Robbie Jenkins is a four year old boy growing up in Blackwood. He is small for h
ID: 3517643 • Letter: R
Question
Robbie Jenkins is a four year old boy growing up in Blackwood. He is small for his age and seems to be growing slowly compared to his friends. Recently, he has been complaining of abdominal pain and he sometimes has diarrhoea. The stool that he passes is watery and floats in the toilet bowl. It smells particularly bad. His parents take him to the doctor. The doctor takes a blood sample and has it analysed for "antibodies to tissue transglutaminase" (tTG or TG2). The results are positive. Robbie then undergoes an endoscopy in Flinders Medical Centre. He is sedated and a fibre-optic endoscope is passed down his oesophagus, through his stomach and into his small intestine. It is noted that the villi in his small intestine do not have the normal finger-like shape; they have very little depth so that the mucosa looks almost flat. These are all typical signs and symptoms of coeliac disease. His parents are told to change his diet to avoid gluten. They talk to a nutritionist and make the appropriate changes and his symptoms and signs improve over the next 2 months. (a) What is gluten? (1 mark)
(b) Where does tissue transglutaminase come from? (1 mark)
(c) What does a "transglutaminse" enzyme do (it's primary role)? (1 mark)
(d) Which 3 cereal grains that are most common in the Australia diet contain sufficient gluten to trigger exacerbations of coeliac disease? (1 mark)
(e) What is gliadin? (1 mark)
(f) Why is Robbie failing to grow at a normal rate? (1 mark)
(g) Why do his stools float in the toilet bowl and smell so bad? (1 mark) (h) Why does Robbie have frequent bouts of abdominal pain? (1 mark)
(i) Why does Robbie have diarrhoea? (1 mark)
(j) Most of us eat lots of gluten and have lots of tissue transglutaminase, yet we don't have celiac disease. There are two specific serotype of an antigen presenting protein (an HLA) that greatly increase someone's risk of developing Coeliac Disease. Which HLA form are they? (1 mark)
Explanation / Answer
Answer-a-gluten is the protein found in wheat,rye,barley and oats.
Answer-b-.tissue transforming see is an enzyme that is present in intracellular as well as extracellular space.it is present in many organs like heart,lung, kidney,etc.it is important in the pathogenesis of the coeliac disease.
Answer-c-transglutaminase play key role in the pathogenesis of coeliac disease.it is an deamination enzymes,that delaminate the gluten and in pathologic condition it overrides the immune response to this gluten.
Answer-d-wheat,barley and oats are sufficient to cause exacerbation of coeliac disease.
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