TABLE 3.

Clinical features and diagnosis of INCPH

Clinical presentation
Age/sex	Usually young to middle age with a male predilection (third to fourth decade) in Indian studies and a female predilection (fifth decade) in studies from Japan and western literature2
Hypersplenism	Pain abdomen, early satiety, postprandial fullness, cytopenias (usually asymptomatic recurrent infections are rare)
Portal hypertension	Well-tolerated episodes of VH Transient ascites (in <1/3) during VH, PVT and infection. Ascites can also be noted in patients with parenchymal extinction and those who develop cirrhosis due to recurrent transfusion-related viral hepatitis (HBV/HCV)
Noninvasive tests
Liver function and coagulation tests	Usually normal until parenchymal extinction. Mild alterations in liver enzymes and serum bilirubin can be noted
Endoscopy	Esophageal, gastric, and ectopic varices
Radiologic findings	Spleen-enlarged PV and hepatic artery-patent and enlarged. Liver-surface smooth or mildly nodular. Hypertrophy of the caudate lobe and segment IV. (In cirrhosis there is hypertrophy of caudate lobe and atrophy of segment IV) Thickened hyperechoic walls or reduced caliber or occlusive thrombosis of intrahepatic portal branches
Transient elastography	Liver stiffness lower than those with cirrhosis but higher than normal individuals, with values between 7 and 12 kPa suggestive of INCPH8,9

Abbreviations: INCPH, idiopathic noncirrhotic portal hypertension; PSVD, portosinusoidal vascular liver disorder; PV, portal vein; VH, variceal hemorrhage.