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Pancreatic and biliary disease

Pancreatic and biliary disease. Intrahepatic cholestasis of pregnancy. Presents with sometimes intense pruritis Functional disorder of bile secretion ALP mod high, Bili high, Transaminases <200, NORMAL FUNCTIONAL TESTS, NORMAL PLTS, NORMAL COAGS

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Pancreatic and biliary disease

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  1. Pancreatic and biliary disease

  2. Intrahepaticcholestasis of pregnancy • Presents with sometimes intense pruritis • Functional disorder of bile secretion • ALP mod high, Bili high, Transaminases <200, NORMAL FUNCTIONAL TESTS, NORMAL PLTS, NORMAL COAGS • Watch for fat malabsorbtion, vitamin deficiencies (decreased bile salts)

  3. Acute Pancreatitis • Causes: • EtOH and Gallstones • 75% of people with negative RUQ u/s have sludge or micoliths • Drugs: Lasix, thiazides, estrogen, azathioprine, tetracycline, sulfa drugs, ddI, ddC, valproic acid, 6-MP, L-asparginase • Hypertriglyceridemia >1000mg/dl • Cystic fibrosis • Hypercalcemia • Trauma • ERCP

  4. Physical exam signs and symptoms: • Pain radiating from epigastrium “boring through” to the back • Cullen’s sign • blue around the umbilicus • Turner’s Sign • purple or green discoloration of the flanks. Acute Pancreatitis (continued)

  5. Dx: • Elevated amylase and lipase, when amy >900 U/L and lipase >6000 U/L--97% specific • Both elevated also in biliary dz, perforation, renal insufficiency • Amylase also high in parotitis, macroamylasemia, chronic EtOH Acute Pancreatitis (continued)

  6. Initial Eval: RUQ U/S of biliary tree, CT after 48 hours if not improved or complication suspected • Don’t worry about MRCP vs ERCP • Prognosis • APACHE II, Ranson’s, and Balthazar have been validated for mortality • Failure of one or more organ system is more clinically useful Acute Pancreatitis (continued)

  7. 57 yo woman s/p traumatic pancreatitis 8 mos ago presents in F/U. She is asymptomatic. No meds. No EtOH. PE: epigastric fullness, no pain. Amylase 180 U/L. Serial CT Scans reveal an 8 cm cystic lesion with a well-defined capsule in the pancreatic body. No  in 6 mos. Best Management? A. Conservative B. Percutaneous drainage C. ERCP with internal drainage D. Surgical drainage E. TPN

  8. Fluid Collections and Pancreatitis • Pancreatitic fluid collection high in amylase may appear in 48 hours, usually resolves • Left pleural effusion common • Fluid collection with clinical signs of infection should be aspirated to r/o infection • Necrosis, within 2 weeks, if infected--surgical debridement • Severe pancreatitis with suspected infection: empiric coverage with imipenem or cefuroxime

  9. Fluid collections • Pseudocyst develops in 10-15% of pts, requires 1-4 weeks to develop • Complications of hemorrhage, rupture, fistula formation • Drainage rec’d only if symptomatic or infected • Abscess develops 4-6 weeks post acute attack • CT guided aspiration 90% accurate • Surgical debridement

  10. Pancreatic Pearls • Abdominal Pain and  amylase don’t always equal acute pancreatitis: • Acute cholecystitis • Intestinal infarction • DKA • Perforated Ulcer • Salpingitis • Ectopic pregnancy • Perforated Diverticulum • Macroamylasemia

  11. Chronic Pancreatitis • 60-70% due to EtOH, usu >10 years. • Other causes CF, pancreas divisum, tumor, hyperparathyroidism • Loss 80-90% of endocrine/exocrine function develop DM and steatorrhea • Increased risk for pancreatic cancer • Dx: • 1) Ca+ on AXR • 2) CT or MRI or EUS or secretin test (bicarb<80 mEq/L) • 3) ERCP

  12. Complications of Chronic Pancreatitis • Gastric varices due to splenic vein thrombosis • B12 malabsorption • Brittle DM, prone to hypoglycemia secondary to loss of pancreatic glucagon • No retinopathy or nephropathy • Jaundice due to obstruction of CBD as it runs through the pancreatic head

  13. Chronic Pancreatitis Treatment • Low fat diet, less than 25 g/d • Pancreatic enzyme replacement has little or no effect on pain but can help with steatorrhea • Must be enteric coated or given with PPI because gastric acid inactivates them • If pancreatic duct is dilated, ERCP or surgery have shown improvement in pain

  14. Pancreatitis Pearls • Microlithiasis may cause recurrent pancreatitis in setting of no EtOH and no gallstones on U/S • Splenic vein thrombosis in severe acute pancreatitis causes gastric not esophageal varices • ERCP • cholangitis/sepsis • TB > 2.5 or dilated CBD on imaging

  15. Pancreatic Neoplasms • Pancreatic Adenocarcinoma: • Classic presentation is painless jaundice • Risk Factors: chronic pancreatitis, diabetes mellitus, smokers (2x), perhaps heavy EtOH users • >80% present with advanced disease • CT is first test, Double duct sign on ERCP, EUS good for staging • If no mets then Whipple procedure, rarely curative

  16. Cystic Neoplasms of the Pancreas • They happen and need biopsy to r/o cystadenocarcinoma • All have malignant potential and need resection

  17. Other Pancreatic Neoplasia • Glucagonoma • Plasma glucagon usually > 1000pg/dl • Scaly necrotizing dermatitis • Necrolytic migratory erythema (NME) • Wt loss • Anemia • Hyperglycemia

  18. Other Pancreatic Neoplasia • Insulinoma • VIPoma • “pancreatic cholera”, profuse watery diarrhea • Gastrinoma • ZE syndrome, elevated gastrin level (off PPI), think about MEN I

  19. Biliary Disease • Cholelithiasis • 20% females, 8% of males • Obesity, Pregnancy • Native American (Pima Indian), Hispanic • Oral contraceptive use, Clofibrate tx, TPN • Ileal disease (Crohn’s) or resection • 80% of stones are radioluscent-cholesterol • good case-pt s/p gastric bypass, rapid wt loss--cholesterol stones

  20. Cholelithiasis • Pigment stones: Clonorchis, Sickle cell dz (i.e., hemolysis) • Dx: U/S 90% sensitive; HIDA best for determining cystic duct obstruction • Tx: • Symptomatic-Elective cholecystectomy • If not surgical candidate: Actigall--cholesterol stones only • Low suspicion for CBD stone: MRCP or EUS • High suspicion CBD stone: ERCP

  21. 70 yo asymptomatic woman undergoes abd U/S after a pulsatile mass is found on physical exam. A 3 cm aortic aneurysm and multiple gallstones are found. The next step in management is: • ERCP with sphincterotomy • Lithotripsy • Elective cholecystectomy • Ursodeoxycholic Acid • Observation/No treatment

  22. Asymptomatic: Observation!!! • Cholangitis • Charcot’s Triad: • Fever • Biliary colic • Jaundice • Tx: • Abx • ERCP for sphincterotomy

  23. Other Diseases of the GB • Calcifications of GB wall on X-Ray highly suggestive of canceropen cholecystectomy • Emphysematous cholecystitisemergent laparotomy • Abx-Gram- and anaerobes, no ceftriaxone (biliary concretions)!

  24. Primary Biliary Cirrhosis • EPIDEMIOLOGY • 95% women • onset 30-65 • incidence 2.7 per 100,000 person years

  25. Primary Biliary Cirrhosis • EPIDEMIOLOGY • clustering in geographic areas • prevalence 1000x greater in families of a patient than general population • no obvious inheritance pattern

  26. Primary Biliary Cirrhosis • SYMPTOMS / PRESENTATION • abnormal LFT’s • fatigue • pruritus • decompensated cirrhosis

  27. Primary Biliary Cirrhosis • SYMPTOMS / PRESENTATION • osteoporosis • osteomalacia • steatorrhea • xanthomata • hyperlipidemia

  28. Primary Biliary Cirrhosis • ASSOCIATED CONDITIONS • rheumatoid arthritis 5-10% • Sjogren’s 40-65% • scleroderma 5-10% • hypothyroidism 20%

  29. Primary Biliary Cirrhosis • PHYSICAL EXAM • Skin hyperpigmentation • Xanthomas • Hepatomegaly • Kayser-Fleischer rings (rare) • not just Wilson’s • Splenomegaly, ascites, etc

  30. Primary Biliary Cirrhosis • LABORATORY • Alk phos - may be only abnormality • AST/ALT - normal or mild elevation • bilirubin - normal early, elevated later

  31. Primary Biliary Cirrhosis • LABORATORY • Antimitochondrial antibody • sensitivity 95% • specificity 98% • IgM - elevated • Eosinophilia

  32. Primary Biliary Cirrhosis • LABORATORY • Hyperlipidemia • elevated in > 50% • mild LDL and VLDL elevations • significantly elevated HDL • no known increased risk of CAD

  33. Primary Biliary Cirrhosis • LIVER BIOPSY • Diagnosis often made prior to liver biopsy • Biopsy may stage the degree of fibrosis (0-4)

  34. Primary Biliary Cirrhosis • NATURAL HISTORY • Mahl et al., Yale, Hepatology 1994 • 250 patients, up to 24 years follow-up • Median survival • symptomatic - 7.5 years • asymptomatic - 16 years

  35. Primary Biliary Cirrhosis • TREATMENT • Malabsorption • Vitamin D • Vitamin A • Vitamin E - in advanced disease • Vitamin K - in advanced disease

  36. Primary Biliary Cirrhosis • TREATMENT • Drugs that didn’t work • steroids • azathioprine • penicillamine • silymarin (milk thistle) • cyclosporine - effective but toxicities

  37. Primary Biliary Cirrhosis • TREATMENT • Ursodeoxycholic acid • UDCA decreases plasma and biliary endogenous bile acid concentrations • UDCA may decrease immune-mediated destruction of hepatocytes by decreasing the expression of HLA class I and II antigens on hepatocytes, which may diminish recognition by the immune system

  38. Primary Biliary Cirrhosis • TREATMENT • Ursodeoxycholic acid • 13-15 mg/day • Moderate to severe disease • decreased likelihood of transplantation or death • 47% versus 66% at 4 years • meta-analysis showed no benefit but many studies short-term

  39. Primary Biliary Cirrhosis • TREATMENT • Ursodeoxycholic acid • Mild to moderate disease • improvement in LFT’s • improved histology

  40. Primary Biliary Cirrhosis • TREATMENT • Colchicine • mechanism unclear • dose 1 mg/day • well-tolerated in studies • less effective than ursodiol • no clear benefit to combination therapy

  41. Primary Biliary Cirrhosis • TREATMENT • Methotrexate • Dose 0.25 mg/kg PO qweek • Conflicting results • No long-term efficacy, safety data

  42. Primary Biliary Cirrhosis • TREATMENT • Liver Transplantation • survival similar to other etiologies of liver disease • recurrence after liver transplant uncommon • similar appearance to chronic rejection

  43. Primary Sclerosing Cholangitis • Characterized by progressive inflammation, fibrosis, and stricturing of the intrahepatic and extrahepatic bile ducts

  44. Primary Sclerosing Cholangitis • “Secondary” sclerosing cholangitis • prior biliary surgery • choledocholithiasis • intra-arterial chemo (floxuridine) • bacterial cholangitis • AIDS cholangiopathy

  45. Primary Sclerosing Cholangitis • EPIDEMIOLOGY • Prevalence 1- 6 per 100,000 in US • 70% men • mean age at diagnosis 40 years

  46. Primary Sclerosing Cholangitis • ASSOCIATION WITH IBD • Among patients with PSC, ulcerative colitis present in 25-90% (likely 90%) • Among patients with ulcerative colitis, PSC present in 5% • Less common but seen in Crohn’s

  47. Primary Sclerosing Cholangitis • PATHOGENESIS • Unknown but proposed • autoimmune (given association with UC), common ANA, ASMA, ANCA • inflammatory reaction in the liver and bile ducts induced by chronic or recurrent entry of bacteria into the portal circulation • ischemic damage to bile ducts

  48. Primary Sclerosing Cholangitis • DIAGNOSIS • Gold standard - ERCP • MRCP also • most patients asymptomatic with abnormal LFT’s • consider if IBD and elevated alk phos

  49. Primary Sclerosing Cholangitis • LABORATORY • alk phos & bili fluctuate • AST/ALT normal or up to 200

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