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Landfills, especially those poorly managed, can negatively affect the environment and human beings through chemical contamination of soils and waters. This study investigates the soils of a historical municipal solid waste (MSW) landfill situated in the heart of a residential zone in the capital of Slovakia, Bratislava, with an emphasis on metal (loid) contamination and its consequences. Regardless of the depth, many of the soils exhibited high metal (loid) concentrations, mainly Cd, Cu, Pb, Sb, Sn and Zn (up to 24, 2620, 2420, 134, 811 and 6220 mg/kg, respectively), classifying them as extremely contaminated based on the geo-accumulation index (I geo >5). The stable lead isotopic ratios of the landfill topsoil varied widely (1.1679-1.2074 for 206 Pb/ 207 Pb and 2.0573-2.1111 for 208 Pb/ 206 Pb) and indicated that Pb contained a natural component and an anthropogenic component, likely municipal solid waste incineration (MSWI) ash and construction waste. Oral bioaccessibility of metal (loid)s in the topsoil was variable with Cd (73.2-106%) and Fe (0.98-2.10%) being the most and least bioaccessible, respectively. The variation of metal (loid) bioaccessibility among the soils could be explained by differences in their geochemical fractionation as shown by positive correlations of bioaccessibility values with the first two fractions of BCR (Community Bureau of Reference) sequential extraction for As, Cd, Mn, Ni, Pb, Sn and Zn. The results of geochemical fractionation coupled with the mineralogical characterisation of topsoil showed that the reservoir of bioaccessible metal (loid)s was calcite and Fe (hydr)oxides. Based on aqua regia metal (loid) concentrations, a non-carcinogenic risk was demonstrated for children (HI = 1.59) but no risk taking into account their bioaccessible concentrations (HI = 0.65). This study emphasises the need for detailed research of the geochemistry of wastes deposited in urban soils to assess the potentially hazardous sources and determine the actual bioaccessibility and human health risks of the accumulated metal (loid)s., Competing Interests: Declaration of competing interest The authors declare that they have no known competing financial interests or personal relationships that could have appeared to influence the work reported in this paper., (Copyright © 2024 The Authors. Published by Elsevier Ltd.. All rights reserved.)

Introduction: Forestry is one of the most dangerous industries worldwide. Workers handle heavy loads and are exposed to numerous adverse factors of the work environment. This study was focused on analyzing severe and fatal occupational accidents over a long period., Methods: Data were gathered from occupational safety and health databases of the State Forests of the Slovak Republic, which manages 879,113 ha of forests in Slovakia. Between 2007 and 2021, 210 severe and64 fatal accidents were recorded. The data analyses included contingency tables, Pearson χ2 test, and Cramer's V coefficient to analyze the relationships between studied variables., Results: The mean annual number of fatalities was 14, occurring to workers aged 42 on average. Almost 80% of all fatal accidents were suffered by contractors of harvesting operations, who were 5.23 times more likely to suffer a fatality than company employees. On average, 1.28 severe and0.75 fatal accidents occurred per 1 million m 3 of harvested timber. The outcomes of the correlation analysis showed a decreasing trend in incidence in the case of severe accidents. Workers ≤ 30 years or between 51 and 60 years of age were the most vulnerable in the case of fatal accidents. In the case of severe accidents, workers between 51 and 60 were the most vulnerable group. Most fatal accidents were suffered by tree fellers (72.2%) during felling (69.8%), who were in contact with falling trees and tree parts (93.7% share for this material agent). Conclusion and Practical Applications: The presented study identified the most vulnerable worker groups as well as the effects of factors on the occurrence of severe and fatal occupational accidents in forestry. The results will serve in development of occupational safety and health strategies in forest enterprises., Competing Interests: Declaration of Competing Interest The authors declare that they have no known competing financial interests or personal relationships that could have appeared to influence the work reported in this paper., (Copyright © 2023 National Safety Council and Elsevier Ltd. All rights reserved.)

The consumption of alcohol in a population is usually monitored through individual questionnaires, forensics, and toxicological data. However, consumption estimates have some biases, mainly due to the accumulation of alcohol stocks. This study's objective was to assess alcohol consumption in Slovakia during the COVID-19 pandemic-related lockdown using wastewater-based epidemiology (WBE). Samples of municipal wastewater were collected from three Slovak cities during the lockdown and during a successive period with lifted restrictions in 2020. The study included about 14% of the Slovak population. The urinary alcohol biomarker, ethyl sulfate (EtS), was analyzed by liquid chromatography-tandem mass spectrometry (LC-MS/MS). EtS concentrations were used to estimate the per capita alcohol consumption in each city. The average alcohol consumption in the selected cities in 2020 ranged between 2.1 and 327 L/day/1000 inhabitants and increased during days with weaker restrictions. WBE can provide timely information on alcohol consumption at the community level, complementing epidemiology-based monitoring techniques (e.g., population surveys and sales statistics).

The psychological consequences of trauma related to the Holocaust have been primarily studied in samples derived from Israel, North America, and Western Europe. Few studies have examined postcommunist countries in Central and Eastern Europe. The present study focused on three generations living in the Czech Republic and Slovakia after World War II (WWII): Holocaust survivors (71-95 years of age), their children (30-73 years of age), and their grandchildren (15-48 years of age). We compared scores on measures of posttraumatic stress symptoms (PTSS; the Posttraumatic Stress Disorder Checklist-Civilian Version) and posttraumatic growth (PTG; the Posttraumatic Growth Inventory) derived from three focal samples with scores from age-matched comparison participants. Higher PTSS scores emerged for Holocaust survivors in all generations, η 2 P=.087 but only participants in the first generation reported higher PTG scores relative to the comparison group, with small effect sizes for the overall group differences, η 2 P=.029 . These results are discussed in the historical and political context of postwar Czechoslovakia., (© 2021 International Society for Traumatic Stress Studies.)

Consumption of alcohol and new psychoactive substances (NPS) in a population or during special events (music festivals) is usually monitored through individual questionnaires, forensic and toxicological data, and drug seizures. However, consumption estimates have some biases due mostly to the unknown composition of drug pills for NPS and stockpiling for alcohol. The aim of this study was to evaluate for the first time the real use of alcohol and the occurrence of NPS in Slovakia by wastewater-based epidemiology (WBE). Urban wastewater samples were collected from nine Slovak cities over two years (2017-2018) and during three music festivals. The study included about 20% of the Slovak population and 50 000 festival attendees. The urinary alcohol biomarker ethyl sulfate (EtS) and thirty NPS were analyzed by liquid chromatography tandem mass spectrometry (LC - MS/MS). EtS concentrations were used for estimating the per capita alcohol consumption in each city. The average alcohol consumption in the selected cities and festivals in 2017-2018 ranged between 7 and 126 L/day/1000 inhabitants and increased during the weekends and music festivals. Five NPS belonging to the classes of synthetic cathinones (mephedrone, methcathinone, buphedrone and pentedrone) and phenethylamines (25-iP-NBoMe) were found in the low ng/L range. Methcathinone was the most frequently detected NPS, while the highest normalized mass load corresponded to mephedrone (3.1 mg/day/1000 inhabitants). Wastewater-based epidemiology can provide timely information on alcohol consumption and NPS occurrence at the community level that is complementary to epidemiology-based monitoring techniques (e.g. population surveys, police seizures, sales statistics)., Competing Interests: Declaration of competing interest The authors declare that they have no conflict of interest., (Copyright © 2020 Elsevier Inc. All rights reserved.)

The US EPA health risk assessment method is currently widely used to assess human health risks for many environmental constituents. It is used for risk assessment from the exposure to various contaminants exceeding tolerable or safe reference doses, determined e.g., for drinking water, soil, air and food. It accepts widely that excess contents of non-essential elements (e.g., As, Pb or Sb) in environmental compartments represent a general risk to human health. However, contrary to toxic trace elements, deficient contents of essential (biogenic) elements e.g., F, I, Se, Zn, Fe, Ca or Mg may represent even higher health risk. Therefore, we propose to extend the human health risk assessment by calculating the health risk for deficient content and intake of essential elements, and to introduce the terms Average Daily Missing Dose (ADMD), Average Daily Required Dose (ADRD) and Average Daily Accepted Dose (ADAD). We propose the following equation to calculate the Hazard Quotient (HQ) of health risk from deficient elements: HQ d = ADRD/ADAD. At present, there are no reference concentrations or doses of essential elements in each environmental compartment in world databases (Integrated Risk Information System IRIS, The Risk Assessment Information System RAIS). ADRD and ADMD can be derived from different regulatory standards or guidelines (if they exist) or calculated from actual regional data on the state of population health and content of essential elements in the environment, e.g., in groundwater or soil. This methodology was elaborated and tested on inhabitants of the Slovak Republic supplied with soft drinking water with an average Mg content of 5.66 mg·L -1 . The calculated ADMD of Mg for these inhabitants is 0.314 mg·kg -1 ·day -1 and HQ d is equal to 2.94, indicating medium risk of chronic diseases. This method extending traditional health risk assessment is the first attempt to quantify deficient content of essential elements in drinking water. It still has some limitations but also has potential to be further developed and refined through its testing in other countries.

Temporal patterns in occupational safety and health can shed light on the efficiency of safety measures companies adopt and identify when workers are prone to occupational accidents. We analyzed these patterns to identify the effects of factors such as the share of salvage logging, experience, age, daytime, weekday, and more on the number of occupational accidents at Forests of the Slovak Republic (FSR). We analyzed a database of 2963 occupational accidents and 443 occupational illnesses suffered by FSR employees and contractors. We then analyzed a subset of said database, containing 401 accident records coded according to European Statistics at Work manual. We used regression and correlation analyses and generalized linear models to test the relationship between the accident frequency and volume of harvested timber and volume of salvage logging. We used logistic regression, chi2 tests, and Cramér's V statistic to test when accidents occur within shifts, weeks, and months. We found the volume of harvested timber significantly affects the frequency of severe and fatal accidents of contractors (R 0.81; p < 0.05), whereas, for employees, the relationship was insignificant. Over time, the number of accidents and incidence rate decreased, and inexperienced or older workers were the most prone to accidents., Competing Interests: The authors declare no conflicts of interest.

Various types of micropollutants, e.g., pharmaceuticals and their metabolites and resistant strains of pathogenic microorganisms, are usually found in hospital wastewaters. The aim of this paper was to study the presence of 74 frequently used pharmaceuticals, legal and illegal drugs, and antibiotic-resistant bacteria in 5 hospital wastewaters in Slovakia and Czechia and to compare the efficiency of several advanced oxidations processes (AOPs) for sanitation and treatment of such highly polluted wastewaters. The occurrence of micropollutants and antibiotic-resistant bacteria was investigated by in-line SPE-LC-MS/MS technique and cultivation on antibiotic and antibiotic-free selective diagnostic media, respectively. The highest maximum concentrations were found for cotinine (6700 ng/L), bisoprolol (5200 ng/L), metoprolol (2600 ng/L), tramadol (2400 ng/L), sulfamethoxazole (1500 ng/L), and ranitidine (1400 ng/L). In the second part of the study, different advanced oxidation processes, modified Fenton reaction, ferrate(VI), and oxidation by boron-doped diamond electrode were tested in order to eliminate the abovementioned pollutants. Obtained results indicate that the modified Fenton reaction and application of boron-doped diamond electrode were able to eliminate almost the whole spectrum of selected micropollutants with efficiency higher than 90%. All studied methods achieved complete removal of the antibiotic-resistant bacteria present in hospital wastewaters.

The water bugs represent a significant component of the freshwater biota, play an important role in trophic webs, and may have considerable economic importance. Nevertheless, systematic research of this group has been underdeveloped in Slovakia (central Europe) for decades. This work presents a list of water bug species of Slovakia based on an exhaustive review of the literature (time span: 1808-2013) and on more than 14,000 individuals collected during extensive field campaigns (2010-2014) or obtained from insect collections. Fifty-six species belonging to 11 families of Heteroptera were recorded from a total of 767 sites. Seven species were recorded for the first time from Slovakia during our research. Among those, the first exact records of Corixa panzeri Fieber, 1848, Sigara (Subsigara) distincta (Fieber, 1848), Notonecta (Notonecta) lutea Müller, 1776, Notonecta (Notonecta) maculata Fabricius, 1794 and Microvelia (Microvelia) buenoi Drake, 1920 are provided here. Confusion concerning the records of two additional species, Arctocorisa carinata carinata (C. R. Sahlberg, 1819) and Hesperocorixa parallela (Fieber, 1860) is clarified. The water bugs species inventory appears to be nearly complete (~97 %) given an asymptotic richness estimate. The occurrence of other species is discussed taking into account their habitat requirements and distribution in neighbouring countries. Recommendations for future research are provided.

At present, there is no relevant information on arbuscular mycorrhiza and the effect of the symbiosis on the growth of wild populations of cyclamens. To fill this gap, two populations of Cyclamen purpurascens subsp. immaculatum, endemic in Nízke Tatry (NT) mountains and Veľká Fatra (VF) mountains, Slovakia, were studied in situ as well as in a greenhouse pot experiment. For both populations, mycorrhizal root colonization of native plants was assessed, and mycorrhizal inoculation potential (MIP) of the soils at the two sites was determined in 3 consecutive years. In the greenhouse experiment, the growth response of cyclamens to cross-inoculation with arbuscular mycorrhizal fungi (AMF) was tested: plants from both sites were grown in their native soils and inoculated with a Septoglomus constrictum isolate originating either from the same or from the other plant locality. Although the MIP of soil at the NT site was significantly higher than at the VF site, the level of AMF root colonization of C. purpurascens subsp. immaculatum plants in the field did not significantly differ between the two localities. In the greenhouse experiment, inoculation with AMF generally accelerated cyclamen growth and significantly increased all growth parameters (shoot dry weight, leaf number and area, number of flowers, tuber, and root dry weight) and P uptake. The two populations of C. purpurascens subsp. immaculatum grown in their native soils, however, differed in their response to inoculation. The mycorrhizal growth response of NT plants was one-order higher compared to VF plants, and all their measured growth parameters were stimulated regardless of the fungal isolates' origin. In the VF plants, only the non-native (NT originating) isolate showed a significant positive effect on several growth traits. It can be concluded that mycorrhiza significantly increased fitness of C. purpurascens subsp. immaculatum, despite the differences between plant populations, implying that AMF symbionts should be taken into account in conservation programs of this endemic plant.

Background: To screen whether E. coli strains encoding type 1 fimbriae, isolated from fecal microflora, produce bacteriocins more often relative to fimA-negative E. coli strains of similar origin., Methods: PCR assays were used to detect presence of genes encoding 30 bacteriocin determinants (23 colicin- and 7 microcin-encoding genes) and 18 virulence determinants in 579 E. coli strains of human and animal origin isolated from hospitals and animal facilities in the Czech and Slovak Republic. E. coli strains were also classified into phylogroups (A, B1, B2 and D)., Results: fimA-negative E. coli strains (defined as those possessing none of the 18 tested virulence determinants) were compared to fimA-positive E. coli strains (possessing fimA as the only detected virulence determinant). Strains with identified bacteriocin genes were more commonly found among fimA-positive E. coli strains (35.6%) compared to fimA-negative E. coli strains (21.9%, p<0.01) and this was true for both colicin and microcin determinants (p=0.02 and p<0.01, respectively). In addition, an increased number of strains encoding colicin E1 were found among fimA-positive E. coli strains (p<0.01)., Conclusions: fimA-positive E. coli strains produced bacteriocins (colicins and microcins) more often compared to fimA-negative strains of similar origin. Since type 1 fimbriae of E. coli have been shown to mediate adhesion to epithelial host cells and help colonize the intestines, bacteriocin synthesis appears to be an additional feature of colonizing E. coli strains.

Mature larva and pupa of Adosomus (s. str.) roridus (Pallas, 1781) (Curculionidae: Lixinae: Cleonini) are described and compared with ten other taxa of Cleonini with known larvae. This weevil is an oligophagous species on the Asteraceae family. From our observations in Slovakia, we found active adults from April to September in dry sunny places within extensively used or fallow vineyards and in ruderal vegetation with host plants. The weevil is a root borer--larvae, pupae and fresh adults were collected from the root necks and roots of Common Tansy (Tanacetum vulgare L.) and rarely from Mugwort (Artemisia vulgaris L.). Each plant was usually occupied by one larva, or more rarely with two or three larvae. The new generation of adult individuals appeared from early summer to autumn. Both larvae and some of the adults overwinter, which is quite unique within Cleonini.

The measurements of radon activity concentration carried out in residential houses of V4 countries (Hungary, Poland and Slovakia) show that radon levels in these countries considerably exceed the world average. Therefore, the new radon data and statistical analysis are required from these four countries. Each partner chose a region in their own country, where radon concentration in residential buildings was expected to be higher. The results of the survey carried out in the period from March 2012 to May 2012 show that radon concentrations are <200 Bq m(-3) in ∼87% of cases. However, dwellings with radon concentration ∼800 Bq m(-3) were found in Poland and Slovakia. It was also found that the distribution of radon frequency follows that of houses according to the year of their construction., (© The Author 2014. Published by Oxford University Press. All rights reserved. For Permissions, please email: journals.permissions@oup.com.)

Objective: The aim of the project was to evaluate criterias for women's contraception application way choice., Design: Non-interventional, multicentric, prospective project., Setting: Dept. of Obstetrics and Gynecology, 1st Faculty of Medicine, Charles University Prague., Methods: The choice of application way of combined hormone contraception was evaluate in group of 1944 women in 183 centers before and after doctor's consultation. Women's opinions, knowledges and socio-demografic predictors were corelated with final decision., Results: The most important factor choice of application way is minding that application is easy. It elevates probability of final choice by 65% for combined oral contraception and by 90% for path and vaginal ring. Vote for vaginal ring and patch is growing with age., Conclusions: Easibility of application, efficacy and cycle control are the most important predictors for contraception choice. The biggest fears are connected with adverse events for pills and low frequency of usage in population for patch and ring.

Celiac disease is diagnosed in children as well as adults. For long now, this has not been a rare diagnosis. Publications by local authors have warned, as early as 1990s, that this disease is extremely under-diagnosed, as confirmed by current clinical practice. Despite this, it is evident that the prevalence ofthis disease in Slovakia and the Czech Republic is high. Currently, it is assumed that the traditional symptomatic form of the disease represents just a fraction of the overall prevalence. As a consequence to the gradual introduction of the disease screening and the efforts of the medical community to diagnose the disease in its early stages, the disease is now thought of in a surgery of a first-contact physician as well as in a specialist surgery. Continually increasing number of diagnosed patients leads to increasing demands related to their needs. Strict gluten-free diet is the only available causal therapy. Gluten-free products are marked with a universally recognised international symbol--crossed-out grain spike--or are labelled "gluten-free". Some specialized family-run businesses focusing on their production have all foodstuffs labelled. However, this labelling is rather lacking on the products sold in high street stores. Based on the increasing incidence of celiac disease in the population, or rather improved diagnostic methods and increased awareness within the medical community of this diagnosis, the authors review the possibility of labelling all food products. This would be a universally recognised symbol, or a clear warning that the product contains gluten. It is recommended that a proposal is submitted to improve legislation related to labelling of gluten-containing products. It is recommended that the products labelled as "gluten-free" contain no gluten. It is proposed that the production of gluten-free food products is submitted to accreditation with well-defined methodology including strict control.

The subject of the study was the ecological and human health consequences of environmental pollution from emissions arising from burning local coal with an arsenic content ranging from 900 to 1,500 g/tonne of dry substance. The first indication of environmental pollution by arsenic-containing emissions was the mass extinction of honeybee colonies. The neurotoxic and carcinogenic aspects of arsenic exposure were followed. On using a group diagnostics approach, significant hearing losses were detected in exposed children in both air and bone conduction audiometry at high frequency range (4,000 and 8,000 Hz, respectively). Exposure assessment of the local population of the Prievidza district, Central Slovakia, was based on biological monitoring. The criterion of higher exposure was arsenic content in hair exceeding concentrations of 3 microg/g of hair. In a 7.5-km radius of the exposed region, live about two-tenths of the district population who were considered as "exposed" and rest of the district served as the "reference" population. The subject of our analysis was a database of 1,503 non-melanoma skin cancer (NMSC) cases (756 in men and 747 in women) collected from 1977 to 1996 in the Prievidza district, Central Slovakia (population approximately 125,000). The age standardized incidence of NMSC (each confirmed by histological examination) in non-occupational settings ranged from 45.9 to 93.9 in men and from 34.6 to 81.4 in women. Analysis of our data demonstrates a positive correlation between human cumulative exposure to arsenic and incidence of NMSC.

Background: The autosomal recessive Nijmegen breakage syndrome (NBS) is a DNA repair disorder due to a mutation in the NBS1 gene on 8q21. Hyperradiosensitivity and high risk for lymphoreticular malignancy are important reasons for early diagnosis and prevention by avoidance of ionisation. The frequency of NBS heterozygotes of the mutation 657de15, which is predominant in the Slavic population was estimated to be in the range of 1:90-1:314 in different parts of Poland, and 1:128-154 among Czech newborns, born 20 years ago., Methods and Results: Lower prevalence of affected homozygotes born in Czechoslovakia in the period 1969- 1992 (24 among 5.2 million newborns corresponds to 1:271000) than expected on the basis of carrier frequency is explained to be due to underdiagnosing because the rate of prenatal lethality in the NBS families is not increased or it is even lower than in the general population. The underdiagnosing of NBS is emphasized also by the mean age at diagnosis (7.5 years) although severe microcephaly is present at birth. The possibility to offer effective prevention of primary and secondary malignancies becomes the motivation for interdisciplinary collaboration with paediatricians, neurologists, immunologists and clinical geneticists. A decrease of the mean age down to 6 months at diagnosis among the 11 newly recognized patients has been achieved in the previous 4 years. The occurrence of homozygotes was relatively higher in Slovakia with 5 million inhabitants (14 patients in 11 families) than in the Czech Republic with a population of 10 million (21 patients in 14 families), and therefore the frequency of NBS heterozygotes was studied among 2996 newborns born in 2002-2003 in 12 maternity hospitals of west, middle and east Slovakia. Surprisingly, only 3 heterozygotes were found., Conclusions: This discrepancy of heterozygote frequency and the number of homozygotes shows that due to traditional subisolates the population is not in the genetic equilibrium. It explains the high prevalence of alcaptonuria in Slovakia in the middle of last century, which is a rare disorder in other countries.

Abdominal and gluteal (waist and hip) circumferences are accepted as indicators of central fat distribution and abdominal (waist) circumference is generally regarded as a simple anthropometric measure of visceral fat distribution. In the years 1987-88 there was carried out an anthropometric survey on the whole region of former Czechoslovakia, which was done in 16,400 of adult men and women, aged 20 to more than 70 years on the basis of the three grade statistical choice. Except for many other anthropometric measures, abdominal and gluteal (waist and hip) circumferences were estimated according to Martin and Saller [1957] (M62/1; M64/1). The results show that gluteal (hip) circumference is longer than abdominal (waist) circumference in adulthood similarly as in childhood. Abdominal circumference is longer in men than in women in agreement with sexual dimorphism. Abdominal circumference is not significantly different in Czech and Slovak men and Czech and Slovak women. Nevertheless, in Slovak women increases their abdominal circumference to maximum at the beginning their fifth decade and then decreases concurrently with lowering of BMI while in Czech women increase in abdominal circumference lasts till seventh decade. Maximal intersexual difference is found in the third decade (21-25 years) and at the end of follow-up in both populations. Gluteal (hip) circumference is not significantly different in men and women to the half of the forth decade in both Czech and Slovak populations. Afterwards it is markedly longer in women probably due to the higher accumulation of subcutaneous fat. In the second part of seventh decade in Czech population and about five years earlier in the Slovak group the circumference is getting shorter on the average value in men. This change can be caused by the age involution of adipose tissue. The examined data were constructed into percentile nets of the abdominal and gluteal circumferences for both men and women and Czech and Slovak populations.

Background: We present a series of 25 patients (from 21 families) with deficiency of lysosomal sphingomyelinase (acid sphingomyelinase, ASM), diagnosed during the last 30 years., Methods and Results: Diagnosis was established by finding specific sphingomyelin storage pattern in bone marrow histiocytes and in some bioptical and postmortem tissues (presence of sphingomyelin liquid crystals) and finally by proving ASM deficiency in white blood cells and in cultured fibroblasts. Range of clinical manifestations of our patients notably exceeded the the known main (A,B) phenotypes described so far. In the group of type A patients (clinically overt neurovisceral symptomatology) there was significant tendency to prolonged course. Classical fulminant course with death between 5 to 45 months of age was seen only in a subgroup of 5 patients. In other type A patients (n = 8) the course was prolonged attaining 5 years of age, the end of the first (8, and 9 years), second (14, 17 years), third (22 years), fourth (32 years) and fifth decades (41 years). Three of these patients (aged 5, 22 and 41 years) are living. The series of patients with dominant visceral involvement (n = 12) consisted of three phenotypically different subgroups. One with chronic purely visceral affection and prolonged course (n = 4) corresponding to the classical type B, the second with chronic course and largely subclinical neurological affection (n = 4) and the third with accelerated fatal visceral affection (death in age range 31 months-9 years) without (n = 1) or with clinically minor signs of brain damage (n = 3)., Conclusions: Study of the presented series of ASM deficient patients disclosed remarkable phenotypical variability. Two main factors seem to be responsible. Variability in the storage intensity in the two main tissue compartments (neuronal and visceral), and the absence of proportionality in their affection in some instances. The described phenotype variability enlarges significantly the known spectrum of phenotypes in ASM deficiency.

Czech and Slovak medical literature (articles in journals and proceedings, book reviews, monographs) is registered in the Bibliographia medica cechoslovaca basis generated by the National Medical Library, Prague. Quantitative trends in the publishing of this literature in the period 1978-1999 are outlined.

Background: Abdominal and gluteal (waist and hip) circumferences are accepted as indicators of central fat distribution and abdominal (waist) circumference is generally regarded as a simple anthropometric measure of visceral fat distribution., Methods and Results: In the years 1987-1988 there was carried out an anthropometric survey on the region of former Czechoslovakia. Crossover study was performed in 16,400 of adult men and women aged from 20 to more than 70 years on the basis of the three level random sample. Except for many other anthropometric measures, abdominal and gluteal (waist and hip) circumferences were assessed according to Martin (M62/1; M64/1). The results show that gluteal (hip) circumference is longer than a abdominal (waist) circumference in adulthood similarly as in childhood. Abdominal circumference is larger in men than in women in agreement with sexual dimorphism. Abdominal circumference is not significantly different in Czech and Slovak men and Czech and Slovak women. Nevertheless, abdominal circumference in Slovak women increases to maximum at the beginning of their fifth decade and then decreases concurrently with lowering of BMI. In Czech women increase in abdominal circumference lasts till seventh decade. This difference can result from the earlier involution in Slovak women. Maximal intersexual difference is found at the beginning of the third decade (21-25 years) and at the end of follow-up in both populations., Conclusions: Gluteal (hip) circumference is not significantly different in men and women to the half of the forth decade in both Czech and Slovak populations. In following decades it is markedly longer in women probably due to the higher accumulation of subcutaneous fat. In the second part of seventh decade in Czech population and about five years earlier in the Slovak group the average circumference in men decreases. This change can be caused by the age involution of adipose tissue. The examined data were constructed into percentile nets of the abdominal and gluteal circumferences for both men and women and Czech and Slovak populations.

Background: Homocystinuria due to cystathionine beta-synthase deficiency is an autosomal recessive disorder of methionine metabolism. It manifests with vascular, central nervous system and connective tissue disturbances, and phenotypically resembles Marfan's syndrome. We analysed the clinical course of homocystinuria in Czech and Slovak patients., Methods and Results: The group of homocystinuric patients consisted of 19 individuals (12 males and 7 females) aged 5-32 years (average age 18 years), who were diagnosed between 1980 and 1999. The overall incidence of homocystinuria in the Czech and Slovak Republics was 1:287,000. The proportion of pyridoxine-responsive patients was 47%. The average follow-up period was 10 years (range 1 month to 19 years). The prevalence of the individual signs in the group was as follows: lens dislocation--95% of patients, progressive myopia--79%, marfanoid habitus--74%, kyfoscoliosis--68%, osteoporosis--63%, psychomotor retardation--58%, other neurologic symptomatology--58% and tromboembolism--21%. The average delay between the first sign of the disease and the time when the diagnosis was made was 4 years (range 1 to 14 years). At the time of diagnosis the average levels of metabolites in plasma were as follows: total homocysteine 348 mumol/l (range 211-536), free homocystine 70 mumol/l (range 0-203) and methionine 359 mumol/l (range 75-937)., Conclusions: Both the clinical course of homocystinuria due to the cystathionine beta-synthase deficiency and its incidence in the Czech and Slovak Republics are similar to those in other populations. Since homocystinuria is a treatable disease, it should be included in the differential diagnosis of Marfan's syndrome, tromboembolism and severe psychomotor retardation.

Invasive meningococcal diseases have become in the Czech Republic since 1993 a serious epidemiological and clinical problem due to a clonus which was not present previously: Neisseria meningitidis C:2a:P1.2,P1.5, ET-15/37. In 1996 a trial was conducted focused on the problem how this altered epidemiological and clinical situation is reflected in carriership of Neisseria meningitidis and Neisseria lactamica in the healthy population. Two age groups were followed up which were most severely affected by the new clonus of the meningococcus: 15-19 years (410 subjects) and 1-4 years (116 subjects). The trial was implemented in Olomouc where in 1993 the new epidemiological situation of the incidence of the invasive meningococcal disease was so serious that targeted vaccination was introduced. Of 116 children in the age group from 1-4 years in none Neisseria meningitidis was detected, in 9 Neisseria lactamica was found (7.7%). On repeated examination of children with a positive cultivation of Neisseria lactamica after two weeks in none Neisseria meningitidis nor Neisseria lactamica were found. Of 410 subjects in the age group from 15-19 years in none Neisseria lactamica was detected and in 35 Neisseria meningitidis (8.5%). Examinations were repeated after two weeks in 33 carriers: in 31 Neisseria meningitidis was again cultivated. Analysis of factors influencing carriership revealed in Neisseria lactamica two factors in young children which significantly promote this carriership: cold and close contact/kissing. A risk factor at the limit of significance are frequent respiratory diseases. In the carriership of Neisseria meningitidis in 15-19 year-old subjects six factors were revealed which promote carriership. A significant risk factor is close contact/kissing, the existence of partnership, participation in activities of the "disco" type, living in a town, flats in the centre of the town. Effort is a risk factor at the limit of significance.

Heart transplantation (HTx) became an established treatment of the end stage heart failure. The limiting factor of transplantation activity is the availability of donors which could be expanded by multiorgan harvesting and distant heart procurement (DHP). In spite of the fact that only 4-5 hours of could ischaemia (CI) is favourable it is still acceptable to organize the DHP within the radius over 1000 km from a recipient hospital. Good co-ordination, he best communication and transportation technology are absolutely necessary. Only in the case when there is no local recipient on the waiting list fr the HTx we offer an organ abroad. Paediatric Transplant Center Motol has been offered such 19 heart donors since 1992. 7 of them were accepted and in 4 (donor age 10-40 y.) successful DHP was performed, the organs were transported (900-1100 km) and HTx were performed at Harefield Hospital, UK. CI was 4:20-5:05 hours (mean 4:36). Two organs were used for orthotopic HTx (OHTx), two for heterotopic HTx (HHTx). Two failed due to the acute rejection. One was successfully retransplanted, went into chronic renal failure and is maintained on dialysis. Another recipient died 1 w. after HHTx due to the failure of his own heart. One recipient has been living already 1 y. since his OHTx. This experience demonstrates the ability and competence to organize and perform the DHP by a local team even in our conditions. We consider it suitable and necessary prerequisite to be able to start our own paediatric HTx programme.