Analysis of seroconversion following COVID-19 vaccination among multiple sclerosis patients treated with disease-modifying therapies in Poland.

CLINICAL RATIONALE FOR THE STUDY: The rapid spread of SARS-CoV-2 throughout the world has highlighted the importance of vaccinations to control the pandemic and to protect people at risk for severe disease courses. Disease-modifying therapies (DMT) in multiple sclerosis (MS), whether immunomodulatory or immunosuppressive, may affect the immune response. Therefore, the question arose as to whether these vaccinations would be effective. AIM OF THE STUDY: We planned a study to assess the immune response to SARS-CoV-2 vaccines by type of therapy. MATERIAL AND METHODS: Participants were recruited from 14 Polish MS centres. The data was obtained by neurologists using a questionnaire. We collected data on 353 MS patients (269 females, 84 males) who received complete primary SARS-CoV-2 vaccination. All persons with MS (PwMS) were treated with disease-modifying therapies. RESULTS: 305 out of 353 PwMS (86.4%) were positive for IgG Abs against SARS-CoV-2 S domain S1 Ag after vaccination. A strong immune response was noted in 129 PwMS (36.5%). The rate of seroconversion after SARS-CoV-2 vaccination in PwMS who received immunomodulatory DMTs (interferon beta, glatiramer acetate, teriflunomide, dimethyl fumarate, natalizumab) was 91.5%, in PwMS receiving immune reconstruction therapy (alemtuzumab, cladribine) was 92%, and in immunosuppressive DMTs (fingolimod, ocrelizumab), the seroconversion rate was 59%. CONCLUSIONS AND CLINICAL IMPLICATIONS: Our study shows that, in PwMS receiving immunomodulatory therapy, the immune response to vaccination is generally excellent. Even in immunosuppressive patients, seroconversion is satisfactory.

Plasma and Peritoneal Fluid Annexin A2 Levels in Patients with Endometriosis.

Introduction: Endometriosis is an inflammatory-related reproductive age disease characterized by the presence of endometrial cells outside the uterine cavity. Current laboratory practice does not provide specific markers for detecting and assessing the advancement of endometriosis in either plasma or peritoneal fluid. The severity of disease is assessed in stages from I to IV based on the results of laparoscopic inspection. The protein annexin A2 (ANXA2) has been reported to be associated with inflammatory processes. Aim of the Study: The study aimed to investigate and compare ANXA2 protein concentration using the ELISA method in plasma and peritoneal fluid in a group of women with endometriosis compared to controls. Materials and Methods: Biological material was collected during a multicenter, cross-sectional study, which was conducted at eight departments during elective laparoscopy from 53 women with and 40 women without endometriosis. Patients were divided by endometriosis stage and infertility status, and then compared with subgroups. Analysis included the Chi-square test for categorical variables, Mann-Whitney U-test and two-sided Wilcoxon rank-sum test for continuous variables. Results: Women with endometriosis had significantly elevated plasma ANXA2 levels compared to women without endometriosis (mean concentrations 28.69 vs 19.61 ng/L, p=0.01). Differences in peritoneal fluid ANXA2 levels were statistically insignificant (mean concentrations of 23.7 vs 22.97 ng/L, p=0.06). Plasma concentrations in patients with stage III and IV endometriosis were significantly higher compared to controls (mean concentrations of 24.19 vs 19.71 ng/L, p=0.03). No such differences were observed in plasma when comparing stages I-II vs III-IV, and stages I-II vs controls (mean concentrations of 33.82 vs 24.19 ng/L, p=0.72 and 33.82 vs 19.71 ng/L, p=0.12, respectively). Comparison of samples from patients with or without infertility, primary or secondary infertility, endometriosis with or without infertility, and non-endometriosis with or without infertility showed no significant differences in the plasma nor in the peritoneal fluid concentrations. Conclusion: ANXA2 is possibly involved in the pathogenesis of endometriosis, especially in advanced stages. Due to the limited group of tested samples, further studies are needed to confirm its role.

Epidemiology and Risk Factors of UTIs in Children-A Single-Center Observation.

Urinary tract infections (UTIs) are one of childhood's most common bacterial infections. The study aimed to determine the clinical symptoms, laboratory tests, risk factors, and etiology of different UTIs in children admitted to pediatric hospitals for three years. Methods: Patients with positive urine cultures diagnosed with acute pyelonephritis (APN) or cystitis (CYS) were analyzed for clinical symptoms, laboratory tests, risk factors, and etiology, depending on their age and sex. Results: We studied 948 children with UTIs (531 girls and 417 boys), with a median age of 12 (IQR 5−48 months). A total of 789 children had clinical symptoms; the main symptom was fever (63.4% of patients). Specific symptoms of UTIs were presented only in 16.3% of patients. Children with APN had shown significantly more frequent loss of appetite, vomiting, lethargy, seizures, and less frequent dysuria and haematuria than children with CYS. We found significantly higher median WBC, CRP, and leukocyturia in children with APN than with CYS. The risk factors of UTIs were presented in 46.6% of patients, of which 35.6% were children with APN and 61.7% with CYS. The main risk factor was CAKUT, more frequently diagnosed in children with CYS than APN, mainly in children <2 years. The most commonly isolated bacteria were Escherichia coli (74%). There was a higher percentage of urine samples with E. coli in girls than in boys. Other bacteria found were Klebsiella species, Pseudomonas aeruginosa, Proteus mirabilis, and Enterococcus species. Conclusions: Patients with APN were younger and had higher inflammatory markers. Often, fever is the only symptom of UTI in children, and other clinical signs are usually non-specific. The most common UTI etiology is E. coli, regardless of the clinical presentation and risk factors.

The Impact of Coexisting Gestational Diabetes Mellitus on the Course of Preeclampsia.

A strict correlation between gestational diabetes mellitus (GDM) and preeclampsia (PE) has been shown in previous studies. This case-control observational study evaluates the influence of concomitant GDM on the severity of PE. Ninety-nine patients were included: thirty-eight with PE without GDM (group 1), fourteen with PE and concomitant GDM (group 2), and forty-seven with uncomplicated pregnancies (group 3). Adverse maternal/fetal and neonatal outcomes were registered. Patients underwent blood sample analysis of serum PlGF, sFlt-1, creatinine levels, and platelet count (PLT). The incidence of preterm birth, FGR, HELLP syndrome, and NICU admission was significantly higher in group 1 in comparison to groups 2 and 3, whereas RDS was diagnosed most often in group 2 in comparison to groups 1 and 3. All studied biochemical parameters differed between the control group and both PE groups; however, there were no differences between patients with PE with and without GDM. The presented study indicates that the coexistence of GDM may mitigate the course of PE. The lack of differences between patients with PE with and without GDM in serum levels of studied biomarkers may also confirm its usefulness in the diagnosis and management of PE in patients with coexisting GDM.

Plasma and Peritoneal Poly (ADP-Ribose) Polymerase Levels in Patients with Endometriosis.

The evidence of poly (ADP-ribose) polymerase (PARP) association with the immune response could be coherent with the immunological theory of endometriosis and suggests the possibility of a new research direction. The aim of the study was to evaluate the levels of PARP in plasma and peritoneal fluid of patients with and without endometriosis. It was a multicenter, cross-sectional study. Plasma and peritoneal fluid samples were collected from patients with and without endometriosis during planned laparoscopic procedures in eight clinical centers. In total, 84 samples of plasma and 84 samples of the peritoneal fluid were included in the final analyses. Double-antibody sandwich enzyme-linked immunosorbent assay was performed in order to assess levels of PARP in collected samples. No statistically significant differences regarding the detected levels of PARP in plasma and peritoneal fluid comparing patients with and without endometriosis were observed. Patients with a history of infertility had significantly higher plasma PARP concentrations (p = 0.04). We have not observed the potential role of PARP concentration levels in plasma nor peritoneal fluid as an endometriosis biomarker. We have determined an association between a higher plasma PARP concentration and a history of infertility.

Plasma and Peritoneal Fluid ZEB Levels in Patients with Endometriosis and Infertility.

Zinc finger E-box-binding homeobox 1 (ZEB1) and zinc finger E-box-binding homeobox 2 (ZEB2) are transcription factors that regulate epithelial−mesenchymal transformation (EMT). The aim of this study was to compare levels of ZEB1 and ZEB2 in the peritoneal fluid and plasma between patients with and without endometriosis in order to assess their utility in the diagnostic process. Plasma and peritoneal fluid samples were collected from 50 patients with and 48 without endometriosis during planned surgical procedures in eight clinical centers. Quantitative ZEB1 and ZEB2 levels analyses were performed using a double-antibody sandwich enzyme-linked immunosorbent assay (ELISA). No significant differences were observed in ZEB1 levels in any of the subanalyses nor any differences regarding ZEB2 levels between patients with and without endometriosis. Plasma ZEB2 levels were significantly higher among patients with infertility compared to fertile women (16.07 ± 12.70 ng/L vs. 12.07 ± 11.92 ng/L; p < 0.04). Both ZEB1 and ZEB2 do not seem to have a significant value in the initial diagnosis of endometriosis as a single marker. The differences in ZEB2 plasma levels between patients with and without infertility indicate the possibility of EMT dysregulation in the pathogenesis of adverse fertility outcomes.

COVID-19 Vaccination Status among Pregnant and Postpartum Women-A Cross-Sectional Study on More Than 1000 Individuals.

Pregnancy is a well-known factor for vaccine hesitancy and immunization remains the most effective form of prevention against coronavirus disease (COVID-19) related complications. The objective was to estimate vaccine uptake and hesitancy rate, characteristics, and factors contributing to a decision-making process among pregnant and postpartum individuals. This was a prospective cross-sectional study on 1033 pregnant (54.1%) and postpartum (45.9%) women conducted between December 2021 and March 2022 in a tertiary center for maternal−fetal medicine. Logistic regression was used to assess characteristics related to the vaccination decision process. Among responders, 74% were vaccinated and 26% were hesitant (9% planning to vaccinate and 17% totally opposed). Only 59.8% were offered a vaccine by healthcare professionals. Women with higher levels of education (OR 2.26, p < 0.0001), who received positive feedback about vaccination (OR 2.74, p = 0.0172), or were informed about COVID-19 complications in pregnancy (OR 2.6, p < 0.0001) were most likely to accept the vaccination. Hesitancy was associated with multiparity (≥3, OR 4.76, p = 0.006), worse educational status (OR 2.29, p < 0.0001), and lack of previous COVID-19 infection (OR 1.89, p < 0.0001). The most common reason for rejection was insufficient safety data (57%). Understanding factors behind vaccination status is crucial in lowering complications in mothers and newborns and targeted action may facilitate the uptake.

Value of short exercise and short exercise with cooling tests in diagnosis of recessive form of myotonia congenita (Becker disease) - are sex differences important?

INTRODUCTION: In myotonia congenita (MC), activation with exercise or cooling can induce transient changes in compound motor action potential (CMAP) parameters, thus providing a guide to genetic analysis. MATERIAL AND METHODS: We performed the short exercise test (SET) and the short exercise test with cooling (SETC) in 30 patients with genetically confirmed Becker disease (BMC) to estimate their utility in the diagnosis of BMC. RESULTS: Although we observed a significant decrease in CMAP amplitude immediately after maximal voluntary effort in both tests in the whole BMC group, in men this decline was significantly smaller than in women, especially in SET. Clinical implications/future directions: In men with a clinical suspicion of BMC, a small decrease in CMAP amplitude in SET together with a typical decline in SETC does not exclude the diagnosis of BMC. Our results show a sex-specific difference in chloride channel function in BMC, which needs further investigation.

Actuarial Patency Rates of Hepatico-Jejunal Anastomosis after Repair of Bile Duct Injury at a Reference Center.

Background: Bile duct injury complicates patients' lives, despite the subsequent repair. Repairing the injury must restore continuity of the bile tree and bring the patient into a state of cure referred to as "patency". Actuarial primary or actuarial secondary patency rates, depending on whether the patient underwent primary or secondary repair of injury, are proposed to be a proper metric in evaluating outcomes. This study was undertaken to assess outcomes of 669 patients with bile duct injuries Strasberg D and E type referred to the department from public surgical wards between 1990 and 2020. In 442 patients, no attempt was made to repair prior to a referral, and in 227 an attempt to repair was made which failed. Methods: Observations were summarized on December 31st, 2020. The retrospective analysis included: primary patency attained (Grade A result), secondary patency attained (Grade C result), patency loss, and actuarial patency rates of the bile tree at 2, 5, and 10 years. Results: Twenty-five (3.7%) patients died after repair surgery. Actuarial patency rates at 2, 5, and 10 years of follow-up were 93%, 88%, and 74% or 86%, 75%, and 55% in patients attaining Grade A and Grade C outcomes, respectively (p < 0.001). Conclusion: Bile duct injury stands out as a surgical challenge, requiring specialized management at a referral center. Improper proceeding after an injury is the factor leading to faster loss of anastomotic patency.

High-Volume Center Experience with Laparoscopic Adrenalectomy over Two Decades.

Background: Study aims to demonstrate single-institution two decades experience with lateral transperitoneal laparoscopic adrenalectomies. Methods: Retrospective study involved 991 operations grouped into 4 cohorts. Data was collected on the patients' age, sex, side and size of the lesion, histopathological type, hormonal activity, conversion to open adrenalectomy, operating time, length of hospital stay, perioperative complications. Results: The operations were right-sided (n = 550), left-sided (n = 422), bilateral (n = 19). Mean tumor size was 41.9 mm. Histopathological examination revealed 442 adenomas, 191 nodular hyperplasias, 218 pheochromocytomas, 33 malignancies and 126 other lesions. 541 patients had hormonally active tumors. Mean operating time for unilateral laparoscopic adrenalectomy was 141 min. Mean length of hospital stay was 5.27 days. Intraoperative complications rate was 2.3%. Conversion rate was 1.5%. 54 of patients had 70 postoperative complications. Reoperation rate was 1%. Mortality rate was 0.1%. Statistically significant differences were found in all factors, apart from age, sex, side and size of the lesion, reoperations rate (p > 0.05). Conversions rate, complications rates, length of hospital stay were highest in the first group (p < 0.05). Operating time shortened in the first decade. Conclusions: Laparoscopic adrenalectomy is a safe procedure with negligible mortality. Conversions rate, perioperative complications rate, and length of hospital stay, significantly decreased over time.

Intraoperative dialysis with the use of a mobile dialysis system during liver transplantation.

PURPOSE: Over the last few years, transplant centers have started to use various intraoperative renal replacement therapy (ioRRT) modalities during liver transplantation (LT) in patients with pre-existing renal impairment. Here, we present a study on the safety and clinical outcomes of intraoperative hemodialysis (ioHD) performed using a mobile dialysis system during LT. PATIENTS AND METHODS: We retrospectively analyzed 102 adult patients undergoing LT with ioHD; pre-existing renal failure and/or intraoperative metabolic derangement were ioHD treatment indications. RESULTS: Our study cohort consisted of three groups: LT with preoperative serum creatinine (sCr) â€‹< â€‹2 â€‹mg/dL (Group 1:n â€‹= â€‹22), LT with preoperative sCr ≥2 â€‹mg/dL (Group 2:n â€‹= â€‹73), and simultaneous liver-kidney transplantation (Group 3:n â€‹= â€‹7). Among the procedures, 30% were re-transplantations. The mean calculated Model for End-stage Liver Disease score in Group 2 was 39.2, and 67% of patients were hospitalized in the intensive care unit. Patients in Group 1 were less acutely ill but developed severe intraoperative derangements and, therefore, underwent urgent ioHD intraoperatively. However, it was delayed when compared to Group 2. All groups achieved post-reperfusion potassium levels <4 â€‹mmol/L and a decrease in central venous pressure. No serious procedural complications occurred. Post-reperfusion syndrome occurred in 12.7% of patients. Elevated mortality was likely due to the high illness severity in the cohort. CONCLUSIONS: Performing ioHD with a mobile dialysis system during LT was safe and effective, while being easier to perform than continuous techniques. Its effect on intra- and postoperative outcomes should be addressed in a study with a control group.

A Retrospective Study of 881 Lateral Transabdominal Laparoscopic Adrenalectomies Performed Between 1997 and 2017 at a Single Center in Poland to Determine Factors Associated with Surgery Time.

BACKGROUND Laparoscopic adrenalectomy is acknowledged as a standard procedure in adrenal lesions management. This retrospective study of lateral transabdominal laparoscopic adrenalectomy performed between 1997 and 2017 in a single center in Poland aimed to determine the factors associated with surgery time. MATERIAL AND METHODS This retrospective study involved 881 patients. The factors identified as potentially affecting surgery time were age, sex, side of the lesion, histopathological type, hormonal activity, size of the lesion, history of previous abdominal operations, presence of intra-abdominal adhesions, and obesity. The following statistical tests were conducted: t test, Mann-Whitney U test, Kruskal-Wallis H test, Pearson correlation test, and multivariate regression modeling. RESULTS The mean surgery time for all operations was 139 min (55-320 min). We observed statistically significant differences for surgery time in the following groups: sex, side of the lesion, presence of intra-abdominal adhesions, obesity, histopathological type, and hormonal activity (P<0.05). The multivariate regression model showed factors affecting surgery time were: sex, side of the lesion, size of the lesion, obesity, histopathological type (nodular hyperplasia and adenoma), and hormonal activity (non-functioning and aldosterone-secreting tumors) (P<0.05). There was no correlation between surgery time and patient age and tumor size (P<0.05). Mean tumor size was 42 mm (6-130 mm). CONCLUSIONS The 20-year experience of laparoscopic adrenalectomy performed at a single center showed that surgery duration was significantly longer in male patients, obese patients, left-sided adrenal tumors, tumors >6 cm in diameter, patients with a diagnosis of pheochromocytoma, and when intra-abdominal adhesions were present.

Simultaneous Liver-Kidney Transplantation and the Use of Intraoperative Dialysis: A Monocenter Study.

BACKGROUND: Simultaneous liver and kidney transplants (SLKT) represent 1.1% of all liver transplants in Poland. Patients undergoing SLKT experience a longer operation time and concurrent kidney dysfunction may aggravate metabolic derangement associated with the procedure. The benefits of intraoperative dialysis (ioHD) in these patients have not been determined. METHODS: A retrospective observational study of all adult patients undergoing SLKT in our center from January 2009 till December 2016. RESULTS: Study group consisted of 10 patients with End-Stage Kidney Disease (0.9% of all liver transplants): 6 patients treated with ioHD during SLKT (group 1) and 4 patients managed conservatively (group 2). All recipients were on chronic dialysis. The mean calculated Model for End-Stage Liver Disease score was 21 ± 0.9 in group 1 and 30 ± 9.5 in group 2 (P = .009). The mean preoperative serum potassium was 4.7 ± 0,6 mmol/L in group 1 and 3.97 ± 1,02 in group 2. Intraoperative serum potassium levels were comparable between the groups, but the maximum lactate and minimum bicarbonate levels were significantly worse in group 2. Postreperfusion syndrome occurred in no patient. Dialysis circuit clotting occurred in 50% of ioHD. Six patients (2 in group 1) required renal replacement therapy after SLKT; no patient was on dialysis on discharge. Three patients died within 1 year after surgery (2 in group 2). CONCLUSIONS: No patient developed intraoperative hyperkalemia or postreperfusion syndrome. We observed a high frequency of circuit system clotting during ioHD. Clinical benefits of intraoperative hemodialysis during SLKT need to be determined in a larger study.

Three-dimensional computed tomography analysis of frontal recess cells according to the International Frontal Sinus Anatomy Classification (IFAC) - difficulties in identification of frontal recess cells in patients with diffuse primary chronic rhinosinusitis?

<br><b>Introduction:</b>The International Frontal Sinus Anatomy Classification (IFAC) is a consensus document created to standardize and specify the naming of cells within the region of the frontal recess and frontal sinus.</br> <br><b>Aim:</b> The aim of this study was to analyze the difficulties in identifying cells according to the IFAC in patients with diffuse primary chronic rhinosinusitis.</br> <br><b>Material and methods:</b> Three independent reviewers examined triplanar computed tomography (CT) scans to assess the anatomy of the frontal recess using the IFAC system. CT scans were chosen randomly and divided into 3 groups: CT scans of patients not presenting sinus complaints (control group), CT scans of patients affected by diffuse primary chronic rhinosinusitis non-type 2, and CT scans of patients affected by diffuse primary chronic rhinosinusitis type 2.</br> <br><b>Results:</b> Identification of all frontal cell types was accurate in patients not presenting sinus complaints (P-value < 0.05). Patients scoring 9 or more points in the Lund-Mackay scoring system demonstrated a statistically increased risk of improper identification of frontal recess cells (P-value < 0.0001).</br> <br><b>Conclusions:</b> Due to a large number of possible anatomical variants and changes caused by the chronic inflammatory disease, the IFAC nomenclatura is easier to apply to non-type 2 primary diffuse CRS patients with low scores in the L-M score scale than to primary diffuse type 2 CRS patients with higher M-L scores.</br>.

Association between air pollution and COVID-19 mortality and morbidity.

Coronavirus disease (COVID-19) pandemic is affecting the world unevenly. One of the highest numbers of cases were recorded in the most polluted regions worldwide. The risk factors for severe COVID-19 include diabetes, cardiovascular, and respiratory diseases. It has been known that the same disease might be worsened by chronic exposure to air pollution. The study aimed to determine whether long-term average exposure to air pollution is associated with an increased risk of COVID-19 cases and deaths in Poland. The cumulative number of COVID-19 cases and deaths for each voivodeship (the main administrative level of jurisdictions) in Poland were collected from March 4, 2020, to May 15, 2020. Based on the official data published by Chief Inspectorate of Environmental Protection voivodeship-level long-term exposure to main air pollution: PM2.5, PM10, NO2, SO2, O3 (averaged from 2013 to 2018) was established. There were statistically significant correlation between COVID-19 cases (per 100,000 population) and annual average concentration of PM2.5 (R2 = 0.367, p = 0.016), PM10 (R2 = 0.415, p = 0.009), SO2 (R2 = 0.489, p = 0.003), and O3 (R2 = 0.537, p = 0.0018). Moreover, COVID-19 deaths (per 100,000 population) were associated with annual average concentration of PM2.5 (R2 = 0.290, p = 0.038), NO2 (R2 = 0.319, p = 0.028), O3 (R2 = 0.452, p = 0.006). The long-term exposure to air pollution, especially PM2.5, PM10, SO2, NO2, O3 seems to play an essential role in COVID-19 prevalence and mortality. Long-term exposure to air pollution might increase the susceptibility to the infection, exacerbates the severity of SARS-CoV-2 infections, and worsens the patients' prognosis. The study provides generalized and possible universal trends. Detailed analyzes of the phenomenon dedicated to a given region require taking into account data on comorbidities and socioeconomic variables as well as information about the long-term exposure to air pollution and COVID-19 cases and deaths at smaller administrative level of jurisdictions (community or at least district level).

COVID-19 Pandemic-Related Anxiety in Pregnant Women.

The COVID-19 pandemic outbreak influenced general and mental health worldwide. The objective of this study was to assess the anxiety level during the COVID-19 pandemic among pregnant women and compare it between COVID-infected and non-infected groups. We prospectively assessed the daily routine and anxiety level using a bespoke questionnaire and GAD-7 scale validated for pregnant women. With logistic regression, we established possible risk factors of generalized anxiety disorder spectrum and main causes of concern. The dataset included 439 responders of our survey. Of which, 21% had COVID-19 infection during pregnancy; 38% were screened for possible generalized anxiety disorder and the proportion was higher in women who suffered from COVID-19 (48% vs. 35%, p = 0.03). Pre-pregnancy anxiety or depression diagnosis and intentional social contact avoidance increased the risk of anxiety (aOR 3.4 and 3.2). Fetal wellbeing was the main concern for 66% of the responders. The COVID-19 pandemic and related restrictions substantially altered daily lives of pregnant women, exaggerating the prevalence of anxiety compared with the pre-COVID-19 studies (38% vs. 15%). COVID-19 infection during pregnancy was associated with increased levels of generalized anxiety scores. Patient-tailored psychological support should be a mainstay of comprehensive antenatal medical care in order to avoid anxiety- and stress-related complications.

Comparison of Intranasal Steroid Application Using Nasal Saline Irrigation and a Mucosal Atomization Device to Treat Chronic Rhinosinusitis.

Introduction: Chronic rhinosinusitis (CRS) is a disease that can significantly reduce patients' quality of life (QoL). Intranasal steroid therapy is the most commonly used treatment for CRS. There are many evaluation tools dedicated to assessing CRS patients' QoL, but none of them evaluates QoL during local steroid therapy. Mucosal atomization devices (MADs) and nasal saline irrigation (NSI) are effective and safe methods of applying intranasal steroids for CRS patients. Materials and Methods: The sample population for this prospective study comprised 43 CRS patients. Following endoscopic sinus surgery, all participants received intranasal steroids administered via an MAD, followed by NSI for 1.5 months. Each participant completed the SNOT-22 (22-item Sino-Nasal Outcomes Test) score and a new questionnaire, the Complementary Topical Nasal Drug Delivery Questionnaire (the Complementary Questionnaire), at the end of 3 months of intranasal steroid therapy. Results: The patients' responses in both the SNOT-22 score and the Complementary Questionnaire revealed significant differences in their adverse experiences. The patients who received intranasal steroid treatment using NSI experienced more frequently delayed nasal drainage, higher frequency of ear symptoms, and facial pain/pressure, while those whose therapy was administered using an MAD reported complaints such as nasal irritation, nasal dryness, and postnasal drip with unpleasant taste/smell. Conclusion: We used the Complementary Questionnaire as an effective tool for assessment of the QoL of CRS patients. The SNOT-22 score and the Complementary Questionnaire make it possible to select an intranasal applicator tailored to a CRS patient's specific complaints.

Observation of the natural course of type 3 spinal muscular atrophy: data from the polish registry of spinal muscular atrophy.

BACKGROUND: Spinal muscular atrophy (SMA) is one of the most frequent and severe genetic diseases leading to premature death or severe motor disability. New therapies have been developed in recent years that change the natural history of the disease. The aim of this study is to describe patients included in the Polish Registry of SMA, with a focus on the course of type 3 SMA (SMA3) before the availability of disease-modifying treatments. RESULTS: 790 patients with SMA were included in the registry (173 with type 1 [SMA1], 218 with type 2 [SMA2], 393 with SMA3, and six with type 4 SMA [SMA4]), most (52%) of whom were adults. Data on SMN2 gene copy number were available for 672 (85%) patients. The mean age of onset was 5 months for SMA1, 11.5 months for SMA2, and 4.5 years for SMA3. In patients with SMA3, the first symptoms occurred earlier in those with three copies of SMN2 than in those with four copies of SMN2 (3.2 years vs. 6.7 years). The age of onset of SMA3 was younger in girls than in boys (3.1 years vs. 5.7 years), with no new cases observed in women older than 16 years. Male patients outnumbered female patients, especially among patients with SMA3b (49 female vs. 85 male patients) and among patients with SMA3 with four copies of SMN2 (30 female vs. 69 male patients). 44% of patients with SMA3 were still able to walk; in those who were not still able to walk, the mean age of immobilization was 14.0 years. Patients with SMA3a (age of onset < 3 years) and three copies of SMN2 had significantly worse prognosis for remaining ambulant than patients with SMA3b (age of onset ≥ 3 years) and four copies of SMN2. CONCLUSIONS: The Registry of SMA is an effective tool for assessing the disease course in the real world setting. SMN2 copy number is an important prognostic factor for the age of onset and ambulation in SMA3. Sex and age of disease onset also strongly affect the course of SMA. Data supplied by this study can aid treatment decisions.

Endoglin Expression and Microvessel Density as Prognostic Factors in Pediatric Rhabdomyosarcoma.

(1) Background: The study proposed to analyze microvessel density (MVD) in rhabdomyosarcoma (RMS) based on the expression of angiogenesis markers and define its prognostic role in this group of patients. (2) Methods: The study included forty-nine pediatric patients diagnosed with RMS. Tumor tissue expression of CD31, CD34, and CD105 was analyzed. MVD was calculated and correlated with clinical RMS prognostic parameters. (3) Results: CD31, CD34, and CD105 are expressed in all RMS cases. MVD/CD105 was significantly higher in the RMS group than in the control group. The mean and median values of MVD/CD105 in RMS were lower than MVD/CD31 and MVD/CD34. MVD/CD105 was significantly higher in patients with alveolar RMS and those with metastatic disease. Patients with higher levels of MVD/CD105 had a higher risk of death (HR = 1.009). (4) Conclusion: CD105 is a relevant angiogenesis marker in pediatric RMS, and MVD/CD105 is an independent risk factor of short overall survival in children with RMS.