hepatopulmonary syndrome


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hepatopulmonary syndrome

a syndrome consisting of liver disease (usually cirrhosis), hypoxemia, and the presence of intrapulmonary vascular dilatations; notable for the common presence of orthodeoxia and platypnea.

hepatopulmonary syndrome

arterial hypoxemia caused by pulmonary vasodilation in conjunction with chronic liver disease, usually occurring as a result of portal hypertension in cirrhosis.

hepatopulmonary syndrome

A condition in which hypoxemia due to intrapulmonary shunting and/or a V/Q mismatch develops in a Pt with liver cirrhosis; usually there is no apparent parenchymal lung disease, but Pts may have orthodeoxia, an unusual finding of ↑ hypoxemia with a change from the supine to the erect position; the pathogenesis of HPS is uncertain but may be due to an ↑ production of endogenous nitric oxide; shunting of HPS may respond to IV methylene blue

hepatopulmonary syndrome

(hĕp″ă-tō-pŭl′mō-năr″ē) [″ + L. pulmo, lung]
A combination of liver disease, decreased arterial oxygen concentration, and dilatation of the blood vessels of the lung. Clinically the patient may have signs and symptoms of liver disease, including gastrointestinal bleeding, esophageal varices, ascites, palmar erythema, and splenomegaly. Pulmonary signs include clubbing of the fingers, cyanosis, dyspnea, and decreased arterial oxygen concentration while in an upright position (orthodeoxia).
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All 5 patients having hepatopulmonary syndrome fall into child's grade C; hepatopulmonary syndrome presents with advanced cirrhosis.
Indications for LT in children with BA Complications of PHT Gastro-intestinal bleeding Recurrent cholangitis Hepatopulmonary syndrome Failure of liver Worsening coagulopathy synthetic function Intractable ascites Spontaneous bacterial peritonitis Nutritional difficulties Failure to thrive Fat-soluble vitamin deficiencies Developmental delay Failing to achieve developmental milestones in infancy Motor difficulties secondary to PHT Delayed puberty Chronic encephalopathy Miscellaneous CLD Intractable pruritus complications Development of focal lesion, suspected hepatocellular carcinoma Osteoarthropathy Poor quality of life LT = liver transplantation; BA = biliary atresia; PHT = portal hypertension; CLD = chronic liver disease.
Occurrence of hepatopulmonary syndrome in Budd-Chiari syndrome and the role of venous decompression.
Hepatopulmonary Syndrome, orthodeoxia, ventilation perfusion scan, agitated saline, liver transplant
In a prospective study, 27 patients with hepatopulmonary syndrome (HPS) had a significantly shorter median survival time than did 84 patients without the condition (10.
Early Later Graft ischemia Rejection-acute and chronic Primary nonfunction Immunosuppressant side effects Hepatorenal syndrome Biliary stenosis Hepatopulmonary syndrome Disease recurrence Hepatic artery thrombosis Malignancy Immunosuppressant toxicity Hyperacute rejection Early acute rejection Portal vein thrombosis Infection and sepsis (a) (a) Can occur at any stage.
The first publication evidencing the association between liver and pulmonary disorders was in 1884 by Fluckieger; however, it was not until 1977 that Kennedy and Knudson coined the term hepatopulmonary syndrome (HPS).
Hepatopulmonary syndrome consists of a triad of hepatic dysfunction and/or portal hypertension, intrapulmonary vascular dilatations and hypoxemia/ widened alveolar-arterial gradient.
Other potential complications include spontaneous bacterial peritonitis, hepatocellular carcinoma, hepatic encephalopathy, hepatorenal syndrome, and hepatopulmonary syndrome.
Information is given about non-alcoholic fatty, cholestatic, and metabolic liver diseases, vascular diseases, tumors, postoperative jaundice, disorders in pregnancy, hepatopulmonary syndrome, portopulmonary hypertension, and transplantation.
The third patient had hepatopulmonary syndrome and had undergone transjugular intrahepatic portosystemic shunting.
37) The hepatopulmonary syndrome (HPS) is a triad of liver disease, increased alveolar-arterial oxygen gradient, and intrapulmonary vascular dilatations.