bluuebunny/crossref_metadata_2025_split huggingface dataset:
Retrospective data collection of psoriasis treatment with fumaric acid esters in children and adolescents in Germany (KIDS FUTURE study)
Authors: Reich Kristian , Hartl Christoph , Gambichler Thilo , Zschocke Ina 
Published: December 2015

SummaryBackgroundGiven that there is no standard systemic treatment for children and adolescents with plaque psoriasis, this non‐interventional, multicenter, retrospective study collected data on the efficacy and safety of long‐term treatment with fumaric acid esters (FAEs) in this particular patient group.Patients and methodsIn patients younger than 18 years of age at the start of FAE treatment, data on efficacy and safety was retrospectively collected for at least 36 months.ResultsData from 127 patients (aged 6–17 years) was collected for treatment durations of up to 60 months. Physician's Global Assessment, Psoriasis Area and Severity Index, and Body Surface Area showed marked improvement in the first six months. After 36 months, these parameters had, on average, improved by up to two‐thirds of baseline values. Thirty‐seven patients experienced at least one adverse event (AE), which was FAE‐related in 36 individuals. Three AEs (proteinuria (one case), flushing (two cases)) persisted during the observation period while on treatment. Fifteen AEs led to the discontinuation of therapy; nearly all of these cases were related to gastrointestinal disorders.ConclusionsThe KIDS FUTURE study – for the first time – included a larger population of children and adolescents with psoriasis who were treated with FAEs. The data obtained suggests that long‐term FAE therapy in this patient group may be effective and safe. The results are currently being verified in an ongoing clinical study.
Misdiagnosed microcystic adnexal carcinoma on the lateral forehead and challenges in reconstruction of a large and bone‐deep defect
Authors: Fallaha Amgiad , Thuile Tobias , Tappeiner Lukas , Pichler Maria , Deluca Jenny , Perino Franco , Eisendle Klaus 
Published: December 2015

SummaryBackgroundMicrocystic adnexal carcinoma (MAC) is a rare cutaneous malignancy characterized by aggressive local infiltration, including a high propensity for perineural invasion. Histologically it can be easily confused with benign adnexal tumors, which often leads to inappropriate initial treatment. As a consequence delayed surgical excision often requires removal of large cutaneous masses which can be followed by challenges in reconstruction.MethodsWe report the case of a challenging reconstruction of a large forehead defect by a modified AT flap with central Burow's triangle grafting and previous wound conditioning using hyaluronic acid after complete micrographic removal of MAC.ResultsA 80‐year‐old healthy female presented with an almost six year history of an asymptomatic slowly progressing skin mass previously biopsied and diagnosed as benign syringoma five years in advance. Deep biopsies confirmed MAC. She underwent staged surgical excision with Tübingen torte technique with removal of the frontal periostium. A skin substitute of esterified hyaluronic acid was applied for three weeks to the bone‐deep 6×7 cm defect leading to good granulation tissue above the bone. Final reconstruction was achieved by an AT flap combined with full thickness skin transplantation of the central dog ear. The cosmetic outcome was satisfactory, no recurrence was observed within 18 months follow‐up.ConclusionsWe confirm the histological difficulties in the diagnosis of this tumor entity and the large extension of MAC due to delay in diagnosis and treatment. Deep tumor removal including the periostium might be necessary to achieve tumor free margins. Fast granulation tissue above the frontal bone might be achieved by applying hyaluronic acid products. The combination an AT flap with transplantation of the adjacent Burow's triangles gives optimal skin texture and color matching and seems to be a good option to close even large defects of the front especially for supra brow defects.
S2k‐Leitlinie Neurodermitis [atopisches Ekzem; atopische Dermatitis] – Kurzversion
Authors: Werfel Thomas , Heratizadeh Annice , Aberer Werner , Ahrens Frank , Augustin Matthias , Biedermann Tilo , Diepgen Thomas , Fölster‐Holst Regina , Gieler Uwe , Kahle Julia , Kapp Alexander , Nast Alexander , Nemat Katja , Ott Hagen , Przybilla Bernhard , Roecken Martin , Schlaeger Martin , Schmid‐Grendelmeier Peter , Schmitt Jochen , Schwennesen Thomas , Staab Doris , Worm Margitta 
Published: December 2015

ZusammenfassungBei dem Krankheitsbild der Neurodermitis handelt es sich um eine chronische oder chronisch‐rezidivierende, nichtkontagiöse, entzündliche Hauterkrankung mit in der Regel starkem Juckreiz. Darüber hinaus besteht ein Risiko für komplizierte Verläufe mit bakteriellen oder viralen Superinfektionen. Sowohl die genetische Prädisposition als auch zahlreiche Auslösefaktoren spielen für die Erstmanifestation und das Auftreten der Erkrankungsschübe eine wichtige Rolle, so dass auch die Therapiekonzepte vielfältig sind. Bei zahlreichen für die Neurodermitis zur Verfügung stehenden Behandlungsoptionen gilt es, in Abstimmung mit den Patienten bzw. Eltern erkrankter Kinder fallorientiert einen optimalen Behandlungsplan aufzustellen, der im Verlauf ggf. erneut angepasst werden muss.Die vorliegende Kurzfassung der S2k‐Leitlinie gibt einen Überblick über alle bisher zur Verfügung stehenden, evidenzbasierten Diagnoseverfahren und Therapiemöglichkeiten sowie über die entsprechenden Empfehlungen, die durch die an dieser Leitlinie beteiligten Fachgesellschaften und Verbände ausgesprochen werden. Diese Empfehlungen wurden auf der Grundlage der bislang zu dem jeweiligen Verfahren vorliegenden klinisch‐wissenschaftlichen Datenlage, die in der ausführlichen Fassung dieser Leitlinie (unter www.awmf.org und jddg.org) beschrieben ist, konsentiert.
Cardiac Complications in 38 Cases of Kawasaki Disease with Coronary Artery Aneurysm Diagnosed by Echocardiography
Authors: Wei Ya Juan , Zhao Xiao Lan , Liu Bao Min , Niu Hua , Li Qian 
Published: December 2015

BackgroundThe long‐term prognosis of patients with Kawasaki disease (KD) complicated by coronary artery aneurysm (CAA) is unclear. The aim of this study was to evaluate the complications of KD with CAAs.MethodWe retrospectively analyzed the clinical data and complications of 38 KD patients with CAAs who were treated and underwent regular follow‐up with echocardiography between January 1989 and May 2013.ResultsDuring a period of 29 days to 19 years after disease onset, complications seen included coronary stenosis and occlusion (six patients), thrombosis (17 patients), myocardial infarction (six patients), and calcification of CAAs (seven patients). Rupture of giant CAAs occurred in two patients and caused sudden death in one of these patients at 29 days and in the other patient at 5 months after disease onset. A total of seven deaths occurred, with five deaths caused by myocardial infarction. Three of these had undiagnosed incomplete KD or had not received regular treatment, while two experienced sudden death after several asymptomatic myocardial infarctions.ConclusionCardiac complications of KD with CAAs include thrombosis, coronary stenosis, myocardial infarction, sudden death, and calcification. Although rare, rupture of giant CAAs is fatal and might occur earlier after the onset of disease. Mortality occurred primarily in the earlier cases when anticoagulant therapy was insufficient and in patients who did not receive regular treatment. Echocardiography can provide reliable information for assessing the progression and prognosis of this condition.
Smoldering multiple myeloma risk factors for progression: a Danish population‐based cohort study
Authors: Sørrig Rasmus , Klausen Tobias W. , Salomo Morten , Vangsted Annette J. , Østergaard Brian , Gregersen Henrik , Frølund Ulf Christian , Andersen Niels F. , Helleberg Carsten , Andersen Kristian T. , Pedersen Robert S. , Pedersen Per , Abildgaard Niels , Gimsing Peter ,   the Danish Myeloma Study Group
Published: December 2015

AbstractSeveral risk scores for disease progression in patients with smoldering multiple myeloma (SMM) have been proposed; however, all have been developed using single‐center registries. To examine risk factors for time to progression (TTP) to multiple myeloma (MM) for SMM, we analyzed a nationwide population‐based cohort of 321 patients with newly diagnosed SMM registered within the Danish Multiple Myeloma Registry between 2005 and 2014. Significant univariable risk factors for TTP were selected for multivariable Cox regression analyses. We found that both an M‐protein ≥30 g/L and immunoparesis significantly influenced TTP (HR 2.7, 95%CI (1.5;4.7), P = 0.001, and HR 3.3, 95%CI (1.4;7.8), P = 0.002, respectively). High free light chain (FLC) ratio did not significantly influence TTP in our cohort. Therefore, our data do not support recent IMWG proposal of identifying patients with FLC ratio above 100 as having ultra high‐risk of transformation to MM. Using only immunoparesis and M‐protein ≥30 g/L, we created a scoring system to identify low‐, intermediate‐, and high‐risk SMM. This first population‐based study of patients with SMM confirms that an M‐protein ≥30 g/L and immunoparesis remain important risk factors for progression to MM.
Spanish consensus guidelines on prophylaxis with bypassing agents for surgery in patients with haemophilia and inhibitors
Authors: Mingot‐Castellano María Eva , Álvarez‐Román María Teresa , López‐Fernández María Fernanda , Altisent‐Roca Carmen , Canaro‐Hirnyk Mariana Isabel , Jiménez‐Yuste Víctor , Cid‐Haro Ana Rosa , Pérez‐Garrido Rosario , Sedano‐Balbas Carmen 
Published: December 2015

AbstractIntroductionPatients with severe haemophilia and inhibitors against factor VIII who require surgery need a prophylactic approach to prevent bleeding complications. Scientific evidence to decide the best prophylactic treatment is very limited and mainly based on retrospective or case series.AimsTo develop evidence‐ and expert opinion‐based guidelines for prophylactic therapy for patients with haemophilia and inhibitors undergoing surgery.MethodsA panel of nine Spanish haematologists undertook a systematic review of the literature and selected publications providing relevant information regarding the prophylactic management of patients with haemophilia and inhibitors undergoing dental extraction, minor surgery or major surgery.ResultsAlthough evidence is very limited, the panel considers that it seems advisable that prophylaxis should be given in most cases with a bypassing agent (aPCC or rFVIIa) and should start immediately before minor or major surgery. Patients should be closely monitored to enable dose/product modification as needed.ConclusionIt is necessary to communicate clinical experience in a detailed way in order to ensure optimal schemes of prophylaxis for patients with haemophilia and inhibitors. Development of objective outcomes to evaluate efficacy is crucial.
Qualitative muscle mass index as a predictor of skeletal muscle function deficit in Asian older adults
Authors: Chang Jae Seung , Kim Tae Ho , Kim Hanul , Choi Eun Hee , Kim Nahyun , Kong In Deok 
Published: December 2015

AimThe present cross‐sectional study was carried out among community‐dwelling Koreans to determine the validity of various muscle mass indices and to propose more clinically relevant diagnostic criteria.MethodsThis study measured the anthropometrics, body composition and physical capability of 415 older Koreans. Skeletal muscle indices were calculated by dividing appendicular lean mass by height or weight. Apart from this, we adjusted appendicular lean mass for body mass index, body surface area or waist‐to‐height ratio, which we then named the qualitative muscle mass index. Skeletal muscle function deficit was defined as a combination of weakness and slowness.ResultsQualitative muscle indices were closely associated with physical capabilities. Receiver operating characteristic and logistic analyses showed that qualitative muscle indices had significantly greater discriminatory powers regarding low muscle function than did the height‐adjusted index in both men and women, and even showed higher discriminatory potentials than the weight‐adjusted index in men (all P < 0.05). The cut‐off values of qualitative muscle indices of body mass index‐, body surface area‐, and waist‐to‐height ratio‐adjusted indices for identifying functional deficits were 0.760, 11.40 and 34.18 for men, and 0.530, 8.91 and 23.07 for women, respectively.ConclusionsThe present study suggests that qualitative muscle indices are more accurate in predicting low muscle function than are height‐ and weight‐adjusted indices, because they consider anthropometric characteristics as part of the definition. The results might provide new avenues for conceptualizations of sarcopenia accompanied by obesity, and can be used as ethnic‐specific reference values of muscle mass indices based on functional outcome in an elderly Korean/Asian population. Geriatr Gerontol Int 2017; 17: 99–107.
Effect of oral cleaning using mouthwash and a mouth moisturizing gel on bacterial number and moisture level of the tongue surface of older adults requiring nursing care
Authors: Kobayashi Kenichiro , Ryu Masahiro , Izumi Sachi , Ueda Takayuki , Sakurai Kaoru 
Published: December 2015

AimTo evaluate the effect of oral cleaning using a mouthwash and a mouth moisturizing gel on the number of bacteria and moisture level of the tongue surface of older adults requiring nursing care.MethodsThe 60 participants were randomly divided into groups according to their use of oral cleaning procedures as follows: group 1, mouthwash and a moisturizing gel (M + m); group 2, mouthwash (M); group 3, water and a moisturizing gel (W + m); and group 4, water (W). The number of anaerobic bacteria, tongue coating index and moisture level of the tongue surface were measured at baseline, and after 1 and 2 weeks after cleaning commenced to compare the effectiveness of oral cleaning among the groups.ResultsThere was no significant difference in baseline measurements among the groups. The numbers of anaerobic bacteria decreased for all groups, and there were significant differences in the rates of decrease after 2 weeks between the M + m and W + m groups, M + m and W groups, and M and W groups. The tongue coating index decreased for all groups. There was no significant difference in the rate of decrease among the groups after 1 week, and there was a significant difference after 2 weeks between the M + m and W groups. The moisture levels of all groups increased, and there were significant differences after 2 weeks between the M + m and M groups, the M + m and W groups, and the W + m and W groups.ConclusionThe most effective cleaning technique was the combination of a mouthwash and a moisturizing gel. Geriatr Gerontol Int 2017; 17: 116–121.
Videoendoscopic evaluation of food bolus preparation: A comparison between normal adult dentates and older adult dentates
Authors: Matsuno Kohei , Nohara Kanji , Fukatsu Hikari , Tanaka Nobukazu , Fujii Nami , Sasao Yasuhiro , Sakai Takayoshi 
Published: December 2015

AimFood bolus preparation plays an important role in swallowing food. The ability to carry out oral functions varies with age, and the same might be true of the ability to prepare food boluses. Previously developed methods for assessing food bolus preparation were not able to evaluate swallowed boluses; that is, the boluses were spat out before they were swallowed. The aim of the present study was to evaluate food bolus preparation in older adults using videoendoscopy, and to compare the food bolus preparation abilities of older adults and younger healthy adults.MethodsThe participants were 30 older adults and 30 younger healthy adults. None of the participants showed any symptoms of dysphagia or eating difficulties. In each examination, an endoscope was inserted into the nasal passage, and the participant was instructed to eat cooked rice. The grinding, mixing and aggregation of each bolus was graded from 0 to 2, with higher numbers indicating more marked grinding, mixing and aggregation. We simultaneously investigated the number of chewing cycles.ResultsThe older adults showed higher grinding scores and lower mixing scores than the younger healthy adults. However, neither of these differences was significant. In contrast, the older adults showed significantly lower aggregation scores and carried out a significantly greater number of chewing cycles than the younger healthy adults.ConclusionsUsing videoendoscopy, we found that older adults showed lower aggregation scores and carried out a greater number of chewing cycles than younger healthy adults. These results suggest that older adults are less able to prepare food boluses than younger healthy adults. Geriatr Gerontol Int 2017; 17: 226–231.
Defining continuity of care from the perspectives of mental health service users and professionals: an exploratory, comparative study
Authors: Sweeney Angela , Davies Jonathon , McLaren Susan , Whittock Margaret , Lemma Ferew , Belling Ruth , Clement Sarah , Burns Tom , Catty Jocelyn , Jones Ian Rees , Rose Diana , Wykes Til 
Published: December 2015

AbstractBackgroundContinuity of care (COC) is central to the organization and delivery of mental health services. Traditional definitions have excluded service users, and this lack of involvement has been linked to poor conceptual clarity surrounding the term. Consequently, very little is known about the differences and similarities in the conceptualization of COC by mental health service users and professionals.ObjectiveTo explore and compare mental health service users’ and professionals’ definitions of COC.MethodsUsing an exploratory, qualitative design, five focus groups with 32 service users each met twice. Data were analysed thematically to generate a service user‐defined model of COC. In a cross‐sectional survey, health and social care professionals (n = 184) defined COC; responses were analysed thematically. Service user and professional definitions were conceptually mapped and compared to identify similarities and differences.ResultsThere was crossover between the service user and professional derived models of COC. Both contained temporal, quality, systemic, staff, hospital and needs‐related elements of COC. Service users prioritized access, information, peer support and avoiding services; health professionals most frequently referred to staff, cross‐sectional and temporal COC. Service users alone identified service avoidance, peer support and day centres as COC elements; professionals alone identified cross‐sectional working.ConclusionsImportant similarities and differences exist in service user and professional conceptualizations of COC. Further research is necessary to explore these differences, prior to integrating service user and professional perspectives in a validated COC framework which could enable the development and evaluation of interventions to improve COC, informing policy and practice.
Reappraisal of hMLH1 promoter methylation and protein expression status in the serrated neoplasia pathway
Authors: Lee Eun‐Jung , Chun Sung‐Min , Kim Mi‐Jung , Jang Se‐Jin , Kim Do Sun , Lee Doo Han , Youk Eui Gon 
Published: December 2015

AimsThe aim of this study was to determine whether human mutL homologue 1 (hMLH1) inactivation precedes the progression of sessile serrated lesion (SSL) into SSL with cytological dysplasia (SSL/D) and to define the histological stage at which promoter methylation and inactivation of hMLH1 occur.Methods and ResultsUsing the MassARRAY EpiTYPER assay and immunohistochemistry, we examined methylation levels and the protein expression status of hMLH1 in 33 SSL/Ds with conventional epithelial dysplasia and compared the results with those of control hyperplastic polyps (HPs) and SSLs. The methylation level of hMLH1 was higher in the dysplastic component than in the non‐dysplastic component of SSL/Ds (P = 0.005), and differed significantly with regard to the degree of dysplasia (P = 0.002). The methylation levels of hMLH1 in the dysplastic component of SSL/Ds tended to be higher than those of control SSLs and HPs (P = 0.063 and P = 0.017, respectively). The loss of hMLH1 protein expression was identified in only 13 of 33 (39.39%) dysplastic components of SSL/Ds.ConclusionPromoter methylation and loss of protein expression of hMLH1 are not parallel processes that occur concurrently. hMLH1 methylation is an early molecular event which occurs even in HP. However, the loss of hMLH1 expression is a much later step, found in approximately 40% of SSL/Ds at various histological stages. Notably, the loss of hMLH1 protein expression does not necessarily precede the development of cytological dysplasia in SSL.
Polymorphisms in STAT4 and IRF5 increase the risk of systemic sclerosis: a meta‐analysis
Authors: Xu Yang , Wang Wenling , Tian Yanli , Liu Jingyang , Yang Rongya 
Published: December 2015

AbstractBackgroundSystemic sclerosis (SSc) is the most severe connective tissue disorder. Recent studies have demonstrated that genetic factors may play a role in the development of SSc. The aim of this study was to investigate the association of signal transducer and activator of transcription 4 (STAT4) rs7574865 and interferon regulatory factor 5 (IRF5) rs2004640 polymorphisms with risk of SSc.MethodsCase–control studies were obtained from the electronic database of PubMed, Medline, Embase, and CNKI (China National Knowledge Infrastructure) up to December 2013. The association between STAT4 and IRF5 polymorphisms and SSc susceptibility was assessed by pooled odds ratios (ORs) and 95% confidence intervals (CI).ResultsSix related studies, including 4746 SSc cases and 7399 healthy controls, were pooled in this meta‐analysis. For STAT4 polymorphism, we observed a statistically significant positive association between risk factor T allele carriers and SSc susceptibility (OR = 1.37, 95% CI = 1.27–1.48, P < 0.00001) in the overall population. The presence of limited cutaneous (lcSSc) and diffuse cutaneous (dcSSc) scleroderma also showed a significant association with each of the genetic models (P < 0.00001). For IRF5 polymorphism, the T allele was shown to be strongly associated with increased SSc risk (OR = 1.27, 95% CI = 1.17–1.39, P < 0.00001). No significant heterogeneity between studies was found.ConclusionsThe results demonstrated that STAT4 rs7574865 and IRF5 rs2004640G/T substitution are associated with a susceptibility to SSc, and they may serve as the SSc genetic susceptibility factor. These data confirmed that genetic polymorphisms may play a role in the development of SSc and have provided new insight into the pathogenesis of SSc.