Here is a quote from Wiki on this disorder:
Quote:
Adult hypophosphatasia can be associated with rickets, premature loss of deciduous teeth, or early loss of adult dentition followed by relatively good health. Osteomalacia manifests in painful feet resulting from recurrent poorly healing metatarsal stress fractures, and discomfort in the thighs or hips due to femoral pseudofractures which, when they appear in radiographic study, are distinguished from most other types of osteomalacia (which occur medially) by their location in the lateral cortices of the proximal femora. Some patients suffer from calcium pyrophosphate dihydrate crystal depositions with occasional overt attacks of arthritis (pseudogout), which appears to be the result of elevated endogenous inorganic pyrophosphate (PPi) levels. These patients may also suffer articular cartilage degeneration and pyrophosphate arthropathy. Radiographs may reveal pseudofractures in the lateral cortices of the proximal femora, stress fractures, and patients may experience osteopenia, chondrocalcinosis, features of pyrophosphate arthropathy, and calcific periarthritis.
Odontohypophosphatasia is present when dental disease is the only clinical abnormality and radiographic and/or histologic studies reveal no evidence of rickets or osteomalacia. Although hereditary leukocyte abnormalities and other disorders usually account for this condition, odontohypophosphatasia may explain some “early-onset periodontitis” cases....The metabolic basis of hypophosphatasia stems from a molecular defect in the gene encoding tissue non-specific alkaline phosphatase (TNSALP). TNSALP is an ectoenzyme tethered to the outer surface of osteoblast and chondrocyte cell membranes. TNSALP normally hydrolyzes several substances, including inorganic pyrophosphate (PPi) and pyridoxal 5’-phosphate (PLP) a major form of vitamin B6.
When TSNALP is low, inorganic pyrophosphate (PPi) accumulates extracellularly and potently inhibits formation of hydroxyapatite (mineralization) causing rickets in infants and children and osteomalacia (soft bones) in adults. PLP is the principal form of vitamin B6 and must be dephosphorylated by TNSALP for PL to cross over the cell membrane. , Vitamin B6 deficiency in the brain impairs synthesis of neurotransmitters which can cause seizures. In some cases, deposition of calcium pyrophosphate dehydrate (CPPD) crystals in the joint can cause pseudogout...Laboratory Testing The pathognomonic symptom is subnormal serum activity of alkaline phosphatase (ALP). In general, clinical severity mirrors the degree of enzyme deficiency.
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This is only for the adult form. This genetic disease has other presentations, all of which are dramatic and disabling, and sometimes fatal.
http://en.wikipedia.org/wiki/Hypophosphatasia
People with this genetic error, will show very low alkaline phosphatase levels, on testing, and many other obvious problems with bone formation. This is a very rare disorder. I hope for your sake this is not your problem.
Vitamin B6 metabolism is very complex. There can be errors in many points along the pathways it takes:
http://www.kegg.jp/kegg-bin/show_pathway?map00750
Pyridoxine is near the center of the map, and is part of a square
showing formation of pyridoxal...and to the left is the fate of pyridoxal. The rectangular boxes, have numbers in them, and those are enzymes that have been coded so far.
This wiki article explains the chemistry further:
https://en.wikipedia.org/wiki/Pyridoxal_phosphate
At one time I read that about 300 reactions depend on P5P...
so this just adds to the complexity.
The most notable pyridoxine error in humans is B6 dependency.
These individuals require really high doses of B6 beyond normal to survive. This is a genetic error and also very rare.
I only provide these links and topics to illustrate how complicated B6 is in the body. And the medical community does not really have a good grasp on all the potential problems involving errors of utilization with it.
One of the primary sources for B6 is meat. Bananas are quite high too and most breakfast cereals and potatoes. Try avoiding these dietary sources,
read all labels of other foods you consume, and get retested in 3 to 6 months and see what your P5P is then.
It is possible there is a source in your food that you have overlooked. This is the most common source. If you are still high after doing a dietary makeover....then genetic issues are what is left.
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All truths are easy to understand once they are discovered; the point is to discover them.-- Galileo Galilei
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Weezie looking at petunias 8.25.2017
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