Occurrence of an abnormal lipoprotein in patients with liver disease

Ross, A.; Murphy, G. M.; Wilkinson, Philip A.; Mills, G. L.; Sherlock, S.

doi:10.1136/gut.11.12.1035

Cited by 30 publications

(19 citation statements)

References 9 publications

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“…There was no peak in the position of LP-X with agarose column chromatography, and no particles like LP-X were seen on electron microscopy. Many other patients with 'cholestasis' have been reported to have had no detectable LP-X in the plasma (Magnani & Alaupovic, 1976;Vergani, Pietrogrande & Grondona, 1973;Ross, Murphy, Wilkinson, Mills & Sherlock, 1970;Ritland, Blomhoff, Elgjo & Gjone, 1973;Mordasini, Berthold, Schlumpf, Keller & Riva, 1975). Seidel (1976) has also claimed that the con centration of LP-X in plasma does not correlate with LCAT activity.…”

Section: Discussionmentioning

confidence: 93%

Lecithin—Cholesterol Acyltransferase and the Lipoprotein Abnormalities of Obstructive Jaundice

et al. 1978

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1. We have studied the plasma lipoprotein abnormalities in obstructive jaundice to test the hypothesis that the abnormalities would correlate with plasma lecithin--cholesterol acyltransferase activity. 2. Very-low-density lipoproteins (VLDL) were normal in composition and had a normal pre-beta electrophoretic mobility when lecithin--cholesterol acyltransferase activity was high. When it was low VLDL had abnormal composition and ran in the beta position. 3. With high lecithin--cholesterol acyltransferase activity, low-density lipoprotein (LDL) was normal and cholestatic LDL (LP-X) was not found. With low lecithin--cholesterol acyltransferase activity up to three LDL fractions were found: (i) large triglyceride-rich particles, (ii) LP-X and (iii) a triglyceride-rich cholesteryl ester-poor particle of normal dimensions. 4. High-density lipoprotein (HDL) concentrations correlated with lecithin--cholesterol acyltransferase activity and HDL were normal in composition and electron-microscopic appearance when the activity was high. When lecithin--cholesterol acyltransferase activity was low HDL was abnormal in composition and 'stacked discs' were seen on electron microscopy as well as normal spherical particles. 5. These results suggest that low lecithin-cholesterol acyltransferase activity may cause at least some of the lipoprotein changes of obstructive jaundice.

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Section: Discussionmentioning

confidence: 93%

Lecithin—Cholesterol Acyltransferase and the Lipoprotein Abnormalities of Obstructive Jaundice

et al. 1978

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“…LpX elevations are quite common in patients with cholestatic liver diseases, occurring in up to 45% of such patients [16]. While up to 41% of patients with PSC have been reported to have TC levels greater than the 95th percentile, extreme elevations are less frequently encountered but generate significant therapeutic uncertainty [17].…”

Section: Discussionmentioning

confidence: 99%

Management of lipoprotein X and its complications in a patient with primary sclerosing cholangitis

Brandt

Regnier

Leung

et al. 2015

Clinical Lipidology

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Lipoprotein X (LpX) is an abnormal lipoprotein found in conditions such as lecithin:cholesterol acyltransferase deficiency and cholestatic states (e.g., primary biliary cirrhosis and primary sclerosing cholangitis). Management of severe hypercholesterolemia due to LpX with drugs and physical removal methods is not well established in the literature. A case is discussed of a 51-year-old woman who presented with multiple electrolyte abnormalities, xanthomas and neuropathy found to be secondary to LpX in the setting of primary sclerosing cholangitis. This case highlights that oral medications, including statins, may be insufficient to normalize lipid levels or improve clinical symptoms of LpX and presents therapeutic plasma exchange as a safe and effective therapeutic option to treat the morbid sequela of LpX hyperlipidemia.

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“…Since complete obstruc tion of extrahepatic bile ducts is proved in infants with biliary atresia, it is no wonder that all the infants with the illness are Lp-X positive in high concentrations. However, in adults with extrahepatic biliary obstruction, the proportion of patients positive for Lp-X varies from 65% to 100% (Ross et al 1970;Vergani et al 1973;Seidel et al 1973a). Such differences may reflect incomplete obstruction, in various degrees, of the extrahepatic biliary tract in patients examined.…”

Section: Discussionmentioning

confidence: 99%

“…Negative reaction to Lp-X has never been reported in infants with extrahepatic biliary atresia having no hepatic decompensation (Poley et al 1973), while in those with neonatal hepatitis syndrome, including paucity of the intrahepatic bile duct, Lp-X has been demonstrated to be undetectable, or much lower in concentration than in those with biliary atresia (Poley et al 1973; Magani and Alaupovic 1976). However, there is some controversy on the usefulness 210 Y. Tazawa and T. Konno of the Lp-X test in differential diagnosis of obstructive jaundice (Ross et al 1970;Vergani et al 1973). In this study, we determined serum Lp-X levels in infants with neonatal hepatitis or biliary atresia, and found semiquantitative determina tion of serum Lp-X valuable in differential diagnosis of these diseases, especially in their early stages.…”

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confidence: 98%

Semiquantitative Assay of Serum Lipoprotein-X in Differential Diagnosis of Neonatal Hepatitis and Congenital Biliary Atresia

Tazawa

Konno

1980

Tohoku J. Exp. Med.

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Occurrence of an abnormal lipoprotein in patients with liver disease

Cited by 30 publications

References 9 publications

Lecithin—Cholesterol Acyltransferase and the Lipoprotein Abnormalities of Obstructive Jaundice

Lecithin—Cholesterol Acyltransferase and the Lipoprotein Abnormalities of Obstructive Jaundice

Management of lipoprotein X and its complications in a patient with primary sclerosing cholangitis

Semiquantitative Assay of Serum Lipoprotein-X in Differential Diagnosis of Neonatal Hepatitis and Congenital Biliary Atresia

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