Multiple lipoprotein and electrolyte laboratory artifacts caused by lipoprotein X in obstructive biliary cholestasis secondary to pancreatic cancer

Sivakumar, T; Chaidarun, Sushela; Lee, Hong Kee; Cervinski, Mark A.; Comi, Richard J.

doi:10.1016/j.jacl.2011.04.004

Cited by 29 publications

(26 citation statements)

References 13 publications

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“…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]. Severe cholesterol elevations result in artifactual electrolyte abnormalities, xanthomas and hyperviscosity [18,19]. In familial LCAT deficiency, LpX is thought to contribute to renal disease due to deposition of LpX in glomeruli [20,21].…”

Section: Discussionmentioning

confidence: 99%

“…There is a paucity of information to guide the management of LpX and its complications. A limited number of case reports have demonstrated the management of cholestatic LpX using several techniques, including mechanical release of biliary obstruction [18], modification of other known obstructive causes (e.g., allograft rejection) [23], lipid apheresis [24] and liver transplantation [25]. For familial LCAT deficiency, low fat, low cal orie diets with high vegetable consumption may reduce kidney damage [26], and low fat diets coupled with angiotensin II receptor blockers may improve renal abnormalities [27].…”

Section: Discussionmentioning

confidence: 99%

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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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Section: Discussionmentioning

confidence: 99%

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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“…However, these cases described patients with cholestasis secondary to either bone marrow transplant with graft versus host disease, primary biliary cirrhosis, hepatitis C or drugs. Only one case reported a patient with cholestasis secondary to a structural abnormality in the case of pancreatic cancer 13. Our case is unique from the other published cases for several reasons.…”

Section: Discussionmentioning

confidence: 63%

Pseudohyponatraemia secondary to hyperlipidaemia in obstructive jaundice

et al. 2017

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A 44-year-old man with uncontrolled diabetes and chronic pancreatitis presented with abdominal pain, jaundice and unintentional weight loss. Laboratory investigations were significant for hyponatraemia, an obstructive pattern of liver enzymes. Imaging was consistent with intrahepatic and extrahepatic biliary obstruction, and endoscopic evaluation revealed a long common bile duct stricture. Intravascular volume depletion, beer potomania and syndrome of inappropriate antidiuretic hormone (with concern for biliary or pancreatic malignancy) were considered in the work-up for the aetiology of the hyponatraemia. After 4 days of conventional treatment, hyponatraemia persisted. Lipid panel obtained revealed very high levels of total cholesterol. The patient underwent a successful biliary diversion and reconstruction surgery. Follow-up after 3 months showed a clinically stable patient with resolution of elevated liver enzymes, hyperlipidaemia and hyponatraemia. We illustrate this rare case of hyponatraemia secondary to hyperlipidaemia in obstructive biliary cholestasis. It is important for physicians to thoroughly investigate the aetiology of hyponatraemia at its onset.

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“…It is often encountered as a component of genetic disorders like familial hypercholesterolemia but less commonly as a result of biliary obstruction [5]. Obstructive causes reported in the literature to produce hypercholesterolemia include pancreatic carcinoma and primary biliary cirrhosis, but it has not been known to be associated with non-Hodgkin's lymphoma [6, 7]. NHLs are a heterogeneous group of lymphoproliferative disorders typically affecting nodal and extranodal sites and producing a myriad of nonspecific clinical features.…”

Section: Discussionmentioning

confidence: 99%

“…Lipoprotein-Y is another lipoprotein rich in triglyceride and fractionating in the LDL range which has also been identified in biliary obstruction [9]. These abnormal lipoproteins in cholestasis contribute to increased cardiovascular risk and their presence in serum can produce false laboratory results leading to delays in medical management [6, 7]. The precise mechanism by which cholestasis leads to hypercholesterolemia is not clear but several theories abound, including one informed by the findings of Fredrickson et al in rats where CBD ligation led to an increase in hepatic cholesterol synthesis [10].…”

Section: Discussionmentioning

confidence: 99%

Severe Hypercholesterolemia: A Unique Presentation of Non-Hodgkin’s Lymphoma in a Patient with Neurofibromatosis Type 1

Chudy-Onwugaje

Anyadike

Tsirlin

et al. 2014

Case Reports in Gastrointestinal Medicine

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We report a case of non-Hodgkin's lymphoma (NHL) with an unusual initial manifestation as severe hypercholesterolemia and obstructive jaundice in a patient with neurofibromatosis type 1 (NF 1). NHL should be considered in the evaluation of obstructive jaundice alone or in combination with severe hypercholesterolemia. Relief of biliary obstruction led to the resolution of hypercholesterolemia in our 59-year-old male patient, followed by doxorubicin-based chemotherapy for the underlying lymphoma. NF 1 is a genetic condition that results from a defect in a tumor-suppressor gene and it is likely that this led to the development of NHL in our patient. It is important that clinicians are familiar with the gastrointestinal manifestations of NF 1, especially its association with intra-abdominal malignancies, when treating patients with a personal or family history. To the best of our knowledge, this is the first case of NHL presenting initially as severe hypercholesterolemia and it is also one of the few instances where NHL has been reported in association with NF 1.

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Multiple lipoprotein and electrolyte laboratory artifacts caused by lipoprotein X in obstructive biliary cholestasis secondary to pancreatic cancer

Cited by 29 publications

References 13 publications

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

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

Pseudohyponatraemia secondary to hyperlipidaemia in obstructive jaundice

Severe Hypercholesterolemia: A Unique Presentation of Non-Hodgkin’s Lymphoma in a Patient with Neurofibromatosis Type 1

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