Pathology- Misc liver and GI diseases

Question 1

what can cause conjugated/direct jaundice?

Answer 1

• obstruction, hepatitis, dubin-johnson

Question 2

what can cause unconjugated/indirect jaundice?

Answer 2

• hemolysis, hepatitis, gilbert

Question 3

why is jaundice common in neonates?

Answer 3

d/t liver immaturity (hepatic machinery for conjugating and excreting bilirubin not fully operational until 2 weeks old)

Question 4

what can severe hyperbiliruninemia do and how can it be tx?

Answer 4

• injures brain and tx with UV lights

Question 5

what is the most common cause of death from liver disease in early childhood?

Answer 5

• biliary atresia- progressive inflammatory and firbosing disease of extrahepatic bile duct –> complete obstruction of bile flow caused by destruction of extrahepatic bile ducts
• causes 1/3 of neonatal choelstasis.

Question 6

what is this?

Answer 6

biliary atresia- fibrosing obliterating an extrahepatic bile duct

Question 7

How do patients with bilairy atresia present?

Answer 7

• jaundiced at 3-6 weeks of age
• have conjugated hyperbilirubninemia

Question 8

lab values for biliary atretic patient?

Answer 8

• disproportionately high GGT
• mild-mod high ALT and AST
• poor prognosis improved by early surgery (hepatoportoenterostomy) but many still need liver transplant

**alk phos in children is more from bone so less useful in diagnosing liver disease.

Question 9

Alagille syndrome presentation?

Answer 9

• neonatal jaundice, pruritis, and cholestasis plus a bunch of other stuff like triangular face etc.
• it’s a syndromic paucity of intrahepatic bile ducts

Question 10

Genetics of alagille syndrome (arteriohepatic dysplasia)?

Answer 10

• AD
• 70% sporadic new mutations
• d/t mutation in JAGGED 1 gene for ligand for NOTCH 1 receptor

Question 11

what is this?

Answer 11

alagille syndrome. trichrome stain of portal triad blood vessels but no bile ducts

• pronosis for prolonged survival good unless misdiagnosed and surgerized.

Question 12

Dubin-Johnson sydrome

Answer 12

• hereditary conjugated hyperbiliruniemia
• defective excretion of bilirubin conjugates and other organic anions across hepatocyte canalicular membrane

brown black pigment in liver = lysosomes containing polymers of epinephrine metabolites

Question 13

presentation of dubin-johnson syndrome?

Answer 13

• usually asymptomatic but may have recurring or fluctuating chronic jaundice
• normal life expectancy

Question 14

Gilbert syndrome

Answer 14

• AR deficiency of uridinge diphosphate glucuronyltransferase (UGT) enzyme that conjugates bilirubin

males > females

Question 15

presentation of gilbert syndrome?

Answer 15

young adult w/ mild primarily unconjugated hyperbilirubinemia (brought on by febrile illness, physical exertion, fasting, or hemolysis) and no other manifestations

Question 16

prognosis of gilbert syndrome?

Answer 16

• benign prognosis may even be better than avg bc pts with gilbert syndrome run a slightly higher bilirubin than avg and incidence of colon cancer mortality in general and atherosclerotic heart disease are inversely proportional to bilirubin level so good!!

Question 17

Primary biliary cirrhosis

Answer 17

• autoimmune slowly progressive cholestatic disease
• female > male
• late to middle aged women

Question 18

presentation of primary biliary cirrhosis?

Answer 18

pruritis, fatigue, hepatomegaly

• elevated alk phosphate
• eventually get jaundice, xanthomas, steatorrhea

Question 19

pathology of primary biliary cirrhosis?

Answer 19

• dense lymphocytic infiltrate in and around interlobular bile ducts w/ granulomas and bile duct destruction and loss and progressive portal fibrosis

Question 20

anti-mitochondrial antibodies associated with what?

Answer 20

primary biliary cirrhosis

Question 21

what is this?

Answer 21

primary biliary cirrhosis- end stage cirrhosis w/ severe cholestasis

Question 22

budd chiari syndrome

Answer 22

• portal htn d/t thrombosis of hepatic veins

Question 23

manifestation of budd- chiari syndrome

Answer 23

hepatomegaly, ascites, abd pain

Question 24

what causes budd-chiari syndrome?

Answer 24

• polycythemia vera, other myeloproliferative disease, pregnancy, postpartum state, OCP, paroxysmal nocturnal hemoglobunuria, abd cancer esp within the liver itself

Question 26

which lobe of liver is budd-chiari syndrome associated with?

Answer 26

• hypertrophy of caudate lobe
• fatal if develops acutely
• 50% 5 year survival if develops chronically

Question 27

ascending cholangitis

Answer 27

infection of intrahepatic biliary ducts

• acute vs chronic

acute d/t bacteria like (Ecoli, klebsiella, clostridium, bacteriodes, ENTEROBACTER, ENTEROCOCCUS)

chronic d/t parasites like (cryotsporidium, fasciola hepatica, schistosoma, clonorchis sinensis)

Question 28

what is this?

Answer 28

• asending cholangitis. small tan areas.

Question 29

symptoms, patholgy, tx of ascending bacterial cholangitis?

Answer 29

• symptoms- fever, chills, abd pain, and jaundice
• path- prurulent bile fills and distends bile ducts, can get liver abscess
• tx- relief of obstruction and antibiotics

Question 30

what is this?

Answer 30

• cholangitis- bile ductules (Arrows) w/ luminal acute inflammatory cells and damage and hepatocytes w. intracellular cholestasis.

Question 31

sigmoid colonic diverticular disease

Answer 31

• pseudo diverticular outpouchings of colonic mucosa and submucosa…doesnt involve all three layers
• colonic diverticula is usually in those over 60
• increased intraluminal pressure d/t exaggerated peristaltic contractions
• low fiber diets –> reduce soft bulky stool to small rock hard like pellets

Question 32

what is this?

Answer 32

colonic diverticula- flask like outpouchings alongside teaenia coli often i nregular distribution

Question 33

what is this?

Answer 33

sigmoid colonic diverticulosis- thin wall composed of flattened atrophic ucosa compressed submucosa and attenuated or more often totally absent musclaris propria.

hypertriphy of circular layer of muscularis propria in affected bowel segment around the diverticula is common