Primary Immunodeficiency Diseases in Oman: 10-Year Experience in a Tertiary Care Hospital
Primary Immunodeficiency Diseases in Oman: 10-Year Experience in a Tertiary Care Hospital
复制标题
DOI：
10.1007/s10875-016-0337-7
复制
发表时间：
2016-11-01
影响因子：
9.1
通讯作者：
Dennison, David
中科院分区：
文献类型：
--
作者：
Al-Tamemi, Salem;Naseem, Shafiq Ur Rehman;Dennison, David
原发性免疫缺陷（PID）疾病是罕见的，复杂的医学疾病，往往被忽视在临床设置。中东人群中出现了新的PID报告。本研究描述了在阿曼的三级护理设置PID患者的特点，并将其与区域和世界范围的报告进行比较。苏丹卡布斯大学医院（SQUH）是一家学术性三级保健医院，专门提供包括PID患者在内的专业医疗保健服务。在诊断时，以电子形式输入患者的社会人口统计学，临床特征，实验室调查和管理。该研究纳入了2005年8月至2015年7月期间就诊的患者。登记了140例患者，估计最低人群患病率为7.0/100,000。男女比例为1.6:1，出现症状的中位年龄为8个月，诊断21个月，延迟13个月。44%的患者家族史呈阳性，76%的患者有血缘关系，36%的患者有前兄弟姐妹死亡，18%的患者总死亡率，诊断后10年生存率为85%。最常见的免疫缺陷类型是吞噬性疾病（35.0%），其次是抗体疾病（20.7%），联合免疫缺陷（17.8%），其他明确的PID综合征（15.0%），免疫失调综合征（3.5%），补体缺陷（3.5%）和未分类免疫缺陷（4.2%）。最常见的感染是肺炎（47.1%）。PID在阿曼并不罕见。患病率与该地区的报告一致，但高于西方人群。本研究结果将有助于提高对PID的认识、管理和政策制定。
Primary immunodeficiency (PID) diseases are rare, complex medical disorders that often are overlooked in clinical settings. There are emerging reports of PID from Middle Eastern populations. This study describes the features of PID patients in a tertiary care setting in Oman and compares them with regional and worldwide reports.Sultan Qaboos University Hospital (SQUH) is an academic tertiary care-level hospital for specialized healthcare, including PID patients. At the time of diagnosis, patients' sociodemographics, clinical features, laboratory investigations, and management were entered in electronic form. This study included patients seen between August 2005 and July 2015.One hundred forty patients were registered with a minimum estimated population prevalence of 7.0/100,000. The male/female ratio was 1.6:1, the median age of onset of symptoms was 8 months, and diagnosis was 21 months with a delay of 13 months. Family history was positive in 44 %, consanguinity was present in 76 %, death of a previous sibling was present in 36 %, and there was an overall mortality in 18 %, with an 85 % probability of survival 10 years following diagnosis. The most common type of immunodeficiency was phagocytic disorders (35.0 %), followed by predominantly antibody disorders (20.7 %), combined immunodeficiency (17.8 %), other well-defined PID syndromes (15.0 %), immune dysregulation syndromes (3.5 %), complement deficiencies (3.5 %), and unclassified immunodeficiency (4.2 %). The commonest presenting infection was pneumonia (47.1 %).PID is not a rare condition in Oman. The prevalence is in concordance with reports from the region but higher than in Western populations. The findings of the current study would help to improve the awareness and management of, and policy making for PID.