Aa Flashcards

Question

HBsAg neg HbeAb neg HbcAb (IGG)

Answer 1

C. Clinical features = Portal hypertension leads to 1. Ascites (fluid in the peritoneal cavity) Hyperspleenism- enlarged spleen eats platelets and rbc /Congestive splenomegaly/hvpersplenism ili. Portosystemic shunts (esophageal varices, hemorrhoids, and capital Medusa iv. Hepatorenal syndrome (rapidly developing renal failure secondary to cirrhosis) 2. Decreased detoxification results in i Mental status changes, asterixis, and eventual coma (due to serum ammonia); metabolic, hence reversible il. Gynecomastia, spider angiomata, and palmar erythema due to hyperestrinism iii. jaundice 3. Decreased protein synthesis leads to i. Hypoalbuminemia with edema ii. Coagulopathy due to decreased synthesis ofclotting factors; degree of deficiency is followed by PT. Dec epixode reductase

Answer 2

IV. ALCOHOL-RELATED LIVER DISEASE A. Damage to hepatic parenchyma due to consumption ofalcohol B. Fatty liver is the accumulation offat in hepatocytes (Fig. 11.9A) 1. Results in a heavy, greasy liver: resolves with abstinence (Fig. 11.9B) C. Alcoholic hepatitis results from chemical injury to hepatocytes: generally seen with binge drinking 1. Acclaldehyde (metabolite of alcohol) mediates damage. 3. Presents with painful hepatomegaly and elevated liver enzymes (AST > ALT); may result in death D. Cirrhosis is a complication oflong-term, chronic alcohol-induced liver damage; 1. NONALCOHOLIC FATTY LIVER DISEASE A. Fatty change, hepatitis, and/or cirrhosis that develop without exposure to alcohol other known insult) B. Associated with obesity C. Diagnosis ofexclusion; ALT > AST

Answer 3

1. Priinary hemochromatosis is due to mutations In the HFE gene, usually C282Y (cysteine is replaced by tyrosine at amino acid 282). Secondary- transfusions C. Presents in late adulthood I. Classic triad is cirrhosis, secondary diabetes mellitus, and bronze skin; other. findings include cardiac arrhythmias and gonadal dysfunction (due to testicular atrophy). Labs show ferritin, inc ; TIBC Dec serum iron inc and inc transferín saturation. 3. Liver biopsy reveals accumulation of brown pigment in hepatocytes Prussian blue stain distinguishes iron (blue) from lipofuscin (Fig. H UB). i. D. Increased risk of hepatocellular carcinoma Bc increase free radicals E. Treatment is phlebotomy,

Answer 4

V. WILSON DISEASE A. Autosomal recessive defect (ATP7B gene) in ATP-mediated hepatovte copper transport I. Results in lack ofcopper transport into bile and lack of copper incorporation into ceruloplasmin B. Copper builds up in hepatocytes, leaks into serum, and deposits in tissues. 1. Copper-mediated production ofhydroxy! free radicals leads to tissue damage. C. Presents in childhood with 1. Cirrhosis • 2. Neurologic manifestations (behavioral changes, dementia, chorea similar to Huntington and Parkinsonian symptoms due to deposition ofcopper in basal ganglia) 3. Kayser-Fleisher rings in the corneal D. Labs show inc urinary copper, Dec serum ceruloplasmin, and inc copper on liver biopsy. E. Increased risk ofhepatocellular carcinoma Bc increase free radicals F. Treatment is D-penicillamine (chelates copper).

Answer 5

VIII.PRIMARY BILIARY CIRRHOSIS A. Autoimmune granulomatous destruction of intrahepatie bile ducts 1. Classically arises in women (average age is 40 years) 2. Associated with other autoimmune discases B. Etiology is unknown; antimitochondrial antibody is present. C. Presents with features of obstructive jaundice D. Cirrhosis is a late complication, IX. PRIMARY SCLEROSING CHOLANGITIS A. Inflammation and fibrosis ofintrahepatic and extrahepatic bile ducts 1. Periductal fibrosis with an onion-skin* discoloration 1. Periductal fibrosis with an onion-skin* apptarance (Fig. 11.12) 2. Uninvolved regions are dilated resulting in a "beaded" appearance on con imaging. B. Etiology is unknown, but associated with ulcerative colitis; p-ANCA is often p C. Presents with obstructive jaundice; cirrhosis is a late complication. D. Increased risk of cholangiocarcinoma Obstructive jaundice Dark urine (due To bilirubinuria) and pale stool Hypercholesterolemia with xanthomas Steatorchea with malabsorption of fat-soluble Bile salts deposits skin thereforePruiTis. Bile can't get to the therefore bile salts can’t help with fat absorption - malabsorption and Steatorrhea

Answer 6

XII. HEPATOCELLULAR CARCINOMA A. Malignant tumor ofhepatocytes B. Risk factors include 1. Chronic hepatitis (e.g., HBV and HCV) 2. Cirrhosis (e.g., alcohol, nonalcoholic fatty liver disease, hemochromatosis, Wilson disease, and AlAT deficiency) 3. Anatoxins derived from Aspergillus- (induce p53 mutations) C. Increased risk for Budd-Chiari syndrome 1. Liver infarction secondary to hepatic vein obstruction 2. Presents with painful hepatomegaly and ascites D. Tumors are often delected late because symptoms are masked by cirrhosis; poor prognosis E. Serum tumor marker is alpha-fetoprotein.

Answer 7

Berry aneurysm

Answer 8

Berry aneurysms, liver cysTs, Mvp, HTN bc of increases renin, hematuria

Answer 9

II. PRERENAL AZOTEMIA A. Due to decreased blood flow to kidneys (e.g., cardiac failure); common cause of ARf B. Decreased blood How results in decreases GFR, azotemia, and oliguria. C. Reabsorption offluid and BUN ensues (scrum BUN: Cr ratio > 15): tubular function remains intact (fractional excretion ofsodium [FENa] < 1% and urine osmolality > 500 mOsm/kg). IV ACUTE TUBULAR NECROSIS A. Injury and necrosis oftubular epithelial cells (Fig, 12,5); most common cause of acute renal failure (intrarenal azotemia) B. Necrotic cells plug tubules; Dysfunctional tubular epithelium results in decreased reabsorption of BUN (serum BUN:Cr ratio < 15), decreased reabsorption ofsodium (FENa > 2%), and inability to concentrate urine (urine osm < 500 mOsm/kg). D. Etiology may be ischemic or nephrotoxic, Ischemia - Decreased blood supply rosults in And this decreased blood flow persists . Often preceded by prerenal il. Proximal tubule and medullary segment ofthe thick ascending limb are particularly susceptible to ischemic damage. 2. Nephrotoxic -Toxic agents result in necrosis oftubules. i. Proximal tubule is particularly susceptible. il. Causes include aminoglycosides (most common), heavy metals (e.g., lead), myoglobinuria (e.g., from crush injury to muscle), ethylene glycol (associated with oxalate crystals in urine), radiocontrast dye, and urate (c.g., tumor lysis syndrome). ill. Hydration and allopurinol are used prior to initiation ofchemotherapy to decrease risk of urate-induced AT. III. POSTRENAL AZOTEMIA to obstruction of urinary tract downstream from the kidney (e.g., ureters). During early stage of obstruction, increased tubular pressure "forces" BUN into the therefore blood (serum BUN;Cr ratio > 15); tubular function remains intact (FENa < 1% and can concentrate urine therefore tubular function is urine osm > 500 mOsm/kg) perserved D. With long-standing obstruction, tubular damage ensues, resulting in decreased reabsorption of BUN (serum BUN:Cr ratio < 15), decreased reabsorption ofsodium (FF^Na > 2%), and inability to concentrate urine (urine osm < 500 mOsm/kg).

Answer 10

V. ACUTE INTERSTITIAL NEPHRITIS A. Drug-induced hypersensitivity involving the interstitium and tubules (Fig. 12.6); results in acute renal failure (intrarenal azotemia) B. Causes include ((NSAIDs, penicillin, and diuretics) C. Presents as oliguria, fever, and rash days to weeks after starting a drug; cosinophils may be seen in urine. D. Resolves with cessation of drug E. May progress to renal papillary necrosis VI, RENAL PAPILLARY NECROSIS A. Necrosis of renal papillae §. Presents with gross hematuria and flank pain C. Causes include 1. Chronic analgesic abuse (e.g.. long-term phenacetin or aspirin use) 2. Diabetes mellitus 3. Sickle cell trait or disease 4. Severe acute pyelonephritis

Answer 11

Renal (nephrotic )

Answer 12

Renal failure (from diffuse proliferative

Answer 13

D. Etiology may be ischemic or nephrotoxic, !. Ischemia- Decreased blood supply results in necrosis of tubules. And this decreased blood flow persists resulting in i. Often preceded by prerenal azotemia Atn ii. Proximal tubule and medullary segment ofthe thick ascending limb are particularly susceptible to ischemic damage. 2. Nephrotoxic-Toxic agents result in necrosis oftubules. i. Proximal tubule is particularly susceptible. ii. Causes include aminoglycosides (most common), heavy metals (e.g., lead), myoglobinuria (e.g., from crush injury to muscle), ethylene glycol (associated with oxalate crystals in urine), radiocontrast dye, and urate (e.g., tumor lysis syndrome). iii. Hydration and allopurinol are used prior to initiation of chemotherapy to decrease risk of urate-induced ATN.

Answer 14

VI, RENAL PAPILLARY NECROSIS A. Necrosis of renal papillae 8. Presents with gross hematuria and flank pain C. Causes include 1. Chronic analgesic abuse (e.g., long-term phenacetin or aspirin use) 2. Diabetes mellitus 3. Sickle cell trait or disease 4. Severe acute pyelonephritis

Answer 15

C. Normal glomeruli on H&E stain (lipid maybe seen in proximal tubule cells. D. EfTacement of foot processes on electron microscopy (F.M, Fig. 12.7B) E. No immune complex deposits; negative immunofluorescence (IF) F. Selective proteinuria (loss of albumin, but not immunoglobulin) G. F.xcellent response to steroids (damage is mediated by cytokines from T cells)- if doesn’t resolve can lead to FSGS Complication of : Usually idiopathic; may be associated with Hodgkin lymphoma I. Cytokines damages the podocyte and Hodgkin is caused by cytokines -

Answer 16

1. BASIC PRINCIPLES A. Glomerular disorders characterized by proteinuria (> 3.5 g/day) resulting in 1. Hypoalbuminemia pitting edema 2. Hypogammaglobulinemia--increased risk ofinfection 3. Hypercoagulable state- -due to loss of antithrombin III Hrperlipidemia and hypercholesterolemia-may result in fatty casts in urine

Answer 17

III. FOCAL SEGMENTAL GLOMERULOSCLEROSIS (FSGS) A. Most common cause of nephrotic syndrome in Hispanics and African Americans Sclerosis - pink deposits Usually idiopathic; maybe associated with HIV, heroin use, and sickle cell disease C. Focal (some glomeruli) and segmental (involving only part of the glomerulus) sclerosis on H&E stain (Fig. 12.8) D. Fffacement of foot processes on EM E. No immune complex deposits; negative IF Foor response to steroids, progresses to chronic renal failure

Answer 18

Thick not thin filtration barrier b of immune complex deposition Epithelial lays down additional basement / membrane creatin‹ IV. MEMBRANOUS NEPHROPATHY dome over deposits. Spikes in A. Most common cause of nephrotic syndrome in Caucasian adults between dome B. Usually idiopathic; may be associated with hepatitis B or C, solid tumors, SLE, or drugs (e.g.. NSAIDs and penicillamine) IV. MEMBRANOUS NEPHROPATHY A. Most common cause of nephrotic syndrome in Caucasian adult B. Usually idiopathic; may be associated with hepatitis B or C, solid tumors, drugs (e.g. NSAIDs and penicillamine) Thick glomerular basement membrane on H&E (Fig. 12.9A) Due to immune complex deposition (granular IE , subepithelial deposits 'spike and dome' appearance on EM (Fig. 12.9C) E. Poor response to steroids: progresses to chronic renal failure Antibodies against Pla2R (phospholipase)

Answer 19

V. MEMBRANOPROLIFERATIVE GLOMERULONEPHRITIS A. Thick glomerular basement membrane on H&E, often with 'tram-track' appearance B. Due to immune complex deposition (granular IF C. Divided into two types based on location of deposits 1. Type I-_ subendothelial (Fig. 12.10); associated with HBV and HCV- tram tracks 2, Type II (dense deposit disease) intra membranous; associated with C3 nephritic factor (autoantibody that stabilizes C3 convertase, leading to overactivation of complement, inflammation, and low levels of circulating C3) Under normal conditions C3 D. Poor response to steroids; progresses to chronic renal failure

Answer 20

Glucose in basement membrane causes leakage of it releasing protein which narrows the lumen. Hyaline arterial in the efferent arteriole therefore inc pressure in glomerulus leading to sclerosis of glom mesangial progresses to nephrotic j and hyper filtration (microalbuminuria -ACEi prevents this bc blocks ang 2 B. Glomerular efferent arteriole is more affected than the afferent arteriole, leading to high glomerular filtration pressure. L. Hyperfiltration Injury leads to microalbuminuria, C. Eventually progresses to nephrotic syndrome 1. Characterized by sclerosis of the mesangium with formation of Kimmelstiel-Wilson nodules (Fig. 12,111 D. ACE inhibitors slow progression of hyper hitration-induced damage

Answer 21

A. Kidney is the most commonly involved organ in systemic amyloidosis. Amyloid deposits in the mesangium, resulting in nephrotic syndrome, C. Characterized by apple-green birefringence under polarized light after staining with Congo red

Answer 22

I. BASIC PRINCIPLES A. Glomerular disorders characterized by glomerular inflammation and bleeding 1. Limited proteinuria (< 3.5 g/day) 2. Oliguria and azotemia • 3. Salt retention With periorbital edema and hypertension SHa0 velenhon 4. RBC casts and dysmorphic RBCs in urine When glomerular bleeds, d casts are formed in the shape of the tubular. B. Biopsy reveals hypercellular, inflamed glomeruli (Fig. 12.12). neu

Answer 23

I. POSTSTREPTOCOCCAL GLOMERULONEPHRITIS (PSGN) A. Nephritic syndrome that arises alter group A |3-he mo lytic streptococcal infection Of the skin (impetigo) or pharynx 1. Occurs with nephritogenic strains (which carry the M protein virulence factor) 2. May occur after infection with nonstreptococal organisms as well B. Presents 2-3 weeks after infection as hematuria (cola-colored urine), oliguria, hypertension, and periorbital edema 1. Usually seen in children, but may occur in adults C. Hypercellular, inflamed glomeruli on H&E D. Mediated by immune complex deposition (granular IF)- immune complex deposition activates C5a which attracts neutrophils ; subepithelial 'humps' on EM ) Deposits start subendothelial, deposits can disappear therefore it resolves E. Treatment is **supportive** 1. Children rarely (1%) progress to renal failure. 2. Some adults (25%) develop rapidly progressive glomerulonephritis (RPGN).

Answer 24

Linear (anti basement membrane of lung and kidney -Goodpasture syndrome) Antibody attacks the basement of lung Granular (immune complex deposition) -PSGN OR RPGN Diffuse proliterative glomerulomephritis is duc to diffusc antigen - a lit ib ody complex deposition, usually sub-endothehal, most common type ofrenal disease in SLE Negative II (pauci-immunc) Do anca B. Characterized by crescents in Bowman space (of glomeruli) on H&E stain; crescents are comprised of fibrin and macrophages (Fig. 12 14).

Answer 25

IV. IgA NEPHROPATHY (BERGER DISEASE) A, IgA immune complex deposition in mesangium ofglomeruli; most common nephropathy worldwide B, Presents during childhood as episodic gross or microscopic hemaluria with RBC easts, usually following mucosal infections (e.g., gastroenteritis) 1. IgA production is increased during infection. C. IgA immune complex deposition in the mesangium is seen on IF (Fig. 12.16). D. May slowly progress to renal failure <1 week after URI

Answer 26

V, ALPORT SYNDROME A. Inherited defect in type IV collagen; most commonly X-linked (**Col4A5 mutation** B. Results in thinning and splitting of the glomerular basement membrane (basket weave pattern -EM C. Presents as isolated hematuria, sensory hearing loss, and ocular disturbances- damaged basement membrane in ears, eyes, kidney in family

Answer 27

I. BASIC PRINCIPLES A. End-stage kidney failure 1. May result from glomerular, tubular, inflammatory, or vascular insults 2. Most common causes are diabetes mellitus, hypertension, and glomerular discasc, B. Clinical Features 1. Uremia--Increased nitrogenous waste products in blood (azotemia) result in nausca, anorexia, pericarditis, platelet dysfunction, encephalopathy with asterixis, and deposition of urea crystals in skin, 2. Salt and water retention with resultant hypertension 3. Hyperkalemia with metabolic acidosis Can't get rid of 4. Anemia due to decreased erythropoietin production agriensi peritubular interstitial cells 5. Hypocalcemia due to decreased I-alpha-hydroxylation ofvitamin D by proximal renal tubule cells and hyperphosphatemia 6, Renal osteodystrophy due to secondary hyperparathyroidism, osteomalacia, and osteoporosis C. Treatment involves dialysis or renal transplant. 1. Cysts often develop within shrunken end-stage kidneys during dialysis, increasing risk for renal cell carcinoma. cysts in already shrunken kidney