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10 Cards in this Set
- Front
- Back
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MECHANISMS: Cystic Fibrosis
(Intestinal effects) |
Absence of epithelial Cystic Fibrosis Transmembrane conductance Regulator (CFTR)
-> Defective intestinal Cl- ion secretion -> Interference with bicarb, Na+, and water secretion -> Defective luminal hydration -> Formation of intraductal concretionsDuct obstruction = Nutrient malabsorption |
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Tx: Cystic Fibrosis
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Oral enzyme supplementation
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MECHANISMS: Coeliac Disease/Coeliac Sprue/Gluten-sensitive enteropathy
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Gluten hypersentivity
Eat gluten (wheat, rye, barley) -> Gluten digested by luminal & brush border enzymes, into amino acids/peptides (inc. 33-amino acid α-gliadin) -> Gliadin peptides triggers Epithelial expression of IL-15 -> Cytotoxic response (CD4 T-cells) = Enterocyte destruction CD4 activation -> Additional cytokine response (B-cells) |
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Coeliac Disease:
Pathological changes |
Intraepithelial infiltration of mucosa by inflammatory lymphocytes + plasma cells
Villous atrophy Crypt atrophy |
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Coeliac Disease:
Clinical features |
Adults: Present most commonly between ages 30-60
2-3x more common in women (due to menstration?) Usually escapes attention (atypical presentation) Some have silent disease (Dx: Serology/villous atrophy) |
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Coeliac Disease:
Adult symptoms |
Anemia
Chronic diarrhoea Bloating Chronic fatigue |
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Coeliac Disease:
Classical childhood symptoms |
Age 6-24mths
Irritability Abdominal distention Anorexia Chronic diarrhoea Failure to thrive Weight loss or muscle wasting |
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Coeliac Disease:
Non-classical childhood symptoms |
Older ages
Abdominal pain Nausea & Vomiting Bloating Constipation Arthritis Joint pain Seizure disorders Stomatitis (mocous membrane in mouth) Iron deficiency anaemia Pubertal delay Short stature |
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Coeliac Disease: Dx
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Sensitive test:
IgA to tissue transglutaminase IgA/IgG to deamidated gliadin Highly specific, not sensitive: Anti-endomysial Ab If serology suggestive, confirm via: Biopsy |
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Coeliac Disease: Complications
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Most common: Enteropathy-associated T-cell lymphoma
Failure to stick to complete gluten-free diet = most common cause of recurrent symptoms Most patients with this disease do well |
