Disorders of Metabolism Affecting Liver (Lecture 4a)

What is liver steatosis? What are the 2 types?

- Acquired, reversible triglyceride accumulation in hepatocytes

- Types: Alcohol associated FLD & non-alcohol FLD

(response to trauma in liver)

Name 3 causes of liver steatosis, including the most common one.

- Obesity (m/c)

- Alcohol abuse

- Starvation

- Cystic fibrosis,

- Pregnancy

- Type II diabetes

- Hyperlipidemia

Symptoms of steatosis

Usually asymptomatic; symptoms from other disorders.

What is biochemistry for liver steatosis?

LFTs may be normal or slightly elevated

What is the treatment for steatosis?

Remove the stimulus causing liver damage.

What does focal fatty infiltration look like sonographically? What part of the body does it typically affect?

- Focal area of increased echogenicity (hyperechoic)

- Segment 4 of liver most commonly affected

What does focal fatty sparing look like sonographically? What part of the body does it typically affect?

- Focal area of decreased echogenicity

- M/c affects gb fossa and along liver margins

Describe the 3 types of diffuse liver steatosis.

Mild

- minimally inc. echogenicity

- normal vis. of diaphragm and borders

Moderate

- moderate inc. echogenicity

- difficult vis. of IH vessels & diaphragm

Severe

- marked inc. echogenicity

- poor penetration (non vis. of diaphragm and IH vessels)

What is glycogen storage disease?

Congenital enzyme deficiency leading to error in synthesis or degradation of glycogen and subsequent use

What is Von Gierke's disease?

Most common glycogen storage disease type.

What is the clinical presentation of glyogen storage disease (pt population, symptoms)?

- Pt population: children (b/c its's congenital)

- Symptoms: Convulsions, hypoglycemia, lethargy, hepatomegaly

What are the sonofeatures of glycogen storage disease? (HINT: 2)

- Hepatomegaly (m/c)

- Diffusely increased echogenicity

- Associated w/ hepatic adenomas

- May develop HCC

What are the treatments for glycogen storage disease? (HINT: 2)

- NG tube feedings

- Surgical removal of adenomas

What is cirrhosis? (HINT: 3 things)

Normal hepatocytes & kupffer cells replaced by fibrous scar tissue (liver failure) - irreversible

- necrosis

- fibrosis

- regeneration

Name 2 causes of cirrhosis

- Alcoholism (m/c)

- Chronic viral hepatitis (m/c)

- Cardiac disease

- Hemochromatosis (iron storage), Wilson's disease (copper storage), biliary cirrhosis

Name 3 symptoms of cirrhosis

- Asymptomatic until progresses too far

- Classic signs: Hepatomegaly, jaundice, ascites

- Other signs: weight loss, weaknesss, signs of PH

TRUE or FALSE: Cirrhosis can present with normal or slightly elevated LFTs.

FALSE: Cirrhosis always has ABNORMAL LFTs

What are the sonofeatures of cirrhosis?

- Volume/lobar redistribution: usually rt --> lt --> caudate lobe shrinks last (C/RL = 0.65 = cirrhosis)

- Coarse echotexture

- Nodular surface (irregular Glisson's capsule)

- Regenerating nodules (normal hepatocytes)

- Dysplastic nodules (malignant or pre-malignant hepatocytes)

- Doppler: narrowing of HVs

- Signs of PH: recanalized paraumbilical vein, ascities,

(regenerating and dysplastic look indisintinguishable on U/S)

What is the treatment for cirrhosis?

Irreversible; focus on symptom management or transplant

What is Budd-Chiari syndrome?

- Occlusion of 1 or more hepatic veins w/ or w/o a blocked IVC

- Can lead to cirrhosis and liver failture

Name 3 causes of Budd-Chiari syndrome.

- Coagulation disorders,

- Tumors (HCC, RCC, adrenal, etc.)

- Trauma

- Pregnancy

- Oral contraceptives

- Congenital

What is the clinical presentation Budd-Chiari syndrome (pt population and symptoms)

- Pt population: young females on birth control

- Symptoms: ascites, RUQ pain, hepatomegaly, asymptomatic if chronic (collateral devlopment)

What are the sonofeatures of Budd-Chiari syndrome?

- Doppler: Absence of all three hepatic veins

- Caudate lobe enlargement (dec. echogenicity)

- Thickened HV walls, thrombosis, IH collaterals

- Intraluminal echogenicity

- Ascites

- Splenomegaly

What is the treatment for Budd-Chiari syndrome?

- Anticoagulants

- Thrombolytic therapy

- Transplant