Skeletal involvement in children who have chronic granulomatous disease

Paul David Sponseller, H. L. Malech, Edward F McCarthy, S. F. Horowitz, G. Jaffe, J. I. Gallin

Research output: Contribution to journalArticle

Abstract

Chronic granulomatous disease is a rare disorder of the oxidative metabolism of the white blood cells that results in immunodeficiency. In a retrospective review of the records of forty-two patients who had chronic granulomatous disease, we identified thirteen patients who had a total of twenty skeletal infections. Two types of infection were noted: Type 1, which resulted from a direct spread of the infection from an adjacent focus, usually of fungus or mycobacteria, and Type 2, which resulted from hematogenous spread, usually of Nocardia or more common bacteria. The thoracic vertebrae, ribs, and metatarsals were the most commonly involved bones. All four patients in whom the vertebrae were involved had a Type-1 fungal infection. Paresis developed in three of these patients, and two of them died. Antibiotics alone effectively eradicated the infection in only one patient. Wide operative debridement combined with administration of antibiotics was the most successful treatment. Comprehensive preoperative imaging and several cultures are necessary to locate the infection in the bone and to identify the organism.

Original languageEnglish (US)
Pages (from-to)37-51
Number of pages15
JournalThe Journal of bone and joint surgery. American volume
Volume73
Issue number1
StatePublished - 1991

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Chronic Granulomatous Disease
Infection
Anti-Bacterial Agents
Thoracic Vertebrae
Bone and Bones
Nocardia
Metatarsal Bones
Mycoses
Paresis
Ribs
Debridement
Mycobacterium
Spine
Leukocytes
Fungi
Bacteria

ASJC Scopus subject areas

  • Orthopedics and Sports Medicine
  • Surgery

Cite this

Skeletal involvement in children who have chronic granulomatous disease. / Sponseller, Paul David; Malech, H. L.; McCarthy, Edward F; Horowitz, S. F.; Jaffe, G.; Gallin, J. I.

In: The Journal of bone and joint surgery. American volume, Vol. 73, No. 1, 1991, p. 37-51.

Research output: Contribution to journalArticle

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