Obliterative portal venopathy: Difference between revisions

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* Periportal fibrosis
* Periportal fibrosis


* Occlusion of small [[portal veins]]
* [[Occlusion of small portal veins]]


* Sclerosis of the portal venous system
* [[Sclerosis]] of the [[portal venous system]]


* Proliferation of small vascular channels within/around portal tracts
* Proliferation of small vascular channels within/around [[portal tracts]]


== Causes ==
== Causes ==
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* [[Drugs]] ([[vitamin A]], [[6-mercaptopurine]]
* [[Drugs]] ([[vitamin A]], [[6-mercaptopurine]]


* autoimmune and connective tissue diseases
* Autoimmune and connective tissue diseases


* systemic or intra abdominal [[infections]]
* Systemic or intra abdominal [[infections]]


* [[Clotting]] factor abnormalities
* [[Clotting]] factor abnormalities
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==Natural History, Complications and Prognosis==
==Natural History, Complications and Prognosis==
===Complications===
* [[Liver failure]]
* [[Ascites]]
* [[Encephalopathy]]
===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.


==Diagnosis==
==Diagnosis==
===Symptoms===
===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 ====
* [[Pancytopenia]]
* Elevated [[liver enzymes]]
* [[Liver function tests]]
==== Endoscopy ====
* [[Esophageal varices]]
==== Biopsy ====
* Evidence of [[sclerosis]] of portal tract


===Physical Examination===
* Delineates the portal system and helps in the identification of lesions.


===Laboratory Findings===
==Treatment==
==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]].  In considering [[spleenectomy]], the high risk of [[thrombosis]] in the [[portal venous system]] should be taken into account


==References==
==References==

Latest revision as of 05:26, 6 August 2012

Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]

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Synonyms and keywords: Hepatoportal sclerosis

Overview

Hepatoportal sclerosis (HPS) is a rare disorder characterized by sclerosis of the intrahepatic portal veins.

Pathophysiology

Pathology

Histopathologic findings are

  • Periportal fibrosis
  • Proliferation of small vascular channels within/around portal tracts

Causes

  • Autoimmune and connective tissue diseases

Differentiating from other Diseases

Differential diagnosis from liver cirrhosis is crucial and may be difficult. A large liver biopsy specimen, together with expertise and awareness of the examiner, are requested for optimal diagnosis.

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.

Natural History, Complications and Prognosis

Complications

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.

Diagnosis

Symptoms

Lethargy and in advanced stages symptoms of liver failure

Physical Examination

Eye

Abdomen

Neurologic

Laboratory Findings

Blood and Biomarker Studies

Endoscopy

Biopsy


  • Delineates the portal system and helps in the identification of lesions.

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. In considering spleenectomy, the high risk of thrombosis in the portal venous system should be taken into account

References


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