Immediate Hypersensitivity Reactions to Biologic Drugs in Children with Inflammatory Bowel Diseases.

INTRODUCTION: The increased use of biologic drugs (BDs) may lead to an increase in immediate hypersensitivity reactions (I-HSRs) in pediatric patients with inflammatory bowel disease (IBD). Our aim was to assess I-HSRs to BDs in pediatric IBD, examining frequency, clinical features, management, and associated risk factors. METHODS: All children diagnosed with IBD at our institution between January 1, 2006, and August 1, 2023, and who developed I-HSRs related to any BD were included in the study. RESULTS: In a study of 197 pediatric IBD patients, 61 used BD. Among these, 52.4% were male, with a median diagnosis age of 145 months (13-215). Out of a total of 1,679 administrations, 6 patients developed I-HSRs (5 with infliximab, 1 with adalimumab), resulting in a frequency of 9.8% per patient and 0.36% per administration. Of these, 66.7% were cases of Type 1 HSRs (skin test positivity n = 1), while the rest were infusion-related reactions (anti-drug antibody positivity n = 4), all of which were mild to moderate in severity. In the age and gender-adjusted logistic regression model, the presence of any comorbid allergic disease was significantly associated with the occurrence of I-HSR (aOR = 8.35; 95% CI = 1.24-56.38; p = 0.029). CONCLUSION: The frequency of I-HSRs to BDs in children with IBD is not rare but not severe in the long term. The presence of any comorbid allergic disease is a risk factor for the development of I-HSRs to BDs.

Lower Body Mass Index and Prognostic Nutritional Index Are Associated with Poor Posttransplant Outcomes in Lymphoma Patients Undergoing Autologous Stem Cell Transplantation.

INTRODUCTION: Pretransplant inflammatory and nutritional status has not been widely explored in terms of its impact on autologous hematopoietic stem cell transplantation (auto-HSCT) outcomes in lymphoma patients. We aimed to evaluate the impact of body mass index (BMI), prognostic nutritional index (PNI), and C-reactive protein to albumin ratio (CAR) on auto-HSCT outcomes. METHODS: We retrospectively analyzed 87 consecutive lymphoma patients who underwent their first auto-HSCT at the Adult Hematopoietic Stem Cell Transplantation Unit at Akdeniz University Hospital. RESULTS: The CAR had no impact on posttransplant outcomes. PNI ≤50 was an independent prognostic factor for both shorter progression-free survival (PFS) (hazard ratio [HR] = 2.43, p = 0.025) and worse overall survival (OS) (HR = 2.93, p = 0.021), respectively. The 5-year PFS rate was significantly lower in patients with PNI ≤50 than in patients with PNI >50 (37.3% vs. 59.9%, p = 0.003). The 5-year OS rate in patients with PNI ≤50 was significantly low when compared with patients who had PNI >50 as well (45.5% vs. 67.2%, p = 0.011). Patients with BMI <25 had higher 100-day transplant-related mortality compared with patients with BMI ≥25 (14.7% vs. 1.9%, p = 0.020). BMI <25 was an independent prognostic factor associated with shorter PFS and OS (HR = 2.98 [p = 0.003], HR = 5.06 [p < 0.001], respectively). The 5-year PFS rate was significantly lower in patients with BMI <25 than patients with BMI ≥25 (40.2% vs. 53.7%, p = 0.037). Similarly, the 5-year OS rate in patients with BMI <25 was significantly inferior compared to patients with BMI ≥25 (42.7% vs. 64.7%, p = 0.002). CONCLUSION: Our study confirms that lower BMI and CAR have negative impacts on auto-HSCT outcomes in lymphoma patients. Furthermore, higher BMI should not be considered an obstacle for lymphoma patients who need auto-HSCT; conversely, it could be an advantage for posttransplant outcomes.

Long-Term Experience with Anaphylaxis and Desensitization to Alglucosidase Alfa in Pompe Disease.

BACKGROUND AND OBJECTIVE: Pompe disease (PD) is an inherited lysosomal storage disease that progresses with glycogen accumulation in many tissues, due to the deficiency of the acid-alpha glucosidase enzyme. Recombinant alglucosidase alfa (rhGAA) is the only disease-specific treatment option, in the form of enzyme replacement therapy (ERT). Anaphylaxis can develop with rhGAA. There is no study evaluating anaphylaxis and its management in PD in the long term. We aimed to evaluate the development of anaphylaxis and rapid drug desensitization (RDD) with rhGAA in children with PD. MATERIALS AND METHODS: All children diagnosed and followed up in our institution with PD over 12 years between January 2009 and September 2021 were evaluated for development of anaphylaxis and RDD with rhGAA from medical records. RESULTS: Fourteen patients, 64% of whom were female and diagnosed with PD (1 juvenile, 13 infantile types) during the study period included in the study. The median age at diagnosis was 3.2 months (1-40 months). The median follow-up time of the patients was 20 months (1-129 months). Thirteen patients were given rhGAA, one died before ERT. Four (30.8%) patients developed moderate to severe anaphylaxis, and RDD was applied with rhGAA. A total of 390 RDDs have been performed so far without any serious breakthrough reactions during all RDDs. CONCLUSIONS: Anaphylaxis with rhGAA is not rare and RDD with rhGAA is safe and effective in the long term.

A novel pediatric Eosinophilic Esophagitis Symptoms and Adaptive Behavior Scale for different ages: GaziESAS v2.0.

BACKGROUND: High-quality scales (HQS) suitable that measure symptoms and adaptive behaviors (AB) with proven validity and reliability are needed for different age groups of children with eosinophilic esophagitis (EoE). OBJECTIVE: To develop a high-quality pediatric EoE symptoms and AB scale for different age groups. METHODS: Children (7-11 years), teens (12-18 years), and parents of 2-18-year-old children with EoE were included. A HQS should have encompassed: the identification of domain and item generation; content validity (CnV) and field test for construct validity (CsV) and reliability. Convergent validity (CgV) was examined for CsV. Correlations between the Pediatric Eosinophilic Esophagitis Symptom Score, version 2.0 (PEESS v2.0) and Gazi University Eosinophilic Esophagitis Symptoms and Adaptive Behavior Scale (GaziESAS) version 2.0 (v2.0) were examined for CgV. Reliability was determined through internal consistency (Cronbach-α) and test-retest reliability (intraclass correlation coefficients: ICC). RESULTS: Nineteen children, 42 teens, and 82 parents completed the study. GaziESAS v2.0 was composed of 20 items with two main domains: symptoms (subdomains: dysphagia and nondysphagia) and AB. CnV indexes were excellent for all items. The CgV varied from good to excellent correlation (r = 0.6 to r = 0.9). GaziESAS v2.0 showed good reliability (Cronbach-α >0.7 and ICC >0.6). CONCLUSION: GaziESAS v2.0 is the first pediatric HQS that measures the frequency of symptoms and AB in EoE within the last month with separate forms for children, teens, and parents.

Comparison of three methods for sealing of the intramedullary femoral canal during total knee arthroplasty; a randomized controlled trial.

PURPOSE: The purpose of this randomized controlled study was to examine the effect of sealing the intramedullary canal with a bone or cement plug or leaving it empty on postoperative bleeding and pain MATERIALS AND METHODS: A total of 120 patients with knee osteoarthritis who underwent unilateral TKA participated in the trial and were assigned to one of three groups. The femoral canal was sealed with an autogenous bone plug (Group I) or cement plug (Group II), or it was left open (Group III). Estimated blood loss, Hemoglobin decline, bleeding into the drain, and postoperative pain w compared between groups. RESULTS: Six patients were excluded due to various reasons, and the remaining 114 patients were included in the final analysis. There were no significant variations in baseline clinical characteristics between the three groups (p > 0.05). Hemoglobin reduction between preoperative and 72 h after the surgery (p: 0.034) and estimated blood loss (p: 0.003) were significantly different between groups. The cement plug group showed the least bleeding. Although there was a significant difference between the cement and empty canal groups (p: 0.03 and p: 0.002, respectively), the difference between the cement and bone groups was similar regarding both hemoglobin reduction and estimated blood loss. The blood volume in the suction drain (p: 0.598) and transfusion rate (p: 0.087) were similar between the groups. VAS at the 12 h after the surgery was similarly high in each group (p: 0.676). It declined at 36 h after surgery, but no significant difference was determined between groups (p: 0.815). CONCLUSIONS: This study showed that estimated blood loss and hemoglobin reduction were significantly lower in the cement plug group than in the empty canal group. But bone plug group did not show any difference with both empty canal and cement plug groups. Sealing the IM canal or leaving it open did not change the bleeding into the drain, transfusion rate, and postoperative pain between groups. Based on these findings, sealing the IM canal with a cement plug might be recommended to diminish bleeding during TKA despite similar transfusion rates. LEVEL OF EVIDENCE: Level I, randomized controlled trial.

Is the Pediatric Eosinophilic Esophagitis Symptom Score v2.0 reliable for telemedicine?

BACKGROUND: Eosinophilic esophagitis (EoE) is a chronic immune-mediated disease. Telemedicine is a healthcare technology used when a patient is separated by distance. The reliability of the Pediatric Eosinophilic Esophagitis Symptom Score, version 2.0 (PEESS v2.0) for telemedicine applications, has not been studied yet. Therefore, we aimed to evaluate the reliability of PEESS v2.0 for telemedicine. METHODS: We sent a telesurvey using questionnaires via electronic telecommunication as the telemedicine method. Children with EoE and their parents were asked to complete PEESS v2.0 with the telesurvey method (unsynchronized with the physician) and attend in-person visits one week apart. Intraclass correlation (ICC), Wilcoxon, and Bland-Altman tests were used as reliability analyses. Reliability was defined as a strong agreement between the measurements in ICC ≥ 0.8 and a p-value of ≤0.05 and no statistically significant difference between the scores of the two methods in the Wilcoxon and Bland-Altman analyses, i.e. a p-value of >0.05. RESULTS: The total scores of children and parents were higher in in-person visits than in the telesurvey (Wilcoxon tests, p ≤ 0.05). Bland- Altman analysis showed that the mean difference in total scores between the two methods was significant for both children and parents (p ≤ 0.05). ICC levels for the children and parent scores for the entire group ranged from 0.595 to 0.763 (moderate agreement). DISCUSSION: Unsynchronized telesurvey use of PEESS v2.0 is unreliable both for children and parents. We suggest testing the reliability of chosen telemedicine methods before using them in clinical and research practice.

Neuropsychiatric adverse drug reactions induced by montelukast impair the quality of life in children with asthma.

INTRODUCTION: Montelukast-induced neuropsychiatric adverse drug reactions (ADRs) have been reported in retrospective studies. This study aimed to reveal the neuropsychiatric ADRs triggered in patients taking montelukast due to asthma in real time, and to evaluate the effect of these ADRs on quality of life (QoL). METHODS: Patients, ages 3-18 years, taking montelukast for the first time and their parents were included. Ages 3-7 years were defined as the preschool and ages 8-18 years as the school-age group. At the beginning of the study and at the end of the second week of treatment, the neuropsychiatric complaint assessment questionnaire and the KINDL QoL scale were administered to patients and their parents. The effect of ADRs on the decrease in QoL was evaluated by multivariable logistic regression. RESULTS: Neuropsychiatric ADRs were reported in 78 (62.4%) of 125 patients, who recovered when the drug was discontinued. Temperamental behavior, nightmares and sleep disorders occurred significantly more often in both groups compared with pretreatment (p < 0.001 for each). In both groups, except in the child-reported family relationships subscale in the school-age group, significant decreases were found in both child and parent proxy-reported QoL total/sub-scores compared with pretreatment (p˂0.001 for each). It was found in the evaluation that the overall QoL of those experiencing ADRs in both age groups was more affected. (Child-reported QoL ORpreschool age=2.66, p = 0.048; ORschool-age=5.95, p = 0.027; parent-proxy QoL ORpreschool age =3.52, p = 0.010, ORschool-age=6.43, p = 0.027). CONCLUSIONS: Montelukast-induced neuropsychiatric ADRs are more frequent than reported in the literature and negatively impact children's QoL.

Anatomic risk factors for the occurrence of medial talar osteochondral lesions: a case-control study.

OBJECTIVE: This study aimed to determine the anatomical risk factors that may play a role in the etiology of medial-sided osteochondral lesions of the talus (OLT) using morphological parameters in magnetic resonance imaging (MRI). SUBJECTS AND METHODS: One hundred twenty-four patients with medial-sided OLT and age- and sex-matched 124 controls were included in this retrospective study. Two examiners conducted independent OLT classification and measurements of five MRI parameters: tibial axis-medial malleolus angle (TMM), the anterior opening angle of the talus (AOT), talus position (TalPos), the ratio of the distal tibial articular surface to the length of the trochlea tali arc (TAS/TAL), depth of the incisura fibularis (IncDep). Statistical analysis included intraclass correlation coefficients, independent t-tests, receiver-operating characteristic (ROC) analysis, area under the curve (AUC) calculation, and logistic regression analysis. A p-value < 0.05 was considered statistically significant. RESULTS: TTM, AOT, TalPos, and TAL values were significantly higher and the TAS/TAL ratio was significantly lower in the case group than in the control group (p < 0.001). Cut-off and AUC values for TMM were 15.15° (AUC 0.763), AOT 13.05° (AUC 0.826), TalPos 0.75 mm (AUC 0.887), TAL 35.45 mm (AUC 0.642), and TAS/TAL ratio 0.82 (AUC 0.784), p < 0.001. Multivariate logistic regression analysis results were odds ratio (OR) = 6.1 for TMM ≥ 15.15°, OR = 8.9 for AOT ≥ 13.05°, OR = 36.1 for TalPos ≥ 0.75 mm, and OR = 6.7 for TAS/TAL ratio ≤ 0.82. CONCLUSION: Ankle morphology might have an influence on OLT development. The talus position (TalPos) and anterior opening angle of the talus (AOT) seemed to be the strongest predisposing factors.

Comparison of ESHG2016 and AAP2017 hypertension guidelines in adolescents between the ages of 13 and 16: effect of body mass index on guidelines.

OBJECTIVE: The diagnosis of hypertension in adolescents aged ≥13 and <16 years is based on the percentile according to age, gender, and height in the European Society of Hypertension guidelines guideline; whereas, the American Academy of Pediatrics guideline uses blood pressure above 130/80 mmHg as a single criterion. Therefore, this study aimed to evaluate the compatibility of these two guidelines in adolescents aged ≥13 and <16 years. METHODS: This study was designed by retrospectively screening the records of 395 adolescents with both office and 24-hour ambulatory blood pressure measurements. Each blood pressure measurement was classified according to both the ESGH2016 and AAP2017 guidelines. Patients were divided into three subgroups according to body mass index. Cohen's kappa analysis was used to evaluate the agreement between the two guidelines. RESULTS: The majority of adolescents were normotensive according to both guidelines, 55.9% by ESHG2016 and 43.1% by AAP2017. For the whole group, the frequency of hypertension was 32.4% with ESHG2016 and 34.4% with AAP2017; while, in obese patients, hypertension frequencies were 38.8% and 43.3%, respectively. The diagnosis of hypertension was demonstrated with the two guidelines, and there was significant agreement at a substantial level, both for the obese subgroup and the whole study group (kappa value = 0.738 and 0.785, respectively). The frequency of white-coat hypertension was higher with the AAP2017 guideline (28.1% versus 16.2%, p < 0.001). CONCLUSION: With our experience in this single-centre study, it seems that both the AAP2017 and the ESHG2016 guidelines can be used in the diagnosis of hypertension in adolescents.

Is there still a necessity for awareness and information about HPV infection and vaccine for adolescent in developing countries?

Increasing awareness of human papilloma virus (HPV) and vaccines among the target population is an important factor for decreasing the rate of malignancies related to HPV. The aim of this cross-sectional study was to determine Turkish high school and college students' knowledge level of HPV and vaccines via a survey. The HPV knowledge scale (HPV-KS) has been developed to measure the level of knowledge about HPV and vaccination. The response rate of 'Yes' to the question of whether the participants have heard of HPV and the vaccine was 60.8% and 58.8%, respectively. The lack of information was the commonest reason for not being vaccinated in 54% of the participants. According to the HPV-KS, the median value of the knowledge level of the participants was 35.7 (IQR; 14.3-57.1). The HPV-KS scores were significantly higher among those with parents who had graduated from university and had an income above the minimum wage than those who did not (p = .013 and p = .029, respectively). Current evidence suggests that Turkish adolescents have limited HPV knowledge. Gender, age, education (particularly university and health-related departments), parents' level of education and income were all important factors in determining the outcome variables. Therefore, it is very important to apply educational programs to raise awareness by medical staff.Impact StatementWhat is already known on this subject? Human papilloma virus (HPV) infection is a major threat due to the reason of many malignancies, especially cervical cancer, and this problem can be overcome by informing the target population about the screening program and vaccination.What do the results of this study add? Lack of knowledge about HPV infection and cost of the vaccine were the main reasons for insufficient vaccination rate among the adolescents.What are the implications of these findings for clinical practice and/or further research? In developing countries such as Turkey, the necessity to raise awareness about HPV infection and vaccination has emerged. As a result, it is critical to implement educational campaigns to enhance awareness, particularly by medical staff.

Compliance of Familial Mediterranean Fever Patients With Regular Follow-up Visits and Associated Factors.

BACKGROUND: Follow-up is crucial to detect asymptomatic complications of familial Mediterranean fever (FMF). The current European League Against Rheumatism recommendations state that patients with FMF should be evaluated at least every 6 months to monitor attacks, acute phase response, and proteinuria. OBJECTIVES: This study aimed to assess compliance of FMF patients with regular follow-up visits and the associated factors. METHODS: Adult patients with a diagnosis of FMF who had their initial visit at least over 1 year ago were included. Demographic and socioeconomic data, family history, and comorbid diseases were obtained from medical records. The International Severity Score for FMF and the Autoinflammatory Disease Damage Index scores were calculated. We defined patients as "compliant with follow-up visits" both if they had at least 2 visits during the previous year and a compatible physician's assessment. The characteristics of the compliant and noncompliant patients were compared, and multivariable logistic regression analysis was used to determine the factors influencing visit compliance. RESULTS: Four hundred seventy-four patients with FMF were included. Two hundred thirty (48.5%) were compliant, and 244 (51.5%) were noncompliant with follow-up visits. A family history of FMF in parents, the absence of a family history of FMF in siblings, treatment with biologic agents, concomitant medication use, multisite involvement during FMF attacks, and treatment satisfaction were independent predictors of visit compliance. CONCLUSIONS: Only half of the patients with FMF were compliant with follow-up visits. Better strategies should be implemented to increase the compliance of FMF patients. Identifying independent predictors would help to build one.

Diagnostic utility of serum biomarkers in large vessel vasculitis and their correlation with positron emission tomography.

OBJECTIVES: To investigate the association between vascular inflammation, as detected by positron emission tomography (PET) imaging and interleukin-6 (IL-6), pentraxin3, and B-cell-activating factor (BAFF) in subjects with LVV. METHODS: The study included newly diagnosed giant cell arteritis (GCA, n = 27) or Takayasu arteritis (n = 9) patients and healthy control (HC, n = 31) subjects. PET scan and blood samples were obtained before the introduction of treatments. IL-6, PTX3, and BAFF levels were determined quantitatively by enzyme-linked immunosorbent assay kits. RESULTS: Thirty-six patients with LVV (20 females, 16 males; age 64.5 ± 16.6 years) and 31 HC (14 females, 17 males; age 37.1 ± 9.6 years) were included. Serum levels of IL-6, PTX3, and BAFF were increased in patients with newly diagnosed LVV compared with healthy control subjects. In receiver operating characteristics (ROC) analysis, serum IL-6 and BAFF provided excellent discrimination of newly diagnosed LVV patients from HC (area under the ROC curve of >0.90 and >0.80, respectively). None of the inflammatory markers correlated with vascular inflammatory activity determined by PET scanning. CONCLUSIONS: Our results suggest that IL-6 and BAFF may serve as markers of large vessel vasculitis, while PTX3 is not useful. None of the inflammatory markers correlated with PET assessed vasculitis activity.

Sleep disturbances in children with cystic fibrosis, primary ciliary dyskinesia and typically developing children during COVID-19 pandemic.

AIM: We aimed to investigate sleep disturbances in children with cystic fibrosis (CF) and primary ciliary dyskinesia (PCD) and typically developing (TD) children during the COVID-19 pandemic. METHODS: Primary care givers of children with CF and PCD aged 3-16 years were asked to enrol in the study. Primary care givers of TD children were included as control group. The Sleep Disturbance Scale for Children (SDSC) was used, and questions related to sleep habits during the pandemic were asked. Results of the three groups were compared. RESULTS: Primary care givers of 33 children with CF, 16 children with PCD and 66 TD children were included in the study. There were no differences in terms of age and gender between the three groups. Changes in sleep patterns during the pandemic were more common among TD children and their families, with 75% of the children and 80% of their families sleeping later than before. The sleep initiation and maintenance disorder scores were higher in TD children (P = 0.001), whereas the sleep breathing disorder scores were higher in children with PCD (P = 0.001), and the sleep hyperhidrosis scores were higher in children with CF and PCD (P = 0.011). No relationships were found between sleep parameters and clinical findings of children with lung disease. CONCLUSIONS: Children's sleep habits have changed during the pandemic. Children with chronic lung diseases and even TD children may experience sleep disturbances during this period.

Diagnostic Role of 18F-Fluorodeoxyglucose Positron Emission Tomography for the Evaluation of Patients With Inflammation of Unknown Origin.

BACKGROUND: Sometimes, the underlying causes of inflammation cannot be established despite meticulous investigation, including medical history, physical examination, laboratory tests, and radiologic procedures. Rheumatologists are often faced with patients whose condition is known as inflammation of unknown origin (IUO). Differential diagnosis of IUO is diverse, and investigation of these cases is challenging and time-consuming. OBJECTIVE: The study aimed to assess the diagnostic role of positron emission tomography/computed tomography (PET/CT) in the evaluation of patients with IUO. METHODS: The study sample consisted of 97 adult patients with IUO who have not been previously diagnosed with an infectious, inflammatory, or malignant disease. The necessary data were collected from January 2015 to June 2018 with a 6-month follow-up period. The patients were screened using PET/CT after a specific diagnosis could not be established with detailed laboratory and radiologic evaluations. RESULTS: A final diagnosis was established at follow-up, and 47 (54%) of the 97 patients had inflammatory diseases, 30 (34.4%) had malignancies, and 10 (11.4%) had infections. Despite meticulous investigation, 10 patients were left undiagnosed in the follow-up. PET/CT aided diagnosis in 59 patients (60.8%), but it was not helpful in 38 patients (39.2%). PET/CT was positive in 30 (63%) of the 47 patients with inflammatory diseases, whose final diagnosis was inflammatory rheumatic disease, as follows: large-vessel vasculitis in 19 patients, polymyalgia rheumatica in 7 patients, and seronegative arthritis or other rare miscellaneous diseases in 4 patients. The sensitivity of PET/CT was 67% with a specificity and diagnostic accuracy of 100% and 71%, respectively. CONCLUSIONS: Investigation of the underlying etiology of IUO is time-consuming and challenging. PET/CT may help identify the final diagnosis more quickly by locating an obscure inflammatory site; thus, it may reduce the number of unnecessary biopsies, diagnostic time, anxiety, work loss, morbidity, and mortality.

Fiberoptic Endoscopic Evaluation of Swallowing in Patients with Type 2 Diabetes Who Have Self-Reported Swallowing Difficulties.

INTRODUCTION: People with diabetes frequently have gastrointestinal problems and related deglutition disorders. OBJECTIVE: The aims of the present study are to determine the symptomatic swallowing complaints and to evaluate the functionality of oropharyngeal swallowing in patients with type 2 diabetes mellitus (T2DM) by using the Turkish Eating Assessment Tool-10 (T-EAT-10) and fiberoptic endoscopic evaluation of swallowing (FEES). METHODS: In this descriptive cross-sectional study, the T-EAT-10 questionnaire was completed by 121 patients with diabetes, and FEES was planned for each individual whose baseline score of the T-EAT-10 was ≥3. Before swallowing trials via samples of nectar-thick consistency, laryngeal sensation and severity of secretion in the hypopharynx were observed. While the swallowing safety was determined using the Penetration-Aspiration Scale (PAS), the Yale Pharyngeal Residue Severity Rating Scale was used to assess the swallowing efficiency. RESULTS: Of the total participants, 22.3% (n = 27) were found to have abnormal swallowing function (T-EAT-10 ≥3), 27.3% (n = 33) had concomitant neuropathy and 28.1% (n = 34) mentioned a reflux complaint. The results of the multivariate linear regression analysis exposed that the T-EAT-10 score was significantly associated with neuropathy (r = 3.763, p < 0.001) and reflux complaint (r = 2.254, p = 0.031). Of the total FEES-tested subjects (n = 20), 95% (n = 19) had a safe swallowing function (PAS = 1). However, diminished laryngeal sensation, increased secretion and presence of residue revealed that patients with T2DM who have self-reported swallowing difficulties have reduced swallowing efficiency. CONCLUSIONS: This study has demonstrated that almost 1 out of 4 T2DM patients reported to have swallowing-related problems, and the score of the T-EAT-10 was found to be independently associated with both neuropathy and reflux complaint. FEES results pointed out that swallowing efficiency was relatively reduced in the target population. However, further research is still necessary before obtaining a definitive answer to oropharyngeal swallowing problems in patients with T2DM.

Folfirinox versus gemcitabine-cisplatin combination as first-line therapy in treatment of pancreaticobiliary cancer

Background/aim: The purpose of this study was to compare efficacy and safety of a combination chemotherapy regimen consisting of oxaliplatin, irinotecan, fluorouracil, and leucovorin (FOLFIRINOX) and gemcitabine-cisplatin as first-line therapy in patients with pancreatic cancer. Materials and methods: Pancreaticobiliary cancer patients who had Eastern Cooperative Oncology Group performance status score of 0 or 1 (on a scale of 0 to 5, with higher scores indicating greater severity of illness) were evaluated to receive folfirinox or gemcitabine plus cisplatin. The primary endpoints were progression-free and overall survival time. Safety analysis was also evaluated as secondary measures. Results: There were 32 patients in the folfirinox group and 36 patients in the gemcitabine-cisplatin group. The median overall survival was 18.1 months (7.5­28.7) in the folfirinox group as compared with 9.7 months (6.5­13) in the gemcitabine-cisplatin group (p = 0.009). Median progression-free survival was 16.2 months (9­23.4) in the folfirinox group and 6.9 months (6.1­7.6) in the gemcitabine- cisplatin group (p = 0.001). Conclusion: Folfirinox is an option for the first-line treatment of patients with pancreatic cancer and good performance status.

Diaphragmatic muscle thickness and diaphragmatic function are reduced in patients with systemic lupus erythematosus compared to those with primary Sjögren's syndrome.

INTRODUCTION: Systemic lupus erythematosus (SLE) is associated with an increased risk of pulmonary infections, as well as a rare condition known as shrinking lung syndrome (SLS). The diaphragm has an important role to play in lung physiology and might also play a role in these adverse events. Here, we aimed to investigate whether SLE patients have impairment in their diaphragmatic muscle thickness and function with respect to another connective-tissue disease: primary Sjögren's syndrome (pSS). METHOD: Patients diagnosed with SLE who were in remission or who had minimal disease activity and had at least one year of follow-up were included in this study. Patients with known lung pathology and smokers were excluded. Patients with pSS constituted the second experimental group. Ultrasonographic evaluation of the diaphragmatic muscle was conducted by an experienced independent sonographer at three time points, diaphragmatic thickness during deep and quiet inspiration and maximum expiration being measured. Diaphragmatic muscle function was evaluated with maximum expiratory pressure (MEP) and maximum inspiratory pressure (MIP). RESULTS: A total of 115 patients were studied (n = 39 SLE; n = 76 pSS). The mean ± standard deviation (SD) thickness of the diaphragmatic muscles during quiet inspiration was significantly reduced in patients with SLE compared to patients with pSS (2.32 mm vs. 2.81 mm; p < 0.05). Similarly, the thickness during deep inspiration and at maximum deep expiration were significantly lower in SLE patients (2.88 mm vs. 3.29 mm and 1.92 mm vs. 2.33 mm, respectively; p < 0.01). MIPs and MEPs, defined as the percentages of expected values, were significantly lower in patients with SLE compared to those with pSS (80% vs. 92% and 76% vs. 120%, respectively; p < 0.05). Diaphragmatic muscle thickness during deep inspiration demonstrated a moderate correlation with MIP (r = 0.434; p = 0.001). CONCLUSION: SLE patients had reduced diaphragmatic muscle thickness compared to those with pSS, which was associated with impaired functional tests. Further prospective studies are needed to investigate whether structural and functional impairments in diaphragmatic muscle play a role in an increased risk of pulmonary infections and SLS in patients with SLE.

Alcohol consumption and alcohol policy

Alcohol is a unique addictive substance used by many people for different reasons. Alcohol use affects not only the users but also the family and the whole society in a negative way. Alcohol is one of the most commonly used substances for entertainment purposes in the world and 1 in 3 people is a current drinker. 2348 billion people (43% of the population) aged 15 and over are current drinkers and males drink about 2 times more frequently than females (53.6%/32.3%). According to the Global Alcohol and Health Report published by the World Health Organization (WHO) (2018), total alcohol per capita consumption (APC) worldwide aged 15 and over increased from 5.7 liters in 2000 to 6.4 liters in 2016. Harmful alcohol consumption is a major public health problem and it is known to be associated with more than 200 diseases and injuries. Policies and measures to prevent alcohol use are not implemented adequately and the burden of alcohol-related illnesses continues to increase tremendously. In order to prevent and reduce the harmful effects of alcohol, alcohol policies should be formulated based on the best evidence from a public health perspective.

Colchicine intolerance in FMF patients and primary obstacles for optimal dosing

Background/aim: Colchicine is the mainstay of treatment in FMF. However, in daily practice it is not easy to maintain effective colchicine doses in a substantial number of patients due to its side effects. In this study, we aimed to investigate prevalence and risk factors for colchicine side effects that limit optimal drug dosing and cause permanent discontinuation. Materials and methods: All patients were recruited from "FMF in Central Anatolia" (FiCA) cohort, 915 adults with a minimum follow- up time of 6 months during which they had obeyed all treatment instructions. Demographic and anthropometric data, FMF disease characteristics, disease severity, complications, and treatment features were recorded on a web-based registry. Prevalence of colchicine intolerance and characteristics of intolerant patients were analyzed. Results: Effective colchicine doses cannot be maintained in 172 (18.7%) subjects. Main side effects that limit optimal dosing were as follows: diarrhea in 99 (10.8%), elevation in transaminases in 54 (5.9%), leukopenia in 10 (%1.1), renal impairment in 14 (1.3%), myopathy in five (0.5%), and allergic skin reaction in two. Colchicine had to be permanently ceased in 18 (2%) patients because of serious toxicity. Male sex and obesity were found to be associated with liver toxicity, and having a normal body weight was associated with diarrhea. Chronic inflammation and proteinuria were more common in colchicine-intolerant patients, and they had reported more frequent attacks compared to those tolerating optimal doses. Conclusion: Colchicine intolerance is an important problem in daily clinical practice, mainly due to diarrhea and liver toxicity. Suboptimal colchicine dosing is associated with complications.

Decrease in Cerebral Oxygen Saturation During the 6-Minute Walk Test in Pediatric Pulmonary Arterial Hypertension.

This observational study aimed to investigate the relationship between regional cerebral oxygen saturation (rSO2) during the 6-minute walk test (6-MWT) and the demographic/clinical features of patients with pulmonary arterial hypertension (PAH). Cerebral oxygenation was evaluated during the 6-MWT in 20 pediatric patients with PAH aged ≥ 7 years [13 male, 7 female; median age 12.25 (range 7-18) years]. In all patients, regional cerebral oxygen saturation    (rSO2), arterial oxygen saturation (SpO2), and heart rate (HR) were measured using near-infrared spectroscopy (NIRS) for 2 min before the test, during the 6-MWT test, and 2 min after the test. The relationship between the changes in rSO2, heart rate, and SpO2 values and clinical and laboratory features was compared statistically. The mean pulmonary arterial pressure (mPAP) was 77 (range 25-126) mmHg, and the median 6-minute walk distance (6-MWD) was 427.5 (300-570) m. The changes in rSO2 measurements ranged between 3.41 and 21.8%, and 70% of the patients had a greater than 10% decrease in rSO2 during the test compared with baseline. Eight patients showed a decrease in rSO2 without SpO2 changes. The present study demonstrated a significant decrease in cerebral oxygenation in pediatric patients with PAH during the 6-MWT. We hypothesized that using a combination of the 6-MWT and regional cerebral oxygenation monitoring in pediatric patients with PAH in order to evaluate exercise capacity, as a reflection of reduced daily activities, would provide more precise predictive values than the 6-MWT alone.