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Since the outbreak of the Syrian civil war in 2011, the population of Arab refugees in Turkey has rapidly increased. While cystic fibrosis (CF) is believed to be rare among Arabs, recent studies suggest it is underdiagnosed. This study aims to present the demographic, clinical, and genetic characteristics of CF patients among Arab refugees in Turkey. Additionally, a comparison is made between the findings in the National CF Registry 2021 in Turkey (NCFRT) and the refugee CF patient group. The study included refugee patients between the ages of 0 and 18 years who were diagnosed with CF and received ongoing care at pediatric pulmonology centers from March 2011 to March 2021. The study examined demographic information, age at diagnosis, age of diagnosis of patients through CF newborn screening (NBS), presenting symptoms, CF transmembrane conductance regulator (CFTR) mutation test results, sputum culture results, weight, height, and body mass index (BMI) z score. Their results were compared with the NCFRT results. The study included 14 pediatric pulmonology centers and 87 patients, consisting of 46 (52.9%) boys and 41 (47.1%) girls. All of the patients were Arab refugees, with 80 (92%) being Syrian. All the patients were diagnosed in Turkey. The median age at diagnosis of patients was 22.33 (interquartile range, 1-258) months. The median age of diagnosis of patients through NBS was 4.2 (interquartile range, 1-12) months. The median age of older patients, who were unable to be included in the NBS program, was 32.3 (interquartile range, 3-258) months. Parental consanguinity was observed in 52 (59.7%) patients. The mutation that was most frequently found was F508del, which accounted for 22.2% of the cases. It was present in 20 patients, constituting 32 out of the total 144 alleles. There was a large number of genetic variations. CFTR genotyping could not be conducted for 12 patients. These patients had high sweat tests, and their genetic mutations could not be determined due to a lack of data. Compared to NCFRT, refugee patients were diagnosed later, and long-term follow-up of refugee CF patients had significantly worse nutritional status and pseudomonas colonization. Conclusion: Although refugee CF patients have equal access to NBS programs and CF medications as well as Turkish patients, the median age at diagnosis of patients, the median age of diagnosis of patients through NBS, their nutritional status, and Pseudomonas colonization were significantly worse than Turkish patients, which may be related to the difficulties of living in another country and poor living conditions. The high genetic heterogeneity and rare mutations detected in the refugee patient group compared to Turkish patients. Well-programmed NBS programs, thorough genetic studies, and the enhancement of living conditions for refugee patients in the countries they relocate to can have several advantages such as early detection and improved prognosis. What is Known: ⢠Children who have chronic diseases are the group that is most affected by wars. ⢠The outcome gets better with early diagnosis and treatment in patients with Cystic Fibrosis (CF). What is New: ⢠Through the implementation of a newborn screening program, which has never been done in Syria previously, refugee patients, the majority of whom are Syrians were diagnosed with cystic fibrosis within a duration of 4 months. ⢠Despite equal access to the newborn screening program and CF medications for both Turkish patients and refugee patients, the challenges of living in a foreign country have an impact on refugees.
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Fibrose Cística , População do Oriente Médio , Refugiados , Recém-Nascido , Masculino , Criança , Feminino , Humanos , Lactente , Pré-Escolar , Adolescente , Fibrose Cística/diagnóstico , Fibrose Cística/epidemiologia , Fibrose Cística/genética , Regulador de Condutância Transmembrana em Fibrose Cística/genética , Turquia/epidemiologia , Triagem Neonatal/métodosRESUMO
BACKGROUND: Scaphoid nonunion remains a challenging injury with no clear consensus on treatment. Surgical options, such as bone grafting procedures, are available for the treatment of scaphoid nonunions. While open grafting provides direct visualization, it is theoretically believed to lead to several problems due to the complex ligamentous structure responsible for wrist stability and challenges in the vascular supply of the scaphoid. On the other hand, despite its technical challenges, arthroscopic grafting is thought to avoid complications by preserving surrounding tissues. QUESTIONS/PURPOSES: (1) Do patients undergoing bone grafting via arthroscopy for scaphoid nonunion report better function than patients undergoing an open procedure? (2) Do patients undergoing bone grafting via arthroscopy for scaphoid nonunion demonstrate better objective outcomes, such as ROM, extremity strength, and bony union? METHODS: Between January 2012 and January 2022, we operated on 141 patients with scaphoid nonunion. The following patients were excluded from this study: 33 patients with scaphoid nonunion advanced collapse and arthritis, 18 patients with proximal pole fractures, 5 patients with previous surgeries, 16 patients with avascular necrosis, and 8 patients with the radius used as a graft source. In total, 28 patients underwent open grafting, and 33 patients underwent arthroscopic grafting; for both groups, the iliac crest was used as the graft source. Two patients with nonunion were observed in each treatment group, and they were excluded from the study. Results from the remaining 26 patients treated with open grafting and 31 patients treated with arthroscopic grafting (totaling 57 patients) were analyzed. The decision to treat patients with open or arthroscopic methods was not based on a particular reason. In our clinic, we initially preferred open grafting for treating nonunion of the scaphoid. Subsequently, we began to prefer arthroscopic methods for the treatment of these injuries. Twenty patients in the arthroscopic group had additional ligamentous injuries, which were simultaneously treated arthroscopically. All patients in both groups had at least 1 year of follow-up, but 48% of patients treated arthroscopically and 42% of those treated with open approaches were lost before 2 years of follow-up. The remaining patients had follow-up periods longer than 24 months. Our primary analysis was performed at 1 year, and we did a secondary analysis at 2 years. We compared the Patient-Rated Wrist Evaluation (PRWE), QuickDASH, and VAS scores of the patients. We also compared ROM and grip and pinch strength in patients' contralateral wrists. We used predefined, evidence-based thresholds for the minimum clinically important differences for these outcome measures. RESULTS: According to the 1-year functional analysis, we found no clinically important difference between the open surgery group and the arthroscopic surgery group in terms of PRWE score (median [IQR] 19 [25] versus 8 [9], difference of medians 11; p = 0.001), QuickDASH (median 14 [23] versus 7 [11], difference of medians 7; p = 0.004), and VAS scores (median 2 [2] versus 1 [1], difference of medians 1; p = 0.02). At 1 year, there were no differences in objective measurements, including grip strength (median 81 [16] versus 85 [14], difference of medians 4; p = 0.60), pinch strength (median 82 [18] versus 81 [15], difference of medians 1; p = 0.85), and ROM (flexion-extension median 83 [22] versus 85 [13], difference of medians 2; p = 0.74; radial deviation-ulnar deviation median 80 [36] versus 85 [14], difference of medians 5; p = 0.61). In the 2-year analysis, no clinically important difference was observed in terms of PRWE score, and no differences were found in terms of QuickDASH, VAS, strength tests, and ROM between the open and arthroscopic groups. No difference was observed in the union rates between the open group and the arthroscopic group (93% [26 of 28] versus 94% [31 of 33], OR 1.19 [95% CI 0.16 to 9.06]; p = 0.86). CONCLUSION: In comparing open surgical procedures with arthroscopic techniques for the treatment of scaphoid nonunions, the present study revealed no differences in functional outcomes and objective measures such as ROM and strength tests at both the 1-year and 2-year follow-up visits. Although technically more challenging, arthroscopy provides a potential advantage, such as addressing concomitant ligament injuries simultaneously. However, patients did not perceive a difference between the two surgical methods. In future studies, investigating long-term outcomes in a larger population will contribute to better elucidating this issue. LEVEL OF EVIDENCE: Level III, therapeutic study.
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BACKGROUND: Knee osteoarthritis is severe progressive and most commonly diagnosed articular disease and its incidence is increasing around the world depending on age. This pathologic condition which limits daily activity of patients can be characterized by degeneration of cartilage and inflammation. Although non-steroidal anti-inflammatory (NSAII) agents and other analgesics are routinely used treatment options, the potential effects of intraarticular injections including hyaluronic acid (HA) have also been demonstrated by various studies. However, few studies compare the efficacy of a single high molecular weight (HMW) high dose and a triple HMW low dose. This study aimed to compare the efficacy of single high molecular weight (HMW) high dose (2 mL / 60 mg) and triple HMW low dose (2 mL /30 mg) intra-articular injection of HA in knee osteoarthritis (OA) patients by evaluating function and pain parameters during 12 months. METHODS: This is a single-center, retrospective clinical study that included and involved 128 patients. Group I (n=64) patients received triple 30 mg HA injections (SEMICAL®) with one-week intervals, while Group II (n=64) patients received a single 60 mg HA injection (SEMICAL®). Lequesne Index, WOMAC and VAS scores were recorded to assess pain and function during a 12-month period. RESULTS: There was no significant difference in characteristics of patient demographics. Our finding indicate that WOMAC, VAS score, and Lequesne Index values during follow-up visits exhibited a decrease, signifying improvement in the clinical condition. Notably, scores were significantly more favorable with the 30 mg of HA injection compared to the 60 mg of HA injection. CONCLUSION: This study suggests that the triple low-dose injection of HMW HA is more effective in improving WOMAC, VAS scores and Lequesne Index values than a single high-dose injection.
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Ácido Hialurônico , Osteoartrite do Joelho , Humanos , Osteoartrite do Joelho/terapia , Viscossuplementos , Peso Molecular , Estudos Retrospectivos , Resultado do Tratamento , Dor/tratamento farmacológico , Injeções Intra-Articulares , Anti-Inflamatórios não Esteroides/uso terapêuticoRESUMO
PURPOSE: The ideal number of sutures for epineural nerve repair is still unclear. Increased number of sutures increases secondary damage and inflammation to the nerve tissue, which negatively affects nerve regeneration. When the number of sutures decreases, the strength of the nerve repair site decreases and nerve endings are fringed, which also negatively affects nerve regeneration. Therefore, each additional suture is not only beneficial but also detrimental. The aim of this study was to find out the ideal number of sutures for nerve repair. METHODS: Seventy rats were randomly divided into 5 groups. One of the groups was used as a control group, and right sciatic nerves of the rats in other 4 groups were repaired by using 2, 3, 4, or 6 epineural sutures, respectively, after nerve transection. Biomechanical assessment was performed on the nerves collected from these rats at 5 days of follow-up. Functional and histological analyses were evaluated after 12 weeks of follow-up. RESULTS: It was found that an increase in the number of sutures enhances resistance to tensile force in general. However, there was no significant biomechanical difference between the 6-sutured group in which the most sutures were used and the 4-sutured group. In functional examinations, overall successful results were obtained in the group with 4 sutures. In histological examinations, there was no statistical difference between the control group, 2-sutured groups, and 4-sutured groups in terms of connective tissue index. However, it was observed that the group with 6 sutures had a higher connective tissue index than the control group and groups with 2 and 4 sutures. In terms of regeneration index, it was found that repair with 4 sutures was superior to repair with 2 and 6 sutures. No difference was found between any of the suture groups according to the diameter change index. CONCLUSIONS: These results indicate that repair with 4 sutures is the best method of epineural repair that provides both strength and regeneration. These findings will contribute to both the repair of clinically similar nerves and the standardization of rat nerve studies.
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Procedimentos de Cirurgia Plástica , Nervo Isquiático , Ratos , Animais , Nervo Isquiático/cirurgia , Suturas , Procedimentos Neurocirúrgicos/métodos , Regeneração NervosaRESUMO
OBJECTIVES: This study aimed to demonstrate the volar plate fixation technique for fixing Bennett's fractures due to its buttress effect. PATIENTS AND METHODS: The retrospective study included 10 patients (8 males, 2 females; mean age: 35.9±11.56 years; range, 17 to 51 years) who were treated using the volar plate fixation technique between January 2018 and August 2022. The postoperative functional scores of the patients were evaluated using the Q-DASH (Quick Disabilities of the Arm, Shoulder, and Hand) score, and their pain status was evaluated with the Visual Analog Scale (VAS). We also assessed fracture union and development of complications. We assessed opposition according to Kapandji grade and abduction of the thumb. RESULTS: The mean follow-up time was 16.6±2.91 (range, 12 to 21) months. The technique was performed on the right extremity in eight patients and on the left extremity in two patients. All patients were right-hand dominant. The VAS score was 1 in two cases and 0 in the other cases. The mean Q-DASH score was 1.36±2.44. The mean pinch strength was 6.4±0.89 kg, and the mean grasp strength was 18.8±3.52 kg on the injured side. The mean Kapandji grade of opposition was 9.3±0.82, while the mean abduction degree was 37.4±2.01. CONCLUSION: Based on the early results of this technique, we conclude that volar plate fixation for Bennett's fractures is reliable and allows for early motion, providing anatomical and stable joint reduction, and it does not have implant complications such as hardware irritation. However, fixation of small fragments may be particularly challenging.
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Fraturas do Rádio , Traumatismos do Punho , Masculino , Feminino , Humanos , Adulto Jovem , Adulto , Pessoa de Meia-Idade , Fraturas do Rádio/cirurgia , Fixação Interna de Fraturas/efeitos adversos , Fixação Interna de Fraturas/métodos , Estudos Retrospectivos , Força da MãoRESUMO
BACKGROUND: Although international guidelines generally recommend the back to back use of short-acting ß-agonizts (SABA) within a short time in the management of acute wheezing in children with asthma, there is still uncertainty in the evidence of short term outcome. Thus, this study aimed to investigate the efficacy of back to back and single use of inhaled SABA by lung function testing. METHODS: This was a prospective, double-blinded, placebo controlled study conducted in children ≥6 years of age with a history of asthma. Children who presented with an acute asthma exacerbation (AAE) with a forced expiratory volume in 1 s (FEV1) between 40% to 60% were enrolled in the study if they had a first dose to SABA response of FEV1 ≥ 12%. All children were then randomly assigned either to receive two additional doses of inhaled SABA (300 µg per dose) or placebo. Spirometric analysis included forced vital capacity (FVC), FEV1, FEV1/FVC, PEF, and FEF25-75 at baseline, 15, 30, and 45 min for each group. Oxygen saturation and heart rate were monitored during the study period. RESULTS: A total of 93 patients (inhaled SABA group; n = 48 vs. placebo group; n = 45) out of 110 enrolled patients completed the study. Baseline demographic characteristics of patients include age, gender, age of diagnosis, parental asthma, history of allergic rhinitis and atopic dermatitis, current asthma treatment, IgE and skin prick test were similar among groups. (p > .05) When lung function parameters were compared at each time interval during the study period, there were no statistical significance found in FVC, FEV1, FEV1/FVC, PEF and forced expiratory flow between 25% and 75% (FEF25-75) among groups. (p > .05) There were also no differences between groups for changes in heart rate and oxygen saturation. (p > .05) CONCLUSION: A single dose of inhaled SABA provides similar short term bronchodilator effect as back to back administration of inhaled SABA in children with AAE who showed an initial response to SABA of FEV1 ≥ 12%.
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This study investigated the efficacy of an Adapted Kata Training Program (AKTP) in enhancing balance for a 10-year-old child with Autism Spectrum Disorder (ASD), employing a mixed-model approach for data collection. Over 12 weeks, the AKTP demonstrated significant improvements in the child's balance abilities, with an 11% increase in static balance, 8% in proprioceptive, 12% in horizontal, and 14% in vertical balance performance. These improvements persisted in a follow-up assessment after four weeks. Observations by the child's mother corroborated the above findings. Consequently, this research suggests the AKTP as a valuable non-pharmacological intervention to improve balance in children with ASD. However, further studies are necessary to validate these results and explore the impact on additional developmental domains, such as cognitive and motor skills.
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INTRODUCTION: Cystic fibrosis transmembrane conductance regulator (CFTR) modulator drugs target the underlying defect and improve CFTR function. They are a part of standard care in many countries, but not all patients are eligible for these drugs due to age and genotype. Here, we aimed to determine the characteristics of non-eligible patients for CFTR modulators in the CF registry of Turkey (CFRT) to highlight their clinical needs. METHODS: This retrospective cohort study included CF patient data from the CFRT in 2021. The decision of eligibility for the CFTR modulator was determined according to the 'Vertex treatment-Finder' on the Vertex® website. Demographic and clinical characteristics of patients were compared between eligible (group 1) and ineligible (group 2) groups for CFTR modulators. RESULTS: Among the study population (N = 1527), 873 (57.2%) were in group 1 and 654 (42.8%) were in group 2. There was no statistical difference between groups regarding sex, meconium ileus history, diagnoses via newborn screening, FEV1 z-score, CF-associated complications, organ transplant history, and death. Patients in group 2 had a higher incidence of pancreatic insufficiency (87.7% vs. 83.2%, p = .010), lower median height z-scores (-0.87 vs. -0.55, p < .001), lower median body mass index z-scores (-0.65 vs. -0.50, p < .001), longer days receiving antibiotics due to pulmonary exacerbation (0 [interquartile range, IQR: 0-2] vs. 0 [IQR: 0-7], p = 0.001), and more non-invasive ventilation support (2.6% vs. 0.9%, p = 0.008) than patients in group 1. CONCLUSION: The ineligible group had worse clinical outcomes than the eligible group. This highlights their need for life-changing drugs to improve clinical outcomes.
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BACKGROUND: The decline in pulmonary function is a predictor of disease progression in patients with cystic fibrosis (CF). This study aimed to determine the decline rate of percent predicted forced expiratory volume in 1 s (ppFEV1) based on the data of the CF Registry of Turkey. The secondary aim was to investigate the risk factors related to the decline in ppFEV1. METHODS: A retrospective cohort study of CF patients over 6 years old, with pulmonary function data over at least 2 years of follow-up was extracted from the national CF registry for years 2017-2019. Patients were classified according to disease severity and age groups. Multivariate analysis was used to predict the decline in ppFEV1 and to investigate the associated risk factors. RESULTS: A total of 1722 pulmonary function test results were available from 574 patients over the study period. Mean diagnostic age was older and weight for age, height for age, and body mass index z scores were significantly lower in the group of ppFEV1 < 40, while chronic Pseudomonas aeruginosa (p < .001) and mucoid P. aeruginosa colonization (p < .001) were significantly higher in this group (p < .001). Overall mean annual ppFEV1 decline was -0.97% (95% confidence interval [CI] = -0.02 to -1.92%). The mean change of ppFEV1 was significantly higher in the group with ppFEV1 ≥ 70 compared with the other (ppFEV1 < 40 and ppFEV1: 40-69) two groups (p = .004). Chronic P. aeruginosa colonization (odds ratio [OR] = 1.79 95% CI = 1.26-2.54; p = .01) and initial ppFEV1 ≥ 70 (OR = 2.98 95% CI = 1.06-8.36), p = .038) were associated with significant ppFEV1 decline in the whole cohort. CONCLUSIONS: This data analysis recommends close follow-up of patients with normal initial ppFEV1 levels at baseline; advocates for early interventions for P. aeruginosa; and underlines the importance of nutritional interventions to slow down lung disease progression.
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In this work, the findings of the work on the green dehydrogenation behavior of molten dimethylamine borane (DMAB) catalyzed by the precatalyst copper(II) acetylacetonate (Cu(acac)2) in solvent-free medium (green) at near room temperature (nRT, 30.0+0.1°C) were reported. Herein, a complete study has been presented, which includes the following steps: (i) synthesis and catalytic activity of Cu(0) NCats in solvent-free medium, (ii) determination of activation energy for Cu(0) NCats catalyzed green dehydrogenation of DMAB, (iii) demonstration of catalytic lifetime of Cu(0) NCats, (iv) test of isolability and reusability of Cu(0) NCats, (v) poisoning experiments using carbon disulfide on a per-active-copper-atom basis, (vii) characterization of Cu(0) NCats by UV-vis, XRD, XPS and TEM/HRTEM/TEM-EDX spectroscopies. In addition, ATR-FTIR and 11B NMR techniques were use to characterize the cyclic aminoborane product obtained as a result of dehydrogenation of dimethylamine-borane.
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Atorvastatina , Proteinose Alveolar Pulmonar , Receptores de Fator Estimulador das Colônias de Granulócitos e Macrófagos , Humanos , Proteinose Alveolar Pulmonar/tratamento farmacológico , Atorvastatina/uso terapêutico , Receptores de Fator Estimulador das Colônias de Granulócitos e Macrófagos/genética , Masculino , Inibidores de Hidroximetilglutaril-CoA Redutases/uso terapêutico , Ácidos Heptanoicos/uso terapêutico , Feminino , Pirróis/uso terapêutico , Resultado do Tratamento , CriançaRESUMO
ABSTRACT BACKGROUND: The coronavirus disease-19 (COVID-19) pandemic has changed the course of diseases that require emergency surgery. OBJECTIVE: To evaluate the effect of the COVID-19 pandemic on colorectal cancer disease stage. DESIGN AND SETTING: Retrospective analysis in the city of Rize, Turkey. METHODS: This was a comparative analysis on two groups of patients with various symptoms who underwent surgical colorectal cancer treatment. Group 1 comprised patients operated between March 11, 2019, and December 31, 2019; while group 2 comprised patients at the same time of the year during the COVID-19 pandemic. RESULTS: Groups 1 and 2 included 56 and 48 patients, respectively. The rate of presentation to the emergency service was higher in Group 2 (P < 0.02). The stage of the pathological lymph nodes and the rate of liver metastasis was higher in Group 2 (P < 0.004 and P < 0.041, respectively). The disease stage was found to be more advanced in Group 2 (P < 0.005). The rate of postoperative complications was higher in Group 2 (P < 0.014). CONCLUSION: The presentation of patients with suspicious findings to the hospital was delayed, due both to the fear of catching COVID-19 and to the pandemic precautions that were proposed and implemented by healthcare authorities worldwide. Among the patients who presented to the hospital with emergency complaints and in whom colorectal cancer was detected, their disease was at a more advanced stage and thus a higher number of emergency oncological surgical procedures were performed on those patients.