ATI RN
Pediatric Endocrine Nursing Questions Questions
Question 1 of 5
Mucopolysaccharidoses (MPS) affect many solid organs, the brain is spared in
Correct Answer: D
Rationale: MPS VI (Maroteaux-Lamy) primarily affects somatic tissues (skeleton, heart) with minimal brain involvement, unlike other MPS types with significant neurological impact.
Question 2 of 5
Matching: For each inborn error of amino acid metabolism, select the correct urine odor - Trimethylaminuria
Correct Answer: D
Rationale: Trimethylaminuria causes a fishy odor (not listed but known), not matching A, B, or C; options incomplete, assumed D as placeholder.
Question 3 of 5
Matching: Match the disease with its enzyme - β-Glucosidase
Correct Answer: B
Rationale: Gaucher disease results from β-glucosidase (glucocerebrosidase) deficiency, causing glucocerebroside accumulation.
Question 4 of 5
Of the following physical findings found in some patients with hyperlipidemia, which one is most likely to be found in a 17-year-old boy with heterozygous familial hypercholesterolemia?
Correct Answer: D
Rationale: Heterozygous familial hypercholesterolemia often causes tendon xanthomas, especially Achilles, in adolescents/young adults.
Question 5 of 5
The lipid storage diseases are a group of disorders that result from specific enzymatic deficiencies. Each statement about these disorders is true EXCEPT
Correct Answer: B
Rationale: Diagnosis typically uses leukocyte enzyme assays or genetic testing, not liver biopsy, which is invasive and less common.