Lecture 70/72 - Liver Diseases 1 and 2 Flashcards
what is the relationship between cirrhosis and portal HTN?
Cirrhosis – -Increased Intra-hepatic resistance due to scarring; initial mechanism leading to portal HTN
Distorted Sinusoidal Architecture
Sinusoids are usually nice and orderly
But now distorted, increased pressures
Leading to increased resistance
what are some signs and symptoms of cirrhosis and portal HTN?
Spider Angiomata -- Pectoral Alopecia Palpable liver Caput Medusae Ascites Clubbing of the Fingers Muscle wasting Gynocomastia Splenomegaly Testicular atrophy and Low testosterone Female escutcheon -- male pubic hair shape (rhomboid) becomes more feminine (triangular) Palmar erythmea Purpura and Petechia -- bleedings
what are complications (potential manifestions) of portal HTN?
varices
Hepatic Encephalopathy
Ascites
what are two common sites for varices?
what is the name of the sign for a bleeding site on endoscopy
gastric and esophageal
red whale sign
Describe three methods for bleeding management of varices?
Hemostasis of Bleeding Esophageal Varices
Sclerotherapy
Endoscopic Ligation
Transjugular Intrahepatic Portal-Systemic Shunt (TIPS Procedure)
What is the a Transjugular Intrahepatic Portal-Systemic Shunt (TIPS Procedure) ?
Use needle to connect hepatic vein system (normal pressure) to the portal system (High pressure in a cirrhotic)
Create a conduit with catheter and expandable metal stent to reduce the pressures in the portal System
Hepatic Encephalopathy – what is the mechanism of onset?
diverting a lot of blood around the liver; therefore unable to metabolize a lot of neurotoxic substances, such as ammonia
can be spontaneous or in the setting of TIPs for example
what are the 4 stages of Hepatic Encephalopathy
Stage 1 – confusion; can be very subtle
□ Have patient draw a clock, ask about date, etc
§ Stage 2 – drowsiness
□ Typically exhibit asterixis (flapping tremor)
§ Stage 3 – somnolence
□ Also exhibit asterixis
§ Stage 4 – Coma
□ Usually have to intubate
what is the best management for hepatic encephalopathy?
Lactulose
Decreases the pH in the colon
-converting the ammonia (NH3) to ammonium (NH4+), which does not cross the mucosa and excreted
Ascites — what is it?
Accumulation of fluid in the abdomen
what is the difference between uncomplicated and refractory ascites ?
§ Uncomplicated Ascites —
Can be controlled with salt restriction +/- Diuretics
§ Refractory Ascites
Cannot be handled with diuresis
how is uncomplicated ascites managed ?
how is refractory ascities managed ?
Uncomplicated: salt restirction, diuresis, Large volume paracentesis + albumin supplment
Refractory: LVP + albumin, TIPS, Portal vein shunt
what are the common Complications of Ascites?
Infection: Spontaneous Bacterial Peritonitis
§ Tense Ascities –
So much pressure, its pushing on the base of the lungs
Umbilical Hernia – if rupture, can lead to hypotension
Hydrothorax – Fluid leaking form the abdominal cavity to above the diaphragm
Hepatocellular Carcinoma –
describe some infectious risk factors? where are these endemic?
describe some non infectious risk factors?
§ Alcoholism and Hep C – US
§ Hepatitis B – World wide (asia and africa)
- Cirrhosis
Hemochromatosis
Wilson’s disease
DMT2 + obesity === NAFLD
what are some indications for liver transplant?
Decompensated cirrhosis, fulminant liver failure, HCC (Milan criteria)
Viral liver disease, auto-immune, inherited, autoimmune, cholestatic, malignancy, NAFLD, Vascular
in general … what is the Milan Criteria ?
Milan criteria are applied as a basis for selecting patients with cirrhosis and hepatocellular carcinoma for liver transplantation.
in general what is the MELD score?
what is it used to determine?
what labs are taken into consideration in the calculation?
Model for End-Stage Liver Disease — used to assess severity of liver disease
bilirubin, creatinine and INR
what are some contra-indications for liver Tx ?
severe Cardiopulmonary disease
hepatic malignancy with vascular invasion
Extrahepatic malignancy
Active infection
active substance abuse, poor compliance, social support
what is the difference between acute liver failure and fulminant liver failure ?
Acute Liver Failurefocus of today – liver injury without AMS
Fulminant liver failure – liver injury with AMS
what are the different classes of etiologies of acute liver failure ?
Toxic – acetaminophen (intrinsic), other idiosyncratic iatrogenic, death cap mushroom
Alcoholic
Viral – Hep A, B, D, E, C
Auto Immune
Hereditary
Pregnancy
Ischemic
what is the most common cause of acute liver failure
Acetominophen Overdose
Acetominophen Overdose – what is the mechanism of liver damage?
Symptoms?
Labs?
treatment? (early vs late)
- When the system is overloaded, cytochrome P450 kicks in and metabolizes acetaminophen to NAPQI, a toxic reactive Intermediate.
- NAPQI reacts with glutathione to yield a non -toxic intermediate. If glutathione stores depleted, NAPQI toxic build up, which may take 2-3 days
Symptoms: Nausea, vomiting, hepatomegaly, abdominal pain, anorexia, encephalopathy
Labs?
Extremely high AST/ALTs (1000s), with possible kidney injury
Treatment:
early (3 hours of ingestion) – Activated Charcoal
up to 48 hours or more – N AcetylCysteine
what is king’s criteria?
Predicts prognosis of acetaminophen toxicity based on < arterial pH 7.3 vs
PTT > 100, AND High Creatinine AND grade 3,4 Encephalopathy
why are alcoholics at greater risk (ie need only lower dose) to see acetaminophen toxicity?
Alcoholics – may be eating less food. therefore low glutathione stores. therefore easier to overload the system and achieve NAPQI toxicitiy
Mushroom Poisoning
name of mushroom?
Symptms?
Labs?
Treatment?
Amanita Phalloides (Death Cap Muschroom)
Severe abd pain, nausea, vomiting
Historically do not survive without transplant
very high ALT/AST
Possible Treatment: PCN G; or Milk Thistle vs transplant
Idiosyncratic Hepatotoxins (other iatrogenic liver injury)
- what drugs most commonly?
- Diagnosis?
- Treatment ?
can occur with almost any drug
Idiosyncratic drug reaction within the first 6 months of use
Treatment: stop the drug
Diagnosis – Dx of exclusion; determining causality is difficult
what viruses can lead to acute liver failure?
what are the typical LFT values?
Labs: High ALT/AST (3K - 5K)
Hepatitis A – rare in the US
Hepatitis B – Most common worldwide
Hepatitis D -
Hepatitis E -
Hepatitis C – most common cause of chronic liver disease, but not acute scenario
Hepatitis A –
- how is it transmitted? sources?
Diagnostic lab ?
Fecal/oral transmission; Shellfish, polluted water, poor sanitary conditions
Dx – Hepatitis A IgM (for acute)
Hepatitis B –
- how is it transmitted?
Diagnostic labs?
transmitted via infectious blood, bodily fluids
Child birth transmission
Check both surface and core antibody IgM
surface antigen persists in persons with the chronic infection
acute setting – Surface and core IgM
Delta Hepatitis
- unique feature about this infection?
Diagnositic labs?
Requires a previously existing or concurrent Hepatitis B infection
HD antibody IgM -
Hepatitis E
how is it transmitted?
diagnostic test?
fecal, oral transmission
Self-limiting disease
Dx – No great test; HE Ab IgM
Acute alcoholic hepatitis –
Epi and describe the mortality…
Lab findings?
Symptoms ?
Histology
Treatment?
Not really considered acute liver failure
assocaiated with poor outcomes (high 6 month mortality w/o transplant)
Labs: ALT/ASTs do not exceed 500
AST:ALT ratio – 2:1
histology: hepatocytes; microsteatosis; macrosteatois; Mallory Bodies
treatment: : Glucocorticoids, Pentoxifyline
Wilson's Disease: - demographics at risk? - how is it acquired? - symptoms -
Autosomal recessive
young women
Symptoms: Neurologic, psychiatric symptoms, motor function; acute liver failure; refractory coagulation, hemolytic anemia, renal failure
Diagnosis of Wilsons?
Labs?
pathognomic occular finding on physical exam for wilson’s disease?
24 hour urine or plasma copper collection
Alk Phosph : Total Bilirubin ratio < 4
Kayser Fleishcer rings
Autoimmune Hepatitis:
mechanism of disease ?
labs?
histo?
Diagnostic test?
cell mediated immune response against the liver
More a chronic disease
Uncommonly presents as acute, fulminant hepatitis
Labs – mixed elevation of AST/ALT and high alk phosph/bilirubin
Histology – can be severe with massive liver necrosis
Dx: Autoantibodies
Liver disease in Pregnanccy and Post Partum?
– when during pregnacny course is this most likely to develop?
- what is the treatment?
Confined to third trimester, and after delivery
Increased fetal and maternal mortality
Treatment: deliver the baby and stabilize mom and baby
causes of Liver disease in Pregnanccy and Post Partum?
(what is HELP syndrome?)
lab range
Acute Fatty Liver Disease of Pregnancy
HELP Syndrome – Hemolysis, Elevated Liver Enzymes, Low Platelets
AST/ALT – 300-500 range
Ischemic Hepatitis – (“shock liver”)
- what is it?
- causes?
- what is a concerning pattern of lab findings?
Liver cells necrosis due to diminished perfusion
- Cardiac arrest, hypotension, thrombosis
Rapid increase of AST/ALT with rapid resolution (this means that the hepatocytes are damaged and not even releasing AST/ALT anymore)
what is Budd Chiari Syndrome
what is it?
what is the presentation triad?
Diagnosis methods?
Underlying causes?
Treatment?
Hepatic Vein thrombosis
triad: severe abdominal pain, massive hepatomegaly, new ascites
Diagnosis – look for the clot on Ultrasound, CT or MRI
Underlying Causes: malignacy, hypercoagulable states, myeloproliferative, women on OCPs
Thrombolytics, anticoagulation, TIPS, surgery, and liver Transplant
HCC –
what are the risk factors?
what are 2 methods for screening?
Imaging ?
any patient with cirrhosis
Imaging, AFP
CT with contrast
HCC –
Treatment methods?
Chemo-embolization; direct chemotherapy
Radio-frequency ablation
Sorafenib -- anti VEGF; but only extends live by about 2 months
Transplant
