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Biomedical subjects

L Malina

Publications and source records attributed to L Malina.

At least 19 recordsLinked to original sources
PubMed2006

[Use of external photoprotective agents in children living in the area of Prague].

BACKGROUND: Development of skin melanoma is related to the higher exposition to ultraviolet light, namely to the repeated sun burning of children. Because no valid information on the use of photoprotection in children can be found, an extensive search on the use of such measures, their galenic form and protective features was undertaken in order to evaluate the situation and to suggest necessary improvements. METHODS AND RESULTS: A group of 140 children aged 3 to 15 years, part of the group living in Prague, another part living outside the city was examined whether they use some local photoprotection during longer exposition to sun. If the answer was positive, it was followed in which season the photoprotection is used and whether it was used repeatedly. We also questioned on the forms of the local photoprotectives and the value of solar protective factor. CONCLUSIONS: Beside the description of sun tanning characteristics of children, we were informed by their parents that 59% of children use the photoprotection regularly, 41% only occasionally. The age of the first use of protection was 3.75 years. Photoprotection was used once a day in 28% of children, repeated use (during bathing, seashore stay, tourism) was reported in 72% of cases. The galenic form of photoprotective was most frequently a sun tanning creme (42%), a sun tanning milk (30%), both forms alternatively (8%), an oil (8%) or some other form (14%). The value of photoprotective factor was reported by 108 out of 131 parents and it was less than 10 in 9 children, 11 to 22 in 60 children, 21 to 40 in 36 children and over 40 in 3 children.

Adolescent↗
PubMed2005

[Is tamoxifene porphyrinogenic?].

BACKGROUND: As reported in previous studies, porphyria cutanea tarda (PCT) developed in several tamoxifene-treated patients with breast cancer. We studied the group of patients with cancer having only tamoxifene therapy after the initial surgery. We evaluated their clinical and laboratory results and compared them with the results of the group of patients suffering also from breast tumor, but treated after the surgery with other systemic therapies, mostly with chemotherapy. METHODS AND RESULTS: 20 patients were complexly studied, 10 of them with only tamoxifene therapy, and 10 without it. Diagnosis of the breast tumor was histologically confirmed in all of them. With the use of laboratory methods we examined their urinary excretion of diagnostically important porphyrins (uro- and coproporphyrin), then total blood count, liver function tests (ALT and AST), blood sugar, cholesterin, serum iron and ferritin, and performed also urinanalysis and detection of possible anti-HCV antibodies. The laboratory examination was repeated in the patient subgroup after three months, urinary uro- and coproporphyrin excretion also in the the control group, for to have an opportunity to follow the dynamics of laboratory changes. All the patients were examined during their regular laboratory controls performed so as not to be bothered with repeated additional phlebotomies. We did not confirm in our patients suffering from breast tumor the results of other autors, suggesting the connection between tamoxifene-therapy and development of porphyria cutanea tarda. CONCLUSIONS: Isolated cases of PCT can be induced through the effect of various hepatotoxic factors. However, the influence of common porphyrinogenically acting noxious substances (alcohol, HCV virus or iron overload as a result of the HFE gene mutations) were not found in our patients.

Aged↗
PubMed2005

[Non-melanocytic dischromias].

Non-melanocytic hyperpigmentations (dyschromias), besides common melanotic ones, develop mostly due to the skin reaction to various chemicals and/or drugs of exogenous origin. They can be however also caused by some endogenously formed (evolving) intermediates of several metabolic diseases. The above mentioned factors are of toxic or allergic character and their clinical dermal manifestation often occurs as an allergic reaction after the exposition of the skin to solar radiation, or as a toxic contact reaction both on the skin and the mucosa. As a therapy of individual skin changes is difficult or impossible, the only treatment consists in reliable photoprotective measures.

Humans↗
PubMed2003

Autoantibodies in sporadic porphyria cutanea tarda.

In an investigation of autoimmune antibodies using indirect immunofluorescence and Western blot analysis in a group of porphyria cutanea tarda patients we did not find any cytosolic antibodies potentially able to inhibit uroporphyrinogen decarboxylase. Furthermore, no known etiological factors were present in any of our patients. We therefore consider the development of the recently reported autoantibody with a molecular weight of 40 kDa a reaction to infection with the hepatitis C virus. The origin of mostly antinuclear antibodies against liver antigens (50, 45 and 56 kDa), detected in seven patients, was not identified and their etiopathogenetic implications remain unknown.

Antibodies, Antinuclear↗
PubMed2003

[Urocanic acid and its role in the photoimmunomodulation process].

Urocanic acid (UCA) is a metabolite of the amino acid histidine. It represents an important chromatophore in epidermis, which can absorb ultraviolet rays in UVB and UVA region and sequentially convert it from trans- to cis-isomer. Cis-isomer is not further degraded; it accumulates in the skin and is excreted with sweat and in shedding keratin scales. UCA has several important functions, the regulation of the homeostasis of the acidic cutaneous surface, the terminal differentiation of epidermal cells and namely the immunomodulatory role. As and immunomodulator UCA can suppress contact allergic reaction and the delayed hypersensitivity of the organism. It can affect reactions mediated by Th-lymphocytes, cytokine system, Langerhans cells, and by some neuropeptides. UCA is related to the development of non-pigmented skin tumors (basaliomas) and indirectly also to pigmented tumors. Cis-UCA can inhibit both the local and systemic resistance to infectious agents. In the immunomodulation some adductive compounds with another important cutaneous chromatophore DNA can participate.

Adjuvants, Immunologic↗
PubMed2002

[Effect of ultraviolet radiation on the immune system and the effect of exogenous photochemoprotecive agents on the ultraviolet radiation induced immunosuppression].

Ultraviolet radiation (UVR) of the UVB- and UVA-region suppresses immunity and in the dose relation manner it induces cutaneous or systemic state of immunotolerance. It was proved by the successful experimental transplantation of a tumor graft to the skin of UV-radiated animals and on the other side by unsuccessful attempts at skin contact-sensitization. UVR induces this condition either by direct inhibition of antigen-presentating function of the Langerhans cells, or indirectly by the stimulation of secretion of immunomodulatory cytokines in keratinocytes. UVR inhibits also functions of the natural killer cells in apoptotic and lytic cell-processes. It directly correlates with carcinogeneity in conditions of the dysbalance in developing expression of the tumor-suppressive gene p53, mutated by the irradiation and that of bcl-2 gene preventing apoptotic changes in somatic cells. Moreover, the immunosuppression is enhanced by the isomerization of urocanic acid and by the development of damaged DNA-photoproducts. Also various neuropeptides, namely propiomelanocortin, have their partial role in immunomodulation. Local photoprotective substances prevent UVR-penetration into the skin and thus block the immunosuppressive effects in relation to their chemical composition and to the SPF (solar photoprotective factor) value. Their effect depends on the width of the UVR-region blocked, which should include both the UVB and UVA band, and also on the concentration, chemical stability and on the method of their application.

Animals↗
PubMed2000

[Significance and prevalence of the C282Y gene mutation of primary hemochromatosis in the pathogenesis of pophyria cutanea tarda].

BACKGROUND: Hitherto studies on the ethiopathogenesis of porphyria cutanea tarda (PCT) show that the major pathogenic factor is iron ion, which acts via inhibition of the uroporphyrinogen decarboxylase. New speculations have appeared on the possible relation of this role of iron and the occurrence of mutation of the recently discovered gene of the hereditary hemochromatosis HFE, which may cause the iron overloading of the organism. Our paper describes prevalence of the C282Y gene mutation (HFE) together with the clinical and laboratory record in PCT patients. METHODS AND RESULTS: PCT was diagnosed mostly on the basis of clinical finding of actinic-traumatic vesicular dermatitis and the typical laboratory record of elevated higher-carboxylic porphyrines in urine and stool. Other laboratory methods tested the liver functions, plasma iron level and its binding capacity, ferritine level. All patient underwent routine haematological testing. Presence of antibodies against hepatitis C was also assayed (Elisa test 2nd generation, Sanofi Pasteur). In patients with prominent laboratory alterations showing possibility of the hepatic structural lesion, histology from the liver punctate was done. Frequency data of the C282Y gene mutation (HFE) in PCT patients was estimated on the basis of the genetic testing using PCR reaction of our own system. Group of PCT patients had 69 persons (63 patients with the sporadic form and 6 patients with familiar form of the disease). Hereditary haemochromatosis C282Y gene mutation (HH) was found in 15 patients, three of them were homozygotes and twelve heterozygotes (three heterozygotes had the familiar form of the disease). Nobody in this group was positive in the HIV antibody testing. In all porphyria patients with the presence of mutated gene who underwent liver biopsy, siderosis of different degrees was identified. In three patients neither the phenotypic observation nor the laboratory testing have shown haemochromatosis. Prevalence of C282Y gene mutation HFE in patients with porphyria cutanea tarda was studied. Such mutation was found in 15 persons (12 heterozygotes and 3 homozygotes) from the group of 69 tested patients (21.7%). Such frequency is significantly higher than in the control--nonporphyric--persons (10%). Patients were without clinical symptoms. Laboratory haematological changes, typical for HH, manifested in some of them only (elevated level of ferritine was found in 10 from 15 porphyria patients, elevated sideremia in one of them). Red blood cell counts were in both homo- and heterozygotes normal. Concurrence of the two porphyrinogenic factors--presence of gene mutation HFE and hepatitis C infection--was not proved. Antibodies against hepatitis C virus were not identified in any of the patients. Siderosis was found to be only a symptomatic sign, which was pronounced in different degree in all 9 porphyria patients with C282Y gene mutation who underwent liver biopsy. CONCLUSIONS: Frequency of C282Y gene mutation in our patients with porphyria cutanea tarda appears similar to that in other Middle European countries. It differs significantly from the frequency found in South European and North European countries (British).

Hemochromatosis↗
PubMed2000

Overt and hidden coinfection with hepatitis B and C viruses in chronic liver disease and porphyria cutanea tarda.

The aim of this study was to assess the rate of hepatitis B virus (HBV) and hepatitis C virus (HCV) coinfection ("the coinfection") in chronic liver disease (CLD) and to reveal overt and hidden HBV infection in patients with antibodies to HCV (anti-HCV). A total of 209 untreated patients (64 with chronic hepatitis B, 79 with chronic hepatitis C and 66 with porphyria cutanea tarda (PCT)) were screened for serological markers of HBV and HCV infection in serum by third generation enzyme-linked immunosorbent assay (ELISA) methods and for HBV DNA and HCV RNA in serum by polymerase chain reaction (PCR). The rate of the overt coinfection in chronic hepatitis B was very low (2/64, 3%). However, in chronic hepatitis C, the rate of the hidden coinfection with HBV was relatively high (19/79, 24%); these patients had higher alanine transaminase (ALT) and asparagine transaminase (AST) levels in serum and a more advanced liver disease. In PCT patients, the rates of HBV and HCV infections were the same, 21% (14/66). In the PCT patients infected with HBV or HCV, the rate of the coinfection was 33% (7/21). The PCT patients with the coinfection had a high serum ALT level and the worst histological picture in the liver. The hidden HBV infection was more frequent than the overt one. The possibility of the overt or hidden coinfection in CLD renders a detailed analysis of all serum samples for both viruses mandatory. Vaccination against HBV infection should be offered to anti-HCV-positive individuals as well as to PCT patients not showing antibodies to HBV (anti-HBV).

Adolescent↗
PubMed1999

[Porphyria cutanea tarda after antineoplastic drugs].

Chronic hepatic porphyria is in some cases a paraneoplasia, in others part of the neoplastic disease. Sometimes it is, on the other hand manifested during antineoplastic treatment. Its development may be associated with this therapy. The presented case-history describes a female patient with breast cancer treated with antineoplastic agents, who developed a sporadic form of late cutaneous porphyria with a classical clinical and laboratory picture. The authors contemplate on the possible porphyrinogenic effect of antineoplastic agents, in particular cyclophosphamide and tamoxifen, which in the latter preparation was not described so far.

Adult↗
PubMed1998

[Chronic hepatic porphyria and hepatitis B and C virus infections].

BACKGROUND: It is known that in patients with porphyria cutanea tarda (PCT) there is an increased prevalence of the hepatitis B virus (HBV) and the hepatitis C virus (HCV). The incidence of anti-HCV in PCT in our country is 21.7% in estimations by the second generation method, however, the incidence of HBV in PCT was not assessed so far. METHODS AND RESULTS: In 60 patients with PCT antigens and antibodies against HBV and HCV were assessed (by the anti-HCV third generation ELISA method) and in subjects with signs of HBV or HCV. HBV DNA and HCV RNA were assessed by the method of the polymerase chain reaction. PCT without detectable HBV or HCV infection was found in 45 subjects (68%). HBV infection only was confirmed in seven subjects (10.6%), however none of the patients had positive HBsAg in serum. All had only antibodies against HBV. HCV infection only was detected in seven patients (10.6%) and HBV and HCV co-infection also in seven patients (10.6%). In the group of patients with HBV and HCV co-infection there was not a single HBsAg positive subject. The mean ALT serum activity was significantly higher as compared with subjects with HBV or HCV infection only (p < 0.05) and the histological finding on liver biopsy was more serious. CONCLUSION: HBV (21%) and HCV (21%) infection participates significantly in the clinical picture of PCT. A special subgroup is formed by patients with PCT and HBV and HCV co-infection who have as a rule a higher ALT activity and more severe histological changes in the liver. The incidence of HBV and HCV infection in PCT in the Czech Republic is double as compared with Germany or Great Britain.

Adult↗
PubMed1998

[Needle-like inclusions in liver cells are specific diagnostic signs of porphyria cutanea tarda].

Needle-shaped cytoplasmic liver cell inclusions are considered to represent a feature typical of porphyria cutanea tarda (PCT). Histological sections of 849 liver biopsies were stained with ferricyanide reduction reaction for demonstration of the inclusions. They were detected in 18 cases, and in all of them the diagnosis PCT was clinically and/or biochemically confirmed. In our group of patients, PCT was associated with alcoholic liver disease in three cases, with chronic hepatitis C in three cases and with hepatocellular carcinoma in two cases. In the liver with hepatocellular carcinoma, the inclusions were present only in non-neoplastic liver tissue, not in the tumourous tissue. No inclusions were found in the liver tissue of patients without clinical signs of PCT.

Adolescent↗
PubMed1996

[Detection of HCV RNA in anti-HCV positive patients with chronic hepatic porphyria].

BACKGROUND: In 1994 the authors published first results of anti-HCV antibodies in the group of 92 porphyria cutanea tarda patients, tested with ELISA second generation enzymatic method. Positivity, found in 21.7 per cent of them, was significantly higher when compared with the results of healthy blood-donors. Anti-HCV reactivity thus ascertained can be, however, non-specific in some cases and need not necessarily indicate existence of an active replication of hepatitis C-virus. The authors have therefore subsequently tried to verify the above results by the more sensitive polymerase chain reaction (PCR), enabling the proof of viral RNA and thus an assessment of the replication-activity of HCV. This study completes their previous findings for the evidence of viraemia in anti-HCV reactive PCT-patients. METHODS AND RESULTS: 21 of 92 PCT-patients were reactive when examined with the second generation ELISA method (Sanofi, Pasteur). HCV RNA was examined in 20 subjects from the above group (one patient died in the meantime) by means of PCR. Positive HCV RNA was found in 17 patients, i.e. in 85 percent of them. Percutaneous liver biopsy without visual control was performed in 17 anti-HCV reactive and HCV RNA positive patients and in 46 anti-HCV negative porphyrics. The biopsy-findings were significantly worse in the HCV RNA-positive group. Also the average activity of ALT and AST was significantly higher in the patients with positive HCV RNA when compared with anti HCV-negative subjects. CONCLUSIONS: High frequency of HCV-infection (21.7 percent) was found in our group of 92 PCT-patients. Viraemia was present in the vast majority (85 per cent) of them, for they had also positive HCV RNA besides anti-HCV reactivity. These patients had also higher ALT- and AST-activity and more severe histological liver-changes. HCV-infection is the main virological cause of their liver lesion, beside ethylism. Hepatocellular carcinoma is rather exceptional in our porphyria patients in spite of their frequent HCV-infection.

Adult↗
PubMed1996

Screening of the gasoline lead load in school children in the Czech Republic.

The determination of erythrocyte zinc protoporphyrin (ZZP) was implemented as an indirect method for assessing lead levels in the blood in the follow-up of Czech children regarding the lead load due to automobile traffic. Simultaneously, basic indicators of the red blood picture were studied. A screening study was conducted with 2668 school-age children residing permanently in three different regions of our country. The differences in basic hematological indicators, i.e. number of erythrocytes, hemoglobin content, hematocrit and red blood cell volume, like the differences in zinc protoporphyrin in the peripheral blood, did not confirm the initial hypothesis on the relatively greater load of the Czech child population due to lead from exhaust fumes of combustion motors. Also, no regional differences were observed. The differences found in the abovementioned indicators reflect the influence of an overall environmental pollution rather than that of a toxic effect of lead.

Child↗
PubMed1996

[Erythrocyte zinc-protoporphyrin and characteristics of iron- deficiency anemia in parturient women and neonates].

The authors examined the concentration of erythrocyte zinc-protoporphyrin (ZPP) by a haematofluorometric method in 175 women during delivery and the levels in their children. They assessed basic haematological indicators, the number of red cells, their volume, number of reticulocytes, haemoglobin concentration, haematocrit and sideraemia. All were average values within the physiological range in both examined groups. Statistical evaluation revealed a significant difference between mean values of sideraemia in mothers and children and mean values of the erythrocyte ZPP in both groups. The authors found also a medium significant correlation between ZPP and the erythrocyte volume and the erythrocyte volume and sideraemia. The correlation of different indicators was more marked after classification of the examined women and neonates into subgroups by haemoglobin concentrations and ZPP. In the subgroup of mothers with Hb values lower than 115 g/l a positive correlation was found with the number of reticulocytes red cell volume and sideraemia. In the subgroup of anaemic women (Hb concentration lower than 105 g/l) a negative correlation with the foetal sideraemia was found.

Adult↗
PubMed1994

[The frequency of viral hepatitis C in patients with porphyria cutanea tarda].

BACKGROUND: High frequency of hepatitis C virus antibodies was recently ascertained in chronic hepatic porphyria in several European countries. Some authors correlate these findings with the development of PCT, considering hepatitis C-infection further factor capable of triggering clinical and laboratory manifestation of this enzymopathy. The task of the present work was to ascertain the frequency of HCV antibodies in PCT in Czech Republic by examining an extensive group of patients, followed at the porphyria-advise center. METHODS AND RESULTS: The frequency of HCV antibodies was investigated in 92 PCT-patients with the second-generation enzymatic ELISA method. At the same time, urinary porphyrin excretion and liver function tests were examined in these subjects. Simultaneously also the results of liver biopsy performed at the first presentation of the patients were taken into account. Statistically significant increase of anti-HCV antibodies (21.7%) was found in PCT-group in comparison with the results of the group of healthy blood donors (0.16%). CONCLUSIONS: HCV-infection might possibly be a factor responsible for the development of PCT in case of proven time-dependent link between the existence of its active viraemic phase and initial symptoms of porphyria. Such an evidence has not been provided yet and remains a task for future studies.

Adult↗