Gene/Protein Disease Symptom Drug Enzyme Compound
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Query: UMLS:C0033774 (pruritus)
 14,546 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

Primary sclerosing cholangitis (PSC) is a chronic cholestatic syndrome of unknown etiology frequently associated with inflammatory bowel disease and characterized by diffuse inflammation and fibrosis of the intra and/or extrahepatic bile ducts. Recent studies seem to favor autoimmunity in the context of a genetic predisposition as the most likely underlying mechanism for the development of the disease, however our knowledge on the pathogenesis of PSC is still incomplete and further work is needed. The most common manifestations are fatigue, pruritus, jaundice and abdominal pain; however, the increasing use of invasive cholangiography has led to diagnosing this condition in a high proportion of asymptomatic patients. PSC usually follows a progressive course leading to biliary cirrhosis with complications of portal hypertension and hepatic failure. Patients with PSC also may develop a number of other complications, including bacterial cholangitis, dominant biliary strictures, conditions of chronic cholestasis, colorectal cancer and cholangiocarcinoma. Currently, no medical therapy aimed at disrupting disease progression is available, although high-dose ursodeoxycholic acid and other medicines are being evaluated in clinical trials. A better understanding of the pathogenesis of the disease will serve as a guide for evaluating new medical approaches. Liver transplantation is the only therapeutic alternative that improves survival in patients with end-stage PSC. Prognostic models are useful in determining the timing of liver transplantation.
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PMID:Primary sclerosing cholangitis. 1648 1

A 66-year-old white male presented with jaundice, pruritus, and a 30-pound weight loss over two months. Physical examination revealed scleral icterus. Laboratory evaluation revealed ALT 161 U/L, AST 290 U/L, alkaline phosphatase 2004 U/L, GGT 2,552 U/L, total bilirubin 10.2 mg/dL, and a carbohydrate antigen 19-9 (CA 19-9) level of 4,374 U/mL. Initial endoscopic retrograde cholangiopancreatography (ERCP) was unsuccessful due to ulceration in the duodenum healed with esomeprazole therapy. Subsequent ERCP showed a possible filling defect in the common bile duct treated with sphincterotomy and balloon sweeping of the common bile duct. Symptoms and jaundice resolved five months after initial presentation with normal labs and studies. While elevated CA 19-9 levels occur in most patients with carcinoma of the pancreas, it can also be elevated in patients with extrapancreatic malignancies and acute cholangitis. This case illustrates the fact that a markedly elevated CA 19-9 can be secondary to causes other than carcinoma.
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PMID:Patient with markedly elevated CA 19-9 not associated with malignancy. 1655 91

A diagnosis of liver metastasis, periportal adenopathy, or hepatobiliary cancer often is accompanied by findings of biliary obstruction. Malignant biliary obstruction frequently is associated with pruritus, anorexia, cholangitis, or hyperbilirubinemia, which that precludes treatment with chemotherapeutic agents that are excreted or metabolized hepatically. In patients with low biliary obstruction, endoscopic stent placement may accomplish drainage of the entire biliary tree without the need for an external device. Patients with high bile duct obstruction, on the other hand, may need a percutaneous approach to drain the target ducts and avoid draining an atrophic segment or lobe. This first of a series of two articles concerning palliative percutaneous biliary intervention will review the indications for biliary drainage and the preprocedure evaluation of this complicated patient population.
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PMID:Palliative percutaneous drainage in malignant biliary obstruction. Part 1: indications and preprocedure evaluation. 1680 29

A 46-year-old woman presented with chronic fluctuated liver function impairment, Raynaud's phenomenon, digital gangrene, pulmonary hypertension, and intense pruritus within a period of 2 years. Laboratory investigations revealed antinuclear antibodies, anticentromere antibodies (ACA), hypergammaglobulinemia, lymphocytic infiltration of the liver parenchyma, and mild cholangitis. The associated symptoms included thyroiditis, conjunctivitis sicca, xerostomia, and polyarthralgia. There was no conspicuous sclerodactyly, calcinosis, or dysphagia. The symptoms were relieved with intravenous, as well as oral, methylprednisolone. This constellation of presentations, including chronic autoimmune hepatitis with mild cholangitis and pulmonary hypertension, suggested that the presence of serum ACA might indicate relentless visceral organ damage.
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PMID:Hepatitis, cholangitis, pulmonary hypertension, digital gangrene, and conjunctivitis sicca in a woman with anticentromere antibodies. 1686 13

Malignant biliary obstruction frequently is associated with pruritus, anorexia, cholangitis, or hyperbilirubinemia; this difficult complication precludes the use of antineoplastic agents that are excreted or metabolized via the liver. In patients with low biliary obstruction, endoscopic stent placement may accomplish drainage of the entire biliary tree without the need for an external device. However, patients with high bile duct obstruction most often require a percutaneous approach to drain the target ducts to maximize drainage and to avoid draining an atrophic segment or lobe. In the first in this series of two articles, the indications for biliary drainage and preprocedure evaluation in malignant biliary obstruction were discussed. This second article describes the mechanisms of percutaneous biliary drainage and postprocedure management, including consideration of possible complications.
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PMID:Palliative percutaneous drainage in malignant biliary obstruction. Part 2: Mechanisms and postprocedure management. 1689 95

Primary sclerosing cholangitis (PSC) is a chronic cholestatic liver disease of unknown aetiology characterised by inflammation and fibrosis of the biliary tree. The mean age at diagnosis is 40 years and men are affected twice as often as women. There is a reported annual incidence of PSC of 0.9-1.31/100,000 and point prevalence of 8.5-13.6/100,000. The onset of PSC is usually insidious and many patients are asymptomatic at diagnosis or have mild symptoms only such as fatigue, abdominal discomfort and pruritus In late stages, splenomegaly and jaundice may be a feature. In most, the disease progresses to cirrhosis and liver failure. Cholangiocarcinoma develops in 8-30% of patients. PSC is thought to be immune mediated and is often associated with inflammatory bowel disease, especially ulcerative colitis. The disease is diagnosed on typical cholangiographic and histological findings and after exclusion of secondary sclerosing cholangitis. Median survival has been estimated to be 12 years from diagnosis in symptomatic patients. Patients who are asymptomatic at diagnosis, the majority of whom will develop progressive disease, have a survival rate greater than 70% at 16 years after diagnosis. Liver transplantation remains the only effective therapeutic option for patients with end-stage liver disease from PSC, although high dose ursodeoxycholic acid may have a beneficial effect.
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PMID:Primary sclerosing cholangitis. 1706 36

Chronic biliary tract disease is the third most common indication for orthotopic liver transplantation (OLT) in the United States. Most patients undergoing OLT for chronic biliary tract disease have end-stage liver disease associated with cirrhosis, but a minority are transplanted in the precirrhotic stage for indications that can include poor quality of life (eg, intractable pruritus or fatigue), recurrent ascending cholangitis, or cholangiocarcinoma. A smaller subset of these patients suffer from severe noncirrhotic portal hypertension that can be associated with histologic features of nodular regenerative hyperplasia (NRH) and/or obliterative portal venopathy. We reviewed 306 liver explants performed for chronic biliary tract disease at 2 institutions during 1995 to 2003 to identify patients who were transplanted in the precirrhotic stage. The following clinical data were recorded: age, sex, type of biliary tract disease, radiology, clinical symptoms, signs of portal hypertension, pretransplant shunting procedures, time between diagnosis and OLT, and primary indication for OLT. Histopathologic data included: explant weight, gross appearance, fibrosis stage (1 to 4), cholangitis, bile duct dysplasia, malignancy, portal vein thrombi, presence of NRH, and presence of obliterative portal venopathy. Twenty-six of 306 (8.5%) patients underwent OLT in the precirrhotic stage (12 females: 14 males, mean age of 46 y, age range 12 to 68 y). At explant, fibrosis stage ranged from 1 to 2 (portal and periportal fibrosis) to 3 (multiple bridging fibrosis). Underlying biliary tract disease included primary sclerosing cholangitis (18 cases), primary biliary cirrhosis (5 cases), autoimmune cholangitis (2 cases), and secondary sclerosing cholangitis (1 case). Primary indications for OLT were recurrent cholangitis and/or decreased quality of life (11 cases), complications of portal hypertension (6 cases), portal hypertension plus cholangitis/decreased quality of life (5 cases), and malignancy (4 cases). Of the 11 patients with portal hypertension as a major indication for transplant, 2 had undergone transjugular intrahepatic portal-systemic shunting and 3 others had portal vein thrombi. Histopathologically, NRH was prominent in 8 of these 11 patients (73%) and obliterative portal venopathy in 6 (55%). NRH was also present in 4 of the 15 (27%) patients who were transplanted for other indications. These results indicate that precirrhotic portal hypertension is a predominant or major contributing factor to OLT in a significant minority (11 of 306, 3.3%) of patients with chronic biliary tract disease. The occurrence of NRH in some patients transplanted for other indications suggests it is a histologic pattern that can precede the development of clinically significant portal hypertension.
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PMID:Liver transplantation in precirrhotic biliary tract disease: Portal hypertension is frequently associated with nodular regenerative hyperplasia and obliterative portal venopathy. 1706 88

Aims of treatment for primary sclerosing cholangitis are as follows: prevention of progression of hepatobiliary disease, reduction of symptoms and consequences of cholestasis (pruritus, osteoporosis), and prevention of complications (colorectal cancer, hepatobiliary cancer). Ursodeoxycholic acid (UDCA) improves biliary secretion and laboratory parameters of cholestasis, but its effects on liver histology and survival are not clear. It reduces the incidence of dysplasias and carcinomas of the colon in patients with colitis and possibly has a beneficial effect on the incidence of bile duct carcinomas. At present, UDCA represents the most promising therapeutic option. Immunosuppressive treatment has not been proven to be effective; it appears to be indicated in the overlap syndrome with autoimmune hepatitis but may be harmful in bacterial cholangitis. Bacterial cholangitis is common in patients with dominant stenoses and requires antibiotic treatment. Endoscopic treatment of dominant stenoses improves cholestasis and prolongs survival in comparison to predicted survival. Pruritus represents a problem in some patients, and cholestyramine represents the first-line treatment. If ineffective, opioid antagonists, rifampin, or ondansetron may be tried. For treatment of osteoporosis and osteopenia, calcium and vitamin D supplementation are recommended, and in selected cases, bisphosphonates may be indicated. In patients with severe cholestasis and coagulation defects, parenteral supplementation of vitamin K may be indicated. During treatment, all patients should be regularly screened for colonic and bile duct carcinomas. Patients with cirrhosis of the liver and its complications are treated accordingly, and in end-stage disease, liver transplantation is indicated.
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PMID:Treatment of primary sclerosing cholangitis. 1739 26

At a workshop on primary sclerosing cholangitis (PSC) held during Digestive Disease Week - Japan 2003, 388 PSC cases in Japan were analyzed. Two peaks in the age distribution were also observed in this survey. Jaundice and itching, major symptoms in PSC patients included in the diagnostic criteria, were observed in only 28% and 16%, respectively. Alkaline phosphatase levels were less than twofold of the upper limit of the normal range in 35%. In this regard, the diagnostic criteria in 2003 from the Mayo Clinic, including cholestatic symptoms and two- to threefold increases in serum alkaline phosphatase, should be modified in Japan. Inflammatory bowel diseases were complicated in 37%, and autoimmune pancreatitis (AIP) in 7.2%. PSC cases with inflammatory bowel diseases were younger than the average, creating the firstpeak in age distribution, and have similar characteristics compared to patients with PSC in foreign countries. In addition, even after the exclusion of cases of sclerosing cholangitis complicated with AIP, the second peak in the age distribution was clearly evident. Recently, a concept of immunoglobulin G4-related sclerosing cholangitis has been postulated, which has a similar pathogenesis to AIP but without apparent pancreatic lesions. PSC patients without apparent involvement of the pancreas may be present in older patients and seem to be specific to Japan.
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PMID:Characteristics of primary sclerosing cholangitis in Japan. 1793 Dec 5

Biliary strictures at the liver hilum are caused by a heterogeneous group of benign and malignant conditions. In the absence of a clear-cut benign etiology, i.e. bile duct damage during surgery, hilar biliary strictures remain a diagnostic and therapeutic challenge for which a multidisciplinary approach is often necessary. A definitive diagnosis can be achieved in only 40-60% of the patients, while in all the other cases strictures are treated as though they are malignant until surgical pathology determines otherwise. Surgical resection is the only treatment that prolongs survival in patients with malignant strictures. Because these tumors frequently extend longitudinally via the hepatic ducts into the liver parenchyma, partial hepatic resection has been gradually added to biliary resection to ensure tumor-free surgical margins. For unresectable cases, endoscopic stenting of biliary obstruction is considered the preferred palliation modality to relieve pruritus, cholangitis, pain and jaundice, while the percutaneous approach has been reserved for cases of failure. Other modalities of treatment such as radiotherapy, chemotherapy, and photodynamic therapy currently remain investigational. For benign post surgical hilar strictures, surgical repair can be difficult and requires specific skills and experience. As an alternative, a multi-stent technique with endoscopic placement of an increasing number of stents over time until complete resolution of the stricture has been proposed.
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PMID:Management of hilar biliary strictures. 1802 6


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