Idiopathic Noncirrhotic Portal Hypertension: An Appraisal
Idiopathic noncirrhotic portal hypertension is a poorly defined clinical condition of unknown etiology. Patients present with signs and symptoms of portal hypertension without evidence of cirrhosis. The disease course appears to be indolent and benign with an overall better outcome than cirrhosis, a...
| Main Authors: | , , |
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| Format: | Article |
| Language: | English |
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Korean Society of Pathologists & the Korean Society for Cytopathology
2016-01-01
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| Series: | Journal of Pathology and Translational Medicine |
| Subjects: | |
| Online Access: | http://www.jpatholtm.org/upload/pdf/jptm-2015-09-23.pdf |
| _version_ | 1818026137552420864 |
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| author | Hwajeong Lee Aseeb Ur Rehman M. Isabel Fiel |
| author_facet | Hwajeong Lee Aseeb Ur Rehman M. Isabel Fiel |
| author_sort | Hwajeong Lee |
| collection | DOAJ |
| description | Idiopathic noncirrhotic portal hypertension is a poorly defined clinical condition of unknown etiology. Patients present with signs and symptoms of portal hypertension without evidence of cirrhosis. The disease course appears to be indolent and benign with an overall better outcome than cirrhosis, as long as the complications of portal hypertension are properly managed. This condition has been recognized in different parts of the world in diverse ethnic groups with variable risk factors, resulting in numerous terminologies and lack of standardized diagnostic criteria. Therefore, although the diagnosis of idiopathic noncirrhotic portal hypertension requires clinical exclusion of other conditions that can cause portal hypertension and histopathologic confirmation, this entity is under-recognized clinically as well as pathologically. Recent studies have demonstrated that variable histopathologic entities with different terms likely represent a histologic spectrum of a single entity of which obliterative portal venopathy might be an underlying pathogenesis. This perception calls for standardization of the nomenclature and formulation of widely accepted diagnostic criteria, which will facilitate easier recognition of this disorder and will highlight awareness of this entity. |
| first_indexed | 2024-12-10T04:27:13Z |
| format | Article |
| id | doaj.art-4113c06207004bb7b666bfdaae8e35d3 |
| institution | Directory Open Access Journal |
| issn | 2383-7837 2383-7845 |
| language | English |
| last_indexed | 2024-12-10T04:27:13Z |
| publishDate | 2016-01-01 |
| publisher | Korean Society of Pathologists & the Korean Society for Cytopathology |
| record_format | Article |
| series | Journal of Pathology and Translational Medicine |
| spelling | doaj.art-4113c06207004bb7b666bfdaae8e35d32022-12-22T02:02:15ZengKorean Society of Pathologists & the Korean Society for CytopathologyJournal of Pathology and Translational Medicine2383-78372383-78452016-01-01501172510.4132/jptm.2015.09.2316569Idiopathic Noncirrhotic Portal Hypertension: An AppraisalHwajeong LeeAseeb Ur RehmanM. Isabel Fiel0 Department of Pathology, The Mount Sinai Medical Center, New York, NY, USAIdiopathic noncirrhotic portal hypertension is a poorly defined clinical condition of unknown etiology. Patients present with signs and symptoms of portal hypertension without evidence of cirrhosis. The disease course appears to be indolent and benign with an overall better outcome than cirrhosis, as long as the complications of portal hypertension are properly managed. This condition has been recognized in different parts of the world in diverse ethnic groups with variable risk factors, resulting in numerous terminologies and lack of standardized diagnostic criteria. Therefore, although the diagnosis of idiopathic noncirrhotic portal hypertension requires clinical exclusion of other conditions that can cause portal hypertension and histopathologic confirmation, this entity is under-recognized clinically as well as pathologically. Recent studies have demonstrated that variable histopathologic entities with different terms likely represent a histologic spectrum of a single entity of which obliterative portal venopathy might be an underlying pathogenesis. This perception calls for standardization of the nomenclature and formulation of widely accepted diagnostic criteria, which will facilitate easier recognition of this disorder and will highlight awareness of this entity.http://www.jpatholtm.org/upload/pdf/jptm-2015-09-23.pdfIdiopathicNoncirrhoticHypertension, portalLiverHistopathology |
| spellingShingle | Hwajeong Lee Aseeb Ur Rehman M. Isabel Fiel Idiopathic Noncirrhotic Portal Hypertension: An Appraisal Journal of Pathology and Translational Medicine Idiopathic Noncirrhotic Hypertension, portal Liver Histopathology |
| title | Idiopathic Noncirrhotic Portal Hypertension: An Appraisal |
| title_full | Idiopathic Noncirrhotic Portal Hypertension: An Appraisal |
| title_fullStr | Idiopathic Noncirrhotic Portal Hypertension: An Appraisal |
| title_full_unstemmed | Idiopathic Noncirrhotic Portal Hypertension: An Appraisal |
| title_short | Idiopathic Noncirrhotic Portal Hypertension: An Appraisal |
| title_sort | idiopathic noncirrhotic portal hypertension an appraisal |
| topic | Idiopathic Noncirrhotic Hypertension, portal Liver Histopathology |
| url | http://www.jpatholtm.org/upload/pdf/jptm-2015-09-23.pdf |
| work_keys_str_mv | AT hwajeonglee idiopathicnoncirrhoticportalhypertensionanappraisal AT aseeburrehman idiopathicnoncirrhoticportalhypertensionanappraisal AT misabelfiel idiopathicnoncirrhoticportalhypertensionanappraisal |