Biomedical subjects
Publications and source records attributed to L Malina.
We report the case of a woman with hereditary porphyria cutanea tarda which manifested 3 weeks after she gave birth to her second child. The mother of the patient had also been diagnosed and treated for porphyria cutanea tarda. Reduced red cell uroporphyrinogen decarboxylase activity was found in the patient, the new-born child and the patient's mother. Normal enzyme activity was found in the patient's first child.
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PCT, though largely genetically determined, can nevertheless be favorably influenced by several therapeutic methods and prolonged clinical and laboratory remission can be achieved. Two effective and reliable methods exist - repeated phlebotomy therapy and prolonged low-dose chloroquine. Although the exact mechanism underlying serial phlebotomy is not yet known, this therapy seems to be the most successful at present; it does not provoke serious adverse side effects, and extended remission after only one course can be expected. Chloroquine, about as effective in PCT, can cause acute toxic reactions at the beginning of its administration. However, when this occurs treatment need not be discontinued as no permanent ill-effects result. Both kinds of therapy can be used in the same patient consecutively. Although the data are sparse, a favorable effect of hydroxychloroquine has been noted. It can rapidly normalize urinary excretion of porphyrins in relapsing cases. The results of other methods hitherto recommended (i.e., chelators, AMP, desferrioxamine, metabolic alkalinization, plasmapheresis, etc.) have not been sufficiently evaluated either with regard to effectiveness or side effects. The use of some of these has already been abandoned.
In a patient with psoriatic erythroderma treated with Methotrexate and later with Buthiopurine, porphyrinuria and later porphyria cutanea tarda developed, following a toxic reaction with bone marrow suppression. Hepatological examination before therapy did not reveal any signs of active liver disease.
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Longer remissions after the phlebotomy therapy than after the low-dose chloroquine treatment were ascertained by means of the long-term follow-up of a large group of porphyria cutanea tarda patients. An attempt to prove the dependence of the length of laboratory and clinical remission on the values of initial porphyrinuria, on the degree of morphological liver changes, and on the total amount of blood withdrawn at phlebotomy was unsuccessful. On the contrary, a direct relationship was observed between the length of remission and the age of the subject on commencing treatment. At the same time, it was impossible to prove a causal relationship between the length of remission and initial porphyrinuria, the degree of morphological liver changes, and the total dose of drug taken, in the group of patients on the low-dose chloroquine therapy.
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In thirty-four patients with porphyria cutanea tarda treated with small doses of chloroquine, liver biopsies were performed before and after treatment. In seventeen cases (50%) the morphological patterns before treatment corresponded to unstabilized fibrosis, while in eleven (32.4%) there were non-specific changes in the form of focal fatty change, haemosiderosis, and mild fibrosis of the portal tracts. Active chronic hepatitis was found in three patients (8.8%), and cirrhosis also in three cases. Although in all patients a clinical and metabolic remission of porphyria cutanea tarda occurred during treatment, the morphological patterns in the liver parenchyma remained on the whole unchanged. Only in five cases was there an increase in the inflammatory changes, while in two patients these changes tended to disappear.
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The analysis of skin porphyrins in biopsies in patients with porphyria cutanea tarda has been achieved by high pressure liquid chromatography. Porphyrins were determined as their methyl ester copper-chelates and 2-,4-,5-,6-,7- and 8-carboxylic porphyrins distinguished and quantified.
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1. Urinary excretion of indolyl-3-acryloylglycine (chromogen of the so-called Kimmig's light band) in 15 normal subjects was highly significantly increased in June-September ("summer") against the November-April ("winter") collection in the same subjects. Possible explanation of this phenomenon is discussed. 2. In the "winter" period, the mean of 23 patients with chronic polymorphous light eruption was significantly higher than the mean of the 29 controls. In the "summer" period, though an increaes of the average against "winter" was also noted, this difference against the control group (29) disappeared. 3. In 24 patients with skin tuberculosis the mean excretion in "winter" was significantly higher than in controls. This increase cannot be simply attributed to heliotherapy. 4. In "winter", there was no significant difference between the normal subjects and 12 patients with lupus erythematosus and 10 patients with porphyria cutanea tarda. In both these groups there was marked "summer" increase in excretion, though in the case of porphyria cutanea tarda, the "summer" mean was significantly lower than that of the controls. 5. All results were expressed on creatinine basis. In part of the subjects it was possible to calculate the excretion per unit time. Identical conclusions could be drawn.
Two brothers with cutaneous hepatic porphyria are reported. One, aged 64 years, had his disease apparently precipitated by the oestrogen treatment given for carcinoma of the prostate.
A screening test for urinary porphyrin excretion was performed amound 1433 diabetes patients (552 males and 881 females). In three patients (0.21)% the diagnosis of porphyria cutanea tarda was ascertained. In spite of the increasing usage of sulfonylurea antidiabetic drugs, previously reported as potential porphyria-inducing agents, the authors could not observe an increased number of porphyria cases among the diabetics compared to the proportions in similar groups examined 10-12 years ago. The sulfonylurea Oradian was the likely etiopathogenic factor in only one out of three patients. The above mentioned observations do not speak in favour of independent etiological porphyrinogenic influence of antidiabetic sulfonylurea drugs.