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.

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.

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).

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.

Reproductive cancer risk factors among relatives of cancer patients in a tertiary oncology center.

BACKGROUND: The aim of this study was to evaluate the prevalence of some gynecological cancer risk factors in a population of female relatives of cancer patients in Hacettepe University Oncology Hospital. Additionally, what are the levels of the women's awareness /behavior toward available screening tools? METHODS: An individual cancer risk assessment questionnaire has been developed in the Department of Preventive Oncology, which questions the medical history, health behaviors and cancer awareness, as well as their behavior toward available cancer screening tools. RESULTS: The mean age of the study population was 45.7 ± 12.2 years. Median age at menarche was 13 years (IQR, 12-14), 6.9% of the women reported their menarche was before age of 12. About 11.1% of the women had intercourse before age of 18. The median age at first delivery was 22 years. Median BMI was 24.9, with 18.3% of population having obesity. Of the women 65% were current/past smokers. Sixty-two percent of the women had never used condom. About 8% of the women were unaware about mammography and 17.7% about the Pap test. CONCLUSIONS: This study has documented high tobacco use, low protective condom use and low rates of physical activity. Percentage of some risk factors like early menarche was lower than what was suggested for general Turkish population. Awareness and behavior of the women were better about mammography when compared to the Pap test. Considering our results, some measures should be put in place to increase people's awareness, and to modify their behavior toward cancer prevention.

Smoke-free Turkey: Evaluation of outdoor areas of public places.

SIGNIFICANCE: Secondhand tobacco smoke (SHS) exposure is a major cause of morbidity and mortality around the world. The objective of this study was to evaluate the presence of smoking in outdoor areas of public places in three largest Turkish cities (Istanbul, Ankara, and Izmir). METHOD: For this cross-sectional observational study, the Turkish Statistical Institute randomly selected 10 sampling points in each city. Around each sampling point, fieldworkers visited the closest bars/nightclubs, cafes, government buildings, hospitals, restaurants, schools, shopping malls, traditional coffee houses, universities, children's playgrounds, parks and open markets. We observed smoking, ashtrays, and cigarette butts at the outdoor areas of public venues within the urban districts of each city. The fieldwork was conducted in April-May 2016. RESULTS: 477 venues were observed, covering 1017 outdoor locations in which 17,737 people were observed. Smoking in outdoor areas ranged from 3.7% around schools to 90% in open markets. Ashtrays were almost ubiquitous in hospitals (95.6%), shopping malls (92.0%), and universities (90.9%). Cigarette butts were more often observed in open markets (100%), shopping malls (96%), universities (95.5%), and parks (93.3%). Smoking at outdoor areas around schools was significantly lower than around other venues. CONCLUSION: Smoking in outdoor areas was common in most public places in Turkey except schools. The current indoor SHS legislation should be extended to cover adjacent outdoor areas of public venues in order to effectively protect people from SHS exposure in Turkey.

Cancer incidence in familial Mediterranean fever patients: a retrospective analysis from central Anatolia.

Although chronic inflammation has been associated with increased cancer risk in various disease including hepatitis or inflammatory bowel disease, a lower incidence of cancer has been reported recently in familial Mediterranean fever (FMF) which is an auto-inflammatory disease with persistent inflammation. We have assessed cancer incidence among FMF patients with or without amyloidosis to investigate this hypothesis. We performed a retrospective review of FMF patients, diagnosed and treated in Hacettepe University hospitals between 2001 and 2018. We identified patients from the hospital medical records using the ICD-10 code for FMF. We collected data on demographic and clinical features, drug history, the presence of amyloidosis and subsequent diagnosis of cancer. The expected cancer incidence was estimated using age- and gender-specific standardized incidence rates (SIRs) in comparison with the general Turkish population according to Turkish National Cancer Registry data at 2014. Total of 3899 FMF patients (120 patients had also amyloidosis) were included. Median age was 22 and 56% were females. Thirty-eight patients were diagnosed with cancer during 100,283 person-years of follow-up. The most common cancer was breast cancer in females (7/28 patients) and leukemia (2/10 patients) in males. The overall cancer incidence among patients with FMF was significantly lower in both males {SIR 0.42 [95% confidence interval; (CI) 0.21-0.75], p = 0.019} and females [SIR 065 (95% CI 0.44-0.93), p = 0.002]. The overall cancer incidence among patients with FMF and amyloidosis was [SIR 1.21 (95% CI 0.49-2.52), p = 0.73] without gender difference. Cancer incidence was significantly lower in FMF patients compared with the general Turkish population. We found no increased cancer incidence in FMF patients having amyloidosis. Possible underlying mechanisms need to be explained.

Evaluation of cancer risk in patients with periodontal diseases

Background/aim: In this study, we aimed to assess the cancer risk among patients with periodontal disease. Materials and methods: Patients diagnosed with periodontal diseases at Hacettepe University between 2007 and 2012 were included and data on the diagnosis of any cancer after periodontal disease were collected from patient files. The age- and sex-standardized incidence rates (SIRs) were calculated using Turkish National Cancer Registry 2013 data. Results: A total of 5199 patients were included. Median follow-up was 7.2 years. Patients with periodontal diseases had 17% increased risk of cancer compared with the expected counts for the corresponding age and sex groups (SIR: 1.17; 95% CI: 1.04­1.3, P = 0.006). The increased cancer risk was statistically significant in women (SIR: 1.24; 95% CI: 1.05­1.45, P = 0.008) but not in men. Among women with periodontal disease, the risks of breast cancer (SIR: 2.19) and head and neck cancer (SIR: 4.71) were significantly increased. Among men, the risks of prostate cancer (SIR: 1.84), head and neck cancer (SIR: 3.55), and hematological cancers (SIR: 1.76) were significantly increased. Conclusion: This study showed that periodontal diseases were associated with increased risk of several cancers. Besides other well-known benefits for health, the provision of oral/dental health should be considered and employed as a cancer prevention measure.

Extra-Renal manifestations of atypical hemolytic uremic syndrome in children.

BACKGROUND: Atypical hemolytic uremic syndrome (aHUS) is a chronic disease characterized by thrombotic microangiopathy and a high risk of end-stage kidney disease. Dysregulation and/or excessive activation of the complement system results in thrombotic microangiopathy. Interest in extrarenal manifestations of aHUS is increasing. This study aimed to determine the clinical characteristics of patients with extrarenal manifestations of aHUS in childhood. METHODS: This study included 70 children with extrarenal manifestations of HUS from the national Turkish aHUS Registry. The demographics, clinical characteristics, genetic test results, all treatments, and renal/hematologic status of aHUS patients with extrarenal involvement were recorded. RESULTS: The most common extrarenal manifestation was neurological system involvement (n = 46 [27.2%]), followed by gastrointestinal (n = 20 [11.8%]), cardiovascular (n = 12 [7%]), and respiratory (n = 12 [7%]) involvement. The patients with neurological involvement had a higher mortality rate and a lower estimated glomerular filtration rate (eGFR) than the other patients at last follow-up. Eculizumab (with or without plasma exchange/plasma infusion) treatment increased the renal and hematologic recovery rates. CONCLUSIONS: The most common and serious extrarenal manifestation of aHUS is neurological involvement and treatment outcome findings presented herein are important to all relevant clinicians.

Distribution of lymphomas in Turkey: data of 4239 cases from a single institution using the WHO classification

Background/aim: Lymphoma cases diagnosed at one of the largest tertiary reference centers in Turkey were reviewed and findings were compared to those reported from other regions of the world. Materials and methods: Lymphomas diagnosed between 2000 and 2017 in the pathology laboratory of Hacettepe University were identified. A total of 4239 cases were analyzed. The WHO 2008 classification was used. Results: Hodgkin lymphomas accounted for almost 20% of cases. T-cell lymphomas were much more frequent (23% of our non- Hodgkin lymphoma (NHL) cases) in comparison to all other regions of the world. The reason for this difference was the high frequency of mycosis fungoides (MF) cases. We had significantly more cases of high-grade B-cell lymphoma (43.9% of NHLs) and fewer cases of low-grade B-cell lymphoma (33.5% of NHLs) in comparison to the rates of developed regions of the world and the reverse was true when compared to developing parts of the world. Burkitt lymphoma frequency (4% of NHLs) was also higher than in most parts of the world. Conclusion: Our data reveal that the frequency of MF, Burkitt lymphoma, and Hodgkin lymphoma are considerably higher, whereas follicular lymphoma rates are considerably lower than in most other parts of the world.

Ezrin Inhibition Up-regulates Stress Response Gene Expression.

Ezrin is a member of the ERM (ezrin/radixin/moesin) family of proteins that links cortical cytoskeleton to the plasma membrane. High expression of ezrin correlates with poor prognosis and metastasis in osteosarcoma. In this study, to uncover specific cellular responses evoked by ezrin inhibition that can be used as a specific pharmacodynamic marker(s), we profiled global gene expression in osteosarcoma cells after treatment with small molecule ezrin inhibitors, NSC305787 and NSC668394. We identified and validated several up-regulated integrated stress response genes including PTGS2, ATF3, DDIT3, DDIT4, TRIB3, and ATF4 as novel ezrin-regulated transcripts. Analysis of transcriptional response in skin and peripheral blood mononuclear cells from NSC305787-treated mice compared with a control group revealed that, among those genes, the stress gene DDIT4/REDD1 may be used as a surrogate pharmacodynamic marker of ezrin inhibitor compound activity. In addition, we validated the anti-metastatic effects of NSC305787 in reducing the incidence of lung metastasis in a genetically engineered mouse model of osteosarcoma and evaluated the pharmacokinetics of NSC305787 and NSC668394 in mice. In conclusion, our findings suggest that cytoplasmic ezrin, previously considered a dormant and inactive protein, has important functions in regulating gene expression that may result in down-regulation of stress response genes.

Turkish pediatric atypical hemolytic uremic syndrome registry: initial analysis of 146 patients.

BACKGROUND: Atypical hemolytic uremic syndrome (aHUS) is a devastating disease with significant morbidity and mortality. Its genetic heterogeneity impacts its clinical presentation, progress, and outcome, and there is no consensus on its clinical management. METHODS: To identify the characteristics of aHUS in Turkish children, an industry-independent registry was established for data collection that includes both retrospective and prospective patients. RESULTS: In total, 146 patients (62 boys, 84 girls) were enrolled; 53 patients (36.3%) were less than 2 years old at initial presentation. Among the 42 patients (37.1%) whose mutation screening was complete for CFH, CFI, MCP, CFB, C3, DGKE, and CHFR5 genes, underlying genetic abnormalities were uncovered in 34 patients (80.9%). Sixty-one patients (41.7%) had extrarenal involvement. During the acute stage, 33 patients (22.6%) received plasma therapy alone, among them 17 patients (51.5%) required dialysis, and 4 patients (12.1%) were still on dialysis at the time of discharge. In total, 103 patients (70.5%) received eculizumab therapy, 16 of whom (15.5%) received eculizumab as a first-line therapy. Plasma therapy was administered to 84.5% of the patients prior to eculizumab. In this group, renal replacement therapy was administered to 80 patients (77.7%) during the acute period. A total of 3 patients died during the acute stage. A total of 101 patients (77.7%) had a glomerular filtration rate >90 mL/min/1.73 m2 at the 2-year follow-up. CONCLUSIONS: The Turkish aHUS registry will increase our knowledge of patients with aHUS who have different genetic backgrounds and will enable evaluation of the different treatment options and outcomes.

Estimation of fetal weight by ultrasonography after preterm premature rupture of membranes: comparison of different formulas.

OBJECTIVE: To compare different ultrasonographic fetal weight estimation formulas in predicting the fetal birth weight of preterm premature rupture of membrane (PPROM) fetuses. METHODS: Based on the ultrasonographic measurements, the estimated fetal weight (EFW) was calculated according to the published formulas. The comparisons used estimated birth weight (EBW) and observed birth weight (OBW) to calculate the mean absolute percentage error [(EBW-OBW)/OBW×100], mean percentage error [(EBW-OBW)/OBW×100)] and their 95% confidence intervals. RESULTS: There were 234 PPROM patients in the study period. The mean gestational age at which PPROM occured was 31.2±3.7 weeks and the mean gestational age of delivery was 32.4±3.2 weeks. The mean birth weight was 1892±610 g. The median absolute percentage error for 33 formulas was 11.7%. 87.9% and 21.2% of the formulas yielded inaccurate results when the cut-off values for median absolute percentage error were 10% and 15%, respectively. The Vintzileos' formula was the only method which had less than or equal to 10% absolute percentage error in all age and weight groups. CONCLUSIONS: For PPROM patients, most of the formulas designed for sonographic fetal weight estimation had acceptable performance. The Vintzileos' method was the only formula having less than 10% absolute percentage error in all gestational age and weight groups; therefore, it may be the preferred method in this cohort. Amniotic fluid index (AFI) before delivery had no impact on the performance of the formulas in terms of mean percentage errors.

Evaluation of complementary and alternative medicine trials registered in clinicaltrials.gov database.

PURPOSE: Complementary and alternative medicine (CAM) products are increasingly used because they are perceived as natural, relatively low-cost and probably effective therapies for various diseases including cancer. We aimed to determine the quantity and major characteristics of recent herbal/alternative medicine trials registered in clinicaltrials. gov in patients with cancer. METHODS: "Cancer AND (herbal OR complementary OR alternative)" key words were used to query clinicaltrials. gov (access date 17 April 2015). From the results, 163 trials which have been conducted in patients with the diagnosis of cancer were identified and included in this analysis. RESULTS: At the date of access, 72 trials were completed, 37 trials were still recruiting patients and 10 trials had been withdrawn. Most common cancer type was breast cancer. Eighty-eight percent of trials were interventional and 60% of trials were randomized. The rate of new trial submission were similar for 5-year periods after 2000. The majority of the trials were conducted in United States of America (55%) and People's Republic of China (11%). Nine and 4 of 37 recruiting trials were recorded as phase II and phase III, respectively. When browsing was restricted to "recruiting" and "interventional" studies, the ratio of herbal/complementary treatment trials to all chemotherapy trials was 1.8 %. CONCLUSION: CAM research in patients with cancer is currently limited, both in terms of quantity and quality. Until high quality scientific and clinical research establishes safety and efficacy of CAM practices, physicians should rigorously inform patients and the public on potential risks and caveats associated with CAM practices.

Response rate of initial conventional treatments, disease course, and related factors of patients with adult-onset Still's disease: Data from a large multicenter cohort.

BACKGROUND: Adult-onset Still's disease (AOSD) is a rare condition, and treatment choices are frequently dependent on expert opinions. The objectives of the present study were to assess treatment modalities, disease course, and the factors influencing the outcome of patients with AOSD. METHODS: A multicenter study was used to reach sufficient patient numbers. The diagnosis of AOSD was based on the Yamaguchi criteria. The data collected included patient age, gender, age at the time of diagnosis, delay time for the diagnosis, typical AOSD rash, arthralgia, arthritis, myalgia, sore throat, lymphadenopathy, hepatomegaly, splenomegaly, pleuritis, pericarditis, and other rare findings. The laboratory findings of the patients were also recorded. The drugs initiated after the establishment of a diagnosis and the induction of remission with the first treatment was recorded. Disease patterns and related factors were also investigated. A multivariate analysis was performed to assess the factors related to remission. RESULTS: The initial data of 356 patients (210 females; 59%) from 19 centers were evaluated. The median age at onset was 32 (16-88) years, and the median follow-up time was 22 months (0-180). Fever (95.8%), arthralgia (94.9%), typical AOSD rash (66.9%), arthritis (64.6%), sore throat (63.5%), and myalgia (52.8%) were the most frequent clinical features. It was found that 254 of the 306 patients (83.0%) displayed remission with the initial treatment, including corticosteroids plus methotrexate with or without other disease-modifying antirheumatic drugs. The multivariate analysis revealed that the male sex, delayed diagnosis of more than 6 months, failure to achieve remission with initial treatment, and arthritis involving wrist/elbow joints were related to the chronic disease course. CONCLUSION: Induction of remission with initial treatment was achieved in the majority of AOSD patients. Failure to achieve remission with initial treatment as well as a delayed diagnosis implicated a chronic disease course in AOSD.

Compliance with smoke-free legislation within public buildings: a cross-sectional study in Turkey.

OBJECTIVE: To investigate public compliance with legislation to prohibit smoking within public buildings and the extent of tobacco smoking in outdoor areas in Turkey. METHODS: Using a standardized observation protocol, we determined whether smoking occurred and whether ashtrays, cigarette butts and/or no-smoking signs were present in a random selection of 884 public venues in 12 cities in Turkey. We visited indoor and outdoor locations in bars/nightclubs, cafes, government buildings, hospitals, restaurants, schools, shopping malls, traditional coffee houses and universities. We used logistic regression models to determine the association between the presence of ashtrays or the absence of no-smoking signs and the presence of individuals smoking or cigarette butts. FINDINGS: Most venues had no-smoking signs (629/884). We observed at least one person smoking in 145 venues, most frequently observed in bars/nightclubs (63/79), hospital dining areas (18/79), traditional coffee houses (27/120) and government-building dining areas (5/23). For 538 venues, we observed outdoor smoking close to public buildings. The presence of ashtrays was positively associated with indoor smoking and cigarette butts, adjusted odds ratio, aOR: 315.9; 95% confidence interval, CI: 174.9-570.8 and aOR: 165.4; 95% CI: 98.0-279.1, respectively. No-smoking signs were negatively associated with the presence of cigarette butts, aOR: 0.5; 95% CI: 0.3-0.8. CONCLUSION: Additional efforts are needed to improve the implementation of legislation prohibiting smoking in indoor public areas in Turkey, especially in areas in which we frequently observed people smoking. Possible interventions include removing all ashtrays from public places and increasing the number of no-smoking signs.

Factors that influence attitude and enforcement of the smoke-free law in Turkey: a survey of hospitality venue owners and employees.

INTRODUCTION: In 2009, Turkey extended the smoke-free legislation to hospitality venues. Compliance, however, remains low in some hospitality venues. We identified characteristics associated with knowledge of health effects that can be prevented by the smoke-free law, the attitude towards and enforcement of the law. METHODS: In 2014, we conducted 400 interviews with hospitality venue owners and employees in 7 cities in Turkey. The venues were identified based on a random sampling strategy in a previous phase of the study. RESULTS: Over one-third (37.3%) of hospitality owners and employees had adequate knowledge of the health effects from secondhand smoke (SHS), 71.3% had a positive attitude towards the law and 19.5% had personally enforced the law. Participants who worked 70 hours or more per week were more likely to have a positive attitude towards the law. Older individuals, women, participants working in bars/nightclubs, venue owners receiving fines for non-compliance and current smokers were less likely to have a positive attitude towards the law. Participants working in traditional coffee houses, former smokers, and participants with a high school education or greater were more likely to enforce the law. Smokers who quit or reduced smoking because of the law were more likely to enforce the law compared with those who were not influenced by the law. CONCLUSIONS: Although the attitude towards the law was positive, interventions are needed to increase knowledge on the health effects of SHS and facilitate enforcement of the law, particularly among subgroups less likely to have a positive attitude and enforce the law.

Development and reliability of a Turkish version of the Short Form-Joint Protection Behavior Assessment (JPBA-S).

STUDY DESIGN: Clinical measurement. PURPOSE: To adapt the original JPBA-S to a Turkish version (TUR-JPBA-S) and to investigate its reliability in assessing patients with rheumatoid arthritis (RA). METHODS: Twenty-two participants with RA and 21 healthy people were videotaped while performing tasks listed in the TUR-JPBA-S. Two raters scored the video recordings for to evaluate inter-rater reliability. One rater re-analyzed the recordings at a different time point for intra-rater reliability. Participants with RA were asked to perform the same tasks after three to four weeks which was also recorded to evaluate test-retest reliability. RESULTS: Internal consistency (Cronbach's α value) was found to be high (0.89) for participants with RA. Our results demonstrate excellent intra-rater (ICC: 0.99, SEM 1.2) inter-rater (ICC: 0.99, SEM 1.7) reliability, apart from excellent test-retest reliability (ICC: 0.96). CONCLUSION: The TUR-JPBA-S is a valid and reliable instrument for assessing JP behavior in patients with RA in Turkey. LEVEL OF EVIDENCE: Level 2.