Influence of hepatitis C virus (HCV) infection on porphyrin and iron metabolism in porphyria cutanea tarda (PCT) patients.

BACKGROUND: Porphyria Cutanea Tarda (PCT) is the most common form of porphyria. It is characterised by lowered activity of uroporphyrinogen decarboxylase. It seems possible that the hepatitis C virus (HCV) infection triggers the symptoms of PCT. MATERIAL AND METHODS: A group of 29 PCT patients (33-73 years old, mean 54 years) was evaluated. In these patients the serum HCV-RNA (by mean of automatic Cobas AmplicorTM Hepatitis C ver. 2.0 assay, Roche Diagnostics), activity of transaminases (ALT, AST), serum protein fractions, iron, total iron binding capacity (TIBC), ferritin, transferrin, transferrin saturation, and urinary porphyrins excretion were assessed. RESULTS: The presence of HCV-RNA was detected in 12 cases (41.4%). Statistically important differences between currently HCV infected patients and other PCT patients concerned the following parameters: mean body mass (68.5 vs. 78.0 kg, p = 0.02), mean body mass index (22.4 vs. 26.8 kg/m2, p = 0.007), mean serum activities of ALT (112.6 vs. 65.4 U/l, p = 0.02) and AST (85.0 vs. 56.5 U/l, p = 0.038), total serum protein concentration (78.0 vs. 71.6 g/l, p = 0.016) and serum alpha-2 protein concentration (7.2 vs. 5.5 g/l, p = 0.009), respectively. Some other not significant differences were also found: mean serum iron level 182 vs. 169 micrograms/dl, transferrin concentration 2.77 vs. 2.56 g/l, transferrin saturation 43 vs. 45%, serum TIBC 445 vs. 397 mg/dl and ferritin concentration 394 vs. 400 micrograms/l, respectively. The mean urinary uroporphyrin concentrations were 643 micrograms/l vs. 366 micrograms/l (p = 0.16) and those of coproporphyrins were 190 micrograms/l vs. 136 micrograms/l (p = 0.37), respectively. CONCLUSION: In the group of PCT patients investigated no significant relationship between current HCV infection and iron or porphyrin metabolism was noted. Nevertheless, HCV infection significantly influences the symptoms of liver damage and seems to influence other metabolic parameters in PCT patients.

Effect of high fiber vegetable-fruit diet on the activity of liver damage and serum iron level in porphyria cutanea tarda (PCT).

BACKGROUND: During the treatment of coronary heart disease with a vegetable-fruit diet, we have observed the positive effect of the treatment on PCT patients. Therefore, we have now examined the short-term results of the diet on the selected PCT activity parameters. The study was approved by our Review Board. MATERIAL AND METHODS: A group of 13 male PCT patients (mean age 52 years) was evaluated. We assessed the body mass index (BMI), serum iron level, activity of transaminases (ALT, AST), severity of skin symptoms, and urinary prophyrins excretions, before and after a three-week period of vegetable-fruit diet. The diet was of natural vegetable/fruit products, and its daily caloric content was ca. 500 kcal/day. RESULTS: The mean BMI before and after the diet period were 26.8 +/- 4.7 vs. 25.8 +/- 4.3 (p = 0.001), the serum activities of ALT 122.0 +/- 60.7 U/l vs. 75.6 +/- 31.8 U/l, and of AST 91.8 +/- 56.0 U/l vs. 55.2 +/- 14.2 U/l (p = 0.001), respectively. The mean serum iron levels were 188.6 +/- 75.7 mg/dl vs. 140.2 +/- 56.4 mg/dl, serum ferritin concentrations 574 +/- 351 vs. 499 +/- 340 ng/ml (p = 0.04), respectively. Severity of skin lesions and urinary coproporphyrins excretion were significantly diminished during the diet; urinary uroporphyrins excretion was also lowered, but not to a statistically significant level. CONCLUSION: In our group of PCT patients, we noticed the beneficial effect of the vegetable-fruit diet on selected disease parameters. The diet may be useful in the treatment of PCT and diseases associated with PCT.

[Porphyria cutanea tarda--new views on its pathogenesis and therapy]. / Porfiria skórna pózna--nowe spojrzenie na patogeneze i terapie.

Literature data and the results of our studies on the prevalence of the HCV infection markers in patients with porphyria cutanea tarda (PCT) are presented. We also present new views on the PCT pathogenesis in some cases, therapy of the cutaneous changes, and the use of interferon in the patients as a prevention of the hepatocellular carcinoma.

[Wilson's disease in personal material--disturbances in hemostasis]. / Choroba Wilsona w materiale wlasnym--zaburzenia hemostazy.

Genetically determined impairment of copper excretion from the liver into the bile in Wilson's disease (WD) cause that "free copper" is accumulated in toxic amounts not only in the liver, but also in other organs. In WD liver biopsy often could not be made because of serious disturbances in hemostasis. The aim of the study was: a) to demonstrate our 9 patients with various form of WD. b) to examine some blood clotting factors and compare the results with these obtained in other liver diseases. The diagnosis of Wilson's disease was made on the basis of disturbed copper metabolism. Among our 9 patients (8 women and 1 man, between 17-33 years old) we diagnosed: 3 patients with fulminant Wilson's disease with all day deep jaundice, hemolytic anemia, haemorrhagic diathesis and liver failure, died, 2 patients with active chronic hepatitis, hemolytic anemia and haemorrhagic diathesis, 2 patients with liver cirrhosis, haemorrhagic diathesis, Kayser-Fleisher ring, neuropsychiatric syndrome, 2 asymptomatic patients without haemorrhagic diathesis. The prothrombin index and the factors of prothrombin stem (II, V, VII, X) were lower than in other kinds of cirrhosis. After treatment with d-penicillamine the clothing factors returned near to the norm, similar as the biochemical and immunological results.

[Diagnostic difficulties in hepatic amyloidosis--clinical analysis and autopsy data of 3 cases]. / Trudnosci diagnostyczne amyloidozy watroby--analiza kliniczna i autopsyjna trzech przypadków.

Three patients with amyloidosis in whom hepatomegaly was the main sign ara presented. In all cases jaundice coexisted which is regarded as a rare sign in amyloidosis. Attention is called to the diagnostic difficulties of amyloidosis especially without a preceding clinically overt disease process. The presence of a particularly high activity of alkaline phosphatase in the serum, and focal absence of 99Tc uptake by the hepatic macrophage system in liver scintigram suggested liver tumour. However, demonstration of monoclonal protein in blood, urine or ascitic fluid, or raised level of alpha 2 globulin, should call attention to the possibility of liver amyloidosis. The authors stress the usefulness of the method of preincubation of tissue biopsy specimens in potassium hypermanganate during routine staining with Congo red for differentiation of amyloid AA from amyloid AL.