Gene/Protein Disease Symptom Drug Enzyme Compound
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Query: UMLS:C0015672 (fatigue)
 51,768 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

"Overlap syndromes" have been reported among various autoimmune liver diseases, particularly between primary biliary cirrhosis and autoimmune hepatitis (AIH) in adults and between AIH and autoimmune cholangitis in children. The overlap syndrome of AIH and primary sclerosing cholangitis (PSC), however, has been scarcely reported. Furthermore, in most of the reported cases of AIH/PSC overlap syndrome, PSC and AIH were believed to occur simultaneously. We report a case of a 34-year-old woman who has ulcerative colitis and PSC (diagnosed by colonoscopy, histology, and cholangiogram) and 7 years later develops rapidly progressive liver failure and hemolytic anemia from AIH. Liver biopsy showed dense portal lymphoplasmacytic infiltrate with interface hepatitis and acidophil bodies confirming AIH. She responded well to immunosuppressive therapy with steroids, both with respect to her liver disease and her autoimmune hemolytic anemia. Additionally, her clinical symptoms of fatigue, jaundice, and pruritus improved markedly and quickly. Overlap or "crossover" syndrome should be considered in all patients with PSC when they present with sudden deterioration of the liver function and changes in liver enzymes. By making the diagnosis of AIH in a patient with well-established PSC, appropriate treatment can be initiated, resulting in the patient's prompt recovery.
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PMID:Rapid progression of autoimmune hepatitis in the background of primary sclerosing cholangitis. 1549 11

Primary sclerosing cholangitis (PSC) happens mainly in middle-aged men and is seldom diagnosed in children. Childhood PSC undergoing liver transplantation is rarely reported. Here we present a 12-year-old girl who was admitted with a 6-day history of fever, abdominal pain, fatigue, jaundice, and splenomegaly. Liver histological examination revealed the destroyed bile ducts and bridging fibrosis. Endoscopic retrograde cholangiopancreatography (ERCP) showed beaded appearance of right intrahepatic ducts and absent left intrahepatic ducts. PSC was diagnosed. Because of decompensated liver function, she received a living-related orthotopic liver transplantation (OLT). The post-transplantation course was uneventful during the 12-month follow-up. She experienced neither additional episode of cholangitis nor recurrence of liver cirrhosis after OLT.
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PMID:A child with primary sclerosing cholangitis successfully treated by liver transplantation. 1562 73

We report a patient with benign bile duct stricture causing difficulty in differential diagnosis from bile duct carcinoma. A 66-year-old woman consulted a local physician because of general fatigue. Blood biochemical tests showed increased levels of biliary tract enzymes. Abdominal ultrasonography (US) revealed tapering and blockage of the midportion of the bile duct and dilation of the intrahepatic bile ducts. Magnetic resonance cholangiopancreatography (MRCP) demonstrated obstruction of the midportion of the bile duct. Later, because a marked increase in biliary tract enzymes and jaundice appeared, percutaneous transhepatic biliary drainage (PTBD) was performed. Post-PTBD cytological examination of bile was negative for cancer. A third biopsy showed slight hyperplasia with no malignant findings. Recholangiography, performed through PTBD, suggested gradual improvement of bile duct stricture, but could not completely exclude the possibility of malignancy; thus, resection of the bile duct including the stricture site was performed, and the resected specimen was submitted for intraoperative frozen section examination. Histopathological diagnosis did not reveal malignant findings. After cholecystectomy and bile duct resection, hepaticojejunostomy (Roux-en-Y) was performed. Because only erosion and desquamation of the mucosal epithelium and mild submucosal inflammatory cell infiltration and fibrosis were observed, chronic cholangitis was diagnosed histopathologically. Surgical resection of the bile duct should be considered for potentially malignant stricture of the bile duct.
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PMID:A case of benign bile duct stricture causing difficulty in differential diagnosis from bile duct carcinoma. 1575 49

The most common biliary problem in patients with inflammatory bowel disease is primary sclerosing cholangitis (PSC). The treatment of this disease is multifaceted and frequently requires a multidisciplinary approach involving internists, nutritionists, gastroenterologists, and surgeons. Unfortunately, other than liver transplantation, no therapy that is currently available has been proven to alter the natural history of PSC or prolong survival. Ursodeoxycholic acid is currently the most promising pharmacologic treatment option for slowing disease progression and should be used in higher than usual doses (20 to 30 mg/kg/d). Treatment of symptoms due to cholestasis, such as pruritis and steatorrhea, is an important aspect of the medical care of patients with PSC. Our preferred treatment of pruritis due to cholestasis is with bile acid binding exchange resins such as cholestyramine or colestipol (which is generally better tolerated than cholestyramine). Endoscopic therapy should be reserved for patients with obstructive jaundice, cholangitis, or symptomatic dominant biliary strictures. We recommend dilation of dominant strictures with graduated or balloon dilators followed by temporary stenting if the postdilation cholangiographic appearance is not improved or adequate biliary drainage cannot be assured. There is indirect evidence that the combination of ursodeoxycholic acid and endoscopic therapy to maintain biliary patency may improve transplant-free survival in patients with PSC, although this remains to be proven. Liver transplantation remains the only effective treatment of advanced PSC, and should be considered in patients with complications of cirrhosis or intractable pruritis or fatigue.
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PMID:Treatment of Biliary Problems in Inflammatory Bowel Disease. 1576 33

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

Autoimmune cholangitis is a rare chronic cholestatic liver disease. Fever of unknown origin is defined as a temperature higher than 38.3 degrees C that lasts for more than 3 weeks with no obvious source despite appropriate investigation. We describe the case of a 62-year-old woman who presented with fever, fatigue and weight loss. The serum biochemical study showed an increase in alkaline phosphatase and gamma-glutamyl transpeptidase levels. Antinuclear, antimitochondrial, anti-smooth-muscle antibodies and antibodies against the cytoplasm of neutrophils were negative. Liver biopsy was compatible with autoimmune cholangitis. The patient was successfully treated with methylprednisolone and ursodeoxycholic acid. We describe here a rare case of fever as preceding and leading symptom of autoimmune cholangitis.
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PMID:Autoimmune cholangitis presented as fever of unknown origin. 1700 78

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

Primary sclerosing cholangitis (PSC) is a chronic cholestatic liver disease characterized by inflammation and fibrosis of the bile ducts, resulting in end-stage liver disease and reduced life expectancy. PSC primarily affects young and middle-aged men, often in association with underlying inflammatory bowel disease. The etiology of PSC includes immune-mediated components and elements of undefined nature. A cholestatic picture of liver biochemistries with elevations in serum alkaline phosphatase, nonspecific autoantibodies such as perinuclear antineutrophilic antibody, antinuclear antibodies and smooth muscle antibodies, and diffuse multifocal biliary strictures, resulting in a 'beaded' appearance on radiographic studies, are the hallmarks of the disease. No effective medical therapy is currently available, although clinical studies are in progress. Ursodeoxycholic acid at high doses (28 mg/kg/day to 30 mg/kg/day) is the most promising agent but is unproven so far. Liver transplantation is currently the only life-extending therapy for patients with end-stage disease, although recurrent disease can be observed in the transplanted liver. The multiple complications of PSC include pruritus, fatigue, vitamin deficiencies, metabolic bone disease, peristomal varices, bacterial cholangitis, dominant biliary strictures, gallbladder stones and polyps, and malignancy, particularly cholangiocarcinoma, which is the most lethal complication of PSC.
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PMID:Primary sclerosing cholangitis. 1870 47

Cholangiocarcinomas (CCAs) are rare malignancies that arise from the biliary epithelium. Intrahepatic CCAs usually present as mass lesions that are asymptomatic or cause nonspecific systemic symptoms such as fatigue, fever, and weight loss. Hilar and extrahepatic tumors most commonly present with jaundice, though cholangitis also can be seen. Tumor markers such as carbohydrate antigen 19-9 and carcinoembryonic antigen have been used to diagnose CCA, but these are nonspecific and may be elevated in infection, inflammation, or any obstruction. Endoscopic retrograde cholangiopancreatography (ERCP) has been used for the diagnosis and management of CCA for many years. This article summarizes the data regarding the application of ERCP in the diagnosis and management of CCA.
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PMID:Endoscopic retrograde cholangiopancreatography in the diagnosis and management of cholangiocarcinoma. 2068 39


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