| Chronic granulomatous disease by Mouy |
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| Both X-linked and autosomal recessive forms of CGD are complicated by invasive aspergillosis and there is approximately a 40% lifetime incidence of this complication in CGD. A few even rarer cases of neutrophil dysfunction complicated by invasive aspergillosis are described. Occasional instances of 2 children from one family developing invasive aspergillosis, usually related to farm exposures have been reported. Most cases present in children, 56% at #5 years and 89% at #10 years. The clinical presentation of invasive aspergillosis in CGD is distinctive. Pneumonia is most common but osteomyelitis is much more frequent, proportionately, than in other host groups (Table). Pneumonia tends to be bilateral, slowly progressive, with little or no fever. Radiologically it usually presents with a fine nodular pattern, widespread patchy shadowing or with a chest wall abscess with underlying consolidation. If a chest wall abscess is present (Table), rib osteomyelitis is invariably present as well. Extension from the lung may be posteriorly into the vertebral column giving rise to Aspergillus osteomyelitis and spinal cord compression. Often several vertebrae are involved. more... |