An update on primary sclerosing cholangitis epidemiology, outcomes and quantification of alkaline phosphatase variability in a population-based cohort.
An update on primary sclerosing cholangitis epidemiology, outcomes and quantification of alkaline phosphatase variability in a population-based cohort.
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DOI：
10.1007/s00535-020-01663-1
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发表时间：
2020-05
影响因子：
6.3
通讯作者：
Eaton JE
中科院分区：
文献类型：
--
作者：
Bakhshi Z;Hilscher MB;Gores GJ;Harmsen WS;Viehman JK;LaRusso NF;Gossard AA;Lazaridis KN;Lindor KD;Eaton JE
目前缺乏描述血清碱性磷酸酶（SAP）的流行病学、自然史和长期波动及其预后相关性的原发性硬化性胆管炎（PSC）人群队列。因此，我们调查了过去41年来明尼苏达州奥姆斯特德县PSC患者的发病率和自然史，并量化了SAP波动。罗切斯特流行病学项目用于确定1976年至2017年奥姆斯特德县诊断为PSC的56名受试者。主要终点（n = 19）包括肝移植、肝功能失代偿和胆管癌。与1976-2000年相比，2001 - 2017年经年龄和性别调整的PSC发病率（每10万人年）几乎翻了一番（1.47; 95% CI 0.99-1.96 vs 0.79; 95% CI 0.42-1.16，p = 0.02）。这种增加意味着在诊断时具有较轻表型标志物的患者增加：正常SAP（26.32%对0%，p < 0.01）和较低的马约PSC风险评分[0.36（-0.57至1.55）对-0.50（-1.25至0.35），p = 0.03]。个体内SAP波动，中位变异系数为36.20%。SAP正常化和降至1.5 ×正常上限（ULN）以下的发生率分别为每年5%和10%。SAP小于1.5 × ULN与PSC相关并发症风险较低相关（风险比0.11; 95% CI 0.03-0.42）。PSC患者越来越多地被诊断为具有较温和的表型。虽然较低的SAP与改善的结局相关，但SAP水平的高个体内差异使人质疑在临床试验中使用单一SAP值作为替代终点的做法。
Contemporary primary sclerosing cholangitis (PSC) population-based cohorts describing the epidemiology, natural history, and long-term fluctuations in serum alkaline phosphatase (SAP) and their prognostic relevance are lacking. Therefore, we investigated the incidence and natural history of PSC and quantified SAP fluctuations among those with PSC in Olmsted County, Minnesota over the last 41 years. The Rochester Epidemiology Project was used to identify 56 subjects diagnosed with PSC between 1976 and 2017 in Olmsted County. The primary endpoint (n = 19) included liver transplantation, hepatic decompensation, and cholangiocarcinoma. The age- and sex-adjusted incidence of PSC (per 100,000 person years) nearly doubled from 2001 to 2017 compared to 1976–2000 (1.47; 95% CI 0.99–1.96 versus 0.79; 95% CI 0.42–1.16, p = 0.02). This increase paralleled a rise in patients with markers of a milder phenotype at the time of diagnosis: normal SAP (26.32% versus 0%, p < 0.01) and lower Mayo PSC risk score [0.36 (− 0.57 to 1.55) versus − 0.50 (− 1.25 to 0.35), p = 0.03]. Intra-individual SAP fluctuates with a median coefficient of variation of 36.20%. SAP normalization and dropping below 1.5 × upper limit of normal (ULN) occurs at a rate of 5% and 10% per year, respectively. SAP less than 1.5 × ULN was associated with a lower risk of PSC-related complications (hazard ratio 0.11; 95% CI 0.03–0.42). The patients with PSC are increasingly being diagnosed with a milder phenotype. While a lower SAP is associated with improved outcomes, the high intra-individual variation of SAP levels calls into question the practice of using a single SAP value as a surrogate endpoint in clinical trials.
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影响因子：
25.7
作者：
Al Mamari, Said;Djordjevic, Jelena;Chapman, Roger W.
通讯作者：
Chapman, Roger W.
DOI：
10.1016/j.cgh.2016.10.039
发表时间：
2017-06
期刊：
Clinical gastroenterology and hepatology : the official clinical practice journal of the American Gastroenterological Association
影响因子：
--
作者：
Shivashankar R;Tremaine WJ;Harmsen WS;Loftus EV Jr
通讯作者：
Loftus EV Jr
影响因子：
25.7
作者：
Card, Tim R.;Solaymani-Dodaran, Masoud;West, Joe
通讯作者：
West, Joe
影响因子：
29.4
作者：
Eaton JE;Talwalkar JA;Lazaridis KN;Gores GJ;Lindor KD
通讯作者：
Lindor KD
影响因子：
12.6
作者：
Lindstrom, Lina;Hultcrantz, Rolf;Bergquist, Annika
通讯作者：
Bergquist, Annika