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'''Portosystemic shunts (PSS)''' are anomalous vascular connections between the portal and systemic venous systems.  These vessels shunt blood from the '''hepatic portal vein''' (deriving from the [[Alimentary - Anatomy & Physiology #Stomach|stomach]], [[Alimentary - Anatomy & Physiology #Small Intestine|intestines]], [[Pancreas - Anatomy & Physiology|pancreas]] and [[Spleen - Anatomy & Physiology|spleen]]) directly into '''systemic venous system''', bypassing the [[Liver - Anatomy & Physiology|liver]].
 
'''Portosystemic shunts (PSS)''' are anomalous vascular connections between the portal and systemic venous systems.  These vessels shunt blood from the '''hepatic portal vein''' (deriving from the [[Alimentary - Anatomy & Physiology #Stomach|stomach]], [[Alimentary - Anatomy & Physiology #Small Intestine|intestines]], [[Pancreas - Anatomy & Physiology|pancreas]] and [[Spleen - Anatomy & Physiology|spleen]]) directly into '''systemic venous system''', bypassing the [[Liver - Anatomy & Physiology|liver]].
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Portosystemic shunts may be '''congenital''' or '''acquired''' in disease the cause portal hypertension.  Congenital shunts represent approximately 70% of the total number diagnosed in dogs and constitute the majority of those diagnosed in cats.  Congenital shunts usually involve a single (or occasionally double) anomalous vessel which may be located outside of the hepatic parenchyma (extrahepatic) or within it (intrahepatic).  Extrahepatic shunts accounts for 63% of single shunts in the dog and they are most commonly found in miniature and toy breeds.  Intrahepatic shunts are usually located within the left lobes of the liver and occur due to persistence of the foetal [[Foetal Circulation - Anatomy & Physiology|ductus venosus]].  This form of shunt is most common in large breed dogs and patent ductus venosus is an inherited condition in Irish wolfhounds.  Intrahepatic shunts running through the central or right liver lobes are recognised and these may have a different embryological origin.
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Portosystemic shunts may be '''congenital''' or '''acquired''' in disease the cause portal hypertension.  In the developing embryo, the cardinal veins form the systemic abdominal veins (the caudal vena cava, azygos, renal and gonadal veins) whereas the vitelline veins form the hepatic sinusoids, the portal vein and its tributaries, of which the largest are the left gastric, splenic, cranial and caudal mesenteric and gastroduodenal veins.  The ductus venosus, a component of the foetal circulation that directs blood directly from the ubilical vein to the vena cava, is also part of the vitelline system.  If a functional connection (ductus venosus or otherwise) remains between the vitelline and cardinal systems after birth, a PSS develops.  However, in the normal animal, there are numerous non-functional connection between the two systems that may open if the pressure in the portal vein rises, leading to the formation of multiple extrahepatic acquired shunts. 
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Congenital shunts represent approximately 70% of the total number diagnosed in dogs and constitute the majority of those diagnosed in cats.  Congenital shunts usually involve a single (or occasionally double) anomalous vessel which may be located outside of the hepatic parenchyma (extrahepatic) or within it (intrahepatic).  Extrahepatic shunts accounts for 63% of single shunts in the dog and they are most commonly found in miniature and toy breeds.  Intrahepatic shunts are usually located within the left lobes of the liver and occur due to persistence of the foetal [[Foetal Circulation - Anatomy & Physiology|ductus venosus]].  This form of shunt is most common in large breed dogs and patent ductus venosus is an inherited condition in Irish wolfhounds.  Intrahepatic shunts running through the central or right liver lobes are recognised and these may have a different embryological origin.
    
Acquired shunts represent approximately 20% of those diagnosed in dogs and they often consist of multiple small vessels.  They arise due to portal hypertension, following an increased resistance to portal blood flow.  The increased pressure in the portal vein and its tributaries causes numerous non-functional microvascular communications with the systemic venous system to open.  The causes of portal hypertension are numerous and they may be divided into pre-hepatic, hepatic and post-hepatic:
 
Acquired shunts represent approximately 20% of those diagnosed in dogs and they often consist of multiple small vessels.  They arise due to portal hypertension, following an increased resistance to portal blood flow.  The increased pressure in the portal vein and its tributaries causes numerous non-functional microvascular communications with the systemic venous system to open.  The causes of portal hypertension are numerous and they may be divided into pre-hepatic, hepatic and post-hepatic:
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**'''Right-sided congestive heart failure''', including '''cardiac tamponade'''.
 
**'''Right-sided congestive heart failure''', including '''cardiac tamponade'''.
 
*'''Hepatic'''
 
*'''Hepatic'''
**'''Acute fulminant hepatitis''', '''[[Hepatic Lipidosis|hepatic lipidosis]]''' or '''[[Hepatic neoplasia|neoplasia]]'''
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**'''Acute fulminant hepatitis''', '''chronic hepatic failure with fibrosis''' or '''[[Hepatic neoplasia|neoplasia]]'''
 
*'''Post-hepatic'''
 
*'''Post-hepatic'''
 
**'''[[Thrombosis|Thrombosis of the portal vein]]''', '''hepatic arteriovenous fistulae''' and '''congenital hypoplasia of the portal vein.'''
 
**'''[[Thrombosis|Thrombosis of the portal vein]]''', '''hepatic arteriovenous fistulae''' and '''congenital hypoplasia of the portal vein.'''
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The pathophysiology of PSS relates to the shunting of blood directly from the systemic circulation, resulting in hyperammonaemia and [[Hepatic Encephalopathy]].
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The pathophysiology of PSS relates to the shunting of portal blood directly from the systemic circulation, resulting in hyperammonaemia and [[Hepatic Encephalopathy|hepatic encepalopathy]].  Animals with PSS also have reduced function of the hepatic component of the monocyte phagcoyte system (chiefly the Kupffer cells) and they often develop bacteraemia.  Gram negative bacteria deriving from the GI tract are involved most commonly.
    
==Signalment==
 
==Signalment==
*Relatively common in dog, especially small breed dogs
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Congenital portosystemic shunts are diagnosed regularly in dogs under one year of age, although they may be ten years old or more at presentation.  Extrahepatic shunts occur most commonly in dogs of the small or toy breeds, including Shih tzus, West Higland white terriers, Maltese terriers and Yorkshire terriers.  Intrahepatic shunts are much less common and occur in larger breeds such as Irish wolfhounds (in which patent ductus venosus is thought to be inherited), golden retrievers and Australian cattle dogs.  The condition occurs occasionally in cats, horses, cows and pigs.
*Purebred dogs are more at risk
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*Occasionally seen in cats, horses, cows and pigs
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Acquired shunts occur in animals with portal hypertension, of which the most common causes are [[Hepatitis, Chronic|chronic hepatitis]] in dogs and cholangitis in cats.
    
==Diagnosis==
 
==Diagnosis==
 
===Clinical Signs===
 
===Clinical Signs===
*Young animals, usually under 1 years of ages, but can sometimes be up to 10 years or older
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All portosystemic shunts are likely to cause [[Hepatic Encephalopathy|hepatic encephalopathy]](HE) and PSS is the major cause of this diseaseHE mainly causes waxing and waning neurological signs, including central blindness, seizures, depression and bizarre behaviour.  Cats often show hpersalivation/ptyalism.
*Failure to thrive, small body stature or weight loss
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*Waxing and waning neurological signs due to [[Hepatic Encephalopathy|hepatic encephalopathy]].  These are usually most severe an hour or two post prandial but this may not be obvious in all cases
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In additon, animals with congenital PSS may show the following signs:
**Bizarre behaviour
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*'''Failure to thrive''', small body size or weight lossIt may be useful to compare the animal to its littermates to assess whether it is stunted.
**[[Forebrain Disease - Pathology #Circling, Head Pressing, Compulsive Walking|Head pressing]]
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*'''Urinary tract signs''', including dysuria, stranguria, haematuria and pollakiuria.  These signs occur because an increased blood ammonia concentration decreases the ability of enzymes to convert [[Urate Metabolism - Pathology|uric acid]] to allantoin, resulting in urate urolithiasis and urethral obstruction.  Ammonium biurate are the most common uroliths to be formed.
**[[Forebrain Disease - Pathology #Seizures|Seizures]]
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*'''Polyuria and polydipsia''' occur for reasons that are not fully understood.  Urea synthesis is reduced in animals with PSS (because ammonia is not taken up by the liver in the normal way) and a reduction in the urea concentration of the renal medulla reduces the ability of the kidney to concentrate urine.  The hypofunctional liver probably also degrades cortisol at a reduced rate, increasing the circulating concentration of this diuretic hormone. 
**Intermittent blindness.   
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*Intermittent '''gastro-intestinal signs''' such as [[Stomach and Abomasum Consequences of Gastric Disease - Pathology|vomiting]] and [[Diarrhoea|diarrhoea]].
*Ptyalism in cats
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*'''Ascites''' may occur due to [[Hypoalbuminaemia|hypoalbuminaemia]].
*Dysuria, stranguria, haematuria, pollakiuria and urethral obstruction
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*'''Bleeding tendencies''' due to coagulopathy, but this condition is often subclinical.
**An increase in ammonium concentration in the blood decreases the ability of enzymes to convert [[Urate Metabolism - Pathology|uric acid]] to allantoin, thereby resulting in urate urolithiasis.
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*Cats may develop '''copper-coloured irises''' because they do not produce the normal yellow/green pigments in their livers.
*Intermittent [[Stomach and Abomasum Consequences of Gastric Disease - Pathology|vomiting]] or [[Diarrhoea|diarrhoea]]
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*Animals may have other '''congenital abnormalities''', including [[Hernia|hernias]] and cryptorchidism.
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Animals suffering from primary liver disease with acquired PSS may show signs associated with the underlying disease, including [[Icterus|icterus]], hepatomegaly, pain on abdominal palpation, inappetance and polyuria and polydipsia.  Such animals may also show ascites due to portal hypertension and this may be a possible source of confusion as the same sign is seen with congenital PSS.
    
===Laboratory Tests===
 
===Laboratory Tests===
 
====Haematology====
 
====Haematology====
*Microcytosis
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Animals with congenital PSS often have '''microcytic normochromic red blood cells''' due to sequestration of iron.  '''Leucocytosis''' may occur as a response to bacteraemia due to a reduction hepatic phagocytic activity.
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Animals with inflammatory liver disease may have '''leucocytosis''' of various types and may have '''acanthocytic''' red blood cells.
    
====Biochemistry====
 
====Biochemistry====
*Decreased blood urea nitrogen (BUN)
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The following features might be expected on a biochemical profile of an animal with a congenital PSS:
*Hypoalbuminaemia
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*'''Decreased serum urea concentration''', due to reduced hepatic synthesis
*Hypocholesterolaemia
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*'''[[Hypoalbuminaemia]]''' due to reduced synthesis.
*Hypoglycaemia
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*'''Hypocholesterolaemia''' due to disruption of the entero-hepatic circulation so that cholesterol must be used constantly to synthesise new bile acids because those returning from the GI tract cannot be extracted by the liver in sufficient quantities.
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*'''Hypoglycaemia''', which often occurs with acute HE.
    
====Other Tests====
 
====Other Tests====
*Increased postprandial ± preprandial [[Liver - Anatomy & Physiology #Bile Acids|bile acids]]
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*'''Increased postprandial ± preprandial [[Liver - Anatomy & Physiology #Bile Acids|bile acid]]''' concentrations.  Bile acids are normally removed from the portal blood by the liver but, in an animal with PSS, they enter the systemic venous system directly.  If the preprandial level is elevated beyond ~100 umol/l, a bile acid stimulation test will not achieve any higher sensitivity for the detection of PSS.  A bile acid stimulation test is performed by measuring preprandial bile acids, feeding the animal and measureing post prandial bile acids two hours later.  The preprandial sample may occasionally be higher than the postprandial if a gall bladder contraction occurred just before the first sample was taken.
*Increased ammonia levels
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*'''Increased blood ammonia concentration''' is diagnostic of PSS for the same reasons as are elevated bile acids.  An ammonia tolerance test can be performed by administering ammonium chloride (or a small meal) to a fasted animal and measuring the change in blood ammonia level.  Samples must be frozen immediately after collection as continued red blood cell metabolism will artificially raise ammonia levels in stored samples.
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*'''Reduced serum iron concentration''' due to sequestration.
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*'''Clotting times''' can be measured in animals where a coagulopathy is suspected.
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*'''Analysis of urine''' will show that it has a low specific gravity and cytological examination may reveal the presence of urate crystals which resemble 'thorn apples' or 'mites'.
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===Diagnostic Imaging===
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====Radiography====
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A definitive diagnosis relies on visualisation of the shunting blood vessel but radiography may reveal changes that are supportive of a diagnosis of PSS:
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*'''Renomegaly''' is thought to occur as the kidneys attempt to remove and metabolise ammonia and
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*'''Microhepatica''' probably occurs because the liver is deprived of growth factors from the pancreas and other abdominal organs which are usually carried to it in the portal blood, for example the somatomedins (insulin-like growth factors, IGFs).
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*'''Urate uroliths''' are '''radiolucent''' and will not be detected by radiographs.
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===Diagnostic Imaging===
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====Ultrasonography====
A definitive diagnosis relies on visualisation of the shunting blood vessel.  This may be done with either ultrasonography or contrast portography or at surgery.
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The shunting vessel may be identified by ultrasound scan, especially if a '''colour flow Doppler''' facility is available.  Congenital shunts are usually thin-walled, tortuous vessels with '''turbulent flow''' and it should be noted that they may run between the tributaries of the portal vein and the azygos vein, rather than the portal vein itself.  Acquired shunting vessels can often be located around the cranial pole of the right kidney.  It may require some skill to find a shunting vessel.   
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Scans of the urinary bladder and urethra may reveal the presence of '''urate uroliths''' and '''thickening of the bladder wall''' due to cystitis.
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====Positive Contrast Portography====
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The gold standard technique for diagnosis of PSS is mesenteric portovenography, which involves catheterisation of a mesenteric vein and injection of a water soluble iodine-containing contrast medium into the portal circulation.  Radiographs are then made to follow the course of the medium through the portal system and liver.  The medium can be injected percutaneously into the spleen (under ultrasound guidance) in a less invasive variant of this technique but the spleen may overly the location of the shunt in the subsequent radiographs.
    
==Treatment==
 
==Treatment==
*Surgical ligation of the anomalous vessels
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In animals with acquired PSS, the underlying cause should be treated and HE should be managed as described [[Hepatic Encephaolopathy|here]].  Acquired shunts should never be ligated as they occur as a compensatory response to portal hypertension.  Ligation would increase portal pressure further with negative consequences for the animal.
**Portal hypertension is possible post ligationFor this reason, a partial ligation is performed initally, followed by a complete ligation a few months later.
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*Medical treatment of [[Hepatic Encephalopathy #Medical Management|Hepatic Encephalopathy]]
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Animals affected by congenital PSS may be managed either medically or surgically but a recent study has shown that those undergoing surgical ligation of the shunting vessel have a longer median survival time.  Medical management is often employed in those animals that show few clinical signs on presentations, are older or which have a shunt that is not amenable to ligation. 
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===Medical Management===
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A suitable regime would incorporate the following features:
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*A '''diet''' with '''protein of high quality''' and with both soluble and insoluble fibre.  Severe protein restriction is only necessary in animals showing signs of HE and it is otherwise detrimental because animals with PSS are often hypoalbuminaemic.  A protein of high quality is selected so that excess amino acids are not available to colonic bacteria and a suitable choice would be cottage cheeseSoluble fibre acts in a similar manner to lactulose (below) whereas insoluble fibre decreases intestinal transit time and helps to prevent constipation.
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*'''Lactulose''', a synthetic disaccharide that causes acidification of the colonic environment when it is fermented by bacteria.  This environment promotes the conversion of ammonia to ammonium and the latter ions are not easily absorbed due to their electrical charge.  The trapped ammonia is therefore excreted in faeces.  Lactulose is also an osmotic laxative that reduces the amount of time available for colonic bacteria to act on surplus amino acids.
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*Administration of '''oral antibiotics''' to reduce the numbers of colonic bacteria.  Neomycin, ampicillin or metronidazole are commonly used for this purpose.
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===Surgical Ligation===
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This is the treatment of choice for congenital PSS but it may cause adverse effects in the post-operative period.  Consideration must also be given to the choice of anaesthetic drugs as animals with PSS have reduced ability to metabolise drugs in the liver and often undergo prolonged recoveries from sedation or anaesthesia.
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====Anaesthesia====
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====Surgical Procedure====
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=====Extrahepatic Shunts=====
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=====Intrahepatic Shunts=====
    
==Prognosis==
 
==Prognosis==
Excellent prognosis in dog for resolution of clinical signs after total surgical ligation.  However, the response of cat to surgical intervention in cats is less promising than in dogs.
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The prognosis for outcome depends on the age of presentation and the urea concentration at presentation, with older dogs with higher levels of urea having better outcomes.  Dogs usually respond very well to total surgical ligation if they do not experience severe adverse effects in the week following the procedure but the response in cats is less favourable.
 
      
==References==
 
==References==
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==From Pathology==
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*seen in dogs and cats
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*Inherited in Irish wolfhounds
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**Not known what mode of inheritance in this breed
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*these are vessles that allow the blood in the portal vein to bypass the [[Liver - Anatomy & Physiology|liver]] tissue (parenchyma)
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*congenital
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**shunting from the portal vein directly into the vena cava, azygos or renal vein
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**this is the common type seen in small dogs and cats - usually a single communication between the vessels, occasionally multiple
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**larger breeds tend to have the shunting to the vena cava take place within the [[Liver - Anatomy & Physiology|liver]] itself (persistent ductus venosus)
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*acquired
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**due to hepatic fibrosis whcih results in increased resistance of flow of blood into the [[Liver - Anatomy & Physiology|liver]] from the portal vein
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**produces hypertension in the portal vein and fluid accumulates in the peritoneal cavity - '''ascites'''
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**several thin-walled tortuous vessels may be seen connecting the mesenteric veins to the vena cava, and the [[Liver - Anatomy & Physiology|liver]] looks atrophic and fibrosed
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*Bacteraemia is a common finding in severe hepatic disease and PSS in humans
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**portal or systemic
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**usually Gram-negatives
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**also seen in dogs with PSS
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**presumably due to reduced effectiveness of phagocytic activity in these [[Liver - Anatomy & Physiology|livers]]
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**or due to shunting of blood around the liver
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NB: portosystemic shunt is a major cause of hepatic encephalopathy (need link), therefore the affected animals are stunted and seem dull or stupid because of the toxic substances in their systemic circulation
   
[[Category:Liver_-_Developmental_Pathology]]
 
[[Category:Liver_-_Developmental_Pathology]]
 
[[Category:Liver_-_Circulatory_Disturbances]]
 
[[Category:Liver_-_Circulatory_Disturbances]]
    
[[Category:To_Do_-_James]]
 
[[Category:To_Do_-_James]]
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