ALB and Hermansky-Pudlak syndrome: Evidence for HPS was recorded, to include all of the following criteria: (1) hypoxemia (Pa02 < 80 mmHg), (2) portal hypertension (any or all of the following: esophageal and/or gastric varices, high serum-ascitic fluid albumin gradient, ascites, portosystemic collaterals, and splenomegaly), and (3) IPVD related intrapulmonary shunting confirmed by contrast echocardiogram or Tc-99m macroaggregated albumin (MAA) lung perfusion scan (6).