Mangeshkar Shaunak, Nazarenko Natalia, Varrias Dimitrios, Spanos Michail, Borkowski Pawel, Alhuarrat Majd Al Deen, Li Weijia, Kishore Preeti, Faillace Robert T
Internal Medicine, Albert Einstein College of Medicine, Jacobi Medical Center, New York, USA.
Cardiovascular Medicine, Massachusetts General Hospital, Harvard Medical School, Boston, USA.
Cureus. 2023 Jul 5;15(7):e41424. doi: 10.7759/cureus.41424. eCollection 2023 Jul.
Type V hyperlipoproteinemia or multifactorial chylomicronemia syndrome is a rare lipid disorder triggered mainly by uncontrolled diabetes, obesity, poor diet, or particular medications. It is associated with an increased risk of acute pancreatitis and accelerated coronary artery disease which may manifest in younger age groups. We present a case of a 42-year-old male who presented to the emergency department (ED) complaining of a non-healing hand injury. Upon laboratory workup, the patient was found to have an elevated total cholesterol (TC) of 1129 mg/dL, very low levels of high-density lipoprotein (HDL) and triglycerides (TG) > 4000 mg/dL with an inability to calculate low-density lipoprotein (LDL). Lipoprotein electrophoresis revealed an actual TG level of > 7000 mg/dL, increased chylomicrons, normal B and pre-B-lipoproteins, and increased L-lipoproteins with an elevated Apolipoprotein B. Despite these derangements, the patient did not exhibit any abdominal complaints, demonstrating a normal lipase level. The physical exam was indicative of bilateral arcus senilis and obesity. Insulin drip was initiated along with intravenous (IV) hydration and it required 12 days to bring triglycerides down to less than 1000 mg/dL. The total cholesterol was also seen to be down trending to around 500 mg/dL and the HDL improved to 22 mg/dL. We present this case as a unique presentation of asymptomatic chylomicronemia resistant to insulin treatment with an elevated ApoB but with no evidence of pancreatitis or coronary artery disease.
V型高脂蛋白血症或多因素乳糜微粒血症综合征是一种罕见的脂质紊乱疾病,主要由未控制的糖尿病、肥胖、不良饮食或特定药物引发。它与急性胰腺炎风险增加以及可能在较年轻年龄组出现的加速冠状动脉疾病相关。我们报告一例42岁男性病例,该患者因手部伤口不愈合就诊于急诊科。经实验室检查,发现患者总胆固醇(TC)升高至1129mg/dL,高密度脂蛋白(HDL)水平极低,甘油三酯(TG)>4000mg/dL,无法计算低密度脂蛋白(LDL)。脂蛋白电泳显示实际TG水平>7000mg/dL,乳糜微粒增加,B和前B脂蛋白正常,L脂蛋白增加且载脂蛋白B升高。尽管有这些紊乱情况,但患者未表现出任何腹部不适,脂肪酶水平正常。体格检查显示双侧角膜弓和肥胖。开始静脉输注胰岛素并进行静脉补液,需要12天时间才能使甘油三酯降至1000mg/dL以下。总胆固醇也呈下降趋势,降至约500mg/dL,HDL升至22mg/dL。我们将此病例作为无症状乳糜微粒血症的独特表现进行报告,该病例对胰岛素治疗耐药,载脂蛋白B升高,但无胰腺炎或冠状动脉疾病的证据。