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Dubin-Johnson Syndrome

 Syn. DJS

Signs & Symptoms

 Itching (negative symptom) #criteria
Ref: Uptodate [ Inherited disorders associated with conjugated hyperbilirubinemia/approach to the patient with abnormal liver biochemical and function tests] Epocrates [ Dubin-Johnson syndrome]
Mild icter #criteria
Note: Serum Total bilirubin: 2 - 5 mg/dL (or 34 - 85 μmol/L). total bilirubin can be occasionaliy be within normal range or as high as 20–25 mg/dL (or 340–430 μmol/L)
Ref: Uptodate [ Inherited disorders associated with conjugated hyperbilirubinemia] Harrison [ 278] Epocrates [ 418]
Benign
Ref: Uptodate [ Inherited disorders associated with conjugated hyperbilirubinemia] Harrison [ 2345] Epocrates [ Dubin-Johnson syndrome]
Intermittent jaundice #criteria
Note: Serum Total bilirubin: 2 - 5 mg/dL (or 34 - 85 μmol/L). total bilirubin can be occasionaliy be within normal range or as high as 20–25 mg/dL (or 340–430 μmol/L)
Ref: Uptodate [ Inherited disorders associated with conjugated hyperbilirubinemia] Harrison [ 278] Epocrates [ 418]

Physical Examination

 Intermittent jaundice #criteria
Note: Serum Total bilirubin: 2 - 5 mg/dL (or 34 - 85 μmol/L). total bilirubin can be occasionaliy be within normal range or as high as 20–25 mg/dL (or 340–430 μmol/L)
Ref: Uptodate [ Inherited disorders associated with conjugated hyperbilirubinemia] Harrison [ 278] Epocrates [ 418]
Mild icter #criteria
Note: Serum Total bilirubin: 2 - 5 mg/dL (or 34 - 85 μmol/L). total bilirubin can be occasionaliy be within normal range or as high as 20–25 mg/dL (or 340–430 μmol/L)
Ref: Uptodate [ Inherited disorders associated with conjugated hyperbilirubinemia] Harrison [ 278] Epocrates [ 418]
Mild jaundice
Note: due to Different gene mutations
Ref: Uptodate [ Inherited disorders associated with conjugated hyperbilirubinemia/approach to the patient with abnormal liver biochemical and function tests] Harrison [ 278-2345] Epocrates [ Dubin-Johnson syndrome]

Lab / Serology

 Unique pattern of urinary excretion of coprporphyrins #criteria
Note: Aids in diognosis
Ref: Uptodate [ Inherited disorders associated with conjugated hyperbilirubinemia] Harrison [ 2346] Epocrates [ Dubin-Johnson syndrome]
Low-grade Predominantly conjugated chronic hyperbilirubinemia
Note: due to Different gene mutations
Ref: Uptodate [ Inherited disorders associated with conjugated hyperbilirubinemia/approach to the patient with abnormal liver biochemical and function tests] Harrison [ 278-2345] Epocrates [ Dubin-Johnson syndrome]
Benign Isolated elevation of the bilirubin
Note: due to Different gene mutations
Ref: Uptodate [ Inherited disorders associated with conjugated hyperbilirubinemia/approach to the patient with abnormal liver biochemical and function tests] Harrison [ 278-2345] Epocrates [ Dubin-Johnson syndrome]

Imagin Study

 Hepathomegaly
Ref: Uptodate [ Inherited disorders associated with conjugated hyperbilirubinemia] Harrison [ 2345] Epocrates [ Dubin-Johnson syndrome]
Hepatosplenomegaly
Ref: Uptodate [ Inherited disorders associated with conjugated hyperbilirubinemia] Harrison [ 2345] Epocrates [ Dubin-Johnson syndrome]

Complication

 Black liver #criteria
Note: This pigment is thought to be derived from epinephrine metabolites that are not excreted normally
Ref: Uptodate [ Inherited disorders associated with conjugated hyperbilirubinemia] Harrison [ 2346] Epocrates [ Dubin-Johnson syndrome]
Ambiguous abdominal pain
Ref: Uptodate [ Inherited disorders associated with conjugated hyperbilirubinemia] Harrison [ 2345] Epocrates [ Dubin-Johnson syndrome]
Hepathomegaly
Ref: Uptodate [ Inherited disorders associated with conjugated hyperbilirubinemia] Harrison [ 2345] Epocrates [ Dubin-Johnson syndrome]

Risk Factors

Cause

 Mild jaundice
Note: due to Different gene mutations
Ref: Uptodate [ Inherited disorders associated with conjugated hyperbilirubinemia/approach to the patient with abnormal liver biochemical and function tests] Harrison [ 278-2345] Epocrates [ Dubin-Johnson syndrome]
Benign Isolated elevation of the bilirubin
Note: due to Different gene mutations
Ref: Uptodate [ Inherited disorders associated with conjugated hyperbilirubinemia/approach to the patient with abnormal liver biochemical and function tests] Harrison [ 278-2345] Epocrates [ Dubin-Johnson syndrome]
Inherited genetic defects
Note: due to Different gene mutations
Ref: Uptodate [ Inherited disorders associated with conjugated hyperbilirubinemia/approach to the patient with abnormal liver biochemical and function tests] Harrison [ 278-2345] Epocrates [ Dubin-Johnson syndrome]

Other