Johnson Johns T, Paul Jinson, Cherian Kripa Elizabeth, Kapoor Nitin, Asha H S, Paul Thomas Vizhalil
Department of Endocrinology, Diabetes and Metabolism, Christian Medical College and Hospital, Vellore, Tamil Nadu, India.
J Family Med Prim Care. 2020 Aug 25;9(8):4451-4453. doi: 10.4103/jfmpc.jfmpc_819_20. eCollection 2020 Aug.
Familial hypercholesterolaemia (FH) is an autosomal dominant inherited disorder of lipoprotein metabolism caused by defects in the low-density lipoprotein receptor (LDLR) gene. It is characterized by high low-density lipoprotein (LDL) cholesterol levels, premature cardiovascular disease (CVD), and tendon xanthomas. We present the case of a 26-year-old gentleman who presented with multiple nodular eruptions over the extensor aspects of upper and lower limbs and was diagnosed as FH on the basis of positive family history, typical lipid profile abnormalities, and biopsy of the nodule consistent with tendon xanthomas. The diagnosis and management of this case is deftly feasible at the primary care level.
家族性高胆固醇血症(FH)是一种常染色体显性遗传的脂蛋白代谢紊乱疾病,由低密度脂蛋白受体(LDLR)基因缺陷引起。其特征为低密度脂蛋白(LDL)胆固醇水平升高、早发性心血管疾病(CVD)和肌腱黄色瘤。我们报告一例26岁男性患者,其上肢和下肢伸侧出现多处结节性皮疹,基于阳性家族史、典型的血脂异常以及与肌腱黄色瘤相符的结节活检结果,被诊断为FH。该病例在基层医疗水平上的诊断和管理是切实可行的。