Hepatobiliary Complications of Chronic Intestinal Failure

Intestinal failure-associated liver disease is a multifactorial process that may occur in patients with chronic intestinal failure on long-term home parenteral nutrition. A very short gut, the lack of enteral feeding, recurrent sepsis, and parenteral overfeeding are major risk factors. Histologic changes include steatosis, steatohepatitis, cholestasis, fibrosis, and cirrhosis. Chronic cholestasis is common, but does not always progress to fibrosis and/or cirrhosis. Preventing harmful factors may dramatically decrease the risk of intestinal failure-associated liver disease. Advanced liver disease is an indication for intestinal and/or multivisceral transplantation. Biliary stone formation is frequent and mainly due to a lack of enteral feeding.
Source: Gastroenterology Clinics of North America - Category: Gastroenterology Authors: Source Type: research

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Biliary atresia is a serious pediatric liver disease. It is among the leading causes of newborn cholestasis, the foremost reason for cirrhosis and liver-related death in children, and the most frequent indication for liver transplant in the pediatric population. The condition results from an idiopathic, rapidly progressive, fibrosclerosing obliterative injury to large bile ducts during the first months of life. Although not an inherited disease, biliary atresia is a rare orphan liver disease that occurs in 1:15  000 to 1:20 000 live births in North America and Western Europe, with the highest incidence rates in Asia (1...
Source: JAMA - Category: General Medicine Source Type: research
AbstractA complex relationship exists between thyroid and liver in health and disease. Liver plays an essential physiological role in thyroid hormone activation and inactivation, transport, and metabolism. Conversely, thyroid hormones affect activities of hepatocytes and hepatic metabolism. Serum liver enzyme abnormalities observed in hypothyroidism may be related to impaired lipid metabolism, hepatic steatosis or hypothyroidism-induced myopathy. Severe hypothyroidism may have biochemical and clinical features, such as hyperammonemia and ascites, mimicking those of liver failure. Liver function tests are frequently abnorma...
Source: Journal of Endocrinological Investigation - Category: Endocrinology Source Type: research
We describe the case of a 50-year-old female patient with class II obesity and primary biliary cirrhosis, evaluated for mixed dyslipidemia with poor control (statins and fibrates were being administered) as well as abnormal liver tests. The investigation carried out revealed primary auto-immune hypothyroidism. After normalisation of thyroid function by treatment with levothyroxine, as well as suspending the administration of statins and fibrates, there was an improvement in the lipid profile, although hypercholesterolemia continued. During this time, the patient was diagnosed with diabetes and she re-commenced statin thera...
Source: Acta Medica Portuguesa - Category: General Medicine Tags: Acta Med Port Source Type: research
In this study, we developed binary classification models to predict drug-induced cholestasis, cirrhosis, hepatitis, and steatosis based on the structure of drugs and their metabolites. DILI-positive data was obtained from post-market reports of drugs and DILI-negative data from DILIrank, a database curated by the Food and Drug Administration (FDA). Support vector machine (SVM) and random forest (RF) were used in developing models with nine fingerprints and one 2D molecular descriptor calculated from drug (152 DILI-positives and 102 DILI-negatives) and drug metabolite (192 DILI-positives and 126 DILI-negatives) structures. ...
Source: Frontiers in Pharmacology - Category: Drugs & Pharmacology Source Type: research
CONCLUSION: This novel TJP2 variant is associated with PFIC4 causing severe liver disease with cirrhosis and primary liver cancer in adolescents/adults. PMID: 32089630 [PubMed - in process]
Source: World Journal of Gastroenterology : WJG - Category: Gastroenterology Authors: Tags: World J Gastroenterol Source Type: research
Abstract Cholestasis is a result of obstruction of the biliary tracts. It is a common cause of liver pathology after exposure to toxic xenobiotics and during numerous other liver diseases. Accumulation of bile acids in the liver is thought to be a major driver of liver injury during cholestasis and can lead to eventual liver fibrosis and cirrhosis. As such, current therapy in the field of chronic liver diseases with prominent cholestasis relies heavily on increasing choleresis to limit accumulation of bile acids. Many of these same diseases also present with autoimmunity before the onset of cholestasis though, ind...
Source: Food and Chemical Toxicology - Category: Food Science Authors: Tags: Food Chem Toxicol Source Type: research
AbstractFamilial lecithin:cholesterol acyltransferase (LCAT) deficiency (FLD) is a rare genetic disease characterized by low HDL ‐C levels, low plasma cholesterol esterification, and the formation of Lipoprotein‐X (Lp‐X), an abnormal cholesterol‐rich lipoprotein particle. LCAT deficiency causes corneal opacities, normochromic normocytic anemia, and progressive renal disease due to Lp‐X deposition in the glomeruli. R ecombinant LCAT is being investigated as a potential therapy for this disorder. Several hepatic disorders, namely primary biliary cirrhosis, primary sclerosing cholangitis, cholestatic liver disease, ...
Source: Pharmacology Research and Perspectives - Category: Drugs & Pharmacology Authors: Tags: ORIGINAL ARTICLE Source Type: research
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Source: Phytomedicine - Category: Drugs & Pharmacology Source Type: research
Authors: Sticova E, Jirsa M Abstract ATP-binding cassette (ABC) subfamily B member 4 (ABCB4), also known as multidrug resistance protein 3 (MDR3), encoded by ABCB4, is involved in biliary phospholipid secretion, protecting hepatobiliary system from deleterious detergent and lithogenic properties of the bile. ABCB4 mutations altering canalicular ABCB4 protein function and expression may have variable clinical presentation and predispose to several human liver diseases. Well-established phenotypes of ABCB4 deficit are: progressive familial intrahepatic cholestasis type 3, gallbladder disease 1 (syn. low phospholipid ...
Source: Annals of Hepatology - Category: Gastroenterology Tags: Ann Hepatol Source Type: research
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Source: Archives of Medical Science - Category: General Medicine Tags: Arch Med Sci Source Type: research
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