Biliary Cirrhosis - Intrahepatic Bile Ductules Obstruction flac album

Whenever there is bile duct dilatation, the first priority is to look for obstruction. Obstruction If there is an obstruction, we first look for gallstones in the bile duct. If there are no gallstones involved, we then look for strictures. The differential diagnosis for a stricture is based on the location. ERCP: Caroli disease with severe intrahepatic duct dilatation. These patients are also at risk of developing biliary cirrhosis and cholangiocarcinoma. Recurrent pyogenic cholangitis of the left lobe with intrahepatic stones. The left lobe is the most common location of the disease due to the delayed drainage of the left system.

PRIMARY BILIARY CIRRHOSIS A chronic cholestatic disease due to a non-suppurative destructive cholangitis of intrahepatic bile ducts, immune mediated. Age range 20-80years, peak at May be associated with other autoimmune diseases. Very insidious onset, may be asymptomatic for decades, pruritis, fatigue, xanthelasmas, leading to frank cholestatic jaundice, cirrhosis % positive antimitochondrial antibodies (AMA) against E2 subunit of pyruvate dehydrogenase complex inner mitochondrial membrane. Characteristically a granulomatous destruction of bile ducts.

Sections Biliary Obstruction. Biliary obstruction refers to the blockage of any duct that carries bile from the liver to the gallbladder or from the gallbladder to the small intestine. This can occur at various levels within the biliary system. The major signs and symptoms of biliary obstruction result directly from the accumulation of bilirubin and bile salts in the blood and the failure of bile to reach its proper destination. The clinical setting of cholestasis or failure of biliary flow may be due to biliary obstruction by mechanical means or by metabolic factors in the hepatic cells. Causes of biliary obstruction can be separated into intrahepatic and extrahepatic. Mechanical or intrahepatic causes are most commonly hepatitis and cirrhosis. Drugs may also cause direct damage to hepatocytes and metabolic obstruction.

Published: August 2010. Primary Biliary Cirrhosis. Primary Sclerosing Cholangitis. Other Conditions Associated with Intrahepatic Cholestasis. The term cholestasis originally derives from the Greek and literally means a standing still of bile. This disruption of bile flow can occur on a cellular level in the hepatocyte, at the level of the intrahepatic biliary ductules, or from an extrahepatic mechanical obstruction of the bile ducts. Commonly, bile flow is only partially disrupted, giving rise to anicteric cholestasis, or cholestasis without jaundice.

In this investigation, the hepatic immunohistochemical expression of B7-1 and B7-2 has been studied, with emphasis on intrahepatic biliary epithelia, using wedge biopsies from 22 patients with primary biliary cirrhosis (PBC), seven with primary sclerosing cholagitis (PSC), and, as controls, eight cases of extrahepatic biliary obstruction, eight of chronic vital hepatitis C, and three histologically normal livers .

As previously mentioned, biliary stones are responsible for most cases of biliary obstruction; other less frequent causes can produce either an extrahepatic obstruction, as in acquired immunodeficiency syndrome (AIDS) cholangiopathy or cholangitis as a result of stricture of a biliary enteric anastomosis, or a mixture of hepatic and extrahepatic obstruction, as in the case of primary sclerosing cholangitis (PSC) and immunoglobulin G. Cholangiographic studies do not reveal pathognomonic findings; they show either a picture of intrahepatic bile duct involvement similar to PSC and/or extrahepatic biliary strictures similar to those found with cholangiocarcinoma and pancreatic cancer. As it is difficult to differentiate IgG4-associated cholangitis from PSC, many studies have investigated the clinical and laboratory findings that correlate with the diagnosis of IgG4.

On the other hand, obstruction of bile flow results in alterations of coagulation, the immune system, and all organ function. This chapter will discuss the physiology of bile secretion, the pathophysiology of bile obstruction, and the management of obstructive jaundice. Humans with an MDR3 deficiency develop progressive familial intrahepatic cholestasis type 3 (Kullak-Ublick, 2004). These patients have no phosphatidylcholine in bile and therefore do not form mixed micelles with bile salts. As a result, toxic bile salts injure the biliary epithelium, resulting in neonatal cholestasis, cholestasis of pregnancy, and cirrhosis in adults. During its passage through the bile ductules, canalicular bile is modified by the absorption and secretion of electrolytes and water. Bicarbonate secretion by the bile ducts plays an important role in bile salt–independent bile flow.

Biliary Obstruction Mechanical blockage of y Large intrahepatic bile duct y Extra hepatic duct Can lead to bacterial cholangitis, sepsis Causes. Primary Biliary Cirrhosis (Rare autoimmune disease) Destruction of bile ductules (within triads of liver) Cholestasis Acute Hepatocyte injury Degeneration Inflammatory cell infiltration (acute inflammation) Repair Restoration of normal architecture Chronic Foamy changes of hepatocytes Focal destruction of parenchyma Unrelieved obstruction y Portal tract fibrosis y End stage cirrhosis.