Obliterative portal venopathy: Difference between revisions
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'''Hepatoportal sclerosis (HPS)''' is a rare disorder characterized by sclerosis of the intrahepatic portal veins | '''''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 | |||
* [[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 ([[arsenic]] or [[vinyl chloride]]) | |||
* [[Drugs]] ([[vitamin A]], [[6-mercaptopurine]] | |||
* Autoimmune and connective tissue diseases | |||
* Systemic or intra abdominal [[infections]] | |||
* [[Clotting]] factor abnormalities | |||
== 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=== | |||
* [[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== | |||
===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 | |||
* 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== | |||
{{reflist|2}} | |||
{{WH}} | |||
{{WS}} | |||
[[Category:Disease]] | |||
[[Category:Grammar]] | |||
Latest revision as of 05:26, 6 August 2012
Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]
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
- Sclerosis of the portal venous system
- Proliferation of small vascular channels within/around portal tracts
Causes
- Exposure to toxic substances (arsenic or vinyl chloride)
- Autoimmune and connective tissue diseases
- Systemic or intra abdominal infections
- Clotting factor abnormalities
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
- Ascites
- Spleenomegaly
- Signs of portal hypertension
Neurologic
Laboratory Findings
Blood and Biomarker Studies
Endoscopy
Biopsy
- Evidence of sclerosis of portal tract
- 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