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Glossary for Portal hypertension

  • Abdominal swelling: Swelling or bloating of the abdomen
  • Adult Cystic Fibrosis: Cystic fibrosis is an inherited condition characterized by the production of thick sticky mucus by the mucus glands in the lungs, intestines, liver and pancreas. The condition is most often diagnosed in children or young adults but occasionally, relatively mild symptoms may lead to frequent misdiagnosis or no diagnosis at all unless the symptoms become worse. The condition may be misdiagnosed as emphysema, asthma or chronic bronchitis. It is usually females with a mild form of the disease who tend to be diagnosed at a later age.
  • Anemia, Blackfan Diamond: Diamond-Blackfan anemia is a rare genetic condition where the bone marrow is unable to make sufficient red blood cells which leads low levels of red blood cells. There are eight subtypes of the condition which differ in the location of the genetic defect and the incidence of additional symptoms such as malformations. The severity of symptoms is variable but most cases are serious.
  • Ascites: Fluid accumulation in abdominal cavity
  • Berry aneurysm, cirrhosis, pulmonary emphysema, and cerebral calcification: A very rare syndrome characterized primarily by brain aneurysms, liver cirrhosis, pulmonary emphysema and calcification of the brain (cerebrum).
  • Biliary Atresia: A rare condition that can affect newborns where the bile duct that carries the bile from the liver to the small intestine is blocked or missing.
  • Bloody stool: Blood appearing in the stool.
  • Budd-Chiari syndrome: Budd-Chiari syndrome is the clinical picture caused by occlusion of the hepatic veins. It presents with the classical triad of abdominal pain, ascites and hepatomegaly.
  • COACH syndrome: A very rare syndrome characterized by ataxia, gaps or holes in various eye structures, mental retardation, liver fibrosis and brain abnormalities.
  • Cerebellar vermis hypoplasia - oligophrenia - congenital ataxia - coloboma - hepatic fibrosis: A very rare syndrome characterized by ataxia, gaps or holes in various eye structures, mental retardation, liver fibrosis and brain abnormalities.
  • Cholestasis: A condition where the bile flow is impaired or completely halted.
  • Chronic Hepatitis: Chronic hepatitis is defined as inflammatory disease of the liver lasting for more than six months.
  • Chronic liver disease: Any form of chronic liver disease
  • Cirrhosis of liver: Chronic liver disease wherein normal liver parenchyma is replaced by fibrous tissue.
  • Cirrhosis of the liver: Scarring of the liver from alcohol or other causes.
  • Congenital hepatic fibrosis: A rare inherited birth disorder characterized by fibrosis (scarring) of the liver which affects its ability to function. The severity of the condition is variable with some patients being symptomatic during infancy while others may be asymptomatic for most of their life.
  • Dark stool: Darkening of stool
  • Diamond-Blackfan anemia: Diamond-Blackfan anemia is a rare genetic condition where the bone marrow is unable to make sufficient red blood cells which leads low levels of red blood cells. There are eight subtypes of the condition which differ in the location of the genetic defect and the incidence of additional symptoms such as malformations. The severity of symptoms is variable but most cases are serious.
  • Esophageal varices: Abnormally dilated blood vessels in the wall of the esophagus. The condition is asymptomatic unless the blood vessel bursts which can result in death.
  • Gastrointestinal bleeding: Internal bleeding in the digestive tract
  • Gaucher disease type 3: A rare inherited biochemical disorder characterized by the deficiency of the enzyme called glucocerebrosidase and accumulation of glycosylceramide (glucocerebroside). There are three forms of this disease: type 1, 2 and 3. Type 3 is a subacute neurological form which often first appears in childhood.
  • Glycosylphosphatidylinositol deficiency: A genetic disorder where the deficiency of a glycolipid called glycosylphosphatidylinositol causes seizures blood problems.
  • Hepatic Venoocclusive Disease with immunodeficiency: An inherited disorder characterized by the association of immunodeficiency and liver disease involving the blockage of small veins in the liver due to swelling and fibrosis.
  • Hepatic amyloidosis with intrahepatic cholestasis: Amyloidosis is a rare group of metabolic disorders where a protein called amyloid accumulates in body organs and tissues where it can cause damage. In hepatic amyloidosis, the amyloid deposits occur primarily in the liver which affects the flow of bile through the organ and results in intrahepatic cholestasis.
  • Hepatic fibrosis, congenital: A rare condition characterized by scarring or fibrosis of the liver that impairs its function.
  • Hepatorenal Syndrome: A condition the occurs as a result of severe liver disease.
  • Hypercoagulability syndrome, due to glycosylphosphatidylinositol deficiency: A genetic disorder where the deficiency of a glycolipid called glycosylphosphatidylinositol causes seizures and blood coagulation problems.
  • Idiopathic liver cirrhosis: Cirrhosis is defined histologically as a diffuse hepatic process characterized by fibrosis and the conversion of normal liver architecture into structurally abnormal nodules. The progression of liver injury to cirrhosis may occur over weeks to years.
  • Interferon gamma, receptor 1, deficiency: A rare form of primary immunodeficiency which results in a high risk of severe, recurring infections such as mycobacterial infections.
  • Liver conditions: Any condition that affects the liver
  • Liver fibrosis: Liver fibrosis is the excessive accumulation of extracellular matrix proteins including collagen that occurs in most types of chronic liver diseases.
  • Mosse syndrome: A condition involving the association of liver cirrhosis with polycythemia which is a chronic myeloproliferative disorder characterized by the excessive production of mainly red blood cells by the bone marrow.
  • NISCH syndrome: A rare syndrome characterized by sparse scalp hair, scaly skin and inflammation and fibrosis of the bile ducts.
  • Neonatal hepatitis: A rare life-threatening condition where iron accumulates in the liver of the fetus and produces severe symptoms after birth. Death is common during the fetal or infant stage.
  • Obliterative portal venopathy: A rare condition characterized by abnormal hardening of liver blood vessels (portal veins), portal hypertension and an enlarged spleen. The condition usually occurs in adults but can rarely occur in children. The exact cause of the condition is unknown but various factors have been implicated e.g. genetic predisposition, workplace exposure to inorganic arsenicals, thorium compounds and vinyl chloride monomer.
  • Pancreatic cancer: Pancreatic cancer is a malignant neoplasm of the pancreas
  • Portal hypertension due to intrahepatic block: A blockage that forms in the portal vein (caries blood from the digestive tract to the liver) due to liver problems.
  • Portal thrombosis: Clotting or obstruction of blood flow along the veins from the intestines and spleen and into the liver. This causes blood to back up and results various problems such as enlarged spleen and abdominal pain. The obstruction can occur acutely (over a short space of time) or chronically (over a longer period of time).
  • Portal vein thrombosis: Clotting or obstruction of blood flow along the portal vein and into the liver. This causes blood to back up and results various problems such as enlarged spleen and abdominal pain. The obstruction can occur acutely (over a short space of time) or chronically (over a longer period of time). The portal vein collects blood from the intestines and spleen and carries it to the liver.
  • Primary biliary cirrhosis: Primary biliary cirrhosis is a chronic and progressive cholestatic disease of the liver.
  • Primary sclerosing cholangitis: Primary sclerosing cholangitis (PSC) is a chronic cholestatic liver disease of unknown etiology that is recognized increasingly in children.
  • Propanolol - Teratogenic Agent: There is evidence to indicate that exposure to Propanolol during pregnancy may have a teratogenic effect on the fetus. A teratogen is a substance that can cause birth defects. The likelihood and severity of defects may be affected by the level of exposure and the stage of pregnancy that the exposure occurred at.
  • Pulmonary hypertension: Pulmonary hypertension refers to high blood pressure in the blood vessels that carry blood to the lungs. Blood pressure in other parts of the body is normal or sometimes even low. The condition may be caused by such things as lung conditions (e.g. emphysema, chronic bronchitis), heart conditions (e.g. congestive heart failure, birth defects involving heart), AIDS or medications such as fenfluramine (a diet drug). Sometimes it occurs for no apparent reason and is called primary pulmonary hypertension.
  • Pure red cell aplasia, congenital: A rare genetic condition where the bone marrow is unable to make sufficient red blood cells which leads low levels of red blood cells. There are eight subtypes of the condition which differ in the location of the genetic defect and the incidence of additional symptoms such as malformations. The severity of symptoms is variable but most cases are serious.
  • Schistosomiasis: Parasitic fluke infection in developing countries
  • Swollen spleen: Swelling or enlargement of the spleen
  • Vein disorders: Any disorder that affects the veins of an individual
  • Vomiting blood: Blood appearing in vomited material.
  • Wilson's Disease: Wilson disease, or hepatolenticular degeneration, is a neurodegenerative disease of copper metabolism.


 » Next page: Clinical Trials for Portal hypertension

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