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What is dermatitis herpetiformis?
Pruritic vesiculopapular rash on the extensor surfaces associated with Celiac Sprue
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Celiac sprue cause:
Infection with Tropheryma whippelii
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Celiac sprue diagnosis:
- Small bowel biopsy - villous atrophy & crypt hyperplasia
- Positive periodic acid-Schiff (PAS) stain for Tropheryma whippelii
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Celiac sprue complications:
Increased risk of small bowel malignancies, especially adenocarcinoma and lymphoma
[Strict adherence to gluten free diet may decrease incidence of malignancy (Kasper et al., 2005, pp. 1673-1675)]
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Ulcerative colitis biopsy findings:
- Granular, friable mucosal surface
- Loss of normal vascular pattern
- Prominent neutrophils in the epithelium
- Cryptitis with focal crypt abscess
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Ulcerative colitis treatment:
- Medical Management
- Moderate - Severe: Prednisone 40mg PO daily with slow taper
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Major liver complication associated with Ulcerative Colitis:
Primary Sclerosing Cholangitis (PSC)
Occurs in up to 3%
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Malignancy associated with Primary Sclerosing Cholangitis:
- Cholangiocarcinoma
- Colon cancer
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What is Hereditary Nonpolyposis Colorectal Cancer Syndrome (HNPCC) caused by?
Germline mutations in DNA mismatch repair (MMR) genes
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Hereditary Nonpolyposis Colorectal Cancer Syndrome diagnostic criteria:
Colorectal cancer involving at least 2 generations & at least one diagnosed before age 50
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What is Peutz-Jeghers Syndrome?
Hamartomatous polyps of the gut as well as mucocutaneous pigmentation changes
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What is Familial Adenomatous Polyposis (FAP) Syndrome?
Mutation of the APC gene resulting in dense colonic polyposis, mandibular osteomas, and universal colon cancer at a young age
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What is Turcot Syndrome?
A variant of FAP in which patients can also develop medulloblastoma, glioblastoma multiform, and hypertrophy of retinal pigmented epithelium.
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What is Cronkhite-Canada Syndrome?
GI polyposis, alopecia, cutaneous hyperpigmentation, malnutrition, and dystrophic fingernails.
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Progressive dysphagia to solid foods without weight loss:
Peptic stricture (associated with long-standing untreated GERD with esophagitis)
Treatment = esophageal dilation & maximal acid suppression (PPI>H2 antag.)
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CA 19-9
- Pancreatic cancer
- Cholangiocarcinoma
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AFP
Hepatocellular carcinoma
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