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Notes: The clinical value of a very rapid spectrofluorometric method for the determination of plasma uroporphyrin levels was studied during follow-up of 122 patients with porphyria cutanea tarda (PCT). Four-hundred and eight measurements were carried out within a 3-year period. In active PCT plasma uroporphyrin varied between 15 and 448 nmol/1 (normal 0–1.4). A high correlation was seen between elevated levels of uroporphyrin in the plasma and urine in new cases (r=0.72, n= 23) and relapses (r=0.92, n= 37). A parallel course between these variables was noted during the treatment of 31 patients. The correlation was less pronounced (r= 0.42) in remission, i.e. in those cases with urinary uroporphyrin levels lower than 240 nmol/24 h. However, only in nine of 249 measurements taken in remission did plasma uroporphyrin exceed 15 nmol/1. In six of these cases a biochemical relapse had occurred at the next follow-up measurement as judged by an increase of uroporphyrin in the urine. It is suggested that treatment of new cases and relapses should continue until plasma uroporphyrin drops under 10 nmol/1. Values between 15 and 23 nmol/1 in individuals already treated raise the suspicion of relapse and should be rechecked in the near future. Retreatment is necessary when the levels exceed 23 nmol/ 1. The use of the method is recommended as a simple and effective way for monitoring the progress of patients with PCT.

Notes: A high incidence of peptic ulcer was found in a retrospective study of 199 Bulgarians with porphyria cutanea tarda. Ulcers were found in 35 patients (17.59%), while its incidence in Bulgaria varies between 2.52 and 3.7%. The site of the ulcer was duodenal in 29 porphyriacs, gastric in three, both duodenal and gastric in two and pyloric in one. The pattern of localization was similar to that seen in the general population. Peptic ulcers became symptomatic before the appearance of porphyria in 30 cases. Six (17.1%) of the patients had perforations, while the frequency of this complication in the general population was 1.7-8.5%. Two porphyriacs with perforations died of peritonitis.Ulcers were found in 21 (24.4%) of 86 patients with normal activity of erythrocyte uroporphyrinogen decarboxylase, i.e. they had sporadic (acquired) porphyria cutanea tarda. Two (10%) of 20 patients suffering from the familial form of the disease (with low erythrocyte uroporphyrinogen decarboxylase activity) had ulcers. The examination of 105 unselected porphyriacs showed a significantly higher incidence of blood group B in comparison with the general population (23.8% vs. 16.6%). The association between the porphyriacs with ulcers and blood group B (8 of 21 examined persons) and the rare occurrence of group O (only 4 of 21) was unexpected. An association between porphyria and some of the haptoglobin types could not be established in 98 unselected patients (including those with and without ulcer).More studies are needed to substantiate the validity of blood groups and uroporphyrinogen decarboxylase as genetic markers for porphyria cutanea tarda combined with peptic ulcer.Porphyria cutanea tarda (PCT) is the commonest human porphyria in most countries of the world. It is characterized by photosensitivity, mild to moderate hepatic siderosis and marked elevation of the highly carboxylated porphyrins in plasma and urine. A significantly higher incidence of peptic ulcer (PU) was reported in PCT patients in Spain in comparison with the general population.1 The reason for this is unknown. The liver damage and the alcohol consumption in most PCT patients could only partially explain such a high frequency.In both diseases, the aetiological role of hereditary and genetic factors have been considered. It is known that in patients with duodenal ulcer, blood group O is particularly common.2 Two forms of PCT exist. In the familial type a decreased activity of the enzyme, uroporphyrinogen decarboxylase (Uro D) is found in erythrocytes. In sporadic (acquired) PCT the enzyme activity in erythrocytes is normal. However, some hereditary predisposition in the sporadic form cannot be excluded.3 This makes an investigation of some genetic markers in PCT a reasonable goal. The HLA system has been already studied in this disease, but the results do not provide a clear-cut conclusion.4–6 We were not able to find any data on two other routinely determined genetic markers—the blood groups and haptoglobin types. As an increased association between PCT and PU could be expected anyway, and not only in Spain, and as it seems appropriate to determine the blood groups and haptoglobin types in PCT, we undertook the present study. Its aim was to examine (i) the incidence of PU among patients with familial and sporadic PCT in Bulgaria and (ii) the distribution of blood group and haptoglobin types in PCT patients as a whole and in the porphyriacs with and without PU.

Notes: Spontaneous genome mutations in ten patients with porphyria cutanea tarda (PCT) amounted to 1 02% as compared to 0.36% in the control group of healthy individuals (P〈0.01). Spontaneous structural chromosome aberrations in patients were seen in 2.39% of the examined cells versus 0.70% in the cells of controls (P〈0.01). The increased percentage of cells with spontaneous genome and chromosome mutations in patients with PCT cannot be associated with alcohol only.

Notes: Serum γ-glutamyl transferase was elevated fourfold in all thirty-four new cases of porphyria cutanea tarda. Phlebotomy treatment decreased it. The twelve patients in relapse showed a considerable increase, while those in remission tended to a gradual elevation in the enzyme over the course of years. γ-Glutamyl transferase is a useful auxiliary parameter in porphyria cutanea tarda particularly in untreated cases.

Notes: Summary Fasting for 2 and 4 days progressively increases the activity of hepatic γ-glutamyl transferase (γ-GT) in rat. A high-protein diet (with 42.6 energy percent of protein) for 45 and 90 days inhibits it. It seems that liver γ-GT is susceptible to nutritional influences.