Lipoprotein X: clinical implications

Crook, M

doi:10.1177/0004563213478804

Cited by 19 publications

(19 citation statements)

References 30 publications

(44 reference statements)

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“…Thus, the agarose electrophoresis results obtained in routine laboratory testing concerning patients with severe hypercholesterolaemia should be analysed very carefully. Eventually, other electrophoretic techniques or methods, such as ultracentrifugation, nuclear magnetic resonance spectroscopy, and immunological analysis, may be useful for LpX detection (6). Unfortunately, these methods are usually available only in specialised laboratories, reducing the potential for their use in routine practice.…”

Section: Discussionmentioning

confidence: 99%

“…However, severe hypercholesterolaemia may also be unrelated to increased LDL-C, resulting instead from the presence of an abnormal lipoprotein fractionlipoprotein X (LpX) (2)(3)(4)(5). LpX is most frequently detected in patients with cholestatic liver disease (6,7) as well as in those with lecithin:cholesterol acyltransferase (LCAT) deficiency, hepatic lipase (HL) deficiency, and after intravenous fat emulsion infusion (6). The LpX was not proved to result in coronary artery disease development (8,9).…”

Section: Introductionmentioning

confidence: 99%

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Detection of lipoprotein X (LPX) – a challenge in patients with severe hypercholesterolaemia

Ćwiklińska

Mickiewicz

Kowalski

et al. 2019

Journal of Medical Biochemistry

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Summary Background Lipoprotein X (LpX) is an abnormal lipoprotein fraction, which can be detected in patients with severe hypercholesterolaemia and cholestatic liver disease. LpX is composed largely of phospholipid and free cholesterol, with small amounts of triglyceride, cholesteryl ester and protein. There are no widely available methods for direct measurement of LpX in routine laboratory practice. We present the heterogeneity of clinical and laboratory manifestations of the presence of LpX, a phenomenon which hinders LpX detection. Methods The study was conducted on a 26-year-old female after liver transplantation (LTx) with severely elevated total cholesterol (TC) of 38 mmol/L and increased cholestatic liver enzymes. TC, free cholesterol (FC), cholesteryl esters (CE), triglycerides, phospholipids, HDL-C, LDL-C, and apolipoproteins AI and B were measured. TC/apoB and FC:CE ratios were calculated. Lipoprotein electrophoresis was performed using a commercially available kit and laboratory-prepared agarose gel. Results Commercially available electrophoresis failed to demonstrate the presence of LpX. Laboratory-prepared gel clearly revealed the presence of lipoproteins with γ mobility, characteristic of LpX. The TC/apoB ratio was elevated and the CE level was reduced, confirming the presence of LpX. Regular lipoprotein apheresis was applied as the method of choice in LpX disease and a bridge to reLTx due to chronic liver insufficiency. Conclusions The detection of LpX is crucial as it may influence the method of treatment. As routinely available biochemical laboratory tests do not always indicate the presence of LpX, in severe hypercholesterolaemia with cholestasis, any discrepancy between electrophoresis and biochemical tests should raise suspicions of LpX disease.

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Section: Discussionmentioning

confidence: 99%

Section: Introductionmentioning

confidence: 99%

Detection of lipoprotein X (LPX) – a challenge in patients with severe hypercholesterolaemia

Ćwiklińska

Mickiewicz

Kowalski

et al. 2019

Journal of Medical Biochemistry

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“…Lp-X induces foam cell formation in human-derived macrophages. 5 Both Lp-X and xanthomas resolve within approximately 2 weeks of the relief of the biliary obstruction.…”

Section: Discussionmentioning

confidence: 99%

Vanishing bile duct syndrome with eruptive xanthomas

Cotter¹,

Biswas

Rao

et al. 2019

Clin Exp Dermatol

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“…Lp-X is a low-density lipoprotein whose presence in the serum is extremely specific for cholestasis. 1 Phospholipids and unesterified cholesterol constitute the bulk of Lp-X molecule. Its lipid composition is similar to lipids found in normal bile but differs significantly from normal plasma lipoproteins.…”

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confidence: 99%

“…In cholestasis, bile lipoprotein refluxes into the plasma pool and binds to albumin to form Lp-X. 1 Cholestasis is also associated with changes in 11b-hydroxysteroid dehydrogenase, which contributes to electrolyte abnormalities similar to a mineralocorticoid excess state. 2 Resolution of hypercholesterolemia coincides with improvement in cholestasis, as occurred with our patient (total cholesterol 1 year following: 121 mg/dl with a normal electrolyte panel).…”

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confidence: 99%

The Case | Hyponatremia in a patient with obstructive jaundice

Ravella

Lefavour

Carayannopoulos

et al. 2015

Kidney International

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A 40-year-old man with a past medical history of autoimmune pancreatitis, lymphoplasmacytic sclerosing cholangitis, biliary stricture with stent placement, hyperlipidemia, and diabetes mellitus presented with fever for 2 days. Review of systems was negative for nausea, vomiting, poor oral intake, and diarrhea. He denied shortness of breath. Pertinent physical exam findings included fever, jaundice, icteric sclerae, moist oral mucosa, non-tender, non-distended abdomen, and absence of edema in lower extremities. No hepatomegaly was noted on exam.Laboratory tests revealed a serum sodium of 119 mEq/l, potassium of 3.3 mEq/l, chloride of 87 mEq/l, and a serum osmolality of 297 mOsm/kg. Total cholesterol was 2109 mg/dl. His lipid panel, however, demonstrated a low-density lipoprotein level of 68 mg/dl, high-density lipoprotein level of 36 mg/dl, and triglycerides of 299 mg/dl. Electrolytes on a repeat blood sample were checked simultaneously using the indirect ion-selective electrode (ISE) method and the direct ISE method. These results are depicted in the table below. Patient's laboratory dataElectrolyte values Indirect ion-selective electrode method Direct ion-selective electrode method Sodium

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Lipoprotein X: clinical implications

Cited by 19 publications

References 30 publications

Detection of lipoprotein X (LPX) – a challenge in patients with severe hypercholesterolaemia

Detection of lipoprotein X (LPX) – a challenge in patients with severe hypercholesterolaemia

Vanishing bile duct syndrome with eruptive xanthomas

The Case | Hyponatremia in a patient with obstructive jaundice

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