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Dubin Johnson syndrome

Dubin-Johnson syndrome is a rare, autosomal recessive, benign disorder that causes an isolated increase of conjugated bilirubin in the serum. Classically, the condition causes a black liver due to the deposition of a pigment similar to melanin Dubin Johnson syndrome (DJS) is a rare, benign genetic liver disorder. It is inherited in an autosomal recessive pattern and is characterized by buildup of bilirubin, which is normally excreted by the liver into the bile

Dubin-Johnson Syndrome | gutsandgrowthDubin-Johnson Syndrome - The Medical Biochemistry Page

Dubin-Johnson syndrome - Wikipedi

Dubin Johnson Syndrome - NORD (National Organization for

Rotor’s Syndrome – Labpedia

Dubin-Johnson-Sprinz Nelson Syndrome. Occurs in both sexes (although males are affected about 1.5 times more often than females) and in all nationalities and races. The highest prevalence in the general population (1:1300) is found in Iranian Jews Dubin-Johnson syndrome (DJS) is a disorder passed down through families (inherited). In this condition, you may have mild jaundice throughout life

A benign, autosomally recessive inherited hyperbilirubinemia characterized by the presence of a dark pigment in the centrilobular region of the liver cells. There is a functional defect in biliary excretion of bilirubin, cholephilic dyes, and porphyrins The primary defect in Dubin-Johnson syndrome (DJS) is impaired transport of glucuronides and other anionic conjugates from the hepatocytes into bile, due to mutation of multidrug resistance-associated protein 2 (MRP2). MRP2 is an integral membrane glycoprotein expressed mainly in the canalicular (apical) membrane of hepatocytes where it. Dubin-Johnson syndrome (DJS) is a rare autosomal recessive disorder characterized by a defect in biliary excretion of conjugated bilirubin Dubin-Johnson syndrome (DJ) is benign, looks like mild viral hepatitis. It is characterized by mild recurrent jaundice with hepatomegaly

Dubin-Johnson syndrome: MedlinePlus Genetic

  1. Dubin-Johnson syndrome is a benign disorder and does not require any specific therapy, although patients should be warned that pregnancy, oral contraceptive use, and intercurrent illness can..
  2. Dubin-Johnson syndrome is a rare genetic liver disorder characterized by elevated levels of bilirubin in blood (hyperbilirubinemia). Persistent yellowing of the skin, mucous membranes, and whites of the eyes (jaundice) is usually the only symptom and, in most cases, does not appear before puberty

Dubin-Johnson syndrome (Concept Id: C0022350

Dubin-Johnson syndrome is a rare hereditary autosomal recessive disorder that was first described in 1954. 1 Incidence of Dubin-Johnson syndrome in the general population is unclear, it appears to be slightly more common in males and its incidence varies widely with ethnicity and geography. 2 Age at onset varies widely, between 10 weeks and 76. Dubin-Johnson syndrome is a rare inherited disorder of bilirubin that is characterized by buildup of bilirubin in the bloodstream (hyperbilirubinemia), jaundice, which is a yellowing of the skin and whites of the eyes. In most affected people jaundice appears during adolescence or early adulthood. Jaundice is typically the only feature of Dubin. Dubin-Johnson syndrome This rare autosomal recessive disorder involves impaired excretion of bilirubin glucuronides. It is usually diagnosed by liver biopsy ; the liver is deeply pigmented as a result of an intracellular melanin-like substance but is otherwise histologically normal Dubin-Johnson syndrome. Etiology: defective multidrug resistance-associated protein 2 → impaired excretion of conjugated bilirubin from the hepatocytes into the bile canaliculi; Inheritance: autosomal recessive; Clinical features. Mild to moderate jaundice. Onset often occurs during adolescenc

Dubin Johnson Syndrome - PubMe

  1. Dubin-Johnson Syndrome (DJS) is a rare genetic disorder, which is characterized by mild jaundice that is present throughout the life of the individual. The basic defect in this condition is that the liver is not able to transport bilirubin out of it. Some individuals may have liver enlargement, liver tenderness, weakness, nausea or vomiting
  2. 41 g/L Dipstick urinalysis No bilirubinuria He has recently complained of coryzal symptoms and.
  3. al cavity include the liver, gallbladder, stomach, small intestine and large intestine
  4. Dubin-Johnson Syndrome. Dubin-Johnson Syndrome is the result of a hereditary mutation in the protein which actively transports conjugated bilirubin across the hepatocyte membrane and into the bile canaliculi. Because the actual enzymatic process of bilirubin conjugation is not affected (See: Heme Degradation) bilirubin is conjugated normally in.
  5. Dubin-Johnson Syndrome. 1 Department of Gastroenterology and Endoscopy, Medical School and University Hospital Dr. Jose E. Gonzalez, Monterrey, Mexico. Correspondence: José Alberto González-González. E-mail: jalbertogastro@gmail.com

[The Dubin-Johnson syndrome: case report and review of

Elevated direct bilirubin. No evidence of biliary tract obstructive disease process. Normal levels of AST, ALT, GGT and alkaline phosphatase. These levels can be elevated for other reasons, but. What is Dubin Johnson syndrome? Dubin Johnson syndrome is a rare genetic disorder of liver which is passed down through families (inherited) A person who has been affected has mild jaundice throughout life

Síndrome de Dubin-Johnson - EcuRed

Dubin-Johnson syndrome is a benign condition, which results from a hereditary defect in biliary secretion of bilirubin pigments, and manifests as recurrent jaundice with conjugated hyperbilirubinemia. The defect is due to the absence of the canalicular protein MRP2 located on chromosome Dubin-Johnson syndrome (DJ) is benign, looks like mild viral hepatitis. It is characterized by mild recurrent jaundice with hepatomegaly. Pathophysiology. This is a rare genetic autosomal recessive disorder. This is due to the inability to transport bilirubin-diglucuronide through the parenchymal hepatic cells into bile canaliculi Although Dubin-Johnson syndrome and Rotor syndrome have similar phenotypes (mild, fluctuating elevation of both unconjugated and conjugated bilirubin in plasma), in Dubin-Johnson syndrome biliary excretion of organic anions, except bile acids, is impaired, while Rotor syndrome is a disorder of hepatic storage

Dubin-Johnson Syndrome: Practice Essentials, Background

  1. Dubin Johnson syndrome (DJS) is an inherited disorder caused by defect of ABCC2 gene which codes protein production that substantial to transport the conjugated bilirubin out from liver. It presented intermittent jaundice and usually come in young adult rarely in elderly. This rare disorder was not easy to diagnose, often misdiagnose
  2. Search for: Rare Disease Profiles; 5 Facts; Rare IQ; Rare Mystery;
  3. Dubin-Johnson syndrome (DJS) mutation of the canalicular multiple drug-resistance protein (MRP2) gene leads to impaired biliary excretion of bilirubin glucuronides. grossly black liver. Rotor syndrome. associated with mutations of the SLCO1B1 and SLCO1B3 genes leading to defect in the hepatic storage of conjugated bilirubin
  4. Dubin-Johnson syndrome is an autosomal recessive disorder that belongs to a family of disorders that result as a consequence of defects in the metabolism and/or excretion of bilirubin. Bilirubin is the by-product of the catabolism of heme. Normal disposition of bilirubin involves its transport to the liver where it is conjugated to the sugar.
  5. Background: Dubin-Johnson syndrome (DJS) is a benign autosomal recessive liver disease involving mutations of the ABCC2 gene. It is characterized by chronic or intermittent conjugated.
  6. Although Dubin-Johnson syndrome may be exacerbated by pregnancy, infection, birth control pills, and alcohol, it is generally benign and has good prognosis. Onset may be in adolescence, adulthood, and in some cases, infancy. Although it may occur in all ethnicities, it appears more commonly in Iranian, Iraqi, and Moroccan Jews

Dubin-Johnson syndrome - Symptoms, diagnosis and treatment

Dubin-Johnson syndrome. Defect is in the multiple organic anion transport protein 2 (MRP2) of the bile canaliculus, causing impaired hepatocyte excretion of conjugated bilirubin into bile. A variable degree of impairment in uptake and conjugation complicates the clinical picture Dubin-Johnson Syndrome is very rare and it is caused solely by genetics meaning that it is an inherited condition. Dubin-Johnson syndrome is an autosomal recessive inheritance meaning that a person might only acquire Dubin-Johnson issue when both parents enjoy the defective gene that causes this condition Dubin-Johnson syndrome (DJS) is a disorder passed down through families (inherited). In this condition, you may have mild jaundice throughout life. Causes. DJS is a very rare genetic disorder. In order to inherit the condition, a child must get a copy of the defective gene from both parents

Four inherited hyperbilirubinemias: Crigler-Najjar

Dubin-Johnson syndrome (DJS) is caused by the mutation of the ABCC2 gene, which leads to the functional defect of its encoded MPR2 protein. This causes endogenous and exogenous anionic. Dubin-Johnson syndrome Du·bin-John·son syndrome (dooґb ə n jonґs ə n) [Isidore Nathan Dubin, American pathologist, 1913â€1981; Frank B. Johnson, American pathologist, born 1919] see under syndrome.. Medical dictionary. 2011 Dubin-Johnson syndrome is a rare autosomal recessive liver disorder characterized by chronic or intermittent conjugated hyperbilirubinemia (non-pruritic) and is caused by deficient, multidrug resistant-associated protein-2 . In women, the disease may be discovered when jaundice appears during pregnancy or with the use of oral contraceptive pills In Japan, Dubin-Johnson Syndrome was found to be highly prevalent in an isolated area of consanguinity. The age at onset has been established between infancy (10 weeks) and as late as 56 years of age. + + + Genetic inheritance + + It is transmitted as autosomal recessive trait. There is a reduced penetrance in females. The gene encoding for the.

The Dubin-Johnson Syndrome (DJS) is characterized by a black liver; chronic nonhemolytic, pre­ dominantly conjugated hyperbilirubinemia; and im­ paired biliary excretion of substances such as Brom­ sulphalein. The nature of the black pigment in the DJS is not known.i Both lipofuscin2-5 and melanin6- Dubin-Johnson syndrome and Rotor syndrome cause conjugated hyperbilirubinemia, but without cholestasis, causing no symptoms or sequelae other than jaundice. Bilirubin may appear in the urine, in contrast to the unconjugated hyperbilirubinemia in Gilbert syndrome (which also causes no other symptoms), in which bilirubin is absent from the urine About Press Copyright Contact us Creators Advertise Developers Terms Privacy Policy & Safety How YouTube works Test new features Press Copyright Contact us Creators. Kartenbeck J, Leuschner U, Mayer R, Keppler D (1996) Absence of the canalicular isoform of the MRP gene-encoded conjugate export pump from the hepatocytes in Dubin-Johnson syndrome. Hepatology 23(5):1061-1066 PubMed Google Schola Dubin-Johnson Syndrome. List of authors. A 48-year-old woman scheduled to receive a laparoscopic cholecystectomy underwent a preoperative evaluation. A laparoscopic exploration, shown in a video.

Dubin-Johnson syndrome Classification & external resources; Bilirubin: ICD-10 E80.6 ICD-9 277.4 OMIM 237500 DiseasesDB 3982 eMedicine med/588 MeSH: D007566 Dubin-Johnson syndrome is an autosomal recessive disorder which causes an increase of conjugated bilirubin without elevation of liver enzymes (ALT, AST). It is usually diagnosed in early. Dubin-Johnson syndrome is a rare, autosomal recessive, benign disorder that causes an isolated increase of conjugated bilirubin in the serum. Classically, the condition causes a black liver due to the deposition of a pigment similar to melanin. This condition is associated with a defect in the ability of hepatocytes to secrete conjugated bilirubin into the bile, and is similar to Rotor. Dubin-Johnson syndrome synonyms, Dubin-Johnson syndrome pronunciation, Dubin-Johnson syndrome translation, English dictionary definition of Dubin-Johnson syndrome. n. 1. A group of symptoms that collectively indicate or characterize a disease, disorder, or other condition considered abnormal. 2. a. A complex of.. Dubin-Johnson syndrome (DJS) is usually diagnosed after puberty, although cases have been reported in neonates. Kondo T, Yagi R. Dubin-Johnson syndrome in a neonate

black colored liver was found ( a), with a gallbladder full of yellow stones, suggestive of high cholesterol (band c). These pathognomonic findings confirm the. diagnosis of Dubin-Johnson syndrome. Dubin-Johnson syndrome (DJS) and Rotor's syndrome result in conjugated hyperbilirubinaemia. Both DJS and Rotor's syndrome have a relatively benign course. Diagnosis is important to avoid unnecessary investigations and alleviate concern. Other causes of conjugated hyperbilirubinaemia, such as benign recurrent intrahepatic cholestasis, may. Dubin-Johnson syndrome has similarities with the Rotor syndrome as it is a defect in the ability of hepatocytes to disseminate bilirubin into the bile. The condition is generally asymptomatic but it can be diagnosed early in infants with the aid of laboratory tests

Neonatal Dubin-Johnson syndrome (DJS) is rarely diagnosed and mutational analysis of multidrug-resistance-associated protein 2 (MRP2) in such patients had not been reported. We aimed to. TY - JOUR T1 - Dubin-Johnson Syndrome. AU - Noe Del Cueto-Aguilera,Angel, AU - García-Compean,Diego, AU - Scharrer Cabello,Susanna Isabel, AU - González-González,José Alberto, PY - 2020/9/4/entrez PY - 2020/9/5/pubmed PY - 2020/9/30/medline SP - 1386 EP - 1386 JF - The American journal of gastroenterology JO - Am J Gastroenterol VL - 115 IS - 9 SN - 1572-0241 UR - https://cancerres. Dubin Johnson syndrome (DJS) is a rare autosomal recessive disorder characterised by chronic or intermittent conjugated hyperbilirubinemia due to absence of functional Multidrug Resistance-associated Protein 2 (MRP2) [].The ABCC2 gene that encodes the MRP2 is in chromosome 10q24. The MRP2 protein is located in the canalicular membrane of hepatocytes and also at the apical membranes of. Dubin-Johnson syndrome is an autosomal recessive disease which presents shortly after birth with an increase of conjugated bilirubin without elevation of liver enzymes (ALT, AST). Dubin-Johnson syndrome Dubin-Johnson Syndrome List of authors. Kazuhiko Morii, M.D., Ph.D., and Takeharu Yamamoto, M.D. Related Articles; A 48-year-old woman scheduled to receive a laparoscopic cholecystectomy.

Dubin Johnson syndrome: (dōō′bĭn-jŏn′sən) n. An inherited defect in hepatic excretory function marked by an increase of serum bilirubin concentration, an excessive urinary excretion of abnormal proportions of a form of coproporphyrin, a retention of dark pigment by hepatocytes, and the nonvisualization of the gall bladder using a. Dubin-Johnson syndrome is a rare and benign condition but it is not easy to diagnose. the condition is often misdiagnosed with gallstone disease and patients are unnecessarily subject to surgery. For this reason, patients should be managed by an interprofessional team Mutations found in patients suffering from the Dubin-Johnson syndrome, along with the amino acid residues which are involved in supporting the transport activity of MRP2, are also summarized. 2002 Elsevier Science B.V Is a 47 gene panel that includes assessment of non-coding variants. Is ideal for patients who have any type of cholestasis including those with clinical suspicion of Alagille syndrome, citrullinemia type 2, Crigler-Najjar syndrome types 1 and 2, Dubin-Johnson syndrome, Gilbert syndrome, intrahepatic cholestasis of pregnancy type 3 or progressive familial intrahepatic cholestasis types 1-4 Dubin-Johnson syndrome: translation Du·bin-John·son syndrome (dooґb ə n jonґs ə n) [Isidore Nathan Dubin, American pathologist, 1913â€1981; Frank B. Johnson, American pathologist, born 1919] see under syndrome

99mTc-HIDA cholescintigraphy was performed in 6 patients with Dubin-Johnson syndrome and 1 patient with Rotor syndrome. In the patients with Dubin-Johnson syndrome, the cholescintigrams had a characteristic pattern of delayed visualization or nonvisualization of the gallbladder and bile ducts in the presence of intense, homogeneous, and prolonged visualization of the liver Support groups for Dubin-Johnson Syndrome. Providers. Healthcare providers in the area. Research. Various sources of research on Dubin-Johnson Syndrome. Financial Resources. Information about disability benefits from the Social Security Administration the Dubin-Johnson syndrome is most helpful in establishing the diagnosis. In general, the lesion observed is a collection of coarse pigment granules within the cytoplasm of the centrilobular hepatocytes and within some Kupffer cells. This pigment imparts a dark brown coloration which may b Dubin Johnson syndrome (DJS) is an autosomal recessive disorder of bilirubin metabolism. It is a very rare diseases and is characterized by persistent and fluctuating jaundice. Jaundice is aggravated by stress, pregnancy, alcoholism, infections, etc. It can be mistaken for viral hepatitis. It carries an excellent prognosis with no adverse impact on life expectancy Disease - Dubin-Johnson syndrome ))) Map to. UniProtKB (1) Reviewed (1) Swiss-Prot. Format. Definition. Autosomal recessive disorder characterized by conjugated hyperbilirubinemia, an increase in the urinary excretion of coproporphyrin isomer I, deposition of melanin-like pigment in hepatocytes, and prolonged retention of sulfobromophthalein.

Tricia Christensen Doctors will often closely monitor the liver function of people with Dubin-Johnson syndrome. One potential cause of jaundice (yellowing of the skin and whites of the eyes) is a relatively rare disorder called Dubin-Johnson syndrome or idiopathic jaundice. This condition cannot be acquired because it is inherited in an autosomal recessive pattern, which means children only. Information on families of 44 patients fulfilling strict criteria for the Dubin-Johnson-Sprinz (DJS) syndrome revealed parental consanguinity in at least 11 cases. Direct and total serum bilirubin levels and sulfobromophthalein retention levels were determined on siblings, parents, and children of these DJS patients. Conjugated hyperbilirubinemia and elevated, increasing serum.

Dubin-Johnson Syndrome Syndromes: Rapid Recognition and

  1. Dubin-Johnson syndrome (DJS) is a type of hereditary hyperbilirubinemia that was first described independently in 1954 by Dubin and Johnson and by Sprinz and Nelson. Hereditary hyperbilirubinemias can be divided into conjugated forms and unconjugated forms
  2. Dubin johnson syndrome has a defect in the multispecific anion transporter gene. Likely a loss of function mutation, since the mutation affects the cytoplasmic domain. Dubin johnson syndrome is a very rare genetic disorder. In order to inherit the condition, a child must get a copy of the defective gene from both parents. Although most patients.
  3. Dubin-Johnson syndrome is a benign condition, which results from a hereditary defect in biliary secretion of bilirubin pigments, and manifests as recurrent jaundice with conjugated hyperbilirubinemia. The defect is due to the absence of the canalicular protein MRP2 located on chromosomes 10q 24, which is responsible for the transport of biliary.
  4. Dubin-Johnson syndrome: Hyperbilirubinemia in the Term Newborn. Health and Medicine Reference Covering Thousands of Diseases and Prescription Drugs. Find information on thousands of medical conditions and prescription drugs
  5. Dubin-Johnson syndrome (DJS) is a rare autosomal recessive disorder characterized by a defect in the transfer of endogenous and exogenous anionic conju-gates from hepatocytes into the bile, which causes conjugated hyperbilirubinemia and deposition of black pigments in the liver [1]. A mutation in the huma
  6. To the Editor: I have some questions and comments about the article by Wolkoff et al. entitled Inheritance of the Dubin-Johnson Syndrome (N Engl J Med 288:113, 1973). The authors, and others previously, have observed both decreased urinary excretion of coproporphyrin III and increased excretion of coproporphyrin I in the Dubin-Johnson.
  7. What is Dubin-johnson Syndrome ? Autosomal recessive disorder. The condition causes an increase of conjugated bilirubin in the serum without elevation of liver enzymes
Rotor Syndrome - The Medical Biochemistry Page

Dubin-Johnson syndrome: MedlinePlus Medical Encyclopedi

Absence of a functional multidrug resistance protein 2 (MRP2; symbol ABCC2) from the hepatocyte canalicular membrane is the molecular basis of Dubin- Johnson syndrome, an inherited disorder associa.. Dubin-Johnson syndrome: Jaundice. Health and Medicine Reference Covering Thousands of Diseases and Prescription Drugs Hyperbilirubinemia and a delayed plasma clearance of sulfobromophthalein was observed in neonatal mutant lambs possessing a syndrome similar to Dubin-Johnson syndrome in man. Characteristic discoloration of the liver due to hepatocellular pigmentation was not discernible at birth when examined by electron and light microscopy. The livers of 2 mutants were first observed to be grossly. Dubin-Johnson syndrome is a rare, autosomal recessive, inherited disorder characterized by biphasic, predominantly conjugated hyperbilirubinemia with no progression to end-stage liver disease. The molecular basis in Dubin-Johnson syndrome is absence or deficienc..

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dubin-johnson syndrome by: Pamela .B. and Lyric .W. or therapy what is it? DJS the Dubin-Johnson syndrome (or djs) is a autosomal recessive disorder, which means it is passed down by the parents both needing the copy of an abnormal gene what happens? this syndrome increases th Dubin-Johnson syndrome may manifest as conjugated hyperbilirubinemia, darkly pigmented liver, presence of abnormal pigment in the parenchyma of hepatocytes and abnormal distribution of the coproporphyrin isomers I and III in the urine. Intrahepatic cholestatic jaundice of pregnancy presents as pruritus, abnormal liver biochemistry and increased. Dubin-Johnson Syndrome. Published on 21/04/2017 by admin. Filed under Pathology. Last modified 21/04/2017. Print this page. Average : rate 1 star rate 2 star rate 3 star rate 4 star rate 5 star. Your rating: none, Average: 3.9 (9 votes) Rate it. This article have been viewed 1455 times. Tweet. Grossly Evident Pigment. These results indicate that, with respect to urinary coproporphyrin excretion, the Dubin-Johnson syndrome is inherited as an autosomal recessive characteristic. THE Dubin-Johnson syndrome is characterized by a black liver and chronic nonhemolytic, predominantly conjugated,.

Dubin-Johnson syndrome is a rare liver disorder characterized by mild jaundice throughout life. Jaundice, which is caused by an incomplete metabolism of bilirubin, may not appear until puberty or adulthood. This condition is aggravated by alcohol, pregnancy, oral contraceptives, infections and other environmental factors World map of Dubin-Johnson syndrome Find people with Dubin-Johnson syndrome through the map. Connect with them and share experiences. Join the Dubin-Johnson syndrome community Rotor syndrome is related to Dubin-Johnson syndrome in that it is a disorder characterized by conjugated hyperbilirubinema. Molecular Biology of Rotor Syndrome. The molecular causes of Rotor syndrome are mutations in two genes. Mutations in both genes have to occur for an individual to manifest the symptoms of Rotor syndrome Dubin-Johnson Syndrome as a Cause of Neonatal Jaundice: The Importance of Coproporphyrins Investigation Y. Haimi-Cohen, MD , P. Merlob, MD , T. Marcus-Eidlits, MD , and J. Amir, MD Clinical Pediatrics 2016 37 : 8 , 511-51 Related to Dubin-Johnson syndrome: Crigler-Najjar syndrome, Gilbert syndrome, Rotor's syndrome, Rotor syndrome hyperbilirubinemia [ ¦hī·pər‚bil·ə‚rü·bə′nē·mē·ə

HyperbilirubinemiaApproach to evaluation of liver disorders

Paulusma CC, Kool M, Bosma PJ, et al. A mutation in the human canalicular multispecific organic anion transporter gene causes the Dubin-Johnson syndrome. Hepatology. 1997 Jun;25(6):1539-42. Frank M, Doss M, de Carvalho DG. Diagnostic and pathogenetic implications of urinary coproporphyrin excretion in Dubin-Johnson syndrome dubin johnson syndrome . By Guest. New Reply Follow New Topic. Guest over a year ago. what stains are used for visualizing liver biopsy in Dubin johnson syndrome? Loading... Quick reply. If you want to get notified by every reply to your post, please register. It is FREE! No, thanks. Background: Dubin Johnson syndrome is a variety of inherited hyperbilirubinemia and is recognized by a low elevation of conjugated bilirubin without other signs of liver damage.Objective: To present perinatal results of a patient diagnosed with Dubin Johnson syndrome. Methods: The clinical file of a patient diagnosed with Dubin Johnson syndrome during pregnancy, treated at the Instituto. Dubin-Johnson syndrome is a rare, autosomal recessive, benign disorder that causes an isolated increase of conjugated bilirubin in the serum. Classically, the condition causes a black liver due to the deposition of a pigment similar to melanin. This condition is associated with a defect in the ability of hepatocytes to secrete conjugated bilirubin into the bile, and is similar to Rotor syndrome Dubin johnson syndrome; Rotor syndrome; Clinical Information. A benign, autosomally recessive inherited hyperbilirubinemia characterized by the presence of a dark pigment in the centrilobular region of the liver cells. There is a functional defect in biliary excretion of bilirubin, cholephilic dyes, and porphyrins