Lecture 9 Flashcards

Question

alpha antityptin deficiency

Answer 1

- misfiled gene product protein aggregates in hepatocellular ER, cirrhosis with PAS positive globules in liver, in lungs, decrease in alpha antitrypsin

Answer 2

cirrhosis -> Portosytemic shunts -> decrease in NH metabolism -> neuropsychiatric dysfunction. triggers: - increase NH production and absorption sue to GI bleeding, constipation and infection - decrease NH, removal due to renal failure Hepatic encephalopathy is a decline in brain function that occurs when the liver can’t adequately remove toxins—especially ammonia—from the blood. These toxins then build up and affect the brain.

Answer 3

common and potentially fatal in patients with cirrhosis and ascites, often asymptomatic, commonly cause abby gram negative organisms,

Answer 4

Heme is degraded in RE system (esp. liver & spleen) heme -> biliverdin ( green) -> bilirubin (red- orange) blood with albumin ( unconjugugated bilurin) Salicylates & sulfonamides can displace bilirubin from albumin & so bilirubin enters CNS causing neural damage

Answer 5

Uptake of Bilirubin by hepatocytes: Bilirubin dissociates from its carrier albumin & enters hepatocytes Conjugation of Bilirubin: In hepatocytes, bilirubin is conjugated with two molecules of glucuronic acid by the enzyme glucuronyl transferase Excretion of bilirubin into bile: Conjugated bilirubin (bilirubin diglucuronide) is transported into bile canalculi & then into bile. Process is energy dependent & is impaired in liver diseases

Answer 6

conjugated bilrubin -> urobilinogen ( bacteria In the intestine) -> stercobillin the the stool urobillinogen -> reabsorbed -> kindeys -> urine urobillin

Answer 7

Yellow color of skin, nail beds & sclera caused by deposition of bilirubin secondary to increased bilirubin levels in blood (hyperbilirubinemia) JAUNDICE IS NOT A DISEASE HOWEVER, IT IS A SIGN OF AN UNDERLYING DISEASE

Answer 8

Hemolytic Jaundice 2- Obstructive Jaundice 3- Hepatocellular Jaundice

Answer 9

Massive lysis of RBCs in hemolytic anemia e.g. sickle cell anemia Bilirubin is produced in a rate faster than rate of conjugation by the liver Blood: Increased blood unconjugated (indirect) bilirubin Urine: Urobilinogen is increased No bilirubin in urine (Color of urine is normal) as it is bound to albumin Stool Dark color Increased stercobilin (produced from increased urobilinogen)

Answer 10

In bile duct obstruction: Conjugated bilirubin is prevented from passing to the intestine. Thus, it is regurged to blood increasing conjugated (direct) bilirubin in blood Excessive conjugated bilirubin is excreted in urine giving the yellowish brown color of urine Blood: Increased conjugated (direct) bilirubin GGT & ALP are markedly elevated (ALT is normal or mildly elevated) Urine: Bilirubin appears in urine Thus, color is yellowish brown Urobilinogen is reduced Stool Pale (low stercobilin)

Answer 11

First Liver damage (by hepatitis or hepatitis) causes low conjugation efficiency leading to increased unconjugated (indirect) bilirubin in blood Second Conjugated bilirubin is not efficiently secreted into bile. Instead, diffuses to blood increasing conjugated (direct) bilirubin in blood Blood Increased BOTH unconjugated (indirect) & conjugated (direct) bilirubin ALT & AST levels are markedly elevated Urine: Bilirubin is present in urine So, urine color is yellowish brown Stool Pale (low stercobilin)

Answer 12

In newborns (especially premature), Bilirubin accumulates as the liver enzyme bilirubin glucuronyl transferase (responsible for conjugation of bilirubin) is low at birth. (The enzymes reaches adult levels in about 4 weeks) Accordingly, unconjugated bilirubin is increased in blood. Elevated bilirubin in excess of the binding capacity of albumin can diffuse into basal ganglia & cause toxic encephalopathy (kernicterus)

Answer 13

all are autosomal recessive Gilbert Syndrome This is the most common and mildest form. The liver has a reduced ability to process bilirubin because the enzyme UDP-glucuronosyltransferase works less efficiently. People usually don’t have symptoms unless they are stressed, sick, or fasting, and even then, they might only have mild jaundice (yellowing of the skin or eyes). The bilirubin here is unconjugated (not processed yet). This condition is harmless and doesn’t need treatment. ⸻ 2. Crigler-Najjar Syndrome This is a much more severe disorder and has two types: * Type I: The liver enzyme is completely absent, so bilirubin can’t be processed at all. This causes very high levels of unconjugated bilirubin, which can build up in the brain (a dangerous condition called kernicterus) and cause serious damage. It shows up early in life. The main treatments are plasmapheresis and phototherapy, which help remove bilirubin, but they don’t fix the root issue. The only permanent cure is a liver transplant. * Type II: This is a milder form of Crigler-Najjar. Some enzyme activity is still present, and it can be treated with a medicine called phenobarbital, which helps increase enzyme production. ⸻ 3. Dubin-Johnson Syndrome In this condition, the liver makes conjugated bilirubin (the processed kind), but it can’t excrete it properly. This leads to a buildup of conjugated bilirubin in the blood. The liver appears dark or black on imaging or at surgery due to pigment buildup. Despite the odd liver color, this condition is benign (harmless) and usually doesn’t need treatment. ⸻ 4. Rotor Syndrome This condition is similar to Dubin-Johnson, but milder. The key difference is that the liver looks normal (not black), and the issue lies in the storage of conjugated bilirubin in the liver. It’s also harmless and doesn’t require treatment.

Answer 14

Biliary atresia is a condition in which the bile ducts are blocked or absent, either inside (intrahepatic) or outside (extrahepatic) the liver. Because of this, bile can’t drain from the liver to the intestine, leading to: Liver damage Cirrhosis Cholestasis (bile buildup in the liver) Type I – Atresia of the common bile duct The common bile duct (the last part of the duct draining into the intestine) is blocked The proximal ducts (inside the liver) are still open Least severe and rarest Type II – Atresia of the hepatic duct The common bile duct is open, but the hepatic ducts (just outside the liver) are blocked Subtypes: Type IIa – gallbladder and common bile duct are normal Type IIb – gallbladder, common bile duct, and cystic duct are all blocked Type III – Atresia at the porta hepatis (most common ~90%) Complete blockage of all extrahepatic bile ducts, including: Common bile duct Hepatic ducts Often intrahepatic ducts as well Most severe form; requires early intervention

Answer 15

Liver infarcts are rare due to the liver’s dual blood supply from the hepatic artery and portal vein. Even if the main hepatic artery is blocked, the liver tissue usually survives because of collateral circulation. However, in liver transplants, hepatic artery thrombosis often leads to organ failure. Localized infarcts can occur if an intrahepatic artery branch is blocked or compressed by conditions like polyarteritis nodosa, embolism, tumors, or infection

Answer 16

Budd-Chiari Syndrome is caused by thrombosis of one or more hepatic veins, leading to hepatomegaly, ascites, and abdominal pain. It is often linked to pro-thrombotic conditions such as polycythemia vera, pregnancy, oral contraceptive use, paroxysmal nocturnal hemoglobinuria, and intra-abdominal cancers like hepatocellular carcinoma. Mechanical causes include abscesses, parasitic cysts, or inferior vena cava obstruction by tumor or clot. Around 10% of cases have no known cause (idiopathic).

Answer 17

Benign Liver Tumors Generally asymptomatic and found incidentally: Hepatic Hemangioma Most common benign liver tumor Vascular tumor Usually requires no treatment unless very large Hepatocellular Adenoma Linked to oral contraceptive use or anabolic steroids Can rupture or rarely transform into cancer Surgical removal if large or symptomatic Focal Nodular Hyperplasia (FNH) Hyperplastic response to vascular abnormality Second most common benign tumor Usually asymptomatic and does not become cancerous Malignant Liver Tumors Can be primary (arising in the liver) or secondary (metastases from other cancers): Primary Malignant Tumors Hepatocellular Carcinoma (HCC) Most common primary liver cancer Strongly associated with cirrhosis, hepatitis B/C, alcohol, and aflatoxin exposure Angiosarcoma Rare, aggressive vascular tumor Linked to exposure to vinyl chloride, arsenic, and thorium dioxide (Thorotrast) metastases: the most common

Answer 18

- primary bilary cholangitis - a chronic autoimmune disease that slowly destroys the small intrahepatic bile ducts in the liver. This leads to: Cholestasis (bile buildup) Liver inflammation and fibrosis Eventually, cirrhosis and liver failure - Primary Sclerosing Cholangitis (PSC) is a chronic, progressive disease of the bile ducts, where inflammation and fibrosis cause: Narrowing (strictures) and Obstruction of bile flow secondary billiary cirrhosis - chronic liver disease that develops when prolonged obstruction of the extrahepatic bile ducts causes: Bile stasis Inflammation Fibrosis And eventually, cirrhosis (scarring of the liver)

Answer 19

Types of Gallstones: 1. Cholesterol Stones * Radiolucent (10–20% opaque due to calcifications) * ~80% of stones * Associated with: * Obesity * Crohn disease * Advanced age * Estrogen therapy * Multiparity * Rapid weight loss * Medications (e.g., fibrates) * Ethnicity (↑ in White & Native American populations) 2. Pigment Stones * Black = radiopaque (Ca²⁺ bilirubinate, hemolysis) * Brown = radiolucent (infection) * Associated with: * Crohn disease * Chronic hemolysis * Alcoholic cirrhosis * Advanced age * Biliary infections * Total parenteral nutrition (TPN) Risk Factors (7 F’s): * Female, Fat, Fertile, Forty, Fair, Fasting, Family history Complications: * Most common = cholecystitis * Can also cause acute pancreatitis, acute cholangitis

Answer 20

1) bilary colic: associated w nausea and vommiting, neurohormal activation triggers contraction of gallbladder forcing the stone into the cystic duct 2)Choledocholithaisis : presence of gall stones leading to elevated ALP 3) Cholecysitis: acute or chronic inflammation of gall bladder. calculous: inflammation and gall bladder thickening walls Acalculous: seen in critically ill patients and Murphy sign is present. galstone ileus: fistula between gallbladder and GI tract 4) acute: infection of bleary tree due to obstruction that leads to bacterial overgrowth

Answer 21

most common primary hepatobiliary carcinoma

Answer 22

slow growing malignancy of the bilary tract which tend to infiltrate locally and metasise later intrahepatic:

Answer 23

polysipia, polyuria, polyphagia ( 3Ps) can be caused by unstopped secretion of GH and epinephrine can cause cataract, small and large vessel disease

Answer 24

type 1: autoimmune T cell mediated destruction of Beta cells Type 2: resistance to insulin, progressive Beta cell failure

Answer 25

diabetic ketoacidosis: insulin noncompliance due to increased stress, we have an increase of ketone bodies, insulin present, ketones present hyperosmolar hyperglycaemic state: profound hyperglycaemia, causes dehydration due to excessive osmotic diuresis, insulin present, ketones deficient

Answer 26

inflammation of the pancreas acute: auto digestion of pancreas by parcreatic enzymes can be due to gallstones chronic pancreatitis: chronic inflammation, atrophy and calcification of pancreas

Answer 27

very aggressive tumor arising from pancreatic ducts, tumor most common in pancreatic head.

Answer 28

1. Insulinoma * Origin: β-cells → ↑ insulin → hypoglycemia * Whipple Triad: * Low blood glucose * Hypoglycemic symptoms (lethargy, syncope, double vision) * Symptom relief with glucose Glucagonoma * Origin: α-cells → ↑ glucagon → hyperglycemia * 6 D’s: * Dermatitis (necrolytic migratory erythema) * Diabetes * DVT * Declining weight * Depression * Diarrhea Somatostatinoma * Origin: δ-cells → ↑ somatostatin → ↓ secretion of many gut hormones (e.g., insulin, gastrin, secretin) Zollinger-Ellison Syndrome (ZES) * Cause: Gastrin-secreting tumor (gastrinoma) in pancreas/duodenum * Effect: Excess acid → refractory peptic ulcers, malabsorption

Lecture 9 Flashcards

(52 cards)