What is Vanishing bile duct?

Vanishing bile duct syndrome (VBDS) is a rare but serious outcome and complication of drug induced liver injury marked clinically by chronic cholestasis and histologically by loss of intrahepatic bile ducts. VBDS typically occurs after a bout of severe cholestatic hepatitis, often with immunoallergic features.

What is chronic cholestasis?

Chronic cholestatic diseases, whether occurring in infancy, childhood or adulthood, are characterized by defective bile acid transport from the liver to the intestine, which is caused by primary damage to the biliary epithelium in most cases.

Where are the bile ducts?

About the bile ducts The bile ducts are a series of thin tubes that go from the liver to the small intestine. Their main job is to allow a fluid called bile to go from the liver and gallbladder into the small intestine, where it helps digest the fats in food.

How does biliary atresia cause cirrhosis?

When a baby has biliary atresia, bile flow from the liver to the gallbladder is blocked. This causes the bile to be trapped inside the liver, quickly causing damage and scarring of the liver cells (cirrhosis), and eventually liver failure.

What is mirizzi?

INTRODUCTION. Mirizzi syndrome is defined as common hepatic duct obstruction caused by extrinsic compression from an impacted stone in the cystic duct or infundibulum of the gallbladder [1-3]. Patients with Mirizzi syndrome can present with jaundice, fever, and right upper quadrant pain.

What is inflammation of the bile duct?

Cholangitis is an inflammation of the bile duct system. The bile duct system carries bile from your liver and gallbladder into the first part of your small intestine (the duodenum). In most cases cholangitis is caused by a bacterial infection, and often happens suddenly. But in some cases it may be long-term (chronic).

What happens if cholestasis goes untreated?

Babies who get cholestasis may show signs of jaundice 3 to 6 weeks after they’re born. If your cholestasis goes untreated, you may have trouble absorbing nutrients. You may not get enough calcium and vitamin D. This can weaken your bones.

What triggers cholestasis?

Causes include acute hepatitis , alcohol-related liver disease , primary biliary cholangitis with inflammation and scarring of the bile ducts, cirrhosis due to viral hepatitis B or C (also with inflammation and scarring of the bile ducts), certain drugs (for example, amoxicillin/clavulanate, chlorpromazine.

How do you unblock your bile duct?

Some of the treatment options include a cholecystectomy and an ERCP. A cholecystectomy is the removal of the gallbladder if there are gallstones. An ERCP may be sufficient to remove small stones from the common bile duct or to place a stent inside the duct to restore bile flow.

What are the common signs and symptoms of biliary disease?

Pancreatic and biliary symptoms can include, but are not limited to:

• Abdominal swelling.
• Dark-colored urine.
• Diarrhea, especially with fat droplets in the stools.
• Gas, hiccups and belching.
• Itchy skin.
• Jaundice—a yellowing of the skin and the whites of the eyes.
• Loss of appetite or unexplained weight loss.
• Nausea.

Is biliary atresia hereditary?

Biliary atresia is not an inherited disease; rare genetic variants are being reported in children who also have non-liver defects (see above).

What is the meaning of the word ductopenia?

Here are all the possible meanings and translations of the word ductopenia. Ductopenia is the histological hallmark of Vanishing Bile Duct Syndrome.The ductopenia is arbitrarily defined as less than 0.5 bile ducts per portal triad. The most common cause of Ductopenia is Primary Biliary Cirrhosis.

What is the clinical course of ductopenia in liver?

Formally, ductopenia is diagnosed when 50% of the portal tracts lack interlobular or septal bile ducts. 61 The clinical course is variable. Some patients follow a benign course, whereas others progress to biliary cirrhosis, necessitating liver transplantation. 62,63 Yoh Zen,

How old do you have to be to have ductopenia?

PBC is a slowly progressive, obliterative autoimmune cholangiopathy involving the small and medium-sized bile ducts. It leads to ductopenia, progressive fibrosis, cholestasis, and liver failure. PBC is predominantly a disease of women (95% of cases) between the ages of 20 and 60 years.

What kind of foci are found in ductopenia?

Foci of small hepatocytes with basophilic cytoplasm and hyperchromatic nuclei (small-cell dysplasia) or hepatocytes with enlarged, pleomorphic nuclei (large-cell dysplasia) are occasionally found. 124 Canalicular cholestasis is unusual in early PBC unless there is a complicating factor such as steroid-induced jaundice.