**Core Concept**
The patient's symptoms of productive cough with foul-smelling sputum, combined with a history of pancreatic insufficiency, suggest a diagnosis of cystic fibrosis (CF) or a similar condition. The presence of colorless, oxidase-positive colonies with a fruity aroma on agar is characteristic of Pseudomonas aeruginosa, a common pathogen in CF patients. The bacteria responsible for this infection likely produce enzymes that break down the patient's lung tissue and sputum, leading to the observed symptoms.
**Why the Correct Answer is Right**
Pseudomonas aeruginosa produces elastase, a zinc metalloprotease that breaks down elastin, a key component of lung tissue. The elastase enzyme is secreted by the bacteria and functions by cleaving the peptide bonds in elastin, leading to tissue damage and inflammation. The function of a GTP-binding protein involved in the elongation step of protein synthesis is most likely inhibited by the bacteria responsible for this boy's infection, as the bacteria's proteases, including elastase, can cleave and inactivate various cellular proteins, including those involved in protein synthesis.
**Why Each Wrong Option is Incorrect**
**Option A:** A CFTR protein lacking a phenylalanine in exon 10 at position 508 is the most common mutation associated with cystic fibrosis, but it is not directly related to the bacterial infection caused by Pseudomonas aeruginosa.
**Option C:** A GTP-binding protein similar to the one coupled with Ξ±2-adrenergic receptors is not directly involved in the pathogenesis of Pseudomonas aeruginosa infection.
**Option D:** A GTP-binding protein similar to the one coupled with Ξ²-adrenergic receptors is also not directly related to the bacterial infection caused by Pseudomonas aeruginosa.
**Clinical Pearl / High-Yield Fact**
Pseudomonas aeruginosa is a common pathogen in patients with cystic fibrosis and other chronic lung diseases, and its presence is often associated with a poor prognosis. The bacteria's production of elastase and other proteases contributes to the progression of lung disease and the development of complications such as bronchiectasis.
β Correct Answer: B. A GTP-binding protein involved in the elongation step of protein synthesis
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