PEDIATRICS Vol. 75 No. 2 February 1985, pp. 321-323
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Progressive Diaphyseal Dysplasia: Evaluation of Corticosteroid Therapy

Yehezkel Naveh MD1, Uri Alon MD1, Joseph K. Kaftori MD1, and Moshe Berant MD1

1 From the Departments of Pediatrics and Diagnostic Radiology, Rambam Medical Center, and Faculty of Medicine, Technion-Israel Institute of Technology, Haifa, Israel

Progressive diaphyseal dysplasia is characterized clinically by crippling leg pain, fatigue, headache, poor appetite, muscle weakness, and waddling gait. Twelve affected patients, aged 2 years 4 months to 40 years, were treated with intermittent courses of low doses of prednisone given in a single dose on alternate mornings for periods ranging from 6 months to 10 years. The average initial dose of prednisone was 0.6 mg/kg/d, and average maintenance dose was 0.3 mg/kg/d. Relief of all crippling symptoms was achieved in all patients. No untoward serious side effects have been observed, and the growth of children was not slowed. However, corticosteroid therapy should be restricted to patients suffering from crippling pain. The mechanism through which steroids act remains undefined.

Key Words: Camurati-Engelmann disease • Engelmann disease • progressive diaphyseal dysplasia • osteosclerosis • corticosteroid therapy

Submitted on November 21, 1983
Accepted on March 22, 1984




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