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Three Novel Immune-deficient Mouse Models of Muscular Dystrophy 三种新的肌肉萎缩症免疫缺陷小鼠模型
PLoS currents Pub Date : 2017-09-01 DOI: 10.1371/CURRENTS.MD.25E9CD5F4740329F2650972A75230188
Christophe Pichavant, Christopher R. R. Bjornson, T. Gallagher, Tawny L. Neal, H. D. Bois, Mital S. Bhakta, M. Calos
{"title":"Three Novel Immune-deficient Mouse Models of Muscular Dystrophy","authors":"Christophe Pichavant, Christopher R. R. Bjornson, T. Gallagher, Tawny L. Neal, H. D. Bois, Mital S. Bhakta, M. Calos","doi":"10.1371/CURRENTS.MD.25E9CD5F4740329F2650972A75230188","DOIUrl":"https://doi.org/10.1371/CURRENTS.MD.25E9CD5F4740329F2650972A75230188","url":null,"abstract":"","PeriodicalId":74464,"journal":{"name":"PLoS currents","volume":" ","pages":""},"PeriodicalIF":0.0,"publicationDate":"2017-09-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"49077425","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
引用次数: 2
Multi-provincial Salmonellosis Outbreak Related to Newly Hatched Chicks and Poults: A Genomics Perspective. 与新孵小鸡和家禽有关的多省沙门氏菌病暴发:基因组学视角。
PLoS currents Pub Date : 2017-08-09 DOI: 10.1371/currents.outbreaks.309af53b9edcc785163539c30c3953f6
Matthew A Croxen, Kimberley A Macdonald, Matthew Walker, Nancy deWith, Erin Zabek, Christy Peterson, Aleisha Reimer, Linda Chui, Lorelee Tschetter, Linda Hoang, Robin K King
{"title":"Multi-provincial Salmonellosis Outbreak Related to Newly Hatched Chicks and Poults: A Genomics Perspective.","authors":"Matthew A Croxen,&nbsp;Kimberley A Macdonald,&nbsp;Matthew Walker,&nbsp;Nancy deWith,&nbsp;Erin Zabek,&nbsp;Christy Peterson,&nbsp;Aleisha Reimer,&nbsp;Linda Chui,&nbsp;Lorelee Tschetter,&nbsp;Linda Hoang,&nbsp;Robin K King","doi":"10.1371/currents.outbreaks.309af53b9edcc785163539c30c3953f6","DOIUrl":"https://doi.org/10.1371/currents.outbreaks.309af53b9edcc785163539c30c3953f6","url":null,"abstract":"<p><strong>Background: </strong>A multi-provincial outbreak of Salmonella enterica serovar Enteritidis was linked to newly hatched chicks and poults from a single hatchery during the spring of 2015. In total, there were 61 human cases that were epidemiologically confirmed to be linked to the chicks and poults and the outbreak was deemed to have ended in the summer of 2015.</p><p><strong>Methods: </strong>PulseNet Canada, in coordination with the affected provinces, used genome sequencing of human and agricultural Salmonella Enteritidis isolates to aid in the epidemiological investigation, while also using traditional typing methods such as phagetyping and pulsed-field gel electrophoresis (PFGE).</p><p><strong>Results: </strong>All human outbreak cases, except one, were Phage Type (PT) 13a. Single nucleotide variant analysis (SNV) was able to provide a level of resolution commensurate with the results of the epidemiological investigation. SNV analysis was also able to separate PT13a outbreak-related isolates from isolates not linked to chicks or poults, while clustering some non-PT13a agricultural strains with the outbreak cluster.</p><p><strong>Conclusions: </strong>Based on conventional typing methods (phagetyping or PFGE), clinical and agricultural PT13a SE isolates would have been considered as part of a related cluster. In contrast, phagetyping would have led to the exclusion of several non- PT13a strains that clustered with the outbreak isolates using the genome sequence data. This study demonstrates the improved resolution of genome sequence analysis for coordinated surveillance and source attribution of both human and agricultural SE isolates.</p>","PeriodicalId":74464,"journal":{"name":"PLoS currents","volume":" ","pages":""},"PeriodicalIF":0.0,"publicationDate":"2017-08-09","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC5614700/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"35516418","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
引用次数: 2
Estimation of Time Period for Effective Human Inhalational Anthrax Treatment Including Antitoxin Therapy. 估计人类吸入性炭疽有效治疗包括抗毒素治疗的时间。
PLoS currents Pub Date : 2017-07-28 DOI: 10.1371/currents.outbreaks.7896c43f69838f17ce1c2c372e79d55d
Lewis Rubinson, Alfred Corey, Dan Hanfling
{"title":"Estimation of Time Period for Effective Human Inhalational Anthrax Treatment Including Antitoxin Therapy.","authors":"Lewis Rubinson,&nbsp;Alfred Corey,&nbsp;Dan Hanfling","doi":"10.1371/currents.outbreaks.7896c43f69838f17ce1c2c372e79d55d","DOIUrl":"https://doi.org/10.1371/currents.outbreaks.7896c43f69838f17ce1c2c372e79d55d","url":null,"abstract":"<p><strong>Introduction: </strong>Infrequent natural human inhalational anthrax cases coupled with high bioterrorism risk have brought about use of animal models to serve as the basis for approval of novel treatments. For inhalational anthrax, protective antigen (PA) drives much of the mortality, and raxibacumab, an anti-PA monoclonal antibody, has been approved for therapeutic use using the Animal Rule. Given the paucity of human inhalational anthrax clinical data including PA kinetics, the post-exposure period for effective treatment of human disease remains unknown. The objective of this investigation was to extrapolate animal PA kinetics to a conceptual human model to estimate the post-exposure period for effective treatment of human inhalational anthrax.</p><p><strong>Methods: </strong>Human PA kinetic parameters were extrapolated from reported rabbit and monkey data. PA profiles were simulated with and without antibiotic induced PA clearance to represent antibiotic-sensitive and -resistant infections, respectively. Antitoxin levels equimolar to or greater than concurrent PA levels were considered protective.</p><p><strong>Results: </strong>For antibiotic sensitive infections, treatment with antibiotics alone ≤4 days after spore exposure prevents toxemia. Administration of raxibacumab together with antibiotics protects ≥ 80% of subjects for 3 additional days (7 days post exposure). In the setting of antibiotic resistance, raxibacumab would be protective for at least 6 days post exposure.</p><p><strong>Conclusions: </strong>Although the animal model of disease does not reflect the potential impact of supportive care (e.g. fluid resuscitation received by critically ill patients) on PA kinetics and raxibacumab PK, the simulations suggest that administration of antitoxin in combination with antibiotics should provide a longer postexposure window for effective treatment than for antibiotics alone. In addition, raxibacumab administration soon after exposure to an antibiotic resistant strain should provide effective treatment.</p>","PeriodicalId":74464,"journal":{"name":"PLoS currents","volume":" ","pages":""},"PeriodicalIF":0.0,"publicationDate":"2017-07-28","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC5555766/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"35459329","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
引用次数: 5
The Enduring Health Challenges of Afghan Immigrants and Refugees in Iran: A Systematic Review. 伊朗境内阿富汗移民和难民的持久健康挑战:系统回顾。
PLoS currents Pub Date : 2017-07-21 DOI: 10.1371/currents.dis.449b4c549951e359363a90a7f4cf8fc4
Nasim Sadat Hosseini Divkolaye, Frederick M Burkle
{"title":"The Enduring Health Challenges of Afghan Immigrants and Refugees in Iran: A Systematic Review.","authors":"Nasim Sadat Hosseini Divkolaye, Frederick M Burkle","doi":"10.1371/currents.dis.449b4c549951e359363a90a7f4cf8fc4","DOIUrl":"10.1371/currents.dis.449b4c549951e359363a90a7f4cf8fc4","url":null,"abstract":"<p><strong>Introduction: </strong>Iran is the third country in the world with the highest number of registered refugees with the majority coming from Afghanistan. They suffer major health and social risks yet their health status has never been comprehensively determined.</p><p><strong>Methods: </strong>This systematic review of the literature highlights major disparities among documented immigrants in health access, communicable and non-communicable diseases and the increasingly desperate plight of undocumented immigrants.</p><p><strong>Results: </strong>Comparing with Iranian population, the findings suggest the higher prevalence of most diseases among Afghan immigrants and refugees. This highlights the importance of increasing the migrants' access to health services from both public health as well as human rights perspectives.</p><p><strong>Discussion: </strong>Although the Iranian government has taken new initiatives to overcome this challenge, certain issues have still remained unaddressed. Potential solutions to improve this process are discussed.</p>","PeriodicalId":74464,"journal":{"name":"PLoS currents","volume":" ","pages":""},"PeriodicalIF":0.0,"publicationDate":"2017-07-21","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC5554007/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"35459328","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
引用次数: 0
Interventions for Prevention of Intimate Partner Violence Against Women in Humanitarian Settings: A Protocol for a Systematic Review. 在人道主义环境中预防亲密伴侣暴力侵害妇女的干预措施:系统审查议定书。
PLoS currents Pub Date : 2017-07-12 DOI: 10.1371/currents.dis.f41d45fbdca13babe4ae5be0f9732e75
Marjan Delkhosh, Ali Ardalan, Abbas Rahimiforoushani, Abbas Keshtkar, Leila Amiri Farahani, Effat Merghati Khoei
{"title":"Interventions for Prevention of Intimate Partner Violence Against Women in Humanitarian Settings: A Protocol for a Systematic Review.","authors":"Marjan Delkhosh, Ali Ardalan, Abbas Rahimiforoushani, Abbas Keshtkar, Leila Amiri Farahani, Effat Merghati Khoei","doi":"10.1371/currents.dis.f41d45fbdca13babe4ae5be0f9732e75","DOIUrl":"10.1371/currents.dis.f41d45fbdca13babe4ae5be0f9732e75","url":null,"abstract":"<p><strong>Introduction: </strong>Humanitarian emergencies and the number of people who are adversely affected are increasing. In such emergencies, the vulnerability of women and girls to gender-based violence increases signifi-cantly and they often experience high levels of intimate partner violence (IPV). There are a limited number of interventions to reduce gender-based violence (GBV) and IPV in the contexts of humanitarian emergencies, and there is uncertainty about the effectiveness of these preventive interventions. This is the protocol for a systematic review that will synthesize the evidence on interventions for primary or secondary prevention of IPV in humanitarian settings, and assess the effect of existing types of IPV-related interventions in these settings.</p><p><strong>Methods and design: </strong>The PRISMA-P 2015 statement has been used to prepare this report. Studies published from January 2000 to January 2017 will be reviewed with no language limits. Any experimental, quasi-experimental, or controlled trials will be included. A combination of four key concepts, including \"IPV\" AND \"population\" AND \"humanitarian setting\" AND \"intervention\" will be used in the search and a variety of information sources will be used: (1) bibliographic databases; (2) special databases and grey literature; (3) and the reference lists of eligible studies. Two reviewers will independently screen articles, extract relevant data and assess study quality. Discrepancies will be resolved through consensus. Risk of bias will be assessed using the Cochrane Risk of Bias tool and the quality of evidence will be assessed using the CONSORT checklist. A narrative synthesis will be provided. If a sufficient number of studies are found, their results will be pooled using a random-effects meta-analysis. For dichotomous outcomes, summaries of intervention effects for each study will be provided by calculating risk ratios with 95% confidence interval. Standardized mean differences will be used for continuous outcomes.</p><p><strong>Discussion: </strong>The review will be useful for IPV management policy and related planning. It will help researchers, policymakers and guideline developers with an interest in reducing violence against women among refugees, internally displaced persons (IDPs), and conflict-affected population.</p>","PeriodicalId":74464,"journal":{"name":"PLoS currents","volume":" ","pages":""},"PeriodicalIF":0.0,"publicationDate":"2017-07-12","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC5553713/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"35459327","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
引用次数: 13
Correction: Effects of the Pimelic Diphenylamide Histone Deacetylase Inhibitor HDACi 4b on the R6/2 and N171-82Q Mouse Models of Huntington's Disease. 修正:苯乙烯二苯酰胺组蛋白去乙酰化酶抑制剂HDACi 4b对R6/2和N171-82Q小鼠亨廷顿病模型的影响。
PLoS currents Pub Date : 2017-07-11 DOI: 10.1371/currents.hd.976177e0cbf724437ea11745c9231a57
{"title":"Correction: Effects of the Pimelic Diphenylamide Histone Deacetylase Inhibitor HDACi 4b on the R6/2 and N171-82Q Mouse Models of Huntington's Disease.","authors":"","doi":"10.1371/currents.hd.976177e0cbf724437ea11745c9231a57","DOIUrl":"https://doi.org/10.1371/currents.hd.976177e0cbf724437ea11745c9231a57","url":null,"abstract":"<p><p>[This corrects the article DOI: 10.1371/currents.hd.ec3547da1c2a520ba959ee7bf8bdd202.].</p>","PeriodicalId":74464,"journal":{"name":"PLoS currents","volume":" ","pages":""},"PeriodicalIF":0.0,"publicationDate":"2017-07-11","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC5510914/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"35459325","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
引用次数: 0
Correction: Differential Synaptic and Extrasynaptic Glutamate-Receptor Alterations in Striatal Medium-Sized Spiny Neurons of Aged YAC128 Huntington's Disease Mice. 修正:老年YAC128亨廷顿病小鼠纹状体中等棘神经元突触和突触外谷氨酸受体的差异改变。
PLoS currents Pub Date : 2017-07-11 DOI: 10.1371/currents.hd.2f36af340a70a0d248b48d4cc9c3e58e
{"title":"Correction: Differential Synaptic and Extrasynaptic Glutamate-Receptor Alterations in Striatal Medium-Sized Spiny Neurons of Aged YAC128 Huntington's Disease Mice.","authors":"","doi":"10.1371/currents.hd.2f36af340a70a0d248b48d4cc9c3e58e","DOIUrl":"https://doi.org/10.1371/currents.hd.2f36af340a70a0d248b48d4cc9c3e58e","url":null,"abstract":"<p><p>[This corrects the article DOI: 10.1371/currents.hd.34957c4f8bd7cb1f5ec47381dfc811c3.].</p>","PeriodicalId":74464,"journal":{"name":"PLoS currents","volume":" ","pages":""},"PeriodicalIF":0.0,"publicationDate":"2017-07-11","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC5510822/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"35459324","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
引用次数: 1
Genomic Characterization Helps Dissecting an Outbreak of Listeriosis in Northern Italy. 基因组特征有助于解剖意大利北部李斯特菌病的爆发。
PLoS currents Pub Date : 2017-07-06 DOI: 10.1371/currents.outbreaks.633fd8994e9f06f31b3494567c7e504c
Francesco Comandatore, Marta Corbella, Giuseppina Andreoli, Erika Scaltriti, Massimo Aguzzi, Stefano Gaiarsa, Bianca Mariani, Marina Morganti, Claudio Bandi, Massimo Fabbi, Piero Marone, Stefano Pongolini, Davide Sassera
{"title":"Genomic Characterization Helps Dissecting an Outbreak of Listeriosis in Northern Italy.","authors":"Francesco Comandatore,&nbsp;Marta Corbella,&nbsp;Giuseppina Andreoli,&nbsp;Erika Scaltriti,&nbsp;Massimo Aguzzi,&nbsp;Stefano Gaiarsa,&nbsp;Bianca Mariani,&nbsp;Marina Morganti,&nbsp;Claudio Bandi,&nbsp;Massimo Fabbi,&nbsp;Piero Marone,&nbsp;Stefano Pongolini,&nbsp;Davide Sassera","doi":"10.1371/currents.outbreaks.633fd8994e9f06f31b3494567c7e504c","DOIUrl":"https://doi.org/10.1371/currents.outbreaks.633fd8994e9f06f31b3494567c7e504c","url":null,"abstract":"<p><strong>Introduction: </strong>Listeria monocytogenes (Lm) is a bacterium widely distributed in nature and able to contaminate food processing environments, including those of dairy products. Lm is a primary public health issue, due to the very low infectious dose and the ability to produce severe outcomes, in particular in elderly, newborns, pregnant women and immunocompromised patients.</p><p><strong>Methods: </strong>In the period between April and July 2015, an increased number of cases of listeriosis was observed in the area of Pavia, Northern Italy. An epidemiological investigation identified a cheesemaking small organic farm as the possible origin of the outbreak. In this work we present the results of the retrospective epidemiological study that we performed using molecular biology and genomic epidemiology methods. The strains sampled from patients and those from the target farm's cheese were analyzed using PFGE and whole genome sequencing (WGS) based methods. The performed WGS based analyses included: a) in-silico MLST typing; b) SNPs calling and genetic distance evaluation; c) determination of the resistance and virulence genes profiles; d) SNPs based phylogenetic reconstruction.</p><p><strong>Results: </strong>Three of the patient strains and all the cheese strains resulted to belong to the same phylogenetic cluster, in Sequence Type 29. A further accurate SNPs analysis revealed that two of the three patient strains and all the cheese strains were highly similar (0.8 SNPs of average distance) and exhibited a higer distance from the third patient isolate (9.4 SNPs of average distance).</p><p><strong>Discussion: </strong>Despite the global agreement among the results of the PFGE and WGS epidemiological studies, the latter approach agree with epidemiological data in indicating that one the patient strains could have originated from a different source. This result highlights that WGS methods can allow to better.</p>","PeriodicalId":74464,"journal":{"name":"PLoS currents","volume":" ","pages":""},"PeriodicalIF":0.0,"publicationDate":"2017-07-06","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC5510990/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"35459326","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
引用次数: 5
Correction: A Comparative Analysis of Disaster Risk, Vulnerability and Resilience Composite Indicators. 修正:灾害风险、脆弱性和复原力综合指标的比较分析。
PLoS currents Pub Date : 2017-06-29 DOI: 10.1371/currents.dis.19f9c194f3e3724d9ffa285b157c6ee3
{"title":"Correction: A Comparative Analysis of Disaster Risk, Vulnerability and Resilience Composite Indicators.","authors":"","doi":"10.1371/currents.dis.19f9c194f3e3724d9ffa285b157c6ee3","DOIUrl":"https://doi.org/10.1371/currents.dis.19f9c194f3e3724d9ffa285b157c6ee3","url":null,"abstract":"<p><p>[This corrects the article DOI: 10.1371/currents.dis.453df025e34b682e9737f95070f9b970.].</p>","PeriodicalId":74464,"journal":{"name":"PLoS currents","volume":" ","pages":""},"PeriodicalIF":0.0,"publicationDate":"2017-06-29","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC5510779/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"35459323","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
引用次数: 5
The PJ Nicholoff Steroid Protocol for Duchenne and Becker Muscular Dystrophy and Adrenal Suppression. PJ nicoloff类固醇治疗Duchenne和Becker肌营养不良和肾上腺抑制的方案。
PLoS currents Pub Date : 2017-06-27 DOI: 10.1371/currents.md.d18deef7dac96ed135e0dc8739917b6e
Kathi Kinnett, Garey Noritz
{"title":"The PJ Nicholoff Steroid Protocol for Duchenne and Becker Muscular Dystrophy and Adrenal Suppression.","authors":"Kathi Kinnett,&nbsp;Garey Noritz","doi":"10.1371/currents.md.d18deef7dac96ed135e0dc8739917b6e","DOIUrl":"https://doi.org/10.1371/currents.md.d18deef7dac96ed135e0dc8739917b6e","url":null,"abstract":"<p><p>Duchenne muscular dystrophy (DMD or Duchenne) is a progressive, life-limiting muscle-wasting disease that requires comprehensive, multidisciplinary care. This care, at minimum, should include neuromuscular, respiratory, cardiac, orthopedic, endocrine and rehabilitative interventions that address both the primary and secondary manifestations of the disease. The care needs of patients evolve over the cdourse of the disease and as they transition from childhood into young adulthood. In the past two decades, life expectancy has increased significantly by the use of corticosteroids and enhanced clinical management. Nevertheless, each year, patients with Duchenne muscular dystrophy are admitted to emergency departments and intensive care units where medical expertise thrives, but where expertise in rare diseases, such as Duchenne, may not. Emergency care for patients with Duchenne can be as complex as the disease process itself. While any illness or injury may occur in a person with Duchenne, some acute scenarios are much more common in the context of the disease. Making decisions about the clinical care of a person with Duchenne who presents with an acute illness can be quite difficult - in part, because of the extensive use of corticosteroids, which can lead to adrenal suppression. The life of a person with Duchenne needing emergency care may therefore depend upon the ability of the clinician on duty in the emergency department to recognize and mitigate adrenal suppression resulting from corticosteroid dependence. With this in mind, and drawing from expertise and experience with other steroid-dependent diseases, the 'PJ Nicholoff Steroid Protocol' was developed. The purpose of this protocol is to provide clinicians information regarding the safe management of corticosteroid during emergency situations in patients who may have accompanying adrenal suppression. The protocol explains how to recognize the signs and symptoms of acute adrenal crisis, how to prevent it with supplemental stress doses of corticosteroids, and how to taper doses after emergency care in order to prevent corticosteroid withdrawal.</p>","PeriodicalId":74464,"journal":{"name":"PLoS currents","volume":" ","pages":""},"PeriodicalIF":0.0,"publicationDate":"2017-06-27","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC5505768/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"35201490","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
引用次数: 19
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