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The 11th Canadian Symposium on Hepatitis C Virus: 'Getting back on track towards hepatitis C elimination'. 第11届加拿大丙型肝炎病毒研讨会:“回到消除丙型肝炎的轨道上”。
Pub Date : 2023-02-01 DOI: 10.3138/canlivj-2022-0034
Ana Maria Passos-Castilho, Sasha Tejna Persaud Udhesister, Guillaume Fontaine, Dahn Jeong, Melisa Dickie, Carrielynn Lund, Rodney Russell, Nadine Kronfli

Hepatitis C virus (HCV) affects approximately 204,000 Canadians. Safe and effective direct-acting antiviral therapies have contributed to decreased rates of chronic HCV infection and increased treatment uptake in Canada, but major challenges for HCV elimination remain. The 11th Canadian Symposium on Hepatitis C Virus took place in Ottawa, Ontario on May 13, 2022 as a hybrid conference themed 'Getting back on track towards hepatitis C elimination.' It brought together research scientists, clinicians, community health workers, patient advocates, community members, and public health officials to discuss priorities for HCV elimination in the wake of the COVID-19 pandemic, which had devastating effects on HCV care in Canada, particularly on priority populations. Plenary sessions showcased topical research from prominent international and national researchers, complemented by select abstract presentations. This event was hosted by the Canadian Network on Hepatitis C (CanHepC), with support from the Public Health Agency of Canada and the Canadian Institutes of Health Research and in partnership with the Canadian Liver Meeting. CanHepC has an established record in HCV research and in advocacy activities to address improved diagnosis and treatment, and immediate and long-term needs of those affected by HCV infection. The Symposium addressed the remaining challenges and barriers to HCV elimination in priority populations and principles for meaningful engagement of Indigenous communities and individuals with living and lived experience in HCV research. It emphasized the need for disaggregated data and simplified pathways for creating and monitoring interventions for equitably achieving elimination targets.

丙型肝炎病毒(HCV)影响了大约204,000名加拿大人。在加拿大,安全有效的直接抗病毒治疗有助于降低慢性丙型肝炎病毒感染率和增加治疗,但消除丙型肝炎病毒的主要挑战仍然存在。第11届加拿大丙型肝炎病毒研讨会于2022年5月13日在安大略省渥太华举行,这是一个主题为“回到消除丙型肝炎的轨道上”的混合会议。会议汇集了研究科学家、临床医生、社区卫生工作者、患者倡导者、社区成员和公共卫生官员,讨论了在2019冠状病毒病大流行之后消除丙型肝炎病毒的优先事项,这对加拿大的丙型肝炎病毒治疗,特别是对重点人群的治疗产生了破坏性影响。全体会议展示了来自著名国际和国内研究人员的专题研究,并辅以精选的抽象报告。本次活动由加拿大丙型肝炎网络(CanHepC)主办,得到加拿大公共卫生署和加拿大卫生研究院的支持,并与加拿大肝脏会议合作。CanHepC在HCV研究和宣传活动方面有良好的记录,以解决改进的诊断和治疗,以及受HCV感染的人的即时和长期需求。研讨会讨论了在重点人群中消除丙型肝炎病毒的剩余挑战和障碍,以及土著社区和具有丙型肝炎病毒研究生活经验的个人有意义参与的原则。它强调需要分类数据和简化途径,以便制定和监测干预措施,公平实现消除目标。
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引用次数: 0
Exploring provider roles, continuity, and mental models in cirrhosis care: A qualitative study. 探讨肝硬化护理中的提供者角色、连续性和心理模型:一项定性研究。
Pub Date : 2023-02-01 DOI: 10.3138/canlivj-2022-0020
Tanya Barber, Lynn Toon, Puneeta Tandon, Lee A Green

BACKGROUND: Advanced cirrhosis results in frequent emergency department visits, hospital admissions and readmissions, and a high risk of premature death. We previously identified and compared differences in the mental models of cirrhosis care held by primary and specialty care physicians and nurse practitioners that may be addressed to improve coordination and transitions in care. The aim of this paper is to further explore how challenges to continuity and coordination of care influence how health care providers adapt in their approaches to and development of mental models of cirrhosis care. METHODS: Cross-sectional formal elicitation of mental models using Cognitive Task Analysis. Purposive and chain-referral sampling took place over 6 months across Alberta for a total of 19 participants, made up of family physicians (n = 8), specialists (n = 9), and cirrhosis nurse practitioners (n = 2). RESULTS: Lack of continuity in cirrhosis care, particularly informational and management continuity, not only hinders health care providers' ability to develop rich mental models of cirrhosis care but may also determine whether they form a patient-centred or task-based mental model, and whether they develop shared mental models with other providers. CONCLUSIONS: The system barriers and gaps that prevent the level of continuity needed to coordinate care for people with cirrhosis lead providers to create and work under mental models that perpetuate those barriers, in a vicious cycle. Understanding how providers approach cirrhosis care, adapt to the challenges facing them, and develop mental models offers insights into how to break that cycle and improve continuity and coordination.

背景:晚期肝硬化导致频繁的急诊科就诊,住院和再入院,以及过早死亡的高风险。我们之前确定并比较了初级和专科护理医生和护士从业人员在肝硬化护理的心理模型上的差异,这些差异可能会改善护理中的协调和过渡。本文的目的是进一步探讨护理的连续性和协调的挑战如何影响卫生保健提供者如何适应他们的方法和肝硬化护理心理模型的发展。方法:采用认知任务分析对心理模型进行横断面形式启发。有目的和连锁转诊抽样在艾伯塔省进行了6个多月,共有19名参与者,包括家庭医生(n = 8),专家(n = 9)和肝硬化执业护士(n = 2)。肝硬化护理缺乏连续性,特别是信息和管理的连续性,不仅阻碍了保健提供者形成丰富的肝硬化护理心理模型的能力,而且还可能决定他们是形成以患者为中心还是以任务为基础的心理模型,以及他们是否与其他提供者形成共享的心理模型。结论:系统障碍和差距阻碍了协调肝硬化患者护理所需的连续性水平,导致提供者创造并在使这些障碍长期存在的心理模式下工作,从而形成恶性循环。了解提供者如何处理肝硬化护理,适应他们面临的挑战,并建立心理模型,可以帮助我们了解如何打破这种循环,提高连续性和协调性。
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引用次数: 0
Albuminuria post-liver transplant is a predictor of kidney disease progression and mortality. 肝移植后蛋白尿是肾脏疾病进展和死亡率的预测指标。
Pub Date : 2023-02-01 DOI: 10.3138/canlivj-2022-0019
Julie Anne Ting, Dilshani Induruwage, Eric M Yoshida, Miten Dhruve, Nadia Y Zalunardo

BACKGROUND: Albuminuria is a marker of chronic kidney disease (CKD) associated with an increased risk of end-stage kidney disease (ESKD) and mortality in the general population, but it is uncertain whether the same association exists in liver transplant (LT) recipients. This study examined the association between albuminuria and kidney failure and mortality in LT recipients.METHODS: Retrospective cohort study of 294 adults who received a LT between January 1, 1989, and December 31, 2011, in British Columbia, Canada. Cox multivariable regression was used to determine the association between ACR and a primary combined outcome of mortality, doubling of serum creatinine, or ESKD; and a secondary outcome of a decrease in estimated glomerular filtration rate (eGFR) ≥30%. RESULTS: At baseline, mean eGFR was 67 (SD 20.9) mL/min/1.73 m2, and 10% had severe albuminuria (ACR >30 mg/mmol). The primary outcome occurred in 20.4% (60) of patients and was associated with ACR >30 mg/mmol (HR 2.77, 95% CI 1.28-6.04; P = 0.01). A decline in eGFR ≥30% occurred in 21.8% (64) of patients, and was associated with ACR >30 mg/mmol (HR 4.77, 95% CI 2.31-9.86; P < 0.0001). CONCLUSIONS: Severe albuminuria (ACR >30 mg/mmol) was associated with an increased risk of loss of kidney function and mortality after LT. Prospective studies are needed to determine if specific interventions directed at reducing albuminuria can improve long-term outcomes in LT recipients.

背景:蛋白尿是慢性肾脏疾病(CKD)的一个标志,与一般人群终末期肾脏疾病(ESKD)和死亡率增加相关,但在肝移植(LT)受者中是否存在同样的关联尚不确定。本研究探讨了肾移植受者蛋白尿、肾功能衰竭和死亡率之间的关系。方法:对1989年1月1日至2011年12月31日期间在加拿大不列颠哥伦比亚省接受过肝移植的294名成年人进行回顾性队列研究。使用Cox多变量回归来确定ACR与死亡率、血清肌酐加倍或ESKD等主要综合结局之间的关系;次要结局是肾小球滤过率(eGFR)降低≥30%。结果:基线时,平均eGFR为67 (SD 20.9) mL/min/1.73 m2, 10%有严重蛋白尿(ACR >30 mg/mmol)。20.4%(60)的患者出现主要结局,并与ACR >30 mg/mmol相关(HR 2.77, 95% CI 1.28-6.04;P = 0.01)。21.8%(64例)的患者eGFR下降≥30%,且与ACR >30 mg/mmol相关(HR 4.77, 95% CI 2.31-9.86;P < 0.0001)。结论:重度蛋白尿(ACR >30 mg/mmol)与肝移植后肾功能丧失和死亡风险增加相关。需要前瞻性研究来确定针对减少蛋白尿的特定干预措施是否可以改善肝移植受者的长期预后。
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引用次数: 1
Prevalence of syphilis coinfection in hepatitis C virus positive prenatal patients from Alberta during a pilot routine screening program. 阿尔伯塔省丙型肝炎病毒阳性产前患者在试点常规筛查方案期间梅毒合并感染的患病率。
Pub Date : 2023-02-01 DOI: 10.3138/canlivj-2022-0017
L Alexa Thompson, Sabrina S Plitt, Jennifer Gratrix, Carmen L Charlton

BACKGROUND: Alberta routinely screens pregnant patients for select communicable diseases. Hepatitis C virus (HCV) was added to the prenatal screening panel as part of a provincial pilot program in February 2020. This retrospective cross-sectional study aimed to characterize the prevalence of syphilis coinfections in prenatal patients infected with HCV following implementation of the pilot program.METHODS: Routine prenatal HCV and syphilis testing data were extracted from the Public Health Laboratory Information System over a 21-month period. HCV positivity was defined as HCV enzyme immunoassay (EIA) reactive with detected HCV ribonucleic acid (RNA) following molecular confirmation, and positive results were examined for syphilis coinfections. All patients reactive on a syphilis EIA and confirmatory Treponema pallidum particle agglutination (TPPA) or follow-up rapid plasma reagin (RPR) test were considered positive for syphilis. Descriptive statistics for coinfected patients were analyzed. RESULTS: Eighty-seven prenatal patients were identified to be positive for HCV. Of those, 19 (21.8%) were reactive on the syphilis EIA and 17 (19.5%) had confirmed infections with the TPPA or RPR tests. For HCV/syphilis coinfected patients, the majority resided in metropolitan regions (64.6%), were from the lowest income quintile neighbourhoods (47.1%) and had previously tested positive for HCV (82.4%) and syphilis (64.6%) at the public health laboratory. CONCLUSIONS: The prevalence of syphilis coinfections in prenatal patients infected with HCV is high in Alberta. HCV/syphilis coinfection prevalence should be further investigated in other jurisdictions and prenatal cohorts to better understand testing and treatment options for prevention of congenital transmission.

背景:艾伯塔省常规筛查孕妇选定的传染病。2020年2月,作为省级试点项目的一部分,丙型肝炎病毒(HCV)被纳入产前筛查小组。本回顾性横断面研究旨在描述试点项目实施后产前感染HCV患者中梅毒合并感染的患病率。方法:从公共卫生实验室信息系统中提取21个月的常规产前丙型肝炎病毒和梅毒检测数据。HCV阳性定义为HCV酶免疫测定(EIA)在分子确认后与检测到的HCV核糖核酸(RNA)反应,并检查梅毒合并感染的阳性结果。所有梅毒环评和梅毒螺旋体颗粒凝集(TPPA)或后续快速血浆反应素(RPR)试验阳性的患者都被认为是梅毒阳性。对合并感染患者进行描述性统计分析。结果:87例产前患者HCV阳性。其中梅毒EIA阳性19例(21.8%),TPPA或RPR阳性17例(19.5%)。对于丙型肝炎病毒/梅毒合并感染的患者,大多数居住在大都市地区(64.6%),来自收入最低的五分之一社区(47.1%),以前在公共卫生实验室检测出丙型肝炎病毒(82.4%)和梅毒(64.6%)阳性。结论:艾伯塔省产前HCV感染患者中梅毒合并感染的发生率较高。应进一步调查其他司法管辖区和产前队列的丙型肝炎/梅毒合并感染患病率,以更好地了解预防先天性传播的检测和治疗方案。
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引用次数: 0
Birth cohort hepatitis C antibody prevalence in real-world screening settings in Ontario. 出生队列丙型肝炎抗体在安大略省现实世界筛查设置患病率。
Pub Date : 2022-08-16 eCollection Date: 2022-08-01 DOI: 10.3138/canlivj-2021-0036
Mia J Biondi, Grishma Hirode, Camelia Capraru, Aaron Vanderhoff, Joel Karkada, Brett Wolfson-Stofko, David Smookler, Steven M Friedman, Kathy Bates, Tony Mazzulli, Joshua V Juan, Hemant Shah, Bettina E Hansen, Jordan J Feld, Harry LA Janssen

Background: Widespread screening and treatment of hepatitis C virus (HCV) is required to decrease late-stage liver disease and liver cancer. Clinical practice guidelines and Canadian Task Force on Preventative Health Care recommendations differ on the value of one-time birth cohort (1945-75) HCV screening in Canada. To assess the utility of this approach, we conducted a real-world analysis of HCV antibody (Ab) prevalence among birth cohort individuals seen in different clinical contexts.

Methods: Cross-sectional study of individuals born between 1945 and 1975 who completed HCV Ab testing at multiple participating centres in Ontario, Canada between January 2016 and December 2020. Differences in prevalence were compared by year of birth, gender, and setting.

Results: Among 16,672 birth cohort individuals tested, HCV Ab prevalence was 3.2%. Prevalence was higher among younger individuals which increased from 0.9% among those born between 1945 and 1956 to 4.6% among those born between 1966 and 1975. Prevalence was higher among males (4.4%) compared with females (2.0%) and differed by test site. In primary care, the prevalence was 0.5%, whereas the prevalence was highest among those tested at drug treatment centres (28.7%) and through community outreach (14.0%).

Conclusions: HCV Ab prevalence remains high in the 1945-1975 birth cohort. These data highlight the need to re-evaluate existing Canadian Preventative Task Force recommendations, to consider incorporating one-time birth cohort and/or other population-based approaches to HCV screening into the clinical workflow as a preventative health measure, and to increase training among community providers to screen for and treat HCV.

背景:广泛筛查和治疗丙型肝炎病毒(HCV)是减少晚期肝病和肝癌的必要条件。临床实践指南和加拿大预防保健工作组的建议在加拿大一次性出生队列(1945- 1975)HCV筛查的价值上存在差异。为了评估这种方法的实用性,我们对不同临床背景下出生队列个体的HCV抗体(Ab)患病率进行了实际分析。方法:对出生于1945年至1975年的个体进行横断面研究,这些个体在2016年1月至2020年12月期间在加拿大安大略省的多个参与中心完成HCV Ab检测。患病率的差异按出生年份、性别和环境进行比较。结果:在16672名出生队列个体中,HCV - Ab患病率为3.2%。年轻人的患病率更高,从1945年至1956年出生的人的0.9%上升到1966年至1975年出生的人的4.6%。男性的患病率(4.4%)高于女性(2.0%),且在不同的检测地点存在差异。在初级保健中,患病率为0.5%,而在药物治疗中心(28.7%)和通过社区外展(14.0%)接受检测的人群中患病率最高。结论:1945-1975年出生队列中HCV - Ab患病率仍然很高。这些数据强调需要重新评估现有的加拿大预防工作组建议,考虑将一次性出生队列和/或其他基于人群的HCV筛查方法纳入临床工作流程,作为预防性健康措施,并增加社区提供者筛查和治疗HCV的培训。
{"title":"Birth cohort hepatitis C antibody prevalence in real-world screening settings in Ontario.","authors":"Mia J Biondi,&nbsp;Grishma Hirode,&nbsp;Camelia Capraru,&nbsp;Aaron Vanderhoff,&nbsp;Joel Karkada,&nbsp;Brett Wolfson-Stofko,&nbsp;David Smookler,&nbsp;Steven M Friedman,&nbsp;Kathy Bates,&nbsp;Tony Mazzulli,&nbsp;Joshua V Juan,&nbsp;Hemant Shah,&nbsp;Bettina E Hansen,&nbsp;Jordan J Feld,&nbsp;Harry LA Janssen","doi":"10.3138/canlivj-2021-0036","DOIUrl":"https://doi.org/10.3138/canlivj-2021-0036","url":null,"abstract":"<p><strong>Background: </strong>Widespread screening and treatment of hepatitis C virus (HCV) is required to decrease late-stage liver disease and liver cancer. Clinical practice guidelines and Canadian Task Force on Preventative Health Care recommendations differ on the value of one-time birth cohort (1945-75) HCV screening in Canada. To assess the utility of this approach, we conducted a real-world analysis of HCV antibody (Ab) prevalence among birth cohort individuals seen in different clinical contexts.</p><p><strong>Methods: </strong>Cross-sectional study of individuals born between 1945 and 1975 who completed HCV Ab testing at multiple participating centres in Ontario, Canada between January 2016 and December 2020. Differences in prevalence were compared by year of birth, gender, and setting.</p><p><strong>Results: </strong>Among 16,672 birth cohort individuals tested, HCV Ab prevalence was 3.2%. Prevalence was higher among younger individuals which increased from 0.9% among those born between 1945 and 1956 to 4.6% among those born between 1966 and 1975. Prevalence was higher among males (4.4%) compared with females (2.0%) and differed by test site. In primary care, the prevalence was 0.5%, whereas the prevalence was highest among those tested at drug treatment centres (28.7%) and through community outreach (14.0%).</p><p><strong>Conclusions: </strong>HCV Ab prevalence remains high in the 1945-1975 birth cohort. These data highlight the need to re-evaluate existing Canadian Preventative Task Force recommendations, to consider incorporating one-time birth cohort and/or other population-based approaches to HCV screening into the clinical workflow as a preventative health measure, and to increase training among community providers to screen for and treat HCV.</p>","PeriodicalId":9527,"journal":{"name":"Canadian liver journal","volume":"5 3","pages":"362-371"},"PeriodicalIF":0.0,"publicationDate":"2022-08-16","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC9473558/pdf/canlivj-2021-0036.pdf","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"33468108","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
Uptake and factors associated with direct-acting antiviral therapy for hepatitis C and treatment outcomes among Canadian immigrants: A retrospective cohort analysis. 加拿大移民丙型肝炎直接抗病毒治疗的吸收和相关因素及治疗结果:一项回顾性队列分析
Pub Date : 2022-08-16 eCollection Date: 2022-08-01 DOI: 10.3138/canlivj-2021-0037
Yelena Petrosyan, John-Graydon Simmons, Erin Kelly, Curtis L Cooper

Background: We sought to compare rates and factors associated with direct acting antiviral (DAA) treatment uptake and sustained virological response (SVR) between Canadian-born and foreign-born patients.

Methods: The study was conducted utilizing a retrospective cohort of hepatitis C virus (HCV)-infected patients assessed at The Ottawa Hospital Viral Hepatitis Clinic between January 2015 and October 2021. Risk factors, income, and clinical characteristics of HCV infection associated with DAA therapy uptake and SVR were compared by immigration status using logistic regression.

Results: Of 1,459 HCV-infected patients, 264 (18.1%) were born outside of the country. A median 17 years passed from immigration to first assessment at the clinic. The proportion of patients initiating DAA therapy was similar between groups (65.2% versus 69.5%, p = 0.17). Characteristics associated with DAA therapy uptake included age at first assessment (OR 1.02; 95% CI 1.01 to 1.03) and being cirrhotic (OR 3.19; 95% CI 1.99 to 2.13). Crude SVR rate was higher in immigrants than in Canadian-born patients (91.5% versus 83.7%, p = 0.01). After controlling for other variables, only advancing age was associated with the likelihood of achieving crude SVR (OR 1.04, 95% CI 1.02 to 1.05).

Conclusions: We found that DAA therapy uptake and HCV cure rates were high in both groups suggesting equity of opportunity in those referred to our program. The older age at presentation suggests missed opportunities to diagnose and engage immigrants in HCV care. These findings emphasize the importance of early large-scale screening and engagement in care for HCV infection of immigrant populations to prevent future complications.

背景:我们试图比较加拿大出生和外国出生的患者之间直接作用抗病毒药物(DAA)治疗的接受率和持续病毒学反应(SVR)的相关因素。方法:该研究利用2015年1月至2021年10月在渥太华医院病毒性肝炎诊所评估的丙型肝炎病毒(HCV)感染患者的回顾性队列进行。采用logistic回归方法比较移民身份与DAA治疗摄取和SVR相关的HCV感染的危险因素、收入和临床特征。结果:1459例hcv感染者中,264例(18.1%)为境外出生者。从移民到在诊所进行第一次评估的平均时间为17年。启动DAA治疗的患者比例在两组之间相似(65.2%对69.5%,p = 0.17)。与服用DAA治疗相关的特征包括首次评估时的年龄(OR 1.02;95% CI 1.01 - 1.03)和肝硬化(OR 3.19;95% CI 1.99 - 2.13)。移民患者的粗SVR率高于加拿大出生患者(91.5%比83.7%,p = 0.01)。在控制了其他变量后,只有年龄增长与实现粗SVR的可能性相关(OR 1.04, 95% CI 1.02至1.05)。结论:我们发现,两组患者DAA治疗的接受率和HCV治愈率都很高,这表明在我们的项目中,机会均等。发病年龄越大,说明移民错过了诊断和参与HCV治疗的机会。这些发现强调了早期大规模筛查和参与移民人群HCV感染护理以预防未来并发症的重要性。
{"title":"Uptake and factors associated with direct-acting antiviral therapy for hepatitis C and treatment outcomes among Canadian immigrants: A retrospective cohort analysis.","authors":"Yelena Petrosyan,&nbsp;John-Graydon Simmons,&nbsp;Erin Kelly,&nbsp;Curtis L Cooper","doi":"10.3138/canlivj-2021-0037","DOIUrl":"https://doi.org/10.3138/canlivj-2021-0037","url":null,"abstract":"<p><strong>Background: </strong>We sought to compare rates and factors associated with direct acting antiviral (DAA) treatment uptake and sustained virological response (SVR) between Canadian-born and foreign-born patients.</p><p><strong>Methods: </strong>The study was conducted utilizing a retrospective cohort of hepatitis C virus (HCV)-infected patients assessed at The Ottawa Hospital Viral Hepatitis Clinic between January 2015 and October 2021. Risk factors, income, and clinical characteristics of HCV infection associated with DAA therapy uptake and SVR were compared by immigration status using logistic regression.</p><p><strong>Results: </strong>Of 1,459 HCV-infected patients, 264 (18.1%) were born outside of the country. A median 17 years passed from immigration to first assessment at the clinic. The proportion of patients initiating DAA therapy was similar between groups (65.2% versus 69.5%, <i>p</i> = 0.17). Characteristics associated with DAA therapy uptake included age at first assessment (OR 1.02; 95% CI 1.01 to 1.03) and being cirrhotic (OR 3.19; 95% CI 1.99 to 2.13). Crude SVR rate was higher in immigrants than in Canadian-born patients (91.5% versus 83.7%, <i>p</i> = 0.01). After controlling for other variables, only advancing age was associated with the likelihood of achieving crude SVR (OR 1.04, 95% CI 1.02 to 1.05).</p><p><strong>Conclusions: </strong>We found that DAA therapy uptake and HCV cure rates were high in both groups suggesting equity of opportunity in those referred to our program. The older age at presentation suggests missed opportunities to diagnose and engage immigrants in HCV care. These findings emphasize the importance of early large-scale screening and engagement in care for HCV infection of immigrant populations to prevent future complications.</p>","PeriodicalId":9527,"journal":{"name":"Canadian liver journal","volume":"5 3","pages":"388-401"},"PeriodicalIF":0.0,"publicationDate":"2022-08-16","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC9473565/pdf/canlivj-2021-0037.pdf","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"33476565","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
Chronic abdominal pain and Budd-Chiari syndrome: A relentless quest for an underlying neoplastic etiology. 慢性腹痛和Budd-Chiari综合征:对潜在肿瘤病因的不懈探索。
Pub Date : 2022-08-16 eCollection Date: 2022-08-01 DOI: 10.3138/canlivj-2021-0039
Natasha Chandok, Said Ishmail, Jeffrey Jaskolka, Sanjeev Sirpal

In this article, we report on a 62-year-old non-cirrhotic male presenting to the emergency department (ED) with chronic abdominal pain, anorexia, and weight loss. Upon initial presentation, physical exam was unremarkable, other than for sarcopenia and splenomegaly. Initial imaging studies revealed a large thrombosis from the iliac vein to the right atrium of the heart. Following discharge, the patient re-consulted to the ED four months later and was re-admitted in renal failure and ascites. The diagnosis of Budd-Chiari syndrome (BCS) was established. Positive immunohistochemistry confirmed a neoplastic ideology of epithelial nature. This case offers a unique perspective on the clinical presentation of secondary BCS, necessitating a consideration in the differential diagnosis of a para-vascular cause. In this case, chronic abdominal pain, often overlooked, may necessitate further workup to establish a clinical diagnosis.

在这篇文章中,我们报告了一位62岁的非肝硬化男性,因慢性腹痛、厌食症和体重减轻而就诊于急诊科。初次就诊时,体格检查无明显异常,除了肌肉减少和脾肿大。最初的影像学检查显示从髂静脉到右心房有一个很大的血栓形成。出院后4个月,患者再次到急诊科就诊,因肾功能衰竭和腹水再次入院。确定Budd-Chiari综合征(BCS)的诊断。免疫组化阳性证实为上皮性质的肿瘤形态。该病例为继发性BCS的临床表现提供了一个独特的视角,需要考虑血管旁原因的鉴别诊断。在这种情况下,慢性腹痛,往往被忽视,可能需要进一步的检查,以建立临床诊断。
{"title":"Chronic abdominal pain and Budd-Chiari syndrome: A relentless quest for an underlying neoplastic etiology.","authors":"Natasha Chandok,&nbsp;Said Ishmail,&nbsp;Jeffrey Jaskolka,&nbsp;Sanjeev Sirpal","doi":"10.3138/canlivj-2021-0039","DOIUrl":"https://doi.org/10.3138/canlivj-2021-0039","url":null,"abstract":"<p><p>In this article, we report on a 62-year-old non-cirrhotic male presenting to the emergency department (ED) with chronic abdominal pain, anorexia, and weight loss. Upon initial presentation, physical exam was unremarkable, other than for sarcopenia and splenomegaly. Initial imaging studies revealed a large thrombosis from the iliac vein to the right atrium of the heart. Following discharge, the patient re-consulted to the ED four months later and was re-admitted in renal failure and ascites. The diagnosis of Budd-Chiari syndrome (BCS) was established. Positive immunohistochemistry confirmed a neoplastic ideology of epithelial nature. This case offers a unique perspective on the clinical presentation of secondary BCS, necessitating a consideration in the differential diagnosis of a para-vascular cause. In this case, chronic abdominal pain, often overlooked, may necessitate further workup to establish a clinical diagnosis.</p>","PeriodicalId":9527,"journal":{"name":"Canadian liver journal","volume":"5 3","pages":"424-427"},"PeriodicalIF":0.0,"publicationDate":"2022-08-16","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC9473557/pdf/canlivj-2021-0039.pdf","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"33468112","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
Early diagnosis and successful long-term management of a rare, severe lysosomal acid lipase deficiency/Wolman disease patient: Infancy to age five. 罕见的严重溶酶体酸性脂肪酶缺乏症/沃尔曼病患者的早期诊断和成功的长期治疗:婴儿期至5岁
Pub Date : 2022-08-16 eCollection Date: 2022-08-01 DOI: 10.3138/canlivj-2021-0041
Antoine Cossette, Julie Castilloux, Chantal Bouffard, Julie Laflamme, Christophe Faure, Sami Benlamlih, Florian Abel, Michael Beecroft, Mira Francis, Régen Drouin

Background: This report describes a unique case of long-term survival of a young girl who was diagnosed with severe, rapidly progressive lysosomal acid lipase deficiency (LAL-D; historically "Wolman disease") at three months of age and began receiving therapeutic interventions at four months of age. This disease involves rapidly progressive multisystemic impairments and limited survival (6-12 months) without treatment.

Methods: Case report taking into account clinical aspects and patient management including a semi-structured interview with the main family caregiver.

Results: Presentation at two months of age: severe malnutrition and chronic diarrhea; hypoalbuminemia; low iron, vitamin A, and vitamin D levels; high triglyceride levels; profound anemia; thrombocytopenia; adrenal calcifications; and mild hepatosplenomegaly. Enzyme replacement therapy (ERT) with sebelipase alfa, parenteral nutrition, and a low-fat diet began at age four months. The patient has received sebelipase alfa for >5 years with good tolerability and is thriving, with a body mass index of 16.35 kg/m2 (80th percentile) despite a stature delay (height <3rd percentile), and mild developmental delay. Optimal medical management requires that family caregivers and health professionals have the knowledge and skills to provide appropriate care and supports multidisciplinary teams through transfer of knowledge to all stakeholders. Effective coordination of services and activities related to child health and development, including navigation of administrative and financial barriers, is also imperative.

Conclusions: Formerly fatal in untreated infants, severe LAL-D, when diagnosed early, can be promptly and effectively treated by combining sebelipase alfa ERT, modified diet, involvement of family caregivers, and multidisciplinary team collaboration.

背景:本报告描述了一个独特的长期生存的年轻女孩谁被诊断为严重的,快速进行性溶酶体酸性脂肪酶缺乏症(LAL-D;历史上称为“沃尔曼病”),并在4个月大时开始接受治疗干预。这种疾病包括快速进展的多系统损伤和有限的生存期(6-12个月)。方法:病例报告,考虑临床方面和患者管理,包括与主要家庭照顾者的半结构化访谈。结果:2月龄时表现:严重营养不良,慢性腹泻;低白蛋白血症;铁、维生素A和维生素D含量低;甘油三酯水平高;深刻的贫血;血小板减少症;肾上腺钙化;轻度肝脾肿大。4个月大时开始使用脂脂酶、肠外营养和低脂饮食的酶替代疗法(ERT)。患者已接受糖脂酶治疗>5年,耐受性良好,健康状况良好,尽管有身材延迟(身高),但体重指数为16.35 kg/m2(第80个百分点)。结论:早期诊断为重症LAL-D的婴儿曾致命,可通过糖脂酶治疗、改善饮食、家庭照顾者的参与和多学科团队合作,及时有效地治疗。
{"title":"Early diagnosis and successful long-term management of a rare, severe lysosomal acid lipase deficiency/Wolman disease patient: Infancy to age five.","authors":"Antoine Cossette,&nbsp;Julie Castilloux,&nbsp;Chantal Bouffard,&nbsp;Julie Laflamme,&nbsp;Christophe Faure,&nbsp;Sami Benlamlih,&nbsp;Florian Abel,&nbsp;Michael Beecroft,&nbsp;Mira Francis,&nbsp;Régen Drouin","doi":"10.3138/canlivj-2021-0041","DOIUrl":"https://doi.org/10.3138/canlivj-2021-0041","url":null,"abstract":"<p><strong>Background: </strong>This report describes a unique case of long-term survival of a young girl who was diagnosed with severe, rapidly progressive lysosomal acid lipase deficiency (LAL-D; historically \"Wolman disease\") at three months of age and began receiving therapeutic interventions at four months of age. This disease involves rapidly progressive multisystemic impairments and limited survival (6-12 months) without treatment.</p><p><strong>Methods: </strong>Case report taking into account clinical aspects and patient management including a semi-structured interview with the main family caregiver.</p><p><strong>Results: </strong>Presentation at two months of age: severe malnutrition and chronic diarrhea; hypoalbuminemia; low iron, vitamin A, and vitamin D levels; high triglyceride levels; profound anemia; thrombocytopenia; adrenal calcifications; and mild hepatosplenomegaly. Enzyme replacement therapy (ERT) with sebelipase alfa, parenteral nutrition, and a low-fat diet began at age four months. The patient has received sebelipase alfa for >5 years with good tolerability and is thriving, with a body mass index of 16.35 kg/m<sup>2</sup> (80th percentile) despite a stature delay (height <3rd percentile), and mild developmental delay. Optimal medical management requires that family caregivers and health professionals have the knowledge and skills to provide appropriate care and supports multidisciplinary teams through transfer of knowledge to all stakeholders. Effective coordination of services and activities related to child health and development, including navigation of administrative and financial barriers, is also imperative.</p><p><strong>Conclusions: </strong>Formerly fatal in untreated infants, severe LAL-D, when diagnosed early, can be promptly and effectively treated by combining sebelipase alfa ERT, modified diet, involvement of family caregivers, and multidisciplinary team collaboration.</p>","PeriodicalId":9527,"journal":{"name":"Canadian liver journal","volume":"5 3","pages":"428-434"},"PeriodicalIF":0.0,"publicationDate":"2022-08-16","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC9473556/pdf/canlivj-2021-0041.pdf","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"33468107","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
Once upon a time in Canadian hepatology, we were accepted into PubMed…. 曾几何时,在加拿大肝病学,我们被PubMed接受了....
Pub Date : 2022-08-16 eCollection Date: 2022-08-01 DOI: 10.3138/canlivj-2022-0024
Eric M Yoshida, Natasha Chandok
{"title":"Once upon a time in Canadian hepatology, we were accepted into PubMed….","authors":"Eric M Yoshida,&nbsp;Natasha Chandok","doi":"10.3138/canlivj-2022-0024","DOIUrl":"https://doi.org/10.3138/canlivj-2022-0024","url":null,"abstract":"","PeriodicalId":9527,"journal":{"name":"Canadian liver journal","volume":"5 3","pages":"327-328"},"PeriodicalIF":0.0,"publicationDate":"2022-08-16","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC9473564/pdf/canlivj-2022-0024.pdf","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"33468110","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
Clinical characterization of patients with primary biliary cholangitis: A report from multiple Canadian centres. 原发性胆道胆管炎患者的临床特征:来自加拿大多个中心的报告。
Pub Date : 2022-08-16 eCollection Date: 2022-08-01 DOI: 10.3138/canlivj-2021-0038
Eric M Yoshida, Mark Gordon Swain, Cynthia Tsien, Edward Tam, Robert James Bailey, Dusanka Grbic, Hin Hin Ko, Alnoor Ramji, Nir Hilzenrat, Magdy Elkhashab, Euiseok Kim, Meaghan O'Brien, Marco Amedeo Puglia, Kevork M Peltekian

Background: Primary biliary cholangitis (PBC) is a rare, chronic autoimmune, cholestatic liver disease affecting approximately 318 per million Canadians. There is limited information regarding the characterization of this patient population in Canada. Consequently, we aim to describe a cohort of PBC patients managed across liver centres serving this type of population.

Methods: A cross-sectional examination of 1,125 PBC patient charts at 15 liver centres across Canada was conducted between January 2016 and September 2017.

Results: Data from 1,125 eligible patients were collected from 7 Canadian provinces. The patient population was largely female (90.2%), had a median overall age of 61.3 years, and a median overall time since diagnosis of 6.4 years. Of the patients included in the study, 89% were on ursodeoxycholic acid (UDCA) therapy at a median dose of 14.0 mg/kg/day and 4.4% were previously treated with UDCA, whereas 6.6% were never treated with UDCA. Of the patients with available data (n = 1067), 289 (27.1%) presented with alkaline phosphatase (ALP) levels ≥200 IU/L and/or total bilirubin levels ≥21 µmol/L. Assessment of UDCA treatment response revealed that 26.6% and 38.3% of patients were inadequate responders according to the Toronto and Paris-II criteria, respectively. Mortality occurred in 1.2% (14) of patients, with liver-related adverse outcomes being more commonly observed in patients who discontinued UDCA compared to those who are currently on treatment (36.3% and 19.6%, respectively).

Conclusion: This study showed that Canadian PBC patients present with demographics and features commonly reported in the literature for this disease. Over one third of PBC patients had inadequate response to UDCA treatment or were not currently being treated with UDCA. Consequently, there is a significant unmet therapeutic need in this Canadian PBC population.

背景:原发性胆道胆管炎(PBC)是一种罕见的慢性自身免疫性胆汁淤积性肝病,大约每百万加拿大人中有318人发病。关于加拿大这一患者群体的特征信息有限。因此,我们的目标是描述一个跨肝脏中心管理的PBC患者队列,为这类人群服务。方法:对2016年1月至2017年9月期间加拿大15个肝脏中心的1125例PBC患者进行横断面检查。结果:从加拿大7个省收集了1,125名符合条件的患者的数据。患者主要为女性(90.2%),中位总年龄为61.3岁,自诊断以来的中位总时间为6.4年。在纳入研究的患者中,89%接受熊去氧胆酸(UDCA)治疗,中位剂量为14.0 mg/kg/天,4.4%以前接受过UDCA治疗,而6.6%从未接受过UDCA治疗。在可获得数据的患者(n = 1067)中,289例(27.1%)呈现碱性磷酸酶(ALP)水平≥200 IU/L和/或总胆红素水平≥21µmol/L。UDCA治疗反应评估显示,根据多伦多和巴黎ii标准,分别有26.6%和38.3%的患者反应不足。1.2%(14)的患者死亡,与目前正在接受治疗的患者相比,停止UDCA治疗的患者更常观察到肝脏相关不良后果(分别为36.3%和19.6%)。结论:本研究表明,加拿大PBC患者具有文献中常见的该病的人口统计学和特征。超过三分之一的PBC患者对UDCA治疗反应不足或目前未接受UDCA治疗。因此,在加拿大PBC人群中存在显著的未满足的治疗需求。
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Canadian liver journal
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