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PORPHYRIA FACTS: PORPHYRIA & GLUCOSE
GLUCOSE PRIMER
IV Glucose is what intraveous solutions porphyria patients normally referred to.
However, intravenous glucose is medically termed DEXTROSE.
Dextrose is formulated from various elements including SUCROSE,
LEVULOSE, DEXTROSE and FRUCTOSE.
All are synonymous with GLUCOSE.
The cheapest form of iv glucose is a "run-of-the-mill" formulary which has a main
basis of FRUCTOSE.
FRUCTOSE however is contraindicated for persons with corn allergies, corn
syrup allergies, and does contain sulphites.
The better grade forumlary DEXTROSE which is widely available is formulated
from sucrose, levulose and dextrose ---without the fructose.
Many diabetics use this DEXTROSE because the contents is not as sweet as
the corn syrup based FRUCTOSE.
Dextrose and levulose are obtained by the inversion of cane sugar or sucrose,
and hence called invert sugar.
Dextrose is chiefly obtained by the action of heat and acids on starch, and hence
called also starch sugar.
Dextrose is an isomer of glucose that is found in honey and sweet fruits.
Dextrose is also known as glucose or grape sugar or dextroglucose.
Levulose is a simple sugar found in fresh ripe fruits and in honey.
"Fruit sugar"; a very sweet six-carbon sugar that serves as a building block for
more complex sugars and carbohydrates
.
Sucrose is a common variety of sugar found in the juices of many plants,as the
sugar cane, sorghum, sugar maple, beet root, etc. It is extracted as a sweet, and
is largely used in the preservation of fruit. Is it also called saccharose or cane
sugar.
Sucrose proper is a dextrorotatory carbohydrate.
Sucroseis changed by the diastase present to invert sugar (dextrose and
levulose).
It is also present in medicl solutions as galactose.
For patients with allergic reaction to corn products, they need to look for
solutions compounded with fructose.
For those with corn syrup allergy due to the preserving agent, sulphites, they
must also avoid solutions including fructose.
Many of today's common food products contain fructose including most canned
sodas.
If the patient reacts to normal DS or DW DEXTROSE, when it has been
administered correctly, then the patient should be tested for HFI.
HFI = Hereditary Fructose Intolerance.
The fructose in this medicine may cause severe side effects in patients with this
condition does not allow patients to use iv infusion of glucose, when the
Dextrose solution contains Fructose.
All forms are required by law to be distributed by Prescription.
A good hint to check out is the cost factors. Corn syrup based iv glucose runs
around $2.00 US for 1,000 ml bag.
The good formulary grade that is safe for all, runs considerably more averages
about $16 a bag at most hospital pharmacies.
Gary R PHm
Richard B R Phm
Carol F R Phm
++++++++++++++
Glucose Metabolism Effects
A primary source of energy for living organisms. It is naturally occurring and is
found in fruits and other parts of plants in its free state. It is used therapeutically
in fluid and nutrient replacement
Glucose Hypertonic Solutions
Solution that is usually 10 percent glucose but may be higher.
An isotonic solution of glucose is 5 percent.
Levulose
A carbohydrate in sweet fruits and honey that is soluble in water, alcohol, or
ether.
It is used as a preservative and an intravenous infusion in parenteral feeding.
SOURCE:
Grant & Hackh's Chemical Dictionary
5th ed
+++++++++++
LEVULOSE
Hexrose
Any monosaccharide of the general formula C6H12O6; the group includes
glucose, fructose, and galactose.
SOURCE:
Ronald Harrington PhD, RPm
Pharmacology
+++++++++++
:Levulose
Levulose is a simple sugar found in honey and in many ripe fruits
Synonyms: fructose, fruit sugar, laevulose
Hexrose
Any monosaccharide of the general formula C6H12O6; the group includes
glucose, fructose, and galactose.
SOURCE:
Ronald Harrington PhD,
RPmPharmacology
+++++++++++
See also: Fructose
See also: Ketohexose
Laevoluse is a pure form of carbohydrate and has been used to treat acute
porphyria patients to bring attacks into remission.
SOURCE:
The treatment of acute intermittent porphyria with laevulose.
Brodie MJ, Moore MR, Goldberg A.
Clinical Science & Molecular Medicine
1977; 53: 365 71.
++++++++++++
Dextrose
Dextrose, USP is chemically designated C6H12O6• H2O (D-glucose
monohydrate), a hexose sugar freely soluble in water.
Water for Injection, USP is chemically designated H2O
SOURCE:
Ronald Harrington PhD,
RPmPharmacology
+++++++++++
dextrose
Pronunciation: 'dekstrows
WordNet Dictionary
Definition: [n] an isomer of glucose that is found in honey and sweet fruits
Synonyms: dextroglucose, grape sugar
See Also: glucose
Webster's 1913 Dictionary
Definition: \Dex"trose`\, n. [See {Dexter}.] (Chem.)
A sirupy, or white crystalline, variety of sugar, {C6H12O6}
(so called from turning the plane of polarization to the
right), occurring in many ripe fruits. Dextrose and levulose
are obtained by the inversion of cane sugar or sucrose, and
hence called invert sugar. Dextrose is chiefly obtained by
the action of heat and acids on starch, and hence called also
{starch sugar}. It is also formed from starchy food by the
action of the amylolytic ferments of saliva and pancreatic
juice.
The solid Dextrose products are known to the trade as grape sugar; the sirupy
products as glucose, or mixing sirup.
These are harmless, but are only about half as sweet as cane or sucrose.
SOURCE:
Ronald Harrington PhD,
RPmPharmacology
+++++++++++
Dextrose is a six-carbon monosaccharide which is the major sugar in the blood
and is an important intermediate molecule in metabolic processes. It is often
given intravenously to replenish fluids and provide nutrients.
In glycolysis, all six-carbon intermediates are derived from glucose and fructose.
In the first step of glycolysis, hexokinase catalyzes the conversion of glucose
and ATP into glucose 6-phosphate.
Also, in the conversion of one molecule of glucose into two molecules of
pyruvate, two molecules of ATP are also generated.
SOURCE:
Russell Morrison PhD
Biochemistry
++++++++++
Dependent on the manufacturer and the shelf life of a bag od Dextrose, sulphites
may be added to the corn syrup to stop yellowing of the product.
Other names for Dextrose include :Cerelose, D-glucopyranose, Glucose.
SOURCE:
Ronald Harrington PhD,
RPmPharmacology
+++++++++++
FORMS OF GLUCOSE
Sucrose
Sucrose, ordinary table sugar, is probably the single most abundant pure
organic chemical in the world and the one most widely known to nonchemists.
Sucrose, commonest of the sugars, a white, crystalline solid disaccharide
(carbohydrate) with a sweet taste, melting and decomposing at 186°C to form
caramel. It is known commonly as cane sugar, beet sugar, or maple sugar,
depending upon its natural source.
Sucrose has the same empirical formula (C12H22O11) as lactose and maltose
but differs from both in structure.
Hydrolysis of sucrose yields D-glucose and D-fructose; the process is called
inversion and the sugar mixture produced is known as invert sugar because,
although sucrose itself rotates plane-polarized light to the right, the mixture
“inverts” this light by rotating it to the left.
SOURCE:
Ronald Harrington PhD, RPm
Pharmacology
+++++++++++
The ingestion of sucrose failed to have any substantial effect on mood
SOURCE:
Physcology & Behavior
58(3):421-7, 1995)
+++++++++++++
Cellulose
Cellulose is another glucose polymer (molecular weight 150,000-1 million) found
in the cell walls of plants.
Over 50% of the total organic matter in the world is cellulose.
For example, wood is about 50% cellulose, and cotton is almost 100% cellulose.
It is a strong, rigid linear molecule, and these features allow it to be used as the
main structural support for plants.
The glucose units are again held together by linkages, but this time every
second glucose unit is flipped over.
These links are called b,1:4 linkages, and human bodies do not possess the
enzymes necessary to break this bond.
Therefore any cellulose we eat passes through the digestive tract undigested,
and acts as roughage.
Grass feeding animals, such as cows, however, can digest cellulose, since they
have extra stomachs to contain the grass for long periods while it is broken down
by special bacteria.
SOURCE:
Introduction to Organic Chemistry
Streitweiser and Heathcock
MacMillan
1981
+++++++++++++++
Dextrose
When dextrose is administered intravenously this solution restores blood
glucose levels in hypoglycemia and provides a source of carbohydrate calories.
Carbohydrate in the form of dextrose may aid in minimizing liver glycogen
depletion and exerts a protein-sparing action.
Dextrose injection undergoes oxidation to carbon dioxide and water.
Water is an essential constituent of all body tissues and accounts for
approximately 70% of total body weight. Average normal adult requirement
ranges from two to three liters (1.0 to 1.5 liters each for insensible water loss by
perspiration and urine production).
Water balance is maintained by various regulatory mechanisms. Water
distribution depends primarily on the concentration of electrolytes in the body
compartments and sodium (Na+) plays a major role in maintaining physiologic
equilibrium.
SOURCE:
On-line Medical Dictionary,
© 1997-98 Academic Medical Publishing
++++++++++++
Dextrose is an isomer of glucose that is found in honey and sweet fruits [syn:
dextroglucose, grape sugar]
SOURCE:
WordNet ® 1.6,
© 1997 Princeton University
++++++++++++
Dextrose is also called corn sugar and grape sugar, dextrose is a naturally
occurring form of glucose.
SOURCE:
Ronald Harrington PhD, RPm
Pharmacology
+++++++++++
Dextrose is a sirupy, or white crystalline, variety of sugar, C6H12O6 (so called
from turning the plane of polarization to the right), occurring in many ripe fruits.
Dextrose and levulose are obtained by the inversion of cane sugar or sucrose,
and hence called invert sugar.
Dextrose is chiefly obtained by the action of heat and acids on starch, and hence
called also starch sugar.
It is also formed from starchy food by the action of the amylolytic ferments of
saliva and pancreatic juice.
SOURCE:
Webster's Revised Unabridged Dictionary,
© 1996, 1998 MICRA
++++++++++++
Dextrose solution is hypertonic. De sure to read the warnings and precautions.
50% Dextrose Injection, USP is a sterile, nonpyrogenic, hypertonic solution of
dextrose in water for injection for intravenous injection as a fluid and nutrient
replenisher.
Each mL of fluid contains 0.5 g dextrose, hydrous which delivers 3.4 kcal/gram.
The solution has an osmolarity of 2.53 mOsmol/mL (caic.), a pH of 4.2 (3.2 to
6.5) and may contain sodium hydroxide and/or hydrochloric acid for pH
adjustment.
The solution contains no bacteriostat, antimicrobial agent or added buffer
(except for pH adjustment) and is intended only for use as a single-dose
injection. When smaller doses are required, the unused portion should be
discarded with the entire unit.
Dextrose, USP is chemically designated C6H12O6• H2O (D-glucose
monohydrate), a hexose sugar freely soluble in water.
Water for Injection, USP is chemically designated H2O.
SOURCE:
H. Winter Griffith MD
Guide to Prescription Drugs
+++++++++
Hyperosmolar syndrome, resulting from excessively rapid administration of
concentrated dextrose may cause mental confusion and/or loss of
consciousness.
Reactions which may occur because of the solution or the technique of
administration include febrile response, infection at the site of injection, venous
thrombosis or phlebitis extending from the site of injection, extravasation and
hypervolemia.
If an adverse reaction does occur, discontinue the infusion, evaluate the patient,
institute appropriate therapeutic countermeasures and save the remainder of the
fluid for examination if deemed necessary.
SOURCE:
RxPulse
Information Resources
2003
++++++++++
Fructose
Fructose is a fruit sugar, simple sugar found in honey and in the fruit and other
parts of plants.
It is much sweeter than sucrose (cane sugar). It is best obtained by hydrolysis
of inulin, a polysaccharide found in dahlia bulbs and the Jerusalem artichoke.
Chemically it is a monosaccharide (see carbohydrate) with the empirical formula
C6H12O6. It has the same formula as glucose but differs from it in structure (see
isomer).
It is often found with glucose in nature. Glucose and fructose are formed in equal
amounts when sucrose is hydrolyzed by the enzyme invertase or by heating with
dilute acid; the resulting equimolar mixture of fructose and glucose, called invert
sugar, is the major component of honey.
Fructose reacts with Fehling’s solution and can be differentiated from glucose by
its reaction with lime water to form a water-insoluble precipitate, calcium
fructosate. In solution, fructose exists as a ring compound in equilibrium with a
straight-chain form.
SOURCE:
The Columbia Encyclopedia,
Sixth Edition. 2001.
++++++++
Diets containing large amounts of sucrose (a disaccharide of glucose and
fructose) can utilize the fructose as a major source of energy.
The pathway to utilization of fructose differs in muscle and liver. Muscle which
contains only hexokinase can phosphorylate fructose to F6P which is a direct
glycolytic intermediate.
In the liver which contains mostly glucokinase, which is specific for glucose as its
substrate, requires the function of additional enzymes to utilize fructose in
glycolysis.
Hepatic fructose is phosphorylated on C-1 by fructokinase yielding
fructose-1-phosphate (F1P). In liver the form of aldolase that predominates
(aldolase B) can utilize both F-1,6-BP and F1P as substrates.
Therefore, when presented with F1P the enzyme generates DHAP and
glyceraldehyde.
The DHAP is converted, by triose phosphate isomerase, to G3P and enters
glycolysis.
The glyceraldehyde can be phosphorylated to G3P by glyceraldehyde kinase or
converted to DHAP through the concerted actions of alcohol dehydrogenase,
glycerol kinase and glycerol phosphate dehydrogenase.
SOURCE:
Ryan Nettles PhD
Biochemistry
++++++++++
Galactose
Galactose, which is metabolized from the milk sugar, lactose (a disaccharide of
glucose and galactose), enters glycolysis by its conversion to
glucose-1-phosphate (G1P).
This occurs through a series of steps. First the galactose is phosphorylated by
galactokinase to yield galactose-1-phosphate.
Epimerization of galactose-1-phosphate to G1P requires the transfer of UDP
from uridine diphosphoglucose (UDP-glucose) catalyzed by
galactose-1-phosphate uridyl transferase.
This generates UDP-galactose and G1P.
The UDP-galactose is epimerized to UDP-glucose by UDP-galactose-4
epimerase.
The UDP portion is exchanged for phosphate generating glucose-1-phosphate
which then is converted to G6P by phosphoglucose mutase.
SOURCE:
Ryan Nettles PhD
Biochemistry
++++++++++
Glucose
Glucose is chemically considered a simple sugar.
It is the main sugar that the body manufactures.
The body makes glucose from all three elements of food, protein, fat and
carbohydrates, but in largest part from carbohydrates.
Glucose serves as the major source of energy for living cells.
Glucose is carried to each cell through the bloodstream. The cells cannot,
however, use the glucose without the help of insulin.
When sampled in blood, glucose is tested in transit.
SOURCE:
Ronald Harrington PhD, RPm
Pharmacology
+++++++++++
The dextrorotatory form of glucose, C6H12O6·H2O, is found naturally in animal
and plant tissue and derived synthetically from starch.
It is also called dextroglucose..
SOURCE:
WordNet ® 1.6,
© 1997 Princeton University
++++++++++++
Glucose is a also known as Glycerin.
A variety of sugar occurring in nature very abundantly, as in ripe grapes, and in
honey, and produced in great quantities from starch, etc., by the action of heat
and acids. It is only about half as sweet as cane sugar.
Called also dextrose, grape sugar, diabetic sugar, and starch sugar.
Glucose is any one of a large class of sugars, isometric with glucose proper,
and including levulose, galactose, etc.
SOURCE:
Webster's Revised Unabridged Dictionary,
© 1996, 1998 MICRA
++++++++++++
Because Glucose is the unit from which starch, cellulose and glycogen are made
up, and because of its special role in biological processes, there are probably
more glucose groups in Nature than any other organic group.
It is extremely important in Nature as one of the main energy sources for living
organisms, both in plants and animals.
Glucose was first isolated in 1747 from raisins by Andreas Marggraf.
The name glucose was coined in 1838 by Jean Dumas, from the greek glycos,
sugar or sweet), and the structure was discovered by Emil Fischer around the
turn of the century.
In fact, there are 2 forms of glucose, the dextrose). In fact, the full name for
common glucose is D-(+)-glucose, and its chemically correct name (using the
IUPAC systematic naming system for organic molecules) is
(2R,3S,4R,5R)-2,3,4,5,6-pentahydroxyhexanol!
Levulose
Dextrose is also called D-Glucose.
Anhydrous dextrose;
Cartose;
Cerelose;
Corn sugar; D-(+)-Glucose;
Dextropur;
Dextrose;
Dextrosol;
Glucolin;
Grape sugar; Sugar, grape; a-d-glucose; component of Kadalex; D-Glucose,
anhydrous; Dextrose, anhydrous;
GLUCOSE (D)
Hexrose
Any monosaccharide of the general formula C6H12O6; the group includes
glucose, fructose, and galactose.
SOURCE:
Ronald Harrington PhD, RPm
Pharmacology
+++++++++++
SOURCE:
Ronald Harrington PhD, RPm
Pharmacology
+++++++++++
SOURCE:
WordNet ® 1.6,
© 1997 Princeton University
++++++++++++
SOURCE:
Webster's Revised Unabridged Dictionary,
© 1996, 1998 MICRA
++++++++++++
Glycerol
The predominant source of glycerol is adipose tissue.
This molecule is the backbone for the triacylglycerols.
Following release of the fatty acid portions of triacylglycerols the glycerol
backbone is transported to the liver where it it phosphorylated by glycerol kinase
yielding glycerol-3-phosphate.
Glycerol-3-phosphate is oxidized to DHAP by glycerol-3-phosphate
dehydrogenase.
DHAP then enters the glycolytic if the liver cell needs energy.
However, the more likely fate of glycerol is to enter the gluconeogenesis
pathway in order for the liver to produce glucose for use by the rest of the body.
SOURCE:
Michael W. King, Ph.D
Biochemistry Department
Indiana University
School of Medicine
++++++++++++
Glucuronate
Glucuronate is a highly polar molecule which is incorporated into proteoglycans
as well as combining with bilirubin and steroid hormones
.It can also be combined with certain drugs to increase their solubility.
Glucuronate is derived from glucose in the uronic acid pathway.
SOURCE:
Michael W. King, Ph.D
Biochemistry Department
Indiana University
School of Medicine
++++++++++++
Mannose
The digestion of many polysaccharides and glycoproteins yields mannose which
is phosphorylated by hexokinase to generate mannose-6-phosphate.
Mannose-6-phosphate is converted to fructose-6-phosphate, by the enzyme
phosphomannose isomerase, and then enters the glycolytic pathway or is
converted to glucose-6-phosphate by the gluconeogenic pathway of
hepatocytes.
SOURCE:
Ryan Nettles PhD
Biochemistry
++++++++++
GLUCOSE TREATMENT
Management of acute attacks
Managing patients with acute intermittent porphyria involves removing the
precipitating factors, increasing carbohydrate (glucose) intake, controlling pain,
and administering medications.
SOURCE:
AACN Clinical Issues
Critical Care Nursing
1994 Feb;5(1):36-41
Caring for patients with acute intermittent porphyria.
Shively BD, et.aL.
++++++++++++++++++++++
Carbohydrates and parenteral nutrition
Adequate calories are necessary and parenteral nutrition withcarbohydrates may
be necessary.
Attacks may also require therapy for hypertension, pain and epileptic seizures.
SOURCE:
Journal of Photodermatololy, Photoimmunology and Photomedicine
1998 Apr;
14(2):48-54
"Management of the acute porphyrias."
Kauppinen R, M.D. Department of Medicine,
University Hospital
Helsinki, Finland.
++++++++++++++++++++++++
The first line of treatment
The first line of treatment has been the use of GLUCOSE infusion. In the1980's
medical researchers discovered that the use of common glucose provided
enough carbohydrate to the liver wherby it would suppress the overproduction of
porphyrins and bring an acute attack to completion.
In some cases however porphyric attacks continue to persist even after 48 hours
of glucose infusion.
Abbott Laboratories produced the pharmaceutical product.
Panhematin to be used to treat those not responding fully to glucose infusion
therapy.
Hematin, as it is known, unfortunately can not be used by those with a history of
phlebitis or other coagulation factors.
Panhematin
"Panhematin is not recommended for those AIP patients whih have a previous
history of DVT, PE, or phbletis.
Surveys have rvealed that about 37% of all porphyria patients have a history of
previously coagulation problems and therefore are not candidates for the use of
the Panhematin."
SOURCE:
Treatment for AIP
Clinica Chimica Acta
1995
Yutaka Horie et. al.
+++++++++++++++++++
Acute porphyria patients may be infused with glucose to repress the
overproduction of porphyrins.
SOURCE:
Bonkovsky HL.
Advances in understanding and treating 'The little imitator', acute porphyria.
Gastroenterology
1993; 105: 590-94.
++++++++++
The infusion of glucose has been found to be effective in bringing into remission
acute attacks of porphyria.
SOURCE:
Elder GH, Hift RJ.
Treatment of acute porphyria.
Hospital Medicine
2001; 62: 422-25.
++++++++++
Acute attacks of porphyria most often can be brought into remission by the
infusion of glucose.
SOURCE:
Kalman DR
Management of acute attacks in the porphyrias.
Clin Dermatol
1998; 16: 299-306.
++++++++++
Acute porphyria patients may be infused with laevulose to repress the
overproduction of porphyrins during an'acute attack.
SOURCE:
Brodie MJ, Moore MR, Thompson GG, Goldberg A.
The treatment of acute intermittent porphyria with laevulose.
Clin Sci Mol Med
1977; 53: 365-71.
+++++++++++
It is vitally important that the acute porphyria patient consume a high
carbohydrate diet and subsequent infusion of glucose to repress the
overproduction of porphyrins and bring the attack into remission.
SOURCE:
Robert TL, Varella L, Meguid MM.
Nutrition management of acute intermittent porphyria.
Nutrition 1994; 10: 551-55.
++++++++++
Problems of hematin
Hematin is not without side effects.Such side effects include thrombophletis
and renal toxicity.
SOURCE:
Treatment for AIP
Clinica Chimica Acta
1995 page 171-5
Yutaka Horie et al.
+++++++++++++++++++++++++
The use of hematin in acute porphyria patients in which 48 hours of continuous
glucose infusion has not brought the attack into remisison may be infused with
hematin to repress the overproduction of porphyrin precursors.
SOURCE:
Bonkovsky HL, et al.
Repression of the overproduction of porphyrin precursors in acute intermittent
porphyria by intravenous infusions of hematin.
Proc Natl Acad Sci
1971; 68: 2725-9.
++++++++++
The use of hematin in the treatment of acute porphyria patients has been found
to have mixed results and is notwithout risks.
SOURCE:
Tenhunen R, Mustajoki P.
Acute porphyria: treatment with heme.
Seminars in Liver Disease
1998; 18: 53-56.
+++++++++
Glucose remains first choice of treatment
Administration of carbohydrate is the first choice of therapy.
If it does not respond after 48 hours of glucose administration or if develop or
progress, treatment by hematin is indicated to suppress the ALA synthetase
activity [over production of porphyrins in the liver.]
SOURCE:
Treatment for AIP
Clinica Chimica Acta
1995 page 171-5
Yutaka Horie et al
+++++++++++++++++++
The glucose effect
Glucose is most commonly used therapy in the intervention of acute porphyria
attacks.
The "glucose effect" reverses or aborts acute porphyric attacks by reducing the
rate of porphyrin synthesis using normal endogenous energy metabolism.
SOURCE:
"Use of glucose in acute porphyria"
Harris, A.L. et. al.
Journal of Family Medicine
+++++++++++++++++++++
Action of carbohydrate
Carbohydrate loadings represses hepatic ALA synthase and therefore the
reason that glucose in used as treatment during acute attacks.
SOURCE:
"The Porphyrias" Karl E. Anderson M.D.
HEPATOLOGY: A Textbook of Liver Disease
W.B. Saunders Company
Philadephia 1996
+++++++++++++++++++++++
Carbohydrate loading (glucose) is the specific therapy for hepatic porphyria
attacks because it represses hepatic ALA synthase and the overproduction of
ALA and PBG.
SOURCE:
Dr. Karl E. Anderson, MD
(University of Texas Medical School - Galveston)
The Porphyrias
Cecil Textbook of Medicine
W.B. Saunders
++++++++++++++++++++
GLUCOSE THERAPY
Glucose can and does diminish the excess or overproduction of heme
precursors in the liver.
By using preventive glucose you can prevent an attack or by using the glucose
as Intevention therapy you can bring about the recovery from anyone one of the
acute hepatic porphyrias [AIP, VP or HCP]
GLUCOSE therapy is simple.
For those who have only occasional episodes he can be iv accessed through a
Hickman.
For those who regular run preventive glucose or are chronic porphyrics, and
need regular intervention care, the place of a PICC or a PORT is most
desirable.
Many porphyics fail to realize the importance or ignore the importance of their
carbohydrate [sugar] intake.
A steady daily amount is necessary to suppress disease activity.
Remember that daily requirements run 300 mg or better of carbohydrate daily.
During attack 400 mg or better is indicated.
During an acute attack a porphyric's requirement runs 500 mg of
carbohydrate total including both consumed and iv infusion of carbohydrate.
Quite often porphyria patients when they go to the clinic or hospitalwhile
experiencing an acute attack often will relate that they have not felt well enough
to eat or have been unable to eat.
Ironically it is the very treatment they need, high consumption of carbohydrate
containing foods.
Often it is felt that if the porphyria patient had not skipped over the
consumption of carbohydrate containing foods, that they would have aborted
their own acut attacks and not needed to present for ermgency interventional
care.
Preventive therapy is most cost effective, as well as most importantly a healthy
move.
Preventing acute attacks, a porphyric reduces the risks of renal failure, liver
failure, scarring of the liver resulting in cirrhosis or hepatic cancer.
Moreover safe guarding onesself from the possibility of respiratory
failure and death is essential by use of preventive therapy."
SOURCE:
"Stopping Porphyrin Overproduction and the Acute Attack"
The Medical Forum
Allan Vasques MD et al
145-8 6:1999
++++++++++++++++++
Prophylaxis is the basic approach to treatment
"Because of the irreversible and basically untreatable nature of acute attacks of
porphyria, prophylaxis is the primary approach.
Glucose must be administered for 48 hours after which time if attack has not
gone into remission heme arginate may be administered."
SOURCE:
Hereditary Hepatic Porphyrias
J. Katz et. al.
"Anesthesia and Uncommon DIsease:
Pathophysiologic and Clinical Correlations"
1973
W.D. Saunders Co. Philadelphia
+++++++++++++++++++++
"Where vomiting precludes an adequate oral intake of carbohydrate, it is
imperative to admit the patient for intravenous glucose therapy. "
SOURCE:
Porphyria Information Centre
MRC/UCT Liver Research Centre
University of Cape Town S.A.
+++++++++++++++++++++
"Electrolyte imbalances should be monitored during acute attacks."
SOURCE:
"The Porphyrias"
Karl E. Anderson M.D.
HEPATOLOGY:
A Textbook of Liver Disease
W.B. Saunders Company
Philadephia 1996
"Management of fluid and electrolyte imbalances, particularly hyponatremia and
hypomagnesemia, is critical during attacks along with the administeration of
glucose."
SOURCE:
Medicine Journal
August 6 2001
Volume 2, Number 8
+++++++++++++++++++++++
"Management of fluid and electrolyte imbalances is critical during attacks along
with the administeration of glucose."
SOURCE:
Maureen Poh-Fitzpatrick, MD
Department of Internal Medicine
Division of Dermatology
University of Tennessee
College of Medicine
++++++++++++++++++++++++++
Disease suppression
A steady daily amount of carbohydrate is necessary to suppress
disease activity.
SOURCE:
"Stopping Porphyrin Overproduction
and the Acute Attack"
The Medical Forum
Allan Vasques MD et al
145-8 6:1999
+++++++++++++++
PREVENTIVE THERAPY
Glucose reduces the activity of hepatic aminolevulinic acid synthase, the
rate-controlling enzyme of hepatic heme synthesis.
SOURCE:
Medicine Journal
August 6 2001
Volume 2, Number 8
++++++++++++++
By using preventive glucose you can prevent an attack or by using the glucose
as Intevention therapy you can bring about the recovery from anyone one of the
acute hepatic porphyrias [AIP,
VP or HCP]
SOURCE:
"Stopping Porphyrin Overproduction
and the Acute Attack"
The Medical Forum
Allan Vasques MD et al
145-8 6:1999
+++++++++++++
Emphasis is on the prevention of acute neurological attacks through avoidance
of triggers and the rapid administration of iv glucose."
SOURCE:
Disorders of Porphyrins or Metals
Dwight Montgomery Bissell M.D.
# 203
pp1182-7
Cecil Textbook of Medicine
Volume 1 18th Edition
+++++++++++++
Preventing acute attacks, a porphyric reduces the risks of renal
failure, liver failure, scarring of the liver resulting in cirrhosis or hepatic cancer.
Moreover safe guarding onesself from the possibility of respiratory
failure and death is essential by use of preventive therapy.
SOURCE:
"Stopping Porphyrin Overproduction and the Acute Attack"
The Medical Forum
Allan Vasques MD et al
145-8 6:1999
Respiratory failure
+++++++++++++
Using iv infusion of glucose on a regular periodic basis will maintain
sufficient levels of blood glucose therefore stopping overproduction of
porphyrins caused by interrupted heme synthesis.
SOURCE:
Robyn Morgan PhD
United Health SYstems
+++++++++++
Safe guarding onesself from the possibility of respiratory failure and
death is essential and can be done by use of preventive therapy.
SOURCE:
Treating porphyria
Valerian Guzick PhD
College of Medicine
University Medical School
+++++++++++++
Safe guarding onesself from the possibility of respiratory failure and death is
essential by use of preventive therapy. It is also cost effective.
SOURCE:
Stopping Porphyrin Overproduction and the Acute Attack"
The Medical Forum
Allan Vasques MD et al
145-8 6:1999
+++++++++++++++
Preventive therapy is most cost effective, aswell as most importantly a healthy
move.
SOURCE:
"Stopping Porphyrin Overproduction
and the Acute Attack"
The Medical Forum
Allan Vasques MD et al
145-8 6:1999
+++++++++++
By using iv infusion of gluvcose one can control the hepatic heme synthesis
and bring acute attacks of porphyria under control or prevent them.
Maureen Poh-Fitzpatrick, MD
Department of Internal Medicine
Division of Dermatology
University of Tennessee
College of Medicine
+++++++++++++
Preventive therapy is most cost effective, as well as most importantly a healthy
move.
Preventing acute attacks, a porphyric reduces the risks of renal failure, liver
failure, scarringof the liver resulting in cirrhosis or hepatic cancer.
More over safe guarding onesself from the possibility of respiratory failure and
death is essential by use of preventive therapy.
SOURCE:
"Stopping Porphyrin Overproduction and the Acute Attack"
The Medical Forum
Allan Vasques MD et al
145-8 6:1999
+++++++++++++
A porphyria patient can have management of their disease by using iv infusion
of glucose on a regular basis along with high catbohydrate intake.
SOURCE:
Jennifer Sollum
Home Infusion Therapy Division
+++++++++++++
For management of the acute types of porphyria the porphyria patient can use
high carbohydrate intake and the intravenous infusion.
SOURCE:
Clinical Biochemistry
June 1989 22(3): pages181-8
Authors: Moore Michael R, and McColl KE
University Department of Medicine,
Western Infirmary, Glasgow, Scotland
+++++++++++++
Preventing acute attacks, a porphyric reduces the risks of renal failure, liver
failure, scarring of the liver resulting in cirrhosis or hepatic cancer.
SOURCE:
"Stopping Porphyrin Overproduction and the Acute Attack"
The Medical Forum
Allan Vasques MD et al
145-8 6:1999
++++++++++
Regular glucose infusion reduces the activity of hepatic aminolevulinic
acid synthase, the rate-controlling enzyme of hepatic heme synthesis which is
ultimately responsible for acute attacks.
SOURCE:
Medicine Journal
August 6 2001
Volume 2, Number 8
Maureen Poh-Fitzpatrick, MD
Department of Internal Medicine
Division of Dermatology
University of Tennessee
++++++++++++
Glucose plays a key role in metabolism and is related to virtually all will meet,
either directly or indirectly.
Free glucose in humans is in a steady state equilibrium between its storage
form, glycogen and degradative or oxidative pathways which lead to
both energy and intermediates in other metabolic pathways.
Through the use of regular iv infusion a steady level is maintained and
heme synthesis is not interrupted.
SOURCE:
Fern Masters PhD
Biochemistry
Glycogen Studies
+++++++++++++++
Glucose reduces the activity of hepatic aminolevulinic acid synthase, the
rate-controlling enzyme of hepatic heme synthesis.
SOURCE:
Medicine Journal
August 6 2001
Volume 2, Number 8
+++++++++++++
A steady daily amount of carbohydrate is necessary to suppress disease activity.
SOURCE:
"Stopping Porphyrin Overproduction
and the Acute Attack"
The Medical Forum
Allan Vasques MD et al
145-8 6:1999
+++++++++++++++
GLUCOSE RISK FACTORS
It is important not to administer glucose too fast.
Keeping the flow about 150 ml per hour has shown to be most beneficial.
SOURCE:
Regina Jensen MNS, RN
Infusion Therapy
+++++++++++
A retrospective study was performed to determine whether there is an increased
incidence of hyperglycemia in patients not predisposed to hyperglycemia (n =
102) who receive total parenteral nutrition (TPN) dextrose in excess of 4 to 5
mg/kg/min.
Of the 37 subjects administered dextrose at > 5 mg/kg/min, 18 exhibited
hyperglycemia. None did so who received dextrose at
< or = 4 mg/kg/min (n = 19).
TPN dextrose infusion rate was positively correlated with blood glucose
concentration, over and above other variables considered in a multiple
regression, including kcal/kg administered, furosemide or dopamine use,
gender, age, or diagnosis.
Thus, TPN dextrose infusion rates > 4 to 5 mg/kg/min increase risk of
hyperglycemia.
SOURCE:
Hyperglycemia associated with high, continuous infusion rates of total parenteral
nutrition dextrose.
Rosmarin DK, Wardlaw GM, Mirtallo J.
Mount Carmel Medical Center, Columbus, OH 43222, USA.
Nutr Clin Pract.
1996 Aug;11(4):151-6.
++++++++++++
Phlebitis can often be experienced during successive iv infusions.
SOURCE:
Risk factors for infusion-related phlebitis with small peripheral venous catheters.
A randomized controlled trial.
Maki DG, Ringer M.
University of Wisconsin Medical School,
Madison, Wisconsin
Annals of Internal Medicine.
1991 May 15;114(10):845-54.
+++++++++++++
For central venous administration: concentrated dextrose should be
administered via central vein only after suitable dilution.
SOURCE:
Regina Jensen MNS, RN
Infusion Therapy
+++++++++++
Electrolyte deficits, particularly in serum potassium and phosphate, may occur
during prolonged use of concentrated dextrose solutions.
Blood electrolyte monitoring is essential and fluid and electrolyte imbalances
should be corrected.
SOURCE:
Robert Johnson MD
Internal Medicine
+++++++++
Concentrated dextrose solutions should not be administered subcutaneously or
intramuscularly.
SOURCE:
Regina Jensen MNS, RN
Infusion Therapy
+++++++++++
To minimize hyperglycemia and consequent glycosuria, it is desirable to monitor
blood and urine glucose and if necessary, add insulin.
SOURCE:
Regina Jensen MNS, RN
Infusion Therapy
+++++++++++
Essential vitamins and minerals also should be provided as needed along with
the iv infusion of glucose.
SOURCE:
Sheryl Wilson MNS, RD
+++++++++++++
Do not use glucose unless the solution is clear and seal is intact.
Discard unused portion.
SOURCE:
Regina Jensen MNS, RN
Infusion Therapy
++++++++++
When concentrated dextrose infusion is abruptly withdrawn, it is advisable to
follow with the administration of 5% or 10% dextrose injection to avoid rebound
hypoglycemia.
Solutions containing dextrose should be used with caution in patients with
known subclinical or overt diabetes mellitus.
SOURCE:
Regina Jensen MNS, RN
Infusion Therapy
+++++++++++
Care should be exercised to insure that the needle is well within the lumen of the
vein and that extravasation does not occur. If thrombosis should occur during
administration, the injection should be stopped and corrective measures
instituted.
SOURCE:
Regina Jensen MNS, RN
Infusion Therapy
+++++++++++
For information regarding IV infusion systems one can contact Bard.
Bard Access Systems Address: 5425 W. Amelia Earhart Drive, Salt Lake City,
UT 80116
Phone: 800-545-0890
Clinical Hotline: 800-555-PICC or 800-555-7422
Fax: 801-595-4948
Internet Site: http://www.bardaccess.com
Bard oversees the following:
PRODUCTS - Catheters and Implanted Ports for Venous Access
Hickman® and Groshong® catheters,
SlimPort® and BardPort® implanted ports,
Opti-Flow® and Niagara™ dialysis catheters,
Per-Q-Cath® and Groshong® catheters,
and the Site-Rite® Ultrasound Device.
+++++++++++++
Hidden dangers of IVs
Those transparent flexible plastic iv bags may not be as innocuous as they look!
A group of chemicals known as phthalates are used to manufacture flexible
plastic products such as plastic wrap, baby bottles, styrene cups, iv bags and
medical tubing. Phthalates do not bind to the plastics that they modify and
readily outgas or leech into fluids. The smell associated with new cars is
primarily produced by phthalates outgassing. The familiar smell of new plastic
products is similarly produced by phthalates. To give an idea of the widespread
use of phthalates one needs only to consider that approximately one billion
pounds of phthalates are manufactured every year?even after being banned in
the US for use in young children's toys!
There has been a growing level of concern over the use of phthalates in medical
supplies such as iv bags and medical tubing.
http://www.anapsid.org/cnd/mcs/vinyl.html
http://www.nursingworld.org/tan/99marapr/ivbags.htm
http://www.cedar.at/mailarchives/infoterra/1999/msg00810.html
http://www.commondreams.org/pressreleases/feb99/022299a.htm
http://www.bbraunusa.com/news/IValert.pdf
High levels of phthalates have been found in solutions contained in iv bags.
Permanent ports or chatheters made of phthalate containing products would
leach these chemical into the body.
Although most of the concern about high levels of phthalates has focused on
their carcinogenic properties another area that may be of equal if not greater
concern for those with porphyria is their abiltiy to act as endocrine disruptors.
Phthalates chemically resemble many hormones making them potent
environmental endocrine disruptors capable of interfering with human
development and increasing hormone-related problems.
The chemical and food industry have launched a vehement campaign claiming
that small amounts of phthalates are harmless.
http://www.tompaine.com/feature.cfm/ID/2896
This may be true in relation to the carcinogenic properties of phthalates but what
they don't tell you as that these chemicals can accumulate in the tissues of the
human body or that much lower levels of these substances can induce hormonal
changes than are required to induce cancer.
http://www.sciencenews.org/20000902/bob1.asp
http://www.sciencenews.org/20000909/fob3.asp
The ability of phthalates to disrupt the delicate hormone balance of the body
may be of even greater concern for those with porphyria than the general
population since hormone distrurbances are known to not only induce the acute
attack but to also trigger cutaneous symptoms.
Those receiving chronic glucose or heme ivs should insist that all plastics used
in ports, bags, tubes etc. be phthalate-free.
Those receiving regular infusions of heme may be at even greater risk as
phthalates leech more readily into blood products.
There are phthalate-free medical supplies available in the US. Phthalate-free
medical supplies are more readily available in Europe at this time due to an
increased level of concern there over problems with phthalates in medical
supplies.
These concerns have been diminished in the US by a number of semi-official
and official reports influened by big business claiming that phthalates are safe.
It is believed that only educated consumer concerns and demands for
phthalate-free medical supplies will drive real change in the marketplace.
California legislators are looking at these concerns and may take actions to ban
phthalate containing medical supplies.
More information about the concerns and dangers of phthalates can be found at
the following links:
http://www.checnet.org/healthehouse/chemicals/chemicals-detail.asp?Main_ID=
281
http://www.greenpeace.org/~toxics/reports/medprods.pdf
http://www.nihe.org/pdfs/Nurses_new.pdf
++++++++++++++++++++
GLUCOSE INFUSION
How does a porphyria patient know how much nutritional value is in a bag of
dextrose?
5% DW at 150 ml per hour = 7.5 grams of glucose per hour
5% DW at 150 ml per hour = 56 calories per hour
For a 24 hour period ( 4 -1,000 bags back-to-back) = 180 grams of glucose.
For a 24 hour period (4 - 1,000 bags back-to-back = 1344 calories
*To bring AIP into remission a total of 400 grams of carbohydrate is needed.
An additional 220 grams of carbohydrate must be consumed.
If DW 10 is being used one would realize 15 grams per hour. which would = 360
grams by infusion with 40 remaining to be consumed.
Formula:
7.5 grams of carbohydrate = 56 calories
SOURCE:
"Me and AIP"
Porphyria Educational Services
+++++++++++++++++++++++++++++++++
What is parental feeding?
If a patients is unable to consume orally an nutrients the patients may undergo
parental feeding.
This is a total intravenous feeding which is important to administer glucose and
other nutrients
Such a feeding is used if an acute attack is severe or prolonged, sufficient
glucose can be given by vein to meet the total energy requirements of a patient.
This is best accomplished by a catheter that is inserted into a large central vein.
Additional nutrients, including vitamins, minerals, amino acids and fat can be
given in the required amounts to maintain all requirements. Provision of total
nutritional needs in this manner by vein is commonly called "total parenteral
nutrition".
SOURCE:
Nutritional Guidelines For Porphyria
AIP Medical Guide
Sheryl Wilson, [HCP], MSN, RD
+++++++++++++++++++++++++
Can parental feeding be added to the iv glucose infusions during acute attacks?
Corrective nutrition may be added to or 'piggy-backed" during intravenous
administration."
SOURCE:
Disorders of Porphyrins or Metals
Dwight Montgomery Bissell M.D.
# 203
pp1182-7
Cecil Textbook of Medicine
Volume 1 18th Edition
+++++++++++++++++++++++++
Is it necessary to eat while having infusions during an acute attack?
Adequate calories are necessary and parenteral nutrition with carbohydrates
may be necessary in adition to glucose or heme infusions.
SOURCE:
Photodermatology, Photoimmunology and Photomedicine
1998 Apr;14(2):48-51
Management of the acute porphyrias.
Kauppinen R.
Department of Medicine,
University Hospital of Helsinki,
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