Understanding the dynamics of compliance to smoke-free policy regulations: Exploring the perspectives of venue owners and staff in Türkiye.

INTRODUCTION: The study aims to understand the facilitators and barriers associated with enforcing and complying with Türkiye's smoke-free policy from the perspective of hospitality venue owners and employees. METHODS: A qualitative open-ended survey was conducted in Istanbul and Ankara in 2021 with 58 respondents from 3 different districts in each city from four types of venues: restaurants, traditional coffee and waterpipe houses, and European-style cafés. The open-ended survey included questions to understand the knowledge, beliefs, and attitudes of respondents about Türkiye's smoke-free policy and their perceptions of the facilitators and/or barriers to smoke-free policy implementation and changes after COVID-19. The data were analyzed using an inductive approach to identify patterns and categorize the data into themes. RESULTS: The respondents expressed that the smoke-free policy aimed to protect employees and customers from secondhand smoke (SHS), respect human health, and improve air quality. Findings suggest that the positive attitude of venue owners and staff toward the smoke-free policy serves as a facilitator. However, fear of financial impact, customers' negative attitudes, difficulties in meeting physical requirements, and insufficient enforcement were found to be barriers to implementing the smoke-free policy. The effects of the COVID-19 pandemic were reported as an initial increase in compliance and awareness among customers and staff, but some respondents noted negative changes due to the emotional and financial effects of prolonged restrictions. These challenges have led to decreased attention on the smoke-free policy among venue owners, staff and customers. Respondents' suggested improvements were related to building infrastructure, such as the ventilation systems and educating the public on the harmful health effects of smoking. CONCLUSIONS: Despite the general understanding of the dangers of secondhand smoke and the smoke-free policy, this study highlights the challenges in implementing smoke-free policy measures and the continued need to raise awareness about the importance of a 100% smoke-free venue. A comprehensive approach to addressing the tobacco epidemic as a multifaceted public health issue is essential.

Battle of polymyxin induced nephrotoxicity: Polymyxin B versus colistin.

OBJECTIVES: Nephrotoxicity is the most serious and common adverse effect that limits the use of polymyxins. This study compared polymyxin E (colistin) and polymyxin B regarding drug-related nephrotoxicity. METHODS: This study was conducted as a retrospective cohort study in a university hospital between January 2020 and July 2022. Patients older than 18 years and who received colistin or polymyxin B were identified using electronic hospital records. Kidney disease improving global outcome criteria were used for assessing nephrotoxicity. RESULTS: A total of 190 patients, 95 in both groups, were evaluated. The incidence of acute kidney injury during the treatment was higher in the colistin group [52.6% (n = 50) and 34.7% (n = 33), P = 0.013]. In patients who were exposed to high-dose, the rate of nephrotoxicity was higher in patients receiving colistin [25% (n = 3) vs. 76.9% (n = 10); P = 0.017]. Nephrotoxicity was reversible in 64.4% (n = 38) of patients and the reversibility rate was similar (70% and 52.6% for colistin and polymyxin; P = 0.248). In the multivariable analysis, colistin treatment [odds ratio (OR): 3.882, 95% confidence interval (95% CI) = (1.829-8.241)], concomitant vasopressor use (OR = 2.08, CI: 1.036-4.179), and age (OR=1.036, CI: 1.014-1.058) were found to be independent markers of nephrotoxicity. CONCLUSION: Nephrotoxicity was more common in patients receiving high-dose colistin than polymyxin B. Therefore, the use of appropriate doses of colistin is important in terms of preventing nephrotoxicity. In addition, advancing age and concomitant use of vasopressors contribute to polymyxin-related nephrotoxicity.

Extracellular-Vesicle-Based Cancer Panels Diagnose Glioblastomas with High Sensitivity and Specificity.

Glioblastoma is one of the most devastating neoplasms of the central nervous system. This study focused on the development of serum extracellular vesicle (EV)-based glioblastoma tumor marker panels that can be used in a clinic to diagnose glioblastomas and to monitor tumor burden, progression, and regression in response to treatment. RNA sequencing studies were performed using RNA isolated from serum EVs from both patients (n = 85) and control donors (n = 31). RNA sequencing results for preoperative glioblastoma EVs compared to control EVs revealed 569 differentially expressed genes (DEGs, 2XFC, FDR < 0.05). By using these DEGs, we developed serum-EV-based biomarker panels for the following glioblastomas: wild-type IDH1 (96% sensitivity/80% specificity), MGMT promoter methylation (91% sensitivity/73% specificity), p53 gene mutation (100% sensitivity/89% specificity), and TERT promoter mutation (89% sensitivity/100% specificity). This is the first study showing that serum-EV-based biomarker panels can be used to diagnose glioblastomas with a high sensitivity and specificity.

The outcomes of renin-angiotensin-aldosterone system inhibition and immunosuppressive therapy in children with X-linked Alport syndrome.

BACKGROUND: Alport syndrome (AS) is characterized by progressive kidney disease. There is increasing evidence that renin-angiotensin-aldosterone system (RAAS) inhibition delays chronic kidney disease (CKD) while the effectiveness of immunosuppressive (IS) therapy in AS is still uncertain. In this study, we aimed to analyze the outcomes of pediatric patients with X-linked AS (XLAS) who received RAAS inhibitors and IS therapy. METHODS: Seventy-four children with XLAS were included in this multicenter study. Demographic features, clinical and laboratory data, treatments, histopathological examinations, and genetic analyses were analyzed retrospectively. RESULTS: Among 74 children, 52 (70.2%) received RAAS inhibitors, 11 (14.9%) received RAAS inhibitors and IS, and 11 (14.9%) were followed up without treatment. During follow-up, glomerular filtration rate (GFR) decreased < 60 ml/min/1.73 m2 in 7 (9.5%) of 74 patients (M/F=6/1). In male patients with XLAS, kidney survival was not different between RAAS and RAAS+IS groups (p=0.42). The rate of progression to CKD was significantly higher in patients with nephrotic range proteinuria and nephrotic syndrome (NS), respectively (p=0.006, p=0.05). The median age at the onset of RAAS inhibitors was significantly higher in male patients who progressed to CKD (13.9 vs 8.1 years, p=0.003). CONCLUSIONS: RAAS inhibitors have beneficial effects on proteinuria and early initiation of therapy may delay the progression to CKD in children with XLAS. There was no significant difference between the RAAS and RAAS+IS groups in kidney survival. AS patients presenting with NS or nephrotic range proteinuria should be followed up more carefully considering the risk of early progression to CKD.

Long-term kidney follow-up after pediatric acute kidney support therapy for children less than 15 kg.

BACKGROUND: In small children, acute dialysis (pediatric acute kidney support therapy (paKST)) is increasingly used; however, it is challenging for many reasons. We compared clinical characteristics and predictors of long-term outcomes of patients < 15 kg on peritoneal dialysis (PD), hemodialysis (HD), and continuous kidney replacement therapy (CKRT). METHODS: Patients with history of paKST (CKRT, HD, PD) weighing < 15 kg and ≥ 6 months of follow-up at Hacettepe University were included. Surviving patients were evaluated at last visit. RESULTS: 109 patients (57 females) were included. Median age at paKST was 10.1 months (IQR: 2-27 months). In total, 43 (39.4%) patients received HD, 37 (34%) PD, and 29 (26.6%) CKRT. 64 (58.7%) patients died a median 3 days (IQR: 2-9.5 days) after paKST. Percentages of patients using vasopressor agents, with sepsis, and undergoing mechanical ventilation were lower in those who survived. After mean follow-up of 2.9 ± 2.1 years, 34 patients were evaluated at mean age 4.7 ± 2.4 years. Median spot urine protein/creatinine was 0.19 (IQR: 0.13-0.37) and 12 patients (35.3%) had non-nephrotic proteinuria. Three patients had estimated glomerular filtration rate (eGFR) < 90 mL/min/1.73m2 and 2 (6%) had hyperfiltration. In total 22 patients (64.7%) had ≥ 1 kidney risk factor (elevated blood pressure/hypertension, hyperfiltration, eGFR < 90 ml/min/1.73m2, and/or proteinuria) at last visit. Among 28 patients on paKST < 32 months, 21 had ≥ 1 risk factor (75%), whereas among 6 patients who had paKST ≥ 32 months, one patient had ≥ 1 risk factor (16.7%), (p = 0.014). CONCLUSIONS: Patients on paKST who undergo mechanical ventilation and vasopressor treatment should be followed-up more closely. After surviving the acute period, patients on paKST need to be followed-up closely during the chronic stage. A higher resolution version of the Graphical abstract is available as Supplementary information.

The Clinical and Mutational Spectrum of 69 Turkish Children with Autosomal Recessive or Autosomal Dominant Polycystic Kidney Disease: A Multicenter Retrospective Cohort Study.

INTRODUCTION: Autosomal recessive polycystic kidney disease (ARPKD) is associated with pathogenic variants in the PKHD1 gene. Autosomal dominant polycystic kidney disease (ADPKD) is mainly associated with pathogenic variants in PKD1 or PKD2. The present study aimed to identify the clinical and genetic features of Turkish pediatric ARPKD and ADPKD patients. METHODS: This multicenter, retrospective cohort study included 21 genetically confirmed ARPKD and 48 genetically confirmed ADPKD patients from 7 pediatric nephrology centers. Demographic features, clinical, and laboratory findings at presentation and during 12-month intervals were recorded. RESULTS: The median age of the ARPKD patients at diagnosis was lower than the median age of ADPKD patients (10.5 months [range: 0-15 years] vs. 5.2 years [range: 0.1-16 years], respectively, [p = 0.014]). At the time of diagnosis, the median eGFR in the ARPKD patients was lower compared to that of ADPKD patients (81.6 [IQR: 28.7-110.5] mL/min/1.73 m2 and 118 [IQR: 91.2-139.8] mL/min/1.73 m2, respectively, [p = 0.0001]). In total, 11 (52.4%) ARPKD patients had malnutrition; 7 (33.3%) patients had growth retardation at presentation; and 4 (19%) patients had both malnutrition and growth retardation. At diagnosis, 8 (16.7%) of the ADPKD patients had malnutrition, and 5 (10.4%) patients had growth retardation. The malnutrition, growth retardation, and hypertension rates at diagnosis were higher in the ARPKD patients than the ADPKD patients (p = 0.002, p = 0.02, and p = 0.0001, respectively). ARPKD patients with malnutrition and growth retardation had worse renal survival compared to the patients without (p = 0.03 and p = 0.01). Similarly, ADPKD patients with malnutrition had worse renal survival compared to the patients without (p = 0.002). ARPKD patients with truncating variants had poorer 3- and 6-year renal outcome than those carrying non-truncating variants (p = 0.017). CONCLUSION: Based on renal survival analysis, type of genetic variant, growth retardation, and/or malnutrition at presentation were observed to be factors associated with progression to chronic kidney disease (CKD). Differentiation of ARPKD and ADPKD, and identification of the predictors of the development of CKD are vital for optimal management of patients with ARPKD or ADPKD.

Derivation and validation of adult Still Activity Score (SAS).

OBJECTIVES: Adult-onset Still's disease (AOSD) is a multi-systemic, autoinflammatory disorder. Several activity scores have been proposed but none of them have been adopted universally. Our aim was to create a clinician-friendly activity scoring system by using simple clinical and laboratory parameters. METHODS: AODS patients, according to Yamaguchi criteria, were included in this cross-sectional, multicenter study. Derivation and validation cohorts were constituted. Demographic, clinical, and laboratory evaluation at the study visit; patients' and physicians' global assessments of disease activity (both VAS/Likert scale) were recorded. To develop the score, an ordinal logistic regression model was used to determine independent predictors of physicians' global assessments of disease activity. Clinically and statistically significant variables were weighted according to regression coefficients. Then, performance of the score was tested on the validation cohort. RESULTS: A total of 197 consecutive AOSD patients (125 in derivation, 72 in validation cohorts) were included. Final Still Activity Score was fever (2 points), arthralgia (2 points, plus 1 point if arthritis was present in≥2 joints), neutrophilia≥65% (1 point) and ferritin≥350ng/mL (1 point) (maximum of 7 points). The SAS yielded an AUC value of 0.98 (0.96-1.00) in the derivation cohort and 0.91 (95%CI: 0.85-0.98) in the validation cohort to discriminate high AOSD activity from moderate-inactive AOSD. The correlation of SAS with PGA was 83% for the derivation cohort and 76% for the validation cohort. CONCLUSIONS: SAS has shown a good test performance to distinguish active AOSD patients from others. SAS may be a useful method for evaluating the disease activity of AOSD patients in daily practice.

Extracorporeal photopheresis for the treatment of chronic graft versus host disease.

OBJECTIVES: Chronic graft versus host disease (chronic GVHD) still remains the leading cause of late morbidity and mortality for allogeneic hematopoietic stem cell transplant (allo-HSCT) recipients. In this retrospective study, 53 consecutive allo-HSCT patients with chronic GVHD refractory to corticosteroids were treated with extracorporeal photopheresis (ECP). METHODS: This study was performed as a retrospective single-center study. Medical records of a total of 59 patients treated with ECP for chronic GVHD were reviewed. RESULTS: Best organ responses to ECP were observed in skin, mouth mucosa, eyes and liver. Overall response rate (ORR) to ECP was 81.2% (CR 17% and PR 64.2%). Overall survival (OS) was 84.9% and 36.7%, at 1 and 3 years, respectively. Female sex appears to have an advantage on ORR. Patients achieving ORR were able to maintain their responses with a prolonged continuation of treatments for +6 and +12 months indicating the benefits of longer ECP treatment. DISCUSSION: We found that patients with chronic GVHD who were treated with ECP for 12 months or longer had a higher response rate. Our findings in line with the data reported previously suggest that patients responding to ECP should continue longer therapy schedules to achieve a better and sustained response. In our cohort, long-term ECP therapy was safe and well-tolerated with no significant adverse effects. Best responses were observed in the patients with skin, eye, liver and oral involvement. The ECP procedure offers the advantage relative to the problems with typical immunosuppressive agents. The female sex appeared to have an advantage based on the cumulative probability of the OR after ECP for chronic GVHD.

Estimating the optimal entry point of the antegrade femoral nailing: Previous and novel morphometric measurements.

OBJECTIVE: This anatomical study aimed to investigate the possible relationships between the proximal femur parameters and verify the optimal entry point in line with the medullary canal. METHODS: Both in single image series and 3D-MPR views, 63 femur bones were evaluated. One-millimeter multidetector computed tomography scans were collected and assessed by OsiriX-Lite version 8 and Horos v3.3.5. Entry point locations, projected and true femoral neck-shaft, anteversion, and newly defined nail entrance angles were measured. RESULTS: The entry points of 16 femurs were placed at the tip of the greater trochanter, and the remaining 47 femurs were in the trochanteric fossa (i.e., piriformis fossa). Thirty-three of the entry points found in the trochanteric fossa were overlapped by the greater trochanter. When the parameters of the right and left sides were compared, it was found that; projected neck angle, nail entrance angle, and the differences between true neck angle and nail entrance angle were found significantly different. The relationship between nail entrance angle and entry point localization was assessed, and the probability of the entry point being at the tip of the greater trochanter is 63 times greater when the NEA is below 90. CONCLUSION: This study has demonstrated that the ideal entry point for straight nails, which is based on the anatomic axis of the femur, is found mainly at the trochanteric fossa, and the greater trochanter overlapped 70.21% of them. LEVEL OF EVIDENCE: Level IV, Diagnostic Study.

Hair diameter measurements for planning follicular unit extraction surgery (FUE): Is there a correlation between the micrometer caliper and scanning electron microscopy (SEM) findings?

BACKGROUND: Hair shaft diameter is one of the most important factors for the outcome of follicular unit extraction (FUE) surgery. In fact, the hair shaft is elliptical. Therefore, it has a long and short axis. Many hair transplantation surgeons use manual micrometer caliper for gauging hair diameter and use the results in the management of recipient and donor area. AIM: In this study, we aimed to identify the dependability of micrometer caliper and also the hair diameter diversity pattern in the donor area. PATIENTS/METHODS: Two hundred and seventy hairs were collected from three males with androgenetic alopecia. Hair samples were obtained from the 1 cm2 boxes from superior to inferior at the mid-point of temporal, parietal, and occipital donor areas. The diameter of each hair was measured both with a micrometer caliper and scanning electron microscopy (SEM). RESULTS: Average diameter measured by scanning electron microscopy was 83.01 µm for the long axis and 51.51 µm for the short axis. The average value for the micrometer caliper measurement was 53.32 µm. Comparison of micrometer caliper results with the short-axis measurements of SEM revealed a strong significant correlation. The hair diameters from superior, middle, and inferior boxes revealed a tendency to decrease toward the inferior regions. CONCLUSIONS: A manual micrometer caliper is a dependable tool for planning FUE surgery, and it measures the short axis of the elliptical hair shaft. Hair diameter tends to decrease toward the inferior regions of the donor area.

Treadmill versus overground gait training in patients with lower limb burn injury: A matched control study.

INTRODUCTION: Gait impairment is commonly seen in patients with a lower limb burn injury (LLBI). Therefore, the aim of this study was to investigate the effects of two different gait training modes on gait symmetry, functional mobility and kinesiophobia in patients with LLBI. METHODS: This matched control study was conducted between January 2017 and August 2018. Patients with LLBI (n=28) were allocated to 2 different groups by matching according to burn localization, age, and gender. Group 1 (overground group: n=14) received overground gait training in addition to standard burn rehabilitation, and Group 2 (treadmill group: n=14) received treadmill gait training in addition to standard burn rehabilitation. The rehabilitation program and gait training were started when the patient was admitted to the hospital and ended on discharge. These physical therapy interventions were performed 5 days per week. The gait training intensity, including walking speed and duration, was determined according to patient tolerance. Gait parameters, functional mobility, kinesiophobia and pain values were evaluated with GAITRite, the timed up-and-go test (TUG), Tampa Kinesiophobia Scale and Visual Analogue Scale, respectively. These evaluations were made twice; on admission and immediately prior to discharge. Gait symmetry was calculated using the Symmetry Index. RESULTS: The baseline characteristics of the groups and initial outcome values were similar. In the comparison of the differences between the overground and treadmill groups, the change in kinesiophobia and TUG values were significantly higher in the treadmill group (p=0.01, p=0.02, respectively). The intragroup comparisons showed significant differences in SI in respect of step length (p=0.004), swing (p=0.006), stance (p=0.008) and velocity (p=0.001), cadence (p=0.001), TUG (p=0.001), kinesiophobia (p=0.001) and pain (p=0.001) in the overground group. Statistically significant differences were determined in step length (p=0.01), swing (p=0.01), stance (p=0.02) and velocity (p=0.001), cadence (p=0.001), TUG (p=0.001), kinesiophobia (p=0.001) and pain (p=0.001) in the treadmill group, when pre and post-training values were compared. CONCLUSIONS: The results of this study have shown that treadmill gait training was more effective in the improvement of functional mobility and reduction in kinesiophobia levels of patients with LLBI compared to overground gait training. Both overground and treadmill gait training also provide greater improvements in the velocity and cadence, and gait symmetry for step length, swing and stance in patients with LLBI. CLINICAL TRIAL REGISTRATION NUMBER: NCT03217526.

Birth Weight Reference Percentiles by Gestational Age for Turkish Twin Neonates.

AIMS: In clinical practice, birth weight reference percentiles for singletons are used to evaluate twin births. The utilization of singleton reference percentiles for twins is not appropriate as they experience different growth trajectories. However, Turkey still lacks such references. Our aim was to create gestational age-specific birth weight references for female and male Turkish twins. MATERIALS AND METHODS: This is a hospital-based, multi-centered, retrospective study. In total, 2544 live-born twins between 2010 and 2019 were included in the study. Gestational age, birth weight, mode of delivery, gender, birth order, chorionicity, maternal age, pregnancy resulting from assisted reproduction techniques, APGAR (Appearance, Pulse, Grimace, Activity, and Respiration) score at 5 minutes, admission to the neonatal intensive care unit (NICU), length of stay in NICU, and death during the NICU stay were recorded. RESULTS: Smoothed reference curves for birth weight by gestational age and separate tables for female and male twin neonates for the 3rd, 10th, 50th, 90th, and 97th percentiles from 26 to 39 weeks of gestational age were constructed. Overall neonatal and infant mortality rates during NICU stay in our twin cohort were 12/1000 and 16/1000, respectively. CONCLUSION: Twin-specific birth weight nomograms could be helpful as a reference for clinicians to identify high-risk neonates and fetuses who need specialized care. However, further studies with larger series are urgently needed for validation and use of these nomograms in clinical practice.

Clinical features and outcomes of COVID-19 in patients with solid tumors: Turkish National Registry Data.

We present demographic, clinical, laboratory characteristics and outcomes of the patients with solid malignancies and novel coronavirus disease (COVID-19) collected from the National COVID-19 Registry of Turkey. A total of 1523 patients with a current or past diagnosis of solid tumors and diagnosed with COVID-19 (confirmed with PCR) between 11 March and 20 May 2020 were included. The primary outcome was 30-day mortality. Median age was 61 (range: 18-94), and 752 (49%) were male. The most common types of cancers were breast (19.8%), prostate (10.9%) and colorectal cancer (10.8%). 65% of the patients had at least one comorbidity. At least one COVID-19-directed therapy was given in 73% of the patients.. Hospitalization rate of the patients was 56.6% and intensive care unit admission rate was 11.4%. Seventy-seven (5.1%) patients died within 30 days of diagnosis. The first multivariate model which included only the demographic and clinical characteristics showed older age, male gender and presence of diabetes and receipt of cytotoxic therapy to be associated with increased 30-day mortality, while breast and prostate cancer diagnoses were associated with lower 30-day mortality. In the second set, we further included laboratory parameters. The presence of leukocytosis (OR 6.7, 95% CI 3.3-13.7, P < .001), lymphocytopenia (OR 3,1, 95% CI 1,6-6,1, P = .001) and thrombocytopenia (OR 3,4 95% CI 1,5-8,1, P = .005) were found to be associated with increased 30-day mortality. Relatively lower mortality compared to Western countries and China mainly results from differences in baseline risk factors but may also implicate the importance of intensive supportive care.

A novel technique for prediction of preterm birth: fetal nasal flow Doppler.

OBJECTIVES: Absence of fetal breathing movements (FBM) has been found to be a good predictor of preterm delivery in symptomatic patients. However, analysis of FBM patterns and Doppler measurement of them for preterm birth prediction have not been performed before. In this study, we aimed to investigate and analyze FBM patterns in symptomatic preterm labor patients by fetal ultrasonography and nasal Doppler. METHODS: This was a multicenter, prospective cohort study. Singleton pregnant patients between 24 and 37 gestational weeks diagnosed with preterm labor were included in the study. Patients were evaluated in three groups: no FBM (Group 1), regular FBM (Group 2), irregular FBM (Group3). RESULTS: Seventy-three patients were available for the final analysis after exclusion. Preterm delivery rate in 24 h in groups were 91.7, 32.7 and 100%, respectively. The absence of FBM (Group 1) was statistically significant for preterm delivery in for both 24 (91.7 vs. 42.6%, p=0.002) and 48 h (91.7 vs. 49.2%, p=0.006) when compared with fetal breathing positive Group 2 and 3. In fetal nasal Doppler analyses in Group 2, the inspiration/expiration number rate was significantly lower in the patients who delivered in 24 h (0.98±0.2 vs. 1.25±0.57, p=0.015). By using fetal nasal Doppler, combination of absence of FBM or irregular FBM or regular FBM with inspiration number/expiration number (I/E) <1.25 detects 94.6% of patients who will eventually deliver in the first 24 h after admission. CONCLUSIONS: Examining FBM patterns and using nasal Doppler may help the clinician to differentiate those who will deliver preterm and may be an invaluable tool for managing preterm labor patients.

Alterations in forearm muscle activation patterns after scapholunate interosseous ligament injury: A dynamic electromyography study.

STUDY DESIGN: Case control. PURPOSE OF THE STUDY: This study aimed to investigate the alterations seen in the activation patterns of the forearm muscles and to demonstrate the associated functional outcomes, in patients with scapholunate interosseous ligament (SLIL) injury. METHODS: The study involved 15 patients with SLIL injury (instability group) and 11 healthy participants (control group). Both groups were evaluated with regard to their pain, grip strength, and upper extremity functional level (disabilities of the arm, shoulder and hand and patient-rated wrist evaluation questionnaires), and they also underwent a dynamic electromyography analysis of their forearm muscle activity. The activation patterns of the extensor carpi ulnaris (ECU), extensor carpi radialis (ECR), flexor carpi ulnaris, and flexor carpi radialis muscles during wrist extension and flexion were recorded by means of surface electromyography. RESULTS: In the instability group, the pain severity was higher and the functional level was worse than in the control group (P < .05). Furthermore, during wrist extension, the ECR activity was lower and the ECU activity was higher in the instability group than in the control group (P < .05). CONCLUSION: Dynamic stabilization of the wrist, flexor carpi ulnaris, and flexor carpi radialis muscles have been shown to play an active role with ECU and ECR. Increased ECU and decreased ECR activation may pose a potential risk in terms of enhancing the scapholunate gap. We, therefore, propose that appropriate preventive neuromuscular exercise strategies implemented as part of a physiotherapy program for patients with SLIL lesions might increase the contribution of the dynamic stability effect of the relevant muscles.

Clinical characteristics of children with congenital anomalies of the kidney and urinary tract and predictive factors of chronic kidney disease.

BACKGROUND: Congenital anomalies of kidney and urinary tract (CAKUT) are the leading causes of chronic kidney disease (CKD) in childhood. Determining the clinical course, outcome, and prognostic factors of this heterogeneous disease group is important to provide appropriate management and follow-up. Therefore, we aimed to identify the risk factors of CKD in CAKUT and the differences in clinical courses between disease subgroups. METHODS: Three hundred patients (M/F: 203/97) divided into 16 CAKUT categories were enrolled in the study. Logistic regression and survival analyses were performed to determine the risk factors for CKD that is defined as estimated GFR (eGFR) lower than 90 ml/min/1.73 m2 for at least 6 months. RESULTS: The median age of the study population at the time of the diagnosis was 0.6 years (IQR; 0.1-4.0 years). Among available prenatal diagnoses (n= 138), hydronephrosis (HN) (n= 83; 60.1%) and multicystic dysplastic kidney (MCDK) (n= 39; 28.2%) were the most frequently encountered ones. A total of 24 patients had CKD, and 13 of them (54.1%) progressed to end stage renal disease (ESRD). Patients with posterior urethral valve (PUV) had CKD and ESRD more frequently when compared to the other diagnostic groups (p < 0.001 for CKD, and p < 0.001 for ESRD). Furthermore, the PUV subgroup progressed to ESRD (median 3.63 years) earlier than the other subgroups. The diagnosis of PUV, proteinuria on the first admission, vesicoureteral reflux, and oligohydramnios were identified as independent predictors for CKD in the multivariate logistic regression analysis. CONCLUSIONS: Knowing predictive factors for CKD in patients with CAKUT is valuable for physicians in order to determine appropriate treatment strategies and prognosis.

COL4A3 mutation is an independent risk factor for poor prognosis in children with Alport syndrome.

BACKGROUND: Alport syndrome (AS) is an inherited glomerular disease caused by mutations in COL4A3, COL4A4, or COL4A5. Associations between clinical manifestations and genotype are not yet well defined. Our study aimed to define clinical and genetic characteristics, establish genotype-phenotype correlations, and determine prognosis of AS in children. METHODS: A total of 87 children with AS from 10 pediatric nephrology centers, whom had genetic analyses performed at the Hacettepe University Nephrogenetics Laboratory between February 2017 and February 2019, were included. Data regarding demographics, family history, clinical and laboratory characteristics, histopathological and genetic test results, treatments, and yearly follow-up results were retrospectively analyzed. RESULTS: Of 87 patients, 16% presented with nephrotic syndrome. In patients with nephrotic syndrome, kidney biopsy findings showed focal segmental glomerulosclerosis (FSGS) in 79%, and COL4A3 mutations were the leading genetic abnormality (50%). Twenty-four percent of all patients progressed to chronic kidney disease (CKD). The rate of progression to CKD and the decline in the glomerular filtration rate of the patients with COL4A3 mutation were higher than other mutation groups (p < 0.001 and p = 0.04, respectively). In kidney survival analysis, nephrotic syndrome presentation, histopathology of FSGS, COL4A3 mutations, and autosomal recessive inheritance were found as independent risk factors for earlier progression to CKD. Cyclosporin A treatment did not improve kidney survival. CONCLUSIONS: We emphasize that genetic testing is important for patients suspected as having AS. Furthermore, COL4A mutations should be considered in patients with FSGS and steroid-resistant nephrotic syndrome. This approach will shed light on the prognosis of patients and help with definitive diagnosis, preventing unnecessary and potentially harmful medications. Graphical abstract.

Quality of life study of patients with unresectable locally advanced or metastatic pancreatic adenocarcinoma treated with gemcitabine+nab-paclitaxel versus gemcitabine alone: AX-PANC-SY001, a randomized phase-2 study.

BACKGROUND: Combination of gemcitabine and nab-paclitaxel has superior clinical efficacy than gemcitabine alone. Nevertheless, health-related quality of life. (QoL) associated with this combination therapy when administered at first-line in advanced pancreatic adenocarcinoma is unknown. METHODS: A total of 125 patients were randomized to combination therapy (1000 mg/m2 gemcitabine + 125 mg/m2 nab-paclitaxel) and single-agent gemcitabine (1000 mg/m2) arms to take treatment weekly for 7 of 8 weeks, and following 3 of 4 weeks, until progression or severe toxicity. Primary endpoints were three-months of definitive deterioration free percent of patients, and QoL. RESULTS: Overall QoL analyses showed that 34 and 58.3% of cases in gemcitabine and gemcitabine+nab-P arms had no deterioration in 3rd month QoL scores (p = 0.018). These proportions were 27.3 and 36.6% in 6th month assessments, respectively (p = 0.357). Median overall survivals in combination and single-agent arms were 9.92 months and 5.95 months, respectively (HR: 0.64, 95% CI: 0.42-0.86, p = 0.038). Median progression free survivals in these treatment arms were 6.28 and 3.22 months, respectively (HR: 0.58, 95% CI: 0.39-0.87, p = 0.008). Median time-to-deterioration were 5.36 vs 3.68 months, and objective response rates were 37.1% vs 23.7% (p = 0.009), respectively in combination and single-agent arms. CONCLUSIONS: Combination therapy with gemcitabine + nab-paclitaxel had better overall and progression-free survival than gemcitabine alone. Also, combination therapy showed increased response rate without toxicity or deteriorated QoL. Combination treatment with gemcitabine and nab-paclitaxel may provide significant benefit for advanced pancreatic cancer. TRIAL REGISTRATION: This study has been registered in ClinicalTrials.gov as NCT03807999 on January 8, 2019 (retrospectively registered).

Infections in Pediatric Burn Patients: An Analysis of One Hundred Eighty-One Patients.

Background: Infectious complications are one of the most life-threatening complications and result in substantial mortality and morbidity in children who have been burned. The goal of the study is to assess the risk factors for sepsis in pediatric burn patients in a referral hospital. Methods: This study was performed at the Pediatric Burn Unit of Ankara Child Health and Diseases Hematology Oncology Training and Research Hospital during the period between January 2014 and June 2017. The patients were evaluated for age, sex, burn etiology, burned body surface area (BSA), the presence of inhalation injury, sepsis, positive cultures, the micro-organisms cultured samples, and septic focus. Results: A total of 181 patients were included in the study. The most common cause of burns was scalds in 120 patients (66.3%). Forty-one patients (22.7%) developed health-care-associated infection and sepsis. Gram-negative micro-organisms were isolated in 40 (97.6%) patients (Acinetobacter spp., Pseudomonas aeruginosa, Klebsiella pneumonia) with sepsis. Carbapenem resistance was detected in 31 (93.8%) of 40 patients. Mortality was observed in 11 patients (6.1%) in the group with sepsis. Burn surface area, burn depth, C-reactive protein (CRP) values, mortality, Garcés index, and Baux index were higher in the group with sepsis (p < 0.05). Multiple regression analysis revealed that mechanism of injury (flame), burned BSA ≥25%, C-reactive protein ≥6 mg/dL (area under the curve [AUC]: 0.76 p < 0.001 and AUC: 0.90, p < 0.001, respectively) at admission were independent parameters for development of sepsis in pediatric burn patients. Conclusion: Multi-drug-resistant Pseudomonas aeruginosa and Acinetobacter baumannii were important agents of blood stream infection in burned children. Burned BSA ≥25% and CRP ≥6 mg/dL were risk factors for developing sepsis in pediatric burn patients.