HVOTO, hepatic venous outflow tract obstruction

  • 文章类型: Journal Article
    缺乏接受包括单独抗凝在内的药物治疗的Budd-Chairi综合征(BCS)患者的长期预后数据。
    连续患者(N=138,平均值[标准差,SD]年龄29.3[12.9]岁;66名男性)患有BCS,仅接受药物治疗,包括抗凝治疗,纳入最少随访12个月.初始反应被分类为完全(CR),部分(PR)或无应答(NR),并作为应答丧失(LoR)或应答维持(MoR)进行随访。基线的关联,评估了具有不同反应的临床和生化参数.
    76例患者(55.1%)有CR,26例(18.8%)有PR,36例(26.1%)有NR。具有PR或NR的人后来都没有CR。在中位随访40(范围12-174)个月时,LoR在PR组比CR组更常见(12[46.2%]vs18[23.7%],P=0.03)。LoR与腹水的存在相关(比值比[OR]1.5;95%置信区间[CI]0.06-0.71),基线和随访期间的胃肠道出血(OR1.33;95%CI0.09-0.82)或黄疸(OR1.01;95%CI0.11-0.97)(OR0.018;95%CI1.006-1.030)。NR(28[77.8%])死亡率高于CR(15[19.7%],P=0.001)和PR(8[30.8%],P=0.001)。在二元逻辑回归分析中,基线时腹水的存在与LoR相关(OR0.303[0.098-0.931]).
    初始CR患者的生存率优于无反应者。三分之一的人在后续行动中有LoR。基线时腹水的存在与LoR相关。
    UNASSIGNED: There is lack of data on long-term outcomes of patients with Budd-Chairi Syndrome (BCS) treated with medical therapy including anticoagulation alone.
    UNASSIGNED: Consecutive patients (N = 138, mean [standard deviation, SD] age 29.3 [12.9] years; 66 men) with BCS, treated with medical therapy alone including anticoagulation, with minimum follow-up of 12 months were included. Initial response was classified as complete (CR), partial (PR) or nonresponse (NR) and on follow-up as loss of response (LoR) or maintenance of response (MoR). The association of baseline, clinical and biochemical parameters with different responses was evaluated.
    UNASSIGNED: Seventy-six patients (55.1%) had CR, 26 (18.8%) had PR and 36 (26.1%) had NR. None with PR or NR had CR later. At a median follow-up of 40 (range 12-174) months, LoR was more common in PR group than in CR group (12 [46.2%] vs 18 [23.7%], P = 0.03). LoR was associated with presence of ascites (odds ratio [OR] 1.5; 95% confidence interval [CI] 0.06-0.71), gastrointestinal bleed (OR 1.33; 95% CI 0.09-0.82) or jaundice (OR 1.01; 95% CI 0.11-0.97) at baseline and duration of follow-up (OR 0.018; 95% CI 1.006-1.030). Mortality was higher in NR (28 [77.8%]) compared with CR (15 [19.7%], P = 0.001) and PR (8 [30.8%], P = 0.001). On binary logistic regression analysis, presence of ascites at baseline was associated with LoR (OR 0.303 [0.098-0.931]).
    UNASSIGNED: Patients with initial CR have better survival than nonresponders. One-third had LoR on follow-up. The presence of ascites at baseline is associated with LoR.
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  • 文章类型: Journal Article
    急性肝衰竭(ALF)是罕见的,不可预测的,各种病因导致的急性肝损伤(ALI)的潜在致命并发症。文献中报道的ALF病因具有区域差异,影响临床表现和自然病程。在旨在反映印度临床实践的共识文章的这一部分中,疾病负担,流行病学,临床表现,监测,和预测已经讨论过了。在印度,病毒性肝炎是ALF的最常见原因,抗结核药物引起的药物性肝炎是第二常见的原因。ALF的临床表现以黄疸为特征,凝血病,和脑病。区分ALF和其他肝衰竭的原因是很重要的,包括慢性急性肝衰竭,亚急性肝功能衰竭,以及某些可以模仿这种表现的热带感染。该疾病通常具有暴发性临床过程,短期死亡率很高。死亡通常归因于脑部并发症,感染,导致多器官衰竭。及时肝移植(LT)可以改变结果,因此,在可以安排LT之前,为患者提供重症监护至关重要。评估预后以选择适合LT的患者同样重要。已经提出了几个预后评分,他们的比较表明,本土开发的动态分数比西方世界描述的分数更具优势。ALF的管理将在本文件的第2部分中描述。
    Acute liver failure (ALF) is an infrequent, unpredictable, potentially fatal complication of acute liver injury (ALI) consequent to varied etiologies. Etiologies of ALF as reported in the literature have regional differences, which affects the clinical presentation and natural course. In this part of the consensus article designed to reflect the clinical practices in India, disease burden, epidemiology, clinical presentation, monitoring, and prognostication have been discussed. In India, viral hepatitis is the most frequent cause of ALF, with drug-induced hepatitis due to antituberculosis drugs being the second most frequent cause. The clinical presentation of ALF is characterized by jaundice, coagulopathy, and encephalopathy. It is important to differentiate ALF from other causes of liver failure, including acute on chronic liver failure, subacute liver failure, as well as certain tropical infections which can mimic this presentation. The disease often has a fulminant clinical course with high short-term mortality. Death is usually attributable to cerebral complications, infections, and resultant multiorgan failure. Timely liver transplantation (LT) can change the outcome, and hence, it is vital to provide intensive care to patients until LT can be arranged. It is equally important to assess prognosis to select patients who are suitable for LT. Several prognostic scores have been proposed, and their comparisons show that indigenously developed dynamic scores have an edge over scores described from the Western world. Management of ALF will be described in part 2 of this document.
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