Evaluation of the patients diagnosed with portal hypertension in childhood by long-term observation in the view of etiology, clinical, laboratory and prognosis

Objective: Portal hypertension is defined as portal pressure over 10 mmHg or pressure gradient over 5 mmHg at portal vein and hepatik vein. Due to complications with high morbidity and mortality rate, this thesis aimed to investigate the etiology clinical findings, laboratory and endoscopic findings, treatment approaches and prognosis of PH in children. Methods and Materials: Files of patients who diagnosed with portal hypertension between 2012-2019 in Cukurova University Medical Faculty Hospital, Department of Pediatric Gastroenterology were retrospectively analyzed. Demographic data, complaints, physical examination findings, laboratory findings, radiological and endoscopic findings, treatment and prognosis of 43 patients were examined. Results: 72.1% of the patients were boys and 27,9% were girls.The average age of the patients included in the study was 108 months (5 months and 210 months), while the average age of diagnosis was 60 months (5 months and 204 months). 76% of the cases were non-cirrhotic and 23,3% were cirrhotic. Anatomically, 55% were prehepatic and 41,8% intrahepatic. 13,9% of the intrahepatic PH cases were presinusoidal and 27,9% were sinusoidal. While cryptogenic liver cirrhosis, caroli disease, budd chiari syndrome, congenital hepatic fibrosis bring about cirrhotic reasons, more than half of the non-cirrhotic group was consisted of PVT. The most common complaint of patients was abdominal swelling. The most common physical examination finding was splenomegaly. In the cirrhotic chamber, GGT, BUN, IgG and IgA were higher (p <0,05). Portal cavernous transformation and PVT were found more frequently in the non-cirrhotic group (p <0,05). When the endoscopic findings were evaluated, the rate of esophageal varices and portal hypertensive gastropathy were similar in the cirrhotic and non-cirrhotic groups. Portal hypertensive duodenopathy was higher in cirrhotic patients (p<0,05). Complications with high mortality and morbidity rates such as hepatorenal syndrome, hepatopulmonary syndrome, and hepatic encephalopathy occurred in 10% of cirrhotic patients, but did not occur in non-cirrhotic patients. Conclusion: Portal hypertension is a significant cause of mortality and morbidity in long-term. Any child with signs of liver disease and splenomegaly should be investigated for portal hypertension. In this study, clinical, laboratory, treatment and complications of patients with PH were examined, and the incidence of GGT, BUN, IgG, IgA, bilirubin levels, incidence of fundic varicose veins and portal hypertensive duodenopathy were significantly higher in cirrhotic patients compared to non-cirrhotic patients.