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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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Intrahepatic cholestasis of pregnancy
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)


Acute pancreatitis
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


Pancreatic and biliary disease

  • 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)


Pancreatic and biliary disease

  • 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)


Pancreatic and biliary disease

Acute Pancreatitis (continued)


Pancreatic and biliary disease

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


Fluid collections and pancreatitis
Fluid Collections and Pancreatitis 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

  • 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


Fluid collections
Fluid collections 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

  • 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


Pancreatic and biliary disease

Pancreatic Pearls 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

  • Abdominal Pain and  amylase don’t always equal acute pancreatitis:

    • Acute cholecystitis

    • Intestinal infarction

    • DKA

    • Perforated Ulcer

    • Salpingitis

    • Ectopic pregnancy

    • Perforated Diverticulum

    • Macroamylasemia


Chronic pancreatitis
Chronic Pancreatitis 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

  • 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


Complications of chronic pancreatitis
Complications of Chronic Pancreatitis 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

  • 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


Chronic pancreatitis treatment
Chronic Pancreatitis 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 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


Pancreatitis pearls
Pancreatitis Pearls 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

  • 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


Pancreatic neoplasms
Pancreatic Neoplasms 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

  • 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


Cystic neoplasms of the pancreas
Cystic Neoplasms of the Pancreas 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

  • They happen and need biopsy to r/o cystadenocarcinoma

  • All have malignant potential and need resection


Other pancreatic neoplasia
Other Pancreatic Neoplasia 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

  • Glucagonoma

    • Plasma glucagon usually > 1000pg/dl

    • Scaly necrotizing dermatitis

      • Necrolytic migratory erythema (NME)

    • Wt loss

    • Anemia

    • Hyperglycemia


Pancreatic and biliary disease

Other Pancreatic Neoplasia 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

  • Insulinoma

  • VIPoma

    • “pancreatic cholera”, profuse watery diarrhea

  • Gastrinoma

    • ZE syndrome, elevated gastrin level (off PPI), think about MEN I


Biliary disease
Biliary Disease 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

  • 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


Cholelithiasis
Cholelithiasis 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

  • 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


Pancreatic and biliary disease

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


Pancreatic and biliary disease

  • Asymptomatic: Observation!!! mass is found on physical exam. A 3 cm aortic aneurysm and multiple gallstones are found. The next step in management is:

  • Cholangitis

    • Charcot’s Triad:

      • Fever

      • Biliary colic

      • Jaundice

    • Tx:

      • Abx

      • ERCP for sphincterotomy


Other diseases of the gb
Other Diseases of the GB mass is found on physical exam. A 3 cm aortic aneurysm and multiple gallstones are found. The next step in management is:

  • 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)!


Primary biliary cirrhosis
Primary Biliary Cirrhosis mass is found on physical exam. A 3 cm aortic aneurysm and multiple gallstones are found. The next step in management is:

  • EPIDEMIOLOGY

    • 95% women

    • onset 30-65

    • incidence 2.7 per 100,000 person years


Primary biliary cirrhosis1
Primary Biliary Cirrhosis mass is found on physical exam. A 3 cm aortic aneurysm and multiple gallstones are found. The next step in management is:

  • EPIDEMIOLOGY

    • clustering in geographic areas

    • prevalence 1000x greater in families of a patient than general population

      • no obvious inheritance pattern


Primary biliary cirrhosis2
Primary Biliary Cirrhosis mass is found on physical exam. A 3 cm aortic aneurysm and multiple gallstones are found. The next step in management is:

  • SYMPTOMS / PRESENTATION

    • abnormal LFT’s

    • fatigue

    • pruritus

    • decompensated cirrhosis


Primary biliary cirrhosis3
Primary Biliary Cirrhosis mass is found on physical exam. A 3 cm aortic aneurysm and multiple gallstones are found. The next step in management is:

  • SYMPTOMS / PRESENTATION

    • osteoporosis

    • osteomalacia

    • steatorrhea

    • xanthomata

    • hyperlipidemia


Primary biliary cirrhosis4
Primary Biliary Cirrhosis mass is found on physical exam. A 3 cm aortic aneurysm and multiple gallstones are found. The next step in management is:

  • ASSOCIATED CONDITIONS

    • rheumatoid arthritis 5-10%

    • Sjogren’s 40-65%

    • scleroderma 5-10%

    • hypothyroidism 20%


Primary biliary cirrhosis5
Primary Biliary Cirrhosis mass is found on physical exam. A 3 cm aortic aneurysm and multiple gallstones are found. The next step in management is:

  • PHYSICAL EXAM

    • Skin hyperpigmentation

    • Xanthomas

    • Hepatomegaly

    • Kayser-Fleischer rings (rare)

      • not just Wilson’s

    • Splenomegaly, ascites, etc


Primary biliary cirrhosis6
Primary Biliary Cirrhosis mass is found on physical exam. A 3 cm aortic aneurysm and multiple gallstones are found. The next step in management is:

  • LABORATORY

    • Alk phos - may be only abnormality

    • AST/ALT - normal or mild elevation

    • bilirubin - normal early, elevated later


Primary biliary cirrhosis7
Primary Biliary Cirrhosis mass is found on physical exam. A 3 cm aortic aneurysm and multiple gallstones are found. The next step in management is:

  • LABORATORY

    • Antimitochondrial antibody

      • sensitivity 95%

      • specificity 98%

    • IgM - elevated

    • Eosinophilia


Primary biliary cirrhosis8
Primary Biliary Cirrhosis mass is found on physical exam. A 3 cm aortic aneurysm and multiple gallstones are found. The next step in management is:

  • LABORATORY

    • Hyperlipidemia

      • elevated in > 50%

      • mild LDL and VLDL elevations

      • significantly elevated HDL

      • no known increased risk of CAD


Primary biliary cirrhosis9
Primary Biliary Cirrhosis mass is found on physical exam. A 3 cm aortic aneurysm and multiple gallstones are found. The next step in management is:

  • LIVER BIOPSY

    • Diagnosis often made prior to liver biopsy

    • Biopsy may stage the degree of fibrosis (0-4)


Primary biliary cirrhosis10
Primary Biliary Cirrhosis mass is found on physical exam. A 3 cm aortic aneurysm and multiple gallstones are found. The next step in management is:

  • 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


Primary biliary cirrhosis11
Primary Biliary Cirrhosis mass is found on physical exam. A 3 cm aortic aneurysm and multiple gallstones are found. The next step in management is:

  • TREATMENT

    • Malabsorption

      • Vitamin D

      • Vitamin A

      • Vitamin E - in advanced disease

      • Vitamin K - in advanced disease


Primary biliary cirrhosis12
Primary Biliary Cirrhosis mass is found on physical exam. A 3 cm aortic aneurysm and multiple gallstones are found. The next step in management is:

  • TREATMENT

    • Drugs that didn’t work

      • steroids

      • azathioprine

      • penicillamine

      • silymarin (milk thistle)

      • cyclosporine - effective but toxicities


Primary biliary cirrhosis13
Primary Biliary Cirrhosis mass is found on physical exam. A 3 cm aortic aneurysm and multiple gallstones are found. The next step in management is:

  • 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


Primary biliary cirrhosis14
Primary Biliary Cirrhosis mass is found on physical exam. A 3 cm aortic aneurysm and multiple gallstones are found. The next step in management is:

  • 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


Primary biliary cirrhosis15
Primary Biliary Cirrhosis mass is found on physical exam. A 3 cm aortic aneurysm and multiple gallstones are found. The next step in management is:

  • TREATMENT

    • Ursodeoxycholic acid

      • Mild to moderate disease

        • improvement in LFT’s

        • improved histology


Primary biliary cirrhosis16
Primary Biliary Cirrhosis mass is found on physical exam. A 3 cm aortic aneurysm and multiple gallstones are found. The next step in management is:

  • TREATMENT

    • Colchicine

      • mechanism unclear

      • dose 1 mg/day

      • well-tolerated in studies

      • less effective than ursodiol

      • no clear benefit to combination therapy


Primary biliary cirrhosis17
Primary Biliary Cirrhosis mass is found on physical exam. A 3 cm aortic aneurysm and multiple gallstones are found. The next step in management is:

  • TREATMENT

    • Methotrexate

      • Dose 0.25 mg/kg PO qweek

      • Conflicting results

      • No long-term efficacy, safety data


Primary biliary cirrhosis18
Primary Biliary Cirrhosis mass is found on physical exam. A 3 cm aortic aneurysm and multiple gallstones are found. The next step in management is:

  • TREATMENT

    • Liver Transplantation

      • survival similar to other etiologies of liver disease

      • recurrence after liver transplant uncommon

        • similar appearance to chronic rejection


Primary sclerosing cholangitis
Primary Sclerosing Cholangitis mass is found on physical exam. A 3 cm aortic aneurysm and multiple gallstones are found. The next step in management is:

  • Characterized by progressive inflammation, fibrosis, and stricturing of the intrahepatic and extrahepatic bile ducts


Primary sclerosing cholangitis1
Primary Sclerosing Cholangitis mass is found on physical exam. A 3 cm aortic aneurysm and multiple gallstones are found. The next step in management is:

  • “Secondary” sclerosing cholangitis

    • prior biliary surgery

    • choledocholithiasis

    • intra-arterial chemo (floxuridine)

    • bacterial cholangitis

    • AIDS cholangiopathy


Primary sclerosing cholangitis2
Primary Sclerosing Cholangitis mass is found on physical exam. A 3 cm aortic aneurysm and multiple gallstones are found. The next step in management is:

  • EPIDEMIOLOGY

    • Prevalence 1- 6 per 100,000 in US

    • 70% men

    • mean age at diagnosis 40 years


Primary sclerosing cholangitis3
Primary Sclerosing Cholangitis mass is found on physical exam. A 3 cm aortic aneurysm and multiple gallstones are found. The next step in management is:

  • 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


Primary sclerosing cholangitis4
Primary Sclerosing Cholangitis mass is found on physical exam. A 3 cm aortic aneurysm and multiple gallstones are found. The next step in management is:

  • 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


Primary sclerosing cholangitis5
Primary Sclerosing Cholangitis mass is found on physical exam. A 3 cm aortic aneurysm and multiple gallstones are found. The next step in management is:

  • DIAGNOSIS

    • Gold standard - ERCP

      • MRCP also

    • most patients asymptomatic with abnormal LFT’s

    • consider if IBD and elevated alk phos


Primary sclerosing cholangitis6
Primary Sclerosing Cholangitis mass is found on physical exam. A 3 cm aortic aneurysm and multiple gallstones are found. The next step in management is:

  • LABORATORY

    • alk phos & bili fluctuate

    • AST/ALT normal or up to 200


Primary sclerosing cholangitis7
Primary Sclerosing Cholangitis mass is found on physical exam. A 3 cm aortic aneurysm and multiple gallstones are found. The next step in management is:

  • LABORATORY

    • elevated IgG 30%

    • elevated IgM 40-50%

    • p-ANCA 30-80%

    • HLA DRw52a 0-100%


Primary sclerosing cholangitis8
Primary Sclerosing Cholangitis mass is found on physical exam. A 3 cm aortic aneurysm and multiple gallstones are found. The next step in management is:

  • LIVER BIOPSY

    • sampling likely so not a good diagnostic tool

    • may stage disease


Primary sclerosing cholangitis9
Primary Sclerosing Cholangitis mass is found on physical exam. A 3 cm aortic aneurysm and multiple gallstones are found. The next step in management is:

  • LOCATION

    • Intra- & extrahepatic bile ducts: 87%

    • Intrahepatic bile ducts alone: 11%

    • Extrahepatic bile ducts alone: 2%

      Kaplan, NEJM 1995


Primary sclerosing cholangitis10
Primary Sclerosing Cholangitis mass is found on physical exam. A 3 cm aortic aneurysm and multiple gallstones are found. The next step in management is:

  • PRESENTATIONS

    • Ascending cholangitis

    • Abnormal LFT’s

    • Pruritus


Primary sclerosing cholangitis11
Primary Sclerosing Cholangitis mass is found on physical exam. A 3 cm aortic aneurysm and multiple gallstones are found. The next step in management is:

  • NATURAL HISTORY

    • complications vary

      • biliary - ascending cholangitis

      • liver failure - portal hypertension, etc

      • cholangiocarcinoma


Primary sclerosing cholangitis12
Primary Sclerosing Cholangitis mass is found on physical exam. A 3 cm aortic aneurysm and multiple gallstones are found. The next step in management is:

  • NATURAL HISTORY

    • mean survival 12 years after diagnosis

      • worse if symptomatic at diagnosis


Primary sclerosing cholangitis13
Primary Sclerosing Cholangitis mass is found on physical exam. A 3 cm aortic aneurysm and multiple gallstones are found. The next step in management is:

  • TREATMENT

    • No proven medical therapy

      • D-penicillamine

      • Steroids

      • Cyclosporine, Tacrolimus

      • Methotrexate

      • Azathioprine, 6-MP

      • Ursodeoxycholic acid (small study, benefit to  dose)


Primary sclerosing cholangitis14
Primary Sclerosing Cholangitis mass is found on physical exam. A 3 cm aortic aneurysm and multiple gallstones are found. The next step in management is:

  • TREATMENT

    • Relieve biliary obstruction

      • risk of infection

    • Dominant stricture

      • r/o cholangiocarcinoma

      • dilate or stent


Primary sclerosing cholangitis15
Primary Sclerosing Cholangitis mass is found on physical exam. A 3 cm aortic aneurysm and multiple gallstones are found. The next step in management is:

  • TREATMENT

    • Liver Transplantation

      • survival similar to other etiologies of liver disease

      • disadvantage if symptoms due to cholangitis and not liver failure in MELD

        • living donor?


Primary sclerosing cholangitis16
Primary Sclerosing Cholangitis mass is found on physical exam. A 3 cm aortic aneurysm and multiple gallstones are found. The next step in management is:

  • CHOLANGIOCARCINOMA

    • 10-15% lifetime risk

    • increased if IBD or cirrhosis

    • contraindication to liver transplant

      • protocol for aggressive chemotherapy


Primary sclerosing cholangitis17
Primary Sclerosing Cholangitis mass is found on physical exam. A 3 cm aortic aneurysm and multiple gallstones are found. The next step in management is:

  • CHOLANGIOCARCINOMA

    • Diagnosis difficult

    • CT/MRI - low sensitivity

    • CA 19-9 - low sensitivity


Autoimmune hepatitis
Autoimmune Hepatitis mass is found on physical exam. A 3 cm aortic aneurysm and multiple gallstones are found. The next step in management is:

  • EPIDEMIOLOGY

    • Female > Male 4:1

    • Two peaks

      • 20’s and Middle age

      • also seen in children

    • 50-200 cases/million


Autoimmune hepatitis1
Autoimmune Hepatitis mass is found on physical exam. A 3 cm aortic aneurysm and multiple gallstones are found. The next step in management is:

  • SYMPTOMS/PRESENTATION

    • Abdominal pain

    • fever

    • anorexia

    • malaise


Autoimmune hepatitis2
Autoimmune Hepatitis mass is found on physical exam. A 3 cm aortic aneurysm and multiple gallstones are found. The next step in management is:

  • SYMPTOMS/PRESENTATION

    • Acute or chronic disease

    • 30 - 80 % cirrhotic at presentation


Autoimmune hepatitis3
Autoimmune Hepatitis mass is found on physical exam. A 3 cm aortic aneurysm and multiple gallstones are found. The next step in management is:

  • ASSOCIATED CONDITIONS

    • Arthropathy

    • Ulcerative colitis

    • Sjogren’s syndrome

    • Autoimmune thyroiditis

    • Fibrosing Alveolitis

    • Glomerulonephritis


Autoimmune hepatitis4
Autoimmune Hepatitis mass is found on physical exam. A 3 cm aortic aneurysm and multiple gallstones are found. The next step in management is:

  • DIAGNOSIS

  • PATHOLOGY

    • Interface hepatitis

    • Lymphocytes and plasma cells

    • Bridging necrosis

    • Cirrhosis


Autoimmune hepatitis5
Autoimmune Hepatitis mass is found on physical exam. A 3 cm aortic aneurysm and multiple gallstones are found. The next step in management is:

  • CLASSIFICATION

  • Type 1

    • ANA, anti-smooth muscle antibody

  • Type 2

    • anti-LKM, liver cytosol antigen

    • girls, young women


Autoimmune hepatitis6
Autoimmune Hepatitis mass is found on physical exam. A 3 cm aortic aneurysm and multiple gallstones are found. The next step in management is:

  • CLASSIFICATION

  • Overlap Syndrome

    • path  autoimmune hepatitis

    • serology  PBC (+AMA)

  • Autoimmune cholangiopathy

    • path  PBC

    • serology  ANA, asma


Autoimmune hepatitis7
Autoimmune Hepatitis mass is found on physical exam. A 3 cm aortic aneurysm and multiple gallstones are found. The next step in management is:

  • TREATMENT

    • Corticosteroids

      • acute management

    • Azathioprine

      • goal - maintain remission

      • 2 mg/kg per day

      • goal to d/c Prednisone


Pancreatic and biliary disease
NASH mass is found on physical exam. A 3 cm aortic aneurysm and multiple gallstones are found. The next step in management is:

  • Nonalcoholic steatohepatitis

  • Nonalcoholic fatty liver disease (NAFLD)


Pancreatic and biliary disease
NASH mass is found on physical exam. A 3 cm aortic aneurysm and multiple gallstones are found. The next step in management is:

  • DEFINITION

    • liver biopsy with macrovesicular steatosis & inflammation

    • minimal or no EtOH

    • negative serologic work-up


Pancreatic and biliary disease
NASH mass is found on physical exam. A 3 cm aortic aneurysm and multiple gallstones are found. The next step in management is:

  • EPIDEMIOLOGY

    • #1 cause of liver disease?

    • Women > men

    • Most 40-60

      • reported in children


Pancreatic and biliary disease
NASH mass is found on physical exam. A 3 cm aortic aneurysm and multiple gallstones are found. The next step in management is:

  • ASSOCIATED CONDITIONS

    • obesity

    • type 2 diabetes mellitus

    • hyperlipidemia

    • medications

    • obesity bypass procedures

    • TPN


Pancreatic and biliary disease
NASH mass is found on physical exam. A 3 cm aortic aneurysm and multiple gallstones are found. The next step in management is:

  • DIAGNOSIS

    • liver biopsy

      • confirms or excludes dx

      • negative serologic work-up


Pancreatic and biliary disease
NASH mass is found on physical exam. A 3 cm aortic aneurysm and multiple gallstones are found. The next step in management is:

  • TREATMENT

    • treat underlying condition

      • obesity, DM, lipids

    • stay tuned...


Alcoholic hepatitis
Alcoholic Hepatitis mass is found on physical exam. A 3 cm aortic aneurysm and multiple gallstones are found. The next step in management is:

  • EPIDEMIOLOGY

    • alcohol

      • cirrhosis  80 gm/d EtOH for 10-20 years

    • other factors

      • female gender

        • reduced gastric ADH activity

        • size

      • co-existing HBV, HCV


Alcoholic hepatitis1
Alcoholic Hepatitis mass is found on physical exam. A 3 cm aortic aneurysm and multiple gallstones are found. The next step in management is:

  • SYMPTOMS

    • fever

    • hepatomegaly

    • jaundice

    • anorexia


Alcoholic hepatitis2
Alcoholic Hepatitis mass is found on physical exam. A 3 cm aortic aneurysm and multiple gallstones are found. The next step in management is:

  • DIAGNOSIS

    • liver biopsy

      • steatosis, inflammation

    • AST > 2x ALT


Alcoholic hepatitis3
Alcoholic Hepatitis mass is found on physical exam. A 3 cm aortic aneurysm and multiple gallstones are found. The next step in management is:

  • PROGNOSIS

    • liver failure

      • coagulopathy

      • encephalopathy

    • Discriminant function =

      (4.6 x [PT - control PT]) + (serum bili, mg/dl)

    • DF > 32: mortality 35-45%


Alcoholic hepatitis4
Alcoholic Hepatitis mass is found on physical exam. A 3 cm aortic aneurysm and multiple gallstones are found. The next step in management is:

  • TREATMENT

    • Supportive care

    • Who gets steroids?

      • DF > 32

      • Encephalopathy

      • No infection, no GI bleeding


Abnormal liver tests
Abnormal Liver Tests mass is found on physical exam. A 3 cm aortic aneurysm and multiple gallstones are found. The next step in management is:

  • Hepatocellular - AST, ALT 

  • Cholestatic - alkaline phosphatase 

    • bilirubin can be elevated in both


Abnormal liver tests1
Abnormal Liver Tests mass is found on physical exam. A 3 cm aortic aneurysm and multiple gallstones are found. The next step in management is:

  • AST 124 U/L

  • ALT 157 U/L

  • Alk phos 149 U/L

  • T. bili 1.6 mg/dl


Abnormal liver tests2
Abnormal Liver Tests mass is found on physical exam. A 3 cm aortic aneurysm and multiple gallstones are found. The next step in management is:

  • AST/ALT mildly elevated (<250 U/L)

    • chronic viral hepatitis

      • HCV Ab, HBV surface antigen

    • alcoholic hepatitis (AST > ALT)

      • drug reaction - consider d/c

      • NSAIDs, statins, antibiotics (INH)

    • hemochromatosis (Fe/TIBC > 45%)

    • steatosis, steatohepatitis


Abnormal liver tests3
Abnormal Liver Tests mass is found on physical exam. A 3 cm aortic aneurysm and multiple gallstones are found. The next step in management is:

  • AST/ALT mildly elevated (<250 U/L)

    • less common causes

    • autoimmune hepatitis

      • ANA, ASMA, a-LKM

    • Wilson’s disease

      • age < 40; check ceruloplasmin, K-F rings

    • Alpha-1-antitrypsin deficiency

      • emphysema; alpha-1-antitrypsin level


Abnormal liver tests4
Abnormal Liver Tests mass is found on physical exam. A 3 cm aortic aneurysm and multiple gallstones are found. The next step in management is:

  • AST/ALT mildly elevated (<250 U/L)

    • non-hepatic causes

    • muscle source

    • hypothyroidism

    • celiac disease

      • diarrhea, Fe deficiency; anti-endomysial IgA

    • adrenal insufficiency


Abnormal liver tests5
Abnormal Liver Tests mass is found on physical exam. A 3 cm aortic aneurysm and multiple gallstones are found. The next step in management is:

  • AST/ALT mildly elevated (<250 U/L)

    • negative serologic work-up

    • consider liver biopsy if persistently abnormal


Abnormal liver tests6
Abnormal Liver Tests mass is found on physical exam. A 3 cm aortic aneurysm and multiple gallstones are found. The next step in management is:

  • AST 1480 U/L

  • ALT 1704 U/L

  • Alk phos 229 U/L

  • T. bili 4.8 mg/dl


Abnormal liver tests7
Abnormal Liver Tests mass is found on physical exam. A 3 cm aortic aneurysm and multiple gallstones are found. The next step in management is:

  • AST/ALT  > 10x ULN

    • acute viral hepatitis

      • hep A IgM

      • hep B core IgM

      • hep D

    • autoimmune hepatitis

    • shock liver (ischemic hepatitis)

    • drug or toxin (acetaminophen)


Abnormal liver tests8
Abnormal Liver Tests mass is found on physical exam. A 3 cm aortic aneurysm and multiple gallstones are found. The next step in management is:

  • AST/ALT  > 10x ULN

    • Rarer forms

    • acute Budd-Chiari syndrome

    • veno-occlusive disease

    • HELLP syndrome

    • acute fatty liver of pregnancy


Abnormal liver tests9
Abnormal Liver Tests mass is found on physical exam. A 3 cm aortic aneurysm and multiple gallstones are found. The next step in management is:

  • AST/ALT  > 10x ULN

    • Acute Liver Failure

    • Elevated PT

      •  factor V

    • Encephalopathy

      • If discharge patient, clarify supervision

    • Transfer to Transplant Center


Abnormal liver tests10
Abnormal Liver Tests mass is found on physical exam. A 3 cm aortic aneurysm and multiple gallstones are found. The next step in management is:

  • Bilirubin

    • unconjugated

      • Overproduction of bilirubin or impaired uptake, conjugation

      • tightly bound to albumin so not filtered and not present in urine

    • conjugated

      • impaired excretion into bile ductules

      • present in the urine


Abnormal liver tests11
Abnormal Liver Tests mass is found on physical exam. A 3 cm aortic aneurysm and multiple gallstones are found. The next step in management is:

  • Unconjugated bilirubin

    • hemolysis

    • impaired bilirubin uptake

      • CHF

      • Portosystemic shunts

      • Certain drugs - rifampin, probenecid


Abnormal liver tests12
Abnormal Liver Tests mass is found on physical exam. A 3 cm aortic aneurysm and multiple gallstones are found. The next step in management is:

  • Unconjugated bilirubin

    • hemolysis

    • impaired bilirubin uptake

    • impaired bilirubin conjugation

      • Gilbert’s

      • Crigler-Najjar

      • hyperthyroidism

      • cirrhosis

      • Wilson’s disease


Abnormal liver tests13
Abnormal Liver Tests mass is found on physical exam. A 3 cm aortic aneurysm and multiple gallstones are found. The next step in management is:

  • Unconjugated bilirubin

    • Gilbert’s Syndrome

      • Uridinediphosphoglucuronate glucuronosyltransferases (UGTs) mediate glucuronidation

      • Mutation of UGT1A

      • 9% western world homozygous

      • 30% heterozygous

        • Slightly higher bili


Abnormal liver tests14
Abnormal Liver Tests mass is found on physical exam. A 3 cm aortic aneurysm and multiple gallstones are found. The next step in management is:

  • Unconjugated bilirubin

    • Gilbert’s Syndrome

      • Bilirubin

        • Usually < 3 mg/dl, rarely > 6

      • Factors

        • Fasting

        • Stress (surgery, etc)

        • Infection


Abnormal liver tests15
Abnormal Liver Tests mass is found on physical exam. A 3 cm aortic aneurysm and multiple gallstones are found. The next step in management is:

  • Unconjugated bilirubin

    • Gilbert’s Syndrome

      • Diagnosis

        • rise in bilirubin concentration following a low lipid, 400 kcal diet

        • administration of IV nicotine

        • seldom necessary in clinical practice


Abnormal liver tests16
Abnormal Liver Tests mass is found on physical exam. A 3 cm aortic aneurysm and multiple gallstones are found. The next step in management is:

  • Conjugated bilirubin

    • Extrahepatic cholestasis

      • PSC

      • intrinsic & extrinsic tumors

      • AIDS cholangiopathy

      • cholelithiasis

      • parasites - ascaris lumbricoides, liver flukes


Abnormal liver tests17
Abnormal Liver Tests mass is found on physical exam. A 3 cm aortic aneurysm and multiple gallstones are found. The next step in management is:

  • Conjugated bilirubin

    • Intrahepatic cholestasis

      • Viral hepatitis

      • Alcoholic hepatitis

      • Nonalcoholic fatty liver disease

      • PBC

      • Drugs, toxins

      • Sepsis


Abnormal liver tests18
Abnormal Liver Tests mass is found on physical exam. A 3 cm aortic aneurysm and multiple gallstones are found. The next step in management is:

  • Conjugated bilirubin

    • Intrahepatic cholestasis

      • Infiltrative diseases - sarcoidosis, amyloid, lymphoma

      • TPN

      • Pregnancy

      • Cirrhosis

      • Dubin Johnson, Rotor syndrome


Abnormal liver tests19
Abnormal Liver Tests mass is found on physical exam. A 3 cm aortic aneurysm and multiple gallstones are found. The next step in management is:

  • AST 32 U/L

  • ALT 29 U/L

  • Alk phos 472 U/L

  • T. bili 1.0 mg/dl

  • (5’-nucleotidase )


Abnormal liver tests20
Abnormal Liver Tests mass is found on physical exam. A 3 cm aortic aneurysm and multiple gallstones are found. The next step in management is:

  • Elevated alkaline phosphatase

    • primary biliary cirrhosis

    • primary sclerosing cholangitis

    • partial bile duct obstruction

    • drugs (androgenic steroids, phenytoin)

    • sarcoidosis

    • metastatic cancer