Dubin-Johnson syndrome

Dubin-Johnson syndrome (DJS) is a benign, inherited liver disorder characterized clinically by chronic, predominantly conjugated, hyperbilirubinemia and histopathologically by black-brown pigment deposition in parenchymal liver cells.



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Narrow down the case reports



Total: 45 (papers)

  


(per page)
Matched Phenotype  Gene  Mutation  MeSH
Rank
(Similarity)
PMID
(PMCID)
35
(11.2%)
12012642
Treatment of severe cholestasis in neonatal Dubin-Johnson syndrome with ursodeoxycholic acid.
Regev RH, Stolar O, Raz A, Dolfin T.
J Perinat Med. 2002;30(2):185-7.
Hyperbilirubinemia
Bilirubin Cholestasis Homo sapiens Hyperbilirubinemia Infant, Newborn Jaundice, Chronic Idiopathic Male
35
(11.2%)
9872548
Postoperative management following massive hepatectomy in a patient with Dubin-Johnson syndrome: report of a case.
Ueno S, Tanabe G, Hanazono K, Ogawa H, Yoshidome S, Aikou T, Yoshida A.
Surg Today. 1998;28(12):1274-8.
Conjugated hyperbilirubinemia
Electron Microscopy Hepatectomy Hepatocyte Growth Factor Homo sapiens Hyperbilirubinemia Jaundice, Chronic Idiopathic Male Middle Aged Plasmapheresis Postoperative Care
35
(11.2%)
7454089
[Constitutional hyperbilirubinemia and mixed bilirubin. Clinical and functional study].
Rapaccini GL, Anti M, Miggiano G, De Vitis I, De Simone F, Fedeli G.
Minerva Med. 1980;71(44):3221-8.
Hyperbilirubinemia
Adult Bilirubin Females Homo sapiens Hyperbilirubinemia Hyperbilirubinemia, Hereditary Jaundice, Chronic Idiopathic Liver Liver Function Tests Male Porphyrins
35
(11.2%)
2051565
[Anesthetic and postoperative management of a patient with Gilbert's syndrome and another with Dubin-Johnson syndrome].
Miyakawa H, Matsumoto K, Matsumoto S, Kinoshita R, Noguchi T, Taniguchi K, Honda N.
Masui. 1991;40(1):119-23.
Conjugated hyperbilirubinemia
Homo sapiens Jaundice, Chronic Idiopathic Male Operative Surgical Procedures Postoperative Complications
45
(10.8%)
20819184
Living donor liver transplantation using a graft from a donor with Dubin-Johnson syndrome.
Liu C, Niu DM, Hsia CY, Loong CC, Lin NC, Tsai HL, Tsou MY, Chin T.
Pediatr Transplant. 2012;16(1):E25-9.
Stroke
Adult Alkaline Phosphatase Bilirubin Biopsy Females Homo sapiens Immunosuppressive Agents Infant Jaundice, Chronic Idiopathic Liver Liver Failure Living Donors Male Stenosis
        

Phenotype(s) retrieved from Orphanet

    Total: 11

HPO ID Term Frequency
HP:0000952 Jaundice Very frequent (99-80%)
HP:0001080 Biliary tract abnormality Very frequent (99-80%)
HP:0001392 Abnormality of the liver Very frequent (99-80%)
HP:0002908 Conjugated hyperbilirubinemia Very frequent (99-80%)
HP:0012086 Abnormal urinary color Very frequent (99-80%)
HP:0004295 Abnormality of the gastric mucosa Frequent (79-30%)
HP:0001928 Abnormality of coagulation Occasional (29-5%)
HP:0001945 Fever Occasional (29-5%)
HP:0002027 Abdominal pain Occasional (29-5%)
HP:0002240 Hepatomegaly Occasional (29-5%)
HP:0012378 Fatigue Occasional (29-5%)


Phenotype(s) retrieved from case reports

    Total: 19

HPO ID Term # of case reports
HP:0002908 Conjugated hyperbilirubinemia 13
HP:0000952 Jaundice 6
HP:0001396 Cholestasis 5
HP:0002904 Hyperbilirubinemia 4
HP:0001082 Cholecystitis 2
HP:0006562 Viral hepatitis 2
HP:0001081 Cholelithiasis 1
HP:0001394 Cirrhosis 1
HP:0001409 Portal hypertension 1
HP:0001733 Pancreatitis 1
HP:0001927 Acanthocytosis 1
HP:0002090 Pneumonia 1
HP:0002586 Peritonitis 1
HP:0002621 Atherosclerosis 1
HP:0003003 Colon cancer 1
HP:0004395 Malnutrition 1
HP:0011980 Cholesterol gallstones 1
HP:0012115 Hepatitis 1
HP:0030242 Portal vein thrombosis 1


Causative gene(s) retrieved from Orphanet

    Total: 1

Gene Symbol Gene Name Entrez Gene ID
ABCC2 ATP binding cassette subfamily C member 2 1244