Hepatoportal sclerosis clinical different evolutionary stages: presentation of 3 cases and literature review
Hepatoportal sclerosis (HPS) is characterized by presinusoidal intrahepatic portal hypertension associated with splenomegaly and anemia in patients with non-cirrhotic liver. Liver biopsy is essential, especially to rule out other processes. Being a disease of unknown etiology, the majority of cases...
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Veröffentlicht in: | Revista española de enfermedades digestivas 2012-02, Vol.104 (2), p.94-97 |
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creator | Martínez-Caselles, Alejandro Pons-Miñano, José Antonio Vargas-Acosta, Ángel Ortiz-Sánchez, María Luisa Martínez-Pascual, Cristina Ruiz-Maciá, José Antonio Ferri, Belén Ortiz-Ruiz, Eduardo Sola, Joaquín |
description | Hepatoportal sclerosis (HPS) is characterized by presinusoidal intrahepatic portal hypertension associated with splenomegaly and anemia in patients with non-cirrhotic liver. Liver biopsy is essential, especially to rule out other processes. Being a disease of unknown etiology, the majority of cases have been described in eastern countries. However, it may be an underdiagnosed disease in the West. Symptoms are related to portal hypertension and the clinical spectrum is wide, ranging from anemia with normal liver function tests to bleeding due to esophageal varices. Treatment is directed to the complications and the prognosis is better than in patients with cirrhosis.We report three cases of HPS presenting at different clinical stages and the findings of liver biopsies, the clinical outcomes and a review of scientific literature. |
doi_str_mv | 10.4321/S1130-01082012000200010 |
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Liver biopsy is essential, especially to rule out other processes. Being a disease of unknown etiology, the majority of cases have been described in eastern countries. However, it may be an underdiagnosed disease in the West. Symptoms are related to portal hypertension and the clinical spectrum is wide, ranging from anemia with normal liver function tests to bleeding due to esophageal varices. 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Liver biopsy is essential, especially to rule out other processes. Being a disease of unknown etiology, the majority of cases have been described in eastern countries. However, it may be an underdiagnosed disease in the West. Symptoms are related to portal hypertension and the clinical spectrum is wide, ranging from anemia with normal liver function tests to bleeding due to esophageal varices. Treatment is directed to the complications and the prognosis is better than in patients with cirrhosis.We report three cases of HPS presenting at different clinical stages and the findings of liver biopsies, the clinical outcomes and a review of scientific literature.</description><subject>Adult</subject><subject>Biopsy</subject><subject>Gastroenterology & Hepatology</subject><subject>Geriatrics & Gerontology</subject><subject>Humans</subject><subject>Hypertension, Portal - diagnosis</subject><subject>Hypertension, Portal - pathology</subject><subject>Infectious Diseases</subject><subject>Liver - pathology</subject><subject>Liver Cirrhosis - diagnosis</subject><subject>Liver Cirrhosis - pathology</subject><subject>Male</subject><subject>Middle Aged</subject><subject>Oncology</subject><subject>Pancytopenia - diagnosis</subject><subject>Pancytopenia - pathology</subject><subject>Pathology</subject><subject>Pharmacology & Pharmacy</subject><subject>Radiology, Nuclear Medicine & Medical Imaging</subject><subject>Splenomegaly - diagnosis</subject><subject>Splenomegaly - pathology</subject><subject>Surgery</subject><issn>1130-0108</issn><fulltext>true</fulltext><rsrctype>article</rsrctype><creationdate>2012</creationdate><recordtype>article</recordtype><sourceid>EIF</sourceid><recordid>eNp9UU1LAzEQzUGxtfoXNDdPrZNNu5t4k6JWEDyo5yVNJpKSbtYkW_Hfu7W1F8HDMPA-5sEbQi4ZTKa8YNcvjHEYAwNRACsAYDsMjsjwQAzIaUorgCkvZ8UJGRQFrwoBsyHxC2xVDm2IWXmatMcYkktUe9c43UPGWYsRm0xxE3yXXWhU_KIpq3dMN7SNmHpSbXEaLOVUq4SJqsZQ7zJGlbuINOLG4ecZObbKJzzf7xF5u797nS_GT88Pj_Pbp7GeAuQxB2Y5orAwA5DcaIZQCamZUJUwRWlQGaZLUxqJshJLq6UEC8ZW1pRKCT4ik93dpB36UK9CF5s-sP6pqv5TVW-42hnaGD46TLleu6TRe9Vg6FItS8GAzaTsldVOqfueUkRbt9Gt-0ZqBvX2Hf9kXOwzuuUazcH3-wv-DbWCh2Q</recordid><startdate>20120201</startdate><enddate>20120201</enddate><creator>Martínez-Caselles, Alejandro</creator><creator>Pons-Miñano, José Antonio</creator><creator>Vargas-Acosta, Ángel</creator><creator>Ortiz-Sánchez, María Luisa</creator><creator>Martínez-Pascual, Cristina</creator><creator>Ruiz-Maciá, José Antonio</creator><creator>Ferri, Belén</creator><creator>Ortiz-Ruiz, Eduardo</creator><creator>Sola, Joaquín</creator><general>Sociedad Española de Patología Digestiva</general><scope>CGR</scope><scope>CUY</scope><scope>CVF</scope><scope>ECM</scope><scope>EIF</scope><scope>NPM</scope><scope>AAYXX</scope><scope>CITATION</scope><scope>7X8</scope><scope>GPN</scope></search><sort><creationdate>20120201</creationdate><title>Hepatoportal sclerosis clinical different evolutionary stages: presentation of 3 cases and literature review</title><author>Martínez-Caselles, Alejandro ; 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Liver biopsy is essential, especially to rule out other processes. Being a disease of unknown etiology, the majority of cases have been described in eastern countries. However, it may be an underdiagnosed disease in the West. Symptoms are related to portal hypertension and the clinical spectrum is wide, ranging from anemia with normal liver function tests to bleeding due to esophageal varices. Treatment is directed to the complications and the prognosis is better than in patients with cirrhosis.We report three cases of HPS presenting at different clinical stages and the findings of liver biopsies, the clinical outcomes and a review of scientific literature.</abstract><cop>Spain</cop><pub>Sociedad Española de Patología Digestiva</pub><pmid>22372805</pmid><doi>10.4321/S1130-01082012000200010</doi><tpages>4</tpages><oa>free_for_read</oa></addata></record> |
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subjects | Adult Biopsy Gastroenterology & Hepatology Geriatrics & Gerontology Humans Hypertension, Portal - diagnosis Hypertension, Portal - pathology Infectious Diseases Liver - pathology Liver Cirrhosis - diagnosis Liver Cirrhosis - pathology Male Middle Aged Oncology Pancytopenia - diagnosis Pancytopenia - pathology Pathology Pharmacology & Pharmacy Radiology, Nuclear Medicine & Medical Imaging Splenomegaly - diagnosis Splenomegaly - pathology Surgery |
title | Hepatoportal sclerosis clinical different evolutionary stages: presentation of 3 cases and literature review |
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