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home - Liver - Vascular Problems - Portal Hypertension Written by Dr Sebastian Zeki
Knowledge


Understands the risk of variceal bleeding as a complication of with
portal hypertension


Knows risk of variceal haemorrhage in cirrhotics who have not bled

Knows risk of bleeding related to variceal size endoscopic findings
and severity of liver dysfunction


Knows range of therapeutic options (both endoscopic and
pharmacological).

Skills
Recognises and can treat portal hypertension.
Behaviours
Manages patients with oesophageal varices with skill and
compassion

Able to convey the serious risks to patients and their relatives.

Also...

Knowledge


Recognises and shows understanding of vascular liver disease
including Budd-Chiari syndrome veno-occlusive disease and
portomesenteric venous thrombosis; understands the underlying
anatomy and physiology of these often complex conditions

Aware of need for investigation for associated myeloproliferative and
procoagulant conditions

Understands the role of anticoagulation and indications for further
intervention including TIPS surgery or transplantation

Skills
Can make careful clinical of these conditions and has
heightened awareness of liver vascular disease in differential
diagnosis

Able to make a potentially difficult diagnosis of less common variants
of vascular conditions

Behaviours
Shows ability to keep patient and relatives informed and to refer
appropriately for specialist management

Portal Hypertension

Written by Dr Sebastian Zeki Staging —A four-stage classification system for IPH has been proposed based upon the evaluation of liver size (by imaging), subcapsular parenchymal atrophy (by portogra-phy) and presence of portal vein thrombosis (by Doppler ultrasound).Stage IV requires the presence of portal vein thrombosis, which has been associated with a poor prognosis.The clinical utility of this staging system needs to be validated in further studies. Aetiology: Clinical presentations:Portal hypertension with preserved liver function.Massive splenomegaly (>10 cm below the left subcostal margin)-present in >95 % of patients.Dilated superficial abdominal veins- in15 %.Mild hepatomegaly (<4 cm below the right subcostal margin)- in 50%. Eggs S. hematobium S. mansonInf. mesentery v.Predom Egypt/ Middle East/ Africa/ S. America S. japonicums. mesentery vPredom AsiaCure rate 70% Most common causes of noncirrhotic portal hypertension in the world. =Portal hypertension without cirrhosis, with histological features of dense portal fibrosis, marked phlebosclerosis, and dilated sinusoids. Idiopathic portal hypertension (noncirrhotic portal fibrosis, hepatoportal sclerosis) Treatment of schistosomiasisPraziquantel and oxamniquine can be used.Cure rates -80 % for S. mansoni and 70 % for S. japonicum.If chronic worms may no longer be laying eggs so no further treatment needed.Also treat the consequences of portal hypertension. Diagnostic tests:Schistosomal ova-can be found in the stool.Biopsies of the rectal mucosa or the liver.Various immunologic assays. S. japonicum worldwide distribution esp Asia.S. mansoni is endemic in Egypt, Africa, Middle East, and South America.Two other species (S. mekongi and S. intercalatum) also cause hepatic infections in endemic areas in southeast Asia and western Africa, respectively. Schistosomiasis Pathophysiology 6.The entrapped ova in the small portal venules elicit an immune response causing increased production of collagen, granuloma formation, and eventually lead to portal fibrosis over time.5. S. japonicum can produce more ova than S. mansoni so get more severe liver disease. 4. Others flow into the portal vein tributaries and become trapped in the terminal portal venules, where they incite chronic inflammation which is subsequently followed by marked fibrosis.3. Some of these eggs pass through the intestinal mucosa and are excreted in the urine or feces to continue their life cycle. 2.Adult worms eventually find their way to inhabit tributaries of the inferior (S. mansoni) or superior (S. japonicum) mesenteric veins, respectively, where they produce several hundred to thousand eggs per day for several years before the end of their lifespan. 1. Schistosomal cercariae enter the body through the skin. Portal Hypertension OverviewPortal pressure = Portal venous flow x portal venous outflow resistance= HVPG >5 mmHg.Portal hypertension classification: prehepatic, intrahepatic (subcategorised as presinusoidal, sinusoidal, or postsinusoidal ), or posthepatic based upon the site of obstruction to flow.Presinusoidal portal hypertension is caused by obstruction to flow through the portal venous system in the extrahepatic portion of the portal vein (extrahepatic presinusoidal portal hypertension) or at the level of portal vein branches within the liver (intrahepatic presinusoidal portal hyperten-sion). Epidemiology : Common in Asia- accounts for20 % of all portal hypertension in India. Recurrent infections — Portal pyemia and pylephlebitis( cause vascular endothelial injury, microthrombosis, sclerosis, and obstruction of small and medium-sized portal venules).Genetic predisposition — Possibly HLA-DR3 related.Hypercoagulability — 50% have hypercoagulable state.HIV infection — or HAART related. Diagnosis of Idiopathic PHTNA common denominator of the morphological alterations associated with IPHTN is occlusive venopathy that may lead to an impairment in the intrahepatic portal perfusion and parenchymal atrophy.Other typical histological findings include an characterized by irregular sclerosis of the portal vein wall, fibrosis of the portal tract, obliteration of small portal venules (occlusive venopathy), dilatation of sinusoids due to increased portal pressure, and emergence of new aberrant portal channels.Uncommon features include pseudonodules, piecemeal necrosis, and regenerative activity. Idiopathic PHTN managementAs per management of portal hypertension Clinical features of schistosomiasisIt acutely presents with eosinophilia and features resembling acute bacterial infection.It chronically presents with presinusoidal portal hypertension Unknown but possibilities include...... Differential:Chronic ingestion of arsenic, exposure to vinyl chloride monomers, copper sulphate, hypervitaminosis A. Haemodynamics —The intrasplenic and portal vein pressures are markedly elevated in patients with IPH. Demonstration of such communication during hepatic venography is supportive of the presence of IPH.

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