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

Obliteration of the terminal hepatic venules with perivenular fibrosis (phlebosclerosis) is a well recognised feature in alcoholic liver disease. Veno-occlusive lesions with intimal obliteration of hepatic veins and a lymphocytic phlebitis of hepatic veins may also be present. We looked for these lesions in 256 liver biopsies and 50 livers obtained at necropsy from patients with alcoholic liver disease. Phlebosclerosis was a universal finding in alcoholic hepatitis and cirrhosis and showed increasing severity with progressive liver injury. Veno-occlusive lesions, however, were found in only 25 of 256 (9.8%) of biopsies and 11 of 50 (22%) of livers obtained at necropsy, showing alcoholic hepatitis or cirrhosis: lymphocytic phlebitis was found in 10 of 256 (3.9%) and two of 50 (4%), respectively. Moreover, veno-occlusive lesions were generally mild. The prevalence of veno-occlusive lesions and lymphocytic phlebitis was considerably less than has been previously documented. Phlebosclerosis may have a different mechanism and be a more important contributory factor in progressive liver injury.
 ...
PMID:Hepatic vein lesions in alcoholic liver disease: retrospective biopsy and necropsy study. 395 32

The nature and significance of vascular lesions in alcoholic liver disease were studied in 200 autopsies. Three principal types of lesions were recognized: (a) Lymphocytic phlebitis, consisting of a chronic inflammatory cell infiltrate of the wall of terminal hepatic venules (central veins) or intercalated (sublobular) veins, was noted in 16.7% of patients with precirrhotic alcoholic hepatitis and 4.3% of patients with cirrhosis. (b) Phlebosclerosis, consisting of perivenular scarring with gradual obliteration of the lumen of terminal hepatic venules and sometimes intercalated veins was found to some degree in all patients with alcoholic hepatitis or cirrhosis. (c) Veno-occlusive lesions, consisting of intimal proliferation, fibrosis, and narrowing of the lumen of terminal hepatic venules, intercalated veins, and occasionally portal veins were found in 52.1% of cases of precirrhotic alcoholic hepatitis with total occlusion of some terminal hepatic venules or intercalated veins, or both, in 14.6%. In alcoholic cirrhosis, veno-occlusive lesions were present to some degree in 74.1% with totally occluded vessels found in 46.8%. Evidence of portal hypertension was present in 47.9% of patients with precirrhotic alcoholic hepatitis and was significantly associated with the degree of both veno-occlusive change and phlebosclerosis, which tend to occur together. It is concluded that both veno-occlusive lesions and phlebosclerosis contribute to the development of portal hypertension in alcoholic liver disease. Veno-occlusive lesions in the cirrhotic liver may contribute to atrophy, with loss of functioning parenchyma. The etiopathogenesis of the vascular lesions in alcoholic liver disease requires further investigation.
 ...
PMID:Occlusive venous lesions in alcoholic liver disease. A study of 200 cases. 710 9

Sarcoidosis often involves the liver with mild elevation of serum enzymes and granulomas histologically. Rarely, chronic cholestasis, portal hypertension, cirrhosis, or nodular hyperplasia may be found. The pathogenesis of the portal hypertension and of the cirrhosis are not understood, in part because large samples of tissue have seldom been described. We describe the clinical and anatomic findings of four patients with sarcoid liver disease in whom the whole livers were available for examination. One patient had cirrhosis, one had diffuse nodular hyperplasia, and two had small regions of parenchymal fibrosis. The first two of these had a history of variceal bleeding and healed portal vein thrombosis. One had chronic cholestasis without cirrhosis. We suggest that the cirrhosis and focal fibrosis were caused by ischemia secondary to primary granulomatous phlebitis of portal and hepatic veins. The portal hypertension in two patients was likely secondary to portal vein thrombosis, because cirrhosis was absent at the onset of variceal bleeding.
 ...
PMID:The role of granulomatous phlebitis and thrombosis in the pathogenesis of cirrhosis and portal hypertension in sarcoidosis. 930 82

Porphyrias are a heterogenous group of diseases that may result in disabling or life threatening neurovisceral symptoms and/or cutaneous photosensitivity. In acute intermittent porphyria, the clinical features, particularly neurological symptoms, may be life-threatening and disabling. Conventional treatment with human hemin, though effective in reducing symptoms, does not reverse neuropathy when structural nerve damage has occurred and may cause intense phlebitis. Liver transplantation (LT) may be considered as treatment for those with repeated life-threatening acute attacks resulting in poor quality of life, requirement of ventilatory support, and progressive loss of venous access due to hemin infusion. Patients with variegate porphyria (VP) present after puberty with neurovisceral symptoms and skin manifestations. LT resolved VP in the 1 patient reported in the literature. Aminolaevulinic acid dehydratase deficient porphyria is a rare autosomal recessive disorder and a child who presented with failure to thrive and required transfusions and parenteral nutrition did not improve with LT. In erythropoietic protoporphyria (EPP), there is excessive production of protoporphyrin in the bone marrow. Protoporphyrin is hepatotoxic and pigment loading of hepatocytes and bile canalicular sludging may result in progressive cholestasis and cirrhosis. LT is beneficial for such patients with end-stage liver disease. Perioperative management includes use of filters on operative lights to prevent skin burns and intestinal perforation. Other concerns include development of neuropathy, biliary complications, and recurrent liver disease. This review addresses the rationale, patient selection, evaluation, management issues, and technique of performing LT in various types of porphyria.
 ...
PMID:Liver transplantation for porphyria: who, when, and how? 1776 98

Sclerosing cholangitis is a heterogenous disease. Sclerosing cholangitis with an unknown cause is abbreviated PSC. PSC affects extra- as well as intra-hepatic bile ducts and since this is a permanently progressing fibrous condition, it leads to liver cirrhosis. The disease is often associated with a development of cholangocarcinoma and idiopathic intestinal inflammation. Causal therapy does not exist; liver transplantation is indicated. IgG4 cholangitis differs from PSC in a number of features. This form is, unlike PSC, linked to autoimmune pancreatitis (AIP) as well as other IgG4 sclerosing diseases. Anatomically, distal region of ductus choledochus is most frequently involved. Icterus is, unlike in PSC, a frequent symptom of AIP. There also is a distinctive histological picture--significant lymphoplasmatic infiltration of the bile duct wall with abundance of IgG4 has been described, lymphoplasmatic infiltration with fibrosis in the periportal area and the presence of obliterating phlebitis is also typical. However, intact biliary epithelium is a typical feature. IgG4 can be diagnosed even without concurrent presence of AIP. IgG4 sclerosing cholangitis is a condition sensitive to steroid therapy. At present, there is no doubt that IgG4 sclerosing cholangitis is a completely different condition to primary sclerosing cholangitis. From the clinical perspective, these diseases should be differentiated in every clinician's mind as (a) AIP is treated with corticosteroids and not with an unnecessary surgery, (b) IgG4 sclerosing cholangitis is mostly successfully treated with corticosteroids and the disease is not, unlike PSC, a risk factor for the development of cholangiocarcinoma.
 ...
PMID:[Autoimmune pancreatitis and IgG-positive sclerosing cholangitis]. 2149 6

IgG4-related sclerosing cholangitis as part of IgG4 systemic-related diseases is commonly associated with autoimmune pancreatitis. Major clinical manifestations of IgG4-related sclerosing diseases are apparent in the organs in which tissue fibrosis with obstructive phlebitis is pathologically induced. IgG4-related sclerosing cholangitis is included within the heterogeneous group of 'sclerosing cholangitis'. Sclerosing cholangitis may be associated with choledocholithiasis, infection or biliary malignancies. Sclerosing cholangitis of unknown etiology is called primary sclerosing cholangitis (PSC). Conservative therapy of PSC is usually unsuccessful, the disease involves extra- and/or intrahepatic biliary tree, and the end point of this disease is liver cirrhosis. Typically, PSC is identified at the age of 30 to 40 years, and the disease is frequently associated with inflammatory bowel diseases. On the other hand, IgG4-related sclerosing cholangitis is not associated with inflammatory bowel diseases. In patients with IgG4-related sclerosing cholangitis, a first symptom can be obstructive jaundice, whereas obstructive jaundice is rarely present in PSC. Clinically, patients with IgG4-related sclerosing cholangitis are older at diagnosis compared to patients with PSC. A typical diagnostic feature of IgG4-related sclerosing cholangitis is elevation of serum immunoglobulin G4. In patients with IgG4-related sclerosing cholangitis, response to steroid therapy is high; in patients with PSC corticosteroid therapy is unsuccessful. Histochemically abundant infiltration of IgG4-positive plasma cells is detected in the biliary duct wall. Histologically, we can identify dense lymphoplasmacytic infiltration of the bile duct wall, transmural fibrosis, lymphoplasmacytic infiltration and fibrosis in the periportal area of the liver - a typically obliterative phlebitis. The biliary epithelium is usually intact in contrast to PSC, where mucosal erosion is often present. Steroids are the first-choice therapy of IgG4-related sclerosing cholangitis. In the literature, cholangiocarcinoma in patients with IgG4- related sclerosing cholangitis was not described, whereas cholangiocarcinoma develops in up to 10-30% of patients with PSC.
 ...
PMID:Immunoglobulin G4-related cholangitis: a variant of IgG4-related systemic disease. 2272 42