)
807
0
1032
1898
1)868
840
0
2326
1484
0
3764
721
0
coproporl)hyrmn,
pg./100
nil.;
D,
From www.bloodjournal.org by guest on June 14, 2017. For personal use only.
1008
CETINGIL
icant
increase
in
fresh
in porphyrin
bone
Unfortunately,
of the
disease
not
there
tal
and
uroporphyrin
did
they
al.m1
cases
vith
opportunity
to
porphyria,
by
gastro
I1I.1
None
resemble
neither
excessive
of
However,
occurrence
industrial
of
patients
these
lel(1
the
four
and
the
agent
in
cent
to
duced
quantities
by
to
exposure
in
heavy
;il.24
bone
signs,
Schwartz
light.
Our
marrow
cases?
nor
marines
use(l
in
The
had
1)een
intoxication
es’ieciallv
of
Agriculture
the
one
latter
was
1lld1
addled
lead.
( Fagopyrum
1)llckwheat
treating
niercurv,
compouundl.
to
observed.
so as i)uckwheat
Europe,
Ministry
as
with
exposedi
been
Should
workers
por)hvrifluria
rarely
believe
contained
industry
found
of
has
do not
conhl)ounds
as
an
no complications
been
postulated
in
#{176}
copper
a
These
these
by
the
possibility
that
the
intoxicant
was
is not usually
followed
by porphyriiitmrii.
great
\VS
diagnosis
in
Germany.
furnished
wheat
seed.
Of
hexachlorobenzene
not
been
in
the
of 0.2
per
uSe(l
in a proportioii
x’heat.
tue
The
mercury
in
was
area.
of
produced
ef
group
fummigicides
two
USCdl,
an’
aS
et
a
these
\Ve
the
chemicals
exhibited
by
porphyria
in
incrimiiinated?
fourth
the
liver
Sclili3id
an(l Schwartz,
signs
and by excretion
by
content
Bernard
ill
toxic
ced
observed
masSive
concerning
diseased
with
followed
is well
known
through
its use in northern
This
cereal
is miot grown
in Turkey.
The
information
content
and
anemia
excretion.
been
poisoning.
of
the
) be
PrPl1yrin
compatible
l)rP11y’ri1
llidl
copropcrphyrin
\VL1S
esculentum
observed
microscopy.
histology,
Hemolytic
neurologic
porphyrin
has
excretion
\Vhat
and
porphohilinogen
of
the
hepatic
tests.
I)rodlt’
aS
experimilental
Coproporphyrimmria
evidence
was
fluorescence
determine
were
intestinal
increased
urinary
cases
fluorescence
by
OZEN
tarda.
l11eny’l11ylrazim3e
had
but
has
no
examined
of these
Sedormid
characterized
et
no
features
cutanea
Experimen
nor
was
All the
of porphyria
of
preparations
liver.
Needle
I)iopsv
showed
abnorinal
was also suggestedi
by the liver
function
Preselit.
xVas
concentration,
marrow
AND
agent
suggestive
that
perhaps
mercury
hound
coniunction
with
will
possibilities
antisvphilitic
to
require
of
an
mercury
mercury
is
Mercury
is still
porphyria
have
a porphvrinogemc
organic
could
amid
radical.
he
imivestigation
the
convincing
as
used
Hexachlorobenzol
a
as
formerly
a
used
diuretic,
ever
I)een
reported.
effect
could
be
etiologic
before
not
was
alone
factor.
definite
It
pro-
All
of
conclusion
or
these
be
can
reached.
The
mechanism
nor
is it
not
the
was
in
result
is
the
of
alteration
1 to
In
4, in
cases
of
has
not
per
been
h’illae
!.09
cent.
cent
no
in
idiopathic
abnormality
porp1iri;i
cases?
vi1l
disappeared
pr
clear
porphyria,
hvperpigmentation
are
of
reported
adrenal
in
the
probably
function
literature,
a feature.1#{176}
these
metabolism
the
mirea
10
as
idiopathic
in
porphyrin
which
is not
and
dysfunction
prognosis
#{176}Phenylmercuric
chlorobenzemie
formation
Hypertrichosis
cortical
dysfunction
What
hullae
cases.
of
detected.
cortical
of
our
Hg;
have
We
he
no
completely
after
phemiylmiiercumrie
a few
acetate
imidication
that
reversible.
dlays
of
1.5
In
this
cases
hospitalization.
per
cent
Hg;
hexa-
From www.bloodjournal.org by guest on June 14, 2017. For personal use only.
TOXI(;
1009
PORPHYRIA
urinary
excretion
PrPIiYri1i
1)1ti’mit5
dlel)arture
1tt;ents
1)ecause
the
their
farms.
1)ut
whemi
Case
Case
5
left
the
he
remained
6
was
fre
that
in
still
his
clinic
increased
urinary
for
still
these
greatly
at
the
cI inic.
Photosensitivity
had
exposed
to less sunlight
than
no I)ullae
evemi
on adimnission
hospital
the
l)ut
is 1)ri:til
tlie
m
were
had
at
a5miiptCIi3atiC
‘as
six
excretimig
‘aS
cases
severe
at
the
large
chronic
the
very
WdS
end
amounts
liver
of
perhaps
decreased
‘as
the
case
on
to
the
liOsl)ital,
still
prp1iyr;mi
months;
time
high.
of
this
erioi
of
porphyrms.
he
It
is Present.
dliSe:lse
TREATMENT
\v;ts
Pyodermia
were
givemi
vitaiiiimis,
all
been
Partial
(Inc
clearing
to better
by
responded
1)rt(in
were
and
cases
to
heesi
have
the
massive
in
1)easamits
of
indicatiomis
in
of
cutamieous
P1tients
extract
their
stay
pius
in
irianifestatiomi,
the
disap-
was
observe(1,
but
this
may
lesions
on the hands
and fingers
urinary
excretion
AND
1)or1)hyria
southeastern
of
porphyrins
was
not
‘ere
buckwheat
tarda
Turkey.
type
The
were
toxic
miot
this
COliSUflie
toxicity;
seed
furthermore,
appear
together
vith
dhsease
did
not
wheat.
this
observed
agemits
two
niercuru’
colill)ouli(ls
to seed
wheat.
The
adided
dlidl
‘cutanea
of
including
Who
SUMMARY
the
of
districts
fungicides,
which
hexachlorohemizel
IPP(’tr
the
All
liver
During
of hvperpigmentation
lwgienic
care.
The
The
tre.ttnient.
to
addition,
treatment.
1 . NuIllerolls
in
In
J)arenteralh’.
CoNuus1oN
recemitlv
‘ell
(bet.
given
‘eight,
gaimied
unaltered.
illfllmence(1
and
high
B12,
patients
remained
locally
calorie,
including
hospital
Pelrel.
have
treated
a high
There
seed
is
were
not
no
cultivated
Turkey.
Six
of the
2.
Clinically
affected
were
patients
the
treated
patients
pres’mitecl
i.e.,
lesions
and
hvpertrichosis,
There
were
no cases
of splenomegalv,
The patiemits
were
found
to have
3.
No
suprarenal
normiial.
fecal
fecal
hemolysis
of
porphyrin
5.
Our
cutanea
6. The
may
not
residemice,
departure
the
type
aixiomimial
and
recorded.
had
or
of
fuinctional
X-ravs
marrow
found
to
signs.
liver
of
Bone
porphyria.
neurologic
tile
disease.
1)ones
were
studies
revealed
111
was
were
greater
negative.
Urinary
present
than
reversed.
The
No fluorescence
was
fluorescence
microscopY.
clinical
and
laboratory
findings
the
he
in excess.
of
that
bone
marrow
recorded
The
imroporphyrin
content
in
consistent
the
fresh
with
the
of porpliyrii.
and
severe
alteration
Althotmgli
excessive
the
repeatedly
relation
hy
reversible.
from
hyperpigmentation
tarda
organic
11 1 was
increased.
type
the
no
coproporphin
not
advanced
he
1 and
smears
was
cases
bullae,
cutanea
Present.
WaS
urine
1)100(1
tarda
an
was
coproporplwrin
in the
umlstaine(1
clinic.
hvperplasia.
uroporplwrin
excretion
tile
and
were
porphobilmogen
1, whereas
of
of
normohlastic’
Urinary
4.
and
sighs
of
dh’sfunction
cortical
No
slight
skin
in our
cachexia,
clinic.
porphyrin
the
of our
hullae
excretion
p;ltients
porphyrin
disappeared
dumring
was
still
present
metabolism
their
hospital
on
their
From www.bloodjournal.org by guest on June 14, 2017. For personal use only.
1010
CETINGIL
7.
For
therapy,
the
and
treated
with
food
preparations.
\Ve
patients
were
liver
can
express
vitamin
no
1. Grand!e
numeros
toxicitate
in
Splenomegalia
non
o neurologic
medulla
ossee
Uroporphyrina
e le
Le
porphyrina
excretion
in
ulle
Nostre
habeva
typo
cutanee
Isto
es
de
hyperplasia
de
Le
morbo
frumento
semine.
lo produce-Sex
hyperpigmentation,
tardive
casos.
de
Nulle
porphyria.
signos
le
abdom-
esseva
in
del
negative.
in le urina
plus
alte
Le
es
de
laboratorial
un
caso
require
de
sanguine.
indicative
de
del
erythrodontia.
pathognomonic
constatation
illo
fluorescential
frottis
e
que
contento
Microscopia
notate
erythrodontia
Iste
esseva
revertite.
e non-tincturate
Esseva
del
Studios
in excesso
III
clinic
porphyria.
e func-
repetitemente
augmentate.
fresc
presente.
presente
esseva
characteristicas
organic
Radiogrammas
normoblastic.
esseva
relation
esseva
hepatic
esseva
coproporphyrina
non
porphyria.
morbo
urinari
III
proque
de
del
comI)ositOs
non
observate.
grados
urina,
de
remarcabile
erythropoietic
esseva
agentes
semine.
bullas,
un
hemolyse
de
le
habeva
tardive
duo
cutanee
esseva
de
fluorescentia
casos
Le
iste
de nostre
signos
fecal
In
le medulla
revelava
5.
leve
I.
treatment.
tardive”
Turchia
typo
in ulle
I e coproporphyrina
feces.
non
del
porphobilinogeno
uroporphyrina
de
this
Turchia.
consumite
adrenocortical
Nulle
pro
rich
B-complex
“cutanee
a frumento
cachexia,
pelle
le patientes
revelava
Examines
4.
protein
trovate.
que
normal.
of
incluse
habeva
presente
dysfunction
esseva
de
addite
exhibiva
del
esseva
trovate
Nulle
ossos
non
of
whole
value
typo
sud-est
es excludite
proque
a nostre
clinica.
lesiones
esseva
del
del
esseva
le patientes
i.e.
3. Esseva
qui
saracen
tractate
2. Clinicamente
the
fungicidas,
que
fermeros
hypertrichosis,
tional.
porphyria
apparentemente
appareva
inal
de
e hexachlorobenzol
Toxicitate
per grano
del patientes
esseva
e
casos
to
diet
B12 and
INTERLINCUA
in le districtos
esseva
mercurio
non
de
recentemente
as
IN
caloric
B1, B2,
opinion
SUMMARIO
observate
a high
given
extracts,
OZEN
AND
del
varietate
addi-
confirmationes
tional.
6.
Le
avantiate
patientes
es
e sever
possibilemente
dispareva
durante
persisteva
quando
le
therapia,
proteina.
In
in
B1,
B2,
B12,
tractamento,
in
plus,
non
de
del
le
es
un
revertible.
del
dieta
con
in nostre
Ben
excretion
a alte
extracto
complexo
exprimer
porphyrina
que
de
le bullas
porphyrina
hospital.
un
tractate
complete
que
de
excesso
recipeva
esseva
pote
lo
quitava
illes
metabolismo
Ic
le patientes
e preparatos
nos
ultra
hospitalisation,
le patientes
7. Como
del
alteration
B.
contento
de
caloric
hepate,
Quanto
e nc
vitamina
al efficacia
de
iste
opinion.
REFERENCES
1.
R.,
Lemberg,
Hematin
Their
and
science
2. Brownlee,
amino
Function.
group
and
\V.:
Bile
Pig-
Constitution,
New
Publishers,
G.:
J.
Legge,
Compounds
ments.
hism
and
The
in
MetaboYork,
Inter-
1949.
metabolismii.
Biochem.J.
C.,
3. Bimington,
and
Porphyrinuria
amide.
33:697,
Hemmings,
following
Lancet
4. Rimimigton,
role of the
aromatic
Porphyrimmric
deranged
pigment
to
1:770,
C., and
sulphanilamide.
action
1939.
A.
\V.:
sulphanil1938.
Hemmings,
of
drugs
Comparison
A.
W.:
related
with
From www.bloodjournal.org by guest on June 14, 2017. For personal use only.
TOXIC
1011
PORPHYRIA
repeated!
and
cauusation
ghobinemia.
J.
J.
Kench,
case
of
N.
(3. Fischer,
of
M.,
ii:806,
umnd
Duesherg,
Porphyrine
7.
bci
\Valdenstr#{246}mii,
j.,
R.,
J.,
The
ferent
Lowry,
9.
R.,
J.:
C.
of
in
Schwartz,
S.,
amu!
content
liver
in
the
A.M.A.
11. Schmid,
Porphyria.
R.,
Advances
1954.
6:235,
Schwartz,
S. and
C. J.: Porphyrins
in the
and
erythrocytes
circulating
perimental
Bioh.&
anemias.
B.,
poisouicd
Biol.&
13. Schwartz,
R.:
79:463,
14. Schmiiid,
I.
Isolation
of
bone
marrow
of
hep:itic
Biosymithiesis
p. 209.
C.:
M.,
and
23.
Type
by
lead!, phenylhydrazine
Proc.Soc.Exper.Biol.&
Med.
1952.
porphyria
Proc.Soc.Exper.Biol.&
94,
24.
ant!
III.
an
Hepatic
type
and
A
urinary
47:
Watson,
method
the
for
corpropor-
evaluation
the
of
analysis.
37:843,
factors
J.Lab.&
Chin.
1951.
Wikoff,
H.
M.:
The
relation
coproporphyrin
to
194:563,
of
and
erythropoiesis.
pro-
J.Biol.
1952.
aspects
of
porphyrin
me-
Vet.Admin.Tech.Bull.
10-
1953.
Bernard,
\1.,
S.: Experi-
L.,
of
tabolism.
den
porphobihino-
improved
Clinical
-:
bagli
Med.
S., Zieve,
An
toporphiynin
Schmid,
II.
Por-
Metal)Ohism.
urinary
1941.
Chemii.
porphyria.
Schwartz,
for
gen.
and
on
and
393,
J.:
to
aniniahs
I)irimn 34:11,
1959.
J., and
Schwartz,
S.:
erythrocyte
1952.
S., Keprios,
R., amid
-,
of
porphyria.
Intoksikasyona
test
Med.
22.
in
Symposiumm
phyrin
S.: Ex-
Studies
porphyrins
determination
Proc.Soc.Exper.
79:459,
Experimental
cx-
Experiembryos.
K.:
Foumidlation
C.
A.:
1955.
Ikedla,
experimental
influmencing
Schwartz,
rabbits.
Med.
prodllmced!
and
light.
mental
and
I from
umroporphrin
in
I.
Ciba
Schwartz,
marrow
Proc.Soc.Exper.
porphyria.
perimental
21.
Watson,
bone
1950.
75:705,
Med.
Hanson,
head!
20.
A.,
H.
chick
with
simple
J.:
C.
Int.Med.
-,
Met!.
and
porfirinleri.
Watson,
C.
1954.
Nev#{233},R.
Aldrich,
in
green
19. Cam,
forms
mt.
Arch.
experi-
14:717,
synthesis
and!
interconwith
special
reference
phyrin
1955,
\Vatsomi.
1).,
and
212:663,
S.,
certain
Physi-
J.
Case,
miietabolism.
porphyrin
version,
bone
porphyrin
and
Fed.Proc.
F.,
J.Biol.Chemii.
of
enJ.BioI.
and
humman
porphyria
Schwartz,
relation
various
L.,
R.
uiicntal
dif-
Am
of
henie
porphyria
Porphyrins
Porphyrin
Tier-
the
Ex-
1955.
in
E.
Labbe,
Schwartz.
porphyria
porphyria.
10. Watson,
12.
and!
other
porphyria.
Talman,
1951.
and
93:167,
17.
Schmid,
of
Porphyrin
miiarrow
of
E.,
S.:
Studies
IV.
1955.
cx-
den
1’.,
Proc.Soc.
1952.
Schwartz,
sedormiiid
S.:
mental
18.
findlings. Tr.A
64:345,
Schmid!,
Schwartz,
Uber
umnd!
P.
V.
formiis
cians.
16.
and
and
precumrsors
S.:
manifestations
to chemical
217:263,
1939.
Hawkinson,
S.:
in
Chem.
a
Ztschr.Physiol.
25.9:157,
C.
zymes
In
ul)er
Porphyrinstoffwechsel.
8. Watson,
E., amid
El:
\Vendt,
F.,
sed!ormiiidh.
81:685,
porphyria.
catahase
chiloride
Studien
J.
Figen,
liver
Arch.Exper.
166:95,
1932.
und
Mccl.
perimiiental
1940.
klinischer
experimiientelle
Chemiiie.
A.
methyl
Lancet
I)erinientel!er
Porphyrie.
Pathi. mm. Phiarmakol.
-,
ac-
1939.
Gillam,
i)y
1)rodluuce(l
Exper.Bioh.&
15.
struc-
Porphyrinumri:m.
ind!ustrial
H.,
the
33:960,
E.:
poisoning.
dill-
miietliaemiio-
for porphyrinumric
Biochemii.J.
5. Chalmers.
tic
of
Definition
tuure responsil)le
tion.
thcrapeu
toxicity,
ciency
H.,
et
Gajdos,
A.,
Ranibert,
Medico-Chirurgicahe.
10534-C
10,
Gajdos-T#{246}r#{246}k,
P.:
Encyclopedic
Vol.
p.
11.
Numtrition,
From www.bloodjournal.org by guest on June 14, 2017. For personal use only.
1960 16: 1002-1011
Toxic Porphyria
ARIF I. CETINGIL and MUHLIS A. ÖZEN
Updated information and services can be found at:
http://www.bloodjournal.org/content/16/1/1002.full.html
Articles on similar topics can be found in the following Blood collections
Information about reproducing this article in parts or in its entirety may be found online at:
http://www.bloodjournal.org/site/misc/rights.xhtml#repub_requests
Information about ordering reprints may be found online at:
http://www.bloodjournal.org/site/misc/rights.xhtml#reprints
Information about subscriptions and ASH membership may be found online at:
http://www.bloodjournal.org/site/subscriptions/index.xhtml
Blood (print ISSN 0006-4971, online ISSN 1528-0020), is published weekly by the American Society of
Hematology, 2021 L St, NW, Suite 900, Washington DC 20036.
Copyright 2011 by The American Society of Hematology; all rights reserved.