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Clinical Pathological Conference

Clinical Pathological Conference. Friday, December 5 th , 2008. Hospital Course. The patient was appropriately resuscitated with crystalloid fluid and blood products

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Clinical Pathological Conference

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  1. Clinical Pathological Conference Friday, December 5th, 2008

  2. Hospital Course • 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.

  3. Abdominal CT Scan:Arterial Phase

  4. Abdominal CT Scan:Portal Venous Phase

  5. Radiology Discussant Dr. Emma Robinson

  6. Faculty Discussant Dr. Gerald Villanueva Department of Medicine Division of Gastroenterology

  7. Interval History Dr. SameerDhalla

  8. Interval History • 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

  9. Pathology Discussant Dr. Cristina Hajdu

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

  11. Final Diagnosis Idiopathic Portal Fibrosis

  12. Key Clinical Elements of Case 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

  13. Idiopathic Portal Fibrosis Historical 19th 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

  14. IPF: Overview Presence of portal hypertension Absence of liver cirrhosis Histological features of dense portal fibrosis, marked phlebosclerosis, and dilated sinusoids.

  15. IPF: Epidemiology 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

  16. IPF: Etiology Unknown Recurrent Infection Autoimmunity Genetic: HLA-DR3 Hypercoagulability HAART Miscellaneous Toxins

  17. IPF: Clinical Approach Variceal Bleed which is surprisingly well tolerated Other signs of portal hypertension Preserved Liver Function Characteristic Hemodynamics Characteristic Path Findings Diagnosis of exclusion

  18. IPH: Histological Findings

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

  20. Back to the Patient…. 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

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

  22. Thank You Dr. Martin Blaser Dr. Anthony Grieco Dr. Emma Robinson Dr. Gerald Villanueva Dr. Cristina Hajdu Dr. Chirayu Gor Dr. Christina Yoon

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