Dubin-Johnson syndrome

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Overview

Dubin-Johnson syndrome Classification and external resources
Bilirubin
ICD-10	E80.6
ICD-9	277.4
OMIM	237500
DiseasesDB	3982
eMedicine	med/588
MeSH	D007566

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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).

Presentation

There is a lack of canalicular multi-drug resistant protein which causes dysfunction on bilirubin transfer to bile canaliculi.

An isoform of this protein is localized to the lateral hepatocyte membrane, allowing transport of glucuronide and glutathione conjugates back into the blood.

Analysis of urine pophyrins show a normal level of coproporphirin but the I isomer accounts for 80% ot the total(normally 25%)

Liver will present with dark brown appearance due to pigment accumulation.

Prognosis

Prognosis is good and there's no need to treat this syndrome.

See also

• Jaundice
• Gilbert syndrome
• Crigler-Najjar syndrome
• Rotor syndrome

v • d • e WikiDoc Research Resources for Dubin-Johnson syndrome (Click show to right to view)
Articles on Dubin-Johnson syndrome	Most recent articles on Dubin-Johnson syndrome • Most cited articles on Dubin-Johnson syndrome • Review articles on Dubin-Johnson syndrome • Articles on Dubin-Johnson syndrome in N Eng J Med, Lancet, BMJ
Media (Slides, Video, Images, MP3) on Dubin-Johnson syndrome	Powerpoint slides on Dubin-Johnson syndrome • Images of Dubin-Johnson syndrome • Photos of Dubin-Johnson syndrome • Podcasts & MP3s on Dubin-Johnson syndrome • Videos on Dubin-Johnson syndrome
Evidence Based Medicine Regarding Dubin-Johnson syndrome	Cochrane Collaboration on Dubin-Johnson syndrome • Bandolier on Dubin-Johnson syndrome • TRIP on Dubin-Johnson syndrome
Cost Effectiveness of Dubin-Johnson syndrome	Cost Effectiveness of Dubin-Johnson syndrome
Clinical Trials Involving Dubin-Johnson syndrome	Ongoing Trials on Dubin-Johnson syndrome at Clinical Trials.gov • Trial results on Dubin-Johnson syndrome • Clinical Trials on Dubin-Johnson syndrome at Google
Guidelines / Policies / Government Resources (FDA/CDC) Regarding Dubin-Johnson syndrome	US National Guidelines Clearinghouse on Dubin-Johnson syndrome • NICE Guidance on Dubin-Johnson syndrome • NHS PRODIGY Guidance • FDA on Dubin-Johnson syndrome • CDC on Dubin-Johnson syndrome
Textbook Information on Dubin-Johnson syndrome	Books and Textbook Information on Dubin-Johnson syndrome
Pharmacology Resources on Dubin-Johnson syndrome	Dosing of Dubin-Johnson syndrome • Drug interactions with Dubin-Johnson syndrome • Side effects of Dubin-Johnson syndrome • Allergic reactions to Dubin-Johnson syndrome • Overdose information on Dubin-Johnson syndrome • Carcinogenicity information on Dubin-Johnson syndrome • Dubin-Johnson syndrome in pregnancy • Pharmacokinetics of Dubin-Johnson syndrome •
Genetics, Pharmacogenomics, and Proteinomics of Dubin-Johnson syndrome	Genetics of Dubin-Johnson syndrome • Pharmacogenomics of Dubin-Johnson syndrome • Proteomics of Dubin-Johnson syndrome
Newstories on Dubin-Johnson syndrome	Dubin-Johnson syndrome in the news • Be alerted to news on Dubin-Johnson syndrome • News trends on Dubin-Johnson syndrome
Commentary on Dubin-Johnson syndrome	Blogs on Dubin-Johnson syndrome
Patient Resources on Dubin-Johnson syndrome	Patient resources on Dubin-Johnson syndrome • Discussion groups on Dubin-Johnson syndrome • Patient Handouts on Dubin-Johnson syndrome • Directions to Hospitals Treating Dubin-Johnson syndrome • Risk calculators and risk factors for Dubin-Johnson syndrome
Healthcare Provider Resources on Dubin-Johnson syndrome	Symptoms of Dubin-Johnson syndrome • Causes & Risk Factors for Dubin-Johnson syndrome • Diagnostic studies for Dubin-Johnson syndrome • Treatment of Dubin-Johnson syndrome
Continuing Medical Education (CME) Programs on Dubin-Johnson syndrome	CME Programs on Dubin-Johnson syndrome
International Resources on Dubin-Johnson syndrome	Dubin-Johnson syndrome en Espanol • Dubin-Johnson syndrome en Francais
Business Resources on Dubin-Johnson syndrome	Dubin-Johnson syndrome in the Marketplace • Patents on Dubin-Johnson syndrome
Informatics Resources on Dubin-Johnson syndrome	List of terms related to Dubin-Johnson syndrome

v • d • e Metabolic pathology / Inborn error of metabolism (E70-90, 270-279)
Amino acid	Aromatic (Phenylketonuria, Alkaptonuria, Ochronosis, Tyrosinemia, Albinism, Histidinemia) - Branched chain (Maple syrup urine disease, Propionic acidemia, Methylmalonic acidemia, Isovaleric acidemia, 3-Methylcrotonyl-CoA carboxylase deficiency) - Transport (Cystinuria, Cystinosis, Hartnup disease, Fanconi syndrome, Oculocerebrorenal syndrome) - Sulfur (Homocystinuria, Cystathioninuria) - Urea cycle disorder (N-Acetylglutamate synthase deficiency, Carbamoyl phosphate synthetase I deficiency, Ornithine transcarbamylase deficiency, Citrullinemia, Argininosuccinic aciduria, Hyperammonemia) - Glutaric acidemia type 1 - Sarcosinemia
Carbohydrate	Lactose intolerance - Glycogen storage disease (type I, type II, type III, type IV, type V, type VI, type VII) - fructose metabolism (Fructose intolerance, Fructose bisphosphatase deficiency, Essential fructosuria) - galactose metabolism (Galactosemia, Galactose-1-phosphate uridylyltransferase galactosemia, Galactokinase deficiency) - other intestinal carbohydrate absorption (Glucose-galactose malabsorption, Sucrose intolerance) - pyruvate metabolism and gluconeogenesis (PCD, PDHA) - Pentosuria - Renal glycosuria
Lipid storage	Sphingolipidoses/Gangliosidoses: GM2 gangliosidoses (Sandhoff disease, Tay-Sachs disease) - GM1 gangliosidoses - Mucolipidosis type IV - Gaucher's disease - Niemann-Pick disease - Farber disease - Fabry's disease - Metachromatic leukodystrophy - Krabbe disease Neuronal ceroid lipofuscinosis (Batten disease) - Cerebrotendineous xanthomatosis - Cholesteryl ester storage disease (Wolman disease)
Other lipid	Lipoprotein/lipidemias: Hyperlipidemia - Hypercholesterolemia - Familial hypercholesterolemia - Xanthoma - Combined hyperlipidemia - Lecithin cholesterol acyltransferase deficiency - Tangier disease - Abetalipoproteinemia Fatty acid: Adrenoleukodystrophy - Carnitine (Primary, I, II)
Mineral	Cu Wilson's disease/Menkes disease - Fe Haemochromatosis - Zn Acrodermatitis enteropathica - PO43− Hypophosphatemia/Hypophosphatasia - Mg2+ Hypermagnesemia/Hypomagnesemia - Ca2+ Hypercalcaemia/Hypocalcaemia/Disorders of calcium metabolism
Fluid, electrolyte and acid-base balance	Electrolyte disturbance - Na+ Hypernatremia/Hyponatremia - Acidosis (Metabolic, Respiratory, Lactic) - Alkalosis (Metabolic, Respiratory) - Mixed disorder of acid-base balance - H2O Dehydration/Hypervolemia - K+ Hypokalemia/Hyperkalemia - Cl− Hyperchloremia/Hypochloremia
Purine and pyrimidine	Hyperuricemia - Lesch-Nyhan syndrome - Xanthinuria
Porphyrin	Acute intermittent, Gunther's, Cutanea tarda, Erythropoietic, Hepatoerythropoietic, Hereditary copro-, Variegate
Bilirubin	Unconjugated (Gilbert's syndrome, Crigler-Najjar syndrome) - Conjugated (Dubin-Johnson syndrome, Rotor syndrome)
Glycosaminoglycan	Mucopolysaccharidosis - 1:Hurler/Hunter - 3:Sanfilippo - 4:Morquio - 6:Maroteaux-Lamy - 7:Sly
Glycoprotein	Mucolipidosis - I-cell disease - Pseudo-Hurler polydystrophy - Aspartylglucosaminuria - Fucosidosis - Alpha-mannosidosis - Sialidosis
Other	Alpha 1-antitrypsin deficiency - Cystic fibrosis - Amyloidosis (Familial Mediterranean fever) - Acatalasia

de:Dubin-Johnson-Syndrom

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Acknowledgement and Attribution Regarding Sources of Content

Some of the initial content on this page may be incorporated in part from copyleft sources in the public domain including wikis such as Wikipedia and AskDrWiki. Drug information for patients came from the The National Library of Medicine. Infectious disease information may have come from the Centers for Disease Control (CDC). Differential Diagnoses are drawn from clinicians as well as an amalgamation of 3 sources: 1.The Disease Database; 2. Kahan, Scott, Smith, Ellen G. In A Page: Signs and Symptoms. Malden, Massachusetts: Blackwell Publishing, 2004:3; 3. Sailer, Christian, Wasner, Susanne. Differential Diagnosis Pocket. Hermosa Beach, CA: Borm Bruckmeir Publishing LLC, 2002:7 .