Pathology- Misc liver and GI diseases Flashcards
what can cause conjugated/direct jaundice?
- obstruction, hepatitis, dubin-johnson
what can cause unconjugated/indirect jaundice?
- hemolysis, hepatitis, gilbert
why is jaundice common in neonates?
d/t liver immaturity (hepatic machinery for conjugating and excreting bilirubin not fully operational until 2 weeks old)
what can severe hyperbiliruninemia do and how can it be tx?
- injures brain and tx with UV lights
what is the most common cause of death from liver disease in early childhood?
- 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.
what is this?
biliary atresia- fibrosing obliterating an extrahepatic bile duct
How do patients with bilairy atresia present?
- jaundiced at 3-6 weeks of age
- have conjugated hyperbilirubninemia
lab values for biliary atretic patient?
- 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.
Alagille syndrome presentation?
- neonatal jaundice, pruritis, and cholestasis plus a bunch of other stuff like triangular face etc.
- it’s a syndromic paucity of intrahepatic bile ducts
Genetics of alagille syndrome (arteriohepatic dysplasia)?
- AD
- 70% sporadic new mutations
- d/t mutation in JAGGED 1 gene for ligand for NOTCH 1 receptor
what is this?
alagille syndrome. trichrome stain of portal triad blood vessels but no bile ducts
- pronosis for prolonged survival good unless misdiagnosed and surgerized.
Dubin-Johnson sydrome
- 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
presentation of dubin-johnson syndrome?
- usually asymptomatic but may have recurring or fluctuating chronic jaundice
- normal life expectancy
Gilbert syndrome
- AR deficiency of uridinge diphosphate glucuronyltransferase (UGT) enzyme that conjugates bilirubin
males > females
presentation of gilbert syndrome?
young adult w/ mild primarily unconjugated hyperbilirubinemia (brought on by febrile illness, physical exertion, fasting, or hemolysis) and no other manifestations
prognosis of gilbert syndrome?
- 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!!
Primary biliary cirrhosis
- autoimmune slowly progressive cholestatic disease
- female > male
- late to middle aged women
presentation of primary biliary cirrhosis?
pruritis, fatigue, hepatomegaly
- elevated alk phosphate
- eventually get jaundice, xanthomas, steatorrhea
pathology of primary biliary cirrhosis?
- dense lymphocytic infiltrate in and around interlobular bile ducts w/ granulomas and bile duct destruction and loss and progressive portal fibrosis
anti-mitochondrial antibodies associated with what?
primary biliary cirrhosis
what is this?
primary biliary cirrhosis- end stage cirrhosis w/ severe cholestasis
budd chiari syndrome
- portal htn d/t thrombosis of hepatic veins
manifestation of budd- chiari syndrome
hepatomegaly, ascites, abd pain
what causes budd-chiari syndrome?
- polycythemia vera, other myeloproliferative disease, pregnancy, postpartum state, OCP, paroxysmal nocturnal hemoglobunuria, abd cancer esp within the liver itself
which lobe of liver is budd-chiari syndrome associated with?
- hypertrophy of caudate lobe
- fatal if develops acutely
- 50% 5 year survival if develops chronically
ascending cholangitis
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)
what is this?
- asending cholangitis. small tan areas.
symptoms, patholgy, tx of ascending bacterial cholangitis?
- 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
what is this?
- cholangitis- bile ductules (Arrows) w/ luminal acute inflammatory cells and damage and hepatocytes w. intracellular cholestasis.
sigmoid colonic diverticular disease
- 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
what is this?
colonic diverticula- flask like outpouchings alongside teaenia coli often i nregular distribution
what is this?
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
