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OBJECTIVE: This study evaluated the effect of brochure-based and video-assisted information provided before spirometry on patient compliance. PATIENTS AND METHODS: This was a randomized controlled clinical trial. Before the test, subjects in intervention groups were shown a leaflet outlining the steps of the spirometry protocol and a video prepared for the same purposes. The control group was given standard routine information by the technician before spirometry. RESULTS: The study included 450 patients. We found a significant correlation between compliance status and age, female sex, being a non-smoker, having no known lung disease, investigating respiratory disease as an indication for spirometry, having first-time spirometry, and receiving pre-test information via leaflets. Variables of age, sex, smoking, indication for spirometry, diagnosis, and previous spirometry, which were found in multivariate analysis to be associated with 'compliance with the test protocol' were further processed using regression analysis which identified 'previous spirometry' as the most decisive variable affecting 'compliance with the test protocol'. CONCLUSIONS: Providing information via brochure-based and video-supported information did not contribute to compliance with the testing protocol, leading us to the conclusion that such informative tools do not provide an additional contribution. Previous spirometry experience was the most decisive parameter influencing adherence to the test protocol. [ABSTRACT FROM AUTHOR]

OBJECTIVE: Tumor necrosis factor-alpha (TNF-a) inhibitors are used to treat autoimmune diseases such as rheumatoid arthritis, ankylosing spondylitis, and inflammatory bowel disease. However, patients using TNF-a inhibitors are at a high risk of developing tuberculosis. Therefore, this study aimed to evaluate the compliance level of patients who were prescribed TNF-a-based tuberculosis prophylactic treatment. PATIENTS AND METHODS: The study included 135 patients who were followed up at a tuberculosis dispensary between December 2020 and June 2021 and agreed to participate in the study. The study was conducted after obtaining necessary permissions from the institution, ethics committee, and patients. Data were collected using a questionnaire and evaluated using the Medication Adherence Report Scale (MARS). The MARS scale scores range from 5 to 25, with high scores indicating a high level of "medication compliance level". RESULTS: Of the included patients, 42.2% were females, 46.7% were primary school graduates, and their mean age was 43.75±11.86 years. Additionally, 35.6% of patients had ankylosing spondylitis, among whom 54.1% had a disease duration of 1-5 years and 57.8% had been using TNF-a inhibitors for a year. Of the patients taking isoniazid (INH) for tuberculosis prophylaxis, 47.4% missed their prescribed INH doses, with "forgetfulness" being the reason in 28.9% of these patients. The patients had a mean MARS score of 15.71±6.18. CONCLUSIONS: Patients using TNF-a inhibitors were found to have "moderate" compliance levels for INH-based prophylactic therapy. It is recommended that the compliance levels of patients and factors influencing their compliance should be regularly monitored. [ABSTRACT FROM AUTHOR]

2019 was a year of global protest. Was it democracy in action, or democracy in crisis? The second decade of this millennium will be remembered as a decade of protest. [...]

Use of unmanned aerial vehicles (UAVs) is spreading at an extremely rapid pace in the agricultural sector as in many other sectors. In many areas such as biomass, disease detection, water stress, irrigation, and soil moisture determination, UAVs can be used in agriculture. The biggest problem of irrigation managers is to not be able to fully determine the irrigation activities in the irrigation area by conventional methods. In this study, the possibility of determining the agricultural parcels where irrigation is carried out in the irrigation scheme was investigated with a UAV. During the study, thermal images obtained with a Matrice 600 pro loaded with Zenmuse XT capable of radiometric temperature measurement with 640*320 resolution were used. Thermal images were interpreted by Agisoft Pro. As a result of the study, it has been determined that the irrigation activities carried out in the scheme can be determined easily, especially with the images taken on the first date of the irrigation. In addition, a thermal camera mounted UAV can be detected easily at the points where the leakage losses occur at the irrigation site. thus, irrigation management is determined in areas that need to be concentrated for maintenance services. [ABSTRACT FROM AUTHOR]

Since the introduction of our lives, Unmanned Aerial Vehicles (UAVs) have been used for a variety of purposes in a variety of professions such as agriculture, forestry, industry and mining. Their superiority, such as high resolution, frequent measurement range, and relatively low cost compared to other remote sensing technologies, increase their chances of being used in agricultural areas. The aim of this study is to determine the crop pattern of the irrigation network using a drone equipped with the imaging system and to transfer this information to the parcel information system. Crop pattern was determined by UAV imaging, was processed in layout sheet of irrigation scheme and it was determined which channels served these parcels. In this respect, tried to help for irrigation management has tried to determine what channels work in the network and how much. Thereby determining water distribution activities accordingly and trying to help protect the water resources that are already inadequate. Unmanned aerial vehicles (UAVs), imaging technology, flight times, payloads, and data transfer speeds seem to have tremendous potential in carrying out such and similar studies. [ABSTRACT FROM AUTHOR]

There are several types of renewable energy platforms. Because of requirements of places, where platforms were built, every platform has different energy systems and qualities. In this paper, the renewable energy potential of Turkish coasts was investigated according to different renewable energy types. It is aimed to find suitable places for a near shore renewable energy platform and create a concept design. Firstly, the regions with highest energy potential according to four renewable energy types; wind, wave, current and solar; were determined and five different regions were selected to analyze. For each region, the power data of the different energy types were converted to same unit and the regions were ranked with respect to these results. Then the existing platform types and alternative energy conversion systems were examined and for each energy type a conversion system was chosen according to the characteristics of selected region. Finally, a concept near shore platform was designed with selected systems. [ABSTRACT FROM AUTHOR]

Objective: Heavy menstrual bleeding (HMB) is a significant health concern that may lead to critical medical care and impaired quality of life. The aim of the present study was to identify the clinical indicators of common causes of HMB in adolescents and evaluate the factors influencing the treatment preferences of clinicians., Methods: Between 2010 and 2022, the electronic medical records of adolescents between the ages of 10-18 with HMB were reviewed, and a final of 205 adolescents was included. Clinical, laboratory, and treatment data were retrospectively analyzed., Results: Hypothalamic-pituitary-ovarian (HPO) axis immaturity was diagnosed in 63.9%, bleeding disorders (BD) in 13.7%, and polycystic ovary syndrome (PCOS) in 12.2% of the patients. The presence of "heavy bleeding" and the bleeding length at presentation were not different between the groups. Patients with a BD presented with lower hemoglobin (Hb) levels (P = 0.019), and more frequently required inpatient admissions (P = 0.005). Of all patients, 34.6% were given only iron, 44.9% were given a low dose, and 15.6% were given a high-dose hormone treatment. Factors influencing high-dose hormone regimens were lower Hb levels (P < 0.001), the place of visit (ER rather than the clinic) (P < 0.001), and the presence of multiple ER visits (P < 0.001)., Conclusion: Physicians base their decisions to start high-dose hormonal therapy on objective parameters such as Hb levels and the need for hospitalization, while more precise guidelines are deemed necessary for initiating low-dose hormonal therapy. Standardized protocols and their practical implementation at the clinical level are needed for the management of HMB in adolescence., (© 2024 International Federation of Gynecology and Obstetrics.)

Since tuberculosis (TB) is an infectious disease, it affects patients not only physically but also socially and patients are reported to experience stigma. This study was conducted to determine the stigma levels of patients in a tuberculosis dispensary in the southeastern region of Turkey. The study was conducted between December 2020 and June 2021 with 79 patients who agreed to participate. 'Questionnaire' and 'Tuberculosis-Related Stigma Scale' (TSS) were used for data collection. The score obtained from the scale ranges between '33 and 132' and the higher the score, the higher the 'stigma level'. It was determined that 54.4% of the patients were male, 45.6% were primary school and the mean age was 44.98 ± 16.09 years. It was found that 60.8% of the patients had pulmonary TB, 40.5% had been on treatment for 3-6 months, 45.6% described the society's view of TB patients as 'bad, negative, and excluding', 64.8% had adverse effects on their communication with their family and close environment after being diagnosed with TB. The mean TSS score of the patients was 103.51 ± 10.65. TB patients were found to have 'very high' levels of stigma. The mean total stigma is higher in smokers, particularly in cases of pulmonary and extrapulmonary tuberculosis, as well as in those whose professional lives are affected.

The improved survival rates of childhood cancers raise the long-term risk of second primary malignancy (SPM) in childhood and adolescent cancer survivors. The intensity of the treatment protocol used, the use of some groups of chemotherapeutics, and radiotherapy were found to be risk factors for the development of second primary malignancies (SPMs). Forty-one patients who developed acute myelocytic leukemia or any solid organ cancer within 25 years of follow-up, after completion of pediatric acute lymphoblastic leukemia (ALL) treatment, were included in the study. The mean duration of initial ALL diagnosis to SPM was 9.3 ± 6.1 years. The 3 most common SPMs were acute myelocytic leukemia, glial tumors, and thyroid cancer. Thirteen (81%) of 16 patients exposed to cranial irradiation had cancer related to the radiation field. In total 13/41 (32%) patients died, and the 5-year overall survival rate was 70 ± 8%. Patients older than 5 years old at ALL diagnosis had significantly worse overall survival than cases younger than 5 years old. In conclusion, children and adolescents who survive ALL have an increased risk of developing SPM compared with healthy populations, and physicians following these patients should screen for SPMs at regular intervals., Competing Interests: The authors declare no conflict of interest., (Copyright © 2024 Wolters Kluwer Health, Inc. All rights reserved.)

Background: Leukocyte and platelet integrin function defects are present in leukocyte adhesion deficiency type III (LAD-III) due to mutations in FERMT3. Additionally, osteoclast/osteoblast dysfunction develops in LAD-III., Aim: To discuss the distinguishing clinical, radiological, and laboratory features of LAD-III., Methods: This study included the clinical, radiological, and laboratory characteristics of twelve LAD-III patients., Results: The male/female ratio was 8/4. The parental consanguinity ratio was 100%. Half of the patients had a family history of patients with similar findings. The median age at presentation and diagnosis was 18 (1-60) days and 6 (1-20) months, respectively. The median leukocyte count on admission was 43,150 (30,900-75,700)/μL. The absolute eosinophil count was tested in 8/12 patients, and eosinophilia was found in 6/8 (75%). All patients had a history of sepsis. Other severe infections were pneumonia (66.6%), omphalitis (25%), osteomyelitis (16.6%), gingivitis/periodontitis (16%), chorioretinitis (8.3%), otitis media (8.3%), diarrhea (8.3%), and palpebral conjunctiva infection (8.3%). Four patients (33.3%) received hematopoietic stem cell transplantation (HSCT) from HLA-matched-related donors, and one deceased after HSCT. At initial presentation, 4 (33.3%) patients were diagnosed with other hematologic disorders, three patients (P5, P7, and P8) with juvenile myelomonocytic leukemia (JMML), and one (P2) with myelodysplastic syndrome (MDS)., Conclusion: In LAD-III, leukocytosis, eosinophilia, and bone marrow findings may mimic pathologies such as JMML and MDS. In addition to non-purulent infection susceptibility, patients with LAD-III exhibit Glanzmann-type bleeding disorder. In LAD-III, absent integrin activation due to kindlin-3 deficiency disrupts osteoclast actin cytoskeleton organization. This results in defective bone resorption and osteopetrosis-like radiological changes. These are distinctive features compared to other LAD types., (© 2023. The Author(s), under exclusive licence to Springer Science+Business Media, LLC, part of Springer Nature.)

Carbon footprint (CF) research has received increasing attention in recent years, as evidenced by a rise in publications and citations, reflecting a growing concern for the environmental impact of human activities. However, the alignment of this scientific literature with the three dimensions of sustainability performance provided by the TBL paradigm (people, planet, and profit) has received limited attention. This study addresses this research gap by undertaking a large-scale bibliometric analysis of 9032 Web of Science (WoS) publications from 1992 to 2020. At the macro (journals) and micro (papers) levels, a methodology approach to classify research publications according to TBL dimensions was designed. The results indicate that the output and impact of CF research are balanced with respect to the environmental (planet) and economic (prosperity/profit) dimensions, while the social impact is balanced with respect to the people+profit dimensions. Other than that, "Affordable and Clean Energy" (3761 publications) and "Climate Action" (3091 publications) are the most frequently represented (and interconnected) objectives. The results obtained contribute to a greater understanding of the contribution of CF research to the attainment of the SDGs., (© 2023. The Author(s), under exclusive licence to Springer-Verlag GmbH Germany, part of Springer Nature.)

The widespread usage of Schiff bases in chemistry, industry, medicine, and pharmacy has increased interest in these compounds. Schiff bases and derivative compounds have important bioactive properties. Heterocyclic compounds containing phenol derivative groups in their structure have the potential to capture free radicals that can cause diseases. In this study, we designed and synthesized eight Schiff bases ( 10 - 15 ) and hydrazineylidene derivatives ( 16 - 17 ), which contain phenol moieties and have the potential to be used as synthetic antioxidants, for the first time using microwave energy. Additionally, the antioxidant effects of Schiff bases ( 10 - 15 ) and hydrazineylidene derivatives ( 16 - 17 ) were studied using by the bioanalytical methods of 2,2'-azino-bis(3-ethylbenzothiazoline-6-sulfonic acid) cation radical (ABTS •+ ) and 1,1-diphenyl-2-picrylhydrazyl (DPPH • ) scavenging activities, and Fe 3+ , Cu 2+ , and Fe 3+ -TPTZ complex reducing capacities. In the context of studies on antioxidants, Schiff bases ( 10 - 15 ) and hydrazineylidene derivatives ( 16 - 17 ) were found to be as powerful DPPH (IC 50 : 12.15-99.01 μg/mL) and ABTS •+ (IC 50 : 4.30-34.65 μg/mL). Additionally, the inhibition abilities of Schiff bases ( 10 - 15 ) and hydrazineylidene derivatives ( 16 - 17 ) were determined towards some metabolic enzymes including acetylcholinesterase (AChE), butyrylcholinesterase (BChE), and human carbonic anhydrase I and II (hCAs I and II), enzymes that are linked to some global disorders including Alzheimer's disease (AD), epilepsy, and glaucoma. In the context of studies on enzyme inhibition, it was observed that the synthesized Schiff bases ( 10 - 15 ) and hydrazineylidene derivatives ( 16 - 17 ) inhibited AChE, BChE, hCAs I, and hCA II enzymes with IC 50 values in ranges of 16.11-57.75 nM, 19.80-53.31 nM, 26.08 ± 8.53 nM, and 85.79 ± 24.80 nM, respectively. In addition, in light of the results obtained, we hope that this study will be useful and guiding for the evaluation of biological activities in the fields of the food, medical, and pharmaceutical industries in the future.

Claude syndrome is a rare midbrain stroke syndrome characterized by ipsilateral third cranial nerve palsy and contralateral hemiataxia. So far, only a few cases have been reported in childhood. We present two children with Claude syndrome at 9 and 15 years of age. The typical clinical picture was consistent with brain magnetic resonance imaging findings. A thorough investigation regarding the underlying etiology revealed no definite diagnosis but clues suggestive of probable neuro-Behcet disease. Awareness of pediatric neurologists on arterial ischemic stroke has been increasing over the past decades, enabling timely diagnosis and appropriate management of rare childhood cases with midbrain stroke., Competing Interests: None declared., (Thieme. All rights reserved.)

Background: Turkey is one of the traditional oriental tobacco producing countries. Oriental tobaccos produced in Turkey are ecotypes specific to certain regions, local varieties and landraces, and they have unique characteristics. The present study was conducted to reveal the general and interregional genetic diversity levels of tobacco genotypes collected from different regions of Turkey based on DNA markers., Methods and Results: A total of 319 ecotypes/lines of Turkish Oriental Tobaccos collected from different regions (Aegean Region, Marmara Region, Karadeniz Region, and East and Southeast Anatolia Regions) of Turkey and cultivars Xanthi 81, Xanthi 2A, Canik Sitmasuyu, Katerini, Canik 190-5 and NC 55 were subjected to SSR marker analysis. Eighty-nine alleles were obtained from 21 markers examined, and the average number of alleles per marker was 4.05. A total of 314 unique tobacco genotypes were found among 319 plants evaluated using 19 polymorphic SSR markers. In the dendrogram constructed using SSR marker data, genotypes were divided into six clades based on geographic regions and levels of genetic diversity., Conclusions: The presence of 314 unique genotypes among 319 tobacco plants evaluated indicated the high level of genetic diversity among Turkish tobaccos. While some of them were N. rustica tobaccos, some others were reminiscent of semi-oriental tobaccos. These genotypes merit further agronomic and technological characterizations, which could allow their utilization in future tobacco breeding., (© 2022. The Author(s), under exclusive licence to Springer Nature B.V.)

Objective: Febrile neutropenia (FN) represents a life-threatening complication in hematological malignancies. We aimed to analyze the utility of soluble vascular cell adhesion molecule 1 (sVCAM-1), intercellular adhesion molecule 1 (sICAM-1), vascular endothelial growth factor (VEGF) levels compared with C-reactive protein (CRP) and procalcitonin (PCT) during febrile neutropenia episodes of pediatric patients with leukemia., Methods: Two plasma samples, on day 0 (initial of episode) and day 3 (48-72 h after episode), for VCAM-1, ICAM-1 and VEGF, CRP and PCT were prospectively collected concomitantly during each febrile neutropenic episode between December 2016 and December 2017. The primary outcome was bacteremia and the secondary outcome was intensive care unit (ICU) admission., Results: Twenty-two (28.6%) acute lymphoblastic lymphoma (ALL), seventeen (22.1%) acute myeloblastic lymphoma (AML) patients and thirty-eight (49.3%) control patients with no known underlying disease or fever were included in this study. Of the 39 patients; 16 (41%) had bacteremia. Mean serum sVCAM1 and sICAM1 levels were significantly higher in control group, compared to FN patients (p < 0.001). Mean serum sVCAM2 level was significantly higher in FN patients with bacteremia compared to FN patients without bacteremia (144.97 ± 70.35 pg/mL vs 85.45 ± 53.76 pg/mL, p = 0.022). Mean sVCAM1 and 2 levels were higher in FN patients with ICU admission. In this study, we found that sVCAM-1 and VEGF, when combined to CRP and PCT, could predict gram-negative bacteremia in FN episodes of pediatric hematological malignancy., Conclusion: Serum endothelial adhesion molecules, excluding sVCAM-1, cannot predict bacteremia and ICU admission alone in FN patients; but may be associated with clinical outcome when used with PCT and CRP., (Copyright © 2021. Published by Elsevier Ltd.)

Introduction: Haemophilia is a bleeding disorder that occurs due to the deficiency of coagulation factors, and the angiogenesis process is an important process underlying the pathophysiology of haemophilic arthropathy. The role of the new adipocytokine endoglin (ENG) in patients with haemophilia is not yet known., Aim: The aim of this study is to evaluate the association between ENG protein and angiogenesis-related cytokines in patients with haemophilia for the first time., Methods: Plasma protein levels and mRNA expressions of ENG and various angiogenesis-associated cytokines were compared in blood samples collected from 28 patients with haemophilia A or B and 29 healthy volunteers. The relationship between the cytokines and ENG were determined by correlation analysis., Results: Plasma ENG levels and angiogenic markers were found to be significantly higher in patients with haemophilia compared to controls. Real-time PCR studies showed that mRNA expressions of ENG, vascular endothelial growth factor A, hypoxia-inducible factor A, and prostaglandin E2 increased in patients with haemophilia. Correlation analysis showed a significant positive correlation between ENG and angiopoietin-2 levels in the haemophilia group. Besides, a significant decrease in annexin-V binding to platelets in haemophilia patients compared to control was found to be related to the bleeding profiles in the patients., Conclusions: This study determined that ENG protein may be involved in the formation of angiogenesis in haemophilia patients and its effects may be related to angiogenetic marker angiopoietin-2 in this process. Our findings contribute to the literature during the determination of target proteins in haemophilia treatment., (© 2021 John Wiley & Sons Ltd.)

Objective: To investigate the value of presepsin and proadrenomedullin (proADM) as new markers for febrile neutropenia, by comparing them with conventional markers., Methods: Plasma specimens for presepsin, proADM, C-reactive protein (CRP), and procalcitonin (PCT) were collected every 3 days during each episode of febrile neutropenia., Results: A total of 39 patients experiencing a collective 47 episodes of febrile neutropenia with hematological malignant neoplasms, as well as 40 healthy control patients without infectious disease, were enrolled in this study. Levels of the studied analytes in the presepsin 1 group (with baseline values taken at admission), presepsin 2 group (values recorded on the 3rd day of febrile neutropenia), and presepsin 3 group (values recorded on the 6th day of hospitalization) were all higher in the subgroups with bacteremia. C-reactive protein 1 (baseline value taken at admission), procalcitonin 1 (as recorded at admission), and procalcitonin 2 (recorded on the 3rd day of febrile neutropenia) were higher in the subroups with bacteremia (P =.03, P = .04, and P = .04, respectively). In multivariate logistic regression analysis, presepsin 1 and/or PCT 1/CRP 1 combined analysis was superior in predicting bacteremia., Conclusion: Presepsin could be used in combination with other biomarkers to detect bacteremia., (© American Society for Clinical Pathology, 2021. All rights reserved. For permissions, please e-mail: journals.permissions@oup.com.)

Background: Invasive fungal infections (IFIs) are a leading cause of morbidity and death in immunocompromised patients. Data on efficacy and pharmacokinetics of posaconazole in pediatric patients are rare (1 to 5). Herein, we retrospectively analyzed adolescent patients who had received posaconazole as antifungal prophylaxis., Methods: We retrospectively analyzed patients who received posaconazole as primary or secondary antifungal prophylaxis., Results: A total of 34 adolescent patients, 19 men (55.9%) and 15 women (44.1%) with a mean age of 15.8±2.1 years were included. Twenty-five of 34 (73.5%) patients were on primary and nine of 34 (26.5%) patients were on secondary antifungal prophylaxis. Diagnosis of the patients receiving posaconazole as primary antifungal prophylaxis were acute myeloid leukemia (n=12, 48%), hematopoietic stem cell transplantation (n=7, 28%), acute lymphoblastic leukemia (n=5, 20%), and Fanconi aplastic anemia (n=1, 4%). Five patients (55.6%) with hematopoietic stem cell transplantation, 1 patient with acute myeloid leukemia (11.1%), 1 patient with Fanconi aplastic anemia (11.1%), and 2 (22.2%) patients with chronic granulomatous disease received posaconazole as secondary antifungal prophylaxis. Twelve of 25 (48%) patients receiving posaconazole as primary antifungal prophylaxis were complicated by IFI; 4 of them were proven, 6 probable, and 2 with possible IFI. Three of 9 patients (33.3%) receiving posaconazole as secondary antifungal prophylaxis was complicated by IFI (P=0.29), 2 of them were probable and 1 was possible IFI. Five of 25 patients (20%) receiving posaconazole as primary prophylaxis died because of IFI., Conclusion: Improvement of antifungal prophylaxis in patients with high risk of invasive infections seems clearly necessary, and analyzing serum posaconazole levels and individualizing dosing may be 1 approach to improve outcomes., Competing Interests: The authors declare no conflict of interest., (Copyright © 2021 Wolters Kluwer Health, Inc. All rights reserved.)

Colchicine is the mainstay of treatment for familial Mediterranean fever. We investigated the frequency of leukopenia in 213 patients with familial Mediterranean fever treated with standard doses of colchicine (0.5-2.0 mg/day). We found that 23 patients (10.8%) had reversible leukopenia, 3 moderate, and none severe and that their rate of infections was not increased., (Copyright © 2020 Elsevier Inc. All rights reserved.)

Objective: This study was conducted to examine the effect of inhaler drug education on fatigue, dyspnea severity, and respiratory function tests in patients with chronic obstructive pulmonary disease (COPD)., Methods: This randomized controlled study was conducted with a total of 85 patients treated at the chest diseases clinic and outpatient clinic of a state hospital. The data were collected using questionnaires, respiratory function tests, and the COPD and Asthma Fatigue Scale (CAFS) and Visual Analogue Scale (VAS-for dyspnea severity)., Results: It was determined that after 4 weeks of inhaler drug education, the CAFS mean score decreased to 35.32 ± 14.36, the dyspnea severity mean score decreased to 4.76 ± 2.50, and the respiratory function tests mean scores increased to forced expiratory volume in 1 s (FEV 1 ) = 58.83 ± 25.48, forced vital capacity (FVC) = 59.04 ± 19.19, and FEV 1 /FVC = 88.39 ± 21.59 in the intervention group (p < 0.05). No change was observed in the patients in the control group except for FEV 1 and FVC mean scores (p > 0.05)., Conclusion: These results demonstrate that inhaler drug education can improve fatigue, dyspnea severity, and respiratory function tests of patients with COPD., Practice Implications: The training by nurses of every patient hospitalized in the clinic regarding inhaler drug therapy, and the regular checking of patients' way of using the inhaled drugs, will contribute to COPD management., Competing Interests: Declaration of Competing Interest All authors certify that there is no conflict of interest with financial, personal or other relationships with other people or organizations within three years of beginning the submitted work that could inappropriately influence, or be perceived to influence, their work., (Copyright © 2019. Published by Elsevier B.V.)

Pelvic fractures may range from highly severe, life-threatening injuries to less acute clinical entities. There are several sub-entities that are summed up as pelvic injuries. Anatomically, there are fractures of the anterior or posterior pelvic ring. Apart from these, there are fractures of the acetabulum that make up about one fifth of all pelvic injuries. The indication for surgical treatment of pelvic ring injuries depends on the type of injury, involvement of anterior and/or posterior elements of the pelvic ring, demands and the general condition of the patient. In acetabular fractures, indications depend on the dislocation of the fracture and of course also the needs of the patient and his general condition. An intraarticular step-off of more than 2 mm is usually considered as an indication for open reduction and osteosynthesis. Usually in all these injuries, a preoperative CT scan is mandatory to allow precise planning of the operative approach and technique. Intraoperatively, the surgeon should be familiar with the acquisition of the 2D standard views, including 2D imaging of the pelvic ring and the acetabulum. These consist of the anteroposterior view for both pelvic ring and acetabular osteosyntheses. For further assessment of pelvic ring treatments, inlet and outlet views are achievable by angulating the C-arm cranially and caudally. To assess aspects of the anterior and posterior column of the acetabulum, iliac oblique views are used. Here, the C-arm is rotated laterally. As evaluation of 2D views can be limited due to anatomy and superposing structures, intraoperative 3D imaging has become common in the last decade. Special C-arms allow the automatic acquisition of large numbers of projections and create CT-like views of the central volume. Although this method has significantly widened the possibilities of intraoperative imaging, some issues remain. Depending on the amount of implants placed in the imaging field, assessment can be seriously impaired due to artefacts caused by the implants. Intraoperative CT imaging promises enhanced image quality for artefacts and allows a considerably larger field of view. The use of radiation-free navigation facilitates implant placement in minimally invasive procedures like screw placement in the sacroiliacal joint or the acetabulum by visualisation of instruments and implants in a pre- or intraoperative 3D data set., Competing Interests: One of the authors received lecture fees from Brainlab AG (Munich)./Einer der Autoren erhielt Vortragshonorare von der Brainlab AG (München)., (Georg Thieme Verlag KG Stuttgart · New York.)

Glucose-6-phosphate isomerase (GPI, EC 5.3.1.9) is a dimeric enzyme that catalyzes the reversible isomerization of glucose-6-phosphate to fructose-6-phosphate, the second reaction step of glycolysis. GPI deficiency, transmitted as an autosomal recessive trait, is considered the second most common erythro-enzymopathy of anaerobic glycolysis, after pyruvate kinase deficiency. Despite this, this defect may sometimes be misdiagnosed and only about 60 cases of GPI deficiency have been reported. GPI deficient patients are affected by chronic non-spherocytic hemolytic anemia of variable severity; in rare cases, intellectual disability or neuromuscular symptoms have also been reported. The gene locus encoding GPI is located on chromosome 19q13.1 and contains 18 exons. So far, about 40 causative mutations have been identified. We report the clinical, hematological and molecular characteristics of 12 GPI deficient cases (eight males, four females) from 11 families, with a median age at admission of 13 years (ranging from 1 to 51); eight of them were of Italian origin. Patients displayed moderate to severe anemia, that improves with aging. Splenectomy does not always result in the amelioration of anemia but may be considered in transfusion-dependent patients to reduce transfusion intervals. None of the patients described here displayed neurological impairment attributable to the enzyme defect. We identified 13 different mutations in the GPI gene, six of them have never been described before; the new mutations affect highly conserved residues and were not detected in 1000 Genomes and HGMD databases and were considered pathogenic by several mutation algorithms. This is the largest series of GPI deficient patients so far reported in a single study. The study confirms the great heterogeneity of the molecular defect and provides new insights on clinical and molecular aspects of this disease.

Familial hemophagocytic lymphohistiocytosis is an autosomal recessive, life-threatening condition characterized by defective immune response. A retrospective analysis was performed on 57 patients diagnosed with familial hemophagocytic lymphohistiocytosis at Hacettepe University Pediatric Hematology Department, Ankara, Turkey. Mutation analysis was performed on 37 patients, and of these: 11 had UNC13D , 10 had PRF1 and 3 had STX11 gene mutation. Of these patients, 44% were found to have central nervous system involvement on admission and spinal cord involvement was also seen in 5 patients. Remission was achieved in 24 patients with the treatment, in a median time of 76 days (min-max: 15-705 days). Time to remission was prolonged 3.1 times in patients with a ferritin level 1500 mg/dL or more. When patients were grouped according to age [Group 1 (≤ 2 years), Group 2 (>2 years)]; patients in Group 1 had higher ferritin and aspartate aminotransferase levels but lower fibrinogen levels. The 5-year survival rate was also lower in Group 1. When patients in Group 1 were divided into two sub-groups according to hepatic involvement, the 5-year survival rate of patients who had hepatic involvement was significantly lower than those patients without hepatic involvement (0.7%, 27%, respectively) ( P =0.002). The 5-year survival rate of patients who underwent hematopoietic stem cell transplantation was significantly higher than the patients who didn't (44%, 16%, respectively) ( P =0.02). In conclusion, age two years and under, ferritin level above 1500 mg/dL, spinal cord or hepatic involvement should be considered as poor prognostic factors in familial hemophagocytic lymphohistiocytosis., (Copyright© 2018 Ferrata Storti Foundation.)

Purpose: Determination of the appropriate treatment of unstable simple elbow dislocations is difficult and a topic of ongoing discussion. The aim of this study was to analyse the outcome and complications after surgery and conservative treatment, with special focus on post-traumatic joint laxity., Methods: In this retrospective study, 118 consecutive patients with simple elbow dislocations underwent stability testing by fluoroscopy after joint reduction and were assigned to groups 1 (slight), 2 (moderate) or 3 (gross) depending on post-traumatic joint laxity. All patients of group 1 underwent conservative treatment, and of group 3 primary ligament repair. In patients with moderate elbow laxity, the treatment was decided individually. All patients underwent a similar functional rehabilitation programme during treatment. Clinical outcome was determined after an average of 3.4 ± 1.5 years using the Mayo Elbow Performance Score (MEPS), and treatment-associated complications and revisions were recorded., Results: Forty-nine patients (41.5 %) were assigned to group 1, 41 patients (34.7 %) to group 2 and 28 patients (23.7 %) to group 3. In group 2, 22 patients underwent ligament repair, while 19 patients were treated conservatively. On average, an excellent MEPS was achieved in group 1 after conservative treatment (MEPS 95.8 ± 9.0), similar to results after ligament repair of grossly unstable elbows in group 3 (91.6 ± 11.7). Interestingly, in group 2 conservative treatment was associated with a slightly lower MEPS (90.0 vs. 95.7), and significantly fewer patients achieved an excellent MEPS (81.8 vs. 52.6 %, p = 0.045). Similarly, conservative treatment in group 2 was associated with a fivefold to sixfold risk of complications (p = 0.032) and revision surgery (p = 0.023)., Conclusions: This study supports the notion that patients with slight elbow laxity can be treated non-operatively, while primary surgical treatment should be performed in patients with moderate and gross laxity to avoid post-traumatic sequelae and decrease revision rates., Level of Evidence: Retrospective Cohort Study, Level III.

Purpose: Sports activity after surgical AC joint stabilisation has not been comprehensively evaluated to date. The aim of this study was to determine rate, level and time to return to sports after AC joint stabilisation and to identify the influence of overhead sports on post-operative sports activity., Methods: In this retrospective case series, a total of 68 patients with a high-grade AC joint dislocation (Rockwood type V) were stabilised using a single TightRope technique. Fifty-five patients (80.9 %) with median age of 42.0 (range, 18-65) years completed questionnaires regarding sports activity before and after surgery. Clinical outcome and complications were also evaluated. Forty-three patients participated in sports regularly before injury. Their sports activity was rated according to Allain, and non-overhead and overhead sports were differentiated., Results: At median follow-up of 24 (18-45) months, 41 of 43 patients (95.3 %) had returned to sports. 63 % returned to the same sports activity as before injury. 16.3 % needed to adapt the type of sports to reduce demanding activities. 11.6 % reduced the frequency and 32.5 % the intensity of sports. The median time to return to sports was 9.5 (3-18) months. Overhead athletes (Allain Type III and IV) had to reduce their sports activity significantly more often (11.8 vs. 53.8 %; p = 0.011) and needed more time to return to sports (9.5 vs. 4.5 months; p = 0.009)., Conclusion: After stabilisation of AC joint dislocation, the majority of patients returned to sports after a substantial period of time. Overhead athletes, in particular, required more time and had to considerably reduce their sports activity. The findings impact therapeutic decision-making after AC joint injury and help with the prognosis and assessment of rehabilitation progress., Level of Evidence: IV.

Background: The aim of this study was to develop and validate a novel self-administered questionnaire for assessing the patient's own range of motion (ROM) of the wrist and the elbow., Methods: In a prospective clinical study from January 2015 to June 2015, 101 consecutive patients were evaluated with a novel, self-administered, diagram-based, wrist motion assessment score (W-MAS) and elbow motion assessment score (E-MAS). The questionnaire was statistically evaluated for test-retest reliability, patient-physician agreement, comparison with healthy population, and influence of covariates (age, gender, affected side and involvement in workers' compensation cases)., Results: Assessment of patient-physician agreement demonstrated almost perfect agreement (k > 0.80) with regard to six out of eight items. There was substantial agreement with regard to two items: elbow extension (k = 0.76) and pronation (k = 0.75). The assessment of the test-retest reliability revealed at least substantial agreement (k = 0.70). The questionnaire revealed a high discriminative power when comparing the healthy population with the study group (p = 0.007 or lower for every item). Age, gender, affected side and involvement in workers' compensation cases did not in general significantly influence the patient-physician agreement for the questionnaire., Conclusion: The W-MAS and E-MAS are valid and reliable self-administered questionnaires that provide a high level of patient-physician agreement for the assessments of wrist and elbow ROM., Level of Evidence: Diagnostic study, Level II.

Background: Conservative treatment of simple elbow dislocations can lead to complications such as persisting pain and restricted joint mobility. The current aim was to identify patients with deteriorated outcome after conservative treatment and to investigate a possible association with initial joint (in)stability., Methods: Sixty-eight patients (mean age 37.1 ± 17.2 years) with simple elbow dislocations received conservative treatment. After closed reduction, joint stability was tested by varus and valgus stress under fluoroscopy. According to the findings under fluoroscopy, three different groups of instability could be identified: (1) slight instability (joint angulation <10°; n = 49), (2) moderate instability (angulation ≥10°; n = 19) and (3) gross instability. Patients with gross instability (re-dislocation under stability testing) were treated with primary surgical ligament repair and therefore excluded from this study. Additionally, MRIs and radiographs were analysed regarding warning signs of instability such as the drop sign and joint incongruence. Main outcome parameters were the Mayo Elbow Performance Score (MEPS), range of motion (ROM), complications and revision rates., Results: After 40.7 ± 20.4 months, the overall MEPS was excellent (94.2 ± 11.3) with a trend of slightly worse clinical results in group 2 (95.8 ± 9.0 vs. 90.0 ± 15.2 points; p = 0.154). In group 1, significantly more patients achieved an excellent result regarding the MEPS scoring system (77.6 vs. 52.6 %; p = 0.043) and elbow extension was significantly worse in group 2 (5.3 ± 9.9° vs. 1.4 ± 3.0°; p = 0.015). Seven treatment complications occurred in group 2 (36.8 %) compared with two in group 1 (4.1 %, p < 0.0001). Six patients (8.8 %) needed secondary surgery with an 8.4-fold higher risk for revision surgery in group 2 (p = 0.007). The presence of a positive drop sign or joint incongruence led to higher odds ratio (OR) for complications (OR = 15.9) and revision surgery (OR = 10.3)., Conclusions: This study demonstrates that patients with moderate joint instability after simple elbow dislocation have a significantly worse clinical outcome, more complications and a higher need for secondary revision surgery following conservative treatment compared to patients with slight elbow instability.