Disease Information for Dubin-Johnson syndrome

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Clinical Manifestations
Signs & Symptoms
Yellow skin discoloration
Choluria/Biliuria in Children
Choluria/Biluria/Bilirubin urine
Jaundice/Yellow skin and eyes
Palpable Liver/Hepatomegaly
Yellow eyeballs/Scleral icterus
Disease Progression
Course/Chronic disorder
Course/Chronic only
Demographics & Risk Factors
Ethnic or Racial Factors
Middle Eastern Population
Population Group
Child
Population/Pediatrics population
Family History
Family history/Liver disease
Sex & Age Groups
Population/Child
Population/Child-Infant Only
Population/Children/all
Laboratory Tests
Abnormal Lab Findings (Non Measured)
Direct bilirubin inc/normal indirect (Lab)
Liver Functions Abnormal (Lab)
URINE Color Brown
Abnormal Lab Findings - Increased
Bilirubin, serum (Lab)
URINE Bilirubin
URINE Coproporphyrin
Diagnostic Test Results
Other Tests & Procedures
TEST/BSP retention
Pathology
BX/Liver biopsy/Abnormal
BX/Liver Hepatocytes pigment/deposition
Isotope Scan
Isotope/Hepatic bilirubin clearance study/abnormal
Associated Diseases & Rule outs
Associated Disease & Complications
Bilirubinemia
Cholestasis/intrahepatic biliary obstruction
Cholestatic Jaundice Syndrome
Dubin-Johnson Syndrome
Factor VII deficiency
Neonatal hyperbilirubinemia
Obstructive jaundice syndrome
Disease Mechanism & Classification
Class
CLASS/Pediatric disorders (ex)
CLASS/Liver involvement/disorder (ex)
CLASS/Liver/gallbladder/ducts (category)
Pathophysiology
Pathophysiology/Genomic indentifiers (polymorphism/snip/mutations)
Process
PROCESS/Autosomal recessive disorder (ex)
PROCESS/Enzyme defect/Metabolic disorder (ex)
PROCESS/Eponymic (category)
PROCESS/Hereditofamilial (category)
PROCESS/Metabolic/storage disorder (category)
PROCESS/Inherited bilirubin metabolism disorder (ex)
Definition

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 gastrointestional symptoms; The liver may be slightly enlarged, and oral and intravenous cholangiography fails to visualize the biliary tract---------------------------------------Dubin Johnson Syndrome: Chronic Idiopathic Jaundice;

Chronic Idiopathic Jaundice; Conjugated Hyperbilirubinemia;

DJS; Hyperbilirubinemia II Disorder; is a rare genetic liver disorder that tends to affect people of Middle Eastern Jewish heritage disproportionately to other groups; It appears to be associated with clotting factor VII in this population; Symptoms may include a yellowish color to the skin (jaundice), and a liver that is sometimes enlarged and tender---------[NORD 2005]--------------------

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External Links Related to Dubin-Johnson syndrome
Google
Wikipedia
Merck
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PubMed (National Library of Medicine)
NGC (National Guideline Clearinghouse)
Medscape (eMedicine)
Harrison's Online (accessmedicine)
NEJM (The New England Journal of Medicine)
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