Cerebrotendinous xanthomatosis (CTX) is a rare familial sterol storage disease, causing multiple xanthomas in tendons and the brain. The underlying biochemical defect is a lack of the hepatic mitochondrial cholesterol 27-hydroxylase involved in the normal biosynthesis of bile acid, resulting in reduced biosynthesis of chenodeoxycholic acid (CDCA). It has been reported that administration of CDCA to CTX patients improves neurological disorders and xanthomas of the Achilles tendon. The present study investigated the effect of CDCA on the mechanism of cholesterol accumulation in macrophages, the major cells in xanthoma. The LDL from the patients in this study was significantly more susceptible to oxidative modification than normal LDL, and supplement therapy with CDCA resulted in an improvement in the susceptibility to oxidative modification. In the incubation of CDCA with plasma, 13% of the CDCA added to serum was recovered in the LDL fraction. In addition, supplementation with CDCA enhanced cholesteryl ester transfer protein (CETP) activity and reduced high-densitylipoprotein cholesterol levels in the plasma. This evidence suggests that the multiple xanthomas observed in CTX may be induced by increased oxidized LDL and the low activity of CETP, both of which are caused by a lack of CDCA. J Atheroscler Thromb, 2004; 11: 167-172.

Aim: We aimed to determine the relationship of red cell and platelet distribution widths with the onset of acute myocardial infarction, to enable the early detection and prevention of acute myocardial infarction.Methods: Red cell and platelet distribution widths were retrospectively determined in 46 patients with stable angina pectoris and 140 patients with acute myocardial infarction who were brought to the emergency department of our institution. Red cell and platelet distribution widths were determined with an automatic blood cell analyzer, and the results were compared between the acute myocardial infarction and angina pectoris groups.Results: Both red cell and platelet distribution width values obtained at onset were significantly higher in the acute myocardial infarction group than in the angina pectoris group (red cell distribution widths, 46.4 AE 0.51% versus 44.5 AE 0.59%; mean difference À1.91 [95% confidence interval (CI), À3.79 to À0.34]; platelet distribution widths, 12.1 AE 0.22 fL versus 11.1 AE 0.17 fL; mean difference À1.03 [95% CI, À1.58 to À0.47]). The red cell distribution widths before onset was not different between the acute myocardial infarction and angina pectoris groups; however, the platelet distribution widths before onset was higher in the acute myocardial infarction group (red cell distribution widths, 46.5 AE 0.85% versus 45.9 AE 0.59%; mean difference À0.71 [95% CI, À2.74 to 1.30]; platelet distribution widths, 11.4 AE 0.39 fL versus 10.6 AE 0.21 fL; mean difference À0.83 [95% CI, À1.66 to 0.11]). Conclusion:Red cell distribution widths and especially platelet distribution widths may contribute to the early detection of acute myocardial infarction.

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