Background Portal hypertension is a clinical syndrome in which the portal venous pressure gradient between portal vein and inferior vena cava exceeding 5mmHg. Clinically significant portal hypertension is diagnosed when clinical manifestations of the disease appear or the portal pressure gradient exceeding 10 mmHg. For better management, it is important to determine the underlying cause. Objective To evaluate the aetiology of portal hypertension in pediatric who attending the hepatology clinic at El-Demerdash hospital, Ain Shams University. Materials and Methods This cross sectional study was done in the department of pediatric hepatology clinic at El-Demerdash hospital, Ain Shams University on 91 consecutive cases of portal hypertension enrolled from 2016 to 2019. All pediatric patients were subjected to full history, clinical examination, investigations and questionnaire about effect of portal hypertension and its complications on QOL. Results Age of children and adolescent was 5 months to 16 years with mean age of 5.55±4.30y and male to female ration was 1.5:1. Out of 91 children (51, 56.1%) developed portal hypertension due to extrahepatic causes and (39, 42.9%) due to hepatic causes. In extrahepatic causes (portal vein thrombosis was found 36, (39.6%) and Budd Chiari syndrome 15(16.5%)). On the other hand, hepatic causes (cong. hepatic fibrosis (CHF) was found in 21(23.1%), cases, hepatic cirrhosis 12(13.2%), extrahepatic biliary atresia 3(3.3%) and autoimmune hepatitis with secondary hepatic fibrosis 3(3.3%)). In extrahepatic cases first variant bleed much earlier than hepatic cases. Conclusion Extrahepatic disease were the most common aetiology of portal hypertension in studied cases. Portal vein thrombosis in extrahepatic cases and congenital hepatic fibrosis in intrahepatic cases were the most common causes.

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Background Ruptured oesophageal varices (OVs) is a major cause of mortality in Portal hypertension (PHT) patients, It has been a great issue of interest and research to screen and early detect OVs via oesophageal varices non-invasive methods. Objective The aim of this study was to assess the reliability of measuring plasma von willibrand factor antigen (VWF-Ag) for prediction of the occurrence of oesophageal varices in patients with portal hypertension. Subjects & Methods This was a prospective cross-sectional study, done on 47 children with portal hypertension. The children were recruited from Pediatrics Hepatology clinic, Ain Shams University. Patient’s data was collected including age, sex, etiology and duration of PHT, along with medical treatment. Also an upper GIT endoscope, abdominal doppler ultrasound, and laboratory tests including measuring of plasma VWF-Ag were done to each patient. Then the children were divided based on their endoscopic findings into two groups; variceal group which included 37 patients, and a nonvariceal group which included 10 patients Results: The results of our study revealed an elevated plasma VWF-Ag in patients with oesophageal varices, whilst normal levels of plasma VWF-Ag in the non-variceal patients. In addition, there was a direct positive correlation between increased plasma VWF-Ag and the degree of oesophageal varices. Conclusion Since the plasma VWF-Ag level correlates with the presence and degree of OVs, it can be used as a noninvasive indicator of the presence and degree of OVs, However, further studies using larger sample might be needed to support this.