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CHOLESTASIS

CHOLESTASIS. Dr Mahsa Khodadoostan Isfahan university of medical science Department of G astroentrology and Hepatology. Cholestasis is an impairment of bile formation and/or bile flow Symptoms of fatigue, pruritus and in its most overt form, jaundice.

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CHOLESTASIS

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  1. CHOLESTASIS DrMahsaKhodadoostan Isfahan university of medical science Department of Gastroentrology and Hepatology

  2. Cholestasis is an impairment of bile formation and/or bile flow • Symptoms of fatigue, pruritus and in its most overt form, jaundice. • Early biochemical markers in often asymptomatic patients • increases in serum alkaline phosphatase (ALP) • γ -glutamyltranspeptidase(γGT) • Conjugated hyperbilirubinaemia at more advanced stages. • Cholestasis • classified as intra-hepatic or extra-hepatic

  3. :Alkaline phosphatase • liver • Bones • Intestines • Pancreas • Kidneys • placenta

  4. :Gamma-glutamyltranspeptidase • hepatocytes and biliary epithelial cells • Kidney • seminal vesicles • Pancreas • Spleen • Heart • brain

  5. 5'-nucleotidase: • it is only released into serum by hepatobiliary tissue

  6. Which of these tests is not LIVER FUNCTION TEST? A: Bilirubin B: Aminotransferase ( ALT and AST) C: Albumin D: INR

  7. 1:Which of these tests in not LIVER FUNCTION TEST? A: Bilirubin B:Aminotransferase ( ALT and AST) C: Albumin D: INR

  8. 2: A 46 year old man is consulted because of incidentaly discovered elevated alkaline phosphatase, • CBC: Normal • ALT= 20, AST= 16, ALP= 610 • Bili= 1, INR= 1 Please select the next step? A: Abdominal sonography B: MRCP C: serum GGT D: AMA

  9. 2: A 46 year old man is consulted because of incidentaly discovered elevated alkaline phosphatase, CBC: Normal ALT= 20, AST= 16, ALP= 610 Bili= 1, INR= 1 Please select the next step? A: Abdominal sonography B: MRCP C:serum GGT D: AMA

  10. 3: Patient is a 52 year old lady who is admitted in ER because of RUQ pain, On PH/E she is icteric, febrile and has RUQ tenderness. • CBC : WBC= 10100 , neut= 80%, Hb= 13, Plt= 143000 • ALT= 73, AST= 60, ALP= 980, Bili. T= 3.1, D= 2.5 • INR= 1 • What do you order as the next test? • A: Abdominal sonography • B: MRCP • C: ERCP • D: GGT

  11. 3: Patient is a 52 year old lady who is admitted in ER because of RUQ pain, On PH/E she is icteric, febrile and has RUQ tenderness. • CBC : WBC= 10100 , neut= 80%, Hb= 13, Plt= 143000 • ALT= 73, AST= 60, ALP= 980, Bili. T= 3.1, D= 2.5 • INR= 1 • What do you order as the next test? • A: Abdominal sonography • B: MRCP • C: ERCP • D: GGT

  12. Abdominal sonography: • Liver has normal echotexture and intrahepatic bile ducts are dilated • CBD was measured up to 11mm in midpart and contained a 10mm stone in distal part • Gallbladder has normal wall thickness and contained two small stones • What’s the next step ? • A: MRCP • B: ERCP then cholecystectomy • C: Cholecystectomy and intraoperative CBD exploration • D: Cholecystectomy then ERCP

  13. Liver has normal echotexture and intrahepatic bile ducts are dilated • CBD was measured up to 11mm in midpart and contained a 10mm stone in distal part • Gallbladder has normal wall thickness and contained two small stones • What’s the next step ? • A: MRCP • B:ERCP then cholecystectomy • C: Cholecystectomy and intraoperative CBD exploration • D: Cholecystectomy then ERCP

  14. 4:Patient is a 56year old man who is referred because of icter, pruritus, abdominal pain and weight loss. • CBC: Normal • ALT= 70, AST= 67, ALP= 1090, Bili= 9 • What is the next step? • A: Abdominal sonography • B: MRCP • C: ERCP • D: GGT

  15. Patient is a 56year old man who is referred because of icter, pruritus, abdominal pain and weight loss. • CBC: Normal • ALT= 70, AST= 67, ALP= 1090, Bili= 9 • What is the next step? • A:Abdominal sonography • B: MRCP • C: ERCP • D: GGT

  16. Abdominal sonography: • Dilated CBD without stone or sludge inside • What’s the next test? • A: MRCP+ MRI • B: Abdominal CT scan • C: Endoscopic ultrasonography • D: All of them

  17. Dilated CBD without stone or sludge inside • What’s the next test? • A: MRCP+ MRI • B: Abdominal CT scan • C: Endoscopic ultrasonography • D: All of them

  18. 6:Patient is a 30 year old man who is referred because of pruritus • ALT= 50, AST= 35, ALP= 890, Bili= 2 • On sonography there was no intra or extrahepatic bile duct ectasia • What is the next step? • A: MRCP • B: ERCP • C: AMA • D: EUS

  19. Patient is a 30 year old man who is referred because of pruritus • ALT= 50, AST= 35, ALP= 890, Bili= 2 • On sonography there was no intra or extrahepatic bile duct ectasia • What is the next step? • A: MRCP • B: ERCP • C: AMA • D: EUS

  20. AMA was negative • What do you order as the next test? • A: MRCP • B: ERCP • C: Biopsy • D: EUS

  21. What do you order as the next test? • A:MRCP • B: ERCP • C: Biopsy • D: EUS

  22. Primary sclerosing cholangitis • multifocal strictures, segmental dilations)

  23. What’s the next diagnostic test in this PSC patient? • A: Upper gi endoscopy • B: Colonoscopy • C: Liver biopsy • D: Abdominal CT scan

  24. What’s the next diagnostic test in this patient? • A: Upper gi endoscopy • B:Colonoscopy • C: Liver biopsy • D: Abdominal CT scan

  25. Primary Sclerosing Cholangitis

  26. Definition • A chronic inflammatory cholestaticdisease • Progressive destruction of bile ducts • May progress to cirrhosis • etiology unknown

  27. Patients with PSC may be asymptomatic and diagnosed as part of the evaluation of abnormal laboratory tests, or they may have symptoms such as fatigue and pruritus. • Physical examination may reveal jaundice, hepatomegaly, splenomegaly, and excoriations, though it is often normal

  28. complications • Fat-soluble vitamin deficiencies (A, D, E, and K) • Metabolic bone disease • Dominant biliary strictures • Cholangitis and cholelithiasis • Cholangiocarcinoma • Gallbladder cancer • Hepatocellular carcinoma (in patients with cirrhosis) • Colon cancer (in patients with concomitant ulcerative colitis)

  29. Relationship to IBD • IBD in 60-90% of PSC patients • UC more common than Crohn’s (2:1) • PSC in Crohn’s disease almost always involves the colon • 2-10% of UC patients have PSC

  30. There are two major goals of treatment in primary sclerosing cholangitis (PSC): • Retardation and reversal of the disease process • Management of progressive disease and its complications

  31. A variety of immunosuppressive and anti-inflammatory agents have been studied in patients with PSC, but none has shown a consistent benefit on overall or transplant-free survival • a role for any medical therapy is unproven

  32. In patients who are already taking UDCA, we suggest stopping UDCA but reinstituting it at standard doses (13 to 15 mg/kg per day in divided doses) if: • they develop worsening pruritus or jaundice

  33. given the uncertainty regarding its benefits, an alternative approach is to start (or continue) UDCA in patients who want to take it despite the uncertain benefits • After six months, if the alkaline phosphatase normalizes or is decreased by at least 40 percent, or if the patient experiences symptomatic improvement, UDCA can be continued. Otherwise, it is stopped

  34. We suggest that patients with a dominant stricture and pruritus and/or cholangitis undergo endoscopic therapy to dilate and/or stent the stricture • Long-term prophylactic antibiotics are indicated for patients with recurrent cholangitis despite efforts to treat a dominant stricture

  35. Mild pruritus: warm baths and emollients may help. Antihistamines may also be helpful • Moderate to severe pruritus: • initially treat with a bile acid sequestrant such as cholestyramine (total daily dose of 4 to 16 grams) • does not provide adequate, relief we switch to rifampin (150 to 300 mg twice daily) • If symptoms persist, we then switch to an opioid antagonist, such as naltrexone (12.5 to 50 mg/day) • Switching to sertraline (75 to 100 mg daily) or phenobarbital 90 mg at bedtime can be tried if other measures fail

  36. 6:Patient is a 56 years old lady who is referred because of icter and pruritus since 2 years ago • CBC: WBC= 4200, HB= 12.5, PLt= 98000 • ALT= 32, AST= 40, ALP= 1400 • Bili.T= 4 , Bili.D= 3.5, INR= 1.5 , Alb= 3.2 • On physical examination she is icteric and has splenomegaly

  37. What’s the most common diagnosis: • A: Pancreatic cancer • B: CBD stone • C: PBC • D: PSC

  38. What’s the most common diagnosis: • A: Pancreatic cancer • B: CBD stone • C: PBC • D: PSC

  39. Primary biliary cholangitis (PBC) is rare,19 to 402 cases per million persons • The vast majority of patients (90 to 95 percent) are women • most patients are diagnosed between the ages of 30 and 65 years

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