HISTOPATHOLOGICAL, CLINICAL AND EPIDEMIOLOGICAL FEATURES OF HEPATOPORTAL SCLEROSIS IN A REFERRAL CENTER FOR LIVER DISEASE IN NORTHEASTERN BRAZIL

ABSTRACT BACKGROUND: Hepatoportal sclerosis HPS or obliterative portal venopathy (OPV), one of the differential diagnoses for non-cirrohtic portal hypertension, is characterized by the disappearance of the portal branches, portal and septal fibrosis, perisinusoidal fibrosis and regenerative nodula...

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Main Authors: Caio ARAÚJO, Vinícius Santos NUNES, Genario SANTOS, Luiz Antônio Rodrigues de FREITAS, Maria Isabel SCHINONI, Raymundo PARANÁ
Format: Article
Language:English
Published: Instituto Brasileiro de Estudos e Pesquisas de Gastroenterologia (IBEPEGE) 2021-10-01
Series:Arquivos de Gastroenterologia
Subjects:
Online Access:http://www.scielo.br/scielo.php?script=sci_arttext&pid=S0004-28032021000300276&tlng=en
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author Caio ARAÚJO
Vinícius Santos NUNES
Genario SANTOS
Luiz Antônio Rodrigues de FREITAS
Maria Isabel SCHINONI
Raymundo PARANÁ
author_facet Caio ARAÚJO
Vinícius Santos NUNES
Genario SANTOS
Luiz Antônio Rodrigues de FREITAS
Maria Isabel SCHINONI
Raymundo PARANÁ
author_sort Caio ARAÚJO
collection DOAJ
description ABSTRACT BACKGROUND: Hepatoportal sclerosis HPS or obliterative portal venopathy (OPV), one of the differential diagnoses for non-cirrohtic portal hypertension, is characterized by the disappearance of the portal branches, portal and septal fibrosis, perisinusoidal fibrosis and regenerative nodular hyperplasia (RNH). It is a spectral disease that may progress to severe portal hypertension. Its etiopathogenesis is still little understood, especially in Brazil, it has been probably misdiagnosed due to its histopatological similarities with the hepatosplenic form of schistosomiasis. OBJECTIVE: To analyze the profile of patients with HPS in Northeastern Brazil and to demonstrate the pathological characteristics of HPS. METHODS: We retrospectively analyzed cases of OPV in liver biopsies and explants from a referral center for liver in Bahia - Brazil. The qualitative and quantitative analysis of the portal tracts and liver parenchyma was made so that comparisons could be done among the HPS findings of our population and the findings described by other authors. RESULTS: From the 62 patients identified with HPS, 42% were male, while 58% were female. The average age at diagnosis was 48.3 years. From this group, we analyzed the liver biopsy of 10 patients whose diagnosis of schistosomiasis could be ruled out. From these 100% (10/10) presented dense portal fibrosis and portal venous obliteration. Liver parenchymal atrophy was present in 60% (6/10) of the patients, sinusoidal dilation was present in 30% (3/10), the presence of portal septa occurred in 50% (5/10) and dense portal fibrosis in all patients analyzed. Nodular regenerative hyperplasia was found in 30% (3/10) of the patients. CONCLUSION: HPS seems to be neglected and misdiagnosed in Brazil, due to its similarities with schistossomiasis. In our study dense portal fibrosis, obliteration of the portal vein branches, parenchymal atrophy, sinusoidal dilatation and parenchymal nodular hyperplasia were the main histopathological findings and were similar to that described in other countries.
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spelling doaj.art-d8c602c88e414e1cb935b5ded55c926d2022-12-21T19:34:39ZengInstituto Brasileiro de Estudos e Pesquisas de Gastroenterologia (IBEPEGE)Arquivos de Gastroenterologia1678-42192021-10-0158327628010.1590/s0004-2803.202100000-48HISTOPATHOLOGICAL, CLINICAL AND EPIDEMIOLOGICAL FEATURES OF HEPATOPORTAL SCLEROSIS IN A REFERRAL CENTER FOR LIVER DISEASE IN NORTHEASTERN BRAZILCaio ARAÚJOhttps://orcid.org/0000-0003-1395-8798Vinícius Santos NUNEShttps://orcid.org/0000-0002-0988-9526Genario SANTOShttps://orcid.org/0000-0001-7766-2238Luiz Antônio Rodrigues de FREITAShttps://orcid.org/0000-0001-5499-9858Maria Isabel SCHINONIhttps://orcid.org/0000-0001-9037-8127Raymundo PARANÁhttps://orcid.org/0000-0002-4019-4597ABSTRACT BACKGROUND: Hepatoportal sclerosis HPS or obliterative portal venopathy (OPV), one of the differential diagnoses for non-cirrohtic portal hypertension, is characterized by the disappearance of the portal branches, portal and septal fibrosis, perisinusoidal fibrosis and regenerative nodular hyperplasia (RNH). It is a spectral disease that may progress to severe portal hypertension. Its etiopathogenesis is still little understood, especially in Brazil, it has been probably misdiagnosed due to its histopatological similarities with the hepatosplenic form of schistosomiasis. OBJECTIVE: To analyze the profile of patients with HPS in Northeastern Brazil and to demonstrate the pathological characteristics of HPS. METHODS: We retrospectively analyzed cases of OPV in liver biopsies and explants from a referral center for liver in Bahia - Brazil. The qualitative and quantitative analysis of the portal tracts and liver parenchyma was made so that comparisons could be done among the HPS findings of our population and the findings described by other authors. RESULTS: From the 62 patients identified with HPS, 42% were male, while 58% were female. The average age at diagnosis was 48.3 years. From this group, we analyzed the liver biopsy of 10 patients whose diagnosis of schistosomiasis could be ruled out. From these 100% (10/10) presented dense portal fibrosis and portal venous obliteration. Liver parenchymal atrophy was present in 60% (6/10) of the patients, sinusoidal dilation was present in 30% (3/10), the presence of portal septa occurred in 50% (5/10) and dense portal fibrosis in all patients analyzed. Nodular regenerative hyperplasia was found in 30% (3/10) of the patients. CONCLUSION: HPS seems to be neglected and misdiagnosed in Brazil, due to its similarities with schistossomiasis. In our study dense portal fibrosis, obliteration of the portal vein branches, parenchymal atrophy, sinusoidal dilatation and parenchymal nodular hyperplasia were the main histopathological findings and were similar to that described in other countries.http://www.scielo.br/scielo.php?script=sci_arttext&pid=S0004-28032021000300276&tlng=enHepatoportal sclerosisobliterative portal venopathyportal hypertensionschistosomiasis
spellingShingle Caio ARAÚJO
Vinícius Santos NUNES
Genario SANTOS
Luiz Antônio Rodrigues de FREITAS
Maria Isabel SCHINONI
Raymundo PARANÁ
HISTOPATHOLOGICAL, CLINICAL AND EPIDEMIOLOGICAL FEATURES OF HEPATOPORTAL SCLEROSIS IN A REFERRAL CENTER FOR LIVER DISEASE IN NORTHEASTERN BRAZIL
Arquivos de Gastroenterologia
Hepatoportal sclerosis
obliterative portal venopathy
portal hypertension
schistosomiasis
title HISTOPATHOLOGICAL, CLINICAL AND EPIDEMIOLOGICAL FEATURES OF HEPATOPORTAL SCLEROSIS IN A REFERRAL CENTER FOR LIVER DISEASE IN NORTHEASTERN BRAZIL
title_full HISTOPATHOLOGICAL, CLINICAL AND EPIDEMIOLOGICAL FEATURES OF HEPATOPORTAL SCLEROSIS IN A REFERRAL CENTER FOR LIVER DISEASE IN NORTHEASTERN BRAZIL
title_fullStr HISTOPATHOLOGICAL, CLINICAL AND EPIDEMIOLOGICAL FEATURES OF HEPATOPORTAL SCLEROSIS IN A REFERRAL CENTER FOR LIVER DISEASE IN NORTHEASTERN BRAZIL
title_full_unstemmed HISTOPATHOLOGICAL, CLINICAL AND EPIDEMIOLOGICAL FEATURES OF HEPATOPORTAL SCLEROSIS IN A REFERRAL CENTER FOR LIVER DISEASE IN NORTHEASTERN BRAZIL
title_short HISTOPATHOLOGICAL, CLINICAL AND EPIDEMIOLOGICAL FEATURES OF HEPATOPORTAL SCLEROSIS IN A REFERRAL CENTER FOR LIVER DISEASE IN NORTHEASTERN BRAZIL
title_sort histopathological clinical and epidemiological features of hepatoportal sclerosis in a referral center for liver disease in northeastern brazil
topic Hepatoportal sclerosis
obliterative portal venopathy
portal hypertension
schistosomiasis
url http://www.scielo.br/scielo.php?script=sci_arttext&pid=S0004-28032021000300276&tlng=en
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