A Woman with Primary Biliary Cirrhosis and Hyponatremia

Farooqi, Midhat S.; Hashim, Ibrahim

doi:10.1373/clinchem.2014.229773

Cited by 4 publications

(7 citation statements)

References 9 publications

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“…Moreover, LpX can interfere with direct LDL-C assays, the degree of interference being dependent on the method used (18). There is also evidence that LpX can interfere with the measurements of apolipoprotein E phenotype analysis (19), serum total protein (20), and electrolytes, causing pseudohyponatremia, pseudohypokalemia, and pseudohypochloremia, in cases where indirect ion-selective electrodes are used (21)(22)(23)(24).…”

Section: Discussionmentioning

confidence: 99%

“…In the case of our patient, the fraction with mobility lower than b was not detected in two electropherograms, whereas only a subtle smearing band with g mobility was observed in the third one, performed one year later ( Figure 1A, lane 2B). In most studies, co-migration of LpX with bmobility lipoproteins (LDL) was observed, with eventually subtle reverse migration in the LDL region (14,22,23,25,26). Phatlhane et al (3), in the case of a patient with LpX, observed intense staining in the b area, with minor cathodic migration and a slight green tinge.…”

Section: Discussionmentioning

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: Discussionmentioning

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 was confirmed before and after the first TPE then disappeared after the ninth TPE (Figure 2), along with the normalization of the patient's lipid panel. However, lipoprotein EP is not 100% sensitive for the detection of Lp‐X and may not always be as clearly resolved as in our case 1,11,37,43,44 . Thus, the lipoprotein EP results obtained in a patient with severe hypercholesterolemia should be analyzed with extreme caution and clinicians should be aware of the limitations.…”

Section: Discussionmentioning

confidence: 70%

“…However, lipoprotein EP is not 100% sensitive for the detection of Lp-X and may not always be as clearly resolved as in our case. 1,11,37,43,44 Thus, the lipoprotein EP results obtained in a patient with severe hypercholesterolemia should be analyzed with extreme caution and clinicians should be aware of the limitations.…”

Section: Discussionmentioning

confidence: 99%

Use of therapeutic plasma exchange to remove lipoprotein X in a patient with vanishing bile duct syndrome presenting with cholestasis, pseudohyponatremia, and hypercholesterolemia: A case report and review of literature

Jung,

Nelson,

Lin

2024

J of Clinical Apheresis

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IntroductionLipoprotein X (Lp‐X) is an abnormal lipoprotein found in multiple disease conditions, including liver dysfunction and cholestasis. High Lp‐X concentrations can interfere with some laboratory testing that may result in spurious results. The detection of Lp‐X can be challenging, and there is currently a lack of consensus regarding the management of Lp‐X other than treating the underlying disease.Case PresentationA 42‐year‐old female with Hodgkin's lymphoma treated with dexamethasone, high dose cytarabine and cisplatin and vanishing bile duct syndrome confirmed by liver biopsy presented with cholestasis, pseudohyponatremia (sodium, 113 mmol/L; reference range 136–146 mmL/L; serum osmolality, 303 mOsm/kg), and hypercholesterolemia (> 2800 mg/dL, reference range < 200 mg/dL). Lp‐X was confirmed by lipoprotein electrophoresis (EP). Although she did not manifest any specific signs or symptoms, therapeutic plasma exchange (TPE) was initiated based on laboratory findings of extreme hypercholesterolemia, spuriously abnormal serum sodium, and HDL values, and the potential for short‐ and long‐term sequelae such as hyperviscosity syndrome, xanthoma, and neuropathy. During the hospitalization, she was treated with four 1.0 plasma volume TPE over 6 days using 5% albumin for replacement fluid. After the first TPE, total cholesterol (TC) decreased to 383 mg/dL and sodium was measured at 131 mmol/L. The patient was transitioned into outpatient maintenance TPE to eliminate the potential of Lp‐X reappearance while the underlying disease was treated. Serial follow‐up laboratory testing with lipoprotein EP showed the disappearance of Lp‐X after nine TPEs over a 10‐week period.Literature ReviewThere are seven and four case reports of Lp‐X treated with TPE and lipoprotein apheresis (LA), respectively. While all previous case reports showed a reduction in TC levels, none had monitored the disappearance of Lp‐X after completing a course of therapeutic apheresis.ConclusionClinicians should have a heightened suspicion for the presence of abnormal Lp‐X in patients with cholestasis, hypercholesterolemia, and pseudohyponatremia. Once Lp‐X is confirmed by lipoprotein EP, TPE should be initiated to reduce TC level and remove abnormal Lp‐X. Most LA techniques are not expected to be beneficial since Lp‐X lacks apolipoprotein B. Therefore, we suggest that inpatient course of TPE be performed every other day until serum sodium, TC and HDL levels become normalized. Outpatient maintenance TPE may also be considered to keep Lp‐X levels low while the underlying disease is treated. Serum sodium, TC, and HDL levels should be monitored while on maintenance TPE.

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“…HCV-or hepatitis B virus (HBV)-related nephrotic syndrome and glycogen storage disease may be accompanied by mixed dyslipidemia, whereas primary biliary cirrhosis or other cholestatic liver diseases may be associated with marked hypercholesterolemia attributed to accumulation of lipoprotein-X. Consequently, the above-mentioned liver diseases as well as the administration of intravenous immunoglobulin (e.g., in liver transplant patients) should be considered as potential causes of spurious hyponatremia [10,[12][13][14][15][16][17].…”

Section: Other Causes Of Hyponatremia In Patients With Liver Diseasesmentioning

confidence: 99%

Hyponatremia in patients with liver diseases: not just a cirrhosis-induced hemodynamic compromise

et al. 2016

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Hyponatremia (Na(+) <135 mmol/l) is the most common electrolyte disorder. Cirrhosis represents a rather frequent cause of hyponatremia mainly due to systemic and splanchnic vasodilation resulting in decreased effective arterial blood volume, which leads to excessive non-osmotic secretion of antidiuretic hormone. However, hyponatremia of multifactorial origin may be seen in patients with liver diseases. The review focuses on the factors and pathogenetic mechanisms of decreased sodium levels other than the hemodynamic compromise of cirrhosis in patients with liver diseases. The mechanisms and causal or contributing role of pseudohyponatremia, hyperglycemia, infections, drugs and toxins as well as of endocrine disorders, renal failure and cardiac disease in patients with liver disease are meticulously discussed. Hyponatremia of multifactorial origin is frequently observed in patients with liver diseases, and special efforts should be made to delineate the underlying causative and precipitating factors as well as the risk factors of the osmotic demyelination syndrome in order to properly manage this serious electrolyte disorder and avoid treatment pitfalls.

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A Woman with Primary Biliary Cirrhosis and Hyponatremia

Cited by 4 publications

References 9 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

Use of therapeutic plasma exchange to remove lipoprotein X in a patient with vanishing bile duct syndrome presenting with cholestasis, pseudohyponatremia, and hypercholesterolemia: A case report and review of literature

Hyponatremia in patients with liver diseases: not just a cirrhosis-induced hemodynamic compromise

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