الفهرس 
H EPATOPULMONARY SYNDROMEOnly 14 pages are availabe for public view
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Abstract
Background: The two pulmonary vascular consequences of portal hypertension and liver cirrhosis are hepatopulmonary syndrome (HPS) and portopulmonary hypertension (POPH). HPS is primarily a gas exchange problem characterized by arterial hypoxemia. While POPH is primarily a hemodynamic problem which can result in right heart failure and death, (HPS) and (POPH) are distinct clinical and pathophysiollogical entities that may accompany liver disease. Although characterized by markedly different clinical and physiological features. Aim: to assess screening of hepatopulmonary syndrome and portopulmonary hypertention in cholestatic patients versus non cholestatic patients. Methods: the study was conducted on 25 cholestatic patients and 25 non cholestatic patients at the pediatric Hepatology Clinic, Faculty of Medicine Ain Shams University and Dr Yassin Abd El Ghaffar charity center for liver disease research. All patients were subjected to detailed medical history, thorough clinical examination, laboratory investigations included Complete blood count, liver function tests, Coagulation profile and oxygen saturation test, blood gases, pulmonary function tests,12 leads ECG, abdominal ultrasonography, conventional 2D echocardiography and agitated saline contrast Enhanced Transthoracic Echocardiogram. Results: cholestatic group in comparison to non cholestatic group, There was no statistically significant difference as regards demographic and anthropometric data and As regards diagnosis there is statistically significant higher frequency in cholestatic group of Progressive familial intrahepatic cholestasis PFIC (that represents 44%), Biliary atresia (that represents 36.0%), while there is statistically significant higher frequency in non cholestatic group as regards diagnosis of Portal vein thrombosis PVT (that represents 36%), Autoimmune hepatitis AIH (that represents 16%), Glycogen storage disease GSD (that represents 16%), Hepatitis C virus HCV(that represents 16%). As regards portal hypertension there was no statistically significant difference between cholestatic group and non cholestatic group, even there is 32 % of cholestatic group patients have portal hypertention and 28% of non cholestatic group patients have portal hypertention. As regards Child-pugh Score, in cholestatic group Child A represented 32%, Child B represented 60% and Child C represented 8% but in non cholestatic group Child A represented 88%, Child B represented 12%. By pulse oximetry oxgygen saturation showed non statistical significant difference between cholestatic group and non cholestatic group even there is 8% abnormality in cholestatic group and 12% abnormality in non cholestatic group. Using VBGs showed no difference between cholestatic group and non cholestatic group, So ABGs were done for hypoxic patients in the two groups showing 4% of cholestatic group had hypoxemia (PaO2 78.9 mmhg). Patients in two groups showed normal Pulmonary function tests. As regards echocardiography, pulmonary hypertension was diagnosed in 36% of cholestatic group and 24% of non cholestatic group, while (4%) of cholestatic group had HPS diagnosed by agitated saline contrast echocardiography, cholestatic group had 20% POPH and non cholestatic group had 16% POPH. Conclusions: The screening for HPS is 4 % of cholestatic patients group diagnosed by oxygenation defect by (oxygen saturation test by pulse oximetry) and intrapulmonary vascular dilatations (IPVD) by agited saline contrast echocardiogram. The screening of POPH is 20% of cholestatic group and 16% of non cholestatic group, Severity of liver disease, as measured by the Child–Pugh was not associated with the presence of POPH.