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本文报道一例7岁半的家族性纯合子高胆固醇血症女性患儿,在经过饮食控制及降低胆固醇药物治疗后,未能达到胆固醇水平的持久下降。经过3次LDL去除术治疗后出现贫血。该患儿以后发生急性心肌梗死征象,心电图示亚急性前间壁心肌梗死,选择性冠状动脉造影显示严重的冠状动脉3支病变伴主干狭窄及左心功能明显受限,EF为27％。检查结束后出现血压下降伴心动过缓,需做紧急主动脉-冠状动脉旁路移植术(冠状动脉搭桥术)。尽管采取上述措施,患儿仍死于抗儿茶酚胺性心源性休克。 讨论 本例证实了这样一个已知事实,家族性纯合子高
This article reports the failure of a 7-and-a-half-year, familial homozygous female with hypercholesterolemia to achieve a sustained decline in cholesterol levels after diet control and cholesterol-lowering drug treatment. After three LDL removal therapy anemia. The patient developed signs of acute myocardial infarction, ECG showed subacute anterior myocardial infarction, and selective coronary angiography showed severe 3 lesions of the coronary artery with stenosis and left ventricular function significantly limited, EF was 27%. Blood pressure dropped with bradycardia at the end of the study and emergency aorto-coronary artery bypass grafting (CABG) was required. Despite these measures, children still die of anti-catecholamyltic cardiogenic shock. Discussion This example demonstrates the known fact that familial homozygotes are high