특발성 문맥압 항진증 8예의 임상 및 병리학적 분석
Clinicopathological Analysis of Eight Cases of Idiopathic Portal Hypertension
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Abstract
Background : Idiopathic portal hypertension (IPH) is a rare clinicopathologic entity that shows clinical evidences of portal hypertension with no pathologic features of cirrhosis. Methods : The clinical and pathologic features of 8 cases with IPH were analyzed via the medical records along with the biopsy or resected liver specimens. Results : Six patients were male and two were female. The chief complaints were sudden variceal bleeding in seven patients and abdominal pain in one patient. Six patients were treated with varix ligation and one was treated with splenectomy after the failure of bleeding control. One patient underwent a liver transplantation due to severe symptoms of portal hypertension. The prognosis of all the patients was excellent. Microscopically, the portal tracts were variably fibrotic, and the portal veins in them were sclerotic, obliterated or dilated in 7 cases; pathologic abnormalities were absent in 1 case. Cirrhosis was absent in all cases, while septal fibrosis was present in one resected liver. Conclusions : IPH is a minor cause of portal hypertension. However, a liver biopsy to show the subtle portal vascular changes and fibrosis in patients who have the clinical symptoms of portal hypertension is important for making the diagnosis of IPH.
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