The Journal of Bone and Joint Surgery, Vol 73, Issue 1 37-51, Copyright © 1991 by Journal of Bone and Joint Surgery, Inc
Skeletal involvement in children who have chronic granulomatous disease
PD Sponseller, HL Malech, EF McCarthy, SF Horowitz, G Jaffe and JI Gallin
Department of Orthopaedic Surgery, Johns Hopkins Medical Institutions, Baltimore, Maryland.
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.
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