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Severe Neonatal Presentation of Progressive Familial Intrahepatic Cholestasis Type 4 in an Omani Infant 阿曼婴儿出现严重的进行性家族性肝内胆汁淤积症4型
Pub Date : 2022-08-23 DOI: 10.18295/squmj.8.2022.052
Samira Al Housni, K. Al-Thihli, Dafalla Rahmatalla, Y. Wali, Yusriya Al Rawahi
Progressive familial intrahepatic cholestasis type 4 (PFIC4) is a relatively newly described autosomal recessive disorder caused by biallelic mutations in the gene encoding tight junction protein 2 (TJP2) which is located in chromosome 9q21. PFIC4 is characterized by cholestasis with or without other extrahepatic manifestations. Bleeding tendency due to vitamin k deficiency is a well-known complication of cholestasis. We present a neonate who presented with cholestasis and multiple intracranial bleeds. He was found to have severe coagulopathy and his genetic work up revealed a homozygous variant mutation in TJP2 gene causing PFIC4. He had persistent cholestasis that necessitated an internal biliary diversion with some clinical improvement. Keywords: Jaundice; Intracranial haemorrhage; Progressive Familial Intrahepatic Cholestasis type 4.
进行性家族性肝内胆汁淤积4型(PFIC4)是一种相对较新的常染色体隐性遗传病,由编码紧密连接蛋白2 (TJP2)的基因双等位基因突变引起,该基因位于染色体9q21上。PFIC4以胆汁淤积为特征,伴有或不伴有其他肝外表现。维生素k缺乏引起的出血倾向是胆汁淤积症的一个众所周知的并发症。我们提出了一个新生儿谁提出了胆汁淤积和多发颅内出血。他被发现有严重的凝血功能障碍,他的基因工作显示TJP2基因的纯合变异突变导致PFIC4。他有持续的胆汁淤积,需要进行胆道内分流术,但临床情况有所改善。关键词:黄疸;颅内出血;进行性家族性肝内胆汁淤积4型。
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引用次数: 0
Nursing Students’ Perception and Attitude toward Objective Structured Clinical Examination (OSCE) in Oman 阿曼护生对客观结构化临床检查(OSCE)的认知与态度
Pub Date : 2021-08-18 DOI: 10.18295/squmj.8.2021.113
Shaikha Alamri, Iman Hamdoon Al Hashmi, Kholah Shruba, S. Jamaan, Zaina Alrahbi, Thuraiya Al kaabi
Objective: To explore undergraduate nursing students’ perception and attitude toward the OSCE. Methods: This cross-sectional descriptive study was conducted between April-May 2020, among undergraduate nursing students in the College of Nursing at Sultan Qaboos University. Two self-administered questionnaires were employed to obtain data on students’ perception and attitude toward OSCE. Students’ feedback on OSCE was collected using open-ended questions about OSCE strengths, weakness, and recommendation for improvement. Surveys responses were based on a 5-point ordinal scale ranging from strongly disagree to strongly agree. Students’ Feedback on OSCE was analyzed as descriptive data by identifying the common themes and categorize them in groups. Results: A total of 160 students completed the study questionnaires. Most agreed that OSCE atmosphere is not good (66.3%) and it shouldn’t be the only mode of practical Examination (40.6%). Also, 64.4% of the students felt that timing allocated to complete the skills was not adequate. The data shows that 33.1% of the examinees felt that OSCE examination is more stressful than clinical examination. In addition, few students supported that OSCE has improved their communication skills and it increased their confidence level to perform nursing skills. Conclusion: The findings of this study appear alarming in regard to students’ perception about the overall process and the validity of OSCE examination as an assessment method. Nursing educators need to consider further evaluation and improvement of the OSCE in order to improve students’ acceptance and appreciation of OSCE as an important form of clinical learning and as an assessment method. Keywords: Objective Structured Clinical Examination; Nursing Education; Baccalaureate; Evaluation; Attitude; Nursing Students; Practical Nursing.
目的:探讨本科护生对欧安组织的认知和态度。方法:本横断面描述性研究于2020年4月至5月在苏丹卡布斯大学护理学院的本科护理学生中进行。采用两份自填问卷,了解学生对欧安组织的认知和态度。学生对欧安组织的反馈是通过开放式问题收集的,这些问题涉及欧安组织的优势、劣势和改进建议。调查的回答是基于5分的顺序,从非常不同意到非常同意。通过确定共同主题并将其分组,将学生对欧安组织的反馈作为描述性数据进行分析。结果:共160名学生完成了研究问卷。大多数人认为欧安组织的氛围不好(66.3%),它不应该是实践考试的唯一模式(40.6%)。此外,64.4%的学生认为完成技能的时间分配不够。数据显示,33.1%的考生认为OSCE考试比临床考试压力更大。此外,很少有学生认为欧安组织提高了他们的沟通技巧,提高了他们执行护理技能的信心水平。结论:本研究的结果在学生对整个过程的看法和欧安组织考试作为一种评估方法的有效性方面显得令人震惊。护理教育工作者需要考虑进一步评估和改进OSCE,以提高学生对OSCE作为临床学习的重要形式和评估方法的接受和欣赏。关键词:目的结构化临床检验;护理教育;学士学位;评估;态度;护理学生;实际的护理。
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引用次数: 1
Resuscitation & Research Conference 2018 复苏与研究会议2018
Pub Date : 2019-03-28 DOI: 10.18295/squmj.2018.18.04.027
Sultan Qaboos University
Conference Abstracts
会议摘要
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引用次数: 0
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Sultan Qaboos University Medical Journal [SQUMJ]
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