Ironjustic
Sun, Mar-11-07, 17:16
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http://tinyurl.com/354tc3
Instrument No.71 of 1994
Statement of Principles
concerning
PORPHYRIA CUTANEA TARDA
ICD CODE: 277.1
Veterans' Entitlements Act 1986 subsection 196B(2)
1=2E Being of the view that there is sound medical-scientific
evidence that indicates that porphyria cutanea tarda and death
from porphyria cutanea tarda can be related to operational
service rendered by veterans, peacekeeping service rendered by
members of Peacekeeping forces and hazardous service rendered
by members of the Forces, the Repatriation Medical Authority
determines, under subsection 196B(2) of the Veterans'
Entitlements Act 1986, that the factors that must as a minimum
exist before it can be said that a reasonable hypothesis has
been raised connecting porphyria cutanea tarda or death from
porphyria cutanea tarda with the circumstances of that
service, are:
(a) being exposed to herbicides in Vietnam before the clinical
onset of porphyria cutanea tarda; or
(b) decanting, mixing, applying, or ingesting polychlorinated
aromatic hydrocarbons before the clinical onset of
porphyria cutanea tarda; or
(c) having psychoactive substance abuse or dependence
involving alcohol before the clinical onset of porphyria
cutanea tarda; or
(d) having active viral hepatitis before the clinical onset of
porphyria cutanea tarda; or
(e) having HIV-1 infection before the clinical onset of
porphyria cutanea tarda; or
(f) having skin exposed to sunlight before the clinical
worsening of porphyria cutanea tarda; or
(g) inability to obtain appropriate clinical management for
porphyria cutanea tarda.
2=2E Subject to clause 3 (below) at least one of the factors
set out in paragraphs 1(a) to (g) must be related to any
service rendered by a person.
3=2E The factors set out in paragraphs 1(f) and (g) apply
only where:
(h) the person's porphyria cutanea tarda was contracted prior
to a period, or part of a period, of service to which the
factor is related; and
(i) the relationship suggested between the porphyria cutanea
tarda and the particular service of a person is a
relationship set out in paragraph 8(1)(e), 9(1)(e),
70(5)(d), or 70(5A)(d) of the Act.
4=2E For the purposes of this Statement of Principles:
"active viral hepatitis" means infection with Hepatitis A,
B, C, D, or E with signs or symptoms or pathological tests
indicating active inflammation of the liver, attracting
ICD code 70;
"being exposed to herbicides in Vietnam" may be said to have
occurred only if the person had:
(j) rendered more than 30 days service on land in Vietnam; or
(k) regularly eaten fish, fish products, crustaceans,
shellfish, or meat from Vietnam; or
(l) regularly eaten food cooked with water from Vietnam
discoloured by sediment, or regularly drunk water from
Vietnam discoloured by sediment; or
(m) regularly inhaled dust in a defoliated area in Vietnam or
regularly inhaled herbicide fog in Vietnam; or
(n) sprayed or decanted herbicides in Vietnam as an
occupational requirement;
"HIV-1 infection" means serological evidence of infection with
human Immunodeficiency Virus Type 1, attracting an ICD code in
the range 42 to 44 (inclusive);
"ICD code" means a number assigned to a particular kind of
injury or disease in the tenth edition of the International
Classification of Diseases 9th Revision, effective date of 1
October 1993, copyrighted by the US Commission on Professional
and Hospital Activities, and having the Library of Congress
number 77-94472;
"polychlorinated aromatic hydrocarbons" means chemical
compounds containing a benzene ring and multiple
chlorine atoms;
"porphyria cutanea tarda" means a disease with abnormalities
of porphyrin metabolism with dermatological manifestations,
attracting an ICD code of 277.1;
"psychoactive substance abuse or dependence" means a
maladaptive pattern of use, attracting an ICD code of 303 or
304, that is indicated by either:
(o) continued use of the substance despite knowledge of having
a persistent or recurrent social, occupational,
psychological or physical problem that is caused or
exacerbated by use of the substance; or
(p) recurrent use of the substance when use is physically
hazardous (for example, driving while intoxicated).
Dated this day of 1994
The Common Seal of the )
Repatriation Medical Authority )
was affixed to this instrument )
in the presence of: )
KEN DONALD
CHAIRMAN -----------------------------------------------------
-----------------------=
---------------------------
<<snip>> ascorbate suppresses hepatic URO accumulation at low,
but not high hepatic iron levels <<snip>>
Effect of iron and ascorbate on uroporphyria in
ascorbate-requiring mice as a model for porphyria cutanea
tarda. Gorman N, Zaharia A, Trask HS, Szakacs JG, Jacobs NJ,
Jacobs JM, Balestra D, Sinclair JF, Sinclair PR Hepatology.
2006 Dec 22; 45(1): 187-194
Excess hepatic iron is known to enhance both porphyria cutanea
tarda (PCT) and experimental uroporphyria. Since previous
studies have suggested a role for ascorbate (AA) in
suppressing uroporphyria in AA-requiring rats (in the absence
of excess iron), the present study investigated whether AA
could suppress uroporphyria produced by excess hepatic iron.
Hepatic URO accumulation was produced in AA-requiring
Gulo(-/-) mice by treatment with
3,3',4,4',5-pentachlorbiphenyl, an inducer of CYP1A2, and
5-aminolevulinic acid. Mice were administered either
sufficient AA (1000 ppm) in the drinking water to maintain
near normal hepatic AA levels or a lower intake (75 ppm) that
resulted in 70
% lower hepatic AA levels. The higher AA intake
% suppressed hepatic
URO accumulation in the absence of administered iron, but not
when iron dextran (300-500 mg Fe/kg) was administered. This
effect of iron was not due to hepatic AA depletion since
hepatic AA content was not decreased. The effect of iron to
prevent AA suppression of hepatic URO accumulation was not
observed until a high hepatic iron threshold was exceeded. At
both low and high AA intakes, hepatic malondialdehyde
(MDA), an indicator of oxidative stress, was increased
three-fold by high doses of iron dextran. MDA was
considerably increased even at low iron dextran doses,
but without any increase in URO accumulation. The level
of hepatic CYP1A2 was unaffected by either AA intake.
Conclusion: In this mouse model of PCT, AA suppresses
hepatic URO accumulation at low, but not high hepatic
iron levels. These results may have implications for the
management of PCT. (HEPATOLOGY 2007;45:187-194.).
Abstract =B7 PubMed FullText =B7 SFX =B7 GS Clip Export
InterDB =B7 Terms Related =B7 Graph Tag =B7 Scopus =B7 Cites
10.1002/hep.21474
--------------------------------------------------------------
--------------=
----------------------
[High-dose vitamin therapy as prophylaxis against porphyria
cutanea uremica.] Wimmershoff F, Gardlo K, Bolsen K, Ruzicka
T, Fritsch C Hautarzt. 2006 Mar ; 57(3): 228-36
50 patients with chronic renal failure undergoing hemodialysis
with or without porphyria cutanea tarda (PCT)-like skin
changes were investigated. The total porphyrin amount in
erythrocytes, plasma and dialysate and the distribution of
porphyrin metabolites in plasma and dialysate were measured.
In plasma, the group of patients with skin changes (referred
as PCU =3D porphyria cutanea uremica) showed significantly
increased uroporphyrin levels as compared to the
non-symptomatic group. In addition, significant differences
concerning the ratio uro-/coproporphyrin in plasma were shown:
non-symptomatic patients with 0.87, as opposed to the PCU
group with 3.7. Considerable differences between the level of
vitamin ingestion were identified between the groups. Patients
with PCU took distinctly less vitamins C, E and B than
patients without symptoms.
--------------------------------------------------------------
--------------=
-----------------
Ann Dermatol Venereol. 2003 Jan;130(1 Pt 1):37-9. Links
[Successful treatment of haemodialysis-related porphyria
cutanea tarda with deferoxamine][Article in French] Pitche P,
Corrin E, Wolkenstein P, Revuz J, Bagot M. Service de
Dermatologie, CHU Henri Mondor, 51, avenue du Marechal de
Lattre de Tassigny, 94010 Creteil.
BACKGROUND: End-stage renal failure and long-term hemodialysis
treatment promote porphyria cutanea tarda. Iron overload is
often associated with this disease and is thought to play a
role in its pathogenesis. We report a case of hemodialysis
related-porphyria cutanea tarda improved by deferoxamine. CASE
REPORT: A 45-year-old man, with end-stage renal failure and
who had received hemodialysis treatment since 1993, presented
a several months-history of blisters of the face and the
dorsum of the hands. Laboratory analysis showed: hemoglobin 10
g/dl; a moderate hepatic cytolysis; ferritin 195 ng/l. HIV,
HBV, HCV serologies were negative. Porphyries analyses showed
a porphyria cutanea tarda pattern. The cutaneous histology was
non specific; direct immunofluorescence was negative. The
patient received deferoxamine (40 mg/kg intravenously every
week for 6 weeks) which led to dramatic improvement of the
symptoms. DISCUSSION: Several treatments are proposed in the
management of dialysis-related porphyria cutanea tarda. This
case confirms that deferoxamine can induce rapid and prolonged
remission.
PMID: 12605155 [PubMed - indexed for MEDLINE]
Who loves ya. Tom
Jesus Was A Vegetarian! http://jesuswasavegetarian.7h.com
Man Is A Herbivore! http://tinyurl.com/a3cc3
DEAD PEOPLE WALKING http://tinyurl.com/zk9fk
--------------=
-----
http://tinyurl.com/354tc3
Instrument No.71 of 1994
Statement of Principles
concerning
PORPHYRIA CUTANEA TARDA
ICD CODE: 277.1
Veterans' Entitlements Act 1986 subsection 196B(2)
1=2E Being of the view that there is sound medical-scientific
evidence that indicates that porphyria cutanea tarda and death
from porphyria cutanea tarda can be related to operational
service rendered by veterans, peacekeeping service rendered by
members of Peacekeeping forces and hazardous service rendered
by members of the Forces, the Repatriation Medical Authority
determines, under subsection 196B(2) of the Veterans'
Entitlements Act 1986, that the factors that must as a minimum
exist before it can be said that a reasonable hypothesis has
been raised connecting porphyria cutanea tarda or death from
porphyria cutanea tarda with the circumstances of that
service, are:
(a) being exposed to herbicides in Vietnam before the clinical
onset of porphyria cutanea tarda; or
(b) decanting, mixing, applying, or ingesting polychlorinated
aromatic hydrocarbons before the clinical onset of
porphyria cutanea tarda; or
(c) having psychoactive substance abuse or dependence
involving alcohol before the clinical onset of porphyria
cutanea tarda; or
(d) having active viral hepatitis before the clinical onset of
porphyria cutanea tarda; or
(e) having HIV-1 infection before the clinical onset of
porphyria cutanea tarda; or
(f) having skin exposed to sunlight before the clinical
worsening of porphyria cutanea tarda; or
(g) inability to obtain appropriate clinical management for
porphyria cutanea tarda.
2=2E Subject to clause 3 (below) at least one of the factors
set out in paragraphs 1(a) to (g) must be related to any
service rendered by a person.
3=2E The factors set out in paragraphs 1(f) and (g) apply
only where:
(h) the person's porphyria cutanea tarda was contracted prior
to a period, or part of a period, of service to which the
factor is related; and
(i) the relationship suggested between the porphyria cutanea
tarda and the particular service of a person is a
relationship set out in paragraph 8(1)(e), 9(1)(e),
70(5)(d), or 70(5A)(d) of the Act.
4=2E For the purposes of this Statement of Principles:
"active viral hepatitis" means infection with Hepatitis A,
B, C, D, or E with signs or symptoms or pathological tests
indicating active inflammation of the liver, attracting
ICD code 70;
"being exposed to herbicides in Vietnam" may be said to have
occurred only if the person had:
(j) rendered more than 30 days service on land in Vietnam; or
(k) regularly eaten fish, fish products, crustaceans,
shellfish, or meat from Vietnam; or
(l) regularly eaten food cooked with water from Vietnam
discoloured by sediment, or regularly drunk water from
Vietnam discoloured by sediment; or
(m) regularly inhaled dust in a defoliated area in Vietnam or
regularly inhaled herbicide fog in Vietnam; or
(n) sprayed or decanted herbicides in Vietnam as an
occupational requirement;
"HIV-1 infection" means serological evidence of infection with
human Immunodeficiency Virus Type 1, attracting an ICD code in
the range 42 to 44 (inclusive);
"ICD code" means a number assigned to a particular kind of
injury or disease in the tenth edition of the International
Classification of Diseases 9th Revision, effective date of 1
October 1993, copyrighted by the US Commission on Professional
and Hospital Activities, and having the Library of Congress
number 77-94472;
"polychlorinated aromatic hydrocarbons" means chemical
compounds containing a benzene ring and multiple
chlorine atoms;
"porphyria cutanea tarda" means a disease with abnormalities
of porphyrin metabolism with dermatological manifestations,
attracting an ICD code of 277.1;
"psychoactive substance abuse or dependence" means a
maladaptive pattern of use, attracting an ICD code of 303 or
304, that is indicated by either:
(o) continued use of the substance despite knowledge of having
a persistent or recurrent social, occupational,
psychological or physical problem that is caused or
exacerbated by use of the substance; or
(p) recurrent use of the substance when use is physically
hazardous (for example, driving while intoxicated).
Dated this day of 1994
The Common Seal of the )
Repatriation Medical Authority )
was affixed to this instrument )
in the presence of: )
KEN DONALD
CHAIRMAN -----------------------------------------------------
-----------------------=
---------------------------
<<snip>> ascorbate suppresses hepatic URO accumulation at low,
but not high hepatic iron levels <<snip>>
Effect of iron and ascorbate on uroporphyria in
ascorbate-requiring mice as a model for porphyria cutanea
tarda. Gorman N, Zaharia A, Trask HS, Szakacs JG, Jacobs NJ,
Jacobs JM, Balestra D, Sinclair JF, Sinclair PR Hepatology.
2006 Dec 22; 45(1): 187-194
Excess hepatic iron is known to enhance both porphyria cutanea
tarda (PCT) and experimental uroporphyria. Since previous
studies have suggested a role for ascorbate (AA) in
suppressing uroporphyria in AA-requiring rats (in the absence
of excess iron), the present study investigated whether AA
could suppress uroporphyria produced by excess hepatic iron.
Hepatic URO accumulation was produced in AA-requiring
Gulo(-/-) mice by treatment with
3,3',4,4',5-pentachlorbiphenyl, an inducer of CYP1A2, and
5-aminolevulinic acid. Mice were administered either
sufficient AA (1000 ppm) in the drinking water to maintain
near normal hepatic AA levels or a lower intake (75 ppm) that
resulted in 70
% lower hepatic AA levels. The higher AA intake
% suppressed hepatic
URO accumulation in the absence of administered iron, but not
when iron dextran (300-500 mg Fe/kg) was administered. This
effect of iron was not due to hepatic AA depletion since
hepatic AA content was not decreased. The effect of iron to
prevent AA suppression of hepatic URO accumulation was not
observed until a high hepatic iron threshold was exceeded. At
both low and high AA intakes, hepatic malondialdehyde
(MDA), an indicator of oxidative stress, was increased
three-fold by high doses of iron dextran. MDA was
considerably increased even at low iron dextran doses,
but without any increase in URO accumulation. The level
of hepatic CYP1A2 was unaffected by either AA intake.
Conclusion: In this mouse model of PCT, AA suppresses
hepatic URO accumulation at low, but not high hepatic
iron levels. These results may have implications for the
management of PCT. (HEPATOLOGY 2007;45:187-194.).
Abstract =B7 PubMed FullText =B7 SFX =B7 GS Clip Export
InterDB =B7 Terms Related =B7 Graph Tag =B7 Scopus =B7 Cites
10.1002/hep.21474
--------------------------------------------------------------
--------------=
----------------------
[High-dose vitamin therapy as prophylaxis against porphyria
cutanea uremica.] Wimmershoff F, Gardlo K, Bolsen K, Ruzicka
T, Fritsch C Hautarzt. 2006 Mar ; 57(3): 228-36
50 patients with chronic renal failure undergoing hemodialysis
with or without porphyria cutanea tarda (PCT)-like skin
changes were investigated. The total porphyrin amount in
erythrocytes, plasma and dialysate and the distribution of
porphyrin metabolites in plasma and dialysate were measured.
In plasma, the group of patients with skin changes (referred
as PCU =3D porphyria cutanea uremica) showed significantly
increased uroporphyrin levels as compared to the
non-symptomatic group. In addition, significant differences
concerning the ratio uro-/coproporphyrin in plasma were shown:
non-symptomatic patients with 0.87, as opposed to the PCU
group with 3.7. Considerable differences between the level of
vitamin ingestion were identified between the groups. Patients
with PCU took distinctly less vitamins C, E and B than
patients without symptoms.
--------------------------------------------------------------
--------------=
-----------------
Ann Dermatol Venereol. 2003 Jan;130(1 Pt 1):37-9. Links
[Successful treatment of haemodialysis-related porphyria
cutanea tarda with deferoxamine][Article in French] Pitche P,
Corrin E, Wolkenstein P, Revuz J, Bagot M. Service de
Dermatologie, CHU Henri Mondor, 51, avenue du Marechal de
Lattre de Tassigny, 94010 Creteil.
BACKGROUND: End-stage renal failure and long-term hemodialysis
treatment promote porphyria cutanea tarda. Iron overload is
often associated with this disease and is thought to play a
role in its pathogenesis. We report a case of hemodialysis
related-porphyria cutanea tarda improved by deferoxamine. CASE
REPORT: A 45-year-old man, with end-stage renal failure and
who had received hemodialysis treatment since 1993, presented
a several months-history of blisters of the face and the
dorsum of the hands. Laboratory analysis showed: hemoglobin 10
g/dl; a moderate hepatic cytolysis; ferritin 195 ng/l. HIV,
HBV, HCV serologies were negative. Porphyries analyses showed
a porphyria cutanea tarda pattern. The cutaneous histology was
non specific; direct immunofluorescence was negative. The
patient received deferoxamine (40 mg/kg intravenously every
week for 6 weeks) which led to dramatic improvement of the
symptoms. DISCUSSION: Several treatments are proposed in the
management of dialysis-related porphyria cutanea tarda. This
case confirms that deferoxamine can induce rapid and prolonged
remission.
PMID: 12605155 [PubMed - indexed for MEDLINE]
Who loves ya. Tom
Jesus Was A Vegetarian! http://jesuswasavegetarian.7h.com
Man Is A Herbivore! http://tinyurl.com/a3cc3
DEAD PEOPLE WALKING http://tinyurl.com/zk9fk