A 20-day old neonate presented with history of non-passage of meconium along with rectal prolapse, poor growth and delayed maturation. She has been hospitalized for the past 10 days due to respiratory tract infection. O/E Increased anteroposterior diameter of the chest Generalized hyperresonance Scattered coarse crackles Digital clubbing Protuberant abdomen Decreased muscle mass X ray abdomen was done. X ray abdomen was done. USG Abdomen ( Seen in antenatal records) All of the following drugs can be used in the treatment of the above-mentioned disease except: – Ivacaftor Dornase alpha Pulmozyme Mannitol
A 20-day old neonate presented with history of non-passage of meconium along with rectal prolapse, poor growth and delayed maturation. She has been hospitalized for the past 10 days due to respiratory tract infection. O/E Increased anteroposterior diameter of the chest Generalized hyperresonance Scattered coarse crackles Digital clubbing Protuberant abdomen Decreased muscle mass X ray abdomen was done. X ray abdomen was done. USG Abdomen ( Seen in antenatal records) All of the following drugs can be used in the treatment of the above-mentioned disease except: – Ivacaftor Dornase alpha Pulmozyme Mannitol
π‘ Explanation
**Core Concept**
The underlying condition described is **Cystic Fibrosis (CF)**, a genetic disorder characterized by abnormal **chloride transport** due to mutations in the **CFTR gene**, leading to thickened secretions and respiratory, gastrointestinal, and other systemic complications.
**Why the Correct Answer is Right**
The correct answer is the drug that does not fit with the treatment of CF. **Ivacaftor** is a **CFTR potentiator**, **Dornase alpha (Pulmozyme)** is an **enzymatic deoxyribonuclease** that breaks down DNA in sputum, making it less viscous. These are specific treatments for CF.
**Why Each Wrong Option is Incorrect**
**Option A:** Ivacaftor is used in CF treatment, specifically for patients with certain CFTR mutations.
**Option B:** Dornase alpha (Pulmozyme) is also used in CF to reduce sputum viscosity.
**Option D:** Mannitol, an **osmotic diuretic**, might be considered in the context of managing certain CF complications but is less directly related to CF treatment compared to the others, yet still not the most incorrect choice given the specific context of the question.
**Why Each Wrong Option is Incorrect (continued)**
Given the context, **Mannitol** is not typically used for the direct management of CF symptoms like the other options, which target the disease's pathophysiology more directly.
**Clinical Pearl / High-Yield Fact**
A key point in CF diagnosis is the presence of **respiratory and gastrointestinal symptoms** combined with **sweat test** results showing increased **sodium and chloride** levels, which is crucial for diagnosis.
**Correct Answer:** D. Mannitol
β Correct Answer: D. All of the above
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