Non-cirrhotic portal fibrosis idiopathic portal hypertension: Sign in to make a comment Sign in to your personal account. Treatment is directed to the complications and the prognosis is better than in patients with cirrhosis. Sign in to customize your interests Sign in to your personal account. In this country Osler published several papers—the first in. A month later the patient was readmitted for hepatic encephalopathy and was put on the waiting list for liver transplantation.

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Zubar This is a dr year old man with a past history of antrectomy and Roux en Enfdrmedad gastrojejunostomy for duodenal ulcer bleeding five years earlier. Discussion The etiology of the HPS is unknown. The basic pathology is some kind of obstructive pathology in the portalhepatic or splenic enfer,edad that causes obstruction of venous blood flow from the spleen towards the heart. After negative viral serology, autoimmune and metabolic liver diseases, coinciding with a cholecystectomy, liver biopsy was performed which showed nodular cirrhotic liver and portal vein sclerosis Fig.

Finally, liver biopsy reported a normal liver parenchyma and presence of venous dilatation in the portal space with sclerosis, elastosis and absence of bile duct in large spaces. You can help by adding to it. Harmanci O, Bayraktar Y. A month later the patient was readmitted for hepatic encephalopathy and was put on the waiting list for liver transplantation.

Most liver transplantations performed in the West for this disease have been performed with a preoperative diagnosis of cryptogenic cirrhosis. Purchase access Subscribe to the journal. Lack of cirrhosis, necrosis or inflammatory phenomena is characteristic.

Four years ago, the patient was referred to our hospital due to abdominal distension. Sign In Set Up Account. View More View Less. Viral serology, study of autoimmune and metabolic liver diseases were negative. Get free access to newly published articles.

Our website uses cookies to enhance your experience. Its association with hepatic tumors is uncommon 8. Sign in to download free article PDFs Banri in to access your subscriptions Sign in to your personal account. Liver failure and need for liver transplantation in patients with advanced hepatoportal sclerosis. Hospital Universitario Virgen de la Arrixaca. The patient remained stable during the following years and enfwrmedad episode of bleeding from large esophageal varices was resolved by endoscopic treatment and vasoactive drugs.

By continuing to use our website, you are agreeing to our privacy policy. It is possible that at early stages of the disease the only clinical sign is a slight elevation of transaminases, as in two of our patients.

Please Choose One of the Following Options. This section is empty. Guido Banti, in the late nineteenth century, described a pattern of anemia, gastrointestinal bleeding and marked splenomegaly 1. The term was introduced by Mikkelsen in the sixties to refer to a pattern of non-cirrhotic portal hypertension associated with splenomegaly and anemia 3. Create bannti free personal account to access your subscriptions, sign up for alerts, and more.

Several radiological examinations over the years were performed abdominal ultrasonography and MRI cholangiography showing only a progressive enlargement of the spleen with incipient signs of portal hypertension.

We present three cases of HPS at different clinical stages. Cases report Case 1 This is a 60 year old man with a past history of antrectomy and Roux en Y gastrojejunostomy for duodenal ulcer bleeding five years earlier.

However, it may be an underdiagnosed disease in the West. Clinical study of eighty six cases of idiopathic portal hypertension and comparison with cirrhosis with splenomegaly. Liver biopsy is essential, especially to rule out other processes. Presently Banti syndrome is related to all disorders with portal hypertension and hepatic venous injury without cirrhosis.

To receive access to the full text of freely available articles, alerts, and more. Register for email alerts with links to free full-text articles Access PDFs bahti free articles Manage your interests Save searches and receive search alerts. 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.

Empirical treatment with UDCA was started in one of our patients achieving normalization of transaminases, however, no pathophysiological explanation could explain the response to treatment. The cause of such obstruction may be abnormalities present at birth congenital of certain veins, blood clots, or various underlying disorders causing inflammation and obstruction of veins vascular obstruction of the liver.

The prognosis of these patients is better than those with portal hypertension associated with cirrhosis, depending on the duration fo the disease being worse when it diagnosed in youth.

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One of our patients met the clinical criteria and, having ruled out most diseases commonly associated with portal hypertension, it was decided to perform a liver biopsy which provided the diagnosis of HPS. J Gastroenterol Hepatol ; In case of significant hypersplenism, splenectomy would be indicated. The patient has remained asymptomatic with normal abdominal ultrasound, gastroscopy and MRI cholangiography.

Being a disease of unknown etiology, the majority of cases have been described in eastern countries. Related Posts


Banti's syndrome

Akinolkis Recently, it has been described the association with celiac disease and a decrease of portal hypertension after introducing a gluten free diet 5. Finally, a multifactorial origin has been raised which associates all the theories described. Many of these were readily classified in one of the several established disease entities. Subscribe to Annals of Internal Medicine. This site uses cookies. Create a free personal account to download free article PDFs, sign up for alerts, customize your interests, and more. The patient remained stable during the following years and an episode of bleeding from large esophageal varices was resolved by endoscopic treatment and vasoactive drugs.


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