Pediatric Examination and Board Review (242 page)

Asbury AK, Cornblath DR. Assessment of current diagnostic criteria for Guillain-Barré syndrome.
Ann Neurol.
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Sladky JT, Ashwal S. Inflammatory neuropathies. In: Swaiman KF, Ashwal S, Ferriero DM, eds.
Pediatric Neurology
:
Principles
&
Practice.
4th ed. Philadelphia, PA: Mosby; 2006:1919.

Smith SA, Ouvrier R. Peripheral neuropathies. In: Swaiman KF, Ashwal S, Ferriero DM, eds.
Pediatric Neurology
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Principles
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Practice.
4th ed. Philadelphia, PA: Mosby; 2006:1887.

Swaiman KF. Neurologic examination after the newborn period until 2 years of age. In: Swaiman KF, Ashwal S, Ferriero DM, eds.
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Chapter 17

RHEUMATOLOGY

CASE 140: A 5-YEAR-OLD BOY WITH FEVERS AND JOINT SWELLING

A 5-year-old previously healthy boy has had a daily spiking fever up to 104°F (40°C) in the evenings for 4 weeks. His temperature is normal between spikes. When febrile, the child is irritable, has malaise, and does not want to play or move. He is often active and playful when afebrile. He appears stiff in the morning. His mother is concerned that his finger and knee joints appear swollen.

SELECT THE ONE BEST ANSWER

1.
This child’s diagnosis is most likely

(A) rheumatic fever

(B) systemic arthritis

(C) periodic fever syndrome

(D) Kawasaki disease (KD)

(E) systemic lupus erythematosus (SLE)

2.
Which of the following laboratory results is not expected in this disease?

(A) decreased platelet count

(B) increased white blood cell count

(C) decreased hemoglobin

(D) increased erythrocyte sedimentation rate

(E) negative antinuclear antibody (ANA)

3.
Which of the following is true about the skin rash in this entity?

(A) it is evanescent, salmon colored, and often accompanies the fever

(B) biopsy shows a leukocytoclastic vasculitis

(C) it may start out with pustular lesions

(D) the lesions frequently ulcerate

(E) in some patients, the rash is fixed, lasting up to 3 days

4.
Commonly described extra-articular involvement in this disease includes

(A) eye inflammation

(B) nephritis

(C) hepatosplenomegaly and lymphadenopathy

(D) mitral insufficiency

(E) myositis

5.
The first medication usually given to treat the fevers and joint symptoms of this disease is

(A) a systemic corticosteroid (CS)

(B) acetaminophen

(C) a nonsteroidal anti-inflammatory drug (NSAID)

(D) methotrexate

(E) an antitumor necrosis factor drug

6.
Systemic CS should not be expected to

(A) stop the progression of joint disease

(B) improve the anemia

(C) help control fevers

(D) decrease symptoms of pericarditis

(E) decrease joint pain and swelling

7.
Patients on methotrexate therapy should be monitored regularly for

(A) hematuria

(B) muscle enzyme elevation

(C) hypertension

(D) liver enzyme elevation

(E) hyperlipidemia

8.
Potential long-term complications of this disease include

(A) growth delay, short stature, and osteoporosis

(B) micrognathia

(C) functional disabilities with fine and gross motor skill limitations

(D) risk of neurologic problems related to cervical spine arthritis

(E) all of the above

9.
A 4-year-old boy has had recurrent fevers up to 40ºC documented for the past year. The fevers occur every 4-6 weeks, last up to 5 days, and are associated with sore throat and occasional mouth sores. Workup for an acute infectious etiology during the fevers has been negative, and he never has had neutropenia. Between febrile episodes he is an active child without physical complaints, and he is growing well. His pediatrician gave him a diagnosis of PFAPA (
p
eriodic
f
ever,
a
phthous stomatitis,
p
haryngitis,
a
denitis). Which of the following is true about PFAPA?

(A) it is a benign entity in which most patients have resolution of the cyclic fevers by the age of 10 years

(B) it is associated with defects of the innate immune system

(C) inheritance pattern is autosomal dominant

(D) inheritance pattern is autosomal recessive

(E) patients frequently have polyserositis during the febrile episodes

10.
A 7-year-old girl was well until 3 months ago when she developed pain in her left knee. Her mother notes that she occasionally limps. There is no history of trauma or fevers. Her physical examination reveals left knee swelling and a mild loss of flexion and extension. She has mild guarding on ranging. She has left quadriceps atrophy. Her other joints are normal. Which of the following should be considered in the differential diagnosis?

(A) pauciarticular arthritis

(B) osteosarcoma

(C) psoriatic arthritis

(D) Lyme disease

(E) all of the above

11.
Diagnostic workup in the patient from question 10 should include

(A) rheumatoid factor

(B) plain film radiograph

(C) joint aspiration for synovial fluid culture

(D) uric acid

(E) HLA-B27 serotyping

12.
Which is a true statement about laboratory results in children with oligoarthritis?

(A) complete blood count (CBC) and erythrocyte sedimentation rate may be normal

(B) ANA is positive in all patients

(C) CRP is always elevated when active joint disease is present

(D) leukocyte count and complement levels are often decreased

(E) rheumatoid factor is present in approximately 25% of these patients

13.
The best way to detect early uveitis in children with arthritis is to

(A) assess visual acuity

(B) perform visual field testing

(C) do a funduscopic examination

(D) perform a slit lamp examination

(E) refer patient to ophthalmology immediately if eye symptoms develop

14.
All of the following statements are true about special school needs for children with chronic arthritis except

(A) a school district can transfer the child to another district if it is unable to provide the requested special services

(B) a public school must evaluate the child for special services if the parent requests them for the child

(C) services that may be requested if needed by the child to ensure proper education within the public school system include transportation between home and school, an extra set of books for home use, homebound instruction if unable to attend school, consultation by physical and occupational therapy services, and adaptive physical education

(D) Congress has passed legislation requiring that public school districts provide services to children with special needs

(E) none of the above

15.
Potential long-term problems in a child with oligoarticular disease include

(A) flexion contracture of the involved joint

(B) blindness secondary to uveitis

(C) leg-length discrepancy secondary to accelerated bone growth if a knee joint is involved

(D) progression to polyarticular disease in a small percentage of these children

(E) more of the above

16.
A 9-year-old boy has had right hip pain for 1-2 weeks, sometimes making ambulation difficult. Your differential diagnosis could include all but which of the following?

(A) Legg-Calvé-Perthes disease

(B) bone tumor

(C) transient (or toxic) synovitis

(D) oligoarticular arthritis

(E) early juvenile ankylosing spondylitis

17.
Which of the following is true regarding juvenile ankylosing spondylitis?

(A) arthritis is limited to the hip and axial (spine and sacroiliac) joints

(B) females are affected more frequently than males

(C) rheumatoid factor is present in up to 20% of patients

(D) enthesitis (inflammation at tendon insertion sites) may be present

(E) patients are always HLA-B27 positive

18.
Benign hypermobility syndrome is a common cause of joint pain in children. Which of these statements is not true regarding this syndrome?

(A) it is most common in children 3-10 years of age and decreases in frequency with age

(B) a criterion for diagnosis is hyperextension at elbows and knees by 10 degrees or more

(C) joint pain secondary to benign hypermobility syndrome is unrelated to physical activity

(D) a criterion for diagnosis is the ability to touch the thumb to the volar forearm

(E) it occurs more often in girls than boys