Canadian liver journal最新文献

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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”。
Canadian liver journal 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
{"title":"The 11th Canadian Symposium on Hepatitis C Virus: 'Getting back on track towards hepatitis C elimination'.","authors":"Ana Maria Passos-Castilho,&nbsp;Sasha Tejna Persaud Udhesister,&nbsp;Guillaume Fontaine,&nbsp;Dahn Jeong,&nbsp;Melisa Dickie,&nbsp;Carrielynn Lund,&nbsp;Rodney Russell,&nbsp;Nadine Kronfli","doi":"10.3138/canlivj-2022-0034","DOIUrl":"https://doi.org/10.3138/canlivj-2022-0034","url":null,"abstract":"<p><p>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.</p>","PeriodicalId":9527,"journal":{"name":"Canadian liver journal","volume":"6 1","pages":"56-69"},"PeriodicalIF":0.0,"publicationDate":"2023-02-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC9997521/pdf/canlivj-2022-0034.pdf","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"9453746","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
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。
Canadian liver journal Pub Date : 2023-02-01 DOI: 10.3138/canlivj-2022-0020
Tanya Barber, Lynn Toon, Puneeta Tandon, Lee A Green
{"title":"Exploring provider roles, continuity, and mental models in cirrhosis care: A qualitative study.","authors":"Tanya Barber,&nbsp;Lynn Toon,&nbsp;Puneeta Tandon,&nbsp;Lee A Green","doi":"10.3138/canlivj-2022-0020","DOIUrl":"https://doi.org/10.3138/canlivj-2022-0020","url":null,"abstract":"<p><p><b>BACKGROUND:</b> 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. <b>METHODS:</b> 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 (<i>n =</i> 8), specialists (<i>n =</i> 9), and cirrhosis nurse practitioners (<i>n =</i> 2). <b>RESULTS:</b> 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. <b>CONCLUSIONS:</b> 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.</p>","PeriodicalId":9527,"journal":{"name":"Canadian liver journal","volume":"6 1","pages":"14-23"},"PeriodicalIF":0.0,"publicationDate":"2023-02-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC9997517/pdf/canlivj-2022-0020.pdf","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"9453749","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
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。
Canadian liver journal Pub Date : 2023-02-01 DOI: 10.3138/canlivj-2022-0019
Julie Anne Ting, Dilshani Induruwage, Eric M Yoshida, Miten Dhruve, Nadia Y Zalunardo
{"title":"Albuminuria post-liver transplant is a predictor of kidney disease progression and mortality.","authors":"Julie Anne Ting,&nbsp;Dilshani Induruwage,&nbsp;Eric M Yoshida,&nbsp;Miten Dhruve,&nbsp;Nadia Y Zalunardo","doi":"10.3138/canlivj-2022-0019","DOIUrl":"https://doi.org/10.3138/canlivj-2022-0019","url":null,"abstract":"<p><p><b>BACKGROUND:</b> 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.<b>METHODS:</b> 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%. <b>RESULTS:</b> At baseline, mean eGFR was 67 (SD 20.9) mL/min/1.73 m<sup>2</sup>, 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; <i>P</i> = 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; <i>P</i> < 0.0001). <b>CONCLUSIONS:</b> 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.</p>","PeriodicalId":9527,"journal":{"name":"Canadian liver journal","volume":"6 1","pages":"2-13"},"PeriodicalIF":0.0,"publicationDate":"2023-02-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC9997516/pdf/canlivj-2022-0019.pdf","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"9453744","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
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。
Canadian liver journal Pub Date : 2023-02-01 DOI: 10.3138/canlivj-2022-0017
L Alexa Thompson, Sabrina S Plitt, Jennifer Gratrix, Carmen L Charlton
{"title":"Prevalence of syphilis coinfection in hepatitis C virus positive prenatal patients from Alberta during a pilot routine screening program.","authors":"L Alexa Thompson,&nbsp;Sabrina S Plitt,&nbsp;Jennifer Gratrix,&nbsp;Carmen L Charlton","doi":"10.3138/canlivj-2022-0017","DOIUrl":"https://doi.org/10.3138/canlivj-2022-0017","url":null,"abstract":"<p><p><b>BACKGROUND:</b> 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.<b>METHODS:</b> 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 <i>Treponema pallidum</i> particle agglutination (TPPA) or follow-up rapid plasma reagin (RPR) test were considered positive for syphilis. Descriptive statistics for coinfected patients were analyzed. <b>RESULTS:</b> 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. <b>CONCLUSIONS:</b> 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.</p>","PeriodicalId":9527,"journal":{"name":"Canadian liver journal","volume":"6 1","pages":"70-75"},"PeriodicalIF":0.0,"publicationDate":"2023-02-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC9997519/pdf/canlivj-2022-0017.pdf","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"9469545","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
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。
Canadian liver journal 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
{"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
Canadian liver journal 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
{"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。
Canadian liver journal Pub Date : 2022-08-16 eCollection Date: 2022-08-01 DOI: 10.3138/canlivj-2021-0039
Natasha Chandok, Said Ishmail, Jeffrey Jaskolka, Sanjeev Sirpal
{"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
Canadian liver journal 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
{"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....
Canadian liver journal 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。
Canadian liver journal 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
{"title":"Clinical characterization of patients with primary biliary cholangitis: A report from multiple Canadian centres.","authors":"Eric M Yoshida,&nbsp;Mark Gordon Swain,&nbsp;Cynthia Tsien,&nbsp;Edward Tam,&nbsp;Robert James Bailey,&nbsp;Dusanka Grbic,&nbsp;Hin Hin Ko,&nbsp;Alnoor Ramji,&nbsp;Nir Hilzenrat,&nbsp;Magdy Elkhashab,&nbsp;Euiseok Kim,&nbsp;Meaghan O'Brien,&nbsp;Marco Amedeo Puglia,&nbsp;Kevork M Peltekian","doi":"10.3138/canlivj-2021-0038","DOIUrl":"https://doi.org/10.3138/canlivj-2021-0038","url":null,"abstract":"<p><strong>Background: </strong>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.</p><p><strong>Methods: </strong>A cross-sectional examination of 1,125 PBC patient charts at 15 liver centres across Canada was conducted between January 2016 and September 2017.</p><p><strong>Results: </strong>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).</p><p><strong>Conclusion: </strong>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.</p>","PeriodicalId":9527,"journal":{"name":"Canadian liver journal","volume":"5 3","pages":"372-387"},"PeriodicalIF":0.0,"publicationDate":"2022-08-16","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC9473559/pdf/canlivj-2021-0038.pdf","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"33468111","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}
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