Capillary gas chromatographic determination of cholestanol/cholesterol ratio in biological fluids. Its potential usefulness for the follow-up of some liver diseases and its lack of specificity in diagnosing CTX (cerebrotendinous xanthomatosis).
The concentration ratios of cholestanol/cholesterol in biological materials (serum, cerebrospinal fluid and tendon biopsy) were determined using a capillary gas chromatographic method. The method was validated by gas chromatography-mass spectrometry. The ratio was determined in several groups of patients: (a) patients with cerebrotendinous xanthomatosis (in serum, cerebrospinal fluid and tendon biopsy), before and during chenodeoxycholic acid therapy, (b) patients receiving cholestyramine therapy (in serum), (c) patients suffering from various liver diseases (in serum) and (d) one patient before and after liver transplantation (in serum). It can be concluded that the cholestanol/cholesterol concentration ratio is a potentially useful parameter for monitoring liver diseases but is not specific for establishing the diagnosis of cerebrotendinous xanthomatosis.
D001928:Brain Diseases, Metabolic / Q000175:diagnosis* / Q000378:metabolism; D002635:Chenodeoxycholic Acid / Q000627:therapeutic use; D004083:Cholestanol / Q000032:analysis* / Q000097:blood / Q000134:cerebrospinal fluid; D002784:Cholesterol / Q000031:analogs & derivatives* / Q000032:analysis* / Q000097:blood / Q000134:cerebrospinal fluid; D002849:Chromatography, Gas; D008401:Gas Chromatography-Mass Spectrometry; D006801:Humans; D008107:Liver Diseases / Q000378:metabolism*; D014973:Xanthomatosis / Q000175:diagnosis* / Q000378:metabolism
D002635:Chenodeoxycholic Acid; D004083:Cholestanol; D002784:Cholesterol