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Diabetes Associated with Porphyria
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PORPHYRIA FACTS: DIABETES ASSOCIATED WITH PORPHYRIA

Diabetes is associated with many genetic disorders including the porphyrias.

SOURCE:
Diabetes secondary to genetic disorders.
Robinson, S., and A. Kessling.
Baillieres
Clinical Endocrinology & Metabolism
1992; 6:867-98..
++++++++++++++

Diabetes II can be associated with the acute porphyrias as a secondary
manifestation of porphyria.

SOURCE:
Robert Joihnson MD
++++++++++++++

The relationship between diabetes and the porphyrias is not new.

The association of diabetes and porphyria been know for many decades.

SOURCE:
Porphyria, diabetes, and their relationship.
Burnham, T.K. and R. P. Fosnaugh.
Archives of Dermatology
1961; 83:717-722.
++++++++++++++++

A diabetic metabolism may stop or prevent attacks of AIP - which is logical
according to the findings in many clinical and experimental studies on the effects
of carbohydrates.

SOURCE:
Effects of diabetes mellitus on patients with acute intermittent porphyria
C. Andersson, I. Bylesjö & F. Lithner
Journal of Internal Medicine
Volume 245 Issue 2 Page 193 -
February 1999
+++++++++++++

The higher prevalence of diabetes among those with porphyria than the general
population is well-known and well documented.

SOURCE:
Cutaneous Manifestations of Diabetes
Chakrabarty, A., R. A. Norman, and T. J. Phillips.
Wounds
2002; 14:267-274.
++++++++++++++

Diabetes generally precedes the onset of porphyria, a possible result of the
nonenzymatic glycosylation of the heme pathway.

SOURCE:
Cutaneous Manifestations of Diabetes
Chakrabarty, A., R. A. Norman, and T. J. Phillips.
Wounds
2002; 14:267-274.
+++++++++++++

Porphyria cutanea tarda (PCT) has a known increased incidence of diabetes
mellitus and hepatic involvement.

SOURCE:
Carbohydrate metabolism in porphyria cutanea tarda.
Franks, A. G., Jr, et. al.
American Journal of Medical Science
1979; 277:163-71..
++++++++++++

Amongst AIP patients with HCC, only one with signs of diabetes was identified
(impaired glucose tolerance test).

Diabetes mellitus may be beneficial for patients with severe AIP in minimizing
acute attacks and at the same time saving AIP patients from HCC.

SOURCE:
Effects of diabetes mellitus on patients with acute intermittent porphyria
C. Andersson, I. Bylesjö & F. Lithner
Journal of Internal Medicine
Volume 245 Issue 2 Page 193 -
February 1999
++++++++++++


Although a change in the lean:fat ratio is common in many neuromuscular
disorders, including the porphyrias, mechanisms other than insulin resistance
would seem to operate.

The increased incidence of diabetes in heterozygotes for some of these genetic
disorders raises the possibility that many common diabetics are, in fact,
heterozygotes for some other disorder.

SOURCE:
Diabetes secondary to genetic disorders.
Robinson, S., and A. Kessling.
Baillieres
Clinical Endocrinology & Metabolism
1992; 6:867-98..
+++++++++++++++

Acute intermittent porphyria was diagnosed according to standard clinical and
biochemical criteria including analysis of urinary ALA and PBG, PBGD and DNA
mutation.

Patients were deemed to have atent AIP when they showed laboratory evidence
of AIP, despite never experiencing symptoms.

Hypertension was defined as a condition treated with antihypertensive drugs,
which was decided on clinical grounds

Diabetes mellitus was defined according to the WHO criteria.

SOURCE:
Effects of diabetes mellitus on patients with acute intermittent porphyria
C. Andersson, I. Bylesjö & F. Lithner
Journal of Internal Medicine
Volume 245 Issue 2 Page 193 -
February 1999
+++++++++++++
Diabetes may be associated with many genetic disorders including the
porphyrias.

SOURCE:
Diabetes secondary to genetic disorders
Clinical Endocrinology & Metabolism
1992; 6:867-98..
++++++++++++++++


In laboratory studies rates with porphyria were diabetically induced.

Studies of the enzymes showed degradationand metabolic alteration of enzymes
in the heme synthesis, stopping overproduction of porphyrins.

Rat subjects were found to no longer present with severe acute attacks.

SOURCE:
Diabetes-induced metabolic alterations
in heme synthesis and degradation
and various heme-containing enzymes
Bitar M & Weiner M.
Diabetes
1984; 33: 37 44.
+++++++++++++++

None of the diabetic patients with AIP had HCC.

Of all the AIP patients with HCC registered, none had diabetes, whereas in a
population-based group of individuals in southern

Sweden (mean age 67 years), diabetes was found in 12.8% of the men and
15.0% of the women.

This suggests that diabetes also counteracts HCC in AIP patients, probably by
normalization of ALA.

SOURCE:
Beneficial Effect of Diabetes on Acute Intermittent Porphyria
Folke Lithner, MD, PHD
Department of Internal Medicine
University Hospital
Umea, Sweden
Diabetes Care
American Diabetes Association, Inc.
2002; 25:797-798, 2002
+++++++++++++

In several syndromes there is an increased incidence of diabetes in otherwise
unaffected relatives of individuals with these syndromes such as the porphyrias.

It is impossible to assess what proportion of common NIDDM or IDDM is made
up of heterozygotes for these genetic syndromes.

SOURCE:
Diabetes secondary to genetic disorders
Robinson, S., and A. Kessling.
Baillieres
Clinical Endocrinology & Metabolism
1992; 6:867-98..
+++++++++++++++

By maintaining blood serum AIC readings within normal range, a porphyria
patient will most likely keep their porphyria in remission.

SOURCE:
Robert Johnsoin MD
Internal Medicine
+++++++++++++++

When a porphyria patient has diabetes and keeps their diabetes AIC within the
normal range, there is clinical evidence that porphyria will remain in remission.

SOURCE:
Effect of diabetes on porphyric attacks.
Yalouris AG & Raptis SA.
British Medical Journal
1987; 295: 1237 8.
+++++++++++++++

Diabetes mellitus may be beneficial for patients with severe AIP.

SOURCE:
Effects of diabetes mellitus on patients with acute intermittent porphyria
C. Andersson et. al.
Journal of Internal Medicine
Volume 245
Issue 2 Page 193 -
February 1999
+++++++++++++++++
Up to 25% of those with PCT, the most common type of porphyria, have
diabetes--a diabetes that is often undiagnosed leading to serious complications.

SOURCE:
Cutaneous Manifestations of Diabetes
Chakrabarty, A., R. A. Norman, and T. J. Phillips.
Wounds
2002; 14:267-274.
+++++++++++++++

Diabetes II has been clinically indicated in at least a quarter if PCT patients.

SOURCE:
Robertr JOhnson MD
Internal Medicine
++++++++++++++


An alteration of the porphyrin metabolism is thought to be responsible for
diabetes is PCT patients.

SOURCE:

Insulin resistance in porphyria cutanea tarda.
Calcinaro, F., et. al.
Journal of Endocrinology Investigation
1989; 12:393-39..
+++++++++++++++

Studies clearly indicates the association of diabetes with PCT.

Since diabetes mellitus may remain asymptomatic in some of the PCT
patients, an early diagnosis of the former is likely to be missed unless looked
for.

SOURCE:
Glucose tolerance and glycosuria in Ethiopian porphyria cutanea tarda patients.
Mengistu, M.
Tropical Geogr Medicine
1987; 39:361-35..
+++++++++++++++


Diabetes is also more common in the acute porphyrias than in the general
population.

SOURCE:
Diabetes secondary to genetic disorders.
Robinson, S., and A. Kessling.
Baillieres
Clinical Endocrinology & Metabolism
1992; 6:867-98..
++++++++++++++

There is a higher prevalence of Diabetes II in the porphyria population than in
the general population.

SOURCE:
Robert Johnsoin MD
Internal Medicine
+++++++++++++

For patients with severe AIP, it is a great relief to be spared the attacks.

For elderly patients with latent AIP or with no attacks in recent years, diabetes
could of course cause diabetic complications such as distal gangrene or cardiac
failure.

However, for these patients diabetes may cause their AIP to be latent in the
future.

SOURCE:
Effects of diabetes mellitus on patients with acute intermittent porphyria
C. Andersson, I. Bylesjö & F. Lithner
Journal of Internal Medicine
Volume 245 Issue 2 Page 193 -
February 1999
+++++++++++++


The mechanism of diabetes is more obscure in acute intermittent porphyria
(AIP), although haem deficiency affecting the cytochrome chain raises
interesting possibilities.

SOURCE:
Diabetes secondary to genetic disorders.
Robinson, S., and A. Kessling.
Baillieres
Clinical Endocrinology & Metabolism
1992; 6:867-98..
++++++++++++++


The scientific importance of these often rare disorders such as the porphyrias,
resides in the insight they may provide into the possible mechanisms of common
diabetes.

The type of diabetes varies in these syndromes.

Non-insulin-dependent diabetes (NIDDM), clinically similar to common NIDDM,
may be found in some syndromes.

SOURCE:
Diabetes secondary to genetic disorders.
Robinson, S., and A. Kessling.
Baillieres
Clinical Endocrinology & Metabolism
1992; 6:867-98..
++++++++++++++

Hypoglycemia occurs when your body's blood sugar, or glucose, is abnormally
low.

Hypoglycemia results when your body's glucose is used up too rapidly, when
glucose is released into the bloodstream more slowly than is needed by your
body.

Hypoglycemia is relatively common in acute porphyria patients

Hypoglycemia and onset of acute porphyria attacks both happen when not
enough carbyhydrate containing food is eaten, or from a sudden increase in the
amount of exercise without an increase in food intake.

Sometimes the cause of hypoglycemia is unknown, or idiopathic but has become
realized in acute porphyria as a "supply and demand" signal for carbohydrate
intake.

People who are not diabetic and who do not have another known causes of
hypoglycemia experience hyperglycemia due to low intake of carbohydrates.

It has long been known that Hypoglycemia can occur because of an liver
disease such as porphyria.

Here is a simple formula to remember the relationship of serum blood glucose
monitoring and onset of porphyria.

Here is a simple formula:

LOW BLOOD SUGAR = LOW CARBOHYDRATE LEVEL = TRIGGERING OF
ACUTE PORPHYRIA

It is a known factor in acute porphyrias, that over production of porphyrins
begins when the body is lacking in carbohydrate.

An acute attack of porphyria can be triggered by inadequate

caloric intake and more specificaly insufficient amounts of

carbohydrate.

When carbohyrate levels falls in the body, the serum blood sugar levels falls as
well, thus when blood testing is done, the results will show low end blood sugar
levels. The normal range runs 70-110 for blood sugar monitoring.

When a porphyria patients is nearing the onset of an attack due to insufficient
carbohydrate levels the body begins overproduction of porphyrins which
eventually trigger an attack.

At this same time when carbohydrate levels are low enough to trigger porphyrin
production, a porphyria patient will often experience hyperglycermia.

Blood testing will indicate blood serum levels below 70, and the symptoms of
hyperglycemia will become noticeable in the patient.

Many acute porphyria patients have found relative sucess in monitoring their
blood sugars to be a good indicator of pending porphyria activity.

When a patient feels tired, ill-feeling, perhaps overly tired without good reason, it
is time to take a blood serum glucose testing.

While a normal fasting blood serum range runs 70-110, anything below 75 for a
porphyria patient who carboloads, should be a "heads up" .

If 60 or below the porphyria patient needs to alert a caretaker and begin
infusion.

Dr. Robert Johnson
Internal Medicine
+++++++++++++++

If you have a diagnosis of an acute hepatic porphyria and diabetes II then you
need to learn as much as possible about carbohydrates.

Low intake of carbohydrate is known to trigger acute attacks of porphyria.

Low intake of carbohydrate also will lead to hypoglycemic levels of blood serum
glucose testing

Acute attacks are caused when an overproduction of porphyrins occurs within
the liver for the forms of AIP, HCP and VP.

While the exact science or reason for glucose is not fully understood, it is known
that the intake of glucose (the purest form of carbohydrate) will stop the over
production of porphyrins and thus bring an acute attack into remission.

Carbohydrates are your body’s main energy source.

Carbohydrates are used not only in your liver to maintain the right level of
porphyrin production which takes place there, but carbohydrate are used
throughout the entire body.

Carbohydrates are used in the brain as its primary source of fuel. That gives
meaning to the old caption stating "Brain Power".

And what makes carbohydrates? At the base of all carbohydrates are sugar
components.

There are different kinds of carbohydrates and this important for porphyria
patients to remember.

Depending on the number of components and how they’re linked, a
carbohydrate is classified as a simple carbohydrate (sugar) or a complex
carbohydrate (starch).

After being consumed the body breaks down complex carbohydrates into simple
sugars.

And what makes up simple sugars? Sweets, milk, fruit and some vegetables
contain simple sugars.

And what makes up complex carbohydrates? Grain products and certain
vegetables contain complex carbohydrates.

Another name for the complex carbohydrates could be that of starches.

Starches are complex carbohydrates and include bread, cereal, rice, pasta,
beans and certain vegetables, such as corn, potatoes and squash.

Simple sugars are contained in fruit and dairy products.

Every form of fruit, from the familiar apples, bananas and oranges to pears
contains simple sugars. Milk and milk products contain simple sugars.

The vegetables with simple sugars includes all nonstarchy vegetables, such as
lettuce, asparagus and zucchini.

When planning your meals for throughout the day is best to eat a mixture of
complex and simple carbohydrates.

The advantage of complex carbohydrates is that it takes your body longer to
break them down into sugar.

This means sugar enters your bloodstream at a prolonged rate.

With some simple carbohydrates, sugar may enter your bloodstream quickly.

For porphyria patients this is excellent for maintaining a continuous intake of
carbohydrate into the liver.

For those porphyria patients who are also diabetic, this continuous flow of
carbohydrates is excellent in maintaining a rather constant blood serum glucose
level.

The more fiber the food contains, the more slowly it’s digested and the more
slowly your blood sugar level rises which benefits diabetes, but is not beneficial
in carbo-loading pending the onset of an attack, however it is good in
maintaining a constant carbohydrate level for controlling porphyrin production as
long as there is a continuous carbohydrate intake.

By spreading carbohydrate intake every 2-3 hours through your waking hours
and also including a carbohydrate "middle of the night" snack, you will effectively
achieve maintaining a continuous carbohydrate level in the blood stream and
liver.

At the same time you will achieve maintaining a steady blood serum glucose
level which will keep your diabetes under control.

The best way to maintain porphyrin production in the liver and at the same time
to control blood serum glucose levels

One is by eating the same amount of carbohydrates at similar times throughout
the day.

Nutritional Guidelines for Porphyria
AIP Medical Guide
Sheryl Wilson (HCP), MSN, RD
+++++++++++++++

The heme pathway is impaired in porphyrias.

A frequent coexistence of diabetes mellitus and porphyria disease has been
found.

SOURCE:
Delta aminolevulinate dehydratase (ALA-D) activity in human and experimental
diabetes mellitus.
Fernandez-Cuartero B, et. al.
Department of Internal Medicine
University Complutense
Madrid, Spain.
Int J Biochem Cell Biol.
1999 Mar-Apr;31(3-4):479-88.
+++++++++

The relationship between diabetes and the porphyrias is not new but has been
know for many decades.

SOURCE:
Porphyria, diabetes, and their relationship
Burnham, T.K. et. al.
Archives of Dermatology
83:717-722.
1961
++++++++++

There are direct parallels between carbohydrate metabolism and diabetes due to
heme pathway impairment in some porphyrias, especially in AIP.

SOURCE:
Lewis Gabbert PhD
Biochemistry
+++++++++++

Heme deficiency in the liver of AIP patients stimulates an increase in
ALA-synthase which triggers an escalating metabolic chain reaction, leading to
an increase in the porphyrin content.

This reaction can be reduced by treating AIP patients with glucose.

SOURCE:
Effects of diabetes mellitus on patients with acute intermittent porphyria
C. Andersson, I. Bylesjö & F. Lithner

Journal of Internal Medicine
Volume 245 Issue 2 Page 193 -
February 1999
++++++++++

Diabetes is also more common in the acute porphyrias than in the general
population:

SOURCE:
Diabetes secondary to genetic disorders
Robinson, S., and A. Kessling.
Baillieres
++++++++++

Porphyria cutanea tarda and diabetes have also been linked to HCV.

SOURCE:
Extrahepatic manifestations of hepatitis C infection.
Mayo MJ.
Department of Internal Medicine
Division of Digestive and Liver Diseases
University of Texas
Southwestern Medial Center
Dallas, Texas 75390-9151
American Journal of Med Science.
2003 Mar;325(3):135-48.
++++++++++++

Often acute porphyria patients develop type II diabetes.

SOURCE:
Type 2 Medicines
Medicine Cabinet
Remedy
Catherine Grillo
Winter 2003
+++++++++++




Porphyria cutanea tarda (PCT) has a known increased incidence of diabetes
mellitus and hepatic involvement.

SOURCE:
Glucose tolerance and glycosuria in PCT
Mengistu, M.
Tropical Geogr Medicine
39:361-35.1987
++++++++++++

There is a higher prevalence of diabetes among porphyria patients than in the
general population.

SOURCE:
Diabetes secondary to genetic disorders
Clinical Endocrinology and Metabolism
6:867-98.1992
++++++++++

Diabetes Type II has normally be treated with medications called sulfonylureas
which stimulate the pancreas to produce more insulin.

*Sulfonylureas are contraindicated for acute porphyria patients.

SOURCE:
Type 2 Medicines
Medicine Cabinet
Remedy
Catherine Grillo
Winter 2003
+++++++++++

Design. A population-based study of porphyria patients having DNA-verified AIP
having type 2 diabetes were studied in Sweden.

Prevalence of diabetes was studied retrospectively in AIP patients with
hepatocellular carcinoma (HCC).

None of the patients showed symptoms of AIP after the onset of their diabetes.

Diabetes mellitus may be beneficial for patients with severe AIP.

SOURCE:
Effects of diabetes mellitus on patients with acute intermittent porphyria
C. Andersson, I. Bylesjö & F. Lithner
Journal of Internal Medicine
Volume 245 Issue 2 Page 193 -
February 1999
++++++++++



Studies show that diabetes is beneficial in stopping severe acute attacks in AIP.

A study was conducted a population-based study on AIP patients aged 18 years
living in northern Sweden, in which 319 participated (95%).

A total of 16 patients (5 women) with AIP and type 2 diabetes were found, with a
mean age of 67 years.

Eight of these patients had AIP symptoms, with three patients suffering severe,
recurring attacks.

After the onset of their diabetes, no patient suffered attacks or any other AIP
symptoms.

SOURCE:
Beneficial Effect of Diabetes on Acute Intermittent Porphyria
Folke Lithner, MD, PHD
Department of Internal Medicine
University Hospital, Umea, Sweden
Diabetes Care
2002; 25:797-798
++++++++++++++++++


Diabetes II been found to benefit AIP patients in stopping acute symptoms.

AIP patients who had had recurrent, severe attacks for many years found that
when their diabetes became manifest, their urinary ALA and PBG levels
decreased and the AIP symptoms resolved, to the relief of the patients.

SOURCE:
Effects of diabetes mellitus on patients with acute intermittent porphyria
C. Andersson, I. Bylesjö & F. Lithner
Journal of Internal Medicine
Volume 245 Issue 2 Page 193 -
February 1999
+++++++++++++++++

The decrease in activity of erythrocyte aminolevulinate dehydratase observed in
diabetic patients, may represent an additional and useful parameter for the
assessment of the severity of carbohydrate metabolism impairment in porphyria
patients.

SOURCE:
Delta aminolevulinate dehydratase (ALA-D) activity in human and experimental
diabetes mellitus.
Fernandez-Cuartero B, et. al.
Department of Internal Medicine
University Complutense
Madrid, Spain.
Int J Biochem Cell Biol.
1999 Mar-Apr;31(3-4):479-88.
+++++++++

Manifest AIP is associated with an increased prevalence of hypertension or
renal lesions, also evident in type 2 diabetes.

SOURCE:
Effects of diabetes mellitus on patients
with acute intermittent porphyria
C. Andersson, I. Bylesjö & F. Lithner
Journal of Internal Medicine
Volume 245 Issue 2 Page 193 -
February 1999
+++++++++++++++++++

Diabetes in conjunction with porphyria beneficial in avoiding HCC as a long term
complication of acute porphyria.

SOURCE:
Robert Johnson MD
++++++++++++++++

AIP patients who had had recurrent, severe attacks for many years found that
when their diabetes became manifest, their urinary ALA and PBG levels
decreased and the AIP symptoms resolved, to the relief of the patients.

SOURCE:
Effects of diabetes mellitus on patients with acute intermittent porphyria
C. Andersson, I. Bylesjö & F. Lithner
Journal of Internal Medicine
Volume 245 Issue 2 Page 193 -
February 1999
++++++++++++++++++
In a clinical review of a Swedish community, a close association
of diabetes II and acute porphyria has been indicated.

SOURCE:
Prevalence and incidence of
diabetes in a Swedish community
Andersson DKG, Svärdsudd K, Tibblin G.
1972-87. Diabetic Med 1991; 8: 428 34.
+++++++++++++++

A population-based study of all patients >18 years of age having DNA-verified
AIP (n = 328) living in the two most northerly counties of Sweden (Norrbotten
and Västerbotten, with 550 000 inhabitants) of whom 16 had type 2 diabetes.

Prevalence of diabetes was studied retrospectively in 26 AIP patients with
hepatocellular carcinoma (HCC).

None of the patients showed symptoms of AIP after the onset of their diabetes.

SOURCE:
Effects of diabetes mellitus on patients
with acute intermittent porphyria
C. Andersson, I. Bylesjö & F. Lithner
Journal of Internal Medicine
Volume 245 Issue 2 Page 193 -
February 1999
++++++++++++++++
Diabetes associated with AIP is also associated with renal symptoms.

SOURCE:
Renal symptomatology in patients with acute intermittent porphyria.
C. Andersson, et. al.
A population-based study.
Journal of Internal Medicine
2000; 248: 4, 319-325
+++++++++++++++++




Studies indicate that having diabetes is beneficial in AIP as to the probability of
progressing to HCC in AIP.

In a study of AIP patients with HCC we found only one with diabetes - and this
disease was diagnosed as latent - when in this age group in Sweden, 12.8% of
men and 15.0% of women are diabetic.

A concomitant diabetes may in the long run reduce the risk of HCC, which
mostly affects elderly persons with AIP.

SOURCE:
Effects of diabetes mellitus on patients
with acute intermittent porphyria
C. Andersson, I. Bylesjö & F. Lithner
Journal of Internal Medicine
Volume 245 Issue 2 Page 193 -
February 1999
+++++++++++++++++++++++
Medical research dindings indicate that Diabetes may benefit patients with
severe AIP.

SOURCE:
Effects of diabetes mellitus on patients
with acute intermittent porphyria
C. Andersson, I. Bylesjö & F. Lithner
Journal of Internal Medicine
Volume 245 Issue 2 Page 193 -
February 1999
++++++++++++++++++++

Often acute porphyria patients develop type II diabetes.

SOURCE:
Type 2 Medicines
Medicine Cabinet
Remedy
Catherine Grillo
Winter 2003
++++++++++++

What have clinical studies hown in regard to AIP and diabetes?



During several weeks in the hospital ward, it was possible to closely examine
and study the course of developing diabetes in patients who had previously
been hospitalized at the Department of Medicine due to AIP attacks on 46
occasions.

After menopause, AIP was still unremitting, with recurrent attacks.

However, after developing a metabolically rather mild type 2 diabetes, the AIP
symptoms subsided.

Mean values on 13 occasions of testing for urinary porphobilinogen (PBG)
during 6 months before and on 11 occasions during the first 4 months after she
developed diabetes were 65.9 and 10.2 µmol/l, respectively (reference levels
1.3–11.0 µmol/l) (P = 0.0001).

Five years later, the patient is still free from AIP symptoms, the levels of ALA
and PBG in her urine fluctuate around normal or just above the upper reference
levels, and she leads "a good life."

SOURCE:
Beneficial Effect of Diabetes
on Acute Intermittent Porphyria
Folke Lithner, MD, PHD
Department of Internal Medicine
University Hospital
Umea, Sweden
Diabetes Care
American Diabetes Association, Inc.
+++++++++++++++++++++

Consumption of a diet with high free glucose can promote the development of
oxidative stress associated with hyperglycemia.

SOURCE:
Oxidative stress is dependent on the free glucose content of the diet.
Folmer V, et. al.
Departamento de Quimica,
Centro de Ciencias Naturais e Exatas,
Universidade Federal de Santa Maria,
97105-900 Santa Maria, RS, Brazil.
International Journal of
Biochemistry & Cell Biology
2002 Oct;34(10):1279-85.
+++++++++++++

Type 2 Diabetes is a disease in which the body is unable to use and / or produce
insulin properly.

SOURCE:
Type 2 Medicines
Medicine Cabinet
Remedy
Catherine Grillo
Winter 2003
+++++++++++++

Prevalence of diabetes was studied retrospectively in acute porphria patients
with hepatocellular carcinoma (HCC).

The end result was that none of the patients showed symptoms of AIP after the
onset of their diabetes.

Three patients had had recurrent, severe porphyriaattacks for many years but
when their diabetes became manifest, their urinary ALA and PBG levels
decreased and the porphyria symptoms resolved.

This study raises the possibility that diabetes mellitus may be beneficial for
patients with severe acute hepatic porphyria."

SOURCE:
Effects of diabetes mellitus on patients with acute intermittent porphyria.
Andersson C, Bylesjo I, Lithner F
Primary Health Care Centre,
Arjeplog, Sweden.
++++++++++++++++

Do acute attacks subside when an AIP patients becomes diabetic?

Recurrant AIP attacks cease when the patients become diabetic.

SOURCE:
Beneficial Effect of Diabetes
on Acute Intermittent Porphyria
Folke Lithner, MD, PHD
Department of Internal Medicine
University Hospital
Umea, Sweden
++++++++++++++++++

Diabetes Type II has normally be treated with medications called sulfonylureas
which stimulate the pancreas to produce more insulin.

*Sulfonylureas are contraindicated for acute porphyria patients.

SOURCE:
Type 2 Medicines
Medicine Cabinet
Remedy
Catherine Grillo
Winter 2003
++++++++++++++
The heme synthetic pathway is impaired in porphyria and a frequent coexistence
of diabetes mellitus and porphyria disease has been reported in humans
and experimental animal models, which can be casually linked to the
delta-ALA-D inhibition found in diabetics.

SOURCE:
Oxidative stress is
dependent on the free glucose
content of the diet.
Folmer V, et. al.
Departamento de Quimica,
Centro de Ciencias Naturais e Exatas,
Universidade Federal de Santa Maria,
97105-900 Santa Maria, RS, Brazil.
International Journal of
Biochemistry & Cell Biology
2002 Oct;34(10):1279-85.
++++++++++++++

n several syndromes there is an increased incidence of diabetes in otherwise
unaffected relatives of individuals with these syndromes.

It is impossible to assess what proportion of common NIDDM or IDDM is made
up of heterozygotes for these genetic syndromes.

SOURCE:
Porphyria, diabetes, and their relationship
Burnham, T.K. et. al.
Archives of Dermatology
83:717-722.
1961
+++++++++++++++

Type II Diabetes drugs include chlorpropamide known as Diabinese and
glimepiride known as Amaryl.

SOURCE:
Type 2 Medicines
Medicine Cabinet
Remedy
Catherine Grillo
Winter 2003
++++++++++++++

The prevalence of AIP patientswith diabetes is lower than the prevalence of
diabetic patients in the general population.

Studies shows that diabetes mellitus may be beneficial for patients
with severe AIP.

SOURCE:
Diabetic metabolism protective
in severe acute intermittent porphyria
Andersson C, Lithner F.
Institute of Medicine
Umea University
Lakartidningen.
2001 Dec 19;98(51-52):5874-6
++++++++++++++++

Diabetes may be associated with many genetic disorders including the
porphyrias.

SOURCE:
Porphyria, diabetes, and their relationship
Burnham, T.K. et. al.
Archives of Dermatology
83:717-722.
1961
++++++++++++++

The higher prevalence of diabetes among those with porphyria than the general
population is well-known and well documented.

Up to 25% of those with PCT, the most common type of porphyria, have
diabetes--a diabetes that is often undiagnosed leading to serious complications.

There are hundreds of papers documenting the relationship between diabetes
and the porphyrias.

SOURCE:
Cutaneous Manifestations of Diabetes
Chakrabarty, A., R. A. Norman, and T. J. Phillips.
Wounds 14:267-274.
2002
++++++++++++

One-fourth of porphyria cutanea tarda (PCT) patients have diabetes.

Diabetes generally precedes the onset of porphyria, a possible result of the
nonenzymatic glycosylation of the heme pathway.

SOURCE:
Insulin resistance in PCT
Calcinaro, F.G. et. al.
Journal of Endocrinology Investigation
12:393-39.1989.
+++++++++++++

Acute intermittent porphyria (AIP) is characterized by attacks of abdominal pain
and neuropsychiatric symptoms.

SOURCE:
Diabetes Care
25:797-798, 2002
++++++++++++

About half of those patients carrying the gene encoding for AIP have have
experienced attacks with abdominal pain, and more severely affecting women.

AIP patients have been found to benefit from co-existing diabetes.

SOURCE:
Folke Lithner, MD, PHD
Department of Internal Medicine
++++++++++++

In biochemical terms, AIP is an autosomal hereditary metabolic aberration
resulting from a partial defect in the activity of the third-step enzyme
(porphobilinogen deaminase [PBGD]) during the course of heme synthesis.

SOURCE:
Beneficial Effect of Diabetes on Acute Intermittent Porphyria
Folke Lithner, MD, PHD
Department of Internal Medicine
University Hospital
Umea, Sweden
++++++++++++

Carbohydrate ingestion blocks d-aminolevulinic acid (ALA)-synthase, as has
been demonstrated in numerous clinical and experimental studies.

The mechanisms by which carbohydrates modulate the components of
porphyrins and heme synthesis are highly complex and only partially elucidated
to date.

SOURCE:
Diabetes Care
25:797-798, 2002
++++++++++++

The main long-term complications of AIP are polyneuropathy, hepatocellular
carcinoma (HCC), and renal insufficiency.

SOURCE:
Beneficial Effect of Diabetes on Acute Intermittent Porphyria
Folke Lithner, MD, PHD
Department of Internal Medicine
University Hospital
Umea, Sweden
++++++++++++
Treatment of AIP patients entails treating both the symptoms and the
complications, but also requires an endeavor to reverse the fundamental
disease by prescribing a carbohydrate-rich diet and by treating the attacks with
intravenous infusions of glucose.

SOURCE:
Diabetes Care
25:797-798, 2002
++++++++++++

In a study study on AIP patients with AIP and type 2 diabetes

it was found that after the onset of their diabetes, no patient suffered attacks or
any other AIP symptoms.

SOURCE:
Beneficial Effect of Diabetes on Acute Intermittent Porphyria
Folke Lithner, MD, PHD
Department of Internal Medicine
University Hospital
Umea, Sweden
++++++++++++

AIP is often unremitting, with recurrent attacks.

However, after developing a metabolically rather mild type 2 diabetes, the AIP
symptoms subside, clinically evidence has shown.

The levels of ALA and PBG in urine fluctuate around normal or just above the
upper reference levels, and one lead "a good life."

SOURCE:
Diabetes Care
25:797-798, 2002
++++++++++++

AIP attacks cease when the porphyria patient becames diabetic.

SOURCE:
Beneficial Effect of Diabetes on Acute Intermittent Porphyria
Folke Lithner, MD, PHD
Department of Internal Medicine
University Hospital
Umea, Sweden
++++++++++++

Porphyria can be linked to the delta-ALA-D inhibition found in diabetics.

SOURCE:
Oxidative stress is dependent on the free glucose content of the diet.
Folmer V, Soares JC, Rocha JB.
Departamento de Quimica
Centro de Ciencias Naturais e Exatas
Universidade Federal de Santa Maria
97105-900 Santa Maria, RS, Brazil.
Int J Biochem Cell Biol.
2002 Oct;34(10):1279-85
++++++++++


The consumption of a diet with high free glucose can promote the development
of oxidative stress that we tentatively attribute to hyperglycemia.

SOURCE:
Oxidative stress is dependent on the free glucose content of the diet.
Folmer V, Soares JC, Rocha JB.
Departamento de Quimica
Centro de Ciencias Naturais e Exatas
Universidade Federal de Santa Maria
97105-900 Santa Maria, RS, Brazil.
Int J Biochem Cell Biol.
2002 Oct;34(10):1279-85
++++++++++


Diabetes also counteracts HCC in AIP patients, probably by normalization of
ALA

SOURCE:
Beneficial Effect of Diabetes on Porphyria
Diabetes Care
25:797-798, 2002
++++++++++++

High cholesterol is commonly associated with porphyria patients with
co-extisting diabetes.

SOURCE:
Prevalence of hyperlipidemia in persons with hypertension and/or diabetes
mellitus
Assmann G & Schulte H.
American Heart Journal
1988; 116: 1713 24.
+++++++++++++




In AIP attacks there is an escalating metabolic chain reaction leading to heme
deficiency and increased levels of porphyrin precursors.

After onset of diabetes AIP patients had their ALA and PBG levels decreased
and the AIP symptoms resolved, to the relief of the patients.

SOURCE:
Diabetic metabolism protective in severe acute intermittent porphyria
Andersson C, Lithner F.
Institute of Medicine
Umea UniversityUmea, Sweden
Lakartidningen.
2001 Dec 19;98(51-52):5874-6.
++++++++++++++

In a study of AIP patients with hepatocellular carcinoma (HCC) none had
diabetes.

This implies that diabetic metabolism may prevent the development of HCC in
patients with AIP.

SOURCE:
Diabetic metabolism protective in severe acute intermittent porphyria
Andersson C, Lithner F.
Institute of Medicine
Umea UniversityUmea, Sweden
Lakartidningen.
2001 Dec 19;98(51-52):5874-6.
++++++++++++++

The prevalence of AIP patients with diabetes was lower than the prevalence of
diabetic patients in the general population.

SOURCE:
Diabetic metabolism protective in severe acute intermittent porphyria
Andersson C, Lithner F.
Institute of Medicine
Umea UniversityUmea, Sweden
Lakartidningen.
2001 Dec 19;98(51-52):5874-6.
++++++++++++++

The heme synthetic pathway is impaired in porphyria and a frequent coexistence
of diabetes mellitus and porphyria disease.

SOURCE:
Oxidative stress is dependent on the free glucose content of the diet.
Folmer V, Soares JC, Rocha JB.
Departamento de Quimica
Centro de Ciencias Naturais e Exatas
Universidade Federal de Santa Maria
97105-900 Santa Maria, RS, Brazil.
Int J Biochem Cell Biol.
2002 Oct;34(10):1279-85
++++++++++


Studies have shown that diabetes mellitus may be beneficial for patients with
severe AIP.

SOURCE:
Diabetic metabolism protective in severe acute intermittent porphyria
Andersson C, Lithner F.
Institute of Medicine
Umea UniversityUmea, Sweden
Lakartidningen.
2001 Dec 19;98(51-52):5874-6.
++++++++++++++

The consumption of a diet with high free glucose can promote the development
of oxidative stress that we tentatively attribute to hyperglycemia.

SOURCE:
Oxidative stress is dependent on the free glucose content of the diet.
Folmer V, Soares JC, Rocha JB.
Departamento de Quimica
Centro de Ciencias Naturais e Exatas
Universidade Federal de Santa Maria
97105-900 Santa Maria, RS, Brazil.
Int J Biochem Cell Biol.
2002 Oct;34(10):1279-85
++++++++++

The heme synthetic pathway is impaired in porphyria and a frequent coexistence
of diabetes mellitus and porphyria disease.

SOURCE:
Oxidative stress is dependent on the free glucose content of the diet.
Folmer V, Soares JC, Rocha JB.
Departamento de Quimica
Centro de Ciencias Naturais e Exatas
Universidade Federal de Santa Maria
97105-900 Santa Maria, RS, Brazil.
Int J Biochem Cell Biol.
2002 Oct;34(10):1279-85
++++++++++

In animals, chronic intake of diets with high proportions of rapidly absorbable
glucose promotes the development of insulin resistance.

High levels of glucose can produce permanent chemical alterations in proteins
and lipid peroxidation.

SOURCE:
Oxidative stress is dependent on the free glucose content of the diet.
Folmer V, Soares JC, Rocha JB.
Departamento de Quimica
Centro de Ciencias Naturais e Exatas
Universidade Federal de Santa Maria
97105-900 Santa Maria, RS, Brazil.
Int J Biochem Cell Biol.
2002 Oct;34(10):1279-85
++++++++++



The consumption of a diet with high free glucose can promote the development
of oxidative stress that we tentatively attribute to hyperglycemia.

SOURCE:
Oxidative stress is dependent on the free glucose content of the diet.
Folmer V, Soares JC, Rocha JB.
Departamento de Quimica
Centro de Ciencias Naturais e Exatas
Universidade Federal de Santa Maria
97105-900 Santa Maria, RS, Brazil.
Int J Biochem Cell Biol.
2002 Oct;34(10):1279-85
++++++++++


Additional resource link:

http://care.diabetesjournals.org/cgi/content/full/25/4/797

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