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GI > Celiac Disease and CF > Flashcards

Celiac Disease and CF Flashcards

1
Q

Protein and carbohydrate assimilation may also be adversely impacted by disease states that reduce the absorptive surface area of the

A

Small intestine

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2
Q

This usually occurs via a selective loss of mature

A

Villus enterocytes

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3
Q

Perhaps the most common disorder in which such a change occurs is

A

Celiac disease

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4
Q

Results in an inappropriate immune response to a dietary constituent known as α-gliadin

A

Celiac disease

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5
Q

A constituent of gluten that is a key structural component of wheat and some other grains

A

α-gliadin

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6
Q

The most severe form of celiacs disease shows what type of mucosa in the small intestine?

A

Flat mucosa

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7
Q

This results in the total loss of intestinal

A

Villi

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8
Q

The site to which both brush border hydrolases and nutrient transporters are localized

A

Small intestine Villi

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9
Q

Patients with celiac’s disease may present with symptoms of impaired

A

Nutrient uptake

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10
Q

Celiac patients also hve undigested/absorbed nutrients remaining in the lumen that are then acted on by bacteria. This results in the symptoms of

-similar to lactose intolerance

A

Bloating and abdominal cramps

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11
Q

The treatment for patients with celiac disease is strict avoidance of foods containing

A

α-gliadin

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12
Q

Within a short time, exclusion of these substances from the diet results not only in the resolution of symptoms, but also restoration of the

A

Intestinal mucosa

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13
Q

What is the classic presentation of Celiac disease?

A

Chronic diarrhea, steatorrhea, and weight loss

-also iron and vitamin deficiencies

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14
Q

Affects women 1.5 times more than men

A

Celiacs disease

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15
Q

Celiac disease develops only in people that habe the

A

HLA-DQ2 or HLA-DQ8 Haplotype

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16
Q

These haplotypes expressed on antigen presenting cell surfaces can bind the deaminated gluten peptide found in

A

Wheat, Rye, and Barley

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17
Q

This triggers the abnormal

A

Immune response

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18
Q

The result is then the development of antibodies against

A

Gliadin, transglutaminase, and endomysin

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19
Q

Seen in 27-50% of patients

A

Diarrhea

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20
Q

May develop due to vitamin D deficiency and secondary hyperparathyroidism that can accompany Celiac disease

A

Osteopenia and osteoporosis

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21
Q

Celiac disease is strongly associated with

-develops secondary to antibodies against epidermal transglutaminase

A

Dermatitis herpetiformis

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22
Q

Celiac disease is far more common in patients with

A

Down Syndrome

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23
Q

Celiac disease increases the risk of other autoimmune disorders including

A

Thyroiditis and type 1 DM

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24
Q

The gold standard for diagnosing Celiac disease is

A

Duodenal biopsy

25
Q

Highly accurate to diagnose celiacs but not perfect

A

Serological testing

26
Q

The initial serologic test for celiacs is to test

A

Tissue glutaminase antibody (IgA tGT)

27
Q

What is the sensitivity and specificity of the Tissue glutaminase antibody (IgA tGT)

A

87% sensitive and 97% specific

28
Q

Another very accurate test which is 87% sensitive and 99% specific

A

Endomysial antibody (IgA EMA)

29
Q

Virtually all patients with celiac disease express

A

HLA-DQ2 or HLA-DQ8 heterodimers

30
Q

Is 100% sensitive, but only 57-75% specific

A

HLA typing

31
Q

Celiac disease can be virtually ruled out in patients who are negative for

A

HLA-DQ2 or HLA-DQ8

32
Q

May be useful in patients who instituted a gluten-free diet before evaluation in whom IgA-tGT and IgA EMA antibody levels may be low due to decreased disease activity

A

HLA typing

33
Q

The most widespread autosomal recessive disorder among the caucasian population

A

Cystic Fibrosis

34
Q

A life-limiting disease which involves multisystem dysfunction mainly in the respiratory tract, gastrointestinal system, pancreas, and reproductive organs

A

Cystic Fibrosis (CF)

35
Q

CF involves a mutation in

A

CFTR

36
Q

Mediates Cl- secretion and it is expressed on the apical surface of all exocrine epithelia that includes lung airways, pancreas, intestine, testis

A

CFTR

37
Q

Shown to regulate the secretion of bicarbonate, glutathione and a variety of other transport proteins and cellular processes

A

CFTR

38
Q

CFTR belong to which family of transporters?

A

ATP-Binding Cassette (ABC)

39
Q

CFTR is primarily regulated by cAMP signaling and

A

Protein phosphorylation

40
Q

Loss of function of CFTR affects all exocrine epithelia due to a failure to

A

Hydrate Macromolecules

41
Q

In the lung, CF results in

-causes chronic infection and inflammation of the local airways

A

Mucus retention

42
Q

In the pancreas, CF leads to decreased secretion and more acidic fluid causing precipitation of

A

Zymogens

43
Q

Falls into six groups according to their mechanism by which they affect the synthesis, traffic and function of CFTR

A

CFTR Mutations

44
Q

The most severe among the groups and results in no protein production

A

Class I

45
Q

Include the synthesis of a misfolded improperly leading to its degradation by the ubiquitin-proteasome pathway

A

Class II Mutations

46
Q

The most common mutation in CF is falls into class II and it is due to the deletion of

A

Phenylalanine at position 508

47
Q

Cause defects in the Cl− channel regulation

-One of the mutations is G551D.

A

Class III

48
Q

Acts as a potentiator by increasing the flow of Cl- ions through the activated CFTR

-Can be used to treat Class III

A

Ivacaftor

49
Q

Mostly due to missense mutations leading to correctly trafficked protein, but with low Cl- flow

A

Class IV mutations

50
Q

Results in reduced levels of, but otherwise normal CFTR

A

Class V Mutations

51
Q

Cause reduced stability of CFTR

A

Class VI

52
Q

The first four classes of CFTR mutations which are more severe than the last two classes lead to

A

Panreatic Insufficiency

53
Q

Pancreatic sufficient mutations

A

Classes V and VI

54
Q

Secrete digestive enzymes including zymogens into the acinar lumen

A

Pancreatic Acinar Cells

55
Q

This is accompanied with the secretion of Cl- and HCO3-
into the ductal lumen which move fluid with them to neutralize the acidic chyme emptied from the stomach into the duodenum and also increase the activity and solubility of these

A

Digestive Enzymes

56
Q

In CF, absence of bicarbonate secretion, hyperacidity, not only leads to the precipitation of the digestive enzymes, but also precipitation of

A

Bile Acids

57
Q

This precipitation of Bile Acids leads to the development of

A

Meconium Ileus

58
Q

Also associated with increased trypsinogen autoactivation into trypsin leading to activation of the activation of zymogens damaging the endocrine functions of the pancreas

A

Hyperacidity in CF

59
Q

Obstruction of ducts starts in utero and continues after birth releasing pancreatic proteins into the circulation, hence the immunoreactive trypsinogen (IRT) for newborn screening of

A

CF

GI (59 decks)

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