hey there, that is great that you have figured out what you're dealing with! i dug up some abstracts on PCT and possibly relevant/related stuff:
Ascorbic acid deficiency in porphyria cutanea tarda1
Journal of Laboratory and Clinical Medicine, Volume 130, Issue 2, Pages 197-201
Porphyria cutanea tarda (PCT), the most common form of porphyria, is manifested as skin photosensitivity caused by excess hepatic production of uroporphyrin and heptacarboxylporphyrin. In experimental animal models, ascorbic acid modulates chemically induced uroporphyrin accumulation. The purpose of this study was to determine whether ascorbic acid is decreased in the plasma of patients with PCT. Plasma was obtained after an overnight fast from 21 PCT patients, 16 of whom were infected with hepatitis C virus (HCV), and from a separate group of 9 patients with HCV infection but not PCT. Thirteen PCT patients were studied when they had active disease and 8 after treatment-induced Plasma ascorbic acid was low (<23 μmmol/L) in 11 (85%) of the 13 untreated PCT patients and deficient (<11 μmol/L) in 8 (62%). Two patients with normal ascorbic acid levels (45 and 62 μmol/L) had consumed multivitamins. In 2 patients with deficient ascorbic acid, plasma levels returned to normal after phlebotomy treatment. Of the 8 patients studied during remission, 4 had normal ascorbic acid values and 4 were deficient (5 to 8 μmol/L). Plasma ascorbic acid values were normal for all patients who had HCV but no PCT. These data suggest that plasma ascorbic acid concentrations are commonly low in PCT, but this decrease is unrelated to HCV infection. Ascorbic acid deficiency may be one of the factors that contributes to the pathogenesis of PCT.
so i wonder about that deficiency/causal notion .. maybe it's just that the high iron in your system causes a drain on ascorbic acid in the body? i think that could be more likely seeing that phlebotomy helps normalize the serum ascorbic acid without any supplements.
Low vitamin E content in plasma of patients with alcoholic liver disease, hemochromatosis and wilson's disease
Journal of Hepatology, Volume 20, Issue 1, Pages 41-46
The RRR-alpha-tocopherol (vitamin E) content in plasma from 46 patients with liver diseases and 23 healthy controls was determined by high performance liquid chromatography and electrochemical detection. Patients were divided into three groups: alcoholic liver diseases (n=17; group A), hemochromatosis (n=17; group B) and Wilson's disease (n=12; group C). Lipid-standardized alpha-tocopherol levels were determined to neutralize differences due to hyperlipemia. The ratio of serum vitamin E to serum lipids (cholesterol, triglycerides, phospholipids) was highest in healthy controls and in patients in group A with cirrhosis and normal transaminases and bilirubin. Patients in group A with acute or chronic ethanol intoxication and high bilirubin levels had a 37% lower lipid-standardized vitamin E level than controls. Patients in group B with hemochromatosis, showing high serum iron (>180 μg/dl), a low free iron binding capacity (<8 μmol/l) and high ferritin-levels (<450 μg/l), had a 34% lower vitamin E/lipid ratio than healthy controls. No significant lowering of the vitamin E/lipid ratio was observed in the other patients in group B. A significant decrease (37%) in the vitamin E/lipid ratio was only detectable in patients with Wilson's disease (group C) showing high free serum copper (>10 μg/dl). The data support a role for free radicals in the pathogenesis of active liver diseases.
Supplemental therapy in isolated vitamin E deficiency improves the peripheral neuropathy and prevents the progression of ataxia
Journal of the Neurological Sciences, Volume 156, Issue 2, Pages 177-179
A 24-year-old male, who suffered since childhood from a progressive form of ataxia associated with peripheral neuropathy, was found severely deficient in serum vitamin E. He walked with bilateral aid and presented severe dysmetria of the limbs and dysarthric speech; muscular strength and trophism were slightly diminished in the distal muscles of four limbs and there was hypotonia of the arms; he presented absent deep tendon reflexes, bilateral Babinski's sign, reduced proprioception at four limbs, pes cavus and fasciculations of the tongue. Intestinal fat malabsorption and other gastrointestinal or haematological conditions associated with deficiency of this vitamin were ruled out. In this patient, after 2 years of a daily supplement of high doses of vitamin E, a further progression of the disease was not observed and, moreover, the neurophysiological characteristics of his neuropathy appeared clearly improved. A longitudinal evaluation of serum vitamin E levels showed values in the normal range after 13 months of therapy. The patient had molecular genetic analysis of chromosome 8 and was found homozygous for the unusual mutation 513insTT in the α-tocopherol transfer protein gene.
Quad Sclavo Diagn. 1982 Sep;18(3):249-57.
[Zinc and porphyria cutanea tarda]
[Article in Italian]
In 30 patients affected by Porphyria Cutanea Tarda we determined: plasmatic, erythrocytary and urinary zinc (by atomic absorption spectrophotometry); total porphyrins in plasma and in urines, coproporphyrins and protoporphyrins in erythrocytes (by spectrophotometric methods); haptoglobin, hemopexin, alpha 2-macroglobulin and albumin (by immunological methods). The results obtained were compared with those of normal subjects. In addition, the variables under consideration were statistically analyzed to bring out possible correlations (both simple and partial). The most interesting result (besides the documentation of an increase in the urinary and plasmatic zinc in porphyric patients) was the finding of correlations between plasmatic zinc, plasmatic porphyrins and alpha 2-macroglobulin.
Clin Sci (Lond). 1983 Oct;65(4):423-8.
Decreased liver delta-aminolaevulinate dehydratase activity in porphyria cutanea tarda and in alcoholism.
delta-Aminolaevulinate dehydratase activity was measured in liver biopsy specimens from 13 patients with porphyria cutanea tarda, 13 alcoholics and 10 control subjects. The mean enzyme activities in liver of porphyria cutanea tarda and alcoholics were 34% (P less than 0.001) and 49% (P less than 0.001) respectively of the control level. Heat treatment of liver supernatant caused slight inhibition of the enzyme activity in porphyria cutanea tarda and in controls. Addition of Zn2+ to liver supernatants slightly increased the enzyme activity in both porphyria cutanea tarda and controls. The addition of liver supernatant from the porphyria cutanea tarda to purified bovine delta-aminolaevulinate dehydratase did not result in suppression of the enzyme activity. The apparent Km value of delta-aminolaevulinate dehydratase was 4.3 X 10(-4) mol/l for porphyria cutanea tarda livers and 3.3 X 10(-4) mol/l for control livers. The difference between the two was not significant.
Arch Dermatol Res. 1979 Oct;266(2):121-6.
Study of erythrocyte delta-aminolevulinic acid dehydratase activity in porphyria cutanea tarda.
The erythrocyte delta-aminolevulinic acid dehydratase activity was studied in porphyria cutanea tarda patients, compared to healthy controls, in an attempt to resolve the contradictions in the relevant literature data. In an in vitro experimental system, a study was also made of how the erythrocyte delta-aminolevulinic acid dehydratase activity varies on the action of the activators -SH and Zn2+. It was found that, compared to the healthy controls, the erythrocyte delta-aminolevulinic acid dehydratase activity of porphyria cutanea tarda patients is significantly decreased, but it is restored to the original activity level on the addition of -SH and Zn2+. Since there is a general -SH requirement of delta-aminolevulinic acid dehydratase, the most obvious explanation for the decrease of the activity in the case of the patients is the shift of the natural redox systems of the erythrocytes, and the decrease of the reduced glutathione/oxidized glutathione ratio
Biochemical and Molecular Actions of Nutrients
A High Fat Diet Inhibits Delta-Aminolevulinate Dehydratase and Increases Lipid Peroxidation in Mice (Mus musculus)
Vanderlei Folmer, Júlio C. M. Soares, Diogo Gabriel and João B. T. Rocha2
The aim of this study was examine the effects of high starch (HS) vs. high fat (HF) feeding on blood glycated hemoglobin (GHbA1c) level, thiobarbituric acid-reactive species (TBA-RS) concentration and delta-aminolevulinate dehydratase (-ALA-D) activity in mice. The GHbA1c level was significantly higher in mice fed the HF diet compared with those fed the HS diet. Hepatic, renal, and cerebral TBA-RS concentrations in mice fed the HF diet were significantly greater than in mice fed the HS diet. In addition, positive correlations were found between the GHbA1c and TBA-RS levels for hepatic (P < 0.05; r = 0.46), renal (P < 0.003; r = 0.65), and cerebral (P < 0.001; r = 0.69) tissues. The d-ALA-D hepatic, renal and cerebral activities of mice fed the HF diet were significantly lower than those of mice fed the HS diet. Furthermore, a negative correlation was found between the GHbA1c level and -ALA-D activity in hepatic (P < 0.001; r = -0.77), renal (P < 0.007; r = -0.60), and cerebral (P < 0.007; r = -0.60) tissues. The results of this study indicate that consumption of a high fat diet promotes oxidative stress related to hyperglycemia, which in turn can stimulate glycation of proteins leading to d-ALA-D inhibition in mice.
if zinc is elevated, may need to keep an eye on your copper status..
Copper deficiency myelopathy produces a clinical picture like subacute combined degeneration
Background: Copper deficiency in ruminants is known to cause an ataxic myelopathy. Copper deficiency as a cause of progressive myelopathy in adults is underrecognized.
Objective: To describe the clinical, biochemical, electrophysiologic, and imaging characteristics in 13 patients with myelopathy associated with copper deficiency.
Methods: The records of patients with a copper deficiency–associated myelopathy were reviewed. Clinical characteristics, laboratory investigations, and responses to therapeutic intervention were summarized.
Results: Thirteen such patients were found, 11 of them in a 15-month period. All patients presented with prominent gait difficulty, reflecting a sensory ataxia due to dorsal column dysfunction and lower limb spasticity. All patients had polyneuropathy. A high or high-normal serum zinc level was seen in 7 of the 11 patients for whom this information was available. Somatosensory evoked potential studies done in eight patients showed impaired conduction in central proprioceptive pathways. Dorsal column signal change on spine MRI was present in three patients. An initial clue to the diagnosis was a very low ceruloplasmin level; further tests of copper metabolism excluded Wilson disease. The cause remained unexplained in most patients. Oral copper supplementation restored normal or near-normal copper levels in 7 of the 12 patients in whom adequate follow-up data were available; parenteral supplementation restored normal level in 3 further patients. Copper supplementation prevented further neurologic deterioration, but the degree of actual improvement was variable.
Conclusions: Unrecognized copper deficiency appears to be a common cause of idiopathic myelopathy in adults. The clinical picture bears striking similarities to the syndrome of subacute combined degeneration associated with vitamin B12 deficiency. Early recognition and copper supplementation may prevent neurologic deterioration.
i can't find out why the zinc would be elevated.. i'm curious if it's somewhat similar to the hypercalcemia seen in excess vit d3.. but anyway, just a bunch of stuff to read