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Pyroluria / HPU

BuzzNutrition
This information is provided for reference only. Anne Buzzelli is a Registered Dietitian, not a physician.
Always consult a qualified and experienced health care provider for their opinion about your care plan.
Pyroluria treatment requires physician care because of the multiple serious consequences of mineral imbalance.

Symptoms of Pyroluria/HPU
This is a list of the most common Pyroluria symptoms1.
If you relate to 15 or more of these symptoms, you may want to get tested!
o
o
o
o
o
o
o
o
o
o
o
o

o
o
o
o

Insomnia
White spots on finger nails
Poor morning appetite / tendency
to skip breakfast
Morning nausea
Pale skin (especially face) / poor
tanning / burn easy in sun
Sensitivity to bright light
Hypersensitive to loud noises
Reading difficulties (e.g. dyslexia)
Poor ability to cope with stress
Mood swings or temper outbursts
Argumentative/enjoy argument
A tendency toward feeling
anxious, fearful and carrying
lifelong inner tension
Much higher capability/alertness
in the evening, versus mornings
White spots under fingernails
Dry skin
Tendency to be edgy

o
o
o
o
o
o
o
o
o
o
o
o
o
o
o

Easily upset by criticism


Difficulty digesting, a dislike of
protein or history of vegetarianism
Tendency toward being a loner
and/or avoiding large groups
Poor sense of smell or taste
Feel very uncomfortable with
strangers
Frequently experience fatigue
Prone to frequent colds or
infections
Poor short term memory
Difficulty recalling past events or
people
Bouts of depression or nervous
exhaustion
A tendency toward anemia
Prone to acne, eczema or psoriasis
Episodic anger/explosive temper
ADHD symptoms
Unpleasant body odor

o
o

o
o
o
o
o
o
o
o

Hypoglycemia
New situations or changes in
routine particularly stressful (i.e.,
traveling, new school schedule)
Tendency to overreact to
tranquilizers, barbiturates, alcohol
or other drugs (small amount
produces a powerful response)
Dramatic (histrionic) tendency
History of mental illness or
alcoholism in family
Little or no dream recall
Delayed puberty; stunted growth,
late growth spurt
Stretch marks on skin
Sweet smell (fruity odor) to breath
or sweat when ill or stressed
Abnormal body fat distribution
Belong to an all-girl family with
look-alike sisters

About Pyroluria/HPU2,11
What is Pyroluria?
A genetic abnormality in hemoglobin synthesis which occurs with stress.
Too much kryptopyrrole is produced resulting in deficiency of zinc and vitamin B6
o Hemoglobin: protein that holds iron in red blood cells & allows transportation of oxygen
o Kryptopyrrole: byproduct of hemoglobin synthesis that tightly binds zinc and B6, and pulls it
out of the body

Whats the problem?
Everyone produces kryptopyrroles as natural byproducts of hemoglobin synthesis
Kryptopyrroles bind to zinc, vitamin B6 (pyridoxine) and remove them from the body.
People with Pyroluria make way too many kryptopyrroles, which remove too much zinc and B6 and
cause deficiencies.
Anne Buzzelli MS RD CBP CMTA
110 W Johnson St #228/12, Staunton VA
Anne@BuzzNutrition.com
phone: 540.414.7525, fax: 206.309.7493

Pyroluria / HPU
BuzzNutrition

o Manganese and Arachidonic acid also become deficient.


o Some believe chromium also becomes deficient.Error! Bookmark not defined.
o These nutrients have crucial roles in enzyme activation, metabolism and immune response.
Amount of kryptopyrrole and severity deficiencies vary for every individual.
Is related to stress- if a person with Pyroluria lives a completely uneventful life and never gets an
infection, they may never see Pyroluria symptoms.


Whats the fallout? Deficiency in these key nutrients can predispose a person to numerous problems.
Immune
Weakened as white blood cells are less able to fight infection & clear out toxins
System
May predispose a person to chronic illness (Lyme infection, fibromyalgia, chronic
fatigue, etc.) and prevent them from ever fully recovering.Error! Bookmark not defined.
With defective heme, less oxygen is carried on the blood (anemia). This promotes
the proliferation of microbes because they love low-oxygen environments.Error!
Bookmark not defined.

Zinc, copper, and iron are extremely important to the white blood cells, the
soldiers of the immune system.
Vitamin B6 (P5P) is required to absorb zinc, manganese, magnesium and
chromium.Error! Bookmark not defined.
This is especially the case in the presence of susceptible HLA haplotypes.
Anxiety,
B6 is required in the last step of serotonin synthesis. Serotonin is one of the
Depression
neurotransmitters that modulate mood by creating a sense of peace and
wellbeing.
Heavy metals Deficiency of zinc may result in excess copper and heavy metals like mercury, lead and
cadmium. When supplementing with zinc, these heavy metals will leave and a binder,
such as chlorella, should be added to the supplement menu.
Alcoholism
Some people with Pyroluria use alcohol to calm their anxiety, feel more sociable and
experience normalcy for a short time. As a result, some people with Pyroluria may
become alcoholics.
Mineral
Zinc and copper compete with each other and must stay in balance.3
imbalance
300 of 1500 known metabolic enzymes are zinc dependent.
Zinc and copper arent just minerals- theyre also regarded as neurotransmitters
and the right ratio is required for stable mood and behavior.
Sources of copper: chocolate, nuts, beans, seeds, grains, some multis,
fungicides/pesticides, IUDs/birth control pills. (*Meat has copper but its balanced
by plenty of zinc.)
When zincs deficient, there will likely be excess copper. Copper toxicity can have
troubling effects, such as sensitive skin and PMS symptoms/painful menstruation
Anne Buzzelli MS RD CBP CMTA
110 W Johnson St #228/12, Staunton VA
Anne@BuzzNutrition.com
phone: 540.414.7525, fax: 206.309.7493

Pyroluria / HPU
BuzzNutrition

and, in extreme cases, anxiety, panic attacks, paranoia and hallucinations.


Nutrients that help reduce copper (antagonists): glutathione, vitamin B1, B3, B6,
folate, inositol, choline, zinc, manganese, iron, sulfur, and molybdenum. (Caution
with iron: its very easy to get too much iron.)


Is Pyroluria more than just genetics?
Its never, ever possible to separate the body, mind and emotions. Every condition has something to
do with the way a person perceives their environment and what emotions are triggered. Never
underestimate the power of the subconscious mind.
Dr. Dietrich Klinghardt believes there are 3 potential causes: genetics, early childhood trauma and
chronic infections (its possible that biotoxins can block enzymes involved with heme synthesis).11
A person diagnosed with Pyroluria should alert their family since it is a genetic disorder.

There is great hope for those with chronic illness who find they have Pyroluria:
Once all of the bodily systems are back online and functioning properly, a few months after
introducing the HPU protocol, patients are essentially made invulnerable to Lyme disease, to molds,
even to heavy metals.11

Involvement in recognized disorders


Pyroluria may be an underlying factor for conditions that are thought to be the main culprit, or for
which causes have been elusive.
These conditions may include: autism, aspergers, alcoholism, schizophrenia, depression, ADD/ADHD,
chronic fatigue, fibromyalgia, frequent infections (bacterial/viral/parasitic/fungal), rheumatoid
arthritis, bipolar disorder, obsessive compulsive disorder, violent behavior, panic attacks, extremely
oppositional/defiant children/teens, Tourette Syndrome and more.
If Pyroluria is not addressed, treatment with both traditional and holistic therapies may be in vain.
11% of the general population has some degree of pyroluria but 52% of schizophrenics and 42% of
psychiatric patients are pyroluric. It is a hereditary condition and will show up to various degrees of
severity in family members. Alcoholic families are very prone to have this condition as 40% of
alcoholics are pyroluric.4

Extra Info:
Pyroluria is classified as an inborn error of metabolism (nice way of saying inherited defect)
Its also a porphyrin disease (heme, the protein in red blood cells, is a porphyrin compound)
Other names for Pyroluria: HPU (hemopyrrollactamuria), KPU (kryptopyrroluria), Mauve Factor,
Malvaria
Discovered separately by Doctors Carl Pfeiffer and Abram Hoffer
Anne Buzzelli MS RD CBP CMTA
Anne@BuzzNutrition.com

110 W Johnson St #228/12, Staunton VA


phone: 540.414.7525, fax: 206.309.7493

Pyroluria / HPU
BuzzNutrition

Lab Tests for Pyroluria/HPU


Pyroluia is diagnosed based on how much kryptopyrrole is excreted in the urine. Many labs offer a test
kit that you complete at home and then send via overnight mail to the lab (shipping price included).
5

1. Health Diagnostics and Research Institute


a. In addition to kryptopyrrole, can add HPL (another indicative compound) for no charge
b. $80 (can only be ordered by health care professionals)
2. Great Plains Laboratory6
a. $89 (anyone can order)
b. Under some insurance plans, Great Plains is an in-network provider. CPT code = 84999
3. Riordan Clinic/ Bio-Center Laboratory7
a. $73 (anyone can order)
4. Pyroluriatesting.com8
a. $80 (anyone can order)

o
o

For best possible results from the urine test,


consider using these instructions instead of those sent with the kit11:
Avoid all supplements for 5-7 days (especially those containing zinc, biotin and B6)
Take a 24-hour urine collection, as opposed to first-mornings urine
o Keep in a clean large container (glass juice jar) for collection.
o Store container in the refrigerator.
o Keep container shielded from light to protect kryptopyrroles (keep in dark cupboard and/or
wrap in aluminum foil)
o Add 500 mg ascorbic acid for every liter (4 cups) of urine, as a preservative.
Be sure to have normal daily stress that day- dont go to the spa (kryptopyrrole excretion increases
during stress).
Wrap aluminum foil around transport tube before mailing it to the lab.
Freeze urine sample properly and make sure cold pack has been frozen for long enough.
Check with lab to determine what days theyre open- be sure it will arrive on one of those days.

o
o
o

*Proper urine handling is very important (I hope you laughed at that)
Kryptopyrroles are very sensitive to heat and bright light.
If the lab result is <3.0, it was probably degraded by heat or light.

Anne Buzzelli MS RD CBP CMTA


Anne@BuzzNutrition.com

110 W Johnson St #228/12, Staunton VA


phone: 540.414.7525, fax: 206.309.7493

Pyroluria / HPU
BuzzNutrition

Diagnosis of Pyroluria/HPU
Kryptopyrrole lab test
o Normal: <10mcg/dl
o Mild Pyroluria: 10-20 mcg/dl
o Moderate Pyroluria: 20-50 mcg/dl
o Severe Pyroluria: >50 mcg/dl

Other lab results can be suggestive of HPU11
o White blood cells <5000/mcL (from zinc deficiency)
o High LDL cholesterol, low HDL
o High MCV (mean corpuscular volume; indicates macrocytic anemia from deficiency
of folate, B6 or B12)
o Low manganese, lithium and molybdenum in a hair mineral test
o Very low or very high hair zinc: both correlate with low plasma levels. Pyroluria
causes a very high Zn excretion in both urine and hair.
o Low normal alkaline phosphatase (<60 U/L)
o Low omega-6 fatty acids in red cell membrane test
o Low taurine in amino acid profile

If all the symptoms fit, yet the test comes back negative:
Assume a false negative and retest.
Try the supplementation regimen (under doctors supervision) and if you feel
better you can assume the test was a false negative.

Anne Buzzelli MS RD CBP CMTA


Anne@BuzzNutrition.com

110 W Johnson St #228/12, Staunton VA


phone: 540.414.7525, fax: 206.309.7493

Pyroluria / HPU
BuzzNutrition

Treatment for Pyroluria/HPU


Zinc, B6, manganese, biotin and Arachidonic acid must be repleted. The proper forms and amounts of
nutrients must be used. A physicians participation is required to ensure safety. This info is provided to
help you and your doctor work together.

Supplementation protocols proposed by Dr. Dietrich Klinghardt11




Simpler alternative:
Core, a product made by BioPure USA that contains B6, zinc, manganese, chromium, molybdenum,
boron, L-Taurine and Magnesium9.
o Caution: Contains 2 forms of vitamin B6 (Pyridoxine HCl and Pyridoxal-5-Phosphate). Some
may not tolerate both, in which case, the more extensive protocol is necessary.
Still need to supplement with Arachidonic acid:
o Foods: eggs, butter, red meat and liver
o Supplements of GLA (gamma linolenic acid): black currant seed oil and evening primrose oil.
Another option for zinc supplementation is 4-6 oysters per day.

More details on supplements
*Since every body is different, adjustment will be necessary to find the right levels of each nutrient.
Manganese is added because it is depleted by high levels of zinc
Anne Buzzelli MS RD CBP CMTA
Anne@BuzzNutrition.com

110 W Johnson St #228/12, Staunton VA


phone: 540.414.7525, fax: 206.309.7493

Pyroluria / HPU
BuzzNutrition

Magnesium is added because it is depleted by high levels of B6.


Niacinamide (vitamin B3) is used to make tryptophan, with is necessary to make serotonin.
Vitamin C and pantothenic acid may help relieve exhaustion from adrenal fatigue experienced from
dealing with ongoing stress.
Omega 3s (in fish oils) may make pyroluria symtoms worse. Omega 3 & 6 compete for certain
enzymes and if omega 6 is already deficient, adding more omega 3 intensifies imbalance.


Results can be very fast!10
For mild cases, major improvements can be seen within a couple days. The more severe the
symptoms, the longer it will take to see results.
Its common to see early improvement, then a plateau followed by further improvement.
Will take longer if person has severe mental illness, other significant chemical imbalance or a
malabsorptive condition. (A few weeks to notice improvement and 3-6 months to become stable.)
o Malabsorption can be caused by reactions to food, especially gluten. Real success may not be
realized until problem foods are removed.

Treatment must include stress management & trauma resolution.
The body will not recover unless the mind is onboard.
Unresolved emotions and damaging subconscious beliefs will perpetuate stress.

*Supplementation must continue for life.
Lower maintenance doses will be needed for life to compensate for bodys genetic tendency to
produce defective heme proteins.
Once supplementation is stopped, symptoms will return in 7-14 days.

Checking on progress:
Recheck kryptopyrroles with urine test.
Enough zinc?: test with zinc tally test (liquid taste test available from Thorne)
o If its hard to replete zinc, heavy metal toxicity may be an issue, especially mercury, cadmium,
lead and copper.
Enough B6?: subjective assessment of ability to regularly remember dreams/daydreams.

Caution:
Since zinc competes with copper for entrance to cells, high doses of zinc may prevent copper from
being absorbed (called a functional deficiency). Copper is important for the immune system and may
need to be supplemented at 3-6 mg/day. Copper levels should be evaluated between months 3 & 4
of an HPU treatment protocol.11
Anne Buzzelli MS RD CBP CMTA
Anne@BuzzNutrition.com

110 W Johnson St #228/12, Staunton VA


phone: 540.414.7525, fax: 206.309.7493

Pyroluria / HPU
BuzzNutrition

Zinc also displaces heavy metals. Two-six weeks after treatment, symptoms of heavy metal detox
could come up. May as well have a proper binding agent on board early to mop up the metals.
Chlorella is the binder of choice (12g/day total: 4 grams, 3 times/day, 30 minutes before meals. After
a few months and symptoms disappear, a lower maintenance dose can be taken- ideally for life).

Pyroluria in Children
Symptoms of Pyroluria increase/intensify with age. In children, it may be less easy to detect. The
following may be indications of Pyroluria.12 Testing a child cant hurt its fun to pee in a cup!
paleness of the skin, especially the face
crowded teeth, many cavities
recurrent ear infections, colds
hypermobility of the joints
allergy, hay fever, skin reactions
growing pains, especially of the left knee
headache, migraine
changes in handwriting
easy bruising
white marks on the nails
anemia
sensitivity to sunlight
upper left abdominal pain
loss of appetite
constipation, or mucus in stool with bloating stretch marks on the skin
light colored stool
sweetish breath odor
learning and behavioral problems
convulsions

Since people with Pyroluria are stress-sensitive, the ideal discipline for a child with Pyroluria is
something that is "short and sweet" and non-violent, rather than "long and lingering."2

Pyroluria likely results in copper toxicity because zinc and copper compete for entrance to cells.
Because copper activates MAO and DAO enzymes the histamine level normally is very low in HPU-
children. These children may look healthy, but they are often over stimulated, hyperactive, depressive
and have a high threshold for pain. Because of their insomnia they turn night into day.12


Resources
Gedgaudas, N. www.primalbody-primalmind.com/?p=398 (lots of good references at end of article)
2
Kaslow, J. http://drkaslow.com/html/pyroluria.html (lots of good references here, too)
3
Kresser, C. http://chriskresser.com/rhr-could-copper-zinc-imbalance-be-making-you-sick
4
Vernon, T. http://www.tvernonlac.com/copper-toxicity.html
5
Health Diagnostics and Research Institute: www.EuropeanLaboratory.nl (Click Lab Tests Specific Tests Kryptopyrrol in urine)
6
Great Plains Laboratory: www.greatplainslaboratory.com/home/eng/kryptopyrrole.asp
7
Riordan Clinic/ Bio-Center Laboratory:
www.riordanclinic.org/mm5/merchant.mvc?Screen=PROD&Store_Code=BCL&Product_Code=LPyr&Category_Code
8
Pyroluriatesting.com:
www.pyroluriatesting.com/?page=shop/flypage&product_id=136&CLSN_1407=13264006121407bab69e96684c534d5e
9
About Core supplement: http://www.biopureeurope.com/core-mineral.html
10
Walsh, W. http://www.alternativementalhealth.com/articles/walshMP.htm
1

Anne Buzzelli MS RD CBP CMTA


Anne@BuzzNutrition.com

110 W Johnson St #228/12, Staunton VA


phone: 540.414.7525, fax: 206.309.7493

Pyroluria / HPU
BuzzNutrition
11

Forsgren, Scott. Explore! Volume 18(6), 2009. Available here:


betterhealthguy.com/joomla/images/stories/PDF/kpu_klinghardt_explore_18-6.pdf
12
Centre for Environmental Medicine (KEAC) in the Netherlands. http://www.hputest.nl/ewhat.htm
http://healthy-family.org/caryn/63/why-am-i-so-edgy-learn-about-pyrrole-disorder-pyroluria
http://altmentalhealth.blogspot.com/2008/08/pyroluria-and-mental-health.html
Copper Toxicity: http://www.tvernonlac.com/copper-toxicity.html

Anne Buzzelli MS RD CBP CMTA


Anne@BuzzNutrition.com

110 W Johnson St #228/12, Staunton VA


phone: 540.414.7525, fax: 206.309.7493

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