1. Hyperbilirubinemia Sara Pape-Salmon NP(F) VIHA, Mental Health & Addictions Services April 13, 2010 Sara Pape-Salmon NP(F) VIHA, Mental Health & Addictions Services April 13, 2010

2. Demographics  H.H.  64 year-old female  Eastern European descent  Resides in Victoria  H.H.  64 year-old female  Eastern European descent  Resides in Victoria

3. PMHx:  Paranoid schizophrenia  Obesity  Over-flow incontinence likely  Paranoid schizophrenia  Obesity  Over-flow incontinence likely

4. PSHx:  Tubal ligation

5. Medications & Allergies:  Loxapine 25 mg OD  Multivitamin  Folic Acid  Vitamin B12  NKDA  Loxapine 25 mg OD  Multivitamin  Folic Acid  Vitamin B12  NKDA

6. Social Hx:  Lives alone  No ETOH, currently non-smoker (remote 20 yr hx), no IVD/illicit drug use  Receives intensive MHAS out-reach services  Family hx is not known  Lives alone  No ETOH, currently non-smoker (remote 20 yr hx), no IVD/illicit drug use  Receives intensive MHAS out-reach services  Family hx is not known

7. Chief Concern  Serum icterus, slight (incidental finding)  Fatigue?  Serum icterus, slight (incidental finding)  Fatigue?

8. Lab Findings:  Serum indices = slight icterus  ALT, AST = normal  Total bilirubin = high (29 umol/L)  Conjugated bilirubin = normal (3 umol/L)  ? Unconjugated bilirubin (was not obtained/tested)  Serum indices = slight icterus  ALT, AST = normal  Total bilirubin = high (29 umol/L)  Conjugated bilirubin = normal (3 umol/L)  ? Unconjugated bilirubin (was not obtained/tested)

9. Dx & Pathophysiology  Gilbert Syndrome  Pathophysiology  Most common inherited cause of unconjugated hyperbilirubinemia (recessive trait UGT1 gene)  Underactivity of the conjugating enzyme system (diphospate glucuronyl transferase)  Benign condition  Gilbert Syndrome  Pathophysiology  Most common inherited cause of unconjugated hyperbilirubinemia (recessive trait UGT1 gene)  Underactivity of the conjugating enzyme system (diphospate glucuronyl transferase)  Benign condition

10. Physiology Review  Bilirubin conjugation  Bilirubin = byproduct of RBC destruction  In plasma bilirubin binds to albumin & is lipid soluble = “unconjugated bilirubin”  Unconjugated bilirubin can cross biologic membranes  Bilirubin conjugation  Bilirubin = byproduct of RBC destruction  In plasma bilirubin binds to albumin & is lipid soluble = “unconjugated bilirubin”  Unconjugated bilirubin can cross biologic membranes

11. Physiology Continued  Bilirubin conjugation cont:  Unconjugated bilirubin moves into sinusoids in the hepatocyte & joins with glucuronic acid & becomes water soluble = “conjugated bilirubin”  Conjugated bilirubin, now H2O soluble, can be excreted  Excreted in urine (sm amt as urobininogen) and in feces (mostly)  Bilirubin conjugation cont:  Unconjugated bilirubin moves into sinusoids in the hepatocyte & joins with glucuronic acid & becomes water soluble = “conjugated bilirubin”  Conjugated bilirubin, now H2O soluble, can be excreted  Excreted in urine (sm amt as urobininogen) and in feces (mostly)

12. Signs & Symptoms  Usually dx around puberty  Often precipitated by intercurrent illness, dehydration, menstrual periods, stress, fasting states  Abdominal cramps  Fatigue  Malaise  Mild jaundice intermittently in some  Many people are asymptomatic  Usually dx around puberty  Often precipitated by intercurrent illness, dehydration, menstrual periods, stress, fasting states  Abdominal cramps  Fatigue  Malaise  Mild jaundice intermittently in some  Many people are asymptomatic

13. Differentials  Hemolysis  Hematoma  Rhabdomyolysis  Acute or chronic liver disease  Infections  Cardiac disease  Medications (e.g. Atazanavir, probenicid, some antibiotics)  Thyrotoxicosis  Hemolysis  Hematoma  Rhabdomyolysis  Acute or chronic liver disease  Infections  Cardiac disease  Medications (e.g. Atazanavir, probenicid, some antibiotics)  Thyrotoxicosis

14. Laboratory Studies  CBC including retics and blood smear (exclude hemolysis, RBC abnormalities)  Lactate dehydrogenase  LFT’s  Conjugated and unconjugated bilirubin  Dx: Normal CBC, retic, blood smear, LFT’s, + unconjugated hyperbilirubinemia on several occasions, + absence of other disease process.  CBC including retics and blood smear (exclude hemolysis, RBC abnormalities)  Lactate dehydrogenase  LFT’s  Conjugated and unconjugated bilirubin  Dx: Normal CBC, retic, blood smear, LFT’s, + unconjugated hyperbilirubinemia on several occasions, + absence of other disease process.

15. Treatment  Reassurance of benign nature  Normal life expectancy  No dietary or activity restrictions  No medications for treatment  Reassurance of benign nature  Normal life expectancy  No dietary or activity restrictions  No medications for treatment

16. References:  McCance, K.L., & Huether, S.E. (2002). Pathophysiology: The biologic basis for disease in adults and children. (4th ed). Mosby Inc. St. Louis, Missouri.  Mukherjee, S. (2009). Gilbert Syndrome. Found on-line at http://emedicine.medscape.com/article/176822- overview. http://emedicine.medscape.com/article/176822- overview  McCance, K.L., & Huether, S.E. (2002). Pathophysiology: The biologic basis for disease in adults and children. (4th ed). Mosby Inc. St. Louis, Missouri.  Mukherjee, S. (2009). Gilbert Syndrome. Found on-line at http://emedicine.medscape.com/article/176822- overview. http://emedicine.medscape.com/article/176822- overview