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101.
Choledochal cysts (CDCs) and biliary atresia (BA) are rare pediatric hepatobiliary anomalies that require surgical intervention due to increased risk of malignancy and liver failure, respectively. The underlying disease and operative procedures place patients at risk for long‐term complications, which may continue to affect them into adulthood. Lack of a transitional care model in the health‐care system potentiates the challenges they will face following aging out of their pediatric providers' care. We sought to elucidate the long‐term complications and challenges patients with CDCs and BA face, review the current literature regarding transitioning care, and propose guidelines aiding adult providers in continued care and surveillance of these patients. A literature review was performed to assess short‐term and long‐term complications after surgery and the current standards for transitioning care in patients with a history of CDCs and BA. While transitional programs exist for patients with other gastrointestinal diseases, there are few that focus on CDCs or BA. Generally, authors encourage medical record transmission from pediatric to adult providers, ensuring accuracy of information and compliance with treatment plans. Patients with CDCs are at risk for developing biliary malignancies, cholangitis, and anastomotic strictures after resection. Patients with BA develop progressive liver failure, necessitating transplantation. There are no consensus guidelines regarding timing of follow up for these patients. Based on the best available evidence, we propose a schema for long‐term surveillance.  相似文献   
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Pulse oximetry is used widely to titrate oxygen therapy and for triage in patients who are critically ill. However, there are concerns regarding the accuracy of pulse oximetry in patients with COVID-19 pneumonitis and in patients who have a greater degree of skin pigmentation. We aimed to determine the impact of patient ethnicity on the accuracy of peripheral pulse oximetry in patients who were critically ill with COVID-19 pneumonitis by conducting a retrospective observational study comparing paired measurements of arterial oxygen saturation measured by co-oximetry on arterial blood gas analysis (SaO2) and the corresponding peripheral oxygenation saturation measured by pulse oximetry (SpO2). Bias was calculated as the mean difference between SaO2 and SpO2 measurements and limits of agreement were calculated as bias ±1.96 SD. Data from 194 patients (135 White ethnic origin, 34 Asian ethnic origin, 19 Black ethnic origin and 6 other ethnic origin) were analysed consisting of 6216 paired SaO2 and SpO2 measurements. Bias (limits of agreement) between SaO2 and SpO2 measurements was 0.05% (−2.21–2.30). Patient ethnicity did not alter this to a clinically significant degree: 0.28% (1.79–2.35), −0.33% (−2.47–2.35) and −0.75% (−3.47–1.97) for patients of White, Asian and Black ethnic origin, respectively. In patients with COVID-19 pneumonitis, SpO2 measurements showed a level of agreement with SaO2 values that was in line with previous work, and this was not affected by patient ethnicity.  相似文献   
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Considering the limitations of medical science and the risks associated with medical treatments, we need to re-examine the connotation of medical science from the perspective of philosophy. Medical science is the natural expression of human kindness and human nature of rescuing the dying and healing the wounded. It is a combination of the natural sciences, social sciences, and humanities. From the perspectives of medical philosophy and humanistic care, this article expounds the concepts and ideas of evidence-based, translational, and precision medicine in modern medicine and emphasizes the importance of avoiding new technical bureaucracy, paying attention to achieving a holistic view and systematic understanding, and avoiding biases in development because of the loss of the humanistic spirit in modern medical practice.  相似文献   
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目的 分析2007-2017年浙江省24 388例罕见病住院病例特征,为制定罕见病防控策略提供依据。方法 收集2007-2017年浙江省10家三级甲等(三甲)医院罕见病住院病例资料和各年度住院数,进行描述性统计分析。结果 罕见病病例共24 388例,占住院总例数的2.69‰(24 388/9 054 201),病例数居前3位的疾病类型依次为"血液和造血器官疾病以及某些涉及免疫机能的异常"(占32.81%,8 001/24 388)、"先天性畸形、变形和染色体异常"(占24.87%,6 065/24 388)和"神经系统疾病"(占19.01%,4 635/24 388);2007-2017年罕见病病例数呈逐年增长趋势,年均增幅19.69%,而罕见病例数占同期住院总例数比例仅在2016-2017年明显上升,各类型罕见病时间分布呈不同特征;罕见病的病例数男女性别比为1.35:1(13 990/10 398),男女性别比最高的3类疾病依次为"消化系统疾病"(4.45:1,1 180/265)、"损伤、中毒和外因的某些其他后果"(3.51:1,281/80)和"神经系统疾病"(2.26:1,3 213/1 422);各年龄段罕见病类型、各类型罕见病年龄分布均呈不同特征;病例数居前10位的疾病占全部罕见病例数的53.55%(13 060/24 388),其中前3位疾病分别为成人粒细胞缺乏症(14.41%,3 515/24 388)、皮质基底核退化症(7.60%,1 854/24 388)和亨诺克-舍恩莱因紫癜(6.01%,1 466/24 388)。结论 本研究分析的浙江省2007-2017年24 388例罕见病住院病例的特征资料,是推动我国罕见病的研究、监测或登记数据库构建、制定防控策略的参考依据。  相似文献   
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