ABSTRACT
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.
RESUMO CONTEXTO: Esclerose hepatoportal EHP ou venopatia portal obliterativa VPO, um dos diagnósticos diferenciais para a hipertensão portal não cirrótica, é caracterizada pelo desaparecimento dos ramos portais, fibrose portal e septal, fibrose sinusoidal e hiperplasia nodular regenerativa HNR. A EHP é um doença espectral, que pode progredir para hipertensão portal severa. Sua etiopatologia é ainda pouco compreendida, especialmente no Brasil, onde ela é provavelmente subdiagnoticada devido as suas similaridades com a forma hepatoesplênica da esquistossomose. OBJETIVO: Analizar o perfil dos pacientes com EHP no Nordeste do Brasil, e demontrar as características patológicas da EHP. MÉTODOS: Analisamos restrospectivamente os casos de VPO em biópsias hepáticas e explantes de um centro de referência em fígado na Bahia, Brasil. A análise qualiquantitativa dos tratos portais e parênquima hepático foi realizada, permitindo a comparação entre os nossos paciente e os achados descritos por outros autores. RESULTADOS: Entre os 62 paciente identificados com EHP, 42% era do sexo masculino, 58% era do sexo feminino. A média de idade no diagnótico foi 48,3 anos. Desse grupo, analizamos a biópsia hepática de 10 pacientes nos quais o diagnóstico de esquistossomose pode ser excluído. Desses pacientes, 100% 10/10 se apresentou com fibrose portal densa e obliteração venosa portal. Atrofia do perênquima hepático estava presente em 60% 6/10 dos pacientes, dilatação sinusiodal em 30% 3/10 a presença de septos portais ocorreu em 50% 5/10 e fibrose portal densa foi achada em todos os pacientes. Hiperplasia nodular regenerativa foi encontrada em 30% dos pacientes. CONCLUSÃO: A EHP parece ser negligenciada e subdiagnosticada no Brasil, devido as suas similaridades com esquistossomose. Em nosso estudo, fibrose portal densa, obliteração dos ramos da veia porta, atrofia do parênquima, dilatação sinusoidal e hiperplasia nodular do parênquima foram os principais achados histopatológicos e foram semelhantes aos descritos em outros países.
Subject(s)
Humans , Male , Female , Hypertension, Portal/etiology , Hypertension, Portal/epidemiology , Referral and Consultation , Sclerosis/epidemiology , Brazil/epidemiology , Retrospective StudiesABSTRACT
Antecedentes: la esclerosis hepatoportal se manifiesta como hipertensión portal no cirrótica. Su etiología parece estar relacionada con alteraciones idiopáticas en la microvasculatura hepática. Las manifestaciones de la esclerosis hepatoportal incluyen sangrado de vías digestivas altas, pancitopenia, esplenomegalia e hipertensión portal no cirrótica. Presentamos el primer caso reportado en Colombia de esclerosis hepatoportal en un paciente con serología positiva para el virus de la inmunodeficiencia humana (VIH). Métodos: paciente masculino de 60 años de edad, VIH-positivo, quien ingresa a nuestra institución por hemorragia de vías digestivas alta (várices esofágicas y fúndicas) y ascitis, cuyo manejo requirió la toma de biopsia hepática. Resultados: se realizó biopsia Trucut de hígado que evidenció la presencia de 6 a 8 espacios porta con parénquima arquitectónico conservado que demostró fibrosis perivenular y dilatación sinusoidal pericentral severa. Conclusión: la esclerosis hepatoportal es una causa de morbilidad en pacientes VIH-positivos. Debe considerarse en cada paciente que manifiesta hipertensión portal no cirrótica asociada con hemorragia de la vía digestiva alta. Sin embargo, una investigación adicional es imprescindible con el fin de describir la relación entre el desarrollo de alteraciones intrahepáticas (microtrombosis), la patogénesis del VIH y el uso de terapia antirretroviral, particularmente el uso de didanosina.
Background: Hepatoportal sclerosis manifests as non-cirrhotic portal hypertension. Its etiology appears to be related to alterations in the idiopathic micro-vasculature of the liver. Manifestations of hepatoportal sclerosis include upper gastrointestinal bleeding, pancytopenia, splenomegaly and non-cirrhotic portal hypertension. We present the first reported case of hepatoportal sclerosis in Colombia which occurred in an HIV positive patient. Methods: A 60-year-old male HIV patient positive was admitted to our institution because of ascites and upper digestive tract bleeding due to esophageal and fundal varices. Management required taking a liver biopsy. Results: A Tru-Cut biopsy needle was used to take a liver biopsy sample percutaneously. The biopsy revealed six to eight portal tracts with preserved architectural parenchyma, perivenular fibrosis and severe pericentral sinusoidal dilatation. Conclusions: Hepatoportal sclerosis is a cause of morbidity in HIV-positive patients and should be considered in each patient manifesting non-cirrhotic portal hypertension associated with upper gastrointestinal bleeding. However, further research is necessary to describe the relationship between the development of intrahepatic alterations (microthrombosis), HIV, and the use of anti-retroviral therapy, particularly the use of didanosine.
Subject(s)
Humans , Male , Middle Aged , Antiretroviral Therapy, Highly Active , Biopsy , Hypertension, Portal , Immunologic Deficiency Syndromes , Liver , SclerosisABSTRACT
A 35 year old female presented with pain abdomen, massive hematemesis and signs of portal hypertension. Death occurred despite of all efforts to manage shock & hematemesis by endoscopic sclerotherapy. Clinical autopsy revealed gangrene of small intestine with mesenteric vein thrombosis; oesophageal varices; hepatoportal sclerosis (HPS) of liver with regenerative nodules and chronic venous congestion of spleen. Histopathology of liver is essential for diagnosis of HPS & to rule out other disease process. Gangrene intestine in HPS is a rare presentation
ABSTRACT
Hepatoportal sclerosis (HPS) is defined as sclerosis of portal areas in the absence of cirrhosis. There is little information about HPS in children in the literature. The aim of this study was to describe the clinical presentation, associated disorders, laboratory characteristics and outcome of children who were diagnosed as HPS. This study included 12 children diagnosed as HPS by the Pathology Department between 2005 and 2011. Data were collected from the gastroenterology clinic charts retrospectively, including demographics, presentation characteristics, laboratory data and recent status of patients. Twelve patients were enrolled (6 girls, 6 boys). The median age of patients was 13.5 yr. Median age at the time of biopsy was 11 yr. Four patients had splenomegaly, 3 had esophageal varices, one had hepatopulmonary syndrome and had been transplanted. Smooth muscle antibody was found positive in 4 patients, without autoimmune hepatitis findings in liver biopsy. One patient had celiac disease and another patient had positive celiac disease serology but pathology findings. Another patient had Turner's syndrome. Mean follow-up time was 39 months (3.3 yr) after biopsy. Hepatoportal sclerosis does not necessarily present with portal hypertension in children.