■多灶性纤维硬化是一种引起多器官系统进行性纤维化的罕见疾病。现有资料显示该病有多种表现,有不同的结果。然而,定量数据很少,促使我们需要进行调查.
■从开始到2022年11月16日进行了全面的系统审查,但英语限制,不包括评论文章。文章筛选和提取是独立进行的,并对入围的文章进行偏倚评估。使用SPSS第25版(IBM®SPSS®Statistics;芝加哥,IL,美国)。数据以频率和百分比表示,置信区间为95%。
■这项审查包括134名患者,男性78人(58.2%)。平均年龄为53.6岁,包括两名儿科患者。最常见的合并症是糖尿病(9.7%)。常见症状包括疼痛(47.8%)和肿胀(35.1%)。平均有2.51个器官或解剖部位受到影响,腹膜后(64.2%)受影响最大。胰腺(30.0%)和消化系统(47.0%)是受影响最大的器官/器官系统。79.1%的患者有良好的结果,87.3%没有复发,91.8%的患者存活下来。
■这项研究的发现提供了对人口统计学的定量测量,介绍,器官表现,和多灶性纤维硬化的结果。我们发现这种疾病在日本或美国的男性中很普遍,腹部及其器官通常受累。
UNASSIGNED: Multifocal fibrosclerosis is a rare disorder causing progressive fibrosis of multiple organ systems. Existing data on the disease show that there are multiple manifestations of the disease, with different outcomes. However, quantitative data are scarce, prompting the need for our investigation.
UNASSIGNED: A comprehensive systematic
review was performed from inception to November 16, 2022, with the restriction of English language, not including
review articles. Article screening and extraction was performed independently, and shortlisted articles were assessed for bias. Analysis was performed using SPSS Version 25 (IBM® SPSS® Statistics; Chicago, IL, USA). Data were presented as frequencies and percentages, with a confidence interval of 95%.
UNASSIGNED: This
review included 134 patients, with 78 (58.2%) males. Mean age was 53.6 years and included two pediatric patients. The most common comorbidity was diabetes (9.7%). Prevalent presenting symptoms included pain (47.8%) and swelling (35.1%). A mean of 2.51 organs or anatomical sites was affected, retroperitoneum (64.2%) being most affected. The pancreas (30.0%) and digestive system (47.0%) were the organs/organ systems most affected. Patients had favorable outcomes in 79.1% of cases, 87.3% had no relapse, and 91.8% of patients survived the condition.
UNASSIGNED: The findings in this study provide a quantitative measurement of the demographics, presentations, organ manifestations, and outcomes of multifocal fibrosclerosis. We found the disease to be prevalent in males in Japan or the United States, with the abdomen and its organs being commonly involved.