Chapter 33: Atopic, Rheumatic, and Immunodeficiency Disorders

Garzon Maaks: Burns’ Pediatric Primary Care, 7th Edition

MULTIPLE CHOICE

1. An 8-year-old child is diagnosed with systemic lupus erythematosus (SLE), and the child’s
parent asks if there is a cure. What will the primary care pediatric nurse practitioner tell the
parent?
a. Complete remission occurs in some children at the age of puberty.
b. Periods of remission may occur but there is no permanent cure.
c. SLE can be cured with effective medication and treatment.
d. The disease is always progressive with no cure and no remissions.
ANS: B
Periods of remission do occur in some children with SLE for unknown reasons, but there is no
permanent remission or cure. For some children with Juvenile Idiopathic Arthritis (JIA),
complete remission occurs at puberty.

2. The primary care pediatric nurse practitioner examines a child who has had stiffness and
warmth in the right knee and left ankle for 7 or 8 months but no back pain. The nurse
practitioner will refer the child to a rheumatology specialist to evaluate for what form of
juvenile idiopathic arthritis (JIA)?
a. enthesitis-related JIA.
b. oligoarticular JIA.
c. polyarticular JIA.
d. systemic JIA.
ANS: B
Oligoarticular JIA is characterized by mild, painless asymmetric joint involvement without
systemic symptoms. Enthesitis-related JIA involves arthritis of the lower limbs, especially the
hips, intertarsal joints, and sacroiliac joints, with swelling, tenderness, and warmth.
Polyarticular JIA involves 5 or more joints. Systemic JIA presents with systemic symptoms,
such as fever.

3. The primary care pediatric nurse practitioner is managing care for a child who has been
diagnosed with juvenile idiopathic arthritis (JIA) has a positive antinuclear antibody (ANA).
Which specialty referral is critical for this child?
a. Cardiology
b. Ophthalmology
c. Orthopedics
d. Pain management
ANS: B
An ophthalmology consultation is critical for children with JIA who have a positive ANA.
Uveitis occurs in up to 35% of children with JIA who have a positive ANA. Other specialists
may be consulted for specific symptoms.

4. The primary care pediatric nurse practitioner is prescribing ibuprofen for a 25 kg child
diagnosed with oligoarticular juvenile idiopathic arthritis (JIA. If the child will take 4 doses
per day, what is the maximum amount the child will receive per dose?
 a. 200 mg
b. 250 mg
c. 400 mg
d. 450 mg
ANS: B
The maximum dose is 40 mg/kg/day divided into 3 to 4 doses. 25 kg  40 mg = 1000/4 = 250
mg.

5. The parent of a school-age child who is diagnosed with oligoarticular juvenile idiopathic
arthritis (JIA) asks the primary care pediatric nurse practitioner what exercises the child may
do to help reduce symptoms. What will the nurse practitioner recommend?
a. Running
b. Swimming
c. Weights
d. Yoga
ANS: B
Swimming is an excellent exercise for children with juvenile idiopathic arthritis (JIA) because
water therapy and the use of heat or cold reduce pain and stiffness, unless they have severe
anemia or cardiac involvement.

6. A 12-year-old child is brought to the clinic with joint pain, a 3-week history of low-grade
fever, and a facial rash. The primary care pediatric nurse practitioner palpates an enlarged
liver 2 cm below the subcostal margin along with diffuse lymphadenopathy. An antinuclear
antibody (ANA) test is positive. Which test may be ordered to confirm a diagnosis of systemic
lupus erythematous (SLE)?
a. Anti-double-strand DNA antibodies
b. Anti-La antibodies
c. Anti-Ro antibodies
d. Anti-Sm antibodies
ANS: A
Anti-double-strand DNA antibodies are present in most people with SLE and are generally
exclusively seen in cases of SLE and not other diseases. Anti-SM antibodies are diagnostic of
SLE but are only seen in 30% of patients with systemic lupus erythematous (SLE).

7. The primary care pediatric nurse practitioner is reviewing the rheumatology plan of care for a
child who is diagnosed with systemic lupus erythematosus (SLE). Besides reinforcing
information about prescribed medications, what will the nurse practitioner teach the family to
help minimize flaring of episodes?
a. Have the child rest between activities.
b. Obtain regular ophthalmology exams.
c. Participate in low-impact exercises.
d. Use ultraviolet A (UVA) and ultraviolet B (UVB) sunscreen daily.
ANS: D
 Sunlight is a known trigger of SLE so patients should be advised to use a UVA and UVB
sunscreen both indoors and out. Resting between activities is recommended for children with
juvenile idiopathic arthritis (JIA). Children should participate in low-impact activities, but this
does not reduce the number of flares. Ophthalmology exams are recommended for children
with juvenile idiopathic arthritis (JIA).

8. An adolescent female reports poor sleep, fatigue, muscle and joint paint, and anxiety lasting
for several months. The primary care pediatric nurse practitioner notes point tenderness at
several sites. What will the nurse practitioner do next?
a. Evaluate the adolescent’s pain using a numeric pain scale.
b. Obtain ANA, CBC, liver function, and muscle enzymes tests.
c. Reassure the adolescent that this condition is not life-threatening.
d. Refer the adolescent to a rheumatologist for further evaluation.
ANS: D
Children with widespread musculoskeletal pain and painful point tenderness may have
fibromyalgia and should be referred. The Widespread Pain Index is used to define the degree
of pain. Laboratory studies are of little benefit when diagnosing fibromyalgia. Even though
children need reassurance that this disease is not life-threatening, this is not the next action.

9. A child has a fever and arthralgia. The primary care pediatric nurse practitioner learns that the
child had a sore throat 3 weeks prior and auscultates a murmur in the clinic. Which test will
the nurse practitioner order?
a. Anti-DNase B test
b. anti-streptolysin O (ASO) titer
c. Rapid strep test
d. Throat culture
ANS: B
This child has symptoms and a history consistent with ARF. The ASO titer peaks in 3 to 6
weeks and will confirm a recent strep infection. The anti-DNase B test will also confirm a
recent strep infection, but this doesn’t peak until 6 to 8 weeks after the initial infection. A
rapid strep test and throat culture do not differentiate the carrier state from a true infection.

10. The primary care pediatric nurse practitioner sees a child for follow-up care after
hospitalization for acute rheumatic Fever (ARF). The child has polyarthritis but no cardiac
involvement. What will the nurse practitioner teach the family about ongoing care for this
child?
a. Aspirin (ASA) is given for 2 weeks and then tapered to discontinue the
medication.
b. Prophylactic amoxicillin will need to be given for 5 years.
c. Steroids will be necessary to prevent development of heart disease.
d. The child will need complete bedrest until all symptoms subside.
ANS: A
ASA is given for arthritis for 2 weeks and then will be tapered. Children with ARF will need
penicillin prophylaxis, not amoxicillin. Steroids are sometimes used for symptomatic relief
but do not prevent chronic heart disease. Bed rest is indicated only when cardiac symptoms
occur.
11. An 8-year-old boy has a recent history of an upper respiratory infection and comes to the
clinic with a maculopapular rash on his lower extremities and swelling and tenderness in both
ankles. The pediatric nurse practitioner performs a UA, which shows proteinuria and
hematuria and diagnoses Henoch-Schönlein Purpura (HSP). What ongoing evaluation will the
nurse practitioner perform during the course of this disease?
a. Antinuclear antibody (ANA)titers
b. Blood pressure measurement
c. Chest radiographs
d. Liver function studies (LFTs)
ANS: B
Hypertension is a serious risk of HSP, so repeated BP measurement is indicated. ANA titers
are not measured with HSP. Chest radiographs are performed only if indicated. LFTs are not
indicated; the predominant risk is to the kidneys.

12. A 10-year-old child has a 1-week history of fever of 104°C that is unresponsive to
antipyretics. The primary care pediatric nurse practitioner examines the child and notes
bilateral conjunctival injection and a polymorphous exanthema, with no other symptoms. Lab
tests show elevated ESR, CRP, and platelets. Cultures are all negative. What will the nurse
practitioner do?
a. Begin treatment with intravenous methyl prednisone.
b. Consider IVIG therapy if symptoms persist one more week.
c. Order a baseline echocardiogram today and another in 2 weeks.
d. Reassure the child’s parents that this is a self-limiting disorder.
ANS: C
An echocardiogram should be obtained as soon as the diagnosis of Kawasaki disease (KD) is
established, as a baseline study, with subsequent studies in 2 weeks and in 6 to 8 weeks. This
child has fever and only two other symptoms, which may be consistent with atypical KD.
Atypical KD is more common in very young children and in children over 9 years of age, and
coronary artery involvement is found more frequently in children with atypical KD. Methyl
prednisone is given for children with IVIG-resistant disease. IVIG should be begun ideally in
the first 10 days of the illness. Although KD is a self-limiting disorder, the risk of coronary
artery involvement is high, so this must be evaluated and treated.

13. The primary care pediatric nurse practitioner is evaluating an 11-month-old infant who has
had three viral respiratory illnesses causing bronchiolitis. The child’s parents both have
seasonal allergies and ask whether the infant may have asthma. What will the nurse
practitioner tell the parents?
a. “Although it is likely, based on family history, it is too soon to tell.”
b. “There is little reason to suspect that your infant has asthma.”
c. “With your infant’s history of bronchiolitis, asthma is very likely.”
d. “Your infant has definitive symptoms consistent with a diagnosis of asthma.”
ANS: A
A genetic predisposition for the development of an IgE-mediated response to aeroallergens is
the strongest identifiable predisposing risk factor for asthma, but asthma is rarely diagnosed
before age 12 months due to the high rate of viral-induced bronchiolitis. The PNP should be
cautious about diagnosing asthma until wheezing without an association to viral illnesses
occurs. This infant has clear risk factors for asthma; however, bronchiolitis is not a known risk
factor.
14. The primary care pediatric nurse practitioner is examining a school-age child who has had
several hospitalizations for bronchitis and wheezing. The parent reports that the child has
several coughing episodes associated with chest tightness each week and gets relief with an
albuterol metered-dose inhaler. What will the nurse practitioner order?
a. Allergy testing
b. Chest radiography
c. Spirometry testing
d. Sweat chloride test
ANS: C
Spirometry testing is the gold standard for diagnosing asthma and is then used on a regular
basis to monitor, evaluate, and manage asthma. Allergy testing should be considered but is not
diagnostic of asthma. Chest radiography should not be routine. A sweat chloride test is used
based on history.

15. A school-age child who uses a short-acting beta2-agonist (SABA) and an inhaled
corticosteroid medication is seen in the clinic for an acute asthma exacerbation. After 4 puffs
of an inhaled short-acting B2-agonist (SABA) every 20 minutes for three treatments,
spirometry testing shows an FEV1 of 60% of the child’s personal best. What will the primary
care pediatric nurse practitioner do next?
a. Administer an oral corticosteroid and repeat the three treatments of the inhaled
SABA.
b. Admit the child to the hospital for every 2 hour inhaled SABA and intravenous
steroids.
c. Give the child 2 mg/kg of an oral corticosteroid and have the child taken to the
emergency department.
d. Order an oral corticosteroid, continue the SABA every 3 to 4 hours, and follow
closely.
ANS: D
Children with an incomplete response (FEV1 between 40% and 69% of personal best) should
be given oral steroids and instructed to continue the SABA every 3 to 4 hours with close
follow-up. Hospitalization is not necessary unless severe distress occurs. An FEV1 less than
40% after treatment indicates a need to be seen in the ED.

16. A child who has been diagnosed with asthma for several years has been using a short-acting
B2-agonist (SABA) to control symptoms. The primary care pediatric nurse practitioner learns
that the child has recently begun using the SABA two or three times each week to treat
wheezing and shortness of breath. The child currently has clear breath sounds and an FEV1 of
75% of personal best. What will the nurse practitioner do next?
a. Add a daily inhaled corticosteroid.
b. Administer 3 SABA treatments.
c. Continue the current treatment.
d. Order an oral corticosteroid.
ANS: A
 The child is showing a need to step up treatment based on the frequency of symptoms, greater
than twice each week. The PNP should order an inhaled corticosteroid maintenance
medication to control symptoms and reduce the need for a SABA. The child is not having an
acute exacerbation, so does not need 3 SABA treatments. Oral corticosteroids are given for
moderate obstruction, <70%.

17. An adolescent who has asthma and severe perennial allergies has poor asthma control in spite
of appropriate use of a short-acting beta2-agonist (SABA) and a daily high-dose inhaled
corticosteroid. What will the primary care pediatric nurse practitioner do next to manage this
child’s asthma?
a. Consider daily oral corticosteroid administration.
b. Order an anticholinergic medication in conjunction with the current regimen.
c. Prescribe a LABA/inhaled corticosteroid combination medication.
d. Refer to a pulmonologist for omalizumab therapy.
ANS: D
Children older than 12 years who have moderate to severe allergy-related asthma and who
react to perennial allergens may benefit from omalizumab as a second-line treatment when
symptoms are not controlled by ICSs. The PNP should refer children to a pulmonologist for
such treatment. Daily oral corticosteroid medications are not recommended because of the
adverse effects caused by prolonged use of this route. Anticholinergic medications are
generally used for acute exacerbations during in-patient stays or in the ED. A LABA/ICS
combination will not produce different results.

18. An adolescent who has exercise-induced asthma (EIA) is on the high school track team and
has recently begun to practice daily during the school week. The adolescent uses 2 puffs of
albuterol via a metered-dose inhaler 20 minutes before exercise but reports decreased
effectiveness since beginning daily practice. What will the primary care pediatric nurse
practitioner do?
a. Counsel the adolescent to decrease the number of practices each week.
b. Increase the albuterol to 4 puffs 20 minutes prior to exercise.
c. Order a daily inhaled corticosteroid medication.
d. Prescribe cromolyn sodium in addition to the albuterol.
ANS: C
Children with EIA should use 2 puffs of a B2-agonist and/or cromolyn MDI 15 to 30 minutes
prior to exercise, but, since tolerance may develop if a B2-agonist is used more than a few
times a week, it should not be used as a controller monotherapy. Those who exercise regularly
should use an ICS as a controller medication. Patients with asthma should be encouraged to
exercise to improve overall health. Increasing the albuterol dose will not overcome the
tolerance. And ICS is a preferred controller medication.

19. A school-age child with asthma is seen for a well child checkup and, in spite of “feeling fine,”
has pronounced expiratory wheezes, decreased breath sounds, and an FEV1 less than 70% of
personal best. The primary care pediatric nurse practitioner learns that the child’s parent
administers the daily medium-dose ICS but that the child is responsible for using the short-
acting beta2-agonist (SABA). A treatment of 4 puffs of a SABA in clinic results in marked
improvement in the child’s status. What will the nurse practitioner do?
a. Have the parent administer all of the child’s medications.
b. Increase the ICS medication to a high-dose preparation.
 c. Reinforce teaching about the importance of using the SABA.
d. Teach the child and parent how to use home PEF monitoring.
ANS: D
Home PEF monitoring is useful for children to identify when symptoms are worsening. This
child does not appear to notice the presence of airway tightness or wheezing and so might
benefit from PEF monitoring to know when to use the SABA. School-age children should be
learning how to manage their chronic disease, so having the parent administer all medications
is not the best choice, especially since use of the SABA is still dependent on the child’s report
of symptoms. Since the child responded well to administration of the SABA, increasing the
dose of Inhaled corticosteroids (ICS) should not be done unless better management is not
effective. Reinforcing the teaching is part of the plan but, unless the child is aware of
symptoms, may not occur.

20. The parent of a school-age child reports that the child usually has allergic rhinitis symptoms
beginning each fall and that non-sedating antihistamines are only marginally effective,
especially for nasal obstruction symptoms. What will the primary care pediatric nurse
practitioner do?
a. Order an intranasal corticosteroid to begin 1 to 2 weeks prior to pollen season.
b. Prescribe a decongestant medication as adjunct therapy during pollen season.
c. Recommend adding diphenhydramine to the child’s regimen for additional relief.
d. Suggest using an over-the-counter intranasal decongestant.
ANS: A
Intranasal corticosteroids are a key component in long-term therapy to manage symptoms
associated with allergic rhinitis (AR). These should be begun 1 to 2 weeks prior to the
beginning of pollen season. Decongestants are not recommended for long-term use because of
side effects. Diphenhydramine causes daytime drowsiness.

21. A 4-month-old infant has a history of reddened, dry, itchy skin. The primary care pediatric
nurse practitioner notes fine papules on the extensor aspect of the infant’s arms, anterior
thighs, and lateral aspects of the cheeks. What is the initial treatment?
a. Moisturizers
b. Oral antihistamines
c. Topical corticosteroids
d. Wet wrap therapy
ANS: A
Moisturization is the first-line therapy to interrupt the itch-scratch-itch cycle. Oral
antihistamines are used mostly to allow sleep during nighttime pruritus. Topical
corticosteroids are used if moisturization is not effective. Wet wrap therapy is used to treat
flares with recalcitrant disease.

22. The primary care pediatric nurse practitioner is performing a well-baby checkup on a 6-
month-old infant and notes a candida diaper rash and oral thrush. The infant has had two ear
infections in the past 2 months and is in the 3rd percentile for weight. What will the nurse
practitioner do?
a. Order a CBC with differential and platelets and quantitative immunoglobulins.
b. Order candida and pneumococcal skin tests and lymphocyte surface markers.
c. Refer the infant to an immunologist for evaluation of immunodeficiency.
d. Refer the infant to an otolaryngologist to evaluate recurrent otitis media.
ANS: A
Infants with warning signs of immunodeficiency, such as recurrent infections, skin infections,
and oral thrush, should be evaluated. The initial step is to order a CBC with differential,
platelets, and immunoglobulins. If this is not helpful, referral to an immunologist for further
testing, such as candida and pneumococcal skin tests and lymphocyte surface markers, is
warranted. Referral to an otolaryngologist is not indicated.