TABLE 1.
Difference between Eastern and Western noncirrhotic PHs
| Variables | Eastern | Western |
|---|---|---|
| Nomenclature | INCPH/NCPF | IPH/PSVD |
| Sex dominance | Male > female | Female > male |
| Age | Third decade | Fifth decade |
| Symptoms and signs of portal hypertension | Always present | May or may not be present |
| Usual presentation | Well-tolerated variceal bleed | Anemia, splenomegaly, or incidentally diagnosed when evaluated for abnormal liver function tests |
| Obliterative portal venopathy | 70% | 80% |
| Nodular regenerative hyperplasia | 18% | 10% |
| Cause | Usually unknown | Known trigger |
| Concomitant autoimmune disease | Rare | Common |
| Coexisting liver disease | Rare | Common |
| Elevated liver chemistries | Uncommon | Common |
| Ascites | Transient during variceal bleed | Common and sign of worsening disease |
| Anorectal varices | Uncommon but seen | Unknown |
| Nonvariceal portosystemic collaterals | More frequent | Less frequent |
| HVPG | Normal-slightly high (upto 8 mm Hg) | Normal |
| Mortality | Usually liver related | Related to underlying disease > liver related |
Abbreviations: INCPH, idiopathic noncirrhotic portal hypertension; IPH, idiopathic portal hypertension; NCPF, noncirrhotic portal fibrosis; PH, portal hypertension; PSVD, portosinusoidal vascular disorder.