bluuebunny/crossref_metadata_2025_split huggingface dataset:
The influence of perceived medical risks and psychosocial concerns on photoprotection behaviours among adults with xeroderma pigmentosum: a qualitative interview study in the UK
Authors: Morgan Myfanwy , Anderson Rebecca , Walburn Jessica , Weinman John , Sarkany Robert 
Published: February 2019

BackgroundA high level of photoprotection is required by people with xeroderma pigmentosum (XP), a rare skin disease, to reduce skin cancer and other risks. However poor photoprotection is thought to be widespread.PurposeThis study examines the influences on photoprotection behaviours in adults with XP.DesignInductive qualitative study with semistructured interviews. Analysis employed a framework approach.SettingNational sample recruited through a specialist XP centre in London.MethodsSemistructured interviews at patients’ homes. All transcripts were coded and themes charted for each participant. Comparisons within and across cases identified common themes and differing motivations and approaches to photoprotection. Credibility of interpretations assessed through patient/carer input and clinic adherence scores.Participants25 adults (17 male, eight female) aged 16–63 years with diagnosed XP attending a specialist centre. 18 lived outside London.ResultsAwareness of risks of ultraviolet radiation (UVR) and photoprotection was high. However, photoprotection behaviours varied according to perceived necessity and concerns. Three behavioural responses were identified: (1)‘dominated’by planning and routines to achieve a high level of photoprotection with significant activity restrictions and psychosocial impacts. (2) ‘resistant’to photoprotection with priority given to avoiding an illness identity and enjoying a normal life. (3) Photoprotection’integrated’ with an individual’s life with little psychosocial impact. These responses were influenced by illness, personal and contextual factors including age, life stage and social support. Only the ‘integrated’ group achieved an equilibrium between perceived ‘necessity’ and ‘concerns’.ConclusionsThe personal balance between perceived risks of UVR and social/psychological ‘concerns’ led to differing behavioural responses and contributes to an understanding of adaptation and normalisation in chronic illness. The study will also inform a series of individualised behavioural interventions to reduce measured UVR exposure among people with XP that are potentially applicable to other conditions with high risks of skin cancer.
Family Violence Curricula in Europe (FAVICUE): a cross-sectional descriptive study protocol
Authors: Gómez Bravo Raquel , Lygidakis Charilaos , Feder Gene , Reuter Robert A P , Vögele Claus 
Published: February 2019

IntroductionFamily violence (FV) is a widespread public health problem of epidemic proportions and serious consequences. Doctors may be the first or only point of contact for victims who may be hesitant or unable to seek other sources of assistance, and they tend not to disclose abuse to doctors if not specifically asked. A comprehensive healthcare response is key to a coordinated community-wide approach to FV, but most of the practising physicians have received either no or insufficient education or training in any aspect of FV. Training of medical students concerning FV is often delivered in an inconsistent or ad hoc manner.The main aim of this project, Family Violence Curricula in Europe (FAVICUE), is to (1) describe current FV education delivery in European medical universities (undergraduate period) and during the specialist training in general practice (GP)/family medicine (FM) (postgraduate residency programme), and (2) compare it with WHO recommendations for FV curriculum.Methods and analysisThis is the protocol of a cross-sectional descriptive study consisting of two self-report online surveys (for undergraduate and postgraduate training, respectively) with 40 questions each. For both surveys, general practitioners, residents, medical students and professionals involved in their education from countries of the European region will be identified through the European Regional Branch of the World Organization of National Colleges, Academies and Academic Associations of General Practitioners/Family Physicians (WONCA Europe) and will be invited to provide information regarding the training on FV. Descriptive tests will be carried out and a thematic analysis will be conducted on the open-ended questions.Ethics and disseminationEthics approval has been obtained by the University of Luxembourg (ERP 17–015 FAVICUE). The results will provide important information concerning current curricula on FV, and can be used for mapping the educational needs and planning the implementation of future training interventions. They will be published and disseminated through WONCA Europe and its networks.
Respiratory chain polymorphisms and obesity in the Spanish population, a cross-sectional study
Authors: de Marco Griselda , Garcia-Garcia Ana Barbara , Real Jose Tomas , Gonzalez-Albert Veronica , Briongos-Figuero Laisa-Socorro , Cobos-Siles Marta , Lago-Sampedro Ana , Corbaton Arturo , Teresa Martinez-Larrad Maria , Carmena Rafael , Martin-Escudero Juan Carlos , Rojo-Martínez Gemma , Chaves Felipe Javier 
Published: February 2019

ObjectiveTo study the association of genes involved in the mitochondrial respiratory chain (MRC) pathway with body mass index (BMI) and obesity risk.DesignThis work studies three cross-sectional populations from Spain, representing three provinces: HORTEGA (Valladolid, Northwest/Centre), SEGOVIA (Segovia, Northwest/centre) and PIZARRA (Malaga,South).SettingForty-eight single nucleotide polymorphisms (SNPs) from MRC genes were selected and genotyped by SNPlex method. Association studies with BMI and obesity risk were performed for each population. These associations were then verified by analysis of the studied population as a whole (3731 samples).ParticipantsA total of 3731 Caucasian individuals: 1502 samples from HORTEGA, 988 from PIZARRA and 1241 from SEGOVIA.Resultsrs4600063 (SDHC), rs11205591 (NDUFS5) and rs10891319 (SDHD) SNPs were associated with BMI and obesity risk (p values for BMI were 0.04, 0.0011 and 0.0004, respectively, and for obesity risk, 0.0072, 0.039 and 0.0038). However, associations between rs4600063 and BMI and between these three SNPs and obesity risk are not significant if Bonferroni correction is considered. In addition, rs11205591 and rs10891319 polymorphisms showed an additive interaction with BMI and obesity risk.ConclusionsSeveral polymorphisms from genes coding MRC proteins may be involved in BMI variability and could be related to the risk to become obese in the Spanish general population.
Are more experienced clinicians better able to tolerate uncertainty and manage risks? A vignette study of doctors in three NHS emergency departments in England
Authors: Lawton Rebecca , Robinson Olivia , Harrison Rebecca , Mason Suzanne , Conner Mark , Wilson Brad 
Published: February 2019

BackgroundRisk aversion among junior doctors that manifests as greater intervention (ordering of tests, diagnostic procedures and so on) has been proposed as one of the possible causes for increased pressure in emergency departments (EDs). Here we tested the prediction that doctors with more experience would be more tolerant of uncertainty and therefore less risk-averse in decision making.MethodsIn this cross-sectional, vignette-based study, doctors working in three EDs were asked to complete a questionnaire measuring experience (length of service in EDs), reactions to uncertainty (Gerrity et al, 1995) and risk aversion (responses about the appropriateness of patient management decisions).ResultsData from 90 doctors were analysed. Doctors had worked in the ED for between 5 weeks and 21 years. We found a large association between experience and risk aversion so that more experienced clinicians made less risk-averse decisions (r=0.47, p<0.001). We also found a large association between experience and reactions to uncertainty (r=−0.50, p<0.001), with more experienced doctors being much more at ease with uncertainty. Mediation analyses indicated that tolerance of uncertainty partially mediated the relationship between experience and lower risk aversion, explaining about a quarter of the effect.ConclusionWhile we might be tempted to conclude from this research that experience and the ability to tolerate uncertainty lead to positive outcomes for patients (less risk-averse management strategies and higher levels of safety netting), what we are unable to conclude from this design is that these less risk-averse strategies improve patient safety.
Exhaustion of the immune system by Group A Streptococcus necrotizing fasciitis: the occurrence of late secondary infections in a retrospective study
Authors: Nawijn Femke , Wassenaar Emma C E , Smeeing Diederik P J , Vlaminckx Bart J M , Reinders Jan Siert K , Wille Jan , Leenen Luke P H , Hietbrink Falco 
Published: February 2019

BackgroundNecrotizing fasciitis is a potentially lethal condition for which early and adequate treatment with surgical debridement and broad-spectrum intravenous antibiotics are essential for survival. It is hypothesized that Group A Streptococcus (GAS) necrotizing fasciitis causes exhaustion of the immune system, making these patients more susceptible for late secondary infections.MethodsA retrospective study was conducted of all patients with necrotizing fasciitis between 2002 and 2016. Patients with necrotizing fasciitis based on macroscopic findings, positive Gram staining, culture or fresh frozen section of fascia biopsies were included. Patients with necrotizing fasciitis were divided into two groups based on the presence of GAS. Of both groups, clinical course, outcome and occurrence of late secondary infections were analyzed. For the occurrence of secondary infections, pneumonia was chosen as reference for late secondary infections.ResultsEighty-one patients with necrotizing fasciitis were included of which 38 (47%) had GAS necrotizing fasciitis and 43 (53%) had non-GAS necrotizing fasciitis. Patients with GAS necrotizing fasciitis were younger (50 vs. 61 years, p=0.023) and more often classified as ASA I (45% vs. 14%, p=0.002) compared with patients with non-GAS necrotizing fasciitis. In-hospital mortality rate for necrotizing fasciitis was 32%. Patients with comorbidities were more likely to die of necrotizing fasciitis compared with patients without comorbidities (OR 7.41, 95% CI 1.58 to 34.63). Twelve patients (39%) with GAS necrotizing fasciitis developed pneumonia compared with four patients (13%) with non-GAS necrotizing fasciitis (p=0.017; OR 4.42, 95% CI 1.124 to 15.79). Median time from diagnosis to development of pneumonia in patients with GAS necrotizing fasciitis was 10 days (IQR 9).ConclusionPatients with GAS necrotizing fasciitis have an increased risk to develop late secondary infections during initial treatment for necrotizing fasciitis compared with patients with necrotizing fasciitis without involvement of GAS. This suggests exhaustion of the immune system after severe GAS infection.Level of evidenceIII
СЕПСИС – СИНДРОМ ЧИ ІНФЕКЦІЙНА ХВОРОБА? ЕВОЛЮЦІЯ ДИЛЕМИ
Authors: Kopcha V. S. 
Published: February 2019

Мета роботи – звернути увагу лікарів на невідповідності «нових» уявлень про сепсис, нав’язаних лікарському співтовариству і безумовно прийнятих не лише анестезіологами-реаніматологами та хірургами, але й багатьма інфекціоністами.
Визначаючи наріжним каменем при сепсисі «системність» запальної відповіді у вигляді цитокінових порушень, автори відповідної концепції змушені визнавати етіологічними факторами практично усіх без винятку збудників інфекційних захворювань (від вірусів до найпростіших), які закономірно перебігають з підвищенням активності цитокінів у сироватці крові. Не применшуючи значення цих важливих компонентів запалення, варто звернути увагу на постулат про те, що не тільки віруси та найпростіші ніколи не спричиняють сепсис, але й усі «небактеріємічні збудники» (клостридії ботулізму та правця, бордетелли кашлюка, дифтерійні палички, холерні вібріони та ін.) за визначенням не бувають етіологічними факторами цього захворювання, хоча й неодмінно зумовлюють тяжкі системні реакції, що супроводжуються збільшенням рівня прозапальних цитокінів.
Тому сепсис – це генералізована ациклічна інфекційна хвороба бактерійної та/або грибкової етіології, що розвивається в імунодефіцитному організмі з характерними патоморфологічними і патогістологічними змінами в органах і тканинах.
Наведено детальний аналіз клінічного випадку сепсису у молодої жінки, яка завдяки адекватній активній хірургічній та потужній і тривалій консервативній терапії благополучно одужала.
Self‐Reported Everyday Psychosocial Stressors Are Associated With Greater Impairments in Endothelial Function in Young Adults With Major Depressive Disorder
Authors: Greaney Jody L. , Koffer Rachel E. , Saunders Erika F. H. , Almeida David M. , Alexander Lacy M. 
Published: February 2019

Background

Despite the epidemiological associations between psychological stress, depression, and increased cardiovascular disease risk, no studies have examined the relation between naturally occurring psychosocial stressors and directly measured microvascular function in adults with major depressive disorder (
MDD
). We tested the hypothesis that young adults with
MDD
exposed to everyday psychosocial stressors would exhibit more severe impairments in endothelium‐dependent dilation (
EDD
) compared with: (1) healthy nondepressed adults (
HCs
); and (2) adults with
MDD
without acute psychosocial stress exposure.

Methods and Results

Twenty
HCs
(22±1 years) and 23 otherwise healthy adults with
MDD
(20±0.3 years) participated in the study. Participants completed a psychosocial experiences survey to document their exposure to any of 6 stressors over the preceding 24 hours (eg, arguments, work stressors). Red cell flux (laser Doppler flowmetry) was measured during graded intradermal microdialysis perfusion of acetylcholine (10
−10
to 10
−1
mol/L).
EDD
was expressed as a percentage of maximum vascular conductance (flux/mm Hg). Multiple linear regression was used to determine the associations between stress,
EDD
, and
MDD
. Adults with
MDD
reported a greater number and severity of psychosocial stressors compared with
HCs
(all
P
<0.05).
EDD
was blunted in adults with
MDD
(
HCs
: 91±2 versus
MDD
: 74±3%;
P
<0.001). Exposure to any stressor was related to more severe impairments in
EDD
in patients with
MDD
(no stressor: 81±3 versus 1+ stressors: 69±5%;
P
=0.04) but not in
HCs
(
P
=0.48).

Conclusions

These data indicate that exposure to everyday psychosocial stressors is associated with greater impairments in endothelial function in patients with
MDD
, suggesting a potential mechanistic link between daily stress and depression in increased cardiovascular risk.
Comprehensive Review of the Genetics of Albinism
Authors: Jauregui Ramon , Huryn Laryssa A. , Brooks Brian P. 
Published: February 2019

Introduction It is important to understand albinism, since it is a disorder associated with visual impairment, predisposition to malignant melanomas, and social stigma. The main objective of this article is to review the genetics and biologic mechanisms of the non-syndromic albinism subtypes and to describe associated clinical manifestations. We also discuss research on its treatments. Methods A review of the published literature on albinism subtypes was performed, spanning basic laboratory research, published case reports, and experiences of people with albinism. Results Clear progress has been made in comprehending the causes of albinism; research has shed light on the complexity of the disorder and has led to the molecular classification of subtypes. Discussion Despite the increase in knowledge with regards to albinism, gaps still exist. It is important to continue the pursuit of unraveling the mechanism of the disorder and to monitor the frequency of the subtypes worldwide in order to aid in the development of treatments. Furthermore, disseminating knowledge of albinism is crucial for future progress. Implications for practitioners Albinism is a disorder characterized by hypopigmentation of the hair, skin, and eyes, with accompanying ocular abnormalities that remain relatively stable throughout life. The disorder is defined by a spectrum of pigmentation where albinism is more evident among individuals of dark complexion than their lighter-pigmented peers. Patients with albinism require protection against sun exposure and special resources to address visual impairments. When albinism patients are diagnosed and properly accommodated, they generally report a positive quality of life.
Two layers of graduated compression stockings can reduce healthy saphenous vein diameters in the standing position
Authors: Yang Anes , Yasaman Bahar , Parsi Yana , Kang Mina , Connor David , Parsi Kurosh 
Published: February 2019

Background Previous studies reported graduated compression stockings can reduce the calibre of deep veins in the standing subjects. However, the literature has been inconclusive on the effect of graduated compression stockings on superficial veins in standing subjects. Objectives To assess the effect of two layers of graduated compression stockings on the diameter of saphenous veins in healthy subjects. Materials and methods In 17 legs of nine healthy subjects, we studied the effect of single layering and double layering of graduated compression stockings (23–32 mmHg, Thigh High Belted Sigvaris, Switzerland) on the diameter of the great saphenous vein mid-thigh, great saphenous vein mid-calf and small saphenous vein mid-calf. The measurements were taken using duplex ultrasound (Toshiba Aplio XG 500, 18-7 MHz transducer), through the fabric using a generous amount of ultrasound gel, on subjects both supine or prone and standing. Results Two layers of Class II graduated compression stockings reduced the calibre of great saphenous vein mid-thigh from (4.1 mm to 3.3 mm, p < 0.05), great saphenous vein mid-calf (2.8 mm to 2.2 mm, p < 0.01) and small saphenous vein (2.7 mm to 1.9 mm, p < 0.01) in the standing position. In the supine or prone position, great saphenous vein showed narrowing but there was no measurable change in the calibre of the small saphenous vein. The degree of narrowing was measurable, but insufficient to approximate the vein walls. Conclusion Superimposition of two Class II graduated compression stockings reduced the calibre of the saphenous veins in the standing position but did not approximate the vein walls. In the supine or prone position, the addition of a second layer of graduated compression stockings did not result in further narrowing of the saphenous veins.
The effects of five days of intensive preoperative inspiratory muscle training on postoperative complications and outcome in patients having cardiac surgery: a randomized controlled trial
Authors: Chen Xiaoyu , Hou Lin , Zhang Yuanyuan , Liu Xiangjing , Shao Bohan , Yuan Bo , Li Jing , Li Min , Cheng Hong , Teng Lei , Guo Mingdi , Wang Zhengqing , Chen Tienan , Liu Jianjun , Liu Yaping , Liu Zhigang , Liu Xiaocheng , Guo Qi 
Published: February 2019

Objective: To determine the prophylactic efficacy of short-term intensive preoperative inspiratory muscle training on the incidence of postoperative pulmonary complications in patients scheduled for cardiac surgery. Design: Single-blind, randomized controlled pilot study. Setting: TEDA International Cardiovascular Hospital, China. Subjects: In total, 197 subjects aged ⩾50 years scheduled for cardiac surgery were selected. Intervention: The intervention group ( n = 98) received five days of preoperative inspiratory muscle training on top of the usual care received by the patients in the control group ( n = 99). Main measures: The primary outcome variable was the occurrence of postoperative pulmonary complications. The secondary outcome variables were inspiratory muscle strength, lung function and length of hospitalization. Results: After cardiac surgery, a total of 10 (10.2%) of the 98 patients in the intervention group and 27 (27.3%) of 99 patients in the control group had postoperative pulmonary complications (risk ratio, 0.23; 95% confidence interval (CI), 0.09–0.58, P = 0.002). The study revealed that, compared with the control group, the intervention group had a significant increase in inspiratory muscle strength (by 10.48 cm H2O, P < 0.001), forced expiratory volume in the first second of expiration (FEV1) %predicted (by 3.75%, P = 0.030), forced vital capacity (FVC) %predicted (by 4.15%, P = 0.008) and maximal voluntary ventilation (MVV) %predicted (by 6.44%, P = 0.034). Length of hospital stay was 7.51 (2.83) days in the intervention group and 9.38 (3.10) days in the control group ( P = 0.039). Conclusion: A five-day intensive pattern of preoperative inspiratory muscle training reduced the incidence of postoperative pulmonary complications and duration of postoperative hospitalization in patients undergoing cardiac surgery.
N-nitrosodimethylamine (NDMA) induced apoptosis dependent on Fas/FasL complex in human leukocytes
Authors: Iwaniuk A , Grubczak K , Ratajczak-Wrona W , Garley M , Nowak K , Jabłońska E 
Published: February 2019

Objective: To investigate the mechanism of apoptosis dependent on the Fas/FasL (Fas ligand) complex in the presence of N-nitrosodimethylamine (NDMA) in human leukocytes. Methods: Polymorphonuclear neutrophils (PMNs) and peripheral blood mononuclear cells (PBMCs) were isolated form whole blood by density centrifugation. The concentration of NDMA was assessed by cellular toxicity assay. Apoptotic cells were assessed with flow cytometry and the expression of pro- and antiapoptotic proteins was investigated by Western blotting in PMNs and PBMCs treated with NDMA and/or FasL. Results: PMNs showed a higher ratio of apoptotic cells than PBMCs after exposure to NDMA and/or FasL. Enhanced apoptosis was related to the increased expression of proapoptotic proteins in neutrophils following exposure to either NDMA or FasL. In PBMCs, the relation was observed after exposure to FasL only. PMNs and PBMCs incubated with NDMA and FasL simultaneously demonstrated the highest increase in protein expression. Conclusions: NDMA shows a stronger proapoptotic effect with PMNs than with PBMCs. The Fas/FasL complex, along with other proapoptotic proteins of the receptor (Fas, FADD) and mitochondrial pathway (Noxa, Puma, Bim), plays a key role in the induction of neutrophil apoptosis. Synergic effects of NDMA and FasL which lead to higher induction of apoptosis in PMNs than in PBMCs indicates a multistage and varied regulation of apoptosis in different populations of leukocytes.