Pediatric rheumatology knowledge assessment
A 1,2, AND 3 ARE TRUE; B 1 AND 3 ARE TRUE; C 2 AND 4 ARE TRUE, D ONLY 4 IS TRUE, E ALL ARE TRUE.
1. A three year old girl with a swollen left knee which came on gradually is found to be antinuclear antibody (ANA) positive. Which of the following statements is/are true?
1. She most likely has systemic lupus erythematosus.
2. She is most likely rheumatoid factor positive.
3. She will probably develop arthritis in most of her large and small joints
within six months.
4. She is at increased risk for iridocyclitis.
2. A twelve year old boy has a swollen right knee and complains of pain in both heels when he walks. Which of the following statements is/are true?
1. He should be carefully examined for evidence of decreased lumbar spine
mobility.
2. He is probably rheumatoid factor negative.
3. His family history is likely to reveal a history of chronic low back pain.
4. He is most likely HLA-B27 positive.
3. A six year old boy presents with high fever, a rash, an a tender, hot swollen knee. He looks acutely ill. Which of the following statements is/are true?
1. He should be immediately started on steroids for systemic onset juvenile
rheumatoid arthritis.
2. An ANA (antinuclear antibody) test should be obtained and SLE considered in
the differential diagnosis.
3. He should be observed without intervention to monitor his fever curve.
4. The joint should be aspirated and appropriate cultures obtained to exclude
the possibility of infection. Broad spectrum antibiotics may be necessary
pending culture results.
4. Which of the following drugs is (are) recognized to be associated with the sudden onset of hepatic failure with prolonged clotting times and diffuse intravascular coagulation [even though used in appropriate dosage] in some children with systemic onset juvenile rheumatoid arthritis?
1. Aspirin - acetasalicylic acid
2. Myochrysine - gold sodium thiomalate
3. Indocin - indomethacin
4. Naprosyn - naproxen
5. Which of the following findings make the diagnosis of systemic onset JRA less likely?
1. High spiking fever which is elevated at all times of day.
2. A platelet count of < 100,000
3. A white blood cell count < 4,000.
4. An erythrocyte sedimentation rate of 120 mm/hr or more.
6. Which of the following are true for childhood systemic lupus erythematosus?
1. All affected children have a butterfly rash.
2. Over half of affected children will have renal involvement
3. The diagnosis cannot be made if the white blood cell count is > 4,000
4. Fatigue, fever, and weight loss are common presenting complaints.
7. A three year old child with a high fever and a swollen left anterior cervical lymph node develops dry cracked red lips, conjunctival injection, and swollen knees on the seventh day of illness despite five days of antibiotics. Which of the following statements is/are true?
1. The lymph node should immediately be biopsied.
2. This is probably a severe drug reaction and should be treated with steroids.
3. He should be hospitalized without further evaluation and begun on broad
spectrum antibiotics.
4. He should be evaluated for Kawasaki Disease.
8. The common laboratory manifestations of Kawasaki Disease include which of the following?
1. Elevated erythrocyte sedimentation rate
2. Decreased platelet count
3. Leukocytosis
4. A positive test for ANA [antinuclear antibodies].
9. A 4 year old child from Suffolk County Long Island presents with a hot
swollen knee in February. He has walked in the fields near his house where deer
have been seen on many occasions and over Christmas vacation he visited
1. Lyme disease
2. Juvenile rheumatoid arthritis
3. Plant thorn synovitis
4. Tuberculosis
10. A 17 year old boy comes to see you because of an inflamed and draining finger lesion. You explore it and note that it contains a calcific nidus. While looking at his hand you note that he has tight dry skin and many small telangiectasias. To confirm the diagnosis of CREST syndrome which of the following must be present.
1. Severe dental carries.
2. Difficulty swallowing meat and other foods
3. Arthritis in both hands and feet.
4. Marked cold sensitivity with Raynaud's phenomenon.
THE FOLLOWING QUESTIONS ARE TRUE OR FALSE.
11. The eye involvement of juvenile rheumatoid arthritis is directly correlated with the arthritis. Thus if the arthritis is under control there is no risk of eye disease. True or False?
12. A child with progressive dermatomyositis will loose his ability to grip objects before he begins to have difficulty going up and down stairs. True or False?
13. Once a child is diagnosed with definite dermatomyositis he/she will have marked muscle weakness and be disabled for the rest of his/her life. True or False?
MARK THE SINGLE CORRECT ANSWER FOR THE FOLLOWING QUESTIONS.
14. Which of the following should prompt immediate hospitalization of a child with dermatomyositis?
A. Frequent coughing when eating or drinking.
B. A positive Gower sign
C. The sudden appearance of subcutaneous calcifications
D. Difficulty opening and closing doors.
15. A Vietnamese child who received BCG presents with a positive PPD test and a swollen tender left knee of two weeks duration. A gram stain was negative and routine cultures on the synovial fluid were 'no growth.' There were 65,000 neutrophils/ml in the synovial fluid.
A. The diagnosis remains uncertain and could be juvenile rheumatoid
arthritis or tuberculosis. A synovial biopsy and
cultures for mycobacteria should be obtained.
B. This child has definite juvenile rheumatoid arthritis and should receive
intra-articular steroids.
C. This child has definite tuberculosis and should be begun on therapy with antituberculous agents.
D. Juvenile rheumatoid arthritis can be differentiated from tuberculosis by a lyme titer.
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