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Portal Hypertension in Children: A Tertiary Center Experience in Turkey
Emine Nur Sunar Yayla1, Sinan Sarı2, Neslihan Gürcan Kaya2
1Department of Pediatrics, Faculty of Medicine, Gazi University, Ankara, Turkey.
Insights
Portal hypertension (PH) in children has varied causes like portal vein thrombosis and biliary atresia. PH without cirrhosis may present more severe complications than PH with cirrhosis.
Area of Science:
- Pediatric Gastroenterology
- Hepatology
- Clinical Research
Background:
- Portal hypertension (PH) significantly impacts pediatric morbidity and mortality.
- Understanding the spectrum of PH in children is crucial for effective management.
Purpose of the Study:
- To evaluate the etiology, clinical, laboratory, and endoscopic findings of pediatric PH.
- To analyze treatment approaches, long-term outcomes, and prognosis in pediatric PH patients.
Main Methods:
- Retrospective study of 222 pediatric patients diagnosed with PH between 1998 and 2016.
- Analysis of clinical, laboratory, radiological features, treatments, and complications.
Main Results:
- Common causes include portal vein thrombosis (20.3%), progressive familial intrahepatic cholestasis (18.9%), and biliary atresia (12.2%).
- Cirrhotic (59.0%) vs. non-cirrhotic (41.0%) groups showed distinct clinical features.
- Non-cirrhotic PH patients had more variceal bleeding, while cirrhotic patients had more ascites and hepatic encephalopathy.
Conclusions:
- Extrahepatic portal vein obstruction, genetic-metabolic diseases, and biliary atresia are key causes of pediatric PH.
- Clinical signs like hepatomegaly and elevated transaminases suggest cirrhotic PH.
- Pediatric PH without cirrhosis may indicate a more severe condition.
Purpose:
Portal hypertension (PH) and its complications have a significant impact on morbidity and mortality. This study aimed to evaluate the etiology; clinical, laboratory, and endoscopic findings; treatment approaches; long-term outcomes; and prognosis of pediatric PH.
Methods:
This retrospective study included 222 pediatric patients diagnosed with PH between 1998 and 2016, and data encompassing clinical, laboratory, and radiological features; treatments; and complications were analyzed.
Results:
The most common causes of PH were portal vein thrombosis (20.3%), progressive familial intrahepatic cholestasis (18.9%), and biliary atresia (12.2%). Among the enrolled patients, 131 (59.0%) were included in the cirrhotic group and 91 (41.0%) in the non-cirrhotic group. Hepatomegaly and increased transaminase levels were more frequent in the cirrhotic group than in the non-cirrhotic group. Additionally, portal gastropathy, esophageal varices, and variceal bleeding were more frequent in the non-cirrhotic group, whereas ascites, hepatopulmonary syndrome and hepatic encephalopathy were more common in the cirrhotic group. The incidence of hepatomegaly was higher in the presinusoidal group than in the prehepatic group (p<0.001). Hyperbilirubinemia was more frequent in the prehepatic group (p=0.046). The frequency of esophageal varices was similar between the prehepatic and presinusoidal groups; however, variceal bleeding was more frequent in the prehepatic group (p=0.002).
Conclusion:
Extrahepatic portal vein obstruction, genetic-metabolic diseases, and biliary atresia were the most prevalent causes of PH in our country. In patients with PH, hepatomegaly, increased transaminase levels, and synthesis dysfunction were suggestive of cirrhotic PH. Notably, PH in patients without cirrhosis might be more severe than that in those with cirrhosis.
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