ATI RN
Pediatric Endocrine Nursing Questions Questions
Question 1 of 5
HbAâ‚C values may be spuriously elevated in
Correct Answer: C
Rationale: Iron deficiency anemia (C) increases HbAâ‚C by altering RBC turnover.
Question 2 of 5
Inborn errors of metabolism (IEM) are hereditary biochemical disorders caused by single-gene mutations that encode specific proteins. The following are the common characteristics of IEM EXCEPT
Correct Answer: B
Rationale: Most IEMs are autosomal recessive (A), require early diagnosis (C), present after birth (D), and vary in severity (E). However, later symptom onset often indicates milder disease, not greater severity, making B the exception.
Question 3 of 5
A 5-year-old mentally retarded girl presented with repeated convulsions and abnormal EEG finding. On examination, she had a light skin, small head, and exaggerated reflexes. Of the following, the MOST appropriate method to confirm the diagnosis is
Correct Answer: D
Rationale: Plasma phenylalanine concentration confirms PKU in this classic presentation (mental retardation, seizures, light skin, microcephaly), offering definitive diagnosis over screening (A, C) or secondary tests (D). Flow cytometry (B) is unrelated.
Question 4 of 5
The MOST appropriate method to confirm the diagnosis of tyrosinemia type 1 is by elevated level of
Correct Answer: D
Rationale: Serum succinylacetone is the specific diagnostic marker for tyrosinemia type 1, reflecting fumarylacetoacetate hydrolase deficiency, more definitive than α-fetoprotein (A), tyrosine (B), methionine (C), or ALA (E).
Question 5 of 5
The MOST appropriate treatment of homocystinuria is
Correct Answer: D
Rationale: Vitamin B6 (pyridoxine) is the primary treatment for homocystinuria, reducing homocysteine levels in responsive patients, more foundational than betaine (A), folic acid (B), vitamin C (C), or methionine restriction (E).