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FELLOW CLINICAL CHALLENGE
A
two year old male child is brought to your clinic for failure to
thrive and limping. The mother gives a history of joint swelling in the hands,
wrists, and fingers, poor joint movement, and morning stiffness of 1 hour that
started at age 12 months and has gradually progressed. She says the child has
had frequent fevers by touch. He has remained at the weight of 10.9 kg since
age 1 year.
Your physical exam
reveals a spunky young infant who orders his mother around. He is afebrile and
has no rash. The joint exam reveals mild swelling of the PIP's with moderate
limitation. His wrists have extension to 50 degrees and flexion to 60 degrees.
His knees and ankles also have mild swelling and limitation. He walks with his
elbows, hips, and knees in flexion.
Laboratory testing
reveals hemoglobin of 11.3 g%, a WBC of 6500 cells/mm3, a platelet count of 344,000 cells/mm3, and an ESR of 12 mm/hour (0-20
mm/hour). His ANA and RF are normal. Slit lamp exam is normal.
He is diagnosed as
juvenile idiopathic arthritis and placed on naproxen at 20 mg/kg/day and begun
on a PT/OT program. When he is brought in by his mother in 6 weeks, he is no
better. He is placed upon 7.5 mg per week of methotrexate by the subcutaneous route
in addition to the naproxen.
The child is not brought to see you for another 3 months. His mother says that he has been on his medications but he is no better. On exam you note that he is more flexed with very limited shoulder range, very limited fingers (curled in more of a claw), and with more hip and knee flexion contractures. His laboratory tests are essentially unchanged. You obtain hip, hand, and back x-rays. Please review the radiographs, CLICK HERE
Your diagnosis now is:
| a | inadequately treated, aggressive JIA |
| b) |
Familial acro-osteolysis |
| c) |
Stickler's syndrome |
| d) |
Congenital syphilis |
| e) | Morquio syndrome (MPS IV) |
| f) | Tricorhinophalangeal dysplasia |
| g) | Spondyloepiphyseal dysplasia |
| h) |
Progressive pseudorheumatoid arthropathy |
| i) | sarcoidosis |
Fellows! Please
choose one answer and e-mail your answer by May 15 to Linda Wagner at
lww@uchicago.edu.
FIRST CORRECT
ANSWER WINS THE CD-ROM OF PEDIATRIC SLIDE COLLECTION OF THE
Note: Please
include your program name and training director so we can verify that you are
indeed a fellow.