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

Background. Osteoporosis is a metabolic disease characterized by increased bone fragility. As it is characterized as a general skeletal disease, changes can also be seen in the stomatognathic system (edentulism, wrong fitting of dentures, etc.). The question is whether early changes in the salivary mineral content and acid-base balance may reflect skeletal status and risk of bone fracture. Objectives. The objective of the study was to evaluate whether minerals in the saliva were associated with skeletal fractures in a population of postmenopausal women. Materials and methods. In this observational study, dental examinations along with the collection of saliva were conducted in 117 randomly recruited women (mean age 64.6 +/- 5.9 years). The study group included 23 study participants with fractures, of which 10 had a history of osteoporotic fractures. Saliva samples for mineral content including copper (Cu), zinc (Zn), calcium (Ca), and phosphorus (P), as well as salivary pH were collected and analyzed to determine associations between salivary mineral content and fracture risk. Results. As a result, the median pH value was 6.8, and the median levels for Cu (0.35 mu mol/L), Zn (0.61 mu mol/L), Ca (0.7 mmol/L), and P (6.64 mmol/L) were observed. No differences were noted in salivary mineral content and acid-basic balance between the fractured and non-fractured participants. Conclusions. The results of our study suggest that salivary mineral content has limited usability in predicting skeletal fragility in postmenopausal women when used alone.

BackgroundAge-related macular degeneration is the primary cause of irreversible blindness in developed countries, whereas the global prevalence of osteoporosis-a major public health problem-is 19.7%. Both diseases may coincide in populations aged >50 years, leading to serious health deterioration and decreased quality of life.ObjectivesThis study aimed to analyze the relationship between age-related macular degeneration and osteopenia, defined as decreased bone mineral density, in the Polish population.MethodsParticipants were derived from the population-based Bialystok PLUS Study. Randomized individuals were stratified into two groups, those with age-related macular degeneration (AMD-1 group) or without age-related macular degeneration (AMD-0 group). Using a cutoff value of -1.0 to identify low bone mass, participants with femoral bone mineral density T-scores above -1.0 were assigned to the normal reference, and those with T-scores below -1.0 were assigned to the osteopenia category. Among 436 Caucasian participants aged 50-80 years (252 women, 184 men), the prevalence of age-related macular degeneration was 9.9% in women and 12.0% in men. Decreased bone mineral density based on T-scores was observed in 36.9% of women and in 18.9% of men. Significant differences in femoral bone mineral density between the AMD-0 and AMD-1 groups were detected only in men (mean difference [95% confidence interval] = 0.11 (0.02; 0.13); p = 0.012 for femoral bone mineral density, and 0.73 [0.015; 0.94]; p = 0.011 for the femoral T-score). No associations were observed between bone mineral density and age-related macular degeneration in women.ConclusionDecreased femoral bone mineral density may be associated with a higher risk of age-related macular degeneration in men, but a causal link remains unclear.

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.

Guidelines to provide an update of the previously published Polish recommendations for the management of women and men with osteoporosis have been developed in line with advances in medical knowledge, evidence-based data, and new concepts in diagnostic and therapeutic strategies. A Working Group of experts from the Multidisciplinary Osteoporosis Forum and from the National Institute of Geriatrics, Rheumatology, and Rehabilitation in Warsaw performed a thorough comprehensive review of current relevant publications in the field (including all age groups of people and management of secondary osteoporosis), and they evaluated epidemiological data on osteoporosis in Poland and the existing standards of care and costs. A voting panel of all co-authors assessed and discussed the quality of evidence to formulate 29 specific recommendations and voted independently the strength of each recommendation. This updated practice guidance highlights a new algorithm of the diagnostic and therapeutic procedures for individuals at high and very high fracture risk and presents a spectrum of general management and the use of medication including anabolic therapy. Furthermore, the paper discusses the strategy of primary and secondary fracture prevention, detection of fragility fractures in the population, and points to vital elements for improving management of osteoporosis in Poland.

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.

Small for gestational age (SGA) births are a significant clinical and public health issue. The objective of this review was to summarize maternal biological and socio-demographic factors and preventive strategies used to reduce the risk of SGA births. A literature search encompassing data from the last 15 years was conducted using electronic databases MEDLINE/PubMed, Google Scholar and Scopus to review risk factors and preventive strategies for SGA. Current evidence shows that primiparity, previous stillbirths, maternal age ≤24 and ≥35 years, single motherhood, low socio-economic status, smoking and cannabis use during pregnancy confer a significant risk of SGA births. Studies on alcohol consumption during pregnancy and SGA birth weight are inconclusive. Beneficial and preventive factors include the “Mediterranean diet” and dietary intake of vegetables. Periconceptional folic acid supplementation, maternal 25-hydroxyvitamin D, zinc and iron levels are partly associated with birth weight. No significant associations between COVID-19 vaccinations and birthweight are reported. A midwifery-led model based on early and extensive prenatal care reduces the risk of SGA births in women with low socio-economic status. Major preventive measures relate to the awareness of modifiable and non-modifiable risk factors of SGA, leading to changes in parents’ lifestyles. These data support that education, monitoring during pregnancy, and implementing preventive strategies are as important as biological determinants in risk reduction of SGA births.

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.

Abstract Background Juvenile dermatomyositis is a systemic, autoimmune inflammatory muscle disorder and vasculopathy. While generally it is well-responsive to the standard line of therapy, in rare cases may develop life-threatening complications and show resistance to treatment. We presented the case of a 7-year-old, who developed rapidly progressive interstitial lung disease and pancreatic dysfunction in the course of fulminant juvenile dermatomyositis with a good outcome after aggressive immunosuppressive therapy with the use of rituximab. Our case illustrates the diagnostic challenges of this condition in the setting of non-specific clinical manifestations and a need for novel molecular mechanism-based treatment to improve the survival of refractory dermatomyositis. Case presentation A 7-year-old girl presented with erythematous rash, muscle weakness, pain of lower extremities and elevated body temperature. The patient was diagnosed with juvenile dermatomyositis through physical examination (typical skin manifestation, proximal muscle weakness) and based on the finding of elevated levels of serum muscle enzymes and myopathic EMG changes. In the course of the disease she developed interstitial lung disease rapidly progressing to respiratory dysfunction and pancreatic failure. She received combined therapy, including methylprednisolone pulse therapy, followed by intravenous immunoglobulin, both in maximum doses per kilogram. On the 20th day of hospitalization, she was admitted to the intensive care unit and placed on ventilation support. On the 11th day of assisted ventilation rituximab was administered. Because of pancreatic dysfunction, complicating the course of the disease, the child was placed on total parenteral nutrition. After 3 doses of rituximab her general state and respiratory condition showed gradual improvements. Eventually, after 23 days of ventilation support, the patient could be successfully extubated. When the enteral nutrition was re-established and the underlying disease reached the stage of satisfactory improvement she was discharged to an out-patient setting. Conclusions In juvenile dermatomyositis, rituximab may be a promising agent for treatment of rapidly progressive interstitial lung disease. It may also play a role in the therapy of simultaneous pancreatic dysfunction. In the future, the efficacy of rituximab in the refractory form of the disease should be further investigated.

Over the past few years, there have been significant advances in our understanding of hypoparathyroidism (HypoPT) in terms of its epidemiology, clinical presentation, etiology, and skeletal and renal complications. Moreover, the available treatment options for Hy- poPT have changed. This position statement of the Expert Group of the Polish Society of Endocrinology summarizes the current state of knowledge and provides recommendations for optimal management to assist clinicians in the diagnosis, treatment, and monitoring of HypoPT in Poland. The specific aspects of HypoPT management in children, pregnant and lactating women, and patients with chronic kidney disease are also discussed.HypoPT is a rare disorder characterized by hypocalcemia and the lack or deficiency of parathyroid hormone (PTH). Hypoparathyroidism can be associated with complications, including nephrocalcinosis, nephrolithiasis, renal insufficiency, cataract, seizures, cardiac arrhythmia, depression, and an increased risk of infection. Minimizing complications of HypoPT requires careful evaluation and close monitoring of laboratory parameters. Conventional management of HypoPT has focused on maintaining serum calcium levels using oral calcium and active vitamin D. However, this approach is limited because it does not restore normal PTH function, is often associated with in- adequate biochemical control, and raises concerns as to long-term side effects. HypoPT is the only classic endocrine insufficiency that is not commonly treated with the substitution of the missing hormone. Recently, recombinant human PTH(1-84) has become available, offering hope that the use of the missing hormone in the treatment of HypoPT will help achieve better control and reduce the risk of complications. However, this treatment is currently unavailable in Poland.

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.

Background: Vitamin D deficiency is reported in rheumatological diseases in adults. The aim was to evaluate the prevalence of vitamin D deficiency in children with juvenile idiopathic arthritis (JIA) and to investigate potential correlations between vitamin D status and clinical factors, laboratory traits, and medical treatment, including methotrexate (MTX) and glucocorticoids (GCs). Methods: In 189 patients aged 3–17.7 years, with JIA in the stable stage of the disease, anthropometry, clinical status, serum 25-hydroxyvitamin D [25(OH)D], calcium (Ca), phosphate (PO4), total alkaline phosphatase (ALP), erythrocyte sedimentation rate (ESR), and C-reactive protein (CRP) were assessed. Results: Median 25(OH)D level was 15.00 ng/mL, interquartile range (IQR) 12.00 ng/mL. Vitamin D deficiency was found in 67.2% and was independent of sex, disease manifestation, and CRP, ESR, ALP, or PO4 levels. Higher doses of MTX corresponded with lower 25(OH)D levels using both univariate and multivariate models (p < 0.05). No such trend was found for GCs treatment. Serum Ca was lower in patients treated with GCs (p = 0.004), MTX (p = 0.03), and combined GCs/MTX (p = 0.034). Conclusions: JIA patients are vitamin D depleted independently of disease activity or inflammatory markers. MTX therapy may be an iatrogenic factor leading to inadequate 25(OH)D levels. Vitamin D supplementation should be considered in all children with JIA, particularly those receiving long-term MTX therapy.

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.

Low bone mineral density (BMD) was diagnosed in 24% of childhood cancer survivors (CCS), whereas very low BMD was relatively uncommon at 8%. We suggest that low BMD in CCS may become alleviated over time. Stem cell transplantation, radiotherapy, and underweight were the strongest independent predictors of decreased BMD. Childhood cancer survivors (CCS) are at risk of premature bone loss, although published studies are inconsistent. The objective of this study was to evaluate the prevalence and pattern of low bone mineral density (BMD) in short- and long-term CCS, and to determine clinical factors affecting skeleton after anticancer treatment. This retrospective study was conducted in a cohort of 326 children and young adult CCS (147 females) who completed anticancer treatment. BMD was determined by dual-energy X-ray absorptiometry (DXA). Low BMD was defined as a Z-score ≤ − 1.0, and the very low BMD as a Z-score ≤ − 2.0. Additionally, the changes in BMD over time were studied in 123 CCS who had been re-examined by DXA during follow-up. Median age at diagnosis was 7.27 years (range, 4.4–10.6); median time between end of treatment and DXA was 6.12 (range, 4.0–22.0). Low BMD was found in 24% of CCS, while very low BMD was relatively uncommon (8%). Based on multivariate analysis, the following were significantly associated with low BMD at the follow-up: hematopoietic stem cell transplantation (OR 3.13, 95% CI 1.02–9.63), head and neck radiotherapy (OR 2.54, 95% CI 1.32–4.90), and body weight below the standard reference (OR 3.57, 95% CI 1.24–10.23). The time-related trajectory showed an improvement (BMDLS) or stabilization (BMDTB) in Z-scores values. These data based on serial DXA measurements, encompassing a long-lasting observation period, show that CCS may not be at risk of premature bone loss in young adulthood. However, it is unknown how the scenario for skeletal mass is until the CCS will achieve older or postmenopausal age.

Background Information about trends in perinatal and child health inequalities is scarce, especially in the Eastern Europe. We analyzed how mortality under 1 year of age has been changing in the Baltic States and the European Union (EU) over 25 years, and what associations occurred between changes in macroeconomic factors and mortality. Methods Data on fetal, neonatal, infant mortality, and macroeconomic factors were extracted from WHO database. Joinpoint regression analysis was performed to analyze time trajectories of mortality over 1990–2014. We also investigated how the changes in health expenditures and Gross Domestic Product (GDP) contributed to the changes in mortality. Results The reduction of fetal, neonatal and infant mortality in the Baltic countries led to convergence with the EU. In Estonia this process was the fastest, and then the rates tended to diverge. The strongest effect in reduction of neonatal mortality was related to the annual increase in health expenditure and GDP which had occurred in the same year, and a decrease in fetal mortality associated with an increase in health expenditure and GDP in the 4th and 5th year, respectively, following the initial change. Conclusions These findings outlined convergences and divergences in mortality under 1 year of age in the Baltic States compared with the patterns of the EU. Our data highlighted a need to define health policy directions aimed at the implementation of effective intervention modalities addressing reduction of risks in prenatal and early life.

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.