Pericolangita
* Introduction
* Signs and symptoms
* Diagnosis
* Treatment
History pericolangita term refers to chronic hepatitis and cholestatic biochemical characteristics typical of patients with inflammatory bowel disease. Nomenclature of this disease was recently questioned because pericolangita and classic sclerosing cholangitis seem to be components of a spectrum of disorders. In particular, studies comparing chronic inflammatory bowel disease, liver biochemistry and records colastatic cholangiography inflammatory bowel disease show similarities between the clinical and liver biopsy.
Moreover some patients with negative initial pericolangita colangiograma elements were demonstrated in long-term monitoring elements typical sclerosing cholangitis. For this reason pericolangita disease formerly called sclerosing cholangitis today refers to the large ducts. There is a close association between inflammatory bowel disease and sclerosing cholangitis of large bile ducts. Approximately 70-80% of patients with sclerosing cholangitis also have inflammatory bowel disease. Most patients have ulcerative colitis, but over 13% of Crohn's disease. It is estimated that more than 2-7. 5% of patients with sclerosing cholangitis have ulcerative colitis.
Patient symptoms are variable depending pericolangita reported data. Diagnoses are often asymptomatic patients with inflammatory bowel disease when performing routine liver biochemical tests reveals a profile colastatic liver. Other patients have jaundice and itching. Jaundice may develop insidiously or suddenly in episodes. Other elements of the colangitei patients have acute, fever, jaundice and abdominal pain.
There is no effective medical treatment pericolangita. We have tried numerous drugs including penicillamine, colchicine, corticosteroids, azathioprine, and pentoxifylline nicotine. They have proved ineffective. Ursodeoxicolic acid may play a therapeutic role in this disease. Liver transplantation is the only treatment proven effective. Pericolangita classic is associated with fibrosis progression to cirrhosis, end-stage liver disease and liver transplantation in a significant number of patients. The average time from diagnosis until death or liver transplant is 12 years. Patients are at increased risk of malignancies, including adenocarconomul, colangiocarcinomul, hepatocellular carcinoma and colorectal dysplasia in inflammatory bowel disease present.
End-stage liver disease complications include: bleeding portal hypertension by hypertensive gastropathy, gastric and esophageal varices, ascites, edema, jaundice, malnutrition, colangiocarcinom, deficiency of fat soluble vitamins, osteopenia and osteoporosis, colony dysplasia and adenocarcinoma in patients with inflammatory bowel disease.
Pathogenesis and causes.
Pericolangita is a chronic cholestatic liver disease of unknown etiology characterized by inflammation and fibrosis of interlobular and septal bile ducts. Although large changes in duct morphology pericolangita may accompany this entity is closer to classic sclerosing cholangitis. Clinical course can be characterized by progressive liver fibrosis and eventually liver transplantation in the absence of complications: cirrhosis, liver failure and death. The rest of the organs and systems remain unaffected by the primary disease process.
Sclerosing cholangitis is associated with classic progression from fibrosis to cirrhosis, liver damage and liver transplantation final necessary in most patients. The average time from diagnosis until death is about 12 years. Patients are at increased risk of cancer, including colangiocarcinomul, hepatocellular carcinoma, colorectal adenocarcinoma and dysplasia in the presence of inflammatory bowel disease.
Pericolangitei etiology is still unknown. Theories proposed include the following: -Autoimmunity, portal bacteremia, colonic absorption of toxins Biliary tree-ischemic injury, viral infections, toxic bile acids in a genetically predisposed individual.
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