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Flashcards in Pediatric GI Disease Cases Deck (20):

What is scalloping of the duodenal folds suggestive of?

Celiac disease


Does celiac disease have a lot of non-GI manifestations?

Yes. Most of them seem to due to malabsorption (such as delayed puberty, Fe-deficiency anemia, osteopenia)... but not all of them.


What genes have strong a strong association with celiac disease?

HLA DQ2, mostly.
Also HLA DQ8.


What's the main antigen in celiac disease?



Type of immune response in celiac disease?

Th1 with IFN-gamma production.


2 sequelae of celiac disease?

Increased intestinal permeablility -> may predispose to autoimmune diseases.


What are 3 antigens against which there are auto-antibodies for which we test for in celiac disease? For each, is IgG or IgA assayed?

Gliadin (AGA) - IgG and IgA
Endomysium (EMA) - IgA
Transglutaminase (TTG) - IgA


Is diagnosis of celiac based on serology or biopsy?

Depends on the country... but in the US, a biopsy is required for diagnosis.


3 histological features of celiac disease.

In the small bowel...
Villous atrophy.
Crypt hyperplasia.
Intra-epithelial lymphocytes.


Does the small bowel epithelium of celiac tend to recover when patient goes on a gluten-free diet?

Yes - and prognosis doesn't seem to be linked to the severity of the damage when diagnosed.


What are 2 diseases quite similar to eosinophilic esophagitis that affect other parts of the GI tract?

Allergic proctocolitis
Eosinophilic gastroenteritis


Is there non-allergic eosinophilic esophagitis? Cause? How does this contrast histologically from allergic esophagitis?

Yes. It can be caused by reflux, and responds to PPIs.
Allergic eosinophilic esophagitis has way more eosinophils than does non-allergic.


Who gets eosinophilic proctocolitis?

Mainly infants


What's the most common antigen in eosinophilic proctocolitis?

Cow's milk - either from infant's diet, or from mom's via breastmilk.


In terms of severity / treatability, how does eosinophilic gastroenteritis compare to esophagitis, proctolitis?
Where does it most often affect?

It's the most difficult to treat.
Affects various places in GI tract, antrum of stomach is most common.


Is eosinophilia of the GI tract always allergy?

No, and "allergies" don't always cause eosinophilia (e.g. celiac).


What genetic disorder mentioned can cause both inflammatory bowel disease and predisposition to infections (esp respiratory infections)?

X-linked chronic granulomatous disease (CGD) - probably a few other things could do this, too.


What's defective in CGD? How does this cause inflammatory bowel disease?

Defect in the ability of phagocytes to make reactive oxygen species -> cant kill bacteria / fungi as well.
It's thought that pathogens penetrate gut -> cause inflammation.


What disease should you think of if you see inflammatory bowel disease and can't find any plasma cells on biopsy?

X-linked agammaglobulinemia.


What can IgA deficiency mimic in the duodenum? In the colon?

Can mimic celiac in the duodenum.
IBD in the colon.