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16 Cards in this Set

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  • Back
Mallory-Weiss Syndrome
Painful mucosal lacerations at the gastroesophaageal junction due to severe vomiting.

Leads to hematemesis.

Alcoholics and bulimics.
Esophageal varices
Painless bleeding of submucosal veins in lower 1/3 of esophagus.
Boerhaave syndrome
Transmural esophageal rupture due to violent retching
Esophageal strictures
Associated with lye ingestion
Esophagitis
Associated with reflux, infection (HSV-1, CMV, Candida) or chemical ingestion
Plummer-Vinson Syndrome
Clinical triad of
- dysphagia
- glossitis
- iron deficiency anemia
Gardner's syndrome
FAP with osseus and soft tissue tumors, retinal hyperplasia
Turcot's syndrome
FAP with possible brain involvement (glioblastoma)
Gilbert's Syndrome
Mildly decreased UDP-glucuronyl traansferase or decreased bilirubin uptake.
Asymptomatic.
Elevated unconjugated bilirubin without overt hemolysis.
Associated with stress.
No clinical consequences.
Crigler-Najjar Syndrome, type I
Absent UDP-glycuronyl transferase.
Presents early in life; patients die within a few years.

Jaundice, kernicterus (bilirubin deposits in the brain), increased unconjugated bilirubin.

Rx - plasmapheresis and phototherapy

Type II is less sever and responds to phenobarbital, which increases liver enzyme synthesis.
Dubin-Johnson Syndrome
Conjugated hyperbilirubinemia due to defective liver excretion.
Grossly black liver.
Benign.

Rotor's syndrome is milder, no black liver.
Budd-Chiari syndrome
occlusion of IVC or hepatic veins with centrilobular congestion and necrosis

leads to congestive liver failure
(hepatomegaly, ascites, abd pain, liver failure)

may develop varices, have visible abd and back veins

Assoc w/ polycythemia, pregnancy, hepatocellular carcinoma
Wilson's disease

aka hepatolenticular degeneration
inadequate copper excretion and failure of copper to enter circulation as ceruloplasmin

Copper accumulates in liver, brain, cornea, kidneys and joints

Findings:
1. Asterixis (flapping tremor of the hand)
2. Basal ganglia degeneration (parkinsonian symptoms)
3. Ceruloplasmin decreased, cirrhosis, corneal deposits (kayser-Fleisher rings), carcinoma, choreiform movements
4. Dementia

Rx - penicillamine, autosomal recessive.
Primary biliary cirrhosis
intrahepatic, autoimmune

severe obstructive jaundice, steatorrhea, pruritis, hypercholesterolemia (xanthoma), inc ALP, Inc serum mitochondrial Ab

Assoc w/ scleroderma and CREST
secondary biliary sclerosis
Due to extrahepatic biliary obstruction.
Inc pressure in intrahepatic ducts, leading to injury and fibrosis

Often complicated by ascending cholangitis, biles stasis "lakes" ALP, inc conjugated bilirubin
Primary sclerosing cholangitis
both intra and extra hepatic
inflammation and fibrosis of bile ducts leading to alternating strictures and dilation
beading on ERCP

concentric onion skin bile duct
Inc ALP, assoc w/ ulcerative cholitis

can lead to secondary biliary cirrhosis