Overview. The Liver and Biliary System 10 cases to guide you. Liver Function. Background/Patterns. Case #1

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1 Overview The Liver and Biliary System 10 cases to guide you Chapy Venkatesan, MD Department of Medicine VCU School of Medicine Inova Campus 1 To be familiar with the approach to differential diagnosis and management hepatobiliary disease will not be all-encompassing Case and image based Interactive Hope for you to get three learning points out of this Will review what we covered at the end motivate/challenge you to read on your own Show you that medicine is FUN! 2 Background/Patterns Liver Function Hepatocellular - ALT, AST > 3x uln; AP < 2x uln Cholestatic/Obstructive - AP > 3x uln; AST, ALT < 2x uln Mixed - ALT and AP > 2-3x uln Bili variable in all Alcohol - AST>ALT in 2-3:1, AST < 300, ALT < 100 ratios outside of these ranges in someone who drinks EtOH suggests a concomitant disorder PT/INR Albumin Glucose 3 4 Case #1 42 year old male with recent onset jaundice Vague epigastric/ruq discomfort No IVDU, transfusions, unprotected sex Recently taking acetaminophen 3g/day for a viral URI BP - normal Alert and oriented, jaundiced INR - 4.2; Cr - 3.0; AST - 14,380; ALT - 6,740; Alk Phos - 70; tb mmanual/plates/125pla5.jsp 6 1

2 photoalbum/ph93.htm UpToDate What is the most likely underlying risk factor for the patient s acute liver disease? alcoholic cirrhosis What is the most likely diagnosis for the acute liver disease? acetaminophen hepatotoxicity What is the therapy/antidote? N-acetylcysteine What are other causes of transaminases in the 1,000 s acute viral hepatitis (risk factors), ischemic hepatitis/shock liver (hypotension, diminished cardiac output) N-acetylcysteine is a substitute Induced by alcohol TOXIC No synthesis due to liver dz 11 Current Diagnosis & Treatment in Gastroenterology - 2nd Ed. (2003) 12 2

3 Rumack BH, Matthew H: Acetaminophen poisoning and toxicity. Pediatrics 1975;55: Lee 333 (17): 1118, Table 2 October 26, NEJM 14 Case #2 24 y.o. female prostitute, IV drug user presents with headache, malaise, fever, arthralgias, and nausea followed by jaundice Hepatomegaly and jaundice on exam AST - 850, ALT - 1,250; bili - 4.0; AP - normal Anti-HAV IgM, Anti-HCV, HBsAg, HBsAb all negative What is the best serologic test to make the diagnosis? HBcAb IgM What is the pattern of vaccination versus hepatitis B; remote hepatitis B? + HBsAb, neg HBcAb; +HBsAb, +HBcAb The patient s liver enzymes fall and then peak 4 weeks later, what is the best way to make the diagnosis? Anti HDV or HDV RNA Twenty years later, the HBsAg is positive. There is no evidence of cirrhosis. The alk phos and AFP are elevated and the pt has fever and polycythemia. What is the most likely diagnosis? hepatocellular carcinoma What are other risk factors for hepatoma? 2004 UpToDate

4 Risk factors for hepatoma Case #3 Cirrhosis of any etiology Alcohol Hepatitis B (does not have to progress through cirrhosis) Hepatitis C Hemochromatosis 20 y.o. female with jaundice for one week Had nausea and abdominal pain for three weeks Friends brought her in d/t hallucinations You note a tremor on exam ALT -170, AST -95; AP -120; bili-8.0 Retic %; LDH , Hct - 33, Coombs negative What are blood and urine tests that support this diagnosis? Wilson s disease - low ceruloplasmin, high 24 hr urine copper What is the medical therapy? penicillamine may need liver transplant - especially if presenting with fulminant hepatic failure (acute liver failure with hepatic encephalopathy) Case #4 52 y.o. white female (nondrinker, diabetic w/ A1C - 9.2%)with incidentally noted ALT - 75, AST - 52, AP - 65, bili normal BMI - 36 LDL - 245, TG U/S demonstrates a hyperechoic liver hep B, C, iron studies, ceruloplasmin, alpha one antitrypsin level, antimitochondrial antibody, anti-smooth muscle antibodies negative What is the best treatment? pt has NASH / NAFLD gradual weight loss and treatment of DM, hyperlipid / triglyceridemia What are other causes of chronically elevated liver enzymes?

5 Causes of Chronically Elevated Aminotransferase Levels History Hypogonadism, Arthritis, DM, CHF Fe studies, genetic studies CK, aldolase, TSH Polyclonal gammopathy, Anti Sm m Ab, Anti LKM Ab Serology Malabsorption, Fe def, osteoporosis, misdx with IBS Obesity, DM, hyperlipidemia; exclusion of other causes KF rings; neuro, Psyc, Hepatic, Heme; ceruloplasmin, urine Cu Emphysema, FmHx liver dz, panniculitis, level, genotype 25 Associations Chronic hepatitis B polyarteritis nodosa (intestinal ischemia, renal failure, vasculitis) membranous GN, MPGN Chronic hepatitis C DM porphyria cutanea tarda lichen planus cryoglobulinemia, MPGN, membranous GN 26 NS14_300.jpg 2004 UpToDate SLE/rbc_cast.jpg 2004 UpToDate

6 Case #5 43 y.o. female with fatigue and pruritis at all times Had a recent pathologic fracture and Z score on DEXA was ALT - 70, Alk Phos UpToDate Case #6 What is the most likely diagnosis? primary biliary cirrhosis What are sources of alk phos? placenta, bile duct epithelium, and bone Paget s disease of bone, bone metastases, primary hyperparathyroidism, Vitamin D deficiency 25 y.o. male returned from a trip to India three months ago Develops two weeks of fever and RUQ pain without diarrhea WBC 14K What is the next diagnostic test to order? amebic liver abscess E. Histolytica serology no aspiration risk of amebic peritonitis, inadvertent puncture of an echinococcal cyst, can use if no better on therapy, or to exclude other diagnoses Next therapy? metronidazole When would you suspect a pyogenic liver abscess and aspirate the abscess? jaundiced, septic, h/o abd surgery or biliary disease, picture not typical of amebic TF/VIR/VIR5/Slide2.JPG

7 Case #7 63 y.o. female with acute onset of fever, chills, n/v T F, P - 120, RR - 28, BP - 80/50 Pt is jaundiced with scleral icterus on exam AST and ALT - 125, Alk Phos - 800, bili , dbili U/S shows gallstones and CBD dilatation Hypotension persists despite 4L of NS 37 What is the next step in addition to supportive care and broad spectrum antibiotics? ERCP for drainage and relief of the obstruction can do abx only with ERCP electively unless: no better over 1st 24 hours, fever > 103, persistent pain, hypotension despite resuscitation 38 Case #8 22 y.o. with ulcerative colitis presents with jaundice, alk phos of 500, ALT of _gfx/pscholangitis.jpg 40 What is the diagnosis? Primary sclerosing cholangitis most have UC, most of UC do not have PSC What is the concern if the patient develops a dominant biliary stricture? Cholangiocarcinoma

8 Case #9 45 y.o. male admitted with an acute MI Found to have a total bili of 6.0, dbili One year later, the bili decreases to 2.5, dbili The remainder of the liver panel is normal, as is the CBC, retic count and peripheral smear 43 What is the next diagnostic test? NONE What is the therapy? NONE Gilbert s syndrome Indirect hyperbilirubinemia hemolysis, ineffective erythropoiesis 44 Case #10A 27 y.o. female, G1P0; at 38 weeks Presents with acute onset N/V, jaundice, encephalopathy AST and ALT ~ 700; Alk Phos - nl; NH3-95; Plt - 45K; PT/PTT - elevated; d-dimer - elevated; fibrinogen - low What is the diagnosis? acute fatty liver of pregnancy can recur with subsequent pregnancies stabilize the mother and deliver the fetus Case #10B 26 y.o. female, G4P3, 34 wks RUQ pain, N/V BP - 145/90; 2+ edema Hct - 24; Plt - 75K; bili (dbili - 0.5); ALT - 300; LDH ; 4+ urine protein; Uric acid

9 Diagnosis? Case #10C HELLP Syndrome 28 y.o. G1P0 living in Pakistan The pt and her husband develop an illness characterized by jaundice, N/V, malaise, anorexia Her husband s illness is self-limited She develops encephalopathy, ALT , and dies Diagnosis? Other pregnancy tidbits Hepatitis E - increased mortality and FHF in pregnancy Hyperemesis gravidarum causes elevations in the transaminases - usually below 300 Benign Recurrent Intrahepatic Cholestasis of Pregnancy - intractable pruritis, elevated alk phos, can recur with subsequent pregnancies What did we cover? Patterns of liver panel Physical findings of cirrhosis Acetaminophen hepatotoxicity and other forms of drug-induced liver disease Causes of AST and ALT > 1000 Drug induced liver disease Hepatitis B serologies Hepatocellular carcinoma RF s What did we cover? Wilson s disease NASH/NAFLD Chronically elevated aminotransferases Associations of hepatitis B and C PBC Amebic liver abscess Cholangitis

10 What did we cover? PSC Cholangiocarcinoma Gilbert s syndrome Pregnancy and liver disease 55 10