The most common presenting symptom was abdominal distension. [ncbi.nlm.nih.gov]

Gangrene intestine in HPS is a rare presentation. Key words: Hepatoportal sclerosis; Portal hypertension; Gangrene; Intestine [scopemed.org]

• Turkish

  We conclude that genetic predisposition might be a possible factor for HPS development in Turkish patients and it should be kept in mind that cholestatic features noticed in histopathological evaluation may represent a variant group in the spectrum of [ncbi.nlm.nih.gov]

  […] sclerosis. ( 17414109 ) Isabel Fiel M....Schiano T.D. 2007 36 Hepatoportal sclerosis as a cause of noncirrhotic portal hypertension in patients with HIV. ( 17640321 ) 2007 37 Clinical features, serum interleukin-6, and interferon-gamma levels of 34 turkish [malacards.org]

• Gangrene

  Gangrene intestine in HPS is a rare presentation. Key words: Hepatoportal sclerosis; Portal hypertension; Gangrene; Intestine [scopemed.org]

  Authors: Vidyavathi, K Harendrakumar, M L Keywords: Hepatoportal Sclerosis (HPS) Portal Hypertension Gangrene Intestine Issue Date: Jul-2014 Citation: Bommanahalli B P, Vidyavathi K, Harendrakumar M L. [imsear.li.mahidol.ac.th]

• 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]

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]

• Liver Enzymes Abnormal

  At that time, MP was discontinued, and the liver enzyme abnormalities gradually returned to normal within three weeks. [academic.oup.com]

  Liver enzymes can be normal or slightly abnormal; presentation with isolated liver enzyme abnormalities was previously reported in 20% of INCPH cases [ 46 ]. [jpatholtm.org]

• 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]

• Helicobacter Pylori

  The upper endoscopy showeda mild portal gastropathy, little esophageal varices and an Helicobacter pylori positive duodenal ulcer. [elsevier.es]

12 Most of these cases occurred during the first 6 months of treatment. [academic.oup.com]

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]

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]

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]

AIMS : We will compare the evolution of thrombosis in patients who received a “curative” treatment for PNH, including eculizumab and bone marrow transplantation, to patients who did not receive one of these 2 types of treatments, either because it was [valdig.eu]

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]

We describe two cases of HPS in patients without known etiology causes associated with this disease. [ncbi.nlm.nih.gov]

We describe two cases of HPS in patients without known etiology causes associated with this disease. Results. [arca.fiocruz.br]

To date, its etiology is unknown. Etiopathogenesis of these rare disorders remains unclear, but the hypothesis of vascular thrombotic mechanism has been suggested. [humpath.com]

Extensive work-up to determine the etiology of any underlying liver disease was unrevealing. NRH and OPV were identified on liver biopsy. [wwww.unboundmedicine.com]

/therapy ; Fibrosis/complications ; Gastrointestinal Hemorrhage/etiology/therapy ; Humans ; Hypertension, Portal/complications/diagnosis/therapy ; Liver Cirrhosis/complications/diagnosis/therapy ; Male ; Melena/etiology ; Portal System ; Protein C Deficiency [orbi.uliege.be]

Author Affiliations From the Department of Epidemiology, Harvard School of Public Health, the Epidemiology and Radiation branches, National Cancer Institute, U.S. [nejm.org]

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]

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]

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]

However, various pathophysiological mechanisms has been postulated, including chronic or recurrent infections and exposure to drugs or toxins. [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]

Etiology and pathophysiology Etiology The etiology of INCPH is unknown [ 1, 4, 9, 14, 16 ]. [ojrd.biomedcentral.com]

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]

Prevention [ 8 ] No specific treatment has been shown to prevent the formation of varices. Prevention of first variceal haemorrhage depends on the size/characteristics of varices. [patient.info]

Primaryprophylaxis of variceal bleeding could be helpful to prevent complicationsand if bleeding occurs, endoscopic and/or a portosystemicshunt are appropriate. [elsevier.es]