Mitigation of portal fibrosis and cholestatic liver disease in ANKS6 ‐deficient livers by macrophage depletion

2022 ◽  
Vol 36 (2) ◽  
Author(s):  
Merlin Airik ◽  
Blake McCourt ◽  
Tugba Tastemel Ozturk ◽  
Amy B. Huynh ◽  
Xiaoyi Zhang ◽  
...  
Keyword(s):  
Liver Disease ◽  
Cholestatic Liver Disease ◽  
Portal Fibrosis ◽  
Macrophage Depletion

scholarly journals A218 A CASE REPORT OF PSEUDOHYPONATREMIA IN CHOLESTATIC LIVER DISEASE

2021 ◽  
Vol 4 (Supplement_1) ◽  
pp. 250-252
Author(s):  
M Shpoliansky ◽  
B Kamath
Keyword(s):  
Liver Disease ◽  
Liver Transplant ◽  
Measurement Techniques ◽  
Growth Failure ◽  
Urine Osmolality ◽  
Normal Serum ◽  
Plasma Sodium ◽  
Cholestatic Liver Disease ◽  
Aqueous Fraction ◽  
Direct Potentiometry

Abstract Background True hyponatremia in the setting of cholestatic liver disease may signify cirrhosis with fluid overload, and is therefore an ominous sign of deteriorating liver function. In pediatric liver transplant candidates, it is associated with increased waitlist mortality. Pseudohyponatremia however, is a falsely low measurement of plasma sodium when measured by indirect potentiometry. Pseudohyponatremia secondary to hypercholesterolemia is a phenomenon that occurs due to a reduced aqueous fraction of the plasma when levels of cholesterol or triglycerides are greatly elevated. Severe hypercholesterolemia due to Lipoprotein X accumulation may be the cause of pseudohyponatremia in biliary obstruction or cholestasis. Aims To describe a case of pseudohyponatremia secondary to hypercholesterolemia in an infant with Alagille syndrome (ALGS) and cholestatic liver disease. Methods This 7 month-old male with ALGS (confirmed JAGGED1 mutation) and severe cholestasis, failure to thrive, and pruritus, developed new-onset progressive hyponatremia as low as 121 mmol/L at an outside institution. He was therefore transferred to our center for liver transplant assessment due to concerns of progressive liver dysfunction and for management of the hyponatremia. Results Upon admission, the patient was jaundiced but euvolemic, with no evidence of ascites or peripheral edema. Laboratory work drawn at our institution showed conjugated bilirubin of 180 mmol/L, ALT 300 U/L, AST 250 U/L, and GGT 1200 U/L. INR was 1.1 and albumin of 35 g/L. The cholesterol was elevated above 16.8 mmol/L, with high triglycerides 2.68 mmol/L, and the serum appeared visibly lipemic. The sodium level was 138 mmol/L as measured by direct potentiometry due to the visible lipemia. The osmolality of 288 mmol/kg was normal with a normal osmolar gap. Urine osmolality and sodium were also normal. He underwent routine evaluation and was listed for a liver transplant due to the profound cholestasis and growth failure. Conclusions Pseudohyponatremia is an important entity to recognize when caring for patients with cholestatic liver disease and hyponatremia. Both direct potentiometry and indirect potentiometry are currently used for sodium testing in blood in biochemistry laboratories. These measurement techniques show good agreement as long as protein and lipid concentrations in blood are normal, however, hyperlipidemia is a well-recognized cause for error in sodium estimation. It is therefore imperative to evaluate apparent hyponatremia correctly, especially when the patient appears euvolemic clinically and by normal serum osmolality. In this clinical setting, pseudohyponatremia is the likely cause and a workup should be carried out to identify possible underlying etiologies, the most probable being hypercholesterolemia. Failure to recognize this phenomenon may lead to unnecessary and potentially harmful treatments and interventions. Funding Agencies None

scholarly journals Heme Oxygenase-1 Induction by Cobalt Protoporphyrin Ameliorates Cholestatic Liver Disease in a Xenobiotic-Induced Murine Model

2021 ◽  
Vol 22 (15) ◽  
pp. 8253
Author(s):  
Jung-Yeon Kim ◽  
Yongmin Choi ◽  
Jaechan Leem ◽  
Jeong Eun Song
Keyword(s):  
Oxidative Stress ◽  
Liver Disease ◽  
Heme Oxygenase ◽  
Murine Model ◽  
Liver Diseases ◽  
Transforming Growth Factor ◽  
Heme Oxygenase 1 ◽  
Cholestatic Liver Disease ◽  
Hepatocyte Apoptosis ◽  
Cobalt Protoporphyrin

Cholestatic liver diseases can progress to end-stage liver disease and reduce patients’ quality of life. Although their underlying mechanisms are still incompletely elucidated, oxidative stress is considered to be a key contributor to these diseases. Heme oxygenase-1 (HO-1) is a cytoprotective enzyme that displays antioxidant action. It has been found that this enzyme plays a protective role against various inflammatory diseases. However, the role of HO-1 in cholestatic liver diseases has not yet been investigated. Here, we examined whether pharmacological induction of HO-1 by cobalt protoporphyrin (CoPP) ameliorates cholestatic liver injury. To this end, a murine model of 3,5-diethoxycarbonyl-1,4-dihydrocollidine (DDC) diet feeding was used. Administration of CoPP ameliorated liver damage and cholestasis with HO-1 upregulation in DDC diet-fed mice. Induction of HO-1 by CoPP suppressed the DDC diet-induced oxidative stress and hepatocyte apoptosis. In addition, CoPP attenuated cytokine production and inflammatory cell infiltration. Furthermore, deposition of the extracellular matrix and expression of fibrosis-related genes after DDC feeding were also decreased by CoPP. HO-1 induction decreased the number of myofibroblasts and inhibited the transforming growth factor-β pathway. Altogether, these data suggest that the pharmacological induction of HO-1 ameliorates cholestatic liver disease by suppressing oxidative stress, hepatocyte apoptosis, and inflammation.

Evaluation of the role of bile acids and serotonin as markers of pruritus in children with chronic cholestatic liver disease

2021 ◽  
Author(s):  
Nehal El Koofy ◽  
Noha Yassin ◽  
Sawsan Okasha ◽  
Hany William ◽  
Wafaa Elakel ◽  
...  
Keyword(s):  
Liver Disease ◽  
Bile Acids ◽  
Cholestatic Liver Disease

P059 Transient cholestatic liver disease during a primary EBV infection: an unusual presentation

2018 ◽  
Vol 50 (4) ◽  
pp. e380
Author(s):  
S. Guercio Nuzio ◽  
E. Ardia ◽  
M.G. Carbone ◽  
G. Cimino ◽  
P. Coccorullo ◽  
...  
Keyword(s):  
Liver Disease ◽  
Unusual Presentation ◽  
Cholestatic Liver Disease ◽  
Ebv Infection

Effects of odevixibat on pruritus and bile acids in children with cholestatic liver disease: Phase 2 study

2021 ◽  
pp. 101751
Author(s):  
Ulrich Baumann ◽  
Ekkehard Sturm ◽  
Florence Lacaille ◽  
Emmanuel Gonzalès ◽  
Henrik Arnell ◽  
...  
Keyword(s):  
Liver Disease ◽  
Bile Acids ◽  
Cholestatic Liver Disease ◽  
Phase 2 ◽  
Phase 2 Study

Bile acid conjugation in early stage cholestatic liver disease before and during treatment with ursodeoxycholic acid

1996 ◽  
Vol 248 (2) ◽  
pp. 175-185 ◽  
Author(s):  
Mario Fracchia ◽  
Kenneth D.R. Setchell ◽  
Andrea Crosignani ◽  
Mauro Podda ◽  
Nancy O'Connell ◽  
...  
Keyword(s):  
Liver Disease ◽  
Bile Acid ◽  
Ursodeoxycholic Acid ◽  
Early Stage ◽  
Cholestatic Liver Disease
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