Fibrosis caused by what three mechanisms?
Inflammation- cytokines signal collagen deposition
Wound healing (scar tissue)
Direct stimulators of fibrosis (HBV, Carbon Tetrachloride)
Types of cirrhosis: Laennec Cirrhosis, other name?
Micronodular Cirrhosis
Attributes of micro nodular cirrhosis?
- Acute manifestation
- < 3 mm in diameter
- No landmarks of lobular
architecture in the form of portal
tracts or central vein - Connective tissue is thin
- Primary Etiology:
- Alcoholic cirrhosis is the prototype
- Hemochromatosis
- Wilson disease
Types of Cirrhosis: Chronic hepatitis, other name?
Macronodular Cirrhosis
Attributes of Chronic Cirrhosis?
- Submassive confluent necrosis in
which the liver is grossly misshapen - Connective tissues are
characteristically broad and contain
elements of portal tracts,
mononuclear inflammatory cells and
proliferated ductules - Micronodular may be converted into a
macro pattern by continued
regeneration and expansion of
existing nodules - Etiology: HBV, HCV
Manifestations of Cirrhosis?
What does albumen do?
Moves fluid
Where is albumen made?
Liver
what happens when liver is impaired and can’t make albumen?
fluid builds up in interstitial tissues and around other vessels
What’s in a portal triad?
branch of bile duct
branch of hepatic portal vein
branch of hepatic artery
Hepatic lobules
Portal Hypertension caused by
Tissue destruction causes loss of hepatic vascular volume, increasing pressure in the portal vein
Portal hypertension results in:
Increased congestion at portocaval anastomoses results in the following manifestations:
* Esophageal varices
* Hemorrhoids
* Caput medusae
* Splenomegaly and hypersplenism with pancytopenia
What disease is this?
Autosomal recessive genetic
disorder
* Defect in WD gene codes
(ATP7B chromosome 13)
* Decreased incorporation of
copper into ceruloplasmin
(copper binding protein)
production
* Defect in hepatic copper
transport into bile
Wilson Disease
Pathogenesis of Wilson Disease?
Accumulation of free copper, toxic to many tissue
Wilson Disease expressions?
- Hepar: Fatty change, chronic hepatitis, micronodular cirrhosis
- Basal Ganglia: Movement disorders similar to Parkinson disease with additional psychiatric
manifestations - Eyes: Kayser-Fleischer rings around the iris
Treatment for Wilson Disease?
Life-Long:
* Penicillamine (Cuprimine, Depen)
* Chelating agent
* Can cause serious side effects à Skin and kidney problems, bone marrow suppression and worsening of neurological symptoms
* Used cautiously if having penicillin allergy
* Suppress vitamin B-6 (pyridoxine)
- Trientine (Syprine)
- Trientine works much like penicillamine but tends to cause fewer side effects
- Neurological symptoms can also worsen
Other treatment for Wilson Disease
- Zinc acetate (Galzin)
- Prevents body from absorbing copper from the diet
- Typically used as maintenance therapy to prevent copper from building up
again after treatment with penicillamine or trientine - Zinc acetate might be used as primary therapy if unable to take penicillamine
or trientine - Zinc acetate can cause stomach upset
Disease of increased blood and tissue iron levels that leads to injury, called what?
Hemochromatosis
Primary (hereditary) hemochromatosis cause?
- Autosomal recessive mutation on HFE gene (C28Y)
- Increased small Intestine absorption of Iron
Secondary Hemochromatosis cause?
Chronic transfusion in anemias, build up of too much iron
Results of Hemochromatosis
- Toxic iron deposits in multiple organs:
- Liver > Micronodular cirrhosis and
increased risk of HCC - Heart > Restrictive cardiomyopathy
- Pancreas > Acquired diabetes mellitus
- Skin > Bronze pigmentation (bronze
diabetes) - Gonads > Hypogonadism
Spectrum of gallbladder diseases
- Stone formation in Gall Bladder
- Colicky pain (waves, with peristalsis)
- Worse with fatty foods
- Diagnose > RUQ Ultrasound
- Tx > Elective cholecystectomy
cholelithiasis
