Secondary biliary cirrhosis

Gastroenterology

Clinicals - History

Fact Explanation
Gallstone disease It is the most common cause of biliary obstruction and commonly seen in women than in men. Long standing obstruction of the bile duct due to stones, tumors, strictures can cause secondary biliary cirrhosis. Initially there's inflammation of the liver which later progresses to fibrosis and cirrhosis. . Gallstone disease
It is the most common cause of biliary obstruction and commonly seen in women than in men. Long standing obstruction of the bile duct due to stones, tumors, strictures can cause secondary biliary cirrhosis. Initially there's inflammation of the liver which later progresses to fibrosis and cirrhosis. .
Yellowish discolouration of skin and sclera bilirubin secretion from liver is blocked therefore it's released into the blood stream and can get deposited in the skin and sclera causing jaundice or yellowish discoloration. Yellowish discolouration of skin and sclera
bilirubin secretion from liver is blocked therefore it's released into the blood stream and can get deposited in the skin and sclera causing jaundice or yellowish discoloration.
Dark urine Urobilinogen is absent as secretion of bilirubin from the liver is blocked. Instead conjugated bilirubin is passed into the urine which gives the dark (tea) color to urine. Dark urine
Urobilinogen is absent as secretion of bilirubin from the liver is blocked. Instead conjugated bilirubin is passed into the urine which gives the dark (tea) color to urine.
Pale stools As urobilinogen is absent, there's no production of stercobilin in intestines therefore stools appear pale. Pale stools
As urobilinogen is absent, there's no production of stercobilin in intestines therefore stools appear pale.
itching (Pruritus) Deposition of bile acids in the is thought to cause pruritus. Recently it has been attributed to opioid release in the body as well itching (Pruritus)
Deposition of bile acids in the is thought to cause pruritus. Recently it has been attributed to opioid release in the body as well
Recurrent bouts of right hypochondrial pain When biliary cirrhosis is secondary to gall stone disease, recurrent attacks of right hypochondrial pain may result and the jaundice is painful. Recurrent bouts of right hypochondrial pain
When biliary cirrhosis is secondary to gall stone disease, recurrent attacks of right hypochondrial pain may result and the jaundice is painful.
Anorexia, nauea These are symptoms of advanced cirrhosis as well as felt during an episode of acute cholecystitis due to gallstones. Anorexia, nauea
These are symptoms of advanced cirrhosis as well as felt during an episode of acute cholecystitis due to gallstones.
Weight loss This is a feature of malignancy and particularly if there's malignant obstruction of the biliary tree by a cholangiocarcinoma or cancer in the pancreas. Weight loss
This is a feature of malignancy and particularly if there's malignant obstruction of the biliary tree by a cholangiocarcinoma or cancer in the pancreas.
Swelling of the abdomen This is a feature of cirrhosis and liver failure, there's accumulation of fluid in the abdominal cavity. This transudation of fluid could be due to imbalance of Starling forces as there's reduced oncotic pressure due to reduced albumin production from the liver and increased hydrostatic pressure due to portal vein hypertension. Swelling of the abdomen
This is a feature of cirrhosis and liver failure, there's accumulation of fluid in the abdominal cavity. This transudation of fluid could be due to imbalance of Starling forces as there's reduced oncotic pressure due to reduced albumin production from the liver and increased hydrostatic pressure due to portal vein hypertension.
Ankle swelling This is also a feature of liver failure. There's transudation of fluid due to imbalance of Starling forces as there's reduced oncotic pressure due to reduced albumin production from the liver and increased hydrostatic pressure due to portal vein hypertension. Ankle swelling
This is also a feature of liver failure. There's transudation of fluid due to imbalance of Starling forces as there's reduced oncotic pressure due to reduced albumin production from the liver and increased hydrostatic pressure due to portal vein hypertension.
Reversal of sleep patterns and altered level of consciousness This is a feature of hepatic encephalopathy and is seen in advanced cirrhosis. This is thought to be due to accumulation of toxic ammonia and imbalance of neurotransmitters in brain. Reversal of sleep patterns and altered level of consciousness
This is a feature of hepatic encephalopathy and is seen in advanced cirrhosis. This is thought to be due to accumulation of toxic ammonia and imbalance of neurotransmitters in brain.

Clinicals - Examination

Fact Explanation
Icterus Bilirubin secretion from liver is blocked therefore it's released into the blood stream and can get deposited in the skin and sclera causing jaundice or yellowish discoloration. Icterus
Bilirubin secretion from liver is blocked therefore it's released into the blood stream and can get deposited in the skin and sclera causing jaundice or yellowish discoloration.
Cachexia This is a feature of malignancy and particularly if there's malignant obstruction of the biliary tree by a cholangiocarcinoma or cancer in the pancreas. Cachexia
This is a feature of malignancy and particularly if there's malignant obstruction of the biliary tree by a cholangiocarcinoma or cancer in the pancreas.
Mass in the abdomen Palpable gall bladder in the presence of obstructive jaundice is a feature of malignancy and provides the basis for Courvoisier's law. Mass in the abdomen could also be the malignant mass of the pancreas or cholangiocarcinoma. Mass in the abdomen
Palpable gall bladder in the presence of obstructive jaundice is a feature of malignancy and provides the basis for Courvoisier's law. Mass in the abdomen could also be the malignant mass of the pancreas or cholangiocarcinoma.
Ascitis This is a feature of cirrhosis and liver failure, there's accumulation of fluid in the abdominal cavity. This transudation of fluid could be due to imbalance of Starling forces as there's reduced oncotic pressure due to reduced albumin production from the liver and increased hydrostatic pressure due to portal vein hypertension. Ascitis
This is a feature of cirrhosis and liver failure, there's accumulation of fluid in the abdominal cavity. This transudation of fluid could be due to imbalance of Starling forces as there's reduced oncotic pressure due to reduced albumin production from the liver and increased hydrostatic pressure due to portal vein hypertension.
Pitting odema This is also a feature of liver failure. There's transudation of fluid due to imbalance of Starling forces as there's reduced oncotic pressure due to reduced albumin production from the liver and increased hydrostatic pressure due to portal vein hypertension. Pitting odema
This is also a feature of liver failure. There's transudation of fluid due to imbalance of Starling forces as there's reduced oncotic pressure due to reduced albumin production from the liver and increased hydrostatic pressure due to portal vein hypertension.
Dilated veins in the abdomen (Caput medusae) This is due to opening up of collaterals between portal and systemic circulation in the abdominal wall due to portal hypertension. Dilated veins in the abdomen (Caput medusae)
This is due to opening up of collaterals between portal and systemic circulation in the abdominal wall due to portal hypertension.
Right hypochondrial tenderness When biliary cirrhosis is secondary to gallstone disease, recurrent attacks of right hypochondrial pain may result and the jaundice is painful Right hypochondrial tenderness
When biliary cirrhosis is secondary to gallstone disease, recurrent attacks of right hypochondrial pain may result and the jaundice is painful
Scratch marks Deposition of bile acids in the is thought to cause pruritus and resultant scratch marks. Recently it has been attributed to opioid release in the body as well. Scratch marks
Deposition of bile acids in the is thought to cause pruritus and resultant scratch marks. Recently it has been attributed to opioid release in the body as well.
Flapping tremor/ Asterexis This is a feature of hepatic encephalopathy in advanced liver failure and attributed to accumulation of ammonia and imbalance of other neurotransmitters in the brain Flapping tremor/ Asterexis
This is a feature of hepatic encephalopathy in advanced liver failure and attributed to accumulation of ammonia and imbalance of other neurotransmitters in the brain

Investigations - Diagnosis

Fact Explanation
Serum bilirubin with indirect and direct fractions Serum bilirubin values (especially direct or the conjugated fraction) are usually elevated. Cholestasis causes elevated conjugated (direct) bilirubin where as liver cell damage causes elevation of unconjugated (Indirect) bilirubin Serum bilirubin with indirect and direct fractions
Serum bilirubin values (especially direct or the conjugated fraction) are usually elevated. Cholestasis causes elevated conjugated (direct) bilirubin where as liver cell damage causes elevation of unconjugated (Indirect) bilirubin
Alkaline phosphatase (ALP) ALP levels are very high in biliary obstruction. Extrahepatic obstruction: ALP levels are elevated to high levels but in intrahepatic obstruction, ALP levels are elevated, but <3 times the upper limit of the reference range. Alkaline phosphatase (ALP)
ALP levels are very high in biliary obstruction. Extrahepatic obstruction: ALP levels are elevated to high levels but in intrahepatic obstruction, ALP levels are elevated, but <3 times the upper limit of the reference range.
Serum Transaminases (ALT, AST) Only moderately high levels are observed in patients with cholestasis Serum Transaminases (ALT, AST)
Only moderately high levels are observed in patients with cholestasis
Gamma glutamyl Transferase (GGT) Biliary obstruction elevates GGT. Gamma glutamyl Transferase (GGT)
Biliary obstruction elevates GGT.
Prothrombin time (PT) and international normalization ratio (PT-INR) PT can be prolonged as a result of vitamin K malabsorption due to inadequate fat absorption. Prothrombin time (PT) and international normalization ratio (PT-INR)
PT can be prolonged as a result of vitamin K malabsorption due to inadequate fat absorption.
Urine full report Urine bilirubin is normally absent but conjugated bilirubin is present. Urine full report
Urine bilirubin is normally absent but conjugated bilirubin is present.
Antimitochondrial antibody The presence of high levels of antimitochondrial antibodies, is indicative of PBC. Antimitochondrial antibody
The presence of high levels of antimitochondrial antibodies, is indicative of PBC.
Hepatitis serology Because viral hepatitis maybe difficult to differentiate from extrahepatic obstructive causes, this is done Hepatitis serology
Because viral hepatitis maybe difficult to differentiate from extrahepatic obstructive causes, this is done
Plain abdominal x-rays Plain radiographs are not veru helpful as only 10% of gall stones are seen abdominal x-ray. Plain abdominal x-rays
Plain radiographs are not veru helpful as only 10% of gall stones are seen abdominal x-ray.
Ultrasound scan of the abdomen This is the most sensitive technique for visualizing the biliary system, mainly gallbladder. But not helpful to visualize bile ducts. It's safe and cheap as well Ultrasound scan of the abdomen
This is the most sensitive technique for visualizing the biliary system, mainly gallbladder. But not helpful to visualize bile ducts. It's safe and cheap as well
CT scan of the abdomen Cause and level of obstruction, with visualization of liver is possible with this CT scan of the abdomen
Cause and level of obstruction, with visualization of liver is possible with this
Endoscopic retrograde cholangiopancreatography (ERCP) This is therapeutic as well and more useful to visualize distal bile duct obstruction with proper visualization of the duct system. Endoscopic retrograde cholangiopancreatography (ERCP)
This is therapeutic as well and more useful to visualize distal bile duct obstruction with proper visualization of the duct system.
Magnetic resonance cholangiopancreatography (MRCP) This is a noninvasive method and the determination of the type of tumor and it's extent is possible. Magnetic resonance cholangiopancreatography (MRCP)
This is a noninvasive method and the determination of the type of tumor and it's extent is possible.
Percutaneous transhepatic cholangiogram (PTC) This is especially useful for proximal lesions and the liver is punctured via a needle to enter into the duct system inside the liver Percutaneous transhepatic cholangiogram (PTC)
This is especially useful for proximal lesions and the liver is punctured via a needle to enter into the duct system inside the liver
Endoscopic ultrasound (EUS) This combines endoscopy and ultrasound to visualize the hepatic-biliary system Endoscopic ultrasound (EUS)
This combines endoscopy and ultrasound to visualize the hepatic-biliary system
CT cholangiography Radiolucent stones may be seen with this technique CT cholangiography
Radiolucent stones may be seen with this technique

Investigations - Management

Fact Explanation
Serum Bilirubin This is included in Child-Pugh score (sometimes the Child-Turcotte-Pugh score) which is used to assess the prognosis of chronic liver disease depending on the level and also in MELD score. Serum Bilirubin
This is included in Child-Pugh score (sometimes the Child-Turcotte-Pugh score) which is used to assess the prognosis of chronic liver disease depending on the level and also in MELD score.
Serum creatinine This is included in MELD score (Model For End-Stage Liver Disease) for planing liver transplant Serum creatinine
This is included in MELD score (Model For End-Stage Liver Disease) for planing liver transplant
Serum electrolytes As the patient is on diuretics in liver failure, monitoring of serum electrolytes is essential. Serum electrolytes
As the patient is on diuretics in liver failure, monitoring of serum electrolytes is essential.
Serum Albumin This is included in Child-Pugh score (sometimes the Child-Turcotte-Pugh score) which is used to assess the prognosis of chronic liver disease depending on the level. Serum Albumin
This is included in Child-Pugh score (sometimes the Child-Turcotte-Pugh score) which is used to assess the prognosis of chronic liver disease depending on the level.
Prothrombin type-international normalization ratio This is included in Child-Pugh score (sometimes the Child-Turcotte-Pugh score) which is used to assess the prognosis of chronic liver disease depending on the level. Prothrombin type-international normalization ratio
This is included in Child-Pugh score (sometimes the Child-Turcotte-Pugh score) which is used to assess the prognosis of chronic liver disease depending on the level.
Serum Albumin Low serum albumin indicates chronic liver cell disease and low albumin causes delayed wound healing and anastomotic healing. Therefore this is done prior to any surgeries Serum Albumin
Low serum albumin indicates chronic liver cell disease and low albumin causes delayed wound healing and anastomotic healing. Therefore this is done prior to any surgeries
Prothrombin time and international normalization ratio Bleeding due to low vitamin K is seen in chronic liver cell disease and this test is done prior to any invasive procedures and correction is done Prothrombin time and international normalization ratio
Bleeding due to low vitamin K is seen in chronic liver cell disease and this test is done prior to any invasive procedures and correction is done
Renal function tests including estimated glomerular filteration rate, serum creatinine, blood urea nitrogen To assess fitness for anesthesia Renal function tests including estimated glomerular filteration rate, serum creatinine, blood urea nitrogen
To assess fitness for anesthesia
Full blood count To exclude anaemia. Full blood count
To exclude anaemia.
Antimitochondrial antibody level The presence of high levels of antimitochondrial antibodies, is indicative of PBC. Antimitochondrial antibody level
The presence of high levels of antimitochondrial antibodies, is indicative of PBC.
Hepatitis serology Because viral hepatitis maybe difficult to differentiate from extrahepatic obstructive causes Hepatitis serology
Because viral hepatitis maybe difficult to differentiate from extrahepatic obstructive causes
Serum bilirubin This is included in Child-Pugh score (sometimes the Child-Turcotte-Pugh score) which is used to assess the prognosis of chronic liver disease depending on the level and also in MELD score. Serum bilirubin
This is included in Child-Pugh score (sometimes the Child-Turcotte-Pugh score) which is used to assess the prognosis of chronic liver disease depending on the level and also in MELD score.
Prothromin time and international normalization ratio This is included in Child-Pugh score (sometimes the Child-Turcotte-Pugh score) which is used to assess the prognosis of chronic liver disease depending on the level. Prothromin time and international normalization ratio
This is included in Child-Pugh score (sometimes the Child-Turcotte-Pugh score) which is used to assess the prognosis of chronic liver disease depending on the level.

Management - Supportive

Fact Explanation
Patient education Patient should be educated regarding the aetiology, nature, course and prognosis of the disease, available options of treatment. Patient education
Patient should be educated regarding the aetiology, nature, course and prognosis of the disease, available options of treatment.
Diet Obesity is regarded as a risk factor to develop gall stones therefore weight reduction maybe helpful. Diet
Obesity is regarded as a risk factor to develop gall stones therefore weight reduction maybe helpful.
Activity Regular exercise may help to reduce weight and obesity Activity
Regular exercise may help to reduce weight and obesity
Liver failure regime When patient develops cirrhosis and liver failure, the liver failure regime including diuretics (Furosemide, ACE inhibitors), vitamin supplements, albumin if indicated, low salt diet is advised. Liver failure regime
When patient develops cirrhosis and liver failure, the liver failure regime including diuretics (Furosemide, ACE inhibitors), vitamin supplements, albumin if indicated, low salt diet is advised.
Bile acid–binding resins Cholestyramine or colestipol reduce the symptoms of pruritus Bile acid–binding resins
Cholestyramine or colestipol reduce the symptoms of pruritus
Antihistamines It also helps to reduce the symptoms of pruritus Antihistamines
It also helps to reduce the symptoms of pruritus
Rifampin This is also known to reduce the symptoms of pruritus . Rifampin
This is also known to reduce the symptoms of pruritus .

Management - Specific

Fact Explanation
Medical therapy for gall stone disease Ursodeoxycholic acid is helpful for treatment for cholesterol containing stones however it tends to recur after stopping the therapy Medical therapy for gall stone disease
Ursodeoxycholic acid is helpful for treatment for cholesterol containing stones however it tends to recur after stopping the therapy
Therapetic Endoscopic retrograde cholangiopancreatography (ERCP) for gall stone disease and strictures ERCP is therapeutic as it relieves obstructions after sphincterotomy, removal of stones, , and the placement of stents in strictures. Therapetic Endoscopic retrograde cholangiopancreatography (ERCP) for gall stone disease and strictures
ERCP is therapeutic as it relieves obstructions after sphincterotomy, removal of stones, , and the placement of stents in strictures.
Extracorporeal shock-wave lithotripsy for gall stone disease This causes dissolution of stones Extracorporeal shock-wave lithotripsy for gall stone disease
This causes dissolution of stones
Surgical care (Cholecystectomy, open bile duct surgery, removal of tumours) When symptoms due to gall stones are seen removal of the gall bladder is done and commonly done laparoscopically. Open bile duct surgery is carried out too but not very commonly. Attempts are also made to resect tumors. Surgical care (Cholecystectomy, open bile duct surgery, removal of tumours)
When symptoms due to gall stones are seen removal of the gall bladder is done and commonly done laparoscopically. Open bile duct surgery is carried out too but not very commonly. Attempts are also made to resect tumors.
Liver transplanation This is the last option in advanced liver failure. Liver transplanation
This is the last option in advanced liver failure.

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