GI/ Hepatobiliary Step 1 Review

1. GI/HepatobiliaryStep 1 Review UMMSM Board Review Series Thursday, February 23rd, 2012 Graham Ingalsbe gingalsbe@med.miami.edu

2. First Aid GI Content • Anatomy – Peritoneal structures, ligaments, vasculature, histology, inguinal canal • Physiology – Hormones, GI cell bio, digestion/absorption, bilirubin • GI Pathology – Esophageal, absorption, stomach, IBD, intestinal/colonic, cancers • Hepatobiliary Pathology – Cirrhosis, liver disease, jaundice, hereditary conditions, biliary disease, gallstones, pancreatitis • Pharmacology – PUD, IBD, anti-emetics

3. GI Topics covered in FA • Salivary gland tumors • Achalasia • GERD • Varices • Esophagitis • Mallory-Weiss • Boerhaave • Esophageal Strictures • Plummor-Vinson • Barrett’s • Esophageal Cancers • Tropical Sprue • Whipple’s • Celiac • Disaccharidase deficiency • Abetalipoproteinemia • Pancreatic insufficiency • Celiac • Acute gastritis • Chronic gastritis • Menetrier’s • Stomach Cancers • Gastric PUD • Duodenal PUD • IBD • IBS • Appendicitis • Diverticulosis • Diverticulitis • Zenker’s • Meckel’s • Intussusception • Volvulus • Hirschsprung’s • Duodenal atresia • Meconium • NEC • Ischemic Colitis • Adhesions • Angiodysplasia • Colonic polyps • Colorectal cancer • Carcinoid • Cirrhosis/Portal HTN • Reye’s • Hepatic Steatosis • ETOH hepatitis • ETOH Cirrhosis • HCC • Nutmeg liver • Budd-Chiari • A1AT • Neonatal Jaundice • Jaundice • Gilbert’s • Crigler-Najjar • Dubin-Johnson • Wilson’s • Hemochromatosis • Secondary Biliary Cirrhosis • PBC • PSC • Gallstones • Cholecystitis • Acute/Chronic Pancreatitis • Pancreatic Cancer • Pharm

4. Jaundice • Hepatitis Serologies • Inflammatory Bowel Disease • The C Word • Questions

5. Jaundice • Due to a disruption in normal heme metabolism • RBC breakdown, transport, hepatic uptake, excretion

6. Jaundice Macrophages: RBC -> Heme -> Unconjugatedbili In the bloodstream: Unconjbili binds albumin (Indirect bilirubin) To the Liver: Uptake, then UDP glucoronyltransferase conjugates with glucuronic acid = Direct Bilirubin (now water soluble) From the hepatocytes to the gut in bile: Urobilinogen in the gut either is excreted in feces (80%, ‘stercobilin’) or reabsorbed (20%, most sent back to the liver, trace amount excreted in urine, ‘urobilin’)

8. Jaundice • Problems with overproduction, absorption, conjugation, or excretion • Often presented in review books as tables of either hepatocellular, obstructive, hemolytic, or mixed

10. Jaundice

11. Jaundice

12. Liver Lab Lingo • AST/ALT: Hepatic enzymes, liver cell necrosis, AST>ALT in ETOH hepatitis • GGT: Intra or extrahepatic bile flow obstruction • Alkaline Phos: Biliary obstruction, if GGT also increased it’s hepatic cholestasis • Albumin: made by liver, hypo in severe dz • PT: Coag factors made in liver, increased coag time = severe hepatic dz • Ammonia: metabolized in urea cycle • Serum IgM, Anti-MitochondiralAb: Primary Biliary Cirrhosis • Anti-Smooth Muscle Ab, ANA: Autoimmune hepatitis • Alpha fetoprotein: Hepatocellular carcinoma (yolk sac tumors)

14. GI Cancers • Esophageal • ABCDEFGH (Adeno in US, squamous worldwide) • Alcohol/Achalasia, Barrett’s, Cigarettes, Diverticuli, Esophageal web, Familial, GERD, Hot Dogs • Stomach • Adeno, Signet ring cells, Virchow’s node, Krukenberg met to ovaries, Linitisplastica

15. GI Cancers • Pancreatic • CEA, CA 19-9, cigarettes and chronic pancreatitis, Courvoisier’s, Trousseau’s • HCC • AFP tumor marker, Hep B, C, Wilson’s, Hemochromatosis, A1AT, ETOH Cirrhosis, aflatoxin

16. GI Cancers • Colon • Pathogensis: • Normal -> lose APC -> Kras mutation -> lose p53 -> CA • 3rd MC Cancer, 3rd most deadly. • FAP (APC gene), Gardner’s, Turcot, HNPCC • IBD, Strep Bovis, tobacco, Peutz-Jeghers • Screen all >50 YO • Carcinoid • Neuroendocrine tumor, produce 5-HT, can cause classic syndrome of wheezing, murmurs, diarrhea, flushing

17. Catchphrases • Epigastric pain worse with food: gastric PUD • Epigastric pain relieved with food: duodenal PUD • Acute pancreatitis: Pain radiating to the back • GET SMASHED! • Gallstones, ETOH, Trauma, Steroids, Mumps, Malignancy, Autoimmune, Scorpion, HyperCa, HyperTG, ERCP, Drug • Meckel’s: 2 inches, 2 feet, 2% • Gallstones: The 4 F’s

19. A 52-year-old man comes to the emergencydepartment because he has had vomiting, nausea,and abdominal pain for the past 12 hours. Hesays he attempted suicide 3 days ago by "takingeverything in the medicine cabinet." He wasstuporous for approximately 12 hours after theoverdose but felt better the following day. At thistime, he has jaundice and pain in the right upperquadrant. Which of the following drugs is mostlikely to have caused the pain, vomiting, andjaundice? (A) Acetaminophen (B) Aspirin (C) Cimetidine (D) Diphenhydramine (E) Triazolam

20. A 25-year-old man comes to the physicianbecause of progressive weakness and anincreasingly protuberant abdomen during thepast 3 years. Physical examination showssplenomegaly. His hematocrit is 28%, andplatelet count is 20,000/mm3. A biopsy specimenof bone marrow shows accumulation of lipidladenmacrophages. Glucocerebroside hasaccumulated in the patient's reticuloendothelialcells. Inheritance of mutantalleles most likely caused impairment of whichof the following enzyme activities in thispatient? (A) Ceramidase (B) α-Galactosidase (C) β-Glucosidase (D) Hexosaminidase (E) α-L-Iduronidase (F) Sphingomyelinase

21. A 6-week-old male infant is brought to theemergency department because of a swollenabdomen. He is refusing to eat and has not had abowel movement for 3 days. His mother saysthat he had constipation since birth and was keptin the hospital an extra day after birth because ofdelayed passage of stool. Physical examinationshows abdominal distention. An x-ray of theabdomen shows distended loops of proximalbowel with an abrupt narrowing to a smallcaliber of the distal 15-cm segment of colon.Which of the following pathologic findings ismost likely in a biopsy specimen of the distalrectum in this patient? (A) Absent myenteric ganglion cells (B) Abundant inspissated mucus (C) Hypertrophy of the muscle wall (D) Nodular lymphoid hyperplasia (E) Transmuralcoagulative necrosis

22. An otherwise healthy 3-week-old boy is broughtto the physician's office because of jaundice anddark urine for the past 2 weeks. He hashepatomegaly, and his stools are loose, claycolored,and acholic. Serum conjugated bilirubinconcentration is increased. Which of thefollowing is the most likely cause of thehyperbilirubinemia? (A) Defect in cholesterol synthesis (B) Deficiency of glucuronosyltransferase (C) Hemolysis (D) Inflammation of the terminal ileum (E) Obstruction of the biliary system

23. A 4-year-old girl has the sudden onset ofabdominal pain and vomiting. She has a mass inthe right lower quadrant and hyperactive bowelsounds. A segment of resected bowel is shown inthe photograph. Which of the following is themost likely diagnosis? (A) Appendicitis (B) Intussusception (C) Meckeldiverticulum (D) Necrotizing enterocolitis (E) Strangulated hernia

24. A 76-year-old man comes to the emergencydepartment because of a 12-hour history of feverand left lower quadrant abdominal pain. He hasnot passed a stool for the past 36 hours. Histemperature is 38.3°C (100.9°F). A tender massis palpable in the left lower quadrant of theabdomen. Stool is negative for occult blood. Laboratory studies show: Hemoglobin 13 g/dLLeukocyte count 17,000/mm3(84% neutrophils) Platelet count 200,000/mm3Serum amylase 115 U/L Urinalysis 0 to 1 WBC/hpf An x-ray of the abdomen shows noabnormalities. The most likely diagnosis is an acute episode of which of the followingdisorders? (A) Cystitis (B) Diverticulitis (C) Infectious colitis (D) Ischemic colitis (E) Pyelonephritis

25. A 5-year-old girl is brought to the emergency department because of fever and severe abdominal pain. Acute appendicitis is diagnosed. In the examination room, she keeps her right hip flexed and resists active extension of the hip. The inflamed structure associated with these symptoms is most likely in contact with which of the following structures? (A) Abdominal wall and the externaloblique muscle (B) Obturatorinternus muscle (C) Psoas major muscle (D) Quadratuslumborum muscle (E) Transversusabdominis muscle

26. A 55-year-old man who has alcoholic cirrhosis isbrought to the emergency department because hehas been vomiting blood for 2 hours. He has a 2-month history of abdominal distention, dilatedveins over the anterior abdominal wall, andinternal hemorrhoids. Which of the followingveins is the most likely origin of thehematemesis? (A) Inferior mesenteric veins (B) Left gastric vein (C) Periumbilical veins (D) Superior rectal vein (E) Superior vena cava

27. A 30-year-old woman has anxiety about episodesofabdominal pain that have alternated withdiarrhea and constipation over the past year. Sheoften has these episodes when she is stressed ortired. Physical examination and laboratorystudies are within normal limits during theseepisodes. Which of the following is the mostlikely diagnosis? (A) Gastroenteritis (B) Generalized anxiety disorder (C) Hypochondriasis (D) Irritable bowel syndrome (E) Major depressive disorder (F) Somatization disorder