1. Friday, December 5 th, 2008

2.  The patient was appropriately resuscitated with crystalloid fluid and blood products  Emergent endoscopy showed large gastric and esophageal varices with stigmata of recent bleeding. No endoscopic therapies or biopsies were performed at the time.  Once stable, a three-phase abdominal CT with IV contrast was performed. A diagnostic test/procedure was then performed.

10. Dr. Emma Robinson

11. Dr. Gerald Villanueva Department of Medicine Division of Gastroenterology

12. Dr. Sameer Dhalla

13.  Stool Culture: Negative  Fecal Leukocytes: Negative  Stool Ova and Parasites: Negative  Hepatitis Serologies: Negative  ANA, AMA: Negative  Ceruloplasmin, anti-trypsin: WNL  Tests for Thrombophilia: All Negative  Anti-Schistosomal Antibodies: Negative  A diagnostic liver biopsy was performed

14. Dr. Cristina Hajdu

17. Findings  MINIMAL PORTAL AND LOBULAR INFLAMMATION  FOCAL PORTAL, PERIPORTAL AND PERICENTRAL VEIN FIBROSIS  MINIMAL MACROVESICULAR STEATOSIS Final Diagnosis Idiopathic Portal Fibrosis

19.  Young previously healthy man from Hong Kong with short history of heavy alcohol use presents with UGIB and hypovolemia  Anemia and Hypoalbuminemia  Clinical and radiographic evidence of portal hypertension: variceal bleed, ascites, Splenomegaly. All out of proportion to mild hepatocellular disease  No cirrhosis on CT. No venous thrombosis

20. Prehepatic Portal vein thrombosis Splenic vein thombosis Splanchnic arteriovenous fistula Splenomegaly (lymphoma, Gaucher's disease) Posthepatic IVC obstruction Cardiac disease (constrictive pericarditis, restrictive cardiomyopathy) Intrahepatic Presinusoidal Schistosomiasis Idiopathic portal hypertension/Noncirrhotic portal fibrosis/Hepatoportal sclerosis Primary biliary cirrhosis Sarcoidosis Congenital hepatic fibrosis Sclerosing cholangitis Hepatic arteriopetal fistula Sinusoidal Arsenic poisoning Vinyl chloride toxicity Vitamin A toxicity Nodular regenerative hyperplasia Postsinusoidal Sinusoidal obstruction syndrome (Veno- occlusive disease) Budd-Chiari syndrome

21. Prehepatic Portal vein thrombosis Splenic vein thombosis Splanchnic arteriovenous fistula Splenomegaly (lymphoma, Gaucher's disease) Posthepatic IVC obstruction Cardiac disease (constrictive pericarditis, restrictive cardiomyopathy) Intrahepatic Presinusoidal Schistosomiasis Idiopathic portal hypertension/Noncirrhotic portal fibrosis/Hepatoportal sclerosis Primary biliary cirrhosis Sarcoidosis Congenital hepatic fibrosis Sclerosing cholangitis Hepatic arteriopetal fistula Sinusoidal Arsenic poisoning Vinyl chloride toxicity Vitamin A toxicity Nodular regenerative hyperplasia Postsinusoidal Sinusoidal obstruction syndrome (Veno- occlusive disease) Budd-Chiari syndrome

22.  Historical 19 th century term was Banti’s Syndrome: Anemia, thrombocytopenia, splenomegaly without hematological cause  Characterized simultaneuosly in the 1960’s -India (1962): Non-Cirrhotic Portal Fibrosis -Japan (1962): Idiopathic Portal hypertension -US (1965): Hepatoportal Sclerosis  After 30 years of competing names for the same disease, the above term has been “generally” adopted

23.  Presence of portal hypertension  Absence of liver cirrhosis  Histological features of dense portal fibrosis, marked phlebosclerosis, and dilated sinusoids.

24.  Present worldwide but most focused in South Asia and East Asia, particularly Japan  Prevalence: 25-30% of non-cirrhotic portal hypertension in Asia. Dramatic decline in a more recent Japanese population survey.  Disparate Male to Female Ratios

25.  Recurrent Infection  Autoimmunity  Genetic: HLA-DR3  Hypercoagulability  HAART  Miscellaneous Toxins

26.  Variceal Bleed which is surprisingly well tolerated  Other signs of portal hypertension  Preserved Liver Function  Characteristic Hemodynamics  Characteristic Path Findings  Diagnosis of exclusion

27. Histological feature* Frequency, percent Irregular intimal thickening of portal veins75-100 Organizing thrombus and/or recanalization of portal veins 20-100 Intralobular fibrous septa95 Abnormal blood vessels in the lobules75 Subcapsular atrophy70 Dense portal fibrosis and portal venous obliteration32-52 Periductal fibrosis of interlobular bile ducts50 Portal inflammation47 Nodular hyperplasia of parenchyma25-40

29.  Few studies of IPF management exist  Acute and Prophylactic regimens for variceal bleed as with cirrhotics  TIPS and surgical anastomosis is often well tolerated  Small subgroup progress to nodular transformation of the liver with extensive subhepatic and portal fibrosis  HCC?

30.  The Patient is doing well on his previous regimen of nadolol and esomeprazole  Furosemide and Aldactone were added for ascites  He is following regularly with a gastroenterologist and has had no recurrent bleeding events since his discharge in October 2008

31. Idiopathic Portal Fibrosis Portal Hypertension Alcohol Abuse Asian Descent Raised in Endemic Area Unknown Mechanisms Gastric/Esophageal Varices complicated by recurrent UGIB Multifactorial Anemia Orthostasis Splenomegal y Mild Elevation in Alk Phos and ALT Steatosis and Mild peri- central vein fibrosis Medication non-adherence Ascites

32.  Dr. Martin Blaser  Dr. Anthony Grieco  Dr. Emma Robinson  Dr. Gerald Villanueva  Dr. Cristina Hajdu  Dr. Chirayu Gor  Dr. Christina Yoon