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Bile acid synthesis disorder

ACOX2 deficiency: A disorder of bile acid synthesis with transaminase elevation, liver fibrosis, ataxia, and cognitive impairment Acyl CoA Oxidase 2 (ACOX2) encodes branched-chain acyl-CoA oxidase, a peroxisomal enzyme believed to be involved in the metabolism of branched-chain fatty acids and bile acid intermediates Chapter 33 - Disorders of bile acid synthesis and metabolism. from Section IV - Metabolic liver disease. By Kenneth D. R. Setchell, Department of Pathology and Laboratory Medicine, Cincinnati Children's Hospital Medical Center and Department of Pediatrics, University of Cincinnati College of Medicine, Cincinnati, OH, USA Disorders of bile acid synthesis are responsible for approximately 2 to 3% of cases of liver disease in infants and children.221 At least six specific enzymatic defects related to modification of the cholesterol nucleus or side chain have been characterized Treatment of bile acid synthesis disorders due to single enzyme defects Adjunctive treatment of peroxisomal disorders including Zellweger spectrum disorders in patients who show signs and symptoms of liver disease, steatorrhea (fatty stools), or complications from decreased fat soluble vitamins absorption (A, D, E, K

Bile acid synthesis disorders (BASDs) are a group of rare, inherited metabolic disorders caused by defects in the enzymes that are involved in making bile acids. Bile acids promote the flow and excretion of bile, excess cholesterol and waste, and help the intestines absorb fat and fat-soluble vitamins. When the body is unable to produce normal. Bile acid synthesis disorder accounted for 2.7% of the screened cases of infantile cholestasis in Saudi Arabia. Three novel mutations causing bile acid synthesis disorder are reported. Serum total bile acid concentration is a sensitive and specific screening tool for bile acid synthesis disorder among infants with cholestasis FDA-approved indication: Treatment of bile acid synthesis disorders due to single enzyme defects and as adjunctive treatment of peroxisomal disorders including Zellweger spectrum disorders in patients who exhibit manifestations of liver disease, steatorrhea or complications from decreased fat soluble vitamin absorption Bile acid synthesis disorders occur when the body lacks the enzymes needed to process cholesterol from the liver into cholic acid. This can result in a build up of potentially harmful by-products in the liver and other organs, and malnutrition from lack of absorption of fat and fat-soluble vitamins Bile Acid Synthesis Disorders due to single enzyme defects are a group of rare genetic disorders that affect the enzymes responsible for making normal, healthy bile acids. A mutation, or change, in one's DNA prevents formation of normal bile acids and causes abnormal, toxic bile acids to build up

Disorders of bile acid synthesis - PubMe

Bile Acid Synthesis Disorder

Description. Congenital bile acid synthesis defect type 2 is a disorder characterized by cholestasis, a condition that impairs the production and release of a digestive fluid called bile from liver cells. Bile is used during digestion to absorb fats and fat-soluble vitamins, such as vitamins A, D, E, and K Bile Acid Synthesis might seem like a vast term, but the disorder creates a bigger set of problems on people. They are a group of metabolic disorders that are characterized based on the defects found in the part of creation of Bile Acids. These acids that are found in the liver plays a crucial role in the entire body Abstract. Two inborn errors of metabolism affect the modifications of the cholesterol nucleus in both major pathways for bile acid synthesis: 3β-hydroxy- Δ 5-C 27-steroiddehydrogenase (3 β-dehydrogenase) deficiency and Δ 4-3-oxosteroid-5 β-reductase (5 β-reductase) deficiency.These disorders produce cholestatic liver disease and malabsorption of fat and fat-soluble vitamins The conversion of cholesterol to bile acids and the secretion of cholesterol into bile represent the major routes for elimination of excess cholesterol from the body. Thus, individuals with disorders of bile acid synthesis can present with liver disease due to impaired bile secretion (cholestasis), with gallstones, steatorrhea, symptoms of fat. Bile Acid Synthesis Disorders by Mass Spectrometry Urinary Bile Acid Profile by FAB-MS Fractionated & Total Serum Bile Acids by LC-MS ____/ _____/ _____Ursodeoxycholic acid can mask detection of bile acid synthetic disorders, the patient should be temporarily taken off URSO® or ACTIGALL® for 5 DAYS before sample collection

What is bile acid synthesis disorders (BASD)? CHOLBAM

Toxic bile acids may cause inflammation, apoptosis, and cell death. On the other hand, bile acid-activated nuclear and GPCR signaling protects against inflammation in liver, intestine, and macrophages. Disorders in bile acid metabolism cause cholestatic liver diseases, dyslipidemia, fatty liver diseases, cardiovascular diseases, and diabetes Biology of Bile Acids. Two aspects of bile acid metabolism are relevant to their role in neurodegenerative disorders, bile acids that circulate systemically and that are synthesized by neurons. Circulating bile acids are largely synthesized from cholesterol in the liver ( Prawitt et al., 2011 ). Ingestion of food causes bile acid secretion from. Bile acid synthesis disorders should be suspected in refractory rickets in infancy, particularly in a clinical setting of FTT, even in the absence of substantial abnormalities in liver-function tests. Vitamin D-deficiency rickets is biochemically characterized by a low-serum 25-hydroxy vitamin D (25OHD) level, normal- or low-serum calcium, low. branched-chain acyl-CoA oxidase; bile acid metabolism; peroxisomal disorder; whole-exome sequencing; idiopathic liver disease; Despite major advances in Mendelian genetics, the role in normal human biology and the impact of mutation of >75% of the 20,000 protein-coding genes in the human genome remain to be determined ().One example of an unmet medical need is idiopathic liver disease, which. Tests aid diagnosis of an under-recognized disorder — Bile acid malabsorption (BAM) is an underdiagnosed cause of chronic diarrhea, due mainly to limited availability of the gold standard SeHCAT retention test. That situation will likely change as new, simpler tests for the disorder are developed and validated

Congenital bile acid synthesis defect type 1: MedlinePlus

Also known as: BASD, bile acid synthesis disorders, bile acid synthesis and metabolism defects. Bile acids are chemicals in the liver that play several important roles in the body, including helping with the breakdown of fat and removing cholesterol from the body With regard to bile acid biosynthesis, several comprehensive reviews of the subject have been published [1-4]; therefore, this chapter provides only an overview of the pathways of bile acid synthesis and metabolism and describes specific inborn errors in bile acid synthesis that have been identified Individuals with disorders of bile acid synthesis may exhibit liver disease due to impaired bile secretion (cholestasis), along with symptoms of fat-soluble vitamin deficiency, gallstones, steatorrhea due to fat malabsorption, and the accumulation of cholesterol in tissues leading to cutaneous xanthomata and atherosclerosis

Overview of bile acid sythesis disorders - Rarecholestasi

  1. Inborn errors of primary bile acid synthesis are rare inherited autosomal recessive disorders. The most frequent defects are the 3β-Δ 5-hydroxy-C 27-steroid oxidoreductase (3β-HSD) deficiency (OMIM 607765) which is due to mutations in HSD3B7; and to a lesser extent the Δ 4 -3-oxosteroid-5β-reductase (Δ 4 -3-oxo-R) deficiency (OMIM 235555) due to mutations in AKR1D1 [1,2,3,4,5,6,7]
  2. Bile Acid Synthesis Disorders. When food enters the small intestine, a series of hormonal and nerve signals triggers the gallbladder to contract and the sphincter of Oddi to relax and open. Bile then flows from the gallbladder into the small intestine to mix with food contents and perform its digestive functions
  3. ishing the production of the toxic metabolites likely causing liver and neurologic dysfunction. This study provides th

ACOX2 deficiency: A disorder of bile acid synthesis with

The FDA has approved oral cholic acid (Cholbam - Retrophin) for treatment of children and adults with bile acid synthesis disorders caused by single enzyme defects and for adjunctive treatment of peroxisomal disorders such as Zellweger spectrum disorders in patients who have liver disease, steatorrhea, or complications from fat-soluble vitamin malabsorption World map of Bile Acid Synthesis Disorders. Find people with Bile Acid Synthesis Disorders through the map. Connect with them and share experiences. Join the Bile Acid Synthesis Disorders community. View map Production. Bile acid synthesis occurs in liver cells, which synthesize primary bile acids (cholic acid and chenodeoxycholic acid in humans) via cytochrome P450-mediated oxidation of cholesterol in a multi-step process. Approximately 600 mg of bile salts are synthesized daily to replace bile acids lost in the feces, although, as described below, much larger amounts are secreted, reabsorbed in. Once those disorders are excluded, inborn errors of bile acid synthesis or bile acid transport must be considered. The serum level of gamma glutamyl transpeptidase (GGT) is a helpful clue. In some forms of these disorders, GGT levels, which would be expected to be markedly elevated in correlation with elevated conjugated bilirubin levels, are. 16p11.2. Bile acid synthesis defect, congenital, 1. 607765. Autosomal recessive. 3. HSD3B7. 607764. TEXT. A number sign (#) is used with this entry because this disorder of bile acid synthesis, referred to here as CBAS1, can be caused by homozygous or compound heterozygous mutation in the gene encoding 3-beta-hydroxy-delta-5-C27-steroid.

From GHR Congenital bile acid synthesis defect type 1 is a disorder characterized by cholestasis, a condition that impairs the production and release of a digestive fluid called bile from liver cells. Bile is used during digestion to absorb fats and fat-soluble vitamins, such as vitamins A, D, E, and K. People with congenital bile acid synthesis defect type 1 cannot produce (synthesize) bile. CHOLBAM is a treatment for infants, children and adults who have a rare condition called bile acid synthesis disorders. This happens when infants are born with defects that result in low bile acid. For individuals with bile acid synthesis disorders, CA, the main bile acid found in ox bile, is the primary bile acid used as a therapy. [24] In addition to protecting hepatocytes, activation of FXR by bile acids induces genes involved in the different phases of detoxification This review focuses on these genetic disorders of bile acid transport. Bile Acid Synthesis and Transport. Under normal physiological conditions, bile acids are shuttled between the liver and small intestine through a process termed the enterohepatic circulation (Fig. 1)

Disorders of bile acid synthesis and metabolism (Chapter

This can lead to vitamin metabolism disorders and hormone synthesis disorders. In addition, mycotoxins can lead to bile acid synthesis excretions disorder, detoxification dysfunctions, free radical metabolism and immune disorders (retinal endothelium phagocytic system), oxidative stress and inflammatory response E78.70 is a billable/specific ICD-10-CM code that can be used to indicate a diagnosis for reimbursement purposes. Short description: Disorder of bile acid and cholesterol metabolism, unsp. The 2021 edition of ICD-10-CM E78.70 became effective on October 1, 2020. This is the American ICD-10-CM version of E78.70 - other international versions of. A number sign (#) is used with this entry because of evidence that this congenital defect in bile acid synthesis, referred to here as CBAS4, is caused by homozygous mutation in the gene encoding alpha-methylacyl-CoA racemase (AMACR; 604489) on chromosome 5p13. For a general description and a discussion of genetic heterogeneity of congenital bile acid synthesis defects, see 607765 In inborn errors of bile acid synthesis, accumulation of toxic bile acid intermediates cause neonatal cholestasis, fibrosis, and hepatitis. 1 Obstruction of bile flow by stones, tumors, or inflammation of the intrahepatic small bile duct or extrahepatic common bile duct increases toxic bile acids and intermediates in the liver, leading to.

Below is a list of common medications used to treat or reduce the symptoms of deficiency of bile acids in bile acid synthesis disorder. Follow the links to read common uses, side effects, dosage. Disorders in bile acid synthesis and metabolism can be broadly classified as primary or secondary. Primary enzyme defects involve congenital deficiencies in enzymes responsible for catalyzing key reactions in the synthesis of cholic and chenodeoxy-cholic acids. The primary defects reported thus far include cholesterol 7-hydroxylas

Bile Acid Synthesis - an overview ScienceDirect Topic

  1. Impaired regulation of bile acid synthesis by FGF19 has been proposed to be a causative factor in the common disorder known as primary bile acid diarrhea or idiopathic bile acid malabsorption . Furthermore, obeticholic acid, a semi-synthetic modified bile acid and a potent FXR agonist, stimulated FGF19 production and improved symptoms in these.
  2. This bile acid test for peroxisomal disorders measures concentrations of C27 bile acids, which are diagnostic markers for peroxisomal biogenesis disorders such as Zellweger syndrome and single enzyme defects of bile acid synthesis such as D-bifunctional protein deficiency and alpha methyl-CoA racemase deficiency
  3. Inborn errors of bile acid synthesis are newly recognized disorders that may cause the phenotypic appearance of neonatal hepatitis or neonatal cholestasis .Liver disease may be caused by the failure of primary bile acid synthesis, the accumulation of potentially hepatotoxic atypical bile acid metabolites, or both
  4. Bile acid synthesis disorder (BASD) The member has a documented diagnosis or a bile acid synthesis disorder due to a single enzyme defect (see Appendix 1). Peroxisomal disorder (PD) The member has a documented diagnosis of a peroxisomal disorder (see Appendix 2); an
  5. s, but that is not the only role they play in the body. The gut microbiota metabolizes bile acids, and in return, bile acids play a role as regulators of the gut microbiome. Additionally, they act as signaling hormones to affect metabolism and inflammation, and associated disorders
  6. An inherited metabolic disorder that affects the metabolism of the lipids. Representative examples include gaucher disease, tay-sachs disease, and niemann-pick disease Condition in which there is a deviation or interruption in the processing of lipids in the body: synthesis, absorption, transport, storage, and utilizatio
  7. Endogenous bile acids including cholic acid enhance bile flow, provide the physiologic feedback inhibition of bile acid synthesis, and regulate bile acid circulation (1). Regulatory Status FDA-approved indications: Cholbam (cholic acid) is a bile acid indicated for: (1) 1. Treatment of bile acid synthesis disorders due to single enzyme defects.

Treating Bile Acid Synthesis Disorders with CHOLBAM

Bile acid synthesis disorders are a group of highly symptomatic conditions that affect approximately one in every 50,000-70,000 live births in the U.S. These diseases are caused by genetic errors. Cholesterol supersaturated bile formation is based primarily upon a relatively reduced bile salt synthesis, and somehow, this relates to fatty acid composition in bile phospholipids in the aspect of the degree of fatty acyl chain unsaturation. 5 Cholesterol is present in bile salt micelles and phospholipid particulate species, namely vesicles. Bile Acid Synthesis Disorders Studies . This trial; Search. The REPLACE Registry for Cholbam® (Cholic Acid) a study on Bile Acid Synthesis Disorders. Summary Location at San Francisco, California and other locations Dates. study started July 2017. estimated completion July 2039. Description

Ho Ching shares story of 3-yr-old with a rare genetic

Congenital defects of bile acid synthesis are rare disorders that cause progressive liver dysfunction. Prolonged neonatal hyperinsulism (PNH) is a separate entity that leads to persistent hypoglycemia secondary to stress. We present a 4-month-old infant who presented with liver failure secondary to a bile acid synthesis defect Congenital Bile Acid Synthesis Defect, Type 1 may not be preventable, since it is a genetic disorder. Genetic testing of the expecting parents (and related family members) and prenatal diagnosis (molecular testing of the fetus during pregnancy) may help in understanding the risks better during pregnanc FDA's approval of a new drug application for cholic acid oral capsules marks the first time children and adults with certain rare bile acid synthesis disorders will be able to receive FDA-sanctioned therapy for the condition, the agency announced on March 17

One of the intermediate products in the bile acid synthesis pathway is 7α-hydoxy-4-cholesten-3-one (C4). This is readily measurable in peripheral blood samples and its serum concentrations have been shown to accurately reflect the activity of hepatic cholesterol 7α hydroxylase (CYP7A1), the first enzyme and the rate-limiting step in bile acid synthesis pathway. 1 Bile acid malabsorption is a condition that happens when your colon doesn't reabsorb bile acids, which your body uses to help digest foods. This can lead to diarrhea and frequently needing to. We will first describe bile acid synthesis and enterohepatic circulation, followed by a discussion of disorders causing bile acid malabsorption (BAM) including their diagnosis and treatment. Bile Acid Synthesis Bile acids are produced in the liver as end products of cholesterol metabolism. Bile acid synthesis occur Fortunately, there's help for parents of children with bile acid synthesis disorders (BASD). BASD is a rare metabolic disorder that impacts the production of bile and hinders the liver's ability to break down and process fats and vitamins. It can be treated with replacement therapy or a liver transplant BILE ACID METABOLISM Bile acids are a metabolic product of cholesterol and are synthesized in the liver via cytochrome p450 activity in he-patocytes with multiple metab olic pathways that facilitate this process (2). The first is the classic pathway of bile acid synthesis where cholesterol is metabolized by cholestero

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Bile Acid Synthesis Disorders - Kaiser Permanent

  1. s, such as vita
  2. istration on March 17, 2015 Another bile acid, ursodeoxycholic acid, is a drug approved by the FDA but not for this reason
  3. This paper will summarize recent advances in our understanding of bile acid signaling regulation of glucose and lipid metabolism and the potentials of developing novel therapeutic strategies that target bile acid metabolism for the treatment of metabolic disorders. 2. Bile Acid Synthesis

Video: Bile Acid Synthesis Disorders Travere Therapeutic

Disorders in bile acid synthesis and metabolism can be broadly classified as primary or secondary. Primary enzyme defects involve congenital deficiencies in en-zymes responsible for catalyzing key reactions in the synthesis of cholic and chenodeoxycholic acids. Th The efficacy of Cholbam for the treatment of patients with bile acid synthesis disorders due to single enzyme defects was assessed in an uncontrolled trial involving 50 patients treated over an 18. This is a serum test for the measurement C27 bile acids, which are a diagnostic marker for peroxisomal biogenesis disorders and single enzyme defects of bile acid synthesis including D-bifunctional protein deficiency and alpha methyl CoA racemase deficiency. This test can also be used for monitoring of treatment efficacy. Testing Algorithm This replaces the bile acids lost in the stools and represents approximately 5% of the bile acid pool, as the enterohepatic circulation is 95% efficient. The synthesis and secretion of bile acids, together with hepatic cholesterol secretion into bile, represents the major pathway for the elimination of cholesterol from the body

Cerebrotendinous xanthomatosis (CTX) is an autosomal recessive lipid storage disorder caused by mutations in the CYP27A1 gene creating dysfunctional bile acid metabolism, afflicting patients with a progressive disarray of symptoms including but not limited to: ataxia, dementia, epilepsy, tendon xanthomas and cataracts Multiple sclerosis (MS) is an inflammatory demyelinating disorder of the CNS. Bile acids are cholesterol metabolites that can signal through receptors on cells throughout the body, including in the CNS and the immune system. Whether bile acid metabolism is abnormal in MS is unknown

Increases in serum C27 bile acids are seen in patients with peroxisomal biogenesis disorders such as Zellweger syndrome or single enzyme defects of bile acid synthesis such as D-bifunctional protein deficiency and alpha methyl CoA racemaces. Total bile acids are metabolized in the liver and can serve as a marker for normal liver function Valid for Submission. E78.79 is a billable diagnosis code used to specify a medical diagnosis of other disorders of bile acid and cholesterol metabolism. The code E78.79 is valid during the fiscal year 2021 from October 01, 2020 through September 30, 2021 for the submission of HIPAA-covered transactions Bile acids are end-products of cholesterol metabolism that have multiple biological functions besides aiding lipid absorption in the gut1. Primary bile acids are mainly produced in the liver, but other tissues are capable of bile acid synthesis through an alternative acidic pathway More information: Central anorexigenic actions of bile acids are mediated by TGR5, Nature Metabolism (2021). DOI: 10.1038/s42255-021-00398-4 Journal information: Nature Metabolis

Members of the gastrointestinal tract (GIT) microbiota initiate bile acid metabolism via a critical first step catalyzed by bile salt hydrolases (BSHs) [].These enzymes hydrolyze and deconjugate the glycine or taurine from the sterol core of the primary bile acids, cholic acid (CA), and chenodeoxycholic acid (CDCA) ().Deconjugated bile acids can subsequently undergo a variety of microbiota. Prior to today's approval, patients with these rare bile acid synthesis disorders had no approved treatment options. The efficacy of Cholbam for the treatment of patients with bile acid synthesis disorders due to single enzyme defects was assessed in an uncontrolled trial involving 50 patients treated over an 18 year period Bile acid synthesis disorders (BASD) are extremely rare, genetic, metabolic conditions that exhibit manifestations of liver disease, steatorrhea, and complications from decreased fat soluble vitamin absorption. Individuals with BASD lack the enzymes needed to synthesize cholic acid. If untreated, these individuals fail to grow and can develop life

We found no guidance from NICE about bile acid replacement therapy for inborn errors of bile acid synthesis. The indication and epidemiology Inborn errors of primary bile acid synthesis are rare congenital genetic disorders which affect enzymes catalysing reactions in the synthesis of the primary bile acids, cholic an The code E78.70 is VALID for claim submission. Code Classification: Endocrine, nutritional and metabolic diseases (E00-E90) Metabolic disorders (E70-E88) Disorders of lipoprotein metabolism and other lipidemias (E78) E78.70 Disorder of bile acid and cholesterol metabolism, unsp. Code Version: 2020 ICD-10-CM The Nuclear Receptor for Bile Acids, FXR. The farnesoid X receptor (FXR; also known as the bile acid receptor or BAR; gene symbol NR1H4) is a member of the nuclear receptor family of transcription factors.FXR functions as the chief sensor of intracellular levels of bile acids (the end products of cholesterol catabolism) and the main executor of bile acid-induced transcriptional programmes Bile acid synthesis disorders due to peroxisomal disorders (PDs), including Zellweger spectrum disorders and ALL of the following: • Individual has peroxisomal disorders with at least one of the following criteria: o An abnormal urinary bile acid analysis consistent with a Zellweger spectrum disorder per b Excessive fecal bile acid (BA) loss causes symptoms in a large proportion of people diagnosed with irritable bowel syndrome with diarrhea, a common functional bowel disorder. This BA diarrhea (BAD) results from increased hepatic synthesis of BAs, with impaired negative feedback regulation by the ileal hormone fibroblast growth factor 19 (FGF19)

Bile acid malabsorption syndrome type I (disorder) ICD-10-CM Alphabetical Index References for 'E78.79 - Other disorders of bile acid and cholesterol metabolism' The ICD-10-CM Alphabetical Index links the below-listed medical terms to the ICD code E78.79 ICD-10-CM Code for Disorders of bile acid and cholesterol metabolism E78.7 ICD-10 code E78.7 for Disorders of bile acid and cholesterol metabolism is a medical classification as listed by WHO under the range - Endocrine, nutritional and metabolic diseases E78.79 is a valid billable ICD-10 diagnosis code for Other disorders of bile acid and cholesterol metabolism.It is found in the 2021 version of the ICD-10 Clinical Modification (CM) and can be used in all HIPAA-covered transactions from Oct 01, 2020 - Sep 30, 2021. ↓ See below for any exclusions, inclusions or special notation E78.70 is a valid billable ICD-10 diagnosis code for Disorder of bile acid and cholesterol metabolism, unspecified.It is found in the 2021 version of the ICD-10 Clinical Modification (CM) and can be used in all HIPAA-covered transactions from Oct 01, 2020 - Sep 30, 2021. ↓ See below for any exclusions, inclusions or special notation Expression of the genes involved in cholesterol transport and bile-acid synthesis in the liver. (A) mRNA expression of Cyp7a1, Cyp8b1, Cyp7b1, and Cyp27a1 (n = 8). (B) Protein expression (left panel) and quantification (middle and right panel) of Cyp7a1 and Cyp7b1 (n = 6). (C) mRNA expression of Fgfr4 and β-klotho (n = 8)

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Bile Acid Synthesis Disorders in Arabs: A 10-year

In bile acid synthesis disorders due to SEDs in the biosynthetic pathway, and in PDs including Zellweger spectrum disorders, deficiency of primary bile acids leads to unregulated accumulation of intermediate bile acids and cholestasis. Bile acids facilitate fat digestion and absorption by forming mixed micelles, and facilitate absorption of fat. Both procedures cause a block in the reabsorption of bile acids. Due to release of feedback regulation from a reduction in bile acids returning to the liver, the conversion of cholesterol into bile acids is greatly enhanced; this in turn causes a drain on body cholesterol Alterations in gut microorganisms found in the appendix of people with Parkinson's disease were associated with elevated digestive bile acids that may play a role in disease development, a study has revealed.. The discovery suggests targeting microbial-derived bile acids as a new approach to treatment, or using these acids as biomarkers to monitor early disease progression and response to. 3. The Functions of Bile Acids. Digestion: As discussed above and in our web page on triacylglycerol metabolism, a major function of bile acids is to act as powerful detergents or emulsifying agents in the intestines to aid the hydrolysis of dietary triacylglycerols and other lipids and the subsequent absorption of unesterified fatty acids, cholesterol, monoacylglycerols, fat-soluble vitamins.

Bilirubin - WikipediaEnterohepatic Circulation