We report on an infant girl with severe RSH or Smith-Lemli-Opitz syndrome with hyperbilirubinemia. The infant died at age 2 months. Sterol analysis of liver and brain tissues showed marked elevations of 7-dehydrocholesterol with decreased levels of cholesterol. Immunocytochemical analysis demonstrat
Low-density lipoprotein-apheresis in two patients with extremely elevated lipoprotein (a) levels
β Scribed by R. Bambauer; R. Schiel; J. Klinkmann; R. Latza
- Publisher
- John Wiley and Sons
- Year
- 1996
- Tongue
- English
- Weight
- 187 KB
- Volume
- 11
- Category
- Article
- ISSN
- 0733-2459
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β¦ Synopsis
Two male Caucasian patients (36 and 42 years old) with heterozygous familial hypercholesterolemia and extremely elevated lipoprotein Lp(a) concentrations, resistant to diet and lipid-lowering drugs, were treated with low-density lipoprotein (LDL)-apheresis for 55 months (liposorber system, Kaneka@, Japan) and 15 months (immunoadsorption system, special Lp(a) columns, Lipopakm, Pocard, Russia). Lp(a) dropped on average by 50%, total cholesterol by 27%, LDL-cholesterol by 42%. triglycerides by 43%, and the fibrinogen concentration by 16%. o 1996 Wiley-Liss, lnc
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Low density lipoprotein-cholesterol (LDL-c) concentration measured by a homogeneous enzymatic assay was reported to correlate well with the modified b-quantification assay, especially in samples with high triglyceride (TG) concentration. In this study, we evaluated a homogeneous enzymatic assay, Cho
A subset of patients with familial hypercholesterolemia (FH) have an inadequate lipid-lowering response to diet and drug treatment and should be considered for low-density lipoprotein (LDL)-apheresis therapy. This procedure selectively removes apolipoprotein B-containing particles [LDL, very-low-den