治疗乳糜泻综合征的定制治疗策略

IF 3.6 3区 医学 Q2 PHARMACOLOGY & PHARMACY
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引用次数: 0

摘要

背景/简介严重的高甘油三酯血症(HTG),大于 1,000 mg/dL,常常是由于循环中乳糜微粒的病理性积聚,称为乳糜微粒血症。这可能导致血浆高粘度和灌注减少,表现为急性胰腺炎、认知能力受损和神经病变。结果病例 1:一名 35 岁男性,有未控制的 T2DM、高血压(HTN)和 HTG 病史,出现持续性搏动性头痛(HA)。他的检查没有任何异常。临床检查发现他患有严重的高血压(9157 毫克/分升;正常值为 30-150),腹部和脑部影像学检查正常。住院治疗包括禁食(NPO)、静脉输液和输注胰岛素,这使得总胆固醇(TG)逐渐下降到 1341 毫克/分升。治疗 7 天后,HA 恢复正常。病例 2:一名 43 岁女性,有未控制的 T2DM、高血压和 HTG 导致的胰腺炎病史,在用完药物一周后出现 HA 和腹痛。她的检查结果显示血压为 180/96 mmHg,四肢伸展面有糜烂性黄疽,腹部有压痛。她被诊断为高温硫化氢诱发的急性胰腺炎,TG 为 5,307 mg/dL,脂肪酶水平升高(86 U/L;正常值为 5-55),CT 发现胰腺水肿,周围有脂肪滞留。患者被列为 NPO,并接受静脉输液和胰岛素输注治疗。入院第 5 天,由于 TG 持续升高和 HA 持续存在,患者开始接受血浆置换术。一个疗程后,总胆固醇从 2,048 毫克/分升降至 534 毫克/分升,患者的症状也得到缓解。出院前,她重新开始服用阿托伐他汀、非诺贝特和欧米伽-3 酸乙酯。结论治疗严重的、有症状的高血脂症包括 NPO 状态、输注胰岛素和口服降脂治疗;通常需要长期住院。血浆置换术虽然费用高昂且具有创伤性,但能迅速降低总胆固醇、缓解症状并预防与高胆固醇血症相关的并发症。未来对这些治疗方式的研究将有助于制定乳糜微粒血症综合征的住院管理指南。
本文章由计算机程序翻译,如有差异,请以英文原文为准。
Tailored Therapeutic Strategies for the Management of Chylomicronemia Syndrome

Background/Synopsis

Severe hypertriglyceridemia (HTG), greater than 1,000 mg/dL, is frequently due to a pathological accumulation of circulating chylomicrons, termed chylomicronemia. This can lead to plasma hyperviscosity with decreased perfusion, manifesting as acute pancreatitis, impaired cognition, and neuropathy. Given the lack of established guidelines, managing symptomatic chylomicronemia underscores the necessity for personalized expert care.

Objective/Purpose

To review the unusual presentations and inpatient management of chylomicronemia syndrome.

Methods

We present two cases of symptomatic, severe HTG, each managed with different treatment modalities.

Results

Case 1: A 35-year-old man, with a history of uncontrolled T2DM, hypertension (HTN), and HTG, presented with a persistent, throbbing headache (HA). His exam was unremarkable. Clinical investigation revealed severe HTG (9,157 mg/dL; normal 30-150) with normal abdominal and brain imaging. Inpatient management included nothing by mouth (NPO), intravenous (IV) fluids, and insulin infusion, which led to a gradual decline in TG to 1,341 mg/dL. HA resolution occurred after 7 days of therapy. The patient was educated on a very low-fat diet, strict glycemic control, lipid lowering therapy compliance, and scheduled outpatient follow-up.

Case 2: A 43-year-old woman, with a history of uncontrolled T2DM, HTN, and pancreatitis due to HTG, presented with a HA and abdominal pain one week after running out of her medications. Her exam was notable for a blood pressure of 180/96 mmHg, eruptive xanthomas on the extensor surfaces of the extremities, and abdominal tenderness. She was diagnosed with HTG-induced acute pancreatitis, supported by a TG of 5,307 mg/dL, elevated lipase levels (86 U/L; normal 5-55), and CT findings of an edematous pancreas with surrounding fat stranding. The patient was made NPO and treated with IV fluids and insulin infusion. On the 5th day of admission, due to persistently elevated TG and an ongoing HA, plasmapheresis was initiated. After a single session, TG decreased from 2,048 mg/dL to 534 mg/dL, and the patient's symptoms resolved. Prior to discharge, she was restarted on atorvastatin, fenofibrate, and omega-3 acid ethyl esters.

Conclusions

Managing severe, symptomatic HTG includes NPO status, insulin infusion, and oral lipid lowering therapies; often requiring prolonged hospitalization. Plasmapheresis, though costly and invasive, offers a rapid reduction in TG, symptom resolution, and prevention of HTG-associated complications. Future studies on these treatment modalities can aid in developing inpatient management guidelines for chylomicronemia syndrome.

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来源期刊
CiteScore
7.00
自引率
6.80%
发文量
209
审稿时长
49 days
期刊介绍: Because the scope of clinical lipidology is broad, the topics addressed by the Journal are equally diverse. Typical articles explore lipidology as it is practiced in the treatment setting, recent developments in pharmacological research, reports of treatment and trials, case studies, the impact of lifestyle modification, and similar academic material of interest to the practitioner. While preference is given to material of immediate practical concern, the science that underpins lipidology is forwarded by expert contributors so that evidence-based approaches to reducing cardiovascular and coronary heart disease can be made immediately available to our readers. Sections of the Journal will address pioneering studies and the clinicians who conduct them, case studies, ethical standards and conduct, professional guidance such as ATP and NCEP, editorial commentary, letters from readers, National Lipid Association (NLA) news and upcoming event information, as well as abstracts from the NLA annual scientific sessions and the scientific forums held by its chapters, when appropriate.
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