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KRYPTOPYRROLE / HPU / HPL / PORPHYRIA / B6 & Zinc

Posted Feb 10 2009 11:32am 1 Comment

Remember that this is juat a post of my blog, and it evolves, so to see the full story go to: www.pochoams.blogspot.com (English) or www.sfc-tratamiento.blogspot.com (Spanish)
My current doc: Josepa Rigau Av Catalunya, 12, 3º, 1ª 43002 Tarragona Spain +34977220358 (I do recommend! hoeopathy and biological medicine, significant improvement)


I post this information on KRYPTOPYRROLES, because is the second time that I indicate a positive in this therothycally genetic deficiency of B6 and Zinc.

The first time I did, it was in KEAC Laboratories in The Netherlads with a result ofHemopyrrollactamcomplex * 0,9 Ref < 0,6 uMol/L (Slightly positive)

The second time I did was last December in ELN Laboratories in The Netherlands, with a result ofHPL---------------------------------*461 Ref Range 15-250 (Positive)

Both of them measure Kryptopyrrole, and when is positive implies a structural deficiency of B6 and Zinc.

Explanation on HPU:

The HPU test measures the amount of haemopyrrollactam complex in the urine. HPU is a situation in which the body excretes a certain compound called HPL-complex, in the urine.

HPU is an abbreviation of hydroxyhaemopyrrollactumuria. The haemopyrrollactam complex, also called HPL or hydroxyl-HPL, consists of hydroxil-2, 3-dimethylpyrrolidone-5-on and hydroxyhaemopyrrolinon-2-on complexed with pyridoxal-5-phosphate (active vitamin B6) and minerals as zinc and manganese.

The excretion of HPL shows a variety of zero till a few decades of micromoles per litre (uMol/l) and is increased in the case of all kinds of stress. Besides pyrrols also increased concentrations of porphyrins, such as coproporphyrin I, are found in the urine of HPU-patients as well.

HPU can be described as a familiar double deficiency of zinc and vitamin B6 (pyridoxal-5-phosphate). These deficits are linked to the production of a group of chemical compounds that are called pyrrols and porphyrins, and are excreted with the urine. That is why HPU is also called porphyrinuria or toxic induced porphyria.

Because a deficit of vitamin B6 (pyridoxal-5-phosphate), reduces the absorption of zinc, chromium and -to a lesser degree- also manganese and magnesium, one could call it a deficit of only pyridoxal-5-phosphae. Vitamin B6 also plays a role in the production of vitamin B3 from trytophan. This vitamin is often decreased in HPU too. The extent of the deficits are so large that they cannot be counterbalanced by foods that are rich in these vitamins or minerals.

Interpretation of the test:

< 0.4 uMol/L HPU absent
0.4-0.6 uMol/L HPU weak, doubtful
0.6-1.0 uMol/L HPU present, light positive
1.0-2.5 uMol/L HPU positive
2.5-4.0 uMol/L HPU strong positive
>4.0 uMol/L HPU very strong positive

HPL was determined according to GLP-Norms. For children other reference values are used than for adults. Children with HPL values above 0.65 uMol/L that are younger than ten years old, or girls older than ten, but do not menstruate yet, as well as children with values above 0.75 uMol/L that are older than ten years old, or girls younger than ten years old that already menstruate, all are considered POSITIVE. Their value will increase during the onset of puberty. A value above 0.6 uMol/L implies that a treatment should be started for at least 4 months.

Present till light positive result:
The excretion of HPL shows a variation between zero and 20 uMol/L, and it increases with all kind of stress: psychological stress, physical stress, such as flu, burns, sun burns, the use of porphyrinogenic pharmaceuticals or the presence of infections will increase the amount of HPL in the urine. The difference between light positive and positive is only arbitrary. A person with a 1.2 value is not necessarily more ill than another one with 0.65. In patients that are chronically ill, much lower values are found, because HPL is not excreted in the morning urine but though out the day, and a 24h urine test is recommended to be certain.

False Positives:
Some illness can influence on the value of HPU excreted on the upside. Mononucleosis, alcoholism, hyperthyroidism, pernicious anaemia, hepatitis, malaria, bartter syndrome, liver cirrhoses, crigler-najjar disease, Gilbert disease, sferocytosis, sickelcell disease, physical stress, psychological stress, short after a heart attack, and short after an operation or accident.
HPU values can also be increased by a chemical load or by foods (a low carbohydrate diet). An extream high value can also indicate another porphyria disease different from HPU.

False Negatives:
The result can give a false negative value if you have taken regularly supplements of vitamin B or biotin, and/or a multivitamin with zinc, manganese or vitamin B for the last few months.
False negative results can also be obtained right after menstruation, or if you have used the toilet during the night previous to the test. Also if you have take diuretics before the test, or after a 3 week relaxed holidays.

Symptoms

a) Complaints caused by deficits of P-5-P, zinc and manganese.

-Reduced muscle building, muscles spams, epileptic like attack, convulsions, cramp attacks.
-Joint problems, hyper-mobility, instability of the pelvis
-Stomach and gut problems (carbohydrate intolerance and faulty protein digestion) allergy.
-Heart and vascular diseases caused by increased homocystein, vascular instability.
-Problems with menstruation, pregnancy and delivery
-Blood sugar problems: reactive hypoglycaemia and diabetes Type II

b) Complaints caused by decreased haem production
-Anaemia
-Fatigue, functional liver problems
-Psychological complains, such as depression, schizophrenia and psychosis.
-Muscle weakness

c) Complaints caused by deviation of the hormonal regulation

-Fatigue
-Headache and or migraine
-Allergy and gluten sensitivity
-Infections
-Low blood pressure
-Infertility
-Overweight
-Auto-immune diseases

d) Complaints caused by deviated liver function

If the haem synthesis id partly blocked, not metabolized porphyrinogens are stored in the tissue. Here they have a toxic effect on the nerve tissue from the central, peripheral or vegetative nervous system. Symptoms can pass from latent to present by the presence of sunburn, infection, stress, pregnancy, porphyrinogenic pharmaceuticals and chemicals. This can overload the faulty enzyme system, completely and definitely.


Further research: When the result is 0.6 or above, further testing is recommended
-HPU blood screening
-Blood test to check the sugar levels by a fructosamine determination.
-IgA Total gluten
-Hystamine
-TSH

Nevertheless, even without this further testing, a treatment can be started.

Treatment:

-Reduce significantly the intake of sugar in your diet.
-Equally divide the food intake during the day. Eat something every 2 hours.
-Reduce the amount of gluten in your diet. If you eat pasta, reduce the amount o grains in other meals, by not eating bread at breakfast or lunch. Eat vegetables and fish.
-Practice daily exercise without exhausting the body (walking, jogging, biking, swimming)
-Do not take high amounts of Vitamin C with high HPL levels. Reduce acid, low amounts of sour milk products, or preserved products with lactate or other acids.
-Do not use food supplements that contain copper in higher dose than 1mgr per day.
-Do not use vitamin B6 in high dose (pyridoxine HCI inactive), however you can use P-5-P.
-Use the following food supplementZinc maximum 30mgr per day (take with dinner)
Manganese maximum 25mgr per day (normally 5mgr per day at dinner)
P-5-P or B6 phosphate 50mgr per day (with breakfast)
The combination of these 3 can be obtained in one single capsule called Depyrrol basis. Take Depyrrol every second day during the first 3 weeks with breakfast. After this periods the amount can be increased if there are no stomach complaints or nausea.

Patients who have or had depression, panic attacks, psychoses or schizophrenia, should start very carefully. Prevent symptoms by starting with one pill every 2 days, or start with the separate ingredients before trying the combination. When you start to feel depressed, stop the treatment and consult with KEAC keac@tip.nl

http://www.hputest.nl/ewhat.htm


Pyroluria is a familial disorder which occurs with stress, where an above-average amount of a substance consisting of "kryptopyrroles" circulate in the body. The substance is harmless in itself, but high levels of these pyrrolles systemically bind with B6 and zinc, preventing the use of these essential nutrients in the brain and body.

Pyroluria (originally known as malvaria) is a genetic abnormality in hemoglobin synthesis resulting in a deficiency of zinc and vitamin B6. People with pyroluria produce excess amounts of a byproduct from hemoglobin synthesis, called OHHPL (hydroxyhemoppyrrolin-2-one). In these people an excess amount of pyrrole is found in the urine. Associated changes in fatty acid metabolism lead to low levels of arachidonic acid (an omega-6 fatty acid). The presence of pyroluria can have a profound effect on mental and physical health.

The list of complaints of HPU is very wide: chronic fatigue, menstruation problems, hypoglycemy, migraines, flatulence, irritable bowel, diarrhea, constipation, pregnancy complaints, anaemia, low blood pressure, returning infections, muscle weakness, overweight, hypermobility, muscle spams, cramp attacks, sleep impairments, allergies, food intolerances, cardiovascular problems, depression...

Of course, having some symptoms does not guaranty that you have HPU, but the more symptoms you recognize as yours, the higher the chance will be to get a positive urine test on HPU.

Some external factors can contribute to a deficiency of Vitamin B6, zinc and manganese, and the risk for health is high. The most important charging factors are stress, the contraceptive pill, medicines, a vegetarian feeding pattern and other sicknesses (for example the sickness of mononucelosis).

Many patients with HPU are tired. Fatigue at HPU can be the consequence of hypoglycemy, a reduced liver capacity, a low histamine quality and/or low activity of the adrenal gland. HPU patients become tired.

Conversations between the two laboratories, regarding the confict of results: a positive HPU (Keac) and a hight P-5-P (ELN)

Me:
My HPU test that came out slightly positive 0,9, indicating a deficiency of P5P, magnesium and zinc, in which you recommended taking Depyrrol. But at the same time I did took some other tests in ELN which showed high levels of P5P, and therefore posted the doubt if I should take any supplement at all regarding B6.

Keac:

Your HPU came positive, With values above 0.6 a treatment is helpfull. The best to use is Depyrrol basic, If not available you cane use at least P5P 50 mg and zinc 30 mg daily for long time.

In the ELN, they do not measure P5P, nor pyridoxin, but PLP, which is much easier to measure.I don't think that ELN has determined P5P. Most laboratory's determine PLP, which is incorrectly presented as P5P. It could also be the fact that you took supplements of B6 before what could be interferring with results.

In the case that it is realy P5P you should take folinic acid with vitamin B12 the lower the P5P levels in you blood. In this case you can take taurine 500 mg each morning in combination with zinc 30 mg with the warm meal.



DETERMINATION OF URINARY KRYPTPYRROLES

(COLORIMETRIC METHOD)

PRINCIPLE

“Kryptopyrrole” is the name given to a pyrrole-zinc-vitamin B6 complex that may be excreted in the urine of affected individuals [1]. Kryptopyrroluria, which may be associated with abdominal pain or a haemolytic crisis, can result in zinc and vitamin B6 deficiency and also schizophrenia. The condition has similarities to acute intermittent porphyria in that coproporphyrins are also excreted. In the scientific literature kryptopyrroles are also referred to as the “Mauve Factor” [2].

The assay procedure described depends on the extraction of the kryptopyrrole (KP) containing fraction from the urine with chloroform and its subsequent reaction with Ehrlich’s acid aldehyde reagent (p-dimethylaminobenzaldehyde in hydrochloric acid). This reaction is non-specific since most indoles, simple pyrroles or more complex pyrrole derivatives such as bilanes, which may be chloroform soluble, will also give a colour. Urobilinogen (UBG), which is a product of bilirubin metabolism, formed as a result of bacterial action in the gut and a normal component of many urine samples, also gives a magenta-red colour with Ehrlich’s reagent which can be extracted into chloroform. Porphobilinogen (PBG), which is an intermediate in the biosynthesis of haem but not a normal component of urine, gives a red colour with Ehrlich’s reagent, but will not extract into chloroform [3]. On the other hand, indican gives a red colour with Ehrlich’s reagent which can be extracted into alkali, but not into organic solvents.

In the situation we are considering,

a) Kryptopyrroles appear in the urine when the patient is suffering from combined zinc and vitamin B6 deficiency.

b) Failure to incorporate these pyrrole rings fully into Hb synthesis results in their appearance in the urine.

c) Such patients may suffer from eg autism or schizophrenia.

It is also possible that a toxin (e.g. a food additive absorbed through the GIT wall) could cause an idiosyncratic disturbance of haemoglobin metabolism and accumulation of pyrroles in the blood and urine, with consequent excessive loss of zinc and vitamin B6. Treatment involves identification and removal of the toxin from the diet, as well as replacement of zinc and vitamin B6. Hence kryptopyrroluria is a secondary coproporphyrinuria (i.e. a porphyrinuria which develops on the basis of a disease other than a primary disturbance of haemoglobin synthesis). The presence of porphyrins in the blood due to an inherited metabolic defect can also cause psychiatric disturbances. However, patients with kryptopyrroluria are not thought to suffer from other recognised porphyrin defects.
Comments (1)
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Hi there, 

This is really interesting, is there a reference from which this information came from?

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