A rapid unfavorable outcome of wegener's granulomatosis in early childhood

L. Nespoli, M. Duse, M. A. Vitiello, G. Perinotto, R. Fiocca, A. Giannetti, A. Colombo

Research output: Contribution to journalArticle

Abstract

Wegener's Granulomatosis was suspected in a 27-month-old female with a nodular, necrotizing lesion of the nose, diffuse subcutaneous nodules, and erythematous desquamation of the entire body. From 20 months of age on she had a purulent nasal discharge, recurrent infections of the upper and lower respiratory tract, a Coombs positive anemia, and enlargement of the spleen and liver. Treatment with azathioprine and corticosteroids produced transient improvement but three months later a dramatic relapse occurred. Cyclophosphamide was substituted for azathioprine but 10 days later the patient died and the autopsy confirmed the diagnosis of Wegener's Granulomatosis. The early age of onset of the disease may explain the unfavorable outcome, despite treatment with cytotoxic agents.

Original languageEnglish
Pages (from-to)277-282
Number of pages6
JournalEuropean Journal of Pediatrics
Volume131
Issue number4
DOIs
Publication statusPublished - Dec 1979

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Granulomatosis with Polyangiitis
Azathioprine
Nose
Cytotoxins
Age of Onset
Respiratory Tract Infections
Cyclophosphamide
Anemia
Autopsy
Adrenal Cortex Hormones
Spleen
Recurrence
Liver
Therapeutics

Keywords

  • Azathioprine and cyclophosphamide
  • Immunodeficiency
  • Wegener's Granulomatosis

ASJC Scopus subject areas

  • Pediatrics, Perinatology, and Child Health

Cite this

Nespoli, L., Duse, M., Vitiello, M. A., Perinotto, G., Fiocca, R., Giannetti, A., & Colombo, A. (1979). A rapid unfavorable outcome of wegener's granulomatosis in early childhood. European Journal of Pediatrics, 131(4), 277-282. https://doi.org/10.1007/BF00444349

A rapid unfavorable outcome of wegener's granulomatosis in early childhood. / Nespoli, L.; Duse, M.; Vitiello, M. A.; Perinotto, G.; Fiocca, R.; Giannetti, A.; Colombo, A.

In: European Journal of Pediatrics, Vol. 131, No. 4, 12.1979, p. 277-282.

Research output: Contribution to journalArticle

Nespoli, L, Duse, M, Vitiello, MA, Perinotto, G, Fiocca, R, Giannetti, A & Colombo, A 1979, 'A rapid unfavorable outcome of wegener's granulomatosis in early childhood', European Journal of Pediatrics, vol. 131, no. 4, pp. 277-282. https://doi.org/10.1007/BF00444349
Nespoli, L. ; Duse, M. ; Vitiello, M. A. ; Perinotto, G. ; Fiocca, R. ; Giannetti, A. ; Colombo, A. / A rapid unfavorable outcome of wegener's granulomatosis in early childhood. In: European Journal of Pediatrics. 1979 ; Vol. 131, No. 4. pp. 277-282.
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