Case Study 18: Cirrhosis Skylar Strobel

Overview Role of liver Scar tissue forms as liver repairs itself Wide range of causes Liver plays a role in digestion through the production of bile, detoxifies chemicals, metabolizes drugs and makes proteins

Patient S.G. 46 yo white male History of chronic alcoholism Abdominal swelling and confusion Gained 15 lbs Lethargic, acting strangely

PMH Cirrhosis, 4 years ago H/O: Father died at age 52 Uncontrolled ascites and peripheral edema Anemia Acute pancreatitis E. coli-induced bacterial peritonitis Father died at age 52 H/O alcohol abuse H/O IVDA and intranasal cocaine ½ ppd for many years Ascites-build of fluid in the lining between the abdomen and abdominal organs

Review of Systems Increasing abdominal girth No complaints of abdominal pain, fever, chills, nausea, hematemesis, tarry stools, cough, chest pain, weakness, blood in urine, diarrhea or dry mouth

Patient Case Question 1: Hematemesis and tarry stools are clinical signs of which serious potential complication of cirrhosis? Hematemesis = vomiting of blood Acute gastrointestinal bleeding

Physical Exam Skin HEENT Chest Abdomen Genit/Rect Neuro Mild jaundice (+) spider nevi on chest (-) palmar erythema Several ecchymoses on lower extremities HEENT (+) icteric sclera Chest (+) gynecomastia Abdomen Moderately distended, firm, slightly tender (+) prominent veins (+) HSM Genit/Rect Testicles atrophied (+) hemorrhoids Neuro Confused, disoriented

Patient Case Question 2: Identify 15 clinical signs and symptoms consistent with a diagnosis of cirrhosis

Unusual violent behavior Skin Weight gain Lethargy Confusion Unusual violent behavior Skin Mild jaundice (+) spider nevi on chest Several ecchymoses on lower extremities HEENT (+) icteric sclera Chest (+) gynecomastia Abdomen Moderately distended, firm, slightly tender (+) prominent veins (+) HSM Genit/Rect Testicles atrophied (+) hemorrhoids Neuro Confused, disoriented

Lab Test Results Na 135 meq/L WBC 4700/mm3 Mg 1.7 mg/dL K 3.5 meq/L PT 15.6 sec AFP 90 ng/mL Cl 101 meq/L PTT 45.1 sec HBsAg (-) HCO3 25 meq/L NH3 250µg/dL HIV (-) BUN 12 mg/dL AST 107 IU/L Anti-HCV (+) Cr .6 mg/dL ALT 86 IU/L HCV RNA 2.8 million/mL Glu 90 mg/dL Alk phos 224 IU/L ANA (-) Hb 14.0 g/dL Bilirubin 2.4 mg/dL Fe 75 µg/dL Hct 39.7% Protein 6.6 g/dL Ferratin 200 ng/mL MCV 90 fL Alb 2.7 g/dL Transferrin saturation 38% Plt 34500/mm3 Ca 8.5 mg/dL Ceruplasmin 37 mg/dL

3. Anemic? Na 135 meq/L WBC 4700/mm3 Mg 1.7 mg/dL K 3.5 meq/L PT 15.6 sec AFP 90 ng/mL Cl 101 meq/L PTT 45.1 sec HBsAg (-) HCO3 25 meq/L NH3 250µg/dL HIV (-) BUN 12 mg/dL AST 107 IU/L Anti-HCV (+) Cr .6 mg/dL ALT 86 IU/L HCV RNA 2.8 million/mL Glu 90 mg/dL Alk phos 224 IU/L ANA (-) Hb 14.0 g/dL Bilirubin 2.4 mg/dL Fe 75 µg/dL Hct 39.7% Protein 6.6 g/dL Ferratin 200 ng/mL MCV 90 fL Alb 2.7 g/dL Transferrin saturation 38% Plt 34500/mm3 Ca 8.5 mg/dL Ceruplasmin 37 mg/dL Look at hemoglobin, hematocrit, MCV, platelets and WBC Then look at iron in the blood

4. Abnormality of CBC? Na 135 meq/L WBC 4700/mm3 Mg 1.7 mg/dL K 3.5 meq/L PT 15.6 sec AFP 90 ng/mL Cl 101 meq/L PTT 45.1 sec HBsAg (-) HCO3 25 meq/L NH3 250µg/dL HIV (-) BUN 12 mg/dL AST 107 IU/L Anti-HCV (+) Cr .6 mg/dL ALT 86 IU/L HCV RNA 2.8 million/mL Glu 90 mg/dL Alk phos 224 IU/L ANA (-) Hb 14.0 g/dL Bilirubin 2.4 mg/dL Fe 75 µg/dL Hct 39.7% Protein 6.6 g/dL Ferratin 200 ng/mL MCV 90 fL Alb 2.7 g/dL Transferrin saturation 38% Plt 34500/mm3 Ca 8.5 mg/dL Ceruplasmin 37 mg/dL Super low platelet count – lower range is around 150,000

5. Sudden progression? Na 135 meq/L WBC 4700/mm3 Mg 1.7 mg/dL K 3.5 meq/L PT 15.6 sec AFP 90 ng/mL Cl 101 meq/L PTT 45.1 sec HBsAg (-) HCO3 25 meq/L NH3 250µg/dL HIV (-) BUN 12 mg/dL AST 107 IU/L Anti-HCV (+) Cr .6 mg/dL ALT 86 IU/L HCV RNA 2.8 million/mL Glu 90 mg/dL Alk phos 224 IU/L ANA (-) Hb 14.0 g/dL Bilirubin 2.4 mg/dL Fe 75 µg/dL Hct 39.7% Protein 6.6 g/dL Ferratin 200 ng/mL MCV 90 fL Alb 2.7 g/dL Transferrin saturation 38% Plt 34500/mm3 Ca 8.5 mg/dL Ceruplasmin 37 mg/dL

6. Four risk factors Alcohol Abuse Hepatitis C Overweight Liver Cancer Anti-HCV test + Overweight Height: 5’7” Weight: 171 lbs BMI = 26.8  overweight Liver Cancer AFP test – 90 ng/mL Normal – 0-15 ng/mL

7. Bacterial peritonitis? Na 135 meq/L WBC 4700/mm3 Mg 1.7 mg/dL K 3.5 meq/L PT 15.6 sec AFP 90 ng/mL Cl 101 meq/L PTT 45.1 sec HBsAg (-) HCO3 25 meq/L NH3 250µg/dL HIV (-) BUN 12 mg/dL AST 107 IU/L Anti-HCV (+) Cr .6 mg/dL ALT 86 IU/L HCV RNA 2.8 million/mL Glu 90 mg/dL Alk phos 224 IU/L ANA (-) Hb 14.0 g/dL Bilirubin 2.4 mg/dL Fe 75 µg/dL Hct 39.7% Protein 6.6 g/dL Ferratin 200 ng/mL MCV 90 fL Alb 2.7 g/dL Transferrin saturation 38% Plt 34500/mm3 Ca 8.5 mg/dL Ceruplasmin 37 mg/dL To diagnose: high WBC, protein greater than 1g/dL, glucose less than 50 mg/dL

8. Why do an ANA test? Na 135 meq/L WBC 4700/mm3 Mg 1.7 mg/dL K 3.5 meq/L PT 15.6 sec AFP 90 ng/mL Cl 101 meq/L PTT 45.1 sec HBsAg (-) HCO3 25 meq/L NH3 250µg/dL HIV (-) BUN 12 mg/dL AST 107 IU/L Anti-HCV (+) Cr .6 mg/dL ALT 86 IU/L HCV RNA 2.8 million/mL Glu 90 mg/dL Alk phos 224 IU/L ANA (-) Hb 14.0 g/dL Bilirubin 2.4 mg/dL Fe 75 µg/dL Hct 39.7% Protein 6.6 g/dL Ferratin 200 ng/mL MCV 90 fL Alb 2.7 g/dL Transferrin saturation 38% Plt 34500/mm3 Ca 8.5 mg/dL Ceruplasmin 37 mg/dL ANA-antinuclear antibodies, may help find autoimmune chronic hepatitis, could cause cirrhosis

9. Hemochromatosis? Na 135 meq/L WBC 4700/mm3 Mg 1.7 mg/dL K 3.5 meq/L PT 15.6 sec AFP 90 ng/mL Cl 101 meq/L PTT 45.1 sec HBsAg (-) HCO3 25 meq/L NH3 250µg/dL HIV (-) BUN 12 mg/dL AST 107 IU/L Anti-HCV (+) Cr .6 mg/dL ALT 86 IU/L HCV RNA 2.8 million/mL Glu 90 mg/dL Alk phos 224 IU/L ANA (-) Hb 14.0 g/dL Bilirubin 2.4 mg/dL Fe 75 µg/dL Hct 39.7% Protein 6.6 g/dL Ferratin 200 ng/mL MCV 90 fL Alb 2.7 g/dL Transferrin saturation 38% Plt 34500/mm3 Ca 8.5 mg/dL Ceruplasmin 37 mg/dL Means there is too much iron in the body, iron overload, all are WNL

10. Wilson’s disease? Na 135 meq/L WBC 4700/mm3 Mg 1.7 mg/dL K 3.5 meq/L PT 15.6 sec AFP 90 ng/mL Cl 101 meq/L PTT 45.1 sec HBsAg (-) HCO3 25 meq/L NH3 250µg/dL HIV (-) BUN 12 mg/dL AST 107 IU/L Anti-HCV (-) Cr .6 mg/dL ALT 86 IU/L HCV RNA 2.8 million/mL Glu 90 mg/dL Alk phos 224 IU/L ANA (-) Hb 14.0 g/dL Bilirubin 2.4 mg/dL Fe 75 µg/dL Hct 39.7% Protein 6.6 g/dL Ferratin 200 ng/mL MCV 90 fL Alb 2.7 g/dL Transferrin saturation 38% Plt 34500/mm3 Ca 8.5 mg/dL Ceruplasmin 37 mg/dL Inherited, too much copper in body’s tissues, normal limits

11. Why can autoimmune hepatitis and primary biliary cirrhosis be ruled out as contributing factors to this patient’s condition? Because both are autoimmune disorders but the ANA was normal

Transferrin saturation 38% Plt 34500/mm3 Ca 8.5 mg/dL Na 135 meq/L WBC 4700/mm3 Mg 1.7 mg/dL K 3.5 meq/L PT 15.6 sec AFP 90 ng/mL Cl 101 meq/L PTT 45.1 sec HBsAg (-) HCO3 25 meq/L NH3 250µg/dL HIV (-) BUN 12 mg/dL AST 107 IU/L Anti-HCV (-) Cr .6 mg/dL ALT 86 IU/L HCV RNA 2.8 million/mL Glu 90 mg/dL Alk phos 224 IU/L ANA (-) Hb 14.0 g/dL Bilirubin 2.4 mg/dL Fe 75 µg/dL Hct 39.7% Protein 6.6 g/dL Ferratin 200 ng/mL MCV 90 fL Alb 2.7 g/dL Transferrin saturation 38% Plt 34500/mm3 Ca 8.5 mg/dL Ceruplasmin 37 mg/dL Both are autoimmune dieases, but tested negative for ANAs

12. Osteoporosis? Na 135 meq/L WBC 4700/mm3 Mg 1.7 mg/dL K 3.5 meq/L PT 15.6 sec AFP 90 ng/mL Cl 101 meq/L PTT 45.1 sec HBsAg (-) HCO3 25 meq/L NH3 250µg/dL HIV (-) BUN 12 mg/dL AST 107 IU/L Anti-HCV (-) Cr .6 mg/dL ALT 86 IU/L HCV RNA 2.8 million/mL Glu 90 mg/dL Alk phos 224 IU/L ANA (-) Hb 14.0 g/dL Bilirubin 2.4 mg/dL Fe 75 µg/dL Hct 39.7% Protein 6.6 g/dL Ferratin 200 ng/mL MCV 90 fL Alb 2.7 g/dL Transferrin saturation 38% Plt 34500/mm3 Ca 8.5 mg/dL Ceruplasmin 37 mg/dL

13. Evidence for ascites Na 135 meq/L WBC 4700/mm3 Mg 1.7 mg/dL K 3.5 meq/L PT 15.6 sec AFP 90 ng/mL Cl 101 meq/L PTT 45.1 sec HBsAg (-) HCO3 25 meq/L NH3 250µg/dL HIV (-) BUN 12 mg/dL AST 107 IU/L Anti-HCV (-) Cr .6 mg/dL ALT 86 IU/L HCV RNA 2.8 million/mL Glu 90 mg/dL Alk phos 224 IU/L ANA (-) Hb 14.0 g/dL Bilirubin 2.4 mg/dL Fe 75 µg/dL Hct 39.7% Protein 6.6 g/dL Ferratin 200 ng/mL MCV 90 fL Alb 2.7 g/dL Transferrin saturation 38% Plt 34500/mm3 Ca 8.5 mg/dL Ceruplasmin 37 mg/dL Alb low, protein high – indicative of the presence of ascites

14. Hepatic encephalopathy? Na 135 meq/L WBC 4700/mm3 Mg 1.7 mg/dL K 3.5 meq/L PT 15.6 sec AFP 90 ng/mL Cl 101 meq/L PTT 45.1 sec HBsAg (-) HCO3 25 meq/L NH3 250µg/dL HIV (-) BUN 12 mg/dL AST 107 IU/L Anti-HCV (-) Cr .6 mg/dL ALT 86 IU/L HCV RNA 2.8 million/mL Glu 90 mg/dL Alk phos 224 IU/L ANA (-) Hb 14.0 g/dL Bilirubin 2.4 mg/dL Fe 75 µg/dL Hct 39.7% Protein 6.6 g/dL Ferratin 200 ng/mL MCV 90 fL Alb 2.7 g/dL Transferrin saturation 38% Plt 34500/mm3 Ca 8.5 mg/dL Ceruplasmin 37 mg/dL Worsening of brain function that occurs when liver is no longer able to remove toxic substances in the blood, primary indicator is ammonia Nh3 VERY high, 18-60ug/dL

15. Grade this encephalopathy? 0: Minimal HE Hard to detect, changes in memory, concentration and temper are minimal 1: Mild HE Short attention span, noticeable mood changes, sleep problems 2: Moderate HE Forgetfulness worsens, lethargic, exhibit inappropriate behavior, slurred speech, and difficulty with mental tasks 3: Severe HE Confused as to where you are or what day it is, extremely sleepy, unable to do basic mental tasks, extremely anxious, act strangely 4: Coma Unconscious, slip into coma

16. Patient’s CTP score? Na 135 meq/L WBC 4700/mm3 Mg 1.7 mg/dL K 3.5 meq/L PT 15.6 sec AFP 90 ng/mL Cl 101 meq/L PTT 45.1 sec HBsAg (-) HCO3 25 meq/L NH3 250µg/dL HIV (-) BUN 12 mg/dL AST 107 IU/L Anti-HCV (-) Cr .6 mg/dL ALT 86 IU/L HCV RNA 2.8 million/mL Glu 90 mg/dL Alk phos 224 IU/L ANA (-) Hb 14.0 g/dL Bilirubin 2.4 mg/dL Fe 75 µg/dL Hct 39.7% Protein 6.6 g/dL Ferratin 200 ng/mL MCV 90 fL Alb 2.7 g/dL Transferrin saturation 38% Plt 34500/mm3 Ca 8.5 mg/dL Ceruplasmin 37 mg/dL Child-Pugh Score used to assess prognosis of chronic liver disease, mainly cirrhosis, look at these lab rseults, then grade ascites and encephatholgy grade to obtain the score (online calculator) Score = 12, Class C, life expectancy of 1-3 years

17. One year survival? Based on his class C CTP score, this patient has a 45% probability of surviving another year

18. Does the patient have any signs of dehydration or hepatorenal syndrome?

Transferrin saturation 38% Plt 34500/mm3 Ca 8.5 mg/dL Na 135 meq/L WBC 4700/mm3 Mg 1.7 mg/dL K 3.5 meq/L PT 15.6 sec AFP 90 ng/mL Cl 101 meq/L PTT 45.1 sec HBsAg (-) HCO3 25 meq/L NH3 250µg/dL HIV (-) BUN 12 mg/dL AST 107 IU/L Anti-HCV (-) Cr .6 mg/dL ALT 86 IU/L HCV RNA 2.8 million/mL Glu 90 mg/dL Alk phos 224 IU/L ANA (-) Hb 14.0 g/dL Bilirubin 2.4 mg/dL Fe 75 µg/dL Hct 39.7% Protein 6.6 g/dL Ferratin 200 ng/mL MCV 90 fL Alb 2.7 g/dL Transferrin saturation 38% Plt 34500/mm3 Ca 8.5 mg/dL Ceruplasmin 37 mg/dL Dehydration look at electrolytes, and hepatorenal syndrome = rapid deterioration in kidney function inidividuals with cirrhosis, look at BUN and Cr

19. The patient’s primary care provider has decided to conduct extensive clinical studies for the diagnosis of liver cancer. Which single abnormal laboratory value has raised a concern that hepatocellular carcinoma may have developed?

Transferrin saturation 38% Plt 34500/mm3 Ca 8.5 mg/dL Na 135 meq/L WBC 4700/mm3 Mg 1.7 mg/dL K 3.5 meq/L PT 15.6 sec AFP 90 ng/mL Cl 101 meq/L PTT 45.1 sec HBsAg (-) HCO3 25 meq/L NH3 250µg/dL HIV (-) BUN 12 mg/dL AST 107 IU/L Anti-HCV (-) Cr .6 mg/dL ALT 86 IU/L HCV RNA 2.8 million/mL Glu 90 mg/dL Alk phos 224 IU/L ANA (-) Hb 14.0 g/dL Bilirubin 2.4 mg/dL Fe 75 µg/dL Hct 39.7% Protein 6.6 g/dL Ferratin 200 ng/mL MCV 90 fL Alb 2.7 g/dL Transferrin saturation 38% Plt 34500/mm3 Ca 8.5 mg/dL Ceruplasmin 37 mg/dL AFP-alpha-fetoprotein, tumor marker, helps in the diagnosis of liver cancers