Small Intestine

Question 1

Answer 1

Abetalipoproteinemia

-problem with TG absorption results in low TG/cholesterol, vitamin E deficiency and steatorrhea

-path is diagnostic if lipid laden enterocytes

Question 2

What is agammaglobulinemia?

Answer 2

no B cells, i.e. no plasma cells

presents with baby boy infants with recurrent sinus infections and diarrhea (w/ giardia)

Question 2

What are the GI manifestations of CVID?

Answer 2

-IBD like disease
-pernicious anemia
-SIBO
-protein wasting enteropathy
-recurrent GI infections

Question 2

How do you diagnose Whipple’s disease?

Answer 2

Pathology from D3/jejunum with PAS (+) foamy macrophages

Question 2

How do you diagnose Whipple’s disease?

Answer 2

Pathology from D3/jejunum with PAS (+) foamy macrophages

Question 3

What are the GI and non-GI manifestations of Whipple’s disease?

Answer 3

Non-GI
-CNS
-cardia (mycarditis)
-arthralgias

GI
-diarrhea, weight loss, occult GI bleeding, protein-loosing enteropathy

Question 3

How do you diagnose Whipple’s disease?

Answer 3

Pathology from D3/jejunum with PAS (+) foamy macrophages

Question 4

How are folate and B12 levels different in celiac disease, topical sprue and SIBO? what about crypto?

Answer 4

CD (proximal small bowel)- low folate, normal B12

tropical sprue (distal small bowel)- low folate, low b12

SIBO- high folate, low B12

crypto- terminal ileum, low B12 (think HIV/AIDS)

Question 5

In celiac disease which EIM improve with GFD?

Answer 5

Question 6

What is the diagnostic algorithm for celiac disease?

Answer 6

Question 7

What condition is this?

Answer 7

celiac disease

Question 8

What condition is this?

Answer 8

celiac disease

Question 9

What condition is this?

Answer 9

dermatitis herpetaformis

Question 10

Low B12, high folate = what condition?

Answer 10

SIBO

Question 11

Key Features of SIBO?

Answer 11

Question 12

Indications for small bowel transplant in SBS

Answer 12

Question 13

What are common small bowel tumors and their features?

Answer 13

• adenocarcinoma (most common proximal SB lesion)
• carcinoid (common distal SB lesion)
  -lymphoma (enteropathy associated Tcell or Bcell from camplyobacter, mantle cell, folicular, burkets)
  -PTLD - EBV associated highest risk in sb transplant then lung
  -GIST- tyrosine kinase, CD117 positive

Question 14

What are the differences between carcinoid and systemic mastocytosis?

Answer 14

both have flushing, palpitations

systemic mastocytosis- hepatomegaly, urticarial reaction with scratching the skin- get a small bowel biopsy to confirm diagnosis

Question 15

Answer 15

glucagonoma
classic rash known as necrolytic migratory erythema and glucose intolerance/diabetes.

Question 16

What supplementation is needed for extensive ileal resection +/- ostomy vs enterocolonic anastamosis

Answer 16

Vitamin B12, selenium, fat soluble vitamins
If enterocolonic anastamosis then, no need for paraenteral fluids
if ostomy, then will need paraenteral fluids

Question 17

Answer 17

Primary intestinal lymphangiectasia (PIL) is a rare disorder characterized by dilated intestinal lacteals resulting in lymph leakage into the small bowel lumen and responsible for protein-losing enteropathy leading to lymphopenia, hypoalbuminemia and hypogammaglobulinemia.

Question 18

Describe the different types of bile acid malabsorption.

Answer 18

Type I BAM related to primary ileal disease; ileum is unable to reabsorb bile salts

Type II BAM related to idiopathic BAM often seen in irritable bowel syndrome (IBS); idiopathic upregulation of bile acid synthesis

Type III BAM - continuous leakage of bile salts after CCY overwhelm ileal absorptive capacity

Type IV BAM - medication induced increased bile acid synthesis (ex. metformin use)

Question 19

Treatment for dermatitis herpetiformis?

Answer 19

dapsone

Question 20

IMO vs SIBO

Answer 20

presence of preceding chronic constipation (rather than diarrhea) makes intestinal methanogen overgrowth more likely, as methanogenic flora are associated with chronic constipation.

Question 21

how do you treat topical sprue?

Answer 21

doxycycline x 3 to 6 months

Question 22

first line and second line therapy for mestastatic GIST

Answer 22

1. imatinib
2. sunitinib

don’t give chemo or radiation

Question 23

What is the mechanism for bile salt diarrhea in IBS-D?

Answer 23

Type II BAM is now considered to be related to reduced production of fibroblast growth factor 19 (FGF-19) by ileal enterocytes and resulting abnormal feedback inhibition of hepatic bile acid synthesis by FGF-19. FGF-19 is normally generated by ileal epithelial enterocytes cells in response to bile acid resorption.

Question 24

What do you do when the serologic testing and duodenal histology are discordant in celiac disease?

Answer 24

Either:
check an alternative serologic test such as anti–endomysial-IgA or anti-deamidated gliadin peptide-IgG antibodies
or
measure human leukocyte antigen DQ2/DQ8

Question 25

small intestinal diaphragm disease

Answer 25

small bowel strictures secondary to the longstanding use of nonsteroidal anti-inflammatory drugs (NSAIDs). Patients may present with abdominal pain, iron deficiency anemia, and subacute small bowel obstruction.

Question 26

What is this lesion? What are the clinical signs, serological markers, and best functional testing?

Answer 26

Small bowel NET
Clinical symptoms- anemia, abdominal pain, bowel obstruciton (NOT carcinoid)
serology- elevated chromogranin a
Functional testing- gallium-68 dotatate PET (>octreotide)

Question 27

What are the types of refractory celiac disease?

Answer 27

Type 1- IEL
Tyle 2- abnormal IEL with loss of CD3 and CD8 expression (higher risk for lymphoma) - detected by flow cytometry of T cells

Question 28

HIV, diarrhea
small bowel bx with PAS+ macrophages, +AFB
lung lesions

Answer 28

disseminated MAC infection
treatment is azithromycin, rifampin, and ethambutol.

Question 29

Multiple myeloma is associated with which GI condition?

Answer 29

AL amyloid
deposits in the muscularis mucosa around blood vessels and nerves
can cause anemia, perforation

Question 30

Which drugs mimic celiac disease? What about NSAIDs?

Answer 30

Clinical scenario: serologically negative enteropathy (villous atrophy and inflammation but negative celiac serologies).
-Some patients with celiac disease may have negative celiac serologies
-medication induced: Mycophenolate mofetil; olmesartan
-NSAIDs- can cause increased IEL but normal villi