What is the most likely underlying condition?

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Pediatric Gi Nclex Practice Questions Questions

Question 1 of 5

What is the most likely underlying condition?

Correct Answer: C

Rationale: A newborn with respiratory distress, bilateral pleural effusions, dysmorphic features (hypertelorism, rotated ears, nuchal skin), and later juvenile myelomonocytic leukemia (JMML) suggests Noonan syndrome (Choice C). This RASopathy is associated with congenital heart defects, lymphatic anomalies (causing effusions), and a predisposition to JMML. Cockayne syndrome (Choice A) involves growth failure and photosensitivity, not JMML. Fanconi anaemia (Choice B) causes marrow failure and malformations but not this lymphatic picture. Omenn syndrome (Choice D) is an immunodeficiency, not matching these features. Noonan syndrome aligns with the presentation.

Question 2 of 5

The following statement is not true about urine culture:

Correct Answer: A

Rationale: Choice A is false; significant colony count for catheterized specimens is ≥50,000 CFU/mL, not 1,000 (IDSA). Choices B-E are true per standard guidelines.

Question 3 of 5

Most common side effects of inhaled glucocorticoids are oral thrush and dysphonia. The cause of oral thrush formation is:

Correct Answer: A

Rationale: Local immunosuppression from inhaled glucocorticoids allows Candida overgrowth, causing thrush (ATS). Other options (B-E) aren’t primary causes.

Question 4 of 5

During therapy for hypernatremic dehydration, the goal is to decrease the serum sodium by:

Correct Answer: A

Rationale: Sodium should drop by less than 12 mEq/L/day to avoid cerebral edema, per guidelines (AAP). Faster or slower (C-E) rates are suboptimal.

Question 5 of 5

All of the following are basic mechanisms of metabolic acidosis except:

Correct Answer: B

Rationale: Increased acid excretion corrects, not causes, metabolic acidosis (UpToDate). A, C-E are true mechanisms.

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