A nurse is caring for a 1-year-old male toddler in an outpatient orthopedist’s office.
Which findings are consistent with osteogenesis imperfecta in this toddler? Select all that apply.
Non-displaced fibula fracture
Blue sclera
Grey teeth
Decreased trabecular and cortical bone volume
Right wrist swelling and limited movement
Capillary refill less than 3 seconds
History of fall at home
Correct Answer : A,B,C,D,E
Choice A rationale: Non-displaced fibula fractures with minimal trauma are common in children with osteogenesis imperfecta due to compromised bone matrix integrity. Type I collagen mutations lead to defective osteoid formation, resulting in brittle bones with reduced tensile strength and elasticity. These children experience frequent fractures even with normal play or mild falls, supporting the diagnosis of osteogenesis imperfecta, which causes microarchitectural failure in long bones and cortical thinning.
Choice B rationale: Blue sclera is a hallmark of osteogenesis imperfecta caused by reduced collagen thickness in the sclera, allowing underlying choroidal veins to show through. This phenotypic finding results from impaired Type I collagen assembly, affecting connective tissue transparency and tensile properties. It is often present in milder forms of the disease and supports clinical suspicion even in infants or toddlers. The presence of blue sclera is highly specific in connective tissue disorders like osteogenesis imperfecta.
Choice C rationale: Grey teeth, or dentinogenesis imperfecta, arise from abnormal dentin structure due to collagen Type I defect, leading to translucency and discoloration. The enamel may chip off easily due to poor underlying support, contributing to rapid tooth wear. Dental manifestations are prominent in osteogenesis imperfecta and correlate with systemic skeletal fragility. The altered mineralization and dentin structure result in brittle, opalescent teeth characteristic of the disease phenotype.
Choice D rationale: Bone biopsy showing decreased trabecular and cortical bone volume confirms defective bone formation consistent with osteogenesis imperfecta. The disorder impairs osteoblast function and collagen synthesis, leading to low bone mass and microstructural failure. Reduced bone density increases fracture risk and can be visualized through histopathologic examination. Bone architecture is crucial in determining mechanical integrity, and its compromise reflects the disease’s fundamental pathophysiology.
Choice E rationale: Wrist swelling and limited movement, in the absence of a known injury, suggests spontaneous fracture or minor trauma-induced injury typical in osteogenesis imperfecta. Frequent unexplained musculoskeletal pain, localized edema, and functional limitation are common presentations. Children with this disorder often experience repeated injuries with minimal external force due to structurally compromised bone. These symptoms demand imaging to confirm fracture presence and disease progression.
Choice F rationale: Capillary refill less than 3 seconds indicates normal peripheral perfusion and is not specific to osteogenesis imperfecta. Circulatory status remains intact in this disorder, which primarily affects the skeletal system. Unless there are concurrent vascular complications or trauma-related circulatory issues, normal capillary refill does not provide diagnostic evidence of connective tissue abnormalities.
Choice G rationale: A history of fall is not diagnostic of osteogenesis imperfecta. While trauma may provoke fracture in both healthy and affected children, the key distinction lies in fracture frequency and severity relative to injury mechanism. Falls are common in toddlers; the disease manifests when even minor trauma results in disproportionate injury, which must be correlated with other clinical signs and biopsy findings
Nursing Test Bank
Naxlex Comprehensive Predictor Exams
Related Questions
Correct Answer is ["B","C","D","G"]
Explanation
Fine Motor Skills
- Rotates utensils to bring them to their mouth ✅ Age-appropriate. At 2 years, children should be able to self-feed with utensils, though messy. Rotating utensils shows intentional use, which is a typical fine motor skill.
? Gross Motor Skills
- Walks independently ✅ Normal. Independent walking is typically achieved by 12–15 months.
- Scoots on bottom up and down stairs ⚠️ Potential delay. By age 2, toddlers typically attempt to walk up and down stairs with assistance or by holding onto rails. Scooting may suggest immature coordination or caution, although it can also reflect personality or lack of opportunity to practice.
?️ Language Skills
- Uses one-word sentences, says five words ⚠️ Likely delayed. By age 2, children typically say at least 50 words and begin combining two words (e.g., “more juice”). Using only five words and relying on one-word sentences may signal a language delay.
? Play/Social Skills
- Participates in parallel play with siblings ✅ Typical. Parallel play (playing near others without interaction) is developmentally appropriate for 2-year-olds.
- Does not notice when others are upset ⚠️ Possible social-emotional delay. By age 2, children start showing early empathy—comforting others or reacting to distress. Lack of awareness could indicate delay in emotional or social development.
? Physical Growth
- Height: 86.4 cm (34 in) ✅ Normal. Within expected growth range for a 2-year-old boy.
- Weight: 11.3 kg (24.9 lb) ✅ Normal. Also within expected percentiles for age.
?? External Concern
- Daycare teachers express concern ⚠️ Important indicator. Teachers observe children in peer settings and may detect delays not seen at home. Their concerns often reflect valid developmental differences.
? Summary
The main red flags suggesting developmental delay:
- Limited expressive language (five words only).
- Lack of empathy or social awareness.
- Unusual gross motor strategy for stairs.
- External concerns from daycare providers.
Correct Answer is D
Explanation
Choice A rationale
Applying a dry gauze dressing twice per day is not the standard of care for a hypospadias repair. The surgical site is often left exposed to the air to promote healing and prevent infection, as moist dressings can create an ideal environment for bacterial proliferation. This approach minimizes friction and allows for easy observation of the incision for signs of complication, such as swelling or redness.
Choice B rationale
Hourly measurements of urinary output are excessive and not typically required for a stable postoperative hypospadias repair patient. While monitoring output is important, it can usually be done less frequently. The focus is on ensuring a steady stream of urine, which indicates patency of the urinary tract and stent, but continuous hourly monitoring is not standard practice and can be disruptive to the infant's rest and recovery.
Choice C rationale
Offering a 6-month-old infant 12 to 18 ounces of fruit juice daily is not appropriate. The American Academy of Pediatrics recommends avoiding fruit juice for infants under 1 year of age. This is because juice can displace more nutritious breast milk or formula and may contribute to dental caries and excessive weight gain due to its high sugar content and lack of fiber.
Choice D rationale
Avoiding a tub bath until the stent is removed is a crucial instruction to prevent infection and protect the surgical site. Submerging the incision in water before it has healed can introduce bacteria from the water into the wound. This can lead to serious infections and complications, such as wound dehiscence. Sponge baths are a safer alternative to maintain hygiene during this period.
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