DSA 6: Chronic hepatitis, Chronic liver disease, Cirrhosis Flashcards
what is the etiology of chronic hepatitis?
how does it present?
group of d/o’s chronic inflam rxn in the liver for at least 6 months
common sxs: fatigue, malaise, anorexia, low-grade fever, jaundice
some pts - complication of cirrhosis
HBV: polyarteritis nodosa
HCV: mixed cryoglobulinemia
what is diagnositic for chronic hepatitis
CBC (platelets), chem panel, coag studies
Bx: historic classification - grade-necrosis/inflam & stage- degree of fibrosis
identify presence/absence of fibrosis (cirrhosis) : serum fibrosure &/or US elastography
what is the etiology of chronic HBV
endemic: Asia & sub-saharan Africa (90% vertical transmission)
Asymp healthy carrier stat - 75% Asia & Pacific rim
younger the age of infection - higher the probablity of chronicity
Risk HCC & cirrhosis: M>F
What increases/decreases progression of HCV?
increase: Male, drink > 50g EtOH daily, acquire infection after 40 yo, immunocompromised, tobacco/cannibis/fatty liver promote fibrosis
high risk of cirrhosis & HCC in genotype 1b
decrease: coffee
HCV Ab + HCV RNA = chronic
most HCV is curable
normal AST/ALT
what is the presentation and PE of AIH
type 1 (MC): Anti-Sm M &/or ANA (F, 30-50)
type 2: anti-LKM Ab
progessive jaundice, epistaxis, amenorrhea
healthy young woman w/ stigmata of cirrhosis - multiple spider telangiectasias, cutaneous striae, acne, hirsutism and hepatomegaly
extrahepatic: AI sxs, UC
what is diagnostic of AIH
AST/ALT may be > 1000
increased total bili
serology:
type 1: hypergammaglobulinemia, SMA, ANA
type 2: anti-LKM Ab
what is Tx for AIH
glucocorticoids
indicated for sxs dz: Bx shows bridging necrosis, 5-10x AST/ALT, hypergammaglobuliemia
what are complications of AIH
leads to cirrhosis –> HCC
what is the etiology of alc liver dz (SAAD > 1.1)
excessive: >80g/day in males & 30-40g/day in females x 10 yrs
(often deny Hx of excess alc use)
fatty liver (steatosis) : asymp- hepatomegaly & mild elevation in biochem livers (reverse if stop EtOH)
what is the clinical presentation of alcohol liver dz
asymp –> severe dz w/ jaundice, ascites, GI bleeds and encephalopathy
anorexia, N/V, fever, jaundice, tender hepatomegaly & RUQ pain
what lab are diagnostic of alcoholic liver dz (SAAG > 1.1)
chem (CMP) - AST 2x > ALT; Bili >=10 mg/dL
CBC: leukocytosis (severe) and leukopenia (sometimes and resolve after drinking), anemia (macrocytic/megaloblastic); thrombocytopenia
PT/PTT/INR: marked prolongation of PT
what imaging diagnostics are found in alcohol liver dz
imaging: US- exclude biliary obstruction; CT w/ IV contrast/MRI: collateral vessels, space-occupying lesions; US elastography: fibrosis
liver Bx: mallory-denk bodies
what is the treatment for alcoholic liver dz
abstinence from alcohol
daily multivitamin, thiamine 100 mg, folic acid 1 mg, zinc: glucose administration increase thiamine requirement & can cause wernicke-korsakoff syndrome if thiamine not co-administered
severe alcoholic hepatitis = steroids, pentoxifylline
liver transplant - abstain from alc for 6 months
what is the prognosis and complications of alcoholic liver dz (SAAG >1.1)
MC precursor to cirrhosis in US –> risk of HCC
wernicke-encephalopathy- treat w/ thiamine
korsakoff (permanent)
adverse prognostic factors: poor prognosis -ascities, variceal hemorrhage, encephalopathy, hepatorenal syndrome. critically ill pt w/ alc hepatitis have 30 day mortality rate >50%
what is labs/symptoms present in severe alcoholic hepatitis
total bili > 8-10 and PTT > 6 = (50% mortality/susceptible to infxn, including invasive aspergilosis)
hypoalbuminemia, azotemia
what is
Maddrey’s discriminant fxn (DF)
Glasgow alc hepatitis score
A model for End-stage liver dz
Maddrey’s discriminant fxn (DF): >=32 - poor prognosis; may benefit from steroids
Glasgow alc hepatitis score: predict mortality based on multivariable model; >= 9 -higher survival rates
A model for End-stage liver dz: >21 significant mortality in alc hepatitis
what is hepatic steatosis and what is the etiology
fatty liver –> fatty liver that causes liver inflam = steatohepatitis –> cirrhosis
etiology: alc related or non-alc fatty liver dz (NAFLD) - < 20 g/day F and <30 g/day M
wha tis the etiology of NAFLD
MCC of chronic liver dz in USA
principal causes & increased risk for NAFLD - metabolic syndrome (obesity, DM, hypertriglyderigemia) - increased risk for CV disease, CKD, colorectal CA
protective: physical activity & coffee consumption
cirrhosis caused by NASH is UNCOMMON in african americans
what is the presentation and PE of NAFLD
what is the complication
asymp or mild RUQ
hepatomeg
complication - cirrhosis
what is diagnositic of NAFLD
labs: mild increase AST/ALT and ALP - may be normal =< 80% hepatic steatosis
imaging - US elastography- assess liver stiffness can be used to ESTIMATE hepatic fibrosis
Liver Bx = Diagnostic - histologic- local infiltration by PMN and mallory hylaine,indistinguishale from alc hepatitis, =NASH
what is the treatment/management of NAFLD
lifestyle modifications- wt loss, dietary fat restriction, exercise, vit E, gastric bypass
statins NOT contraindicated
in advanced cirrhosis –> liver transplant
What is the etiology of a1-antitrypsin def
AR
low level of a1-antitrypsin def –> build up in hepatocytes –> liver damage & decrease protease inhibitors
pulmonary emphysema
panacinar emphysema (at young age) - lower lobes; **smokers –> COPD/emphysema of upper lobe
liver dz
MC diagnosed inherited hepatic disorder in infants & children
what is diagnostic of a1-antitrypsin def
low a1-antitrypsin def level
check a1-antitrypsin def phenotype
PIZZ - homozygous (reduction in enzyme)
what are treatments of a1-antitrypsin def?
if not treated, what are complications?
smoking abstinence/cessation
liver transplant - MC genetic cause requiring liver transplant in kids
complication - emphysema at young age; cirrhosis –> HCC
what is the etiology of PBC
progressive non-suppurative destructive intrahepatic cholangitis
infxn w/ Novosphingobium aromaticivorans or chlymagophilia -may trigger/cause it
= AI destruction of small intrahepatic bile ducts/cholestasis
Female, median = 50 yo
asymp isolated elevation of ALP or impaired bile excretion
what are the RF and clinical manifestation of PBC
RF: UTI, smoking, use of hormone replacement therapy, hair dye
clinical manifestation: pruritus, progressive jaundice, xanthelasma, osteoporsis, HSM, portal HTN
associated w/ AI d/o’s
what is diagnositic of PBC
AMA, increasd IgM
increased ALP, GGT (liver), bilirubin, cholesterol
what is the treatment/management of PBC
ursodeoxycholic acid
liver transplantation for end stage dz
Ca2+, vit D, bisphosphonate for osteoporosis
what complications may occur w/ PBC
what is the prognosis
cirrhosis –> liver failure
prognosis –> correlated w/ age, serum bili, serum albumin, PT, edema
what is the etiology of hemochromatosis
HFE gene mutation - increased Fe abs in duodenum
increase Fe saturation or ferritin or Fhx
increased accumulation of Fe as gemosiderin in the liver, pancreas, heart, adrenals, testes, pituitary and kidneys
what is the clinical manifestation of hemochromatosis
tetrad: cirrhosis w/ hepatomegaly, abnormal skin pigment, DM, cardiac dysfxn
early sxs = nonspecifc
late sxs= skin pigmentation (bronze color), DM (bronze DM), erectile dysfxn (hypgonadism)
what is diagnostic of hemochromatosis
Labs: HFE gene mutation, mildly abnormal liver tests; elevated plasma iron > 45% transferring saturation; elevated serum ferritin
Imaging: MRI & CT - iron overload of the liver; MRI quantitate hepatic iron stores and hep assess the degress of hepatic fibrosis
liver Bx: homozygous - C282Y - check for cirrhosis
screening - test for HFE mutation for pt w/ iron overload and all-first degree family members
what is the treatment and mangement for hemochromatosis
phlebotomy therapy: goal - iron store depletion achieved
avoid foods rich in iron
monitor HCT and serum iron
give PPI
hemochromatosis and anemia/thalassemia (can’t tolerate phlebotomy)
use deferoxamine
liver transplantation
what are risks for advanced fibrosis in hemochromatosis
who is at increased risk for infxn
male, excess EtOH, DM
increased risk for infxn - v. vulnificus, l. monocytogenes, y. enterocolitica and siderophilic organism
(overall slight increase in mortality)
what is the etiology of wilsons dz
AR - ATP7B; excess abs from SI or decreased excretion by liver
< 40 yo
impaired copper excretion into bile acid and failure to incorporate copper inot ceruloplasmin
accumulation of copper in liver, brain and eyes ; also cause hemolytic anemia
liver dz in teens & neuropsychiatric dz in young adults
what is diagnostic for wilson dz
increased urinary copper excretion
low serum ceruloplasmin
hepatic copper concentration are high
MRI of the brain
molecular analysis - ATP7B
what is the clinical presentation and PE of wilson’s dz
child/young adults: hepatitis, hemolytic anemia, neurologic/psychiatric abnormalities, splenomegaly w/ hypersplenism, coombs-neg, portal HTN
kayser-fleischer ring - fine pigmented granular deposits in sescemet membrane in cornea
assocaited w/ renal canliculi, hemolytic anemia, subQ lipoma
what is the treatment of wilson dz
oral penicillamine (increases urinary excretion of chelated copper)
liver transplant
what is the etiology of liver in HF
RHF –> passive congestion of the liver (nutmeg liver)
ischemic hepatitis - acute fall in CO
statin therapy prior to admission may protect against ischemic hepatitis
what is the presentation of liver dx due to heart failure
what are complications
hypotension absent or unwitnessed
hepatojugular reflux w/ tricuspid regurg
jaundice is associated w/ worse outcomes
complications: mortality rate due to underlying dz high
what are diagnostics of liver dz due to heart failure
AST/ALT mildly elevated (absence or superimposed ischemia)
markedly elevated serum N-terminal-proBNP or BNP
hallmark = Shock Liver - rapid & striking elevation of AST/ALT (> 5000) & early rapid rise in serum LDH
MILD elevations inf ALP and bili
what is the epidemiology & RF of cirrhosis
peak incidence 40-60
RF: alcohol, IV drug abuse, obesity, viral, genetics, AI, meds
life expectancy - markedly reduced
what is the pathophys of cirrhosis
fibrosis of liver –> destroy the liver’s vascular and lobular architecture –> progressively deminish blood flow, decrease fxn, formation of regenerative nodules
liver insufficiency
higher coffee and tea consumption -reduce risk of cirrhosis
MC: hep C, alcoholic liver dz, NAFLD
what is historical info and clinical presentation of cirrhosis
hepatic cell dysfxn, portosystemic shunting, portal htn
jaundice, ascites, encephalopathy
RF = viral, alc consumption, Hx of viral hep or jaundice
what are PE findings of cirrhosis or liver dz
jaundice/icterus
gynecomastia
ascites
testicular atrophy
esophageal varices (splenomegaly)
palmar erythema
spider angioma
caput medusae
m. wasting
asterixis
dupuytran’s contracture
umbilical hernia
hypoalbumin - muehrcke’s lines & terry’s nails
what are diagnostic labs for cirrhosis ?
CBC - decrease HBC, HgB, platelets
chem - CMP, cholesterol decrease, PTT/INR, ammonia, AFP (last increased)
(look at attached pic)
what are diagnostic imaging findings for cirrhosis
ultrasonography + doppler (RUQ/liver)
SAAG- serum albumin - ascites albumin (>1.1 portal htn, >250 PMNs/mL think SBP)
several noninvasice predictive indicies for hepatic fibrosis - imaging - US elastography; more specific serum - fibrosure
what are possible complications of cirrhosis
HCC
HIV co-infxn
decompensated cirrhosis
what are treatments for cirrhosis
what are managements for cirrhosis
abstain from alcohol, recretaional drugs, smoking, liver toxic medicaitons/herbs
stop nsaids
endoscopy to screen for varices
monitor labs
AFP and US every 6 months to sceen for HCC
immunizations
what are survival prognostic scores of cirrhosis
MELD/MELD-Na -order bilirubin, CK, Na, INR
Child-turcotte-pugh (CTP) - order bilirubin, albumin, PT/INR, assess PE for encephalopathy and ascites
always check these
liver transplant
what is the etiology of ascities
pathologic of fluid in periotneal cavity
MCC of ascites is portal HTN 2ndary to CLD
what is the hx/PE of ascities
Hx - increasing abd girth (+/-) abd pain
PE: asterixis, shifting dullness, elevated jugualr venous pressure, large tender liver, signs of CLD, firm LN - think intraabd malignancy
what are diagnostic features of ascities
abd US (+ doppler)- Abd paracentesis
CT: detect budd-chiari, LAD & masses
Labs - SAAG, WBC most important, culture/gram stain
what is the tx for ascites
paracentesis: dx and tx
treat the cause
draw out SAAG decision tree
what are sxs for esophageal varices
what increases chances of bleed
acute GI hemorrhage - melena, hematochesia, hematoemesis
can be serious/life threatenting - bleeding can lead to hypovolemia
bleeding RF = size, red walte marking, severity of liver dz, active alcohol abuse
(how do you tx and how do you prevent rebleed)
what is the etiology/pathophys of hepatic encephalopathy
alteration of mental status in presence of liver failure
amonia levels are typically elevated in encephalopathy
what are precipitants of hepatic encephalopathy
GI bleeding, constipation, sepsis
azotemia, high protein meal, hypoK alkolosis, CNS depressant drugs
what is diagnostics for hepatic encephalopathy
how do you treat this
clincal diagnosis
ammonia levels are typically elevated
tx: Lactulose (nonabsorbable disaccharide) results in colonic acidification and diarrhea; goal = 2–3 soft stools/day
what is the presentation of encephalopathy
what are the grades of overt encephalopathy
asterixis (flapping temor)
confusion, slurred speech, change in personality, sleepy, difficult to arouse
overt enceophalopathy: mild confusion, droswy, stupor, coma
what is primary peritonitis
what is secondary peritonitis
what is the etiology and clinical presentation/PE of HCC
male, 50-60s, high incidence Asia & Africa, Aflatoxin exposure
cachexia, abd pain, fever, jaundice, asthenia, itching, tremors, disorientation, HSM, ascites, peripheral edema
how do you screen/prevent HCC
and how do you dx
AFP and US every 6 months
Dx: pt w/ known liver dz - abnormality on US or increase in AFP
how do you treat HCC
Surgical resection or liver transplantation (Tx, but rarely successful)
Radiofrequency ablation
Transcatheter arterial embolization (TACE)
when are liver transplants considered
irreversible, progressive CLD, acute liver failure, metabolic dz in which the metabolic defect
if pt w/ alcoholism - abstinent for 6 months
consider in pts w/ worsening fxnal status - based on MELD/MELD-Na score (MELD >14)
immnosuppresion needed after transplant
