Search Results (146)

Since severe acute respiratory syndrome coronavirus 2 (SARS-CoV-2) was first reported in Wuhan, China, in December 2019, it has evolved, leading to variants that differ in their transmissibility, severity of disease, and susceptibility to therapy. Our goal was to describe the dynamics of the emergence of SARS-CoV-2 variants among the population of the southern part of Poland (Silesia) in the period from September 2021 to August 2022. Our results showed that, like in the rest of Poland or in neighboring countries (Czech Republic, Slovakia), Delta was replaced by the Omicron BA.1 variant, isolated for the first time in December 2021, and subsequently Omicron BA.2 and its derivative subvariants acquiring further mutations. Finally, in August 2022, only the BA.5.2.26 subvariant was present in Silesia. However, we noted differences in the dynamics of emergence and spread of some Omicron subvariants compared to the rest of Poland and the neighboring countries, which may be due to differences in population density or import of the virus from other regions. Full article

Background: Post-COVID syndrome is characterized by persistent symptoms such as dyspnea, fatigue, and reduced exercise tolerance, which can significantly impair pulmonary function and quality of life. Pulmonary rehabilitation has been proposed as a potential intervention to address these challenges. This study aimed to evaluate the effects of a pulmonary rehabilitation program on pulmonary function, inflammatory markers, and quality of life in patients with post-COVID syndrome. Methods: A prospective, interventional, non-randomized clinical trial was conducted involving 77 participants (mean age 59.4 ± 11.6 years; 39% female) who attended a post-COVID care clinic in Rzeszów, Poland. The intervention included supervised respiratory and aerobic exercises, muscle strengthening, and body balance therapy, alongside motivational breathing therapy. Pulmonary function (spirometry, plethysmography, gasometry), inflammatory markers (CRP, WBC, D-dimer), and quality of life (WHOQOL-BREF) were assessed pre- and post-intervention. Results: Significant improvements were observed in pulmonary function parameters post-rehabilitation, including increases in forced vital capacity (FVC, 75% to 78.4%, p < 0.001), forced expiratory volume in one second (FEV1, 78.2% to 80.5%, p < 0.001), and total lung capacity (TLC, 67.3% to 71%, p < 0.001). The diffusing capacity for carbon monoxide (DLCO) improved by 6.2% (p < 0.001). Arterial oxygen pressure (PaO₂) increased by 7.6 mmHg (p < 0.001). Markers of inflammation, including CRP (8.9 to 1.3 mg/dL, p < 0.001) and d-dimer (1722.2 to 203.4 ng/mL, p < 0.001), showed significant reductions. Quality of life improved across physical, psychological, and environmental domains. Sex, BMI, and baseline inflammatory markers were significant determinants of rehabilitation outcomes. Conclusions: A pulmonary rehabilitation program significantly improved pulmonary function, reduced systemic inflammation, and enhanced quality of life in individuals with post-COVID syndrome. The findings highlight the importance of tailored rehabilitation in mitigating long-term post-COVID sequelae. Future research should explore the long-term effects of rehabilitation and its applicability in diverse populations. Full article

Background: Food neophobia, defined as reluctance to try new foods, may lead to nutritional deficiencies and complicate dietary management—especially in individuals with Down syndrome, who often present with oral-motor dysfunction. This condition may result in nutritional deficiencies and difficulties in adhering to dietary recommendations, particularly in individuals with comorbidities. In individuals with Down syndrome (DS), who frequently present with oral motor disorders and chronic diseases, the problem may be especially pronounced. Objectives: The aim of the study was to assess the risk of food neophobia and feeding difficulties in children, adolescents, and young adults with Down syndrome, as well as their associations with age, gender, and body weight. Methods: The research was conducted using the CAWI method among 310 caregivers of individuals with DS in Poland. Two validated tools were employed: the Montreal Children’s Hospital Feeding Scale (MCH-FS) and the Food Neophobia Scale for Children (FNSC). Body mass index (BMI), comorbidities, and demographic data were also analyzed. Results: Findings revealed that the majority of participants (55.2%) had normal body weight, while 19.4% were undernourished and 6.5% were classified as obese. Feeding difficulties of moderate to very high severity were reported in 26.5% of the participants. A high risk of food neophobia was identified in 41.3% of respondents, most frequently in the preschool age group. A statistically significant association was observed between age and the severity of both feeding difficulties and neophobia (p < 0.05). However, no significant relationships were found with gender or body weight. Conclusions: Feeding difficulties and food neophobia are prevalent among individuals with Down syndrome, particularly in preschool-aged children. The findings highlight the necessity of an interdisciplinary therapeutic approach and the individualization of dietary interventions, taking developmental age into account. Further studies are warranted, with consideration of environmental and psychosocial factors. Full article

Introduction: Among aortic diseases in children, congenital defects such as coarctation of the aorta (CoA), interrupted aortic arch (IAA), hypoplastic aortic arch (HAA), and hypoplastic left heart syndrome (HLHS) predominate. Tissue patches are applied in pediatric cardiovascular surgery for the repair of congenital aortic defects as a filling material to replenish missing tissue or as a substitute material for the complete reconstruction of the vascular wall along the course of the vessel. This retrospective single-center study aimed to present the safety and feasibility of extracellular matrix (ECM) biological scaffolds in pediatric aortic surgery. Patients and methods: There were 26 patients (17 newborns and nine children), who underwent surgical procedures in the Department of Pediatric Cardiac Surgery (Poznań, Poland) between 2023 and 2024. The patients’ population was divided into two subgroups according to the hemodynamic nature of the primary diagnosis of the congenital heart defect and the performed pediatric cardiovascular surgery. The first group included 18 (72%) patients after aortic arch repair for interrupted aortic arch and/or hypoplastic aortic arch, while the second group included seven (28%) patients after aortopulmonary anastomosis. In the first group, patches were used to reconstruct the aortic arch by forming an artificial arch with three separate patches sewn together, primarily addressing the hypoplastic or interrupted segments. In the second group, patches were applied to augment the anastomosis site between the pulmonary trunk and the aortic arch, specifically at the connection points in procedures, such as the Damus–Kaye–Stansel or Norwood procedures. The analysis was based on data acquired from the national cardiac surgery registry. Results: The overall mortality in the presented group was 15%. All procedures were performed using median sternotomy with a cardiopulmonary bypass. The cardiopulmonary bypass (CPB) and aortic cross-clamp (AoX) median times were 144 (107–176) and 53 (33–79) min, respectively. There were two (8%) cases performed in deep hypothermic circulatory arrest (DHCA). The median postoperative stay in the intensive care unit (ICU) was 284 (208–542) h. The median mechanical ventilation time was 226 (103–344) h, including 31% requiring prolonged mechanical ventilation support. Postoperative acute kidney failure requiring hemodiafiltration (HDF) was noticed in 12% of cases. Follow-up data, collected via routine transthoracic echocardiography (TTE) and clinical assessments over a median of 418 (242.3–596.3) days, showed no evidence of patch-related complications such as restenosis, aneurysmal dilation, or calcification in surviving patients. One patient required reintervention on the same day due to a significantly narrow ascending aorta, unrelated to patch failure. No histological data from explanted patches were available, as no patches were removed during the study period. The median (Q1–Q3) hospitalization time was 21 (16–43) days. Conclusions: ProxiCor^® biological patches derived from the extracellular matrix can be safely used in pediatric patients with congenital aortic arch disease. Long-term follow-up is necessary to confirm the durability and growth potential of these patches, particularly regarding their resistance to calcification and dilation. Full article

Objectives: The cultural and linguistic adaptation of the Boston Carpal Tunnel Questionnaire (BCTQ) to Polish and the assessment of its psychometric properties among patients undergoing extracorporeal shock wave therapy (ESWT). Methods: This was a cross-sectional study with repeated measures during retest examinations. Subjects from an outpatient rehabilitation center in Poland (n = 103) with mild to moderate carpal tunnel syndrome (CTS) were evaluated three times. Test 1 and test 3 (after a series of four treatments using EWST) included the following: completing the BCTQ, QuickDASH, and SF-36 questionnaires, the VAS pain scale, performing the Tinel–Hoffmann and Phalen tests, and an assessment of grip strength. Test 2 (test–retest BCTQ) was performed two to seven days after test 1. Results: The Polish version of the BCTQ demonstrated a high internal consistency, with a Cronbach’s alpha of 0.861 for the Symptom Severity Scale (SSS) and 0.924 for the Functional Status Scale (FSS). It also showed excellent test–retest reliability, with Intraclass Correlation Coefficients (ICCs) of 0.941 for the SSS and 0.925 for the FSS. The Standard Error of Measurement (SEM) was 0.16 for the SSS and 0.21 for the FSS, while the Minimal Detectable Change (MDC) was 0.43 and 0.59, respectively. It has a high construct validity as 80% of the a priori adopted hypotheses were confirmed. The mean decrease after ESWT on the SSS was 1.04 points, and on the FSS was 0.77 points. The ES value for the SSS scale was 1.62 and for FSS 0.99, and the SRM was 1.35 for the SSS, and 1.01 for the FSS, which proves a higher sensitivity to changes in the BCTQ-PL. Conclusions: The BCTQ-PL is a valid and reliable tool for assessing CTS-related symptoms and functional status in Polish-speaking patients. Full article

Background and Objectives: Hemophagocytic lymphohistiocytosis (HLH) is a rare hyperinflammatory condition characterized by excessive activation of cytotoxic lymphocytes and macrophages, resulting in a cytokine storm, multiorgan damage, and high mortality. HLH is classified into primary (genetic) and secondary (acquired) forms, with diagnosis often challenging due to nonspecific symptoms. Macrophage activation syndrome (MAS) refers to the secondary HLH triggered by rheumatic diseases. In this study, we retrospectively analyzed the clinical and laboratory features of patients with secondary HLH to enhance understanding of this life-threatening condition and summarize emerging management strategies. Materials and Methods: This single-center retrospective study analyzed medical records of patients hospitalized with HLH at the University Hospital in Kraków, Poland, from 2013 to 2024, based on HLH-2009 criteria and HScore > 169 points. Diagnostic criteria included clinical, laboratory, and histological findings, e.g., hemophagocytosis in bone marrow, circulating cytopenia, and elevated ferritin levels. Results: A total of 21 patients met the criteria for HLH diagnosis, with a median age of 35 (range: 19–67) years, including 12 women (57.1%). The median HScore among the patients was 244 (range: 208–304) points. Fever was the most common presenting symptom, occurring in all cases. High ferritin, hypertriglyceridemia, and hypofibrinogenemia in peripheral blood were also prevalent. Bone marrow hemophagocytosis was confirmed in 66.7% of cases (n = 12/18 of available data). Regarding immunosuppressive therapy, glucocorticosteroids were the most frequently used (used in all cases). Four (19.0%) patients died during HLH (cases triggered by lymphoma [twice], Epstein–Barr virus infection, unknown reason). Compared to survivors, these patients had lower counts of white blood cells, neutrophils, and lymphocytes at diagnosis (p < 0.05 for all). Conclusions: Secondary HLH is a severe syndrome requiring rapid diagnosis and timely intervention to improve patient outcomes. Lower white blood cell, neutrophil, and lymphocyte counts present worse prognostic factors. Full article

Poor air quality within hospitals can contribute to a range of health issues, collectively known as sick hospital syndrome, encompassing respiratory, skin, and nonspecific symptoms. Aspergillus poses a significant risk of severe respiratory infections, particularly within intensive care unit (ICU) patients often with compromised immune systems. This study was conducted in the intensive care units of three selected hospitals in Cracow, Poland. Air samples were obtained using the single-stage impactor MAS-100 NT Ex (Merck). The air samples were taken from each ward, seasonally, four times a year. Sampling of intensive care units’ air for Aspergillus revealed the significant diversity in fungal concentration and unveiled seasonal fluctuations in culturable fungi levels. The highest concentration of Aspergillus species complexes was detected during autumn, while the lowest was in spring. The disturbing occurrence of Aspergillus in hospitals’ air emphasizes the importance of monitoring fungal air bioburden and assessing air control efficiency and highlights the urgent need to develop and validate microbiological standards for fungal air contamination in hospitals. Full article

Background: The vaccination programmes after the 2nd World War and the application of safe and effective vaccines were expected to eliminate infectious diseases within the World Health Organization (WHO) European Region. However, epidemiological indexes show isolated cases and local epidemiology outbreaks of viral measles, that draw attention to re-evaluate the effectiveness and obstacles of infectious disease eradication programmes. Methodology: This study analyses the available Polish governmental epidemiological data concerning the WHO European Region for the years 2003–2014 and evaluates the effectiveness of the WHO Measles Eradication Programme implementation, since 2001. Most of these epidemiological data are either available in Polish or scattered in governmental reports only. Thus, we have compiled selected available data to present an overview focusing on Poland’s measles epidemiological situation. Results: The analysis of the available data clearly shows that infection cases of measles are on the rise in the European Region or are steady at a relatively high level depending on the country. The critical factors to prevent measles are maintaining a vaccination level at a minimum of 95% using two doses of MMR, adequate infection detection, rapid reporting, controlling and enforcing identification, and mass media campaigns to inform societies about the necessity of measles vaccination and the safety of using MMR. Conclusions: Besides the current SARS-CoV-2 pandemics, measles is possibly the most dominating infectious disease on the rise in the European Region, including Poland. The eradication programme modifications to increase its effectiveness are of upmost importance, as measles is one of the most infectious diseases with acute syndromes, especially affecting children. Full article

Background: The purpose of this study was to assess bone densitometry parameters in patients with classical and hypermobile Ehlers-Danlos syndrome (EDS) and to determine whether the hypermobile subtype increases the risk of low bone mass, which is particularly important in this patient group, since the genetic mutation responsible for this subtype is still unknown. Material and Methods: In order to conduct this study, we collaborated with the EDS society in Poland. A total of 30 females of reproductive age who were included in the study were divided into two groups: Group 1—those with classical EDS (n = 9) and Group 2—those with hypermobile EDS (n = 21). Routine laboratory test results, bone turnover markers, and densitometry parameters were evaluated and compared. Results: The study groups showed no differences in terms of densitometry parameters or markers of calcium-phosphate metabolism. A multivariate logistic analysis demonstrated no increase in the risk of low bone mass (defined as a Z-score lower than −2) in patients with hypermobile EDS (OR 0.067 [95% Cl 0.0–20.927]; p = 0.356). Conclusions: The hypermobile subtype of EDS does not increase the risk of low bone mass; there were no significant differences between patients with hypermobile EDS and those with classical EDS in terms of either densitometry parameters or markers of calcium-phosphate metabolism. Although patients with hypermobile EDS are not at a higher risk of developing low bone mineral density, they should be regularly monitored for any calcium and phosphate metabolism abnormalities. Full article

Background/Objectives: DDX3X syndrome is often misdiagnosed as autism spectrum disorder (ASD, Rett Syndrome, and Dandy–Walker Syndrome). Precise phenotyping is needed with reference to neurodevelopmental diagnosis. Observation of behavior and communication in parents with DDX3X syndrome in the USA, France, and Poland; conversations with the parents of patients; and rudimentary information in evidence-based medical articles prompted us to identify differences in communication, play, and social interaction between children with ASD only, those with both ASD and DDX3X, and those with DDX3X only. Methods: As diagnostic tool for DDX3X patients, we created a questionnaire divided into four sections: medical, social, play, and communication. Results: The results showed inconsistent diagnoses in different countries where children could have been diagnosed with DDX3X. In a comparative analysis, individuals with DDX3X exhibited greater social skills than individuals with ASD. Furthermore, those with DDX3X demonstrated higher levels of social functioning compared to children with ASD. Therefore, parents of children recently diagnosed with ASD or similar conditions are encouraged to complete a survey to determine if their child is likely to have features of DDX3X syndrome. Conclusion: Identification of early behavioral markers that differentiate children with ASD and those with DDX3X could lead to the earliest opportunity for identification and intervention, and can significantly impact developmental trajectories, leading to better long-term outcomes. Full article

Background: WFS1-spectrum disorders are caused by a mutation in the WFS1 gene. The term includes a wide range of rare disorders, from the most severe Wolfram syndrome with autosomal recessive inheritance to milder clinical manifestations with a single causative variant in the WFS1 gene, such as Wolfram-like syndrome, low-frequency sensorineural hearing loss (LFSNHL), isolated diabetes mellitus (DM), nonsyndromic optic atrophy (OA), and isolated congenital cataracts. Methods: The aim of this study was to evaluate genotype–phenotype correlations in Polish patients with WFS1-spectrum disorders. The study group constituted 22 patients (10 F; 12 M), including 10 patients (3 F; 7 M) referred to the Outpatient Clinic for Rare Diseases in Children and Adolescents and Diabetogenetics between 2019 and 2024 with clinical symptoms suggestive of WFS1-spectrum disorders, and 12 of their first-degree relatives (7 F; 5 M) from 10 families in Poland. Molecular testing was performed using tNGS (Targeted Next Generation Sequencing; Illumina) and analyzed for variants in the WFS1 gene. Results: Thirteen different variants in the WFS1 gene were found in 22 individuals (10 patients and family members), including the identification of two new variants (c.1535T>C and c.2485C>G). All patients had hyperglycemia or DM, hearing impairment, OA, or a combination of these symptoms. Four patients in the study group were diagnosed with Wolfram syndrome and all were compound heterozygotes for variants in the WFS1 gene. Conclusions: The evaluation of molecular characteristics in combination with clinical symptoms broadens the understanding of WFS1-spectrum disorders and allows more accurate management and prognosis for patients with this diagnosis. Full article

It is assumed that climate change (global warming) worsens the living conditions for conifers and at the same time favours the cultivation of deciduous trees, including oaks. In fact, in Poland, for example, many more oaks are now being planted as forest-forming tree species than in the 1980s and 1990s. However, the monitoring of the health status of European forests (according to the International Co-operation Project) does not confirm these optimistic assumptions, and oak has been cited as one of the most damaged tree species in terms of defoliation in recent decades. The prospects for oak cultivation in European forestry are therefore a combination of abiotic conditions and biotic damage factors. This review article focuses in particular on the new threats posed by pathogenic organisms causing emerging diseases. These include newly identified bacteria responsible for the so-called Acute Oak Decline (AOD), oomycetes (especially those specialised in damaging fine roots, such as Phytophthora quercina T.Jung) and semi-parasites of the genus Loranthus. At the same time, the pressure from commonly observed insects and fungi described in connection with the complex syndrome of oak decline, which is divided into predisposing, inciting, and contributing factors (according to Manion’s disease spiral), has not abated. Therefore, international, interdisciplinary research (such as that proposed in Oakland) is needed, using modern technologies (RS remote sensing) based on the comparison of satellite images (from different years), not only to inventory the most valuable oak stands in Europe (microrefugia) but also to identify trends in changes in their condition and biodiversity. As RS has its limitations (e.g., resolution), aerial monitoring should be complemented by quantitative and qualitative inventory from the ground, e.g., monitoring of the presence of soil microorganisms using effective molecular biological methods (e.g., Next-Generation Sequencing NGS). Full article

Objectives: This study aims to evaluate the prevalence of premenstrual syndrome (PMS) among Polish adolescents and explore its associations with mental health outcomes, lifestyle factors, and risk behaviours. Additionally, it seeks to examine the impact of PMS on quality of life, contributing to the foundation for initiatives that enhance adolescent menstrual health. Methods: This research is part of the POLKA 18 study, a youth-led cross-sectional survey conducted between April and December 2019. Final-year high school students were surveyed using paper-based, self-reported questionnaires. Statistical analysis was performed using the R programming language in RStudio, with a significance threshold of p-value < 0.05. Results: A total of 1545 valid responses were analysed. PMS was reported by 33.9% of respondents, with over 80% experiencing premenstrual symptoms. Adolescents with negative mental health outcomes, such as self-harm or suicidal ideation, were significantly more likely to report PMS (p ≤ 0.001). PMS was also strongly associated with anxiety and panic attacks (p < 0.001). Risk behaviours, including smoking (p = 0.006), illicit substance use (p < 0.01), and increased alcohol consumption in the past 30 days (p < 0.001), were more prevalent among adolescents with PMS. Furthermore, respondents with PMS reported poorer school performance (p = 0.002), higher stress levels (p < 0.001), and a more negative perception of their overall health (p < 0.001) compared to peers without PMS. Conclusions: PMS is significantly associated with mental health issues, engagement in risky behaviours, higher stress, and diminished self-perceived health and academic performance. These findings highlight the need for further research and the development of targeted interventions to improve adolescent menstrual health. Full article

Background: Women with an aplastic uterus (ESHRE/ESGE classification) or Müllerian agenesis (ASRM MAC 2021) might present with functional uterine remnants. Our study aimed to report the clinical course of symptomatic uterine rudiments in adolescents and adults with Mayer–Rokitansky–Küster–Hauser syndrome (MRKHS). Methods: This study involved 20 patients with MRKHS who, between 2012 and 2023, underwent surgery for symptomatic uterine horns at the mean age of 25.2 years in the Division of Gynaecology, Gynaecological and Obstetric Clinical Hospital, Poznan University of Medical Sciences, Poland. The records of the patients were retrospectively analysed. Results: The volume of the uterine horns ranged from 0.8 to 58.3 cm³, and the volume of the endometrial cavity within the horns ranged from 0.03 to 12 cm³, with no significant difference between adolescents and adults (p = 0.36). In five patients (25%), MRKHS was identified during the diagnosis of recurrent pelvic pain at the age of 12.6–14.8 years. In 19 patients, uterine rudiments were removed: unilaterally in 3 patients (16%), bilaterally in 16 patients (84%), and laparoscopically in 89% of cases. In one patient, the horn was preserved (horno-neovaginal anastomosis). Histopathology confirmed the presence of the endometrium in uterine rudiments ipsilateral to the pain location in 75% of cases. Four patients (20%) were diagnosed with endometriosis. Conclusions: Recurrent pelvic pain in patients with MRKHS should prompt the diagnosis of functional uterine rudiments. The resection of symptomatic uterine horns can result in the complete resolution of pain. Patients with endometriosis are at risk of pain recurrence. In some patients strongly desirous of menstruation, horno-neovaginal anastomosis can be cautiously attempted. Full article

Background/Objectives: Hemolytic uremic syndrome (HUS) is a known cause of acute kidney injury in children, but there are few recent reports on its epidemiology and outcome. We aimed to investigate trends in the incidence and the long-term outcomes of both Shiga toxin-producing Escherichia coli -HUS (STEC-HUS) and atypical HUS (aHUS) in Poland over the last 12 years (2012—2023), based on the Polish Pediatric HUS and Pediatric Renal Replacement Therapy (RRT) Registries. Methods: A total of 436 patients (301 with STEC-HUS and 135 with aHUS) were included. Results: The incidence of STEC-HUS increased during the observation period, with a mean of 3.9 cases per million age-related population (marp). The incidence of aHUS was relatively constant with a mean of 1.8/marp. The majority of patients fully recovered, although kidney sequelae were observed at 5-year follow-ups in 31% of children with STEC-HUS, 57% of aHUS subjects in the pre-eculizumab era, and 37% of aHUS subjects who had received eculizumab. The overall mortality rate was 2% for STEC-HUS and 3.7% for aHUS, with no deaths reported in children on eculizumab and mortality mainly attributed to neurological damage. A decreasing incidence of chronic kidney disease stage 5 (CKD5) due to HUS was observed. Conclusions: Despite an unchanging incidence of aHUS and an increasing incidence of STEC-HUS, the kidney outcomes of both diseases have improved significantly over the last 12 years. Mortality from HUS has dropped due to improved symptomatic treatment and the introduction of anti-C5 therapy. The development of CKD5 in childhood as a consequence of HUS has become exceptional. Full article