Suha Ahmed, Marwa Elgizouli, Eric S Kilpatrick, Timothy J Morris
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引用次数: 0
摘要
合并或混合型高脂血症的特点是高胆固醇血症和高甘油三酯浓度。在英国,大约每 100 人中就有 1 人患有此病。大多数病例是继发于代谢综合征、糖尿病(尤其是控制不佳)或高酒精摄入量等潜在疾病。混合型高脂血症也是某些原发性高脂血症的特征之一,如家族性联合高脂血症(FCH)或 III 型高脂血症(脂蛋白异常血症)。混合型高脂血症的一个容易被忽视的鉴别诊断是,潜在诊断为家族性高胆固醇血症(FH)的患者同时还伴有其他原因引起的高甘油三酯血症。这些患者的总胆固醇和低密度脂蛋白胆固醇(LDL-C)浓度可能非常高,甘油三酯浓度也会中度升高。在本文中,我们报告了 4 例家族性高胆固醇血症病例,这些患者最初表现为高甘油三酯血症,此外还伴有高总胆固醇和低密度脂蛋白胆固醇。本文讨论了与这种表现相关的诊断难题,并强调了在这些特殊情况下直接测量低密度脂蛋白胆固醇有助于诊断的关键作用。
Familial hypercholesterolaemia with high triglycerides: A diagnostic challenge.
Combined or mixed hyperlipidaemia is characterised by hypercholesterolaemia together with high triglyceride concentrations. It is found in approximately 1 in 100 people in the United Kingdom. Most cases are secondary to an underlying condition such as the metabolic syndrome, diabetes mellitus (especially poorly controlled) or individuals with a high alcohol intake. Mixed hyperlipidaemia is also a feature of some primary hyperlipidaemia conditions such familial combined hyperlipidaemia (FCH) or type III hyperlipidaemia (dysbetalipoproteinaemia). One differential diagnosis for mixed hyperlipidaemia that can easily be overlooked is a patient with an underlying diagnosis of familial hypercholesterolaemia (FH) who also has a hypertriglyceridaemia due to any other cause. Those patients may have very high total and low-density lipoprotein cholesterol concentrations (LDL-C) with a moderately elevated triglyceride concentration. In this article, we report 4 cases of familial hypercholesterolaemia, confirmed by genetic testing, in patients initially presenting with hypertriglyceridaemia in addition to high total cholesterol and LDL-C. This article discusses the diagnostic challenges associated with this presentation and highlights the key role of directly measuring LDL-C to aid diagnosis in these specific situations.
期刊介绍:
Annals of Clinical Biochemistry is the fully peer reviewed international journal of the Association for Clinical Biochemistry and Laboratory Medicine.
Annals of Clinical Biochemistry accepts papers that contribute to knowledge in all fields of laboratory medicine, especially those pertaining to the understanding, diagnosis and treatment of human disease. It publishes papers on clinical biochemistry, clinical audit, metabolic medicine, immunology, genetics, biotechnology, haematology, microbiology, computing and management where they have both biochemical and clinical relevance. Papers describing evaluation or implementation of commercial reagent kits or the performance of new analysers require substantial original information. Unless of exceptional interest and novelty, studies dealing with the redox status in various diseases are not generally considered within the journal''s scope. Studies documenting the association of single nucleotide polymorphisms (SNPs) with particular phenotypes will not normally be considered, given the greater strength of genome wide association studies (GWAS). Research undertaken in non-human animals will not be considered for publication in the Annals.
Annals of Clinical Biochemistry is also the official journal of NVKC (de Nederlandse Vereniging voor Klinische Chemie) and JSCC (Japan Society of Clinical Chemistry).