Decreased quality of life (QoL) is a significant complication of most rheumatic conditions in adults, whereas data on health-related quality of life (HRQoL) in children with juvenile idiopathic arthritis (JIA) are limited. Patient-Reported Outcomes Measurement Information System® (PROMIS® ) instruments assessing quality of life (QoL) components can be scored using disease-specific severity thresholds. Our study aimed to compare the results of generic and one of the JIA-specific scorings regarding PROMIS® Pediatric Mobility, Pain Interference, and Fatigue questionnaires, introduce PROMIS® Pediatric Global Health 7 questionnaire as a valuable method to assess QoL specifically in patients with JIA and determine differences in self-reported QoL in juvenile idiopathic arthritis (JIA) patients and their healthy peers in the Polish population. In this single-center cross-sectional study, four self-reported questionnaires derived from the PROMIS® Pediatric Item Bank (Global Health, Pain Interference, Fatigue, Mobility) were administered to 52 patients with JIA (8–17 years; mean 13.2 ± 2.9; girls 59.6

Age-related sarcopenia, resulting from a gradual loss in skeletal muscle mass and strength, is pivotal to the increased prevalence of functional limitation among the older adult community. The purpose of this meta-analysis of individual patient data is to investigate the difference in health-related quality of life between sarcopenic individuals and those without the condition using the Sarcopenia Quality of Life (SarQoL) questionnaire. A protocol was published on PROSPERO. Multiple databases and the grey literature were searched until March 2023 for studies reporting quality of life assessed with the SarQoL for patients with and without sarcopenia. Two researchers conducted the systematic review independently. A two-stage meta-analysis was performed. First, crude (mean difference) and adjusted (beta coefficient) effect sizes were calculated within each database; then, a random effect meta-analysis was applied to pool them. Heterogeneity was measured using the Q-test and I-2 value. Subgroup analyses were performed to investigate the source of potential heterogeneity. The strength of evidence of this association was assessed using GRADE. From the 413 studies identified, 32 were eventually included, of which 10 were unpublished data studies. Sarcopenic participants displayed significantly reduced health-related quality of life compared with non-sarcopenic individuals (mean difference = -12.32; 95 % CI = [-15.27; -9.37]). The model revealed significant heterogeneity. Subgroup analyses revealed a substantial impact of regions, clinical settings, and diagnostic criteria on the difference in health-related quality of life between sarcopenic and non-sarcopenic individuals. The level of evidence was moderate. This meta-analysis of individual patient data suggested that sarcopenia is associated with lower health-related quality of life measured with SarQoL.

Introduction. Percentiles of birth weight by gestational age (GA) are an essential tool for clinical assessment and initiating interventions to reduce health risks. Unfortunately, Poland lacks a reference chart for assessing newborn growth based on the national population. This study aimed to establish a national reference range for birth weight percentiles among newborns from singleton deliveries in Poland. Additionally, we sought to compare these percentile charts with the currently used international standards, INTERGROWTH-21 and WHO. Materials and Methods. All singleton live births (n = 3,745,239) reported in Poland between 2010 and 2019 were analyzed. Using the Lambda Mu Sigma (LMS) method, the Generalized Additive Models for Location Scale, and Shape (GAMLSS) package, smoothed percentile charts (3–97) covering GA from 23 to 42 weeks were constructed. Results. The mean birth weight of boys was 3453 ± 540 g, and this was higher compared with that of girls (3317 ± 509 g). At each gestational age, boys exhibited higher birth weights than girls. The weight range between the 10th and 90th percentiles was 1061 g for boys and 1016 g for girls. Notably, the birth weight of Polish newborns was higher compared to previously published international growth standards. Conclusion. The reference values for birth weight percentiles established in this study for Polish newborns differ from the global standards and are therefore useful for evaluating the growth of newborns within the national population. These findings hold clinical importance in identifying neonates requiring postbirth monitoring.

BACKGROUND:Air pollution is a major risk factor for public health worldwide, but evidence linking this environmental problem with the mortality of children in Central Europe is limited.OBJECTIVE:To investigate the relationship between air pollution due to the emission of industry-related particulate matter and mortality due to respiratory diseases under one year of age.METHODS:A retrospective birth cohort analysis of the dataset including 2,277,585 children from all Polish counties was conducted, and the dataset was matched with 248 deaths from respiratory diseases under one year of age. Time to death during the first 365 days of life was used as a dependent variable. Harmful emission was described as total particle pollution (TPP) from industries. The survival analysis was performed using the Cox proportional hazards model for the emission of TPP at the place of residence of the mother and child, adjusted individual characteristics, demographic factors, and socioeconomic status related to the contextual level.RESULTS:Infants born in areas with extremely high emission of TPP had a significantly higher risk of mortality due to respiratory diseases: hazard ratio (HR) = 1.781 [95% confidence interval (CI): 1.175, 2.697], p = 0.006, compared with those born in areas with the lowest emission levels. This effect was persistent when significant factors were adjusted at individual and contextual levels (HR = 1.959 [95% CI: 1.058, 3.628], p = 0.032). The increased risk of mortality was marked between the 50th and 150th days of life, coinciding with the highest exposure to TPP.CONCLUSIONS:The emission of TPP from industries is associated with mortality due to respiratory diseases under one year of age. A considerable proportion of children's deaths could be prevented in Poland, especially in urban areas, if air pollution due to the emission of particle pollution is reduced.

Background: The objective of this retrospective cohort study was to present the experience of 20-year-long comprehensive care of pediatric patients with familial hypercholesterolemia (FH) in a single academic center. Methods and Results: The study included 84 children aged 1–18 years with FH. For the whole study group, 535 medical visits were recorded. The mean follow-up period was 33.6 months. Molecular testing performed in 55 children (65%) provided genetic confirmation of the diagnosis in 36 children (43%). Twenty-seven children (32%) were treated pharmacologically with statins. Follow-up during the treatment averaged 29 months. Treatment with statins was associated with a mean reduction in total cholesterol and LDL-cholesterol levels of 24 and 33% from the baseline. Symptoms of statin intolerance occurred incidentally and did not require amendment in the treatment protocol. Significantly higher values of body weight, height, and BMI were found only among girls older than 10 years who were treated with statins. Conclusions: These data confirm a high efficacy and a good safety profile of statin treatment in children with FH, demonstrating no harm to physical development. However, there is a need for further cause-and-effect research regarding associations between long-term treatment with low-cholesterol, low-fat diets, statin therapy, and excessive weight gain.

Vitamin D, known for its essential role in calcium and bone homeostasis, has multiple effects beyond the skeleton, including regulation of immunity and modulation of autoimmune processes. Several reports have shown suboptimal serum 25 hydroxyvitamin D [25(OH)D] levels in people with different inflammatory and autoimmune rheumatic conditions, and an association between 25(OH)D levels, disease activity and outcomes. Although most available data pertain to adults, insights often are extended to children. Juvenile rheumatic diseases (JRDs) are a significant health problem during growth because of their complex pathogenesis, chronic nature, multisystemic involvement, and long-term consequences. So far, there is no definitive or clear evidence to confirm the preventive or therapeutic effect of vitamin D supplementation in JRDs, because results from randomized controlled trials (RCTs) have produced inconsistent outcomes. This review aims to explore and discuss the potential role of vitamin D in treating selected JRDs. Medline/PubMed, EMBASE, and Scopus were comprehensively searched in June 2023 for any study on vitamin D supplementary role in treating the most common JRDs. We used the following keywords: “vitamin D” combined with the terms “juvenile idiopathic arthritis”, “juvenile systemic scleroderma”, “juvenile systemic lupus erythematosus”, “juvenile inflammatory myopathies”, “Behcet disease”, “periodic fever syndromes” and “juvenile rheumatic diseases”. Observational studies have found that serum 25(OH)D concentrations are lower in juvenile idiopathic arthritis, juvenile systemic lupus erythematosus, juvenile systemic scleroderma, Behcet disease and proinflammatory cytokine concentrations are higher. This suggests that vitamin D supplementation might be beneficial, however, current data are insufficient to confirm definitively the complementary role of vitamin D in the treatment of JRDs. Considering the high prevalence of vitamin D deficiency worldwide, children and adolescents should be encouraged to supplement vitamin D according to current recommendations. More interventional studies, especially well-designed RCTs, assessing the dose–response effect and adjuvant effect in specific diseases, are needed to determine the potential significance of vitamin D in JRDs treatment.

Background: Despite numerous studies of women having children later in life, evidence of the relationship between maternal factors and newborn outcomes in Central and Eastern European countries is limited. This study aimed to examine the association between maternal age, biological determinants, including parity and sex of the newborn, demographic and social background, and birth weight in 3.8 million singleton live births in Poland. Methods: The effect of maternal age on birth weight (in grams and Z-scores) adjusted for confounders was assessed using Generalized Linear Models. Results: The mean (±SD) birth weights of neonates born to primiparous women and multiparous women were 3356.3 ± 524.9 g and 3422.7 ± 538.6 g, respectively, which corresponded to a Z-score of −0.07 ± 0.96 and 0.14 ± 1.00, respectively (p ≤ 0.001). After controlling for biological, demographic, and social factors, a significant decrease in birth weight was found for primiparous women of the age group ≥30 years and multiparous women aged ≥35 years compared to the age group of 25–29 years. The lowest neonatal birth weight was observed in the case of women aged ≥45 years. Confounders did not affect birth weight Z-scores among primiparous women, whereas among multiparous women, together with educational factors, they reversed Z-scores from positive to negative values. The lower birth weight of neonates was overall associated with lower maternal education. Conclusions: Regardless of parity, advanced maternal age is strongly associated with a decreased neonatal birth weight, implying complications in early pregnancy and the antenatal period as well as obstetric complications. Counseling to support women’s family planning decisions and improving women’s education during their reproductive age may help to alleviate unfavorable newborn outcomes.

The aim of this cross-sectional study was to assess the influence of simvastatin treatment in children with familial hypercholesterolemia (FH) on parameters of cellular immunity. Twenty-six children with FH were included, of which thirteen were treated with 10 mg simvastatin for at least 26 weeks, and thirteen were age- and sex-matched with a low-cholesterol diet only. Total WBC count and lipid profile were measured. Flow cytometry was used to identify lymphocyte subsets and determine the expression of adhesion molecules (AM) and toll-like receptors (TLRs) on leukocytes. No differences were found in the basic values of peripheral blood count and subpopulations of lymphocytes between groups. The percentage of granulocytes with the expression of AM was higher in those treated with statins. The TLR-2 expression on granulocytes and monocytes showed higher values, whereas the TLR-4 expression was lower on lymphocytes and granulocytes in simvastatin-treated children. Treatment with simvastatin in children with FH is not associated with alterations in the amounts of granulocytes and monocytes. There is no association between statin treatment and the pattern of peripheral blood lymphocyte subpopulations. The role of AM and TLRs needs further investigation, given the effect of statins on the innate immunity may be important for their efficacy and safety during growth.

The study was aimed to review a rare coexistence of type 1 diabetes (T1D) and juvenile idiopathic arthritis (JIA) regarding different clinical approaches to the management and treatment options. Medical complications of the two autoimmune disorders in children and adolescents have been evaluated, particularly in those treated with glucocorticosteroids (GCS) and insulin. A review of the literature regarding reports on concomitant T1D and JIA was conducted using resources available in Medline, Google Scholar, and Web of Science databases, with a specific focus on the combination of T1D and JIA in a pediatric population. The review was extended by our analysis of two patients treated in a single center for this comorbidity. Eligible reports of four cases were found, and including our two original records, a total of six pediatric patients (5 females) were analyzed, of which three had also other autoimmune diseases (thyroiditis, coeliac disease, autoimmune hepatitis), whereas four had been treated with a long-term GCS, and two were receiving biological therapy (etanercept or adalimumab). Only one of them had good metabolic control of diabetes. Diabetes in childhood may coexist with other autoimmune diseases, including rheumatologic conditions. Hyperglycemia can worsen JIA therapy by induction and maintaining inflammation. Using modern diabetes technologies (like personal insulin pumps and continuous glucose monitoring) helps to minimize the deteriorating effect of JIA exacerbations and the rheumatoid treatment on metabolic control of diabetes.

Juvenile idiopathic arthritis (JIA), as a chronic condition, is associated with symptoms negatively impacting health-related quality of life (HRQL). Regarding growing interest in the implementation of the patient-reported outcome measures (PROMs), we aimed to review the non-disease specific PROMs addressing HRQL assessment, potentially useful in the clinical care of JIA and daily practice. A systematic literature search was conducted using MEDLINE/PubMed, Google Scholar, Scopus and Embase databases (1990 to 2021), with a focus on the recent 5-years period. Entry keywords included the terms: “children”, “adolescents”, “JIA”, “chronic diseases”, “HRQL”, “PROMs” and wordings for the specific tools. Several available PROMs intended to measure HRQL, non-specific to JIA, were identified. The presented outcomes differed in psychometric properties, yet all were feasible in assessing HRQL in healthy children and those with chronic diseases. Both EQ-5D-Y and PedsQL have already been tested in JIA, showing relevant reliability, validity, and similar efficiency as disease-specific measurements. For PROMIS® PGH-7 and PGH-7 + 2, such validation and cross-cultural adaptation need to be performed. Considering the future directions in pediatric rheumatology, the large-scale implementation of PROMIS® PGH-7 and PGH-7 + 2 in JIA offers a particularly valuable opportunity. The PROMs reflect the patient perception of the chronic disease and allow to understand child’s opinions. The PROMs may provide an important element of the holistic medical care of patients with JIA and a standardized tool for clinical outcomes, monitoring disease severity and response to treatment.

Sarkopenia związana z podeszlym wiekiem nalezy do zjawisk inwolucyjnych polegających na ubytku masy, jakości i funkcji mieśni szkieletowych. Ze wzgledu na trendy demograficzne wydluzania zycia staje sie ona coraz powazniejszym problemem geriatrycznym. Sarkopenia stanowi skladową zespolu kruchości ( frailty syndrome ), odzwierciedlającego postepujące wyczerpanie rezerw organizmu i prowadzi — niezaleznie od innych czynnikow — do pogorszenia sprawności fizycznej, obnizenia ogolnej aktywności, ryzyka upadkow i zlaman, zwlaszcza u osob powyzej 75. roku zycia. Poniewaz kluczowymi nastepstwami sarkopenii są subiektywne odczucie oslabienia i zmeczenia, liczne ograniczenia oraz zaburzenie funkcjonowania psychospolecznego, za najwazniejszy wykladnik progresji tego schorzenia uwaza sie pomiar jakości zycia. W niniejszym opracowaniu przedstawiono nową, niedawno adaptowaną do populacji polskiej, metode oceny jakości zycia u ludzi starszych z sarkopenią, czyli kwestionariusz Sarcopenia and Quality of Life (SarQoL®), opisując rowniez proces translacji, walidacji i analizy psychometrycznej. Jest to specyficzna ankieta adresowana do osob powyzej 65. roku zycia, zawierająca 22 pytania przypisane do siedmiu domen funkcjonowania: zdrowie fizyczne i psychiczne ( Physical & Mental Health ), poruszanie sie ( Locomotion ), sklad ciala ( Body Composition ), funkcjonowanie ( Functionality ), aktywności zycia codziennego ( Activities of Daily Living ), rozrywka i spedzanie wolnego czasu ( Leisure Activities ) oraz leki ( Fears ). Metoda ta, dostepna obecnie w tlumaczeniach na 30 jezykow, pozwala wiarygodnie zbadac jakośc zycia seniorow z sarkopenią, zaś polska wersja jest spojna, powtarzalna i porownywalna z oryginalem pod wzgledem rzetelności i wlaściwości psychometrycznych. SarQoL® w polskiej wersji moze byc wykorzystywany nie tylko do badan naukowych, ale rowniez w praktyce klinicznej. Ze wzgledu na wspolistnienie lub powiązanie patogenetyczne niektorych schorzen reumatologicznych z zespolem kruchości w populacji geriatrycznej, ocena jakości zycia moze miec tu istotne znaczenie rokownicze. SarQoL® oferuje pewne obiecujące mozliwości zastosowania w monitorowaniu starszych pacjentow reumatologicznych z sarkopenią.

Searchable abstracts of presentations at key conferences on calcified tissues ISSN 2052-1219 (online)

Background: Bone metabolism disturbances are commonly observed in patients with newly diagnosed celiac disease (CD). The only available treatment for CD-the intake of a gluten-free diet (GFD)-has been found to be insufficient in effectively improving bone health in some patients. Therefore, there is an urgent need to modify the GFD so as to allow for the provision of all the necessary nutrients and improved absorption. Prebiotics intake reportedly improves the absorption of bone-related vitamin D and calcium as well as bone metabolism. The effect of prebiotic intake on bone health in CD patients has not been studied yet. This study aimed to evaluate the effect of oligofructose-enriched inulin intake on bone metabolism and immune response in children with CD on a GFD. Methods: A total of 34 children with CD were randomised into two groups receiving 10 g of oligofructose-enriched inulin (Synergy 1) or a placebo (maltodextrin) for three months, together with a strict GFD. The children's bone metabolism marker levels and cytokine profiles were analysed before and after the intervention. Results: After supplementation, the concentration of osteocalcin increased significantly in children receiving Synergy 1, while the concentration of bone alkaline phosphatase increased in both groups, independent of supplementation. After the intervention, the level of pyridinoline increased significantly in the placebo group, resulting in a concentration that was two times higher than that in the Synergy 1 group, in which it remained stable. Moreover, the plasma concentrations of N-terminal telopeptides of type I collagen decreased in both the groups, whereas the tartrate-resistant acid phosphatase 5b level increased particularly in the Synergy 1 group. The intervention did not lead to immunological response changes. Conclusions: The proposed supplementation beneficially altered bone metabolism, through increased bone formation rates and decreased bone resorption process rates. Supplementation of GFD with prebiotic oligofructose-enriched inulin may be a promising auxiliary therapy for bone metabolism improvements in children with CD.

It is reported that alterations in protein glycosylation are present in adult rheumatic diseases; however, the data related to pediatric rheumatic conditions are very scarce. The aim of this study was to assess the effect of juvenile idiopathic arthritis (JIA) on the serum glycosylation profile of transferrin isoforms. Twenty-five patients with different clinical forms of an active JIA and 22 healthy controls were studied. Serum samples were analyzed by capillary electrophoresis on MINICAP electrophoretic system (Sebia, France) to determine the levels of transferrin isoforms. In patients with JIA, tetrasialotransferrin (median 82.6%; range 68.8-99.5) concentration was lower (P = 0.032), and pentasialotransferrin (median 14%; range 0.5-31.2) was higher (P = 0.020) in comparison to controls (median 84.45; range 79.8-87.4; median 11.55; range 9.7-16.1, respectively). No significant correlations between concentration of transferrin isoforms and disease activity score (JADAS 27) or the degree of disability (VAS and CHAQ) were found. Erythrocyte sedimentation rate and CRP levels correlated positively with disialotransferrin (R = 0.493, P = 0.017; R = 0.850, P < 0.001, respectively) and pentasialotransferrin (R = 0.533, P = 0.006; R = 0.491, P = 0.045, respectively), and negatively with trisialotransferrin (R = - 0.546, P = 0.007; R = - 0.515, P = 0.049, respectively) and tetrasialotransferrin (R = - 0.436, P = 0.029; R = - 0.504, P = 0.039, respectively). This preliminary study shows the shifts in transferrin isoforms profile among patients with JIA. Our data indicate a potential clinical utility of the transferrin isoforms measurement, especially tetrasialotransferrin and pentasialotransferrin. Further prospective studies on larger groups of patients should be conducted to validate the results.

Objective Both vitamin D and K2 are involved in a number of metabolic processes, including bone metabolism; however, associations between the vitamins are not fully understood. The aim of the study was to evaluate serum concentrations of 25-hydroxyvitamin D [25(OH)D] in adult patients receiving long-term acenocoumarol (AC) treatment. Participants and methods In this cross-sectional study, 58 Caucasian patients (31 women, 27 men) with a median age of 65 years receiving long-term AC therapy were evaluated and compared with 35 age- and gender-matched healthy controls. The AC treatment was used due to recurrent venous thromboembolism (34.5%), atrial fibrillation (31%), or mechanical heart valve prostheses (34.5%). Medical records and a questionnaire were used to obtain information about chronic diseases, smoking habits, and the duration of therapy and weekly dose of AC. Anthropometric measurements were performed, and serum concentration of 25(OH)D and total alkaline phosphatase (ALP) activity were measured. Results Among the 58 patients receiving long-term AC treatment, a high proportion (46.6%) demonstrated significant vitamin D deficiency with concentrations of 25(OH)D lower than 20 ng/mL. The median concentration of 25(OH)D in subjects receiving AC was significantly lower compared to the control group [20.4 (17.4; 26.1) vs. 28.2 (24; 32.7); p < 0.001]. No differences were found between women and men receiving AC therapy. In patients receiving AC, a negative correlation was found between the concentration of 25(OH)D and the weekly dose of AC (r = −0.337, p = 0.01). Patients with concentrations of 25(OH)D < 20 ng/mL were found to have a significantly higher median dose of AC, compared to those with concentrations of 25(OH)D ≥ 20 ng/mL [21 (17; 31) vs. 17 (12; 28); p = 0.045]. Conclusion In conclusion, treatment with AC is associated with low 25-hydroxyvitamin D levels, although the path leading to this phenomenon is not entirely clear. Long-term administration of AC in adults may increase the risk of chronic vitamin D deficiency, thus, effective supplementation of vitamin D in these individuals needs careful consideration.

To evaluate the extent to which the population of Polish preadolescents is vitamin D deficient and to assess seasonal variations in vitamin D status.

Prebiotics have been shown to improve absorption of some nutrients, including vitamins. This pilot study evaluated the effect of the prebiotic oligofructose-enriched inulin (Synergy 1) on fat-soluble vitamins status, parathormone, and calcium-related elements in pediatric celiac disease (CD) patients (n = 34) on a strict gluten-free diet (GFD). Participants were randomized into a group receiving 10 g of Synergy 1 or placebo (maltodextrin) together with a GFD. At baseline and after 3 months of intervention, 25-hydroxyvitamin D [25(OH)D], parathormone, vitamin E and A, calcium, phosphate, magnesium, total protein, and albumin were determined. Concentration of 25(OH)D increased significantly (p < 0.05) by 42% in CD patients receiving Synergy 1 in GFD, whereas no change was observed in placebo. Vitamin D status reached an optimal level in 46% of patients receiving Synergy 1. No significant difference in parathormone, calcium, and phosphate levels was observed. Concentration of vitamin E increased significantly (p < 0.05) by 19% in patients receiving Synergy 1, but not in the placebo. Vitamin A levels were not changed. Supplementation of GFD with Synergy 1 improved vitamin D and vitamin E status in children and adolescents with CD and could be considered a novel complementary method of management of fat-soluble vitamins deficiency in pediatric CD patients.

Recently, SarQoL® (Sarcopenia and Quality of Life), a quality of life (QoL) questionnaire specific to sarcopenia, was successfully developed. For practical reasons, there is a great interest in validating this questionnaire in other populations. The aim of this cross-sectional study was to translate and adjust the SarQoL® into Polish and to standardize the validity of this method for the assessment of sarcopenic individuals in Poland with regard to psychometric properties. The English version was used for the translation process. A total of 106 community-dwelling Caucasian subjects aged 73.3 ± 5.94 years (65.1% females) were studied, with 60 participants being diagnosed sarcopenic. The translation and cross-cultural adaptation was carried out in five phases according to specific standard guidelines. There were no major linguistic issues in the translation process. The data confirmed a good discriminant validity, i.e., significantly lower scores for all domains (reduced global QoL in sarcopenic subjects compared to non-sarcopenic ones; 54.9 ± 16.5 vs. 63.3 ± 17.1, p = 0.013), and high internal consistency (Cronbach’s alpha coefficient was 0.92). The significant correlation of the SarQoL® scores with those of other questionnaires (SF-36v2® Health Survey and EuroQoL-5-Dimension) that are supposed to have similar dimensions indicated the consistent construct validity of the SarQoL®-PL questionnaire. No floor/ceiling effects were found. An excellent agreement was found between the test and the re-test (intraclass coefficient correlation (ICC): 0.99). The first Polish version of the SarQoL® questionnaire is valid and consistent and therefore may be used with reliability for clinical and research purposes regarding QoL assessment of sarcopenic individuals. However, further research, in particular prospective studies, is needed to determine potential limitations and the suitability of the new tool for the Polish scenario and specificity.

Searchable abstracts of presentations at key conferences on calcified tissues ISSN 2052-1219 (online)