PATH - General Flashcards

Question

Ileus

Answer 1

Intestinal hypomotility without obstruction-->constipation and DEC flatus Associated with abdominal surgeries, opiates, hypokalemia, sepsis.

Answer 2

In *cystic fibrosis, meconium plug obstructs intestine, preventing stool passage at birth.

Answer 3

Seen in *premature, *formula-fed infants with *immature immune system Necrosis of intestinal mucosa (primarily *colonic) with possible perforation, which can lead to *pneumatosis intestinalis, free air in abdomen, portal venous gas.

Answer 4

diffuse bridging fibrosis (via stellate cells) and regenerative nodules disrupt normal architecture of liver; INV risk for hepatocellular carcinoma (HCC) Etiologies include alcohol (60–70% of cases in the US), nonalcoholic steatohepatitis, chronic viral hepatitis, autoimmune hepatitis, biliary disease, genetic/metabolic disorders.

Answer 5

INC pressure in portal venous system Etiologies include cirrhosis (most common cause in Western countries), vascular obstruction (portal vein thrombosis, Budd-Chiari syndrome), schistosomiasis.

Answer 6

Rare, often fatal childhood hepatic encephalopathy mitochondrial abnormalities, fatty liver (microvesicular fatty change), hypoglycemia, vomiting, hepatomegaly, coma Associated with viral infection (especially VZV and influenza B) that has been *treated with aspirin *Avoid aspirin in children, except in those with *Kawasaki disease.

Answer 7

Thrombosis or compression of hepatic veins with *centrilobular congestion and necrosis-->congestive liver disease Associated with hypercoagulable states, polycythemia vera, postpartum state, HCC May cause *nutmeg liver (mottled appearance).

Answer 8

Misfolded gene product protein aggregates in hepatocellular ER-->cirrhosis with PAS⊕ globules in liver Codominant trait In lungs, DEC α1-antitrypsin-->uninhibited elastase in alveoli-->DEC elastic tissue-->panacinar emphysema.

Answer 9

Abnormal yellowing of the *skin and/or *sclera due to *bilirubin deposition. Hyperbilirubinemia 2° to INC production or DEC disposition (impaired hepatic uptake, conjugation, excretion)

Answer 10

Hemolytic physiologic (newborns) Crigler-Najjar Gilbert syndrome

Answer 11

*Biliary tract obstruction: gallstones, cholangiocarcinoma, pancreatic or liver cancer, liver fluke Biliary tract disease: - 1° sclerosing cholangitis - 1° biliary cirrhosis Excretion defect: - Dubin-Johnson syndrome - Rotor syndrome

Answer 12

Hepatitis | cirrhosis

Answer 13

At birth, immature *UDP-glucuronosyltransferase-->unconjugated hyperbilirubinemia-->jaundice/ kernicterus (bilirubin deposition in brain, particularly basal ganglia) Occurs after first 24 hours of life and usually resolves without treatment in 1–2 weeks TX: phototherapy (non-UV)

Answer 14

Recessive mutations in hepatocyte *copper-transporting ATPase (*ATP7B gene; chromosome 13)-->inadequate copper excretion into bile and blood (*DEC serum ceruloplasmin, INC urine copper). Copper accumulates, especially in liver, brain, cornea, kidneys, joints. Presents before age 40 with liver disease, neurologic disease (dysarthria, dystonia, tremor, parkinsonism), psychiatric disease, **Kayser-Fleischer rings (deposits in Descemet membrane of cornea), hemolytic anemia, renal disease (eg, Fanconi syndrome). TX: chelation with penicillamine or trientine, oral zinc.

Answer 15

Recessive mutations in *HFE gene (C282Y>H63D, chromosome 6)-->abnormal iron sensing and INC intestinal absorption (INC ferritin, INC iron, DEC TIBC-->INC transferrin saturation) *Iron accumulates, especially in liver, pancreas, skin, heart, pituitary, joints. *Hemosiderin (iron) can be identified on liver MRI or biopsy with Prussian blue stain Classic triad of cirrhosis, diabetes mellitus, skin pigmentation (“bronze diabetes”) Also causes dilated cardiomyopathy (reversible), hypogonadism, arthropathy (calcium pyrophosphate deposition) *HCC is common cause of death. TX: repeated phlebotomy, chelation with deferasirox, deferoxamine, oral deferiprone

Answer 16

May present with pruritus, jaundice, dark urine, light-colored stool, hepatosplenomegaly Typically with cholestatic pattern of LFTs (INC conjugated bilirubin, INC cholesterol, INC ALP).

Answer 17

Biliary tract disease Unknown cause of concentric *“onion skin” bile duct fibrosis-->alternating strictures and dilation with *“beading” of intra- and extrahepatic bile ducts Classically in *middle-aged men with IBD Associated with *ulcerative colitis. p-ANCA ⊕ INC IgM Can lead to 2° biliary cirrhosis. INC risk of cholangiocarcinoma and gallbladder cancer

Answer 18

Biliary tract disease Autoimmune reaction-->lymphocytic infiltrate+granulomas-->destruction of *intralobular bile ducts. Classically in *middle-aged women. Anti-mitochondrial antibody⊕ INC IgM ``` Associated with other autoimmune conditions (eg, Sjögren syndrome, Hashimoto thyroiditis, CREST, rheumatoid arthritis, celiac disease). ```

Answer 19

Biliary tract disease Extrahepatic biliary obstruction-->INC pressure in intrahepatic ducts-->injury/fibrosis and bile stasis. Patients with known obstructive lesions (gallstones, biliary strictures, pancreatic carcinoma). May be complicated by ascending cholangitis.

Answer 20

INC cholesterol and/or bilirubin, DEC bile salts, and gallbladder stasis all cause stones 2 types of stones: *Cholesterol stones (radiolucent with 10–20% opaque due to calcifications) -80% of stones -Associated with obesity, Crohn disease, advanced age, estrogen therapy, multiparity, rapid weight loss, Native American origin. *Pigment stones (black=radiopaque, Ca2+ bilirubinate, hemolysis; brown=radiolucent, infection) -seen in patients with Crohn disease, chronic hemolysis, alcoholic cirrhosis, advanced age, biliary infections, total parenteral nutrition (TPN). Risk factors (4 F’s): 1. Female 2. Fat 3. Fertile (pregnant) 4. Forty Most common complication is *cholecystitis; also acute pancreatitis, ascending cholangitis. Charcot triad of *cholangitis: -Jaundice -Fever -RUQ pain

Answer 21

Acute or chronic inflammation of gallbladder usually from cholelithiasis (stone at neck of gallbladder with gallbladder wall thickening) Gallstones most commonly blocking the cystic duct-->2° infection rarely acalculous due to ischemia and stasis, or 1° infection (CMV) Murphy sign⊕ INC ALP if bile duct becomes involved (ascending cholangitis)

Answer 22

Calcified gallbladder due to chronic cholecystitis usually found incidentally on imaging TX: *prophylactic cholecystectomy due to high rates of gallbladder cancer (mostly adenocarcinoma).

Answer 23

Autodigestion of pancreas by pancreatic enzymes Diagnosis by 2 of 3 criteria: -acute epigastric pain often radiating to the back -*INC serum amylase or lipase (more specific) to 3× upper limit of normal -characteristic imaging findings Complications: pseudocyst (lined by granulation tissue, not epithelium), necrosis, hemorrhage, infection, organ failure (ARDS, shock, renal failure), hypocalcemia (precipitation of Ca2+ soaps).

Answer 24

Chronic inflammation, atrophy, calcification of the pancreas Mutations in CFTR (cystic fibrosis) can cause chronic pancreatic insufficiency Major causes are alcohol abuse and idiopathic Can lead to pancreatic insufficiency-->Steatorrhea, fat-soluble vitamin deficiency, diabetes mellitus