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Wegener's Granulomatosis in a Child Observations on Pathogenesis and Treatment

Stacy A. Roback, MD; Roger C. Herdman, MD; John Hoyer, MD; Robert A. Good, MD, PhD
Am J Dis Child. 1969;118(4):608-614. doi:10.1001/archpedi.1969.02100040610014.
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WEGENER'S granulomatosis, a rare disease in adults, is particularly infrequent in children. Only three instances of this disorder in individuals less than 15 years of age have been reported.1-3 This communication concerns studies of a boy whose first symptoms of Wegener's granulomatosis occurred at 14 years of age. New observations relative to the pathogenesis and therapy of this disease are described.

Report of a Case  A 16-year-old white boy had the first of many episodes of spontaneous epistaxis at age 14. On several occasions, bleeding was severe enough to require hospitalization, yet at these times no abnormality of coagulation could be demonstrated. At 14½ years of age he developed nasal stuffiness, intermittent low-grade fever, lethargy, and anorexia. At this time, the histological condition of a nasal biopsy was interpreted as nonspecific granulation with minimal granuloma formation (Fig 1). Skin tests for mycobacteria were negative, and over a period


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