Presentation
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]
Entire Body System
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Leg Edema
On admission, the patient was noted to have significant abdominal distention and tenderness, and pitting leg edema, although no cutaneous stigmata of chronic liver disease were observed on physical exam. [academic.oup.com]
Neurologic
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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]
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Lethargy
Diagnosis Symptoms Lethargy and in advanced stages symptoms of liver failure Physical Examination Eye Jaundice Abdomen Ascites Spleenomegaly Signs of portal hypertension Neurologic Confusion Encephalopathy Laboratory Findings Blood and Biomarker Studies [wikidoc.org]
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]
Serum
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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]
Microbiology
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Helicobacter Pylori
The upper endoscopy showeda mild portal gastropathy, little esophageal varices and an Helicobacter pylori positive duodenal ulcer. [elsevier.es]
Other Pathologies
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Small Liver
Small liver volume, significant portal fibrosis, and phlebosclerosis may contribute to hepatic synthetic dysfunction in patients with HPS. [ncbi.nlm.nih.gov]
Small liver volume, significant portal fibrosis, and phlebosclerosis may contribute to hepatic parenchymal loss and subsequent synthetic compromise. * The Lillian & Henry M. [journals.lww.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]
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]
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]
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]
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]
Pathophysiology
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]
Prevention
Elsevier), Environmental Research (Elsevier), Journal of Trace Elements in Biology & Medicine (Elsevier), Ecotoxicology and Environmental Safety (Elsevier), Human & Experimental Toxicology, Oxidative Medicine & Cell Longevity, Environmental Health & Preventive [books.google.de]
Primaryprophylaxis of variceal bleeding could be helpful to prevent complicationsand if bleeding occurs, endoscopic and/or a portosystemicshunt are appropriate. [elsevier.es]
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]