scholarly journals Plasma cholesterol level determines in vivo prion propagation

2017 ◽  
Vol 58 (10) ◽  
pp. 1950-1961 ◽  
Author(s):  
Véronique Perrier ◽  
Thibaud Imberdis ◽  
Pierre-André Lafon ◽  
Marina Cefis ◽  
Yunyun Wang ◽  
...  
1987 ◽  
Vol 117 (9) ◽  
pp. 1527-1531 ◽  
Author(s):  
Shigeru Saeki ◽  
Hiroyuki Nishikawa ◽  
Shuhachi Kiriyama

1989 ◽  
Vol 53 (12) ◽  
pp. 3127-3132 ◽  
Author(s):  
Masamichi KOSEKI ◽  
Keisuke TSUJI ◽  
Yasue NAKAGAWA ◽  
Masako KAWAMURA ◽  
Tomio ICHIKAWA ◽  
...  

1988 ◽  
Vol 52 (1) ◽  
pp. 71-76
Author(s):  
Hisanao TAKEUCHI ◽  
Miki FUJISHIRO ◽  
Yasushi WATANABE ◽  
Keiichiro MURAMATSU

PEDIATRICS ◽  
1990 ◽  
Vol 86 (6) ◽  
pp. 977-981
Author(s):  
Peter F. Belamarich ◽  
Richard J. Deckelbaum ◽  
Thomas J. Starc ◽  
Beth Ellen Dobrin ◽  
G. Stephen Tint ◽  
...  

In this report, an 11-year-old boy with diffuse use tendinous and tuberous xanthomatosis and a plasma sterol concentration of 555 mg/dL, consisting primarily of cholesterol, is described. Three months after changing from an unrestricted diet to a cholesterol-lowering diet, his plasma sterol concentration decreased to 221 mg/dL. Because of the degree and rapidity of his response to diet, sitosterolemia was suspected. According to results of capillary gas-liquid chromatography of his plasma sterols, there was a sitosterol concentration of 31.3 mg/dL (normal <1.0 g/dL), establishing the diagnosis of sitosterolemia. Addition of cholestyramine therpy (8 g/d) to a low sterol diet further lowered his plasma sterol concentration to 73 mg/dL and led to complete regression of all tuberous xanthomata. Tendinous xanthomata regressed at a slower rate. These findings show that the diagnosis of sitosterolemia should be suspected in severely hypercholesterolemic children (total cholesterol >400 mg/dL) whose plasma cholesterol level is highly responsive to dietary manipulation. The rapid and sustained lowering of plasma cholesterol and regression of xanthomata after treatment with diet and cholestyramine suggest that sitosterolemia is a treatable cause of premature atherosclerosis.


Sign in / Sign up

Export Citation Format

Share Document