GI Re-Up #6 Flashcards
Primary Biliary Cirrhosis/Cholangitis (PBC) is idiopathic autoimmune disorder of intrahepatic small bile ducts that leads to decreased bile salt excretion, cirrhosis, and end-stage liver disease.
This is MC in ____________.
What are some symptoms?
-MC in middle-aged women (30-60 years old)
-Symptoms: Fatigue (first symptom), pruritus, RUQ discomfort, hepatomegaly, jaundice, xanthelasma, osteoporosis. Cirrhosis symptoms late in disease.
What is the initial diagnostic study done?
What gives a definitive diagnosis?
What do labs show (there is a specific lab)
-Initial: US
-Definitive: Liver biopsy
Labs (cholestatic pattern): increased Alkaline phosphatase and GGT
–Antimitochondrial antibody HALLMARK
First line treatment for PBC
-Ursodeoxycholic acid (like cholelithiasis)
-Liver transplant is definitive
On the other hand, Primary Sclerosing Cholangitis (PSC), is autoimmune progressive cholestasis leading to diffuse fibrosis of intra AND extra hepatic biliary ducts.
This is MC associated with what other condition?
Name some symptoms
-Associated with IBD (Ulcerative Colitis)
Symptoms: jaundice, pruritus, fatigue, RUQ pain, hepatomegaly, splenomegaly (similar to PBC)
What do labs show for PSC (there is a specific one)
-Cholestatic pattern: Increased Alkaline phosphatase and GGT
-Positive P-ANCA HALLMARK
What is the most accurate imaging study for PSC and what is seen?
-MRCP and ERCP - beaded appearance of biliary ducts (narrowing, strictures)
What is Wilson’s Disease?
What is the pathophysiology of this condition?
rare autosomal recessive disorder leading to copper accumulation in the body (liver, brain, kidney, joints, cornea)
Defect in chromosome 13 leads to decreased biliary copper excretion due to decreased ceruloplasmin
Symptoms of Wilson’s Disease
What is seen on a slit-lamp examination?
-Cirrhosis, liver failure, hepatosplenomegaly
-Dysarthria, bradykinesia, tremor, hallucinations, dementia, seizures, ataxia
-Psychosis, Delusions
-Arthralgias from copper deposition
Slit Lamp: Kayser-Fleischer Rings (brown or green rings due to copper deposition in cornea)
What is seen on labs for Wilson’s Disease?
-Decreased serum ceruloplasmin
-Molecular genetic testing for ATP7B
-Elevated transaminases
-Hemolytic Anemia
-Increased 24 hour urinary copper excretion**
What gives a definitive diagnosis of Wilson’s Disease?
Treatment options
-Liver biopsy
-Treatment
–Trientine or D-Penicillamine (Copper chelating agents)
–Give pyridoxine (B6) with Penicillamine to prevent B6 deficiency
–Zinc supplementation
What is the pathophysiology of acute pancreatitis?
2 MCC of this condition, along with others?
-Acinar cell injury –> intracellular activation of pancreatic enzymes –> auto digestion of pancreas
-2 MCC are gallstones and alcohol abuse.
-Others: Thiazides, Protease Inhibitors, Exenatide, Valproic Acid, Scorpion Sting, ERCP, CF, Infection
Symptoms of acute pancreatitis (remember there are two named physical exam findings)
-Epigastric pain: constant, boring pain that radiates to the back or other quadrant. Pain worse if supine, relieved with leaning forward, sitting, or fetal position.
-Nausea, vomiting, fever common
-Decreased bowel sounds
-Cullen Sign: periumbilical ecchymosis
-Grey Turner Sign: flank ecchymosis
Best initial test for acute pancreatitis?
Increased amylase and lipase (lipase more specific)
What else is seen on labs for pancreatitis?
Hypocalcemia (necrotic fat binds to calcium)
Diagnostic imaging of choice for pancreatitis?
What is seen on abdominal XR?
Abdominal CT (DOC)
XR: sentinel loop: localized ileus of segment of small bowel in LUQ. Colon Cutoff Sign: abrupt collapse of colon near pancreas.
Management for acute pancreatitis
-90% recover in 3-7 days with just supportive measures (rest the pancreas)
-NPO, high volume IVF (Lactated Ringers), Analgesia
-ABX not routinely used
MCC of chronic pancreatitis?
There is a triad of clinical manifestations with this condition…what is it?
-ETOH abuse most common
-Others: idiopathic
Calcifications + steatorrhea + Diabetes Mellitus
-Others: weight loss, back pain, epigastric pain
With diagnostics with chronic pancreatitis, amylase and lipase are usually normal or mildly elevated. What is seen on CT scan?
What is seen on abdominal radiographs?
What is the most sensitive and specific test for chronic pancreatitis?
-CT: calcification of pancreas.
-Abdominal XR: calcified pancreas
Pancreatic function testing (fecal elastase) most sensitive and specific
Treatment for chronic pancreatitis
-ETOH abstinence, pain control, low fat diet, vitamin supplementation
-oral pancreatic enzyme replacement
-Pancreatectomy if refractory
70% of pancreatic carcinoma is found in which part?
What is the MC type?
Name some important risk factors for this condition?
-head of pancreas
-Adenocarcinoma
-Smoking, > 55 years old, males, Obesity, chronic pancreatitis, AA