Page 914 - Saunders Comprehensive Review For NCLEX-RN

Priority Concepts: Clotting; Safety
Reference: McKinney et al. (2018), p. 1133.

304. Answer: 2

Rationale: β-Thalassemia is an autosomal recessive disorder characterized by the
reduced production of 1 of the globin chains in the synthesis of hemoglobin (both
parents must be carriers to produce a child with β-thalassemia major). This disorder
is found primarily in individuals of Mediterranean descent. Options 1, 3, and 4 are
incorrect.
Test-Taking Strategy: Focus on the subject, the child at greatest risk for β-
thalassemia major. Think about the pathophysiology of the disorder. Remember that
this disorder occurs primarily in individuals of Mediterranean descent.
Level of Cognitive Ability: Applying
Client Needs: Physiological Integrity
Integrated Process: Teaching and Learning
Content Area: Pediatrics: Hematological
Health Problem: Pediatric-Specific: Anemias
Priority Concepts: Gas Exchange; Perfusion
Reference: McKinney et al. (2018), pp. 1128-1129.

305. Answer: 4

Rationale: β-Thalassemia is an autosomal recessive disorder characterized by the
reduced production of 1 of the globin chains in the synthesis of hemoglobin (both
parents must be carriers to produce a child with β-thalassemia major). The major
complication of long-term transfusion therapy is hemosiderosis. To prevent organ
damage from too much iron, chelation therapy with either deferasirox or
deferoxamine may be prescribed. Deferoxamine is classified as an antidote for acute
iron toxicity. Dalteparin is an anticoagulant used as prophylaxis for postoperative
deep vein thrombosis. Meropenem is an antibiotic. Metoprolol is a beta blocker used
to treat hypertension.
Test-Taking Strategy: Focus on the subject, chelation therapy. Specific knowledge
regarding the antidote for iron toxicity is needed to answer this question. One way
to remember this is to look at the prefix in the generic name of the medication used
to treat iron overdose. Remember to associate defer- and removal of iron.
Level of Cognitive Ability: Analyzing
Client Needs: Physiological Integrity
Integrated Process: Nursing Process—Planning
Content Area: Pediatrics: Hematological
Health Problem: Pediatric-Specific: Anemias
Priority Concepts: Clinical Judgment; Gas Exchange
Reference: McKinney et al. (2018), pp. 1128-1129.

306. Answer: 4

Rationale: Sickle cell crises are acute exacerbations of the disease, which vary

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