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Abstract：
BACKGROUND: Severe hypertriglyceridemia (SHTG) is a rare condition associated with serious complications, such as acute pancreatitis (AP), and the best treatment is still a matter of discussion. The aim of this study is to outline the demographics, management, and outcomes (recurrence and mortality) of complications in patients with SHTG.
METHODS: A retrospective, observational, and analytical study was carried out by obtaining clinical data from the electronic health records of patients with SHTG admitted to the Internal and Intensive Medicine units from the 1st of January 2009 to the 31st of December 2020 in a university hospital.
RESULTS: The cohort included 17 patients. The most common complication was AP (13/17 = 76.5%). Admission to the intensive care unit (ICU) was observed in 84.2%. Among patients with AP, the most commonly administered therapies were insulin (82.4%) and fibrates (76.5%). Plasmapheresis was used in 58.8%, and the criteria for using this technique were mainly based on clinical and laboratory abnormalities. There were no deaths. The readmission rate at 30 days was 36.3%.
CONCLUSIONS: This study shows the morbidity profile associated with SHTG, with a high level of ICU admissions and also a high level of the use of plasmapheresis. In our population, this approach had good results, and this should be highlighted as there are no clear international guidelines for this intervention. Distinguishing between patients with familial chylomicronemia syndrome or with multifactorial chylomicronemia is important as recent specific therapy for lipoprotein lipase (LPL) genetic deficit is available. In the near future, the performance of a genetic study should be considered in patients with SHTG as an attempt to avoid the high recurrence rate of complications of this disease.
METHODS: A retrospective, observational, and analytical study was carried out by obtaining clinical data from the electronic health records of patients with SHTG admitted to the Internal and Intensive Medicine units from the 1st of January 2009 to the 31st of December 2020 in a university hospital.
RESULTS: The cohort included 17 patients. The most common complication was AP (13/17 = 76.5%). Admission to the intensive care unit (ICU) was observed in 84.2%. Among patients with AP, the most commonly administered therapies were insulin (82.4%) and fibrates (76.5%). Plasmapheresis was used in 58.8%, and the criteria for using this technique were mainly based on clinical and laboratory abnormalities. There were no deaths. The readmission rate at 30 days was 36.3%.
CONCLUSIONS: This study shows the morbidity profile associated with SHTG, with a high level of ICU admissions and also a high level of the use of plasmapheresis. In our population, this approach had good results, and this should be highlighted as there are no clear international guidelines for this intervention. Distinguishing between patients with familial chylomicronemia syndrome or with multifactorial chylomicronemia is important as recent specific therapy for lipoprotein lipase (LPL) genetic deficit is available. In the near future, the performance of a genetic study should be considered in patients with SHTG as an attempt to avoid the high recurrence rate of complications of this disease.
摘要：
背景：严重的高甘油三酯血症（SHTG）是一种与严重并发症相关的罕见疾病，如急性胰腺炎（AP），最好的治疗方法仍在讨论中。这项研究的目的是概述人口统计学，管理,SHTG患者并发症的结局（复发和死亡率）。
方法：回顾性研究，观察,和分析研究是通过从2009年1月1日至2020年12月31日在大学医院住院的内部和重症医学科的SHTG患者的电子健康记录中获取临床数据进行的。
结果：该队列包括17名患者。最常见的并发症是AP(13/17=76.5%)。接受重症监护病房（ICU）的比例为84.2％。在AP患者中，最常用的治疗是胰岛素(82.4%)和贝特类药物(76.5%).58.8%的人使用了血浆置换，使用该技术的标准主要基于临床和实验室异常。没有死亡。30天再入院率为36.3%。
结论：这项研究显示了与SHTG相关的发病率，ICU入院率很高，血浆置换的使用也很高。在我们的人口中，这种方法有很好的效果，应该强调这一点，因为这种干预没有明确的国际准则。区分家族性乳糜微粒血症综合征或多因素乳糜微粒血症的患者很重要，因为最近有针对脂蛋白脂酶（LPL）遗传缺陷的特异性疗法。在不久的将来,应考虑在SHTG患者中进行基因研究,以避免该疾病并发症的高复发率.
方法：回顾性研究，观察,和分析研究是通过从2009年1月1日至2020年12月31日在大学医院住院的内部和重症医学科的SHTG患者的电子健康记录中获取临床数据进行的。
结果：该队列包括17名患者。最常见的并发症是AP(13/17=76.5%)。接受重症监护病房（ICU）的比例为84.2％。在AP患者中，最常用的治疗是胰岛素(82.4%)和贝特类药物(76.5%).58.8%的人使用了血浆置换，使用该技术的标准主要基于临床和实验室异常。没有死亡。30天再入院率为36.3%。
结论：这项研究显示了与SHTG相关的发病率，ICU入院率很高，血浆置换的使用也很高。在我们的人口中，这种方法有很好的效果，应该强调这一点，因为这种干预没有明确的国际准则。区分家族性乳糜微粒血症综合征或多因素乳糜微粒血症的患者很重要，因为最近有针对脂蛋白脂酶（LPL）遗传缺陷的特异性疗法。在不久的将来,应考虑在SHTG患者中进行基因研究,以避免该疾病并发症的高复发率.
