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]

• Splenectomy

  In considering splenectomy, the high risk of thrombosis in the portal venous system should be taken into account. Prognosis Current overall prognosis is good (80% survival at ten years after the diagnosis). [orpha.net]

  Symptomatic hypersplenism or painful splenic lump merits splenectomy or splenic artery embolization. [cancertherapyadvisor.com]

  The role of congestion (portal hypertension) in socalled Banti’s syndrome: a clinical and pathologic study of thirtyone cases with the late results following splenectomy. JAMA 1936; 107: 1788–93. 18. Whipple AO. [jpatholtm.org]

  and primary myeloproliferative disorder in one; sclerotherapy in two, including one who also had abdominal sepsis; surgical portocaval shunt in one; adenocarcinoma of the colon in one; and splenectomy and protein S deficiency in one. [gut.bmj.com]

• Chronic Infection

  Chronic infection by hepatotropic viruseswas discarded and a screening of other frequent causes of chronic liverdamage did not reveal infectious, autoimmune or metabolic diseases.A hypercoagulable state was discarded. [elsevier.es]

  Chronic infections Data indicate that intestinal infection with E. coli might cause recurrent septic embolization and subsequent obstruction of small portal veins, a probable trigger of INCPH. [ojrd.biomedcentral.com]

  This disorder is linked with several conditions such as immunological disorders, chronic infections, prothrombotic disorders, genetic predisposition and/or toxins. There is no specific positive diagnostic test. [rarediseasesjournal.com]

• Fever

  Case Report A 2-year-old boy with coryza, cough, fever and mouth wounds suffered episodes of melena and a fall in hemoglobin (5.1 g dl −1 ), requiring replacement with blood derivatives. [academic.oup.com]

  Portal pylephlebitis should be suspected if spiky fevers, tenderness, shock, and sepsis-related cholestasis are seen. – Chronic EHPVO. [cancertherapyadvisor.com]

• Cough

  Case Report A 2-year-old boy with coryza, cough, fever and mouth wounds suffered episodes of melena and a fall in hemoglobin (5.1 g dl −1 ), requiring replacement with blood derivatives. [academic.oup.com]

• Gastropathy

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

  Portal hypertensive gastropathy is uncommon and is a rare cause of upper gastrointestinal (UGI) bleed at the first presentation in patients who have NCPF. [cancertherapyadvisor.com]

• Abdominal Distension

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

• Odynophagia

  Dysphagia/odynophagia (pain on swallowing; uncommon). Confusion secondary to encephalopathy (even coma). Signs Peripherally shut down. Pallor. Hypotension and tachycardia (ie shock). Reduced urine output. Melaena. Signs of chronic liver disease. [patient.info]

• Hepatomegaly

  It is manifested by splenomegaly, portal hypertension, and variable ascites and hepatomegaly. Liver histology is initially normal but subsequently shows periportal fibrosis without cirrhosis. [ncbi.nlm.nih.gov]

  Presenting features were gastrointestinal(GI) bleeding (9 patients) or the fortuitous finding of anenlarged spleen (21 patients), raised serum alanineaminotransferase activity (6 patients), thrombocytopenia(6 patients), hepatomegaly (3 patients), abnormal [dokumen.tips]

  Mild hepatomegaly may be present. The diagnosis of NRH is secured by histopathology demonstrating regenerative nodules without parietal thickening of portal venules, and no or minimal perisinusoidal and portal fibrosis on reticulin staining. [wjgnet.com]

  Nodular regenerative hyperplasia: a cause of ascites and hepatomegaly after chemotherapy for leukemia. Am J Gastroenterol. 1991;86(1):86–88. 53. Al-Mukhaizeem KA, Lamoureux E, Rosenberg A, Sherker AH. [dovepress.com]

• Ulcer

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

  Pulmonary hypertension 32 [ 11, 24 - 44 ] Rheumatoid arthritis/Felty’s syndrome 30 [ 31, 41, 44 - 55 ] Human immunodeficiency virus infection 20 [ 21, 56 - 64 ] Lupus erythematosus 12 [ 27, 40, 65 - 70 ] Crohn’s disease/ulcerative colitis 13 [ 17, 28, [wjgnet.com]

  […] this study lacks of follow up so it is unknown whether portal hypertension may develop in some of the OPV patients as previously described by Cazals-Hatem et al. b Thirteen patients (22%) had portal vein thrombosis at diagnosis c Celiac disease (12%), Ulcerative [rarediseasesjournal.com]

• Stroke

  […] disorders (n=4) • Neurological disorder (n=2) • Renal insufficiency (n=2) • Infection (n=1) Liver related (n=4) • Sepsis (n=2) • Hepatopulmonary syndrome (n=1) • Multiorgan failure (n=1) 3 of them 1 year after LT Non-liver related (n=1) Hemorrhagic stroke [rarediseasesjournal.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]

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]

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]

However, drug treatment may stop bleeding in the majority of these patients. [patient.info]

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]

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]

Non-cirrhotic idiopathic portal hypertension (NCIPH), also known as hepatoportal sclerosis (HPS) is a disease of uncertain etiology. [biblat.unam.mx]

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

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]

However, many pathophysiological mechanisms has been postulated, including thrombophilia, chronic recurrent infections and exposure to drugs or toxins. [biblat.unam.mx]

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]

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]