Edit concept Question Editor Create issue ticket

Hepatoportal Sclerosis

Obliterative Portal Venopathy


Presentation

  • The aim of this study was to describe the clinical presentation, associated disorders, laboratory characteristics and outcome of children who were diagnosed as HPS.[ncbi.nlm.nih.gov]
  • Seven patients presented with bleeding varices and 5 had concomitant ascites.[ncbi.nlm.nih.gov]
  • The most common presenting symptom was abdominal distension.[ncbi.nlm.nih.gov]
  • Abstract The clinical, angiographic, and pathologic features of hepatoportal sclerosis in five children are presented. Hepatosplenomegaly with preservation of liver function and esophageal varices were demonstrated.[ncbi.nlm.nih.gov]
  • It should be considered in the differential diagnosis of HIV patients presenting with variceal bleeding.[ncbi.nlm.nih.gov]
Neglect
  • An early diagnosis of APS, an acquired thrombophilia, should not be neglected since treatment with anti-coagulant drugs can prevent a new thrombotic event.[academic.oup.com]

Workup

  • Make a comprehensive diagnostic workup with data from ancillary techniques and molecular findings whenever appropriate. Effectively grasp complex topics and streamline decision- making by using extensive tables, graphs, and flowcharts.[books.google.com]
  • Provides all the necessary tools to make a comprehensive diagnostic workup including data from ancillary techniques and molecular findings whenever appropriate.[books.google.com]
Enlargement of the Liver
  • […] in young and middle-aged adults • Better prognosis than cirrhotic portal hypertension because of preserved liver function • Typically presents with signs of portal hypertension Usually normal or near normal liver function tests Macroscopic • Normal, enlarged[basicmedicalkey.com]
Microcytic Anemia
  • His labs were significant for leukocytosis, microcytic anemia, and a normal platelet count. The liver chemistry tests were significant only for a low albumin (3.0 g/dL) and a slightly prolonged prothrombin time (17.2 s).[academic.oup.com]

Treatment

  • This case underscores the potential serious liver adverse events that may arise silently and go undetected during treatment with mercaptopurine, and should alert clinicians as to the potential need to discontinue mercaptopurine in this setting.[ncbi.nlm.nih.gov]
  • Management and treatment Treatment of active bleeding and its prophylaxis can follow the recommendations for patients with cirrhosis. In considering splenectomy, the high risk of thrombosis in the portal venous system should be taken into account.[orpha.net]
  • . 12 Most of these cases occurred during the first 6 months of treatment.[academic.oup.com]
  • In general, hepatic synthetic function is preserved and treatment is aimed at relief of the portal hypertension. In this study, we present the clinical and pathologic features of HPS cases who underwent liver transplantation (LT).[ncbi.nlm.nih.gov]
  • Treatment Variceal bleeding is the main cause for morbidity and mortality. Treatment of active bleeding and its prophylaxis can follow the recommendations for patients with cirrhosis.[wikidoc.org]

Prognosis

  • Prognosis Current overall prognosis is good (80% survival at ten years after the diagnosis). Male patients and patients with disease onset before 40 years of age show a poorer prognosis.[orpha.net]
  • Natural History, Complications and Prognosis Complications Liver failure Ascites Encephalopathy Prognosis Current overall prognosis is good (80% survival at ten years after the diagnosis).[wikidoc.org]
  • The liver lesion does not progress so the prognosis is good. Links: portal hypertension[gpnotebook.co.uk]
  • 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[ncbi.nlm.nih.gov]
  • […] medications Prothrombotic states Autoimmune disorders Many cases idiopathic Clinical Issues • Up to 40% of patients with portal hypertension in India and Japan Increasingly recognized in Western countries • Most common in young and middle-aged adults • Better prognosis[basicmedicalkey.com]

Etiology

  • We describe two cases of HPS in patients without known etiology causes associated with this disease.[ncbi.nlm.nih.gov]
  • To date, its etiology is unknown.[ncbi.nlm.nih.gov]
  • The etiology of hepatoportal sclerosis is uncertain, but the disease appears to be only slowly progressive. Control of variceal bleeding by central portosystemic shunts in this condition is associated with a 50% incidence of hepatic encephalopathy.[ncbi.nlm.nih.gov]
  • We describe two cases of HPS in patients without known etiology causes associated with this disease. Results.[arca.fiocruz.br]
  • BACKGROUND AND AIM: Hepatoportal sclerosis (HPS) is a clinical syndrome of unspecified etiology depicted by enlarged spleen and portal hypertension in the lack of other chronic liver disease findings, hematological disorders or any infectious disease[ncbi.nlm.nih.gov]

Epidemiology

  • Summary Epidemiology Prevalence is unknown. Distribution is worldwide, with many cases reported in Asia. The disease may occur at any age. Both sexes are equally affected.[orpha.net]
  • Epidemiology and Demographics The prevalence of this disease is unknown, and the distribution is worldwide, with many cases reported in Asia. Age The disease can occur at any age. Gender Both males and females are equally affected.[wikidoc.org]
  • Author Affiliations From the Department of Epidemiology, Harvard School of Public Health, the Epidemiology and Radiation branches, National Cancer Institute, U.S.[nejm.org]
  • Epidemiologic studies indicate an increased incidence not only of liver disease, but also of cancers of the brain, lung, and possibly other organs. FULL TEXT[annals.org]
  • Each chapter extensively reviews epidemiology, etiopathogenesis, clinical features, diagnosis, medical treatment, and surgical treatment.[books.google.de]
Sex distribution
Age distribution

Pathophysiology

  • However, various pathophysiological mechanisms has been postulated, including chronic or recurrent infections and exposure to drugs or toxins.[ncbi.nlm.nih.gov]
  • Although the etiology is obscure, a number of theories such as immunologic and vascular endothelial cellular abnormalities have been put forward to explain the underlying pathophysiology.[ncbi.nlm.nih.gov]
  • Pathophysiology Pathology Histopathologic findings are Periportal fibrosis Occlusion of small portal veins Sclerosis of the portal venous system Proliferation of small vascular channels within/around portal tracts Causes Exposure to toxic substances ([wikidoc.org]
  • Pathophysiology: The prehepatic block of EHPVO with normal hepatic sinusoidal pressure and a high pressure in the obstructed splanchnic bed results in the formation of multiple hepatopetal thick, winding collaterals.[cancertherapyadvisor.com]

Prevention

  • Primaryprophylaxis of variceal bleeding could be helpful to prevent complicationsand if bleeding occurs, endoscopic and/or a portosystemicshunt are appropriate.[elsevier.es]
  • Transjugular intrahepatic portosystemic shunts compared with endoscopic sclerotherapy for the prevention of recurrent variceal hemorrhage. A randomized, controlled trial. Ann Intern Med 1997; 126:849-857. ‎[books.google.de]
  • The current case also raises the question of how to best predict and prevent liver damage in patients treated with AZA/MP.[academic.oup.com]
  • An early diagnosis of APS, an acquired thrombophilia, should not be neglected since treatment with anti-coagulant drugs can prevent a new thrombotic event.[academic.oup.com]

Ask Question

5000 Characters left Format the text using: # Heading, **bold**, _italic_. HTML code is not allowed.
By publishing this question you agree to the TOS and Privacy policy.
• Use a precise title for your question.
• Ask a specific question and provide age, sex, symptoms, type and duration of treatment.
• Respect your own and other people's privacy, never post full names or contact information.
• Inappropriate questions will be deleted.
• In urgent cases contact a physician, visit a hospital or call an emergency service!