ORIGINAL ARTICLE

Postprandial hypertriglyceridaemia in patients with Tangier disease

G Kolovou¹, D Daskalova¹, K Anagnostopoulou¹, I Hoursalas¹, V Voudris¹, D P Mikhailidis² and D V Cokkinos¹

¹ Cardiology Department, Onassis Cardiac Surgery Centre, 17674 Athens, Greece
² Department of Clinical Biochemistry (Vascular Disease Prevention Clinics), Royal Free Hospital, Royal Free and University College Medical School, London NW3 2Q9, UK

Correspondence to:
Correspondence to:
Dr G D Kolovou
Onassis Cardiac Surgery Centre, 356 Sygrou Avenue, 17674 Athens, Greece; g\|[lowbar]\|kolovou{at}yahoo.co.uk

Background: Tangier disease (TD) is the phenotypic expression of rare familial syndromes with mutations in the ABCA1 transporter. TD results in extremely low high density lipoprotein (HDL) cholesterol and reduced low density lipoprotein cholesterol, with normal or mildly increased fasting triglyceride (TG) concentrations. Although there is a close relation between HDL cholesterol values and atherogenesis, the risk of coronary artery disease is variable in TD. Raised fasting or postprandial TG values frequently accompany low HDL cholesterol and can add to the risk of a vascular event.

Aims: To investigate the postprandial TG response in TD.

Patients and methods: Five patients (three homozygotes (HTD) and two heterozygotes (hTD)) from one family were studied. One was defined by DNA analysis as homozygous for a new mutation (C2033A) resulting in truncation of the ABCA1 protein. Their TG concentrations were measured before and four, six, and eight hours after a standardised fat load and compared with a control group.

Results: Two patients with HTD had high fasting TG concentrations. The third patient with HTD, the two with hTD, and the control group had TG concentrations within the reference range. The patients with HTD had increased postprandial peak TG values when compared with those with hTD and controls.

Conclusion: Patients with HTD, with or without fasting hypertriglyceridaemia, may have an increased TG response to a fatty meal. The small number of patients does not allow definitive conclusions to be made. However, postprandial hypertriglyceridaemia could be a reason why some patients with TD develop premature atherosclerosis.

Keywords: postprandial hypertriglyceridaemia; Tangier disease; triglycerides; ABCA1

Abbreviations: ABCA1, ATP binding cassette A1 transporter; AUC, area under the curve; HDL, high density lipoprotein; hTD, heterozygous Tangier disease; HTD, homozygous Tangier disease; LDL, low density lipoprotein; TD, Tangier disease; TG, triglyceride; VLDL, very low density lipoprotein

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