Sensitization to Aspergillus species in the congenital neutrophil disorders chronic granulomatous disease and hyper-IgE syndrome☆,☆☆,★
Section snippets
Case report 1
The patient is a 35-year-old bank accountant who was referred for immunologic evaluation in 1997 by an infectious disease specialist. In 1992, the patient was found to have a lung abscess of the left upper lobe caused by S aureus , which was surgically excised in 1993. Over the following years, the patient had recurrent right upper lobe infiltrates and hilar adenopathy and required repeated therapeutic bronchoscopic removal of impacted mucoid material from the right upper lobe bronchus. Fungal
RESULTS
Eighteen patients with either CGD or HIE were studied, 11 with CGD (mean age, 16 ± 11.7 years; 9 male and 2 female subjects) and 7 with HIE (mean age, 35 ± 14 years; 4 male and 3 female subjects). All male patients with CGD had the X-linked form of the disease, as determined by family history, molecular analysis, or both. A compilation of the clinical courses and outcomes of the patients is shown in Table I, Table II.
From the 18 patients studied, 12 (67%, 10 of 11 with CGD and 2 of 7 with HIE)
DISCUSSION
In CGD and HIE, invasive pulmonary Aspergillus infections are one of the more common disease manifestations.5, 6, 7, 8, 10 Aspergillus infections can cause pneumonia, disseminated interstitial disease, and granulomata in CGD and carry a significant mortality rate.9, 28 In HIE syndrome Aspergillus infections may produce lung abscesses and erosion of pulmonary vasculature, leading to hemoptysis.11 In contrast, in ABPA Aspergillus species serve as an immune stimulant, but tissue invasion does not
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Supported in part by grant USPHS AI 42349 and the Ernest S. Bazley Grant to Northwestern Memorial Hospital and Northwestern University. Thomas Eppinger, MD, was a fellow of the Jeffrey Modell Foundation.
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Reprint requests: Charlotte Cunningham-Rundles, MD, PhD, Mount Sinai Medical Center, Division of Clinical Immunology, Box 1089, 1425 Madison Ave, New York, NY 10029.
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0091-6749/99 $8.00 + 0 1/1/102871