WD, Wilson disease

WD,威尔逊病
  • 文章类型: Journal Article
    未经评估:坚持药物治疗是包括肝移植(LT)后患者在内的任何慢性疾病的整体管理的重要组成部分。因此,本研究旨在评估印度儿童肝病患者(包括LT术后患者)对药物治疗的依从性,并确定影响其发生的变量.
    UNASSIGNED:从2021年5月至2021年10月,对患有Wilson病(WD)和自身免疫性肝病(AILD)的儿科(<18岁)受试者以及LT后接受者进行了一项横断面研究。使用预先验证问卷(药物依从性测量和儿童和青少年对药物依从性问卷)的结构化工具来收集与不依从性患病率(基于错过和晚期剂量)和影响依从性的因素相关的数据。
    未经评估:共有152名儿童被纳入研究(WD39.5%,AILD32.9%,和后期27.6%)。错过和晚期剂量不依从性的患病率(在≥20%的临界值)为12.5%和16.4%,分别。年龄较大(奇数/O。R1.185),留在农村地区(O.R5.08),和障碍,如不良口味的药物(O.R4.728)和难以记住的药物(O。R7.180)与不依从性独立相关(P<0.05)。
    未经评估:总的来说,12-16%的患者不依从,即,大约六分之一的病人,在LT后接受者中观察到的不依从最少(0-2.4%)。患者年龄较大,农村居住地和难以记住/健忘和不良药物口味等个人障碍被确定为导致不依从性的独立因素。
    UNASSIGNED: Adherence to medication(s) is an essential component of holistic management in any chronic disease including in post liver transplant (LT) patients. Thus, this study aimed to assess adherence to medications in Indian pediatric liver disease patients (including post LT recipients) and to identify variables affecting its occurrence.
    UNASSIGNED: A cross-sectional study was conducted among pediatric (<18 years of age) subjects with Wilson disease (WD) and autoimmune liver disease (AILD) along with post LT recipients from May 2021 to October 2021. Structured tools using prevalidated questionnaires (Medication adherence measure and the Child & Adolescent Adherence to Medication Questionnaire) were used to collect data related to nonadherence prevalence (based on missed and late doses) and factors influencing the adherence.
    UNASSIGNED: A total of 152 children were included in the study (WD 39.5%, AILD 32.9%, and post LT 27.6%). Prevalence of missed and late dose nonadherence (at a cut-off of ≥20%) was 12.5% and 16.4%, respectively. Older age (odd\'s ratio/O.R 1.185), stay in a rural area (O.R 5.08), and barriers like bad taste of medication (O.R 4.728) and hard to remember the medication (O.R 7.180) were independently associated with nonadherence (P < 0.05).
    UNASSIGNED: Overall, nonadherence was seen in 12-16%, i.e., around one-sixth of the patients, with least nonadherence seen in post LT recipients (0-2.4%). Older age of the patient, rural place of stay and personal barriers like hard to remember/forgetfulness and bad medication taste were identified as factors independently leading to nonadherence.
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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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