Skip to Content

Dubin-Johnson syndrome

Pronunciation: du'bin jon'son

Definition: an inherited defect in hepatic excretory function characterized by jaundice with levels of serum bilirubin up to about 6 mg/dL, over half of which is conjugated, and excretion of abnormal proportions of coproporphyrin I in urine. There is also retention of a dark pigment in the hepatocytes that is derived either from melanin or catecholamines, but otherwise liver histology examination provides normal results. Oral cholecystogram fails to visualize the gallbladder; excretion of test substances (bromosulfothalein) by the liver is abnormal. The basic defect is apparently in canalicular transport. No therapy is necessary; autosomal recessive inheritance caused by mutation in the canalicular multispecific organic anion transporter gene (CMOAT) on 10q.

Synonym(s): chronic idiopathic jaundice

Disclaimer: This site is designed to offer information for general educational purposes only. The health information furnished on this site and the interactive responses are not intended to be professional advice and are not intended to replace personal consultation with a qualified physician, pharmacist, or other healthcare professional. You must always seek the advice of a professional for questions related to a disease, disease symptoms, and appropriate therapeutic treatments.
© Copyright 2018 Wolters Kluwer. All Rights Reserved. Review Date: Sep 19, 2016.