[Tangier disease--a "free radical"-associated disease. Results of HDL and anti-oxidant therapy with selenium and D-alpha tocopherol].
In a 26-year-old patient there have been benign enlargements of the lymphatic nodes and a splenomegaly since the end of the adolescence. In the 21st year of age the diagnosis of a Tangier disease was made. Allogenic HDL-rich serum fraction (COHN IV/1-fraction, prepared according to the modified method 6) infused under therapeutic aspect led to a prolonged increase of the serum total cholesterol and of the thrombocytes. The results pled for an activation of the reverse cholesterol transport. Excessively high malonic dialdehyde concentrations in the serum were relating to a "free radical"-associated metabolic defect, which was caused by the hypocholesterolaemia, the reduced transport capacity of vitamin E in the plasma and the nutrition poor in selenium and cholesterol, respectively. Under a nutritive antioxidant supplementation with sodium selenite and D-alpha-tocopherol a slight increase of the total cholesterol, of the thrombocytes as well as a normalization of the MDA values could be reached. The chronic oxidative stress appeared in the patient in a distinct lipofuscinosis of the skin and formations of naevus-cell naevi as an expression of massive denaturations of protein-lipids. In the Tangier disease we must reckon with an increased mutagenic effect of free radicals with an additional DNS repair capacity as well as an increased sensitivity to radical-generating cancerogenic xenobiotics.(ABSTRACT TRUNCATED AT 250 WORDS)
Kuklinski
B
B
Klinik für Innere Medizin, Universität Leipzig.
Zimmermann
R
R
Rühlmann
C
C
Nagel
R
R
Tessmann
D
D
D000328:Adult; D002784:Cholesterol / Q000097:blood; D008076:Cholesterol, HDL / Q000097:blood; D003131:Combined Modality Therapy; D005230:Fatty Acids, Nonesterified / Q000097:blood; D005609:Free Radicals; D006801:Humans; D015227:Lipid Peroxidation / Q000187:drug effects / Q000235:genetics; D008075:Lipoproteins, HDL / Q000008:administration & dosage*; D008297:Male; D008315:Malondialdehyde / Q000097:blood*; D012643:Selenium / Q000627:therapeutic use*; D018038:Sodium Selenite; D013631:Tangier Disease / Q000097:blood / Q000235:genetics / Q000628:therapy*; D014280:Triglycerides / Q000097:blood; D014810:Vitamin E / Q000008:administration & dosage*
D008076:Cholesterol, HDL; D005230:Fatty Acids, Nonesterified; D005609:Free Radicals; D008075:Lipoproteins, HDL; D014280:Triglycerides; D014810:Vitamin E; D008315:Malondialdehyde; D002784:Cholesterol; D012643:Selenium; D018038:Sodium Selenite
Tangier Disease--eine "freie-radikal"-assoziierte Erkrankung. Resultate einer HDL- und Antioxidantientherapie mit Selen und D-alpha-Tocopherol.