Jaundice, Chronic Idiopathic

Last update 08 May 2025

Basic Info

Synonyms

Black liver-jaundice syndrome, CHRONIC IDIOPATHIC JAUNDICE, Chronic Idiopathic Jaundice

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Introduction

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. Affected persons may be asymptomatic or have vague constitutional or gastrointestinal symptoms. The liver may be slightly enlarged, and oral and intravenous cholangiography fails to visualize the biliary tract.

Clinical Trials associated with Jaundice, Chronic Idiopathic

CTRI/2014/03/004511

/ RecruitingNot ApplicableIIT

Randomised control trial to compare pain during DoubleJ stent removal using Flexible cystoscope and Semirigid Ureteroscope

Start Date10 Jun 2013

Sponsor / Collaborator

Jawaharlal Institute of Postgraduate Medical Education and Research

100 Clinical Results associated with Jaundice, Chronic Idiopathic

100 Translational Medicine associated with Jaundice, Chronic Idiopathic

0 Patents (Medical) associated with Jaundice, Chronic Idiopathic

705

Literatures (Medical) associated with Jaundice, Chronic Idiopathic

23 Feb 2025·Cureus

An Incidental Finding of an Indigo-Blue Liver in a Patient With Dubin-Johnson Syndrome Confirmed via Genetic Testing

Article

Author: Pérez, Miguel ; Morales, Emilio ; Barrera, Francisco ; Sotomayor, Camila ; Martínez, Jorge A ; Torres, Javiera ; Viñuela, Macarena

We present the case of a 29-year-old female with chronic jaundice and a history of dyshidrosis, previously treated with cyclosporine. She also had hypertriglyceridemia managed with fibrates and was referred for evaluation of gallstone disease. Imaging confirmed gallstones without bile duct abnormalities. Laboratory tests revealed conjugated hyperbilirubinemia (total bilirubin: 3.0 mg/dL, direct bilirubin: 2.95 mg/dL). During laparoscopic cholecystectomy, a striking blue liver appearance was observed. A liver biopsy confirmed Dubin-Johnson syndrome (DJS). Genetic analysis revealed two pathogenic ABCC2 variants: c.2077G>A (p.Gly693Arg) and a novel mutation, c.513del (p.Tyr172Thrfs*6). This report highlights a rare "blue liver" presentation of DJS, thereby expanding the differential diagnosis of blue liver syndrome.

01 Sep 2024·Archives of Industrial Hygiene and Toxicology

Factors predicting the level of vaccine protection against hepatitis B virus infection among physicians and nurses in Šabac, Serbia

Article

Author: Maletić, Jelena Stojčević ; Milutinović, Dragana ; Stevanović, Goran ; Vasić, Slobodanka Bogdanović ; Svitlica, Branislava Brestovački ; Ružić, Maja ; Aranđelović, Branimirka ; Savić, Nikola

AbstractAs healthcare workers run a high and constant occupational risk of hepatitis B virus (HBV) infection through exposure to biological material, vaccination is mandatory as well as the monitoring of antibody levels one to two months after complete immunisation. The aim of this descriptive cross-sectional study was to determine HBV vaccine coverage of 200 primary and secondary healthcare workers (100 each) from Šabac, Serbia and their blood anti-HBs titre. We also wanted to identify factors that could predict the titre. Anti-HBV vaccination covered all participants, of whom 89.5 % were fully vaccinated, and 85 % had a protective antibody titre. We found a statistically significant association between antibody titre and the number of received vaccine doses, chronic jaundice, autoimmune disease, and cancer in our participants. The fact that 15 % did not achieve the protective antibody titre confirms the necessity of its control after immunisation, which is not routinely carried out in most countries, Serbia included. It is, therefore, necessary to develop a detailed strategy for monitoring vaccination and serological status of healthcare workers in order to improve their safety at work. An important role should also be given to continuous education of healthcare workers from the beginning of schooling to the end of their professional career.

01 May 2024·Journal of Gastroenterology and Hepatology

Diagnostic algorithm for neonatal intrahepatic cholestasis integrating single‐gene testing and next‐generation sequencing in East Asia

Article

Author: Shin, MinSoo ; Seong, Moon Woo ; Lee, Heerah ; Ko, Jae Sung ; Hahn, Jong Woo ; Moon, Jin Soo

AbstractBackground and AimAdvances in molecular genetics have uncovered causative genes responsible for neonatal cholestasis. Panel‐based next‐generation sequencing has been used clinically in infants with neonatal cholestasis. We aimed to evaluate the clinical application of single‐gene testing and next‐generation sequencing and to develop a diagnostic algorithm for neonatal intrahepatic cholestasis.MethodsFrom January 2010 to July 2021, patients suspected of having neonatal intrahepatic cholestasis were tested at the Seoul National University Hospital. If there was a clinically suspected disease, single‐gene testing was performed. Alternatively, if it was clinically difficult to differentiate, a neonatal cholestasis gene panel test containing 34 genes was performed.ResultsOf the total 148 patients examined, 49 (33.1%) were received a confirmed genetic diagnosis, including 14 with Alagille syndrome, 14 with neonatal intrahepatic cholestasis caused by citrin deficiency, 7 with Dubin–Johnson syndrome, 5 with arthrogryposis‐renal dysfunction‐cholestasis syndrome, 5 with progressive familial intrahepatic cholestasis type II, 1 with Rotor syndrome, 1 with Niemann‐Pick disease type C, 1 with Kabuki syndrome, and 1 with Phenylalanyl‐tRNA synthetase subunit alpha mutation. Sixteen novel pathogenic or likely pathogenic variants of neonatal cholestasis were observed in this study. Based on the clinical characteristics and laboratory findings, we developed a diagnostic algorithm for neonatal intrahepatic cholestasis by integrating single‐gene testing and next‐generation sequencing.ConclusionsAlagille syndrome and neonatal intrahepatic cholestasis caused by citrin deficiency were the most common diseases associated with genetic neonatal cholestasis. Single‐gene testing and next‐generation sequencing are important and complementary tools for the diagnosis of genetic neonatal cholestasis.

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Jaundice, Chronic Idiopathic

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