L7: Diffuse Liver Diseases Flashcards
what are examples of Diffuse Liver Diseases?
- Non-alcoholic fatty liver disease (NAFLD)
- Autoimmune hepatitis (AIH)
- Inherited Metabolic liver diseases:
a) Wilson’s Disease
b) Hemochromatosis
c) α 1 antitrypsin deficiency
Def of NAFLD
Prevelance of NAFLD
- Most common cause of liver disease in North America
- Growing in Parallel With Obesity and Metabolic Syndrome
- Most common liver disorder in Western industrialized Countries
- Affects ~25% of adult population worldwide
Causes & Risk Factors of NAFLD
CP of NAFLD
Symptoms
Asymptomatic
Rt hypochondrial pain
Dyspepsia
Symptoms of the cause as DM
General Signs
Increased body weight
May be xanthoma & Xanthelasma
Signs of the cause as DM
C) Local examination
Enlarged, soft liver with rounded boarders & may be tender
Investigations of NAFLD
- Lab
- Rad
- Bx
Lab investigations in NAFLD
Rad investigations in NAFLD
Bx in NAFLD
TTT of NAFLD
1) Treatment of the cause as malnutrition
2) Reduction of body weight
3) Reduce CHO & fats in diet
4) Statins
5) Insulin sensitizers as metformin & TZDs
6) Anti-oxidants as vitamin A, C &E
Role of Vit E in NAFLD
Role of Pioglitazone in NAFLD
Is metformin Recommended in NAFLD?
Nope
Progression of steatosis into cirrhosis
Def of Autoimmune Hepatitis
- It is a chronic (>6 months) progressive immune-mediated inflammation of the liver
- Disease in which the body’s own immune system attacks the hepatocyte and causes it to become inflamed.
Incidence of Autoimmune Hepatitis
About 70 percent of those with autoimmune hepatitis are female.
Types of Autoimmune Hepatitis
Pathology of Autoimmune Hepatitis
Mild Autoimmune Hepatitis
M
Severe Autoimmune Hepatitis
Cirrhosis Autoimmune Hepatitis
CP of Autoimmune Hepatitis
Asymptomatic.
Vague symptoms: Fever, headache, anorexia, malaise.
Hepatic symptoms: RUQ pain, jaundice, LCF is late.
Liver: enlarged & firm, may be splenomegaly
Extrahepatic symptoms: GN, arthritis.
Investigations for Autoimmune Hepatitis
TTT of Autoimmune Hepatitis
Def of Wilson’s Disease
- An AR defect in copper metabolism characterized by copper accumulation in hepatocytes and other extra hepatic organs.
- Deficit in gene ATP7B on chromosome 13
Pathophysiology of Wilson’s Disease
- Decreased biliary excretion of copper
- Decreased incorporation of copper into ceruloplasmin
CP of Wilson’s Disease
Investigations of Wilson’s Disease
LFTs in Wilson’s Disease
Ceruplasmin in Wilson’s Disease
Genetic Testing in Wilson’s Disease
Mutation analysis of the ATP7B gene
Bx in Wilson’s Disease
TTT of Wilson’s Disease
what is another name of Hemochromatosis?
Bronzed DM
Def of Hemochromatosis
- Inherited condition leading to increased total body iron with excess deposition in tissues.
- Total body stores of iron increased to 20-40 g (normal 1 g)
Pathology of Hemochromatosis
Etiology of Hemochromatosis
- Primary (hereditary) hemochromatosis
- Secondary hemochromatosis
Parenteral iron overload (e.g. transfusions)
Chronic hemolytic anemia: thalassemia.
Excessive iron intake
Sidroblastic anemia
Causes of Secondary Hemochromatosis
- Parenteral iron overload (e.g. transfusions)
- Chronic hemolytic anemia: thalassemia.
- Excessive iron intake
- Sidroblastic anemia
CP of Hemochromatosis
Investigations for Hemochromatosis
- Blood Tests
- Genetic Testing
- bx
Blood tests in Hemochromatosis
Genetic Testing in Hemochromatosis
for the specific genetic markers —> HFE gene
Bx in Hemochromatosis
TTT of Hemochromatosis
Main TTT of Hemochromatosis
phlebotomy
Alternative therapy in Hemochromatosis
First steps in suspected NAFLD
What is a fibroscan?
- Fibro Scan is an imaging-based diagnostic test developed by Echosens that looks at liver fibrosis (stiffness, scarring) and steatosis (fat) using transient- elastography technology.
- Fibro Scan can be used on patients in need of staging of their NAFLD/NASH
FibroScan Score Ranges