Gene/Protein Disease Symptom Drug Enzyme Compound
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Query: UMLS:C0023890 (cirrhosis)
 42,195 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

beta-glucoronidase (EC 3.2.1.31) is a lysosomal enzyme catylysing the decomposition of beta-D-glucoronides--compounds arising as a result of the combination of beta-D-glucoronic acid and a number of compounds both exo- and endogenous, containing hydroxylic, carboxylic, amine, imine or thiol groups. The most common test evaluating the activity of the enzyme is that using phenolphtalein glucoronide as a biosynthetic substrate. The freed aglycons are colorimetrically assayed. The activity of beta-glucoronidase increases in many pathological conditions: liver infammations, cirrhosis of the liver, inflammations of other organs, cholestatic jaundice, tuberculosis, sarcoidosis and also in neoplasms. Many authors point to beta-glucoronidase as a sensitive indicator signalling cell damage.
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PMID:Beta-glucuronidase in physiology and disease. 1532 23

The presence of granulomas in the liver raises consideration of a wide differential diagnosis, but in most Western series, sarcoidosis accounts for a majority of cases. This review will focus specifically on the diagnosis of and therapy for hepatic sarcoidosis. Sarcoidosis is a systemic granulomatous disease of unknown etiology. Hepatic involvement of sarcoidosis was described in 11.5% of 736 patients enrolled in the ACCESS study. However, presence alone of granulomas in an organ in sarcoidosis does not dictate treatment. The decision to treat should be based on symptoms and severity of disease. Although hepatic involvement usually is asymptomatic, a minority of patients progress to chronic cholestatic disease, portal hypertension, and cirrhosis that may require liver transplantation. Treatment of hepatic sarcoidosis should be reserved for patients who manifest this spectrum of disease. Glucocorticoid treatment is first-line therapy for hepatic sarcoidosis, improving symptoms and abnormal laboratory values but generally having no effect on progression of disease. In addition to glucocorticoids, immunomodulators such as azathioprine, methotrexate, hydroxychloroquine, and infliximab have been used with some positive effects on symptoms, liver enzyme abnormalities, and hepatomegaly, but none has been shown to prevent progression of disease. Ultimately, in cases of overt liver failure, liver transplantation is the definitive treatment. Overall, treatment for hepatic sarcoidosis is targeted toward alleviation of symptoms but has no curative potential at this time. Focus should be on discovering the etiology of the disease to target therapy at prevention, not cure.
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PMID:Diagnosis and treatment of hepatic sarcoidosis. 1708 81

In this article, we present the CT and MR imaging characteristics of the cirrhotic liver. We describe the altered liver morphology in different forms of viral, alcoholic and autoimmune end-stage liver disease. We present the spectrum of imaging findings in portal hypertension, such as splenomegaly, ascites and varices. We describe the patchy and lacelike patterns of fibrosis, along with the focal confluent form. The process of hepatocarcinogenesis is detailed, from regenerative to dysplastic nodules to overt hepatocellular carcinoma. Different types of non-neoplastic focal liver lesions occurring in the cirrhotic liver are discussed, including arterially enhancing nodules, hemangiomas and peribiliary cysts. We show different conditions causing liver morphology changes that can mimic cirrhosis, such as congenital hepatic fibrosis, "pseudo-cirrhosis" due to breast metastases treated with chemotherapy, Budd-Chiari syndrome, sarcoidosis and cavernous transformation of the portal vein.
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PMID:Cirrhosis: CT and MR imaging evaluation. 1714 54

Sarcoidosis is a multisystem disease of unknown aetiology. Histological evidence of non-caseating granulomas represents the main finding. It affects mostly young people, targeting primary the lung and hilar lymph nodes although liver involvement is often encountered. Hepatic sarcoidosis covers a broad spectrum from asymptomatic hepatic granulomas formation and slightly deranged liver function tests to clinically evident disease with cholestasis or, in advanced cases, cirrhosis and portal hypertension. Other granulomatous diseases (mainly systemic infections like tuberculosis) should be excluded prior to treatment, as longstanding corticosteroid administration is the main stem of therapy. In advanced cases, liver transplantation represents the ultimate therapeutic option.
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PMID:Hepatic sarcoidosis. 1715 76

Although autopsy series report liver granulomas in up to 70% of patients, computed tomography detection of hepatic and splenic lesions is described in 5% and 15% of sarcoidosis cases, respectively. A rather rare case of liver sarcoidosis mimicking macronodular cirrhosis is presented by this current article. Imaging findings in our patient were in compliance with the diagnosis of liver cirrhosis, although liver biopsy findings eventually revealed sarcoid granulomas located in the portal and lobular areas without fibrotic lesions. Histological and imaging modalities in liver sarcoidosis are discussed.
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PMID:A case of liver sarcoidosis mimicking cirrhosis. 1718 48

There is dispute about the cause of Beethoven's death; alcoholic cirrhosis, syphilis, infectious hepatitis, lead poisoning, sarcoidosis and Whipple's disease have all been proposed. In this article all primary source documents related to Beethoven's terminal illness and death are reviewed. The documents include his letters, the report of his physician Andreas Wawruch, his Conversation Books, the autopsy report, and a new toxicological report of his hair. His terminal illness was characterised by jaundice, ascites, ankle oedema and abdominal pain. The autopsy data indicate that Beethoven had cirrhosis of the liver, and probably also renal papillary necrosis, pancreatitis and possibly diabetes mellitus. His lifestyle for at least the final decade of his life indicated that he overindulged in alcohol in the form of wine. Alcohol was by far the most common cause of cirrhosis at that period. Toxicological analysis of his hair showed that the level of lead was elevated. During the eighteenth and early nineteenth centuries, lead was added illegally to inexpensive wines to sweeten and refresh them. These findings strongly suggest that liver failure secondary to alcoholic cirrhosis, associated with terminal spontaneous bacterial peritonitis, was the cause of death. This was complicated in the end stages by renal failure. If the presence of endogenous lead was verified by analysis of Beethoven's skeletal remains, it would suggest that the lead was derived from wine that he drank. Lead poisoning may account for some of his end-of-life symptoms. There is little clinical or autopsy evidence that Beethoven suffered from syphilis.
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PMID:Beethoven's terminal illness and death. 1721 30

Primary biliary cirrhosis (PBC) and systemic sarcoidosis are granulomatous diseases of unknown etiology whose hepatic manifestations may infrequently be imitative of one another. Described herein is the first reported case in the medical literature of systemic sarcoidosis developing after liver transplantation for PBC. The presented patient, who suffered from typical clinical, laboratory, and pathologic manifestations of PBC, developed decompensated liver cirrhosis within a course of 8 years, necessitating orthotopic liver transplantation. A year and a half after transplantation, the patient developed diffuse, biopsy-proven, dermatologic and pulmonary manifestations of systemic sarcoidosis, which promptly responded to corticosteroid treatment. In retrospect, the patient's longstanding liver disease was probably caused by an unrecognizable, isolated hepatic form of sarcoidosis or an overlap between PBC and sarcoidosis. This patient illustrates the complexity that may be rarely encountered in differentiating between PBC and hepatic sarcoidosis. Discussed are the clinical, laboratory, and pathologic overlaps between hepatic sarcoidosis and PBC, and clues that may aid in the diagnosis and differentiation between the 2 disorders. Hepatologists and liver transplantation specialists should be aware of the rare possibility of hepatic sarcoidosis imitating PBC, and exacerbating systemically after liver transplantation.
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PMID:Late-onset sarcoidosis after liver transplantation for primary biliary cirrhosis. 1742 76

Sarcoidosis is a multisystem disorder commonly affecting the lungs, but also the liver, with cirrhosis and portal hypertension occurring in fewer than 1% of cases. Although hepatopulmonary syndrome (HPS) is seen in 15% to 20% of patients with cirrhosis of varying causes, it has rarely been associated with sarcoidosis. Also, although a brain abscess is not uncommon in patients with discrete pulmonary arteriovenous malformations, it is rarely seen in patients with the much smaller intrapulmonary vascular dilations that characterize HPS. A patient with an unusual series of uncommon sarcoidosis complications, including cirrhosis with HPS, brain abscess and finally Nocardia meningitis, is reported. The possibility of HPS should be considered in sarcoidosis patients with liver involvement, if gas-exchange abnormalities are out of proportion to the degree of lung involvement. These patients may also be susceptible to a cerebral abscess by paradoxical embolization, and to opportunistic infections due to cirrhosis.
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PMID:Sarcoidosis complicated by cirrhosis and hepatopulmonary syndrome. 1843 52

Here we report a case of a 25-year-old woman with mesenteric and hepatic sarcoidosis without lung involvement complicated by severe noncirrhotic portal hypertension. In 1992, at the clinical presentation, she had abdominal pain, asthenia, and weight loss. Splenomegaly, signs of flogosis, sideropenic anemia, and cholestasis were observed. Laparoscopic abdominal exploration and histological analysis demonstrated noncaseating granulomas of the liver, abdominal lymph nodes, and mesenteric connective tissue. The clinical course was severe with episodic remissions and recrudescences characterized by ascites (mild or moderate), elevation of bilirubin levels (mean: 1.1 mg/dl; range: 0.9-3.5 mg/dl), reduction of albumin levels (mean: 4 g/dl; range: 3.4-4.2 g/dl), and prolongation of elevated international normalized ratio (mean: 1; range: 0.9-1.4). In 1997, the patient had variceal bleeding. Complete hemostasis was obtained with band ligation. Liver function was preserved, and until 2000 the disease remained stable. In 2001, the patient became pregnant. At the 36th week of gestation, the patient delivered a healthy female infant and afterwards remained in clinical remission. This report stresses that sarcoidosis can have a hepatic and mesenteric involvement in absence of thoracic lymphadenopathy. Portal hypertension may be severe, and in absence of cirrhosis it may be associated with portal thrombosis. Finally, portal hypertension in patients with hepatic sarcoidosis and preserved liver function should not be considered as an absolute contraindication to pregnancy.
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PMID:Hepatic and mesenteric sarcoidosis without thoracic involvement: a case of severe noncirrhotic portal hypertension and successful pregnancy. 1878 74

Intrahepatic portal venopathy leads to various entities that are important causes of portal hypertension. Noncirrhotic portal fibrosis (NCPF) occurs in the Indian subcontinent, whereas idiopathic portal hypertension (IPH) occurs in Japan although the pathogenesis and presentation of both are similar. NCPF presents mainly with upper gastrointestinal bleeding; IPH presents with massive splenomegaly. The liver functions are preserved. Wedged hepatic venous pressure is normal, but portal venous pressure is high indicating a presinusoidal block. Patients are best managed with endoscopic therapy or surgery, with better results than in patients with cirrhosis. Nodular regenerative hyperplasia is a histological diagnosis characterized by development of nodules in the liver due to uneven perfusion of the portal venous blood. These patients may develop portal hypertension and if they bleed would require treatment as in NCPF/IPH. Schistosomiasis produces portal hypertension by the development of fibrous tissue around the portal veins as a response to schistosome eggs. Gratifying results have been reported with praziquantel therapy. Rarely sarcoidosis and chronic biliary obstruction may also produce portal venopathy.
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PMID:Intrahepatic portal venopathy and related disorders of the liver. 1881 80


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