A CASE OF PORTAL HYPERTENSION

• A 71-year-old Hispanic male with a past medical

history of hypertension (on lisinopril 10 mg daily
for ten years) and hepatic amyloidosis was
evaluated for ascites. Over five years, his alkaline
phosphatase and bilirubin levels trended up from
605 to 983 IU and1.7 to 5.5 g/dl respectively. A
liver workup including imaging studies did not
show any other causes of liver disease; however,
moderate ascites was present.
•
 A case of portal hypertension
• A progression of amyloid deposition in the
hepatic arterial wall, space of Disse, and portal
tracts was noted. Moreover, nodular
regenerative hyperplasia (NRH) without
cirrhosis, progressive narrowing of hepatic
artery and absence of bile ducts on review of
18 portal tracts was documented five years
later on the second liver biopsy.
 A case of portal hypertension
• A right hepatic venography revealed right
atrial, free hepatic vein and hepatic vein
wedge pressures at 5, 5 and 22 mm Hg
respectively, with a hepatic venous gradient of
17 mm Hg. An echocardiogram was normal.
Esophageal variceal banding was done for
grade 4 varices and he was referred for a liver
transplantation.
 I. synthetic
Plasma protein (albumin) Hypoproteinimea → oedema

Coagulants Haemorrhagic disorders

Enzymes Hepatocellular disorders
Urea / removal of NH3 ↓ bld urea, ↑bld NH3

II. Metabolic
Carbohydrate ↓ glycogen – more damage
↓ bld. Glucose – muscle weakness, personality changes,
tremors, slurred speech, convulsion, coma , death → pre
hepatic coma

Protein metabolism ↑ blood ammonia – aminoaciduria

lipid metabolism Acc. Of FA in liver → fatty liver →pre hepatic hepatitis→
fibrosis→ cirrhosis→ ↑ portal pressure→ portal hypertension
 Extra-hepatic Portal venous
obstruction
• A distinct vascular disorder of the liver
characterized by obstruction of the
extrahepatic PV with or without involvement
of intrahepatic PV radicles or splenic or
superior mesentric veins
• Excludes all cases of acute and chronic portal
vein thrombosis
• Isolated splenic or SMV thrombosis is also not
included
 Obliterative Venopathy
(OPV)/Hepatopetal sclerosis

• Liver biopsy longer than 1 cm and containing more

than 5 complete portal tracts; more than 66% of the
complete portal tracts harboring thickening of the
vein’s smooth muscle wall
• Thrombi, perhaps largely composed of platelet
aggregates formed in the portal venous circulation or
spleen, embolize to the liver and results in obliterative
vascular lesions. Atrophy and regenerative nodule
formation occur in response to the interruption of the
portal blood supply.
 Idiopathic portal hypertension
• Idiopathic portal hypertension in Japan;
associated with immunological abnormalities;
frequently ANA positive
• Criteria include splenomegaly, normal LFT,
minimal bone marrow colloid uptake, patent
portal and hepatic veins, WHVP not as high as
cirrhosis, no evidence of cirrhosis, marked portal
fibrosis and elevated portal vein pressure;
thrombosis not the etiology instead portal vein
fibrosis is seen
 Cells in the liver
• Hepatocytes
• Kupfer cells
• Sinusoidal cells
• NK cells
• Hepatic stellate cells (ito cells/vitamin A
storage cells)
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