Purohit Purvi, Khera Daisy, Sharma Praveen, Singh Kuldeep
Department of Biochemsitry, AIIMS Jodhpur, Jodhpur, India.
Department of Pediatrics, AIIMS Jodhpur, Jodhpur, India.
Indian J Clin Biochem. 2017 Oct;32(4):493-495. doi: 10.1007/s12291-016-0626-1. Epub 2016 Nov 16.
Type V hypertriglyceridemia in children is a rare condition since it has often been associated with obesity, type II diabetes, metabolic syndrome and hormone therapy. We encountered a case of massive hypertriglyceridemia (1900 mg/dl) in a 6 years old boy with complains of acute pancreatitis but no physical manifestations. There was no family history of sudden cardiac death, father and younger male sibling were found to be normal. The mother however had hypertriglyceridemia. The child was managed by dietary changes, omega 3 capsules and low dose fenofibrate. On follow up showed there was reduction in lipid profile and lipoprotein electrophoretic pattern showed bands for VLDL and chylomicron indicating type V hyperlipidemia. Early diagnosis via screening for lipid profile of such patients and their family can improve prognosis and quality of life of these children.
儿童V型高甘油三酯血症是一种罕见病症,因为它常与肥胖、II型糖尿病、代谢综合征及激素治疗相关。我们遇到一例6岁男孩,患有严重高甘油三酯血症(1900毫克/分升),主诉急性胰腺炎,但无身体表现。无心脏性猝死家族史,发现其父亲和弟弟正常。然而,母亲患有高甘油三酯血症。该患儿通过饮食调整、服用ω-3胶囊及低剂量非诺贝特进行治疗。随访显示血脂谱有所降低,脂蛋白电泳图谱显示极低密度脂蛋白(VLDL)和乳糜微粒条带,提示为V型高脂血症。通过筛查此类患者及其家族的血脂谱进行早期诊断,可改善这些儿童的预后和生活质量。