Clinical characteristics of Polish patients with ANCA-associated vasculitides-retrospective analysis of POLVAS registry.

OBJECTIVE: Antineutrophil cytoplasmic antibody (ANCA)-associated vasculitides (AAV) are rare small to medium-size vessel systemic diseases. As their clinical picture, organ involvement, and factors influencing outcome may differ between countries and geographical areas, we decided to describe a large cohort of Polish AAV patients coming from several referral centers-members of the Scientific Consortium of the Polish Vasculitis Registry (POLVAS). METHODS: We conducted a systematic multicenter retrospective study of adult patients diagnosed with AAV between Jan 1990 and Dec 2016 to analyze their clinical picture, organ involvement, and factors influencing outcome. Patients were enrolled to the study by nine centers (14 clinical wards) from seven Voivodeships populated by 22.3 mln inhabitants (58.2% of the Polish population). RESULTS: Participating centers included 625 AAV patients into the registry. Their distribution was as follows: 417 patients (66.7%) with GPA, 106 (17.0%) with MPA, and 102 (16.3%) with EGPA. Male-to-female ratios were almost 1:1 for GPA (210/207) and MPA (54/52), but EGPA was twice more frequent among women (34/68). Clinical manifestations and organ involvement were analyzed by clinical phenotype. Their clinical manifestations seem very similar to other European countries, but interestingly, men with GPA appeared to follow a more severe course than the women. Fifty five patients died. In GPA, two variables were significantly associated with death: permanent renal replacement therapy (PRRT) and respiratory involvement (univariate analysis). In multivariate analysis, PRRT (OR = 5.3; 95% confidence interval (CI) = 2.3-12.2), respiratory involvement (OR = 3.2; 95% CI = 1.06-9.7), and, in addition, age > 65 (OR = 2.6; 95% CI = 1.05-6.6) were independently associated with death. In MPA, also three variables were observed to be independent predictors of death: PRRT (OR = 5.7; 95% CI = 1.3-25.5), skin involvement (OR = 4.4; 95% CI = 1.02-19.6), and age > 65 (OR = 6.3; 95% CI = 1.18-33.7). CONCLUSIONS: In this first multicenter retrospective study of the Polish AAV patients, we have shown that their demographic characteristics, disease manifestations, and predictors of fatal outcome follow the same pattern as those from other European countries, with men possibly suffering from more severe course of the disease.

Linear perturbation renormalization group for the two-dimensional Ising model with nearest- and next-nearest-neighbor interactions in a field.

The linear perturbation renormalization group (LPRG) is used to study the phase transition of the weakly coupled Ising chains with intrachain (J) and interchain nearest-neighbor (J_{1}) and next-nearest-neighbor (J_{2}) interactions forming the triangular and rectangular lattices in a field. The phase diagrams with the frustration point at J_{2}=-J_{1}/2 for a rectangular lattice and J_{2}=-J_{1} for a triangular lattice have been found. The LPRG calculations support the idea that the phase transition is always continuous except for the frustration point and is accompanied by a divergence of the specific heat. For the antiferromagnetic chains, the external field does not change substantially the shape of the phase diagram. The critical temperature is suppressed to zero according to the power law when approaching the frustration point with an exponent dependent on the value of the field.

Optimisation of vasculitis disease assessments in clinical trials, clinical care and long-term databases.

The systemic vasculitides are a group of rare, chronic, relapsing, but often progressive inflammatory conditions. They are associated with a significant burden of morbidity both due to scarring from the disease itself and as a consequence of treatment with glucocorticoids and other potent immunosuppressive agents. Careful assessment of disease activity is critical to guide appropriate use of these potentially toxic therapies. It is also important to differentiate features of active disease from those attributable to damage, which will not respond to immunosuppression. As these are chronic complex conditions, the impact on a patient's functional ability and quality of life are also important considerations. Given the lack of a reliable biomarker for assessment of disease activity or damage in systemic vasculitis, clinical tools developed and validated for use initially in clinically trials are key outcome measures in the evaluation of these patients. While the conduct of randomised clinical trials in vasculitis has been significantly enhanced by the development and use of validated outcome measures, regular use of validated disease activity and damage measurements as part of routine care offers a structured approach, which can serve as the basis of justifying treatment decisions. The authors review the concepts of clinical assessment tools used in the evaluation of patients with systemic vasculitis in the setting of clinical practice, clinical trials and long term databases with particular emphasis on disease activity, damage, prognosis and function.

Critical point of a para-ferrimagnetic phase transition of the ANNNI model in a field.

The finite field para-ferrimagnetic phase transition in the axial next-nearest-neighbor Ising (ANNNI) model is studied by using the linear perturbation real space renormalization group transformation. The method is examined in systems of ferromagnetic and antiferromagnetic Ising chains coupled by ferromagnetic interactions in the longitudinal field. As one would expect, the external field in the first case destroys the continuous phase transition and in the second shifts the critical point toward a lower temperature according to the square law. For the ferromagnetic chains coupled by the competing interchain nearest-neighbor J(1) < 0 and next-nearest-neighbor J(2) < 0 interactions, the external field changes the character of the phase transition from continuous to discontinuous. However, it has been found that for the ratio J(2)/J(1) around 0.5 there is a critical value of the field for which an isolated critical point exists. The temperature dependences of the specific heat for several values of J(2)/J(1) and the external field are presented.

[Gastric neurofibroma--still a diagnostic problem]. / Neurofibroma zoladka--nadal aktualny problem diagnostyczny.

We report a case of gastric neurofibroma encountered in 41-year-old woman who complained of dyspepsia and physical examination revealed palpable mass in her abdomen. It was not possible to determine the nature and origin of the tumor by radiological and endoscopic investigations. At laparotomy the tumor was found to be pendiculated and growing extramurally from the anterior wall of the stomach. Wedge gastric resection, including the mass, was performed. Histological examination revealed a spindle cell gastric tumor, immunohistochemically differentiated as a neurofibroma.

[Hypersensitivity to house dust mites and incidence of acute respiratory tract infections in children with bronchial asthma]. / Uczulenie na roztocza kurzu domowego a czestosc wystepowania ostrych zakazen ukladu oddechowego dzieci z astma oskrzelowa.

The aim of the study was to evaluate the relationship between h.d.m. allergy and incidence of respiratory tract infections in children with episodic, mild and moderate asthma. 32 children with bronchial asthma being followed up at the I. Department of Children Diseases P.A.I.P. CMUJ in Cracow were included into the study. The positive skin prick test (SPT) with H.D.M. allergens was confirmed in 18 children. The negative SPT allergens were observed in 14 patients. All patients were examinated by the retrospective inquiry which included the degree of asthma, severity of therapy schedule and incidence of acute respiratory tract infections during the last 6 months. The anthropometric measurement (length, weight, body mass index, thorax circumference) and spirometric parameters with physical examination challenge were introduced. The mean number of infections in group of patients sensitive to H.D.M. was 6.8; in non sensitive group-4.6. There was no significant difference in incidence of acute respiratory tract infections between both groups. It seems likely that small number of patients may influence the statistics. To verify the results, prospective observation of wider group of patients is planned in forthcoming winter.

Poland-US collaborative study on cardiovascular epidemiology: classification agreement between US National Cholesterol Education Program and European Atherosclerosis Society hyperlipidemia guidelines in selected Polish and US populations.

Data from two epidemiological studies are used to measure the degree to which two well-known guidelines agree in measuring hyperlipidemia in population samples in the US and Poland. The epidemiological studies are the US Lipid Research Clinics Program Prevalence Study and the Pol-MONICA project in Poland and the guidelines are those adopted by the US National Cholesterol Program (USNCEP) and by the European Atherosclerosis Society (EAS). EAS guidelines were analyzed in two ways: Method 1 used triglycerides and total cholesterol only in classifying persons as hyperlipidemics or non-hyperlipidemics; Method 2 used triglycerides, total cholesterol and nine additional risk factors in the classification process. USNCEP guidelines used total cholesterol, low density lipoprotein cholesterol and the same additional nine risk factors used in EAS Method 2 in classifying hyperlipidemics. Classification differences between the two sets of guidelines were high when EAS Method 1 guidelines were compared with USNCEP guidelines. However, EAS Method 2 which included risk factors, compared favorably with USNCEP guidelines in all three populations under study.

A case of abetalipoproteinaemia in a Polish family.

Abetalipoproteinaemia (ABLP) was diagnosed in a brother and sister, 9 and 13 years old, presenting with symptoms of malabsorption during the neonatal period. Both children showed most of the main clinical features of ABLP, including neurological, and ophthalmic symptoms, and mental retardation. Acanthocytosis of erythrocytes was almost complete in the affected children, while in most of the remaining 11 members of their three-generation family, it was found in less than 50% of red blood cells. Absence of apoprotein B and low concentrations of apo A-I and lipids were found only in ABLP-affected children. Among five siblings only the two affected children had ABLP-characteristic lipid storage in enterocytes. The latter features correlated better with clinical symptoms than did the acanthocytosis of erythrocytes.

[Utilization of risk factors in medical practice for ischemic heart disease evaluation]. / Uzyteczny w praktyce lekarskiej wskaznik ryzyka zachorowania na chorobe niedokrwienna serca.

The Multivariate risk of developing Ischaemic Heart Disease (IHD) during period of 6 years was estimated for men aged 40-59 years on the basis of 2 Polish populations. Coefficients of multivariate logistic function (MLF) were calculated using sample of 4831 men with 240 cases of IHD. The significant risk factors were: age, total serum cholesterol level, diastolic blood pressure, number of cigarettes smoked, familial history of IHD. For these five significant factors new MLF coefficients were calculated. Discriminant power and goodness of fit for both functions were analysed and no significant differences found. The reduced MLF was applied to construct a simple questionnaire of IHD risk test.

A comparative analysis of the spectrum of blood lipoproteins in random population samples. A Soviet-Polish cooperative lipid project.

Mean values of cholesterol contents in low- and high-density lipoproteins, and the 10% and 90% cut-off points of their distribution were determined in random samples of men aged 35-64 years and resident in three Soviet cities (Kiev, Moscow, Novosibirsk) and two Polish regions (Warsaw, and Tarnobrzeg province). Comparative analysis revealed that, while having identical mean total cholesterol levels, the studied populations differ both in the character of cholesterol distribution in atherogenic and nonatherogenic lipoproteins and in their age dynamics. The lipoprotein spectrum was also compared with data of overall mortality and that from ischaemic heart disease and stroke in the respective areas. The comparison suggested a potential association between the incidence of these diseases and the incidence of individual lipid risk factors in the population. The regional specifics of the blood lipoprotein spectrum must also be considered when developing programmes of atherosclerosis prevention.

[12-year trends in cardiovascular disease mortality]. / Dwunastoletnie trendy umieralnosci z powodu chorób ukladu krazenia.

The coefficients and trends of mortality in years 1976-1987, based on data of Central Statistical Office, has been estimated in population of men and women aged 25 to 64 years, inhabitants of the Warsaw city region or the county region of Tarnobrzeg province . The trend of decrease of general mortality due to diseases of circulatory system has been statistically significant one similarly as that of mortality due to other heart diseases or ischaemic heart disease in men (group aged 25-34 or 35-44 years) in the Tarnobrzeg province . The analogously significant trend of mortality due to diseases of circulatory system or ischemic heart diseases in men aged 35-44 years has been stated in the Warsaw inhabitants. In the group of women an increase of general mortality has been noted in the youngest age group in Warsaw. In Warsaw and the Tarnobrzeg province in 1987 deaths due to diseases of circulatory system represented 33.2% and 39.9%, respectively, of total death pool among subjects aged 25 to 64 years. The respective percentages in the group of women were 29.1% and 39.4%.

[Myocardial infarction incidence and mortality among urban and rural populations during the 3-year period]. / Zachorowalnosc i smiertelnosc spowodowana zawalem serca populacji wielkomiejskiej i rolniczej w okresie 3-letniej obserwacji.

In years 1984-1986 3639 men and 1885 women were registered with suspected myocardial infarction from right-side Warsaw, simultaneously 2371 men and 917 women with the same suspected disease were registered from the Tarnobrzeg province population. The myocardial infarction diagnosis was confirmed in 54% of men and 43% of women from the Warsaw population as well as in 77% of men and 66% of women from Tarnobrzeg province population. In Warsaw the standardized incidence due to myocardial infarction increased during years of study from 466 to 588/100,000 among men and from 178 to 206/100,000 among women. The analogous increase among the Tarnobrzeg province population was 302 to 559/100,000 among men and 64 to 195 among women. Fatality during 28 days from the onset of the disease did not show any relationship with year of study and corresponded yearly among men to 45% and among women to 37% in Warsaw and to 42% among men and 33% among women in the Tarnobrzeg province . High fatality was noted during first 24 hours of hospitalization (Warsaw: 36% among men and 26% among women, Tarnobrzeg: 39% among men and 32% among women). The highest fatality was noted in the youngest of analysed groups.

[Stroke incidence and mortality among urban and rural populations during the 3-year period]. / Zachorowalnosc i smiertelnosc na udar mózgu w populacji wielkomiejskiej i rolniczej w okresie trzyletniej obserwacji.

In years 1984-1986 582 men and 338 women have been registered with clinical diagnosis of cerebral stroke. The respective numbers in the Tarnobrzeg province were 340 and 263. Preliminary diagnosis of cerebral stroke was confirmed according to criteria of registration in 82% of men or women from Warsaw and in 81% of men and 84% of women in the Tarnobrzeg province . Standardized incidence due to cerebral stroke was significantly higher in every of analysed years in the Warsaw population both among men and women in compare with that in the Tarnobrzeg province . In both population studied the incidence was significantly higher among men than among women. Standardized fatality during 28 days from the start of sickness was higher in the Tarnobrzeg province population than in the Warsaw population so far as both men and women are concerned. In both populations among men and women the most frequently diagnosis was acute disease of cerebral vessels.

[Characteristics of the main risk factors of coronary disease among the population of Warsaw and rural agricultural population of the Tarnobrzeg province]. / Charakterystyka glównych czynników ryzyka choroby niedokrwiennej serca w populacji wielkomiejskiej Warszawy i rolniczo-przemyslowej województwa tarnobrzeskiego.

Basing on results of first screening of the Pol-MONICA Project in 1984 in two centres that is the Tarnobrzeg province and two districts of Polish capital Warsaw (Praga South and Praga North) the level of 11 main coronary disease risk factors was compared in 2469 men and 2729 women. The following 5 risk factors were statistically significant for both men and women: the Durnin index of body fat content, the total cholesterol concentration in the plasma, triglyceride level, numbers of daily meals. The mean level of two factors that is the HDL-cholesterol concentration and the HDL-cholesterol/total cholesterol ratio was lower in the Warsaw sample what speaks in favor of concept that the ischemic heart disease threat is greater in that population. Among men the Warsaw population exhibited moreover the significantly higher value of diastolic blood pressure as well as of Quetelet body mass index; age and numbers of cigarettes smoked daily has also been higher in that population. In women from the Tarnobrzeg province ++ the diastolic blood pressure was higher than that in the Warsaw population. The two populations studied showed also highly significant differences so far as the complex of all 11 features is concerned what enabled the qualification of 72.3% of men, and 68.4% of women as belonging to the big city population or to the agricultural-industrial population using the discriminant Fisher function.

[Effect of inter-population differences in the levels of selected factors on the differences in arterial blood pressure]. / Wplyw miedzypopulacyjnych róznic w poziomie wybranych czynników na róznice w cisnieniu tetniczym krwi.

According to the Pol-MONICA program the random selected population samples were studied in inhabitants of Warsaw or the Tarnobrzeg province . After excluding from analysis the subjects treated with the hypotensive++ or hypolipemic drugs the differences between populations studies with regard to range of mean pressure value, except systolic pressure (RRs) in women, appeared significant ones. In populations studied the arterial blood pressure (CTK) was influenced by: age, sex, education, family history with regard to the circulatory system, the alcohol intake, smoking, heart action frequency the Quetelet coefficient value, triglyceride concentration and daily sodium intake. After analysis of inter-population differences in values of above factors the mean RRs values in populations studied did not differed significantly whereas differences in mean values of diastolic pressure (RRr) were highly statistically significant.

[Plasma lipid and lipoprotein levels among rural and urban populations in Poland in relation to risk factors of coronary disease]. / Stezenie lipidów i lipoprotein osocza w populacji rolniczej i przemyslowej Polski zaleznie od czynników ryzyka choroby niedokrwiennej serca.

There are significant differences in plasma total cholesterol, LDL-cholesterol, HDL-cholesterol, triglycerides and blood pressure, prevalence of cigarette smoking, obesity, education level and alcohol consumption found between the rural and industrial populations in Poland. It was found that the differences in plasma lipids and lipoproteins concentration are related to the differences in age, sex, education level, alcohol consumption, obesity, cigarette smoking and blood pressure. Increase of education level was related to increase in plasma concentration of total cholesterol. LDL-cholesterol and triglycerides but it was related to decrease of HDL-cholesterol. After the adjustment to all above factors the differences in total cholesterol and LDL-cholesterol between the studied populations appeared insignificant. The differences in HDL-cholesterol and triglycerides remained significant.

[Comparison of parameters and factors affecting the differences in obesity among urban population of Warsaw and the rural agricultural-industrial population of the Tarnobrzeg province]. / Porównanie parametrów i czynników mogacych wplywac na zróznicowanie otylosci w populacji wielkomiejskiej Warszawy i rolniczo-przemyslowej województwa tarnobrzeskiego.

Comparison of the parameters of obesity between two populations, urban from Warsaw and agricultural-industrial from Tarnobrzeg province revealed that skinfolds thickness, Quetelet index and body fat index, and per cent of overweight were statistically significantly higher in men from urban population. In the group of women these parameters were also statistically higher in Warsaw population with the exception of triceps skinfold and Quetelet index--differences of no statistical significance. There were also differences in the set of variables which correlate with the parameters of obesity between these two tested populations.