Irritable Bowel Syndrome
- Pathophysiology:
- Normal perception of abnormal gut motility, vs abnormal perception of normal gut motility
- Use Rome III criteria for diagnosing
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- >=12 wks of abdo disomfort over 12mo and has 2/3:
- 1. Relieved by defecation
- 2. Associated with change in frequency of stool
- 3. Associated with change in consistency of stool
- Supporting criteria: (not essential for dx)
- Abnormal frequency
- Anbormal form
- Abnormal passage
- Mucous
- Bloating
- RED FLAGS: (if present unlikely IBS)
- Wt loss
- Fever
- Nocturnal defecation
- Blood/Pus in stool
- Hematuria
- Abnormal Fex-sig
- >=12 wks of abdo disomfort over 12mo and has 2/3:
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Esophageal Varices
- Almost always from portal hypertension (often with stomach varices)
- Risk of bleeding - 30% in first year
- Risk of re-bleeding 50-70%
- Need endoscopy
- Management.
- ABC's - Resuscitate
- IV Octreotide
- Causes splanchnic vasoconstriction
- Decreases poral collateral circulation and pressure - Endoscopic therapy: variceal ligation or sclerotherapy
- Long term tx to decrease recurrence:
- B-Blocker (i.e. non-cardiospecific such as nadolol or propranolol)
- Repeat ligation or sclerotherapy
- Nitrates - Persistent or Recurrent
- Transjugular intrahepatic portosystemic shunt (TIPS)
- Balloon tamponade
- Liver transplant
- Splanchnic vasoconstriction
- Somatostatin
- Octreotide
- Terlipressin
- Review Article: Improved survival with Patients with Variceal Bleeds (Int. J Hepatol 2011)
- Resuscitation to achieve (Hb >70-80g/L, but avoid fluid overload)
- Antibiotic prophylaxis: IV ceftriaxone or postendoscopic norfloxacin reduced infection, rebleeding rates, length of hosp., all-cause mortality
- Band ligation superior to injection sclerotherapy.
Sclerosing Cholangitis
- Inflammation of biliary tree (intra and/or extrahepatic bile ducts) --> scarring and obliteration.
- Two types:
- Primary/idiopathic
- most common
- Associated with IBD, more commonly UC in up to 70% of pts (usually male)
- One of the most common indications for transplant.
- Secondary -less common
- Long-term choledocholithiasis
- Cholangiocarcinoma
- Surgical/traumatic injury (iatrogenic)
- contiguous inflammatory process
- Post-ERCP
- Associated with HIV/AIDS ("HIV cholangiopathy")
- Primary/idiopathic
- Symptoms:
- Often insiduous... fatigue, pruritis
- May have episotic bacterial cholangitis due to biliary obstruction.
- Diagnosis:
- High ALP, less often high bili
- Mildily increased AST (<300 U/L)
- p-ANCA (30-80%), elevated IgM (40-50%)
- ERCP shows narrowing and dilatations of bile ducts that may result in "beading", both intrahepatic and extrahepatic bile ducts.
- If intrahepatic narrowing only, do Antimitochondria antibody (AMA) to r/o PBC.
- Complications:
- Repeated bouts of cholangitis may lead to biliary
Primary Biliary Cirrhosis (PBC)
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Chronic inflammation and fibrous obliteration of intrahepatic ductules.
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Often autoimmune (associated Sjogren's syndrome, scleroderma, CREST syndrome, RA, thyroiditis)
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Classically affects middle-aged women (9:1)
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Classic presentation:
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Middle aged woman presents with fatigue, pruritis, and edema... Has elevated cholestatic liver enzymes.
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Symptoms:
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Asymptomatic
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Pruritis, fatigue.
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Chronic: Jaundice, melanosis (darkening skin),
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High incidence of osteoporosis.
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Labs
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Increased ALP, GGT, bilirubin (rises at later stage)
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Positive Anti-mitochondrial antibody (AMA) --> 95% specificity
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Increased serum cholesterol. (mild increase LDL and larger increase HDL)
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May have xanthelasmas, xanthomas
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Gold standard: liver biopsy.
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Many "overlap" symptoms with autoimmune cholangitis, autoimmune hepatitis, sclerosing cholangitis.
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Treatment:
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Treat w. ursodiol (less frequently colchicine, methotrexate)
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Mechanism: Ursodiol replaces endogenous bile acids that are more toxic.
1. Increased hydrophilicity index of bile acids
2. Stiulation of hepatocellular ductal secretion3. Cytoprotection against bile acid and cytokine induced injury
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Pruritis:
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cholestyramine
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rifampin (150mg bid)
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Calcium and Vitamin D for low bone density, bisphosphonates if osteoporisis.
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monitor TSH
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Liver transplant if disease severe, progressive.
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Secondary Biliary Cirrhosis
- Cirrhosis from prolongued major bile duct obstruction
- Acquired: post-op strictures, chronic pancreatitis, sclerosing cholangitis, stones in duct.
- Congenital: cystic fibrosis, congenital biliary atresia, choledochal cysts.
- Investigations:
- Cholangiography and liver biopsy.
- Tx:
- Treat obstruction
- Give Antibiotics for cholangitis prophylaxis.
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