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31.
Clinical Spectrum of Capillary Malformation–Arteriovenous Malformation Syndrome Presenting to a Pediatric Dermatology Practice: A Retrospective Study
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Nicole A. Weitz M.D. Christine T. Lauren M.D. Gerald G. Behr M.D. June K. Wu M.D. Jessica J. Kandel M.D. Philip M. Meyers M.D. Sally Sultan M.D. Kwame Anyane‐Yeboa M.D. Kimberly D. Morel M.D. Maria C. Garzon M.D. 《Pediatric dermatology》2015,32(1):76-84
Capillary malformation–arteriovenous malformation syndrome (CM‐AVM) is an autosomal dominant disorder caused by RASA1 mutations. The prevalence and phenotypic spectrum are unknown. Evaluation of patients with multiple CMs is challenging because associated AVMs can be life threatening. The objective of this study was to describe the clinical characteristics of children presenting with features of CM‐AVM to an academic pediatric dermatology practice. After institutional review board approval was received, a retrospective chart review was performed of patients presenting between 2009 and 2012 with features of CM‐AVM. We report nine cases. Presenting symptoms ranged from extensive vascular stains and cardiac failure to CMs noted incidentally during routine skin examination. All demonstrated multiple CMs, two had Parkes Weber syndrome, and two had multiple infantile hemangiomas. Seven patients had family histories of multiple CMs; three had family histories of large, atypical CMs. Six had personal or family histories of AVMs. Genetic evaluation was recommended for all and was pursued by six families; four RASA1 mutations were identified, including one de novo. Consultations with neurology, cardiology, and orthopedics were recommended. Most patients (89%) have not required treatment to date. CM‐AVM is an underrecognized condition with a wide clinical spectrum that often presents in childhood. Further evaluation may be indicated in patients with multiple CMs. This study is limited by its small and retrospective nature. 相似文献
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ABSTRACTBoth international tourism and migration of people with low English proficiency (LEP) to Australia are increasing. Thus, health-care practitioners (HPs) increasingly use interpreters to communicate with patients with LEP. Although qualified interpreters are the most suggested and policy-endorsed mechanism for communicating with patients with LEP, family members (FMIs) are also used as interpreters. This study investigated (a) when do health professionals consider it appropriate to use FMIs and (b) what characteristics of family members health professionals believe make them suitable to act as FMIs. As part of a larger project examining the decision-making processes of HPs regarding interpreter use, 69 HPs from neonatal and pediatric departments in one hospital in Queensland Australia were interviewed. Results indicated HPs thought the appropriateness of using FMIs depended on the type of information, such that it was either completely acceptable (e.g., explaining some basic or non-medical information) or completely unacceptable (e.g., confidential information or consent). However, in an emergency, when no other options were available, FMIs were used. The characteristics of suitable FMIs included age, level of English proficiency and medical understanding, and the relationship between patient and FMI. Results were to some extent consistent with Queensland government policies but there were notable differences, including using children and regarding FMIs as first preference. Improving HP’s knowledge of policies may increase their confidence in their practice and appropriate use of FMIs, thereby improving their care delivery to patients and families with LEP. 相似文献
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Erin L. Sutfin Jennifer Cornacchione Ross Allison J. Lazard Elizabeth Orlan Cynthia K. Suerken Kimberly D. Wiseman 《Health communication》2019,34(3):343-351
ABSTRACTAdolescents and young adults smoke waterpipe tobacco (WT) and cigarillos, at least in part, based on erroneous beliefs that these products are safer than cigarettes. To address this challenge, we used a systematic, three-phase process to develop a health communication campaign to discourage WT and cigarillo smoking among at-risk (tobacco users and susceptible non-users) 16- to 25-year-olds. In Phase 1, we used a national phone survey (N = 896) to determine salient message beliefs. Participants reported constituents (i.e., harmful chemicals) emitted by the products were worrisome. In Phase 2, we developed and evaluated four message executions, with varying imagery, tone, and unappealing products with the same constituents, using focus groups (N = 38). Participants rated one execution highly, resulting in our development of a campaign where each message: (1) identified a tobacco product and constituent in the smoke; (2) included an image of an unappealing product containing the constituent (e.g., pesticides, gasoline) to grab attention; and (3) used a humorous sarcastic tone. In Phase 3, we tested the campaign messages (17 intervention and six control) with a nationally representative online survey (N = 1,636). Participants rated intervention and control messages highly with few differences between them. Exposure to messages resulted in significant increases in all risk beliefs from pre to post (p < 0.05). For WT, intervention messages increased beliefs about addiction more than control messages (p < 0.05). This systematic, iterative approach resulted in messages that show promise for discouraging WT and cigarillo use. 相似文献
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Kimberly M. Avallone 《The Journal of asthma》2015,52(5):498-504
Objective: Despite its negative effects, smoking is more common among individuals with asthma compared to those without. Anxiety sensitivity (fear of arousal-related sensations) is associated with both smoking and asthma; however, no research, to date, has examined the interplay between these three factors. Thus, the purpose of the current study was to evaluate the mediating role of anxiety sensitivity in the association between asthma diagnosis and smoking status. Methods: The current study was a secondary analysis of data from three existing datasets of non-smokers and smokers with and without asthma (n?=?433; 56.3% female, Mage?=?34.01 years, SD?=?13.9). Participants provided information on their asthma diagnosis status and smoking status and completed self-report measures. Results: As hypothesized, after controlling for gender, race and age, there was a significant indirect effect of asthma diagnosis on smoking status through anxiety sensitivity (95% CI?=?0.07–0.48). Conclusions: These results indicate that the association between asthma diagnosis and smoking status appears to be driven, in part, by anxiety sensitivity and suggest that anxiety sensitivity may serve as an important target for prevention and intervention efforts for smokers with asthma. 相似文献
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Fiene Marie Kuijper Uma V. Mahajan Seul Ku Daniel A.N. Barbosa Sheila M. Alessi Sherman C. Stein Kyle M. Kampman Brandon S. Bentzley Casey H. Halpern 《Neuromodulation》2022,25(2):253-262
ObjectivesCocaine is the second most frequently used illicit drug worldwide (after cannabis), and cocaine use disorder (CUD)-related deaths increased globally by 80% from 1990 to 2013. There is yet to be a regulatory-approved treatment. Emerging preclinical evidence indicates that deep brain stimulation (DBS) of the nucleus accumbens may be a therapeutic option. Prior to expanding the costly investigation of DBS for treatment of CUD, it is important to ensure societal cost-effectiveness.AimsWe conducted a threshold and cost-effectiveness analysis to determine the success rate at which DBS would be equivalent to contingency management (CM), recently identified as the most efficacious therapy for treatments of CUDs.Materials and MethodsQuality of life, efficacy, and safety parameters for CM were obtained from previous literature. Costs were calculated from a societal perspective. Our model predicted the utility benefit based on quality-adjusted life-years (QALYs) and incremental-cost-effectiveness ratio resulting from two treatments on a one-, two-, and five-year timeline.ResultsOn a one-year timeline, DBS would need to impart a success rate (ie, cocaine free) of 70% for it to yield the same utility benefit (0.492 QALYs per year) as CM. At no success rate would DBS be more cost-effective (incremental-cost-effectiveness ratio <$50,000) than CM during the first year. Nevertheless, as DBS costs are front loaded, DBS would need to achieve success rates of 74% and 51% for its cost-effectiveness to exceed that of CM over a two- and five-year period, respectively.ConclusionsWe find DBS would not be cost-effective in the short term (one year) but may be cost-effective in longer timelines. Since DBS holds promise to potentially be a cost-effective treatment for CUDs, future randomized controlled trials should be performed to assess its efficacy. 相似文献
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Marie Ohata Susumu Fujiwara Ai Yoshioka Hiroshi Nagai Shintaro Sugita Nozomi Yamano Yusuke Inoue Chikako Nishigori 《Pediatric dermatology》2019,36(6):997-998
Fibroblastic connective tissue nevus (FCTN) is a benign cutaneous mesenchymal lesion characterized by proliferation of CD34‐positive fibroblastic/myofibroblastic spindle‐shaped cells. We report a case of agminated FCTN on the right lower abdomen of a 1‐year‐old boy. 相似文献
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Marie Warrer Petersen Tine Sylvest Meyhoff Marie Helleberg Maj-Brit Nørregaard Kjær Anders Granholm Carl Johan Steensen Hjortsø Thomas Steen Jensen Morten Hylander Møller Peter Buhl Hjortrup Mik Wetterslev Gitte Kingo Vesterlund Lene Russell Vibeke Lind Jørgensen Klaus Tjelle Thomas Benfield Charlotte Suppli Ulrik Anne Sofie Andreasen Thomas Mohr Morten H. Bestle Lone Musaeus Poulsen Mette Friberg Hitz Thomas Hildebrandt Lene Surland Knudsen Anders Møller Christoffer Grant Sølling Anne Craveiro Brøchner Bodil Steen Rasmussen Henrik Nielsen Steffen Christensen Thomas Strøm Maria Cronhjort Rebecka Rubenson Wahlin Stephan Jakob Luca Cioccari Balasubramanian Venkatesh Naomi Hammond Vivekanand Jha Sheila Nainan Myatra Christian Gluud Theis Lange Anders Perner 《Acta anaesthesiologica Scandinavica》2020,64(9):1365-1375