liver patho part 2

Glycogen Storage Disease (GSD)

rare genetic disorder resulting in abnormally large deposits of glycogen in the liver

GSD AKA

Von Gierke Disease Type 1 AKA

associated w/ GSD

fatty infiltration and adenomas

GSD sono findings

hepatomegaly, adenomas, enlarged kidneys, splenomegaly, and increased liver echogenicity

Wilson’s Disease

rare, inherited disorder that causes accumulation of copper in liver, brain, and vital organs due to scarring as liver tries to heal

Wilson’s disease can lead to _____ and _____

cirrhosis, liver failure

hepatitis

inflammation and infection of the liver that can be caused by infection elsewhere in body like mono, or drug toxicity

viral hepatitis

local infection and accounts for most cases; consists of hep a/b/c

hep a transmission

fecal/oral

hep b/c transmission

blood/bodily fluid

Hep _____ is the most dangerous to ______ workers

B, healthcare

HCV and its associated _____ is the most frequent indication for ______

chronic liver disease, liver transplant

hepatitis can be fatal due to _____, _____, _____

portal hypertension, cirrhosis, HCC risk

acute hepatitis sono findings

starry night (periportal cuffing), hepatosplenomegaly, hyperechoic PV walls, thickened GB wall

acute hepatitis labs

elevated AST/ALT/bilirubin

chronic hepatitis

lasting >6 mo and usually progresses to cirrhosis and liver failure

chronic hepatitis sono findings

hyperechoic parenchyma, small liver, decreased echogenicity due to fibrosis in PV walls

portal hypertension normal pressure

5-10 mmHg

portal hypertension clinical findings

upper GI hemorrhage due to esophageal varices rupture, hematesis, hepatic encephalopathy, caput medusa

hematesis

vomiting blood

hepatic encephalopathy

toxins accumulate in brain

portal hypertension sono findings

splenomegaly, ascites, portal systemic collaterals

portal hypertension surgical treatment

portacaval shunt, splenorenal/distal splenorenal shunt, and TIPS

distal splenorenal shunt aka

warren shunt aka

splenorenal shunt aka

linton shunt aka

recanalized umbilical vein

collateral from LPV to epigastric veins to IVC

portal systemic collaterals

gastroesophageal, splenorenal, intestinal, and rectal varices

gastroesophageal varices

left gastric vein drains both the gastric walls, forms loop with right gastric and drains into PV. Then communicates with lower esophageal veins through several anast channels and is the most dangerous due to risk of hemorrhage

splenorenal varices

blood is diverted from splenic to renal vein

intestinal varices

veins of retroperitoneal organs anast with systemic tributaries

rectal varices (hemorrhoids)

IMV drains into rectal veins which connect systemic tributaries

portal systemic collateral clinical findings

caput medusa, hemorrhoids, ascites

TIPS placement

via IJV, in between RHV/RPV

normal TIPS velocity

90-190 cm/s

*why TIPS is used*

helps avoid rupture of gastroesophageal varices and accumulation of ascites

TIPS malfunction

<50 or 190< cm/s, hepatopedal LPV/RPV, hepatofugal MPV, absent flow

Left Portal Vein with TIPS

may have either flow if umbilical vein is recanalized

Gore Viatorr endoprosthesis

most common material used for TIPS but can retain air

portal vein thrombosis

mostly results from PV hypertension, cavernous transformation of PV when extensive

pv thrombosis sono findings

thrombus, dilated, cavernous transformation, portal systemic collaterals

pv thrombosis non-tumoral causes

pancreatitis, cirrhosis/hepatitis, IBD, trauma, splenectomy, hypercoagulation, portal lymphadenopathy

pv thrombosis tumoral causes

HCC, liver mets, pancreatic carcinoma

caput medusa

dilated abdomen wall veins

budd chiari

hepatic vein thrombosis with possible IVC involvement but not related to heart

budd chiari sono findings

thrombus, ascites, hepatosplenomegaly

budd chiari causes

idiopathic (50%), congenital (hypercoagulable), infection, pregnancy, tumors

budd chiari results in ______ and decreased blood flow which cause rt/lt lobe ______

portal vein hypertension, atrophy

low elasticity and stiffness

may signal disease in elastography

contrast CEUS

IV agent that uses microbubbles to demonstrate blood flow

portal vein gas in infants

due to necrotizing enterocolitis and affects premies due to bowel ischemia, GI tract inflammation, or post-op

necrotizing entercolitis

intestines are infected and can die

assoc. w/ HIV/AIDS

hep b/c, cholangitis, and other biliary duct pathologies

MELD

model for end-stage liver disease

most common cause for liver transplant in adults is ______ and in children is _____

cirrhosis, biliary atresia

post-op, the _____ is the only blood supply to the biliary tree and thrombosis/stenosis leads to ______

hepatic artery, biliary complications