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

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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Among these patients, two were complicationfree, whereas the rest had chronic complications, such as cholangitis, portal hypertension, variceal bleeding, hypersplenism, hepatopulmonary syndrome, and cholangioblastic hepatoblastoma.
Hepatopulmonary Syndrome. Hepatopulmonary syndrome is defined by the triad of liver failure, abnormal arterial oxygenation, and intrapulmonary vascular dilatations [3].
The hepatopulmonary syndrome is a severe complication of liver disease and it significantly affects the patient's prognosis.
Common bile duct ligation in the rat: a model of intrapulmonary vasodilatation and hepatopulmonary syndrome. Am J Physiol 1997; 272: G779-G784.
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.
Named after the Star Wars hero Luke Skywalker, Luke was diagnosed with hepatopulmonary syndrome, a rare complication of liver disease that causes an abnormal function of the lungs.
The ILTS will fund Michael Ramsay, MD, of Baylor University Medical Center, in Dallas, TX who will speak on Hepatopulmonary Syndrome and Portopulmonary Hypertension and Critical Aspects of Anesthesia for Liver Transplantation.
He was diagnosed with severe hyponatremia and hepatopulmonary syndrome.