Disorders of the Hepatobiliary system
Dr. Mehzabin Ahmed
Normal functions
The liver is an important organ involved primarily for metabolism. It has
varied functions like:
1. Carbohydrate metabolism: it serves as a storage organ for glycogen and
is the source of plasma glucose
2. Fat metabolism: it converts dietary fats into lipoproteins
3. Protein metabolism: it is involved in the synthesis of plasma proteins
like albumen, coagulation proteins as well as the catabolism of proteins
resulting in the formation of urea.
4. Bile synthesis: the liver secretes bile, which contains bilirubin,
cholesterol, electrolytes and bile salts.
5. Storage organ for glycogen, iron, copper and fat soluble vitamins
6. Detoxification organ: it serves to metabolize nitrogenous compounds,
hormones and drugs.
General clinical features of liver disorders
Jaundice: yellowish discoloration of sclera and skin.
Causes: increased hemolysis (increased formation of bilirubin), liver disease
( impaired conjugation and/or excretion), or biliary obstruction
Dark urine due to increased Urobilinogen
Pale faeces in case of biliary obstruction due to lack of bile pigments
Steatorrhoea passage of fatty stools due to malabsorption of fat due to
lack of bile in biliary obstruction
Ascites and edema due to decreased oncotic pressure (decreased
albumen synthesis). Ascites may also be due to portal hypertension.
Xanthelasma are lipid deposits in the skin due to increased
cholesterol in the blood in biliary obstruction
Pruritis: itching due to bile salt accumulation
Easy bruising and bleeding disorders due to impaired synthesis
of clotting factors
Hepatomegaly increased size of the liver in hepatitis, tumor or
fat/ amyloid infiltration
Haematemesis due to ruptured esophageal varices due to portal
hypertension
Encephalopathy results due to failure of the liver to remove
exogenous or endogenous substances mimicking or altering the
balance of the neurotransmitters.
Major causes of the diseases of the liver
Toxic substances: drugs and alcohol
Infections: viruses, bacteria, parasites
Vascular disorders: results in the disturbances in the blood flow
Disturbances in the bile secretion
Neoplasms of the liver parenchyma or the biliary architecture
Clinical conditions produced by hepatic
disorders
Acute /Chronic hepatitis
Alcoholic liver disease
Chronic liver disease
Acute hepatic failure
Cirrhosis
Cholestasis
Hepatitis
Inflammation of the hepatocytes is called hepatitis.
Causes:
Infectious agents
- Hepatitis virus- five types A, B, C, D & E.
- spread by the feco-oral route (A, E) or by blood (B, C).
- The hepatitis B, C and D infections cause chronic hepatitis
and frequently present as carriers
Drugs
Alcohol
Clinical features:
They produce symptoms like fever, nausea and vomiting, jaundice
(yellowish discoloration of the skin, sclera and the increased yellowish
discoloration of the urine) and tender hepatomegaly (increases in the
size of the liver, tender means painful when palpated).
Hepatitis
Lab investigations: reveal an increase in bilirubin levels in the
blood, and increased levels of ALT & AST (liver enzymes).
Non- viral infections may present as
abscesses- bacterial, protozoal (amoebic-the suppurative material is of
brown color or is called anchovy sauce color),
Cysts: hydatid cysts- due to the dog tape worm (parasite) infestation.
ALCOHOLIC LIVER DISEASE
It is due to chronic alcohol consumption.
The first changes noted are the fatty liver (accumulation of fat
globules in the hepatocytes/ also seen in malnutrition and hypoxic
conditions),
Acute hepatitis (inflammation of the liver) and
Alcoholic liver cirrhosis (fibrosis of the liver with regeneration of
nodules of the hepatocytes).
Chronic liver disease
Some follow an acute liver injury or may remain silent for a long
time.
Seen in
Chronic hepatitis B &C virus infection,
Chronic alcoholism
Inborn metabolic disorders
Metabolic diseases affecting the liver
Hemochromatosis: metabolic error resulting in accumulation of
iron as hemosiderin
Liver cirrhosis + Hemosiderin deposition in the endocrine
organs like the pancreas resulting in the destruction of the islets
cells & gives a brown color in the organs- Bronze Diabetes.
It may b primary (congenital) or secondary (due to repeated
blood transfusions or high iron intake in the diet)
Wilson’s disease: inherited disorder of copper metabolism-
accumulation of copper in the liver, brain (in the basal ganglia) and
cornea (Kayser- Fleischer ring in the cornea)
ACUTE HEPATIC FAILURE
It results in
jaundice,
hepatic encephalopathy (delirium, followed by coma- due to
failure to eliminate the toxic nitrogenous waste), and
bleeding disorders due to deficient synthesis of coagulation
factors like II, VII, IX and X.
The patient presents with
edema,
bleeding disorder,
ascites, encephalopathy,
renal failure,
loss of secondary sex characteristics and gynaecomastia and
appearance of spider naevi (small vascular lesions seen in the skin
in pregnancy) due to hyperoestrogenism.
ACUTE HEPATIC FAILURE
Lab investigations:
The liver function tests reveal the type of liver disease. The ALT &
AST are increased in necrosis of the liver cells,
Alkaline phosphatase- increased in biliary obstruction
Bilirubin- levels are raised
Albumen- synthesis is decreased resulting in edema (decreased
oncotic pressure)
Hematological disorders: coagulation factors are deficient resulting
in an abnormal prothrombin time (seen in chronic liver disease and
failure.
CIRRHOSIS
It is the replacement of normal liver tissue by nodules of regenerating
hepatocytes separated by fibrosis.
It is the end stage of many liver diseases like:
1. Alcoholic liver disease
2. Hepatitis B, C infections
3. Idiopathic
4. Biliary obstruction
5. Hemochromatosis
Classified as
1. Morphologically as micronodular (upto 3mm diameter-alcoholic
cirrhosis) or macronodular (>3mm diameter- greater risk of cancer
development)
2. Etiologically as viral, alcoholic, hemochromatosis, autoimmune, biliary
obstruction, Wilson’s diseqse
Clinically the patient presents with
longstanding cirrhosis resulting in liver failure and portal hypertension and
predisposes to hepatocellular carcinoma
Hepatic stellate cells are converted into collagen producing myofibroblasts,
by :
1. Disruption of the extra cellular matrix
2. Secretion of cytokines by the Endothelial cells, Lymphocytes, Kuppfer cells,
Hepatocytes
3. Direct action of the toxins on the Hepatic stellate cells
Vascular disorders of the liver
Passive venous congestion of the liver in right sided heart failure- nutmeg
liver
Portal hypertension: results from obstruction to blood flow in the portal
system
Causes:
Pre hepatic: portal vein thrombosis and massive splenomegaly
Hepatic: cirrhosis is the most common cause, sarcoidosis, schistosomiasis,
chronic active hepatitis
Post hepatic: cardiac failure, constrictive pericarditis
Clinical features: The patients present with
splenomegaly (increased size of the spleen),
ascites (fluid collection in the peritoneum),
dilation of the porta- systemic shunts in the lower esophagus (esophageal
varices), anus (hemorrhoids) and around the umbilicus (caput medusae)
Diseases of the gall bladder &
extra hepatic biliary ducts
Cholelithiasis
Formation of stones in the gall bladder.
There are two types of gall stones, cholesterol and pigment stones.
The risk factors for development of cholesterol stones include female
gender, obesity and diabetes mellitus.
Pigment stones occur in hemolytic anemias (increased production of
bilirubin from the hemoglobin of the hemolysed RBCs).
Consequences of gallstones are
cholecystitis (inflammation),
predisposition to carcinoma,
obstruction to the bile flow resulting in jaundice,
infections of the static bile,
pancreatitis and
formation of mucocele (accumulation of mucus in the gall bladder
resulting in its distension)
Cholecystitis: inflammation of the gallbladder
Acute cholecystitis: usually due to obstruction of the bile outflow.
Intense inflammation and infection can result in empyema ( collection
of pus in a hollow organ or cavity)
Chronic cholecystitis: almost always associated with gallstones. The
walls of the GB are thickened due to fibrosis
Carcinoma of the gall bladder:
Adenocarcinoma occurs almost invariably in association with
gallstones.
Obstructive jaundice
Bile duct obstruction may be due to :
a. gallstones
b. carcinoma of the common bile duct
c. carcinoma of the head of the pancreas
d. inflammatory stricture of the common bile duct
e. accidental surgical ligation of the common bile duct
The patient presents with
jaundice,
pale stools and
dark urine.
If the obstruction persists the static bile may undergo infection
causing cholangitis and liver abscesses and
malabsorption of fat and fat-soluble vitamins.
Diseases of the exocrine pancreas
Acute pancreatitis-
Results in acute abdomen,
Usually due to duct obstruction, shock, alcohol ingestion,
mumps infection, trauma and action of toxin.
It is often hemorrhagic.
Fat necrosis in the surrounding tissue occurs due to the action of
the enzymes
Chronic pancreatitis-
most commonly caused by chronic alcoholism.
Fibrosis and exocrine atrophy occurs.
Malabsorption may result due to loss of pancreatic secretions
