PEDIATRIC PUZZLER. OCTOBER 30 th , 2007 Rachel and Caroline, MDs Best Peds Chiefs Ever. HPI. Pt is a 9 yo autistic boy who presents to his PCP with R hip pain and a limp.
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OCTOBER 30th, 2007
Rachel and Caroline, MDs
Best Peds Chiefs Ever
What are your top 3 differentials?
What 3 lab tests do you want to order?
What are your top 3 diagnoses?
What studies do you want now?
48s 42 l 5m 5e
What further workup should be done?
59s 36l 4m 1e
CMP, Coags, IgGAME : normal
9 yo boy with autism presents with limb pain and progressive decrease in ambulation followed by a rash and gingival hypertrophy.
Multi-organ presentation: joints, skin, oral mucosa
Remember our patient is autistic. Could his autism be playing a role in his disease?
Characterized by abnormalities in social interaction, communication and behavior (DSM IV Criteria)
“Rituals — Apparently inflexible adherence to specific, nonfunctional routines or rituals is another characteristic feature of autism. These may manifest during various aspects of daily life, such as the need to always eat particular foods in a specific order, or to follow the same route from one place to another without deviation. Rituals may also manifest as repetitive ordering of toys, or mimicking the actions or dialogue from television or video”
*Of note, autistic children are at risk for a variety of vitamin deficiencies: A, D, and C especially!
Tie together the joint pain, the MRI changes, the rash and the gingival swelling…
A radiograph of the left wrist (Panel A) shows irregularity with widening of the distal ulnarphysis (arrow). However, there is normal mineralization of the zone of provisional calcification on the metaphyseal side of the growth plates and surrounding the epiphyses. (The curved band is a tube outside the patient\'s hand.) A radiograph of the right knee (Panel B) shows additional findings typical of scurvy: metaphyseal irregularities with spurring (Pelkan\'s sign, black arrows); white lines surrounding the epiphyses (Wimberger\'s sign), indicative of osteoporosis; a white line of Frankl in the zone of provisional calcification (white arrowhead) with a lucent line immediately below this (Trummerfeld zone or scurvy line, black arrowheads); and periosteal reactions along the metaphyses (white arrows). The estimated bone age is 2 years behind the patient\'s chronologic age.
Widened and Irregular growth plate
Osteoporosis of epiphysis with sclerotic ring
Subperiosteal hemorrhages lead to fragmentation and metaphyseal spurs
Dense Zone of calcification at margins of growth platePlain Films made the diagnosis!
Hope you enjoyed another edition of our Pediatric Puzzler!