Gene/Protein Disease Symptom Drug Enzyme Compound
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Query: UMLS:C0027960 (mole)
 21,279 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

Hepatopulmonary syndrome is defined as the clinical triad of advanced liver disease, arterial deoxygenation and intrapulmonary vascular dilatation. Its pathogenesis is not completely understood. Excessive pulmonary nitric oxide production seems to be one of the factors that contribute to the intrapulmonary vascular dilatation. Other mediators such as endothelin-1 and the heme oxygenase-1/carbon monoxide system have recently been found to be important contributors. The major clinical manifestations are arterial hypoxemia, clubbed fingers and spider nevi. Orthodeoxia is the characteristic clinical feature. Contrast-enhanced echocardiography is the preferred screening test. 99mTechnetium macroaggregated albumin (Tc-99m MAA) lung perfusion scan can further specify the diagnosis of hepatopulmonary syndrome and quantify the magnitude of shunting. No clearly effective medical treatments have been found. Although liver transplantation seems feasible to reverse this situation, it is associated with increased postoperative morbidity and mortality. A preoperative arterial oxygen tension of 50 mmHg or less and Tc-99m MAA shunt fractions of 20% or more are strong predictors of postoperative mortality that can be used to stratify patients with better outcome.
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PMID:Recent advances in hepatopulmonary syndrome. 1632 92

Hepatopulmonary syndrome, also known as hepatorenal syndrome, is a triad of liver disease, impaired oxygenation, and intrapulmonary vascular abnormalities. Forty-seven percent of patients with end-stage liver disease may have hepatopulmonary syndrome, an independent predictor of poor prognosis. Gross dilatation of pulmonary precapillary and capillary vessels, as well as an absolute increase in the number of dilated vessels, is the classic pathological description. We report a young man with cirrhosis demonstrating extrapulmonary shunting of 99mTc-MAA to kidneys with extensive peripheral arteriovenous (cutaneous) vasodilation, which we prefer to name as the "mosaic sign" in the absence of spider nevi or erythema.
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PMID:Hepatorenal cutaneous syndrome demonstrated by 99mTc macro aggregated albumin whole-body scintigraphy. 2421 35

Hepatopulmonary syndrome (HPS) increases the mortality of patients who suffered from liver cirrhosis, especially patients plagued by severe hypoxemia. Gene polymorphisms are reported to be related to the risk of HPS in cirrhotic patients. Thus, our study aims to elucidate the correlation between MMP-2 and MMP-9 gene polymorphisms and HPS in cirrhotic patients. A total of 152 cirrhotic patients suffering from HPS as well as another 152 cirrhotic patients without HPS were recruited. Polymerase chain reaction-restriction fragment length polymorphism (PCR-RFLP) was performed for MMP-2 and MMP-9 gene polymorphisms and logistic regression analysis for the relationship between clinicopathological features and HPS occurrence in cirrhotic patients. There were significant differences in genotype and allele frequency of MMP-2 rs243865 and MMP-9 rs3918242 polymorphisms between the HPS and control groups. CC/CT genotype and C allele of MMP-2 rs243865 polymorphism as well as CC/TT genotype and T allele of MMP-9 rs3918242 polymorphism increased the risk of HPS in cirrhotic patients. Genotypes of rs243865 and rs3918242 polymorphisms had remarkable correlations with spider nevi, clubbed fingers (toes), transaminase elevation, portal vein width, esophageal varices, Child-Pugh classification and partial pressure of arterial oxygen (PaO2). Logistic regression analysis showed that rs243865 and rs3918242 polymorphisms, spider nevi, clubbed fingers (toes), esophageal varices, and Child-Pugh classification were closely associated with the occurrence of HPS in cirrhotic patients. Our findings demonstrate that MMP-2 rs243865 polymorphism and MMP-9 rs3918242 polymorphism can increase the risk of HPS occurrence in cirrhotic patients, which provides a potential target for prevention of HPS in cirrhotic patients.
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PMID:Correlations of MMP-2 and MMP-9 gene polymorphisms with the risk of hepatopulmonary syndrome in cirrhotic patients: A case-control study. 3039 70