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Table 1 Clinical, physiological, radiographical, and pathological data relating to mimicking HP and IPAI in the 3 CGD patients

From: Mimicking hypersensitivity pneumonitis as an uncommon initial presentation of chronic granulomatous disease in children

CGD patient Case 1 Case 2 Case 3
Age (yr) 4 8 5
Sex male female male
Data of mimicking HP
Exposure to an offending environment Antigens probably relating to rotten fruits Antigens probably in musty cornhusks Antigens probably in musty cornhusks
Symptom/sign Dry cough, dyspnea, fever, bilateral basilar rales Spiking fever with chills, dry cough, dyspnea, chest stuffy, bilateral basilar rales Spiking fever with chills, dry cough, dyspnea, bilateral basilar rales
Pulmonary function FEV1, 0.48 L (51.2% predicted); FVC, 0.65 L (68.3% predicted) FEV1, 0.8 L (58.7% predicted); FVC, 0.92 L (58.9% predicted); DLCO, 6.46 ml/min/mmHg (46.6% predicted) FEV1, 0.59 L (57.3% predicted); FVC, 0.72 L (70.5% predicted)
Chest HRCT scan Diffuse nodular opacities and slight ground-glass in bilateral inferior field Bilaterally diffuse ill-defined centrilobular nodules and slight ground-glass Bilaterally diffuse ill-defined centrilobular nodules and slight ground-glass
BALF cells AM: 46%; Lym: 52%; Eos: 2%; CD4+/CD8+: 0.62 AM: 41%; Lym: 56%; Eos: 3%; CD4+/CD8+: 0.65 AM: 48%; Lym: 50%; Neu: 2%; CD4+/CD8+: 0.73
Lung biopsy Bronchiolo centric lymphocytic, non-necrotizing granulomas and no evidence of fungal or bacterial elements Not available Not available
Bacterial/viral/fungal cultures Negative Negative Negative
Treatment 1 mg/kg/d prednisone 1 mg/kg/d prednisone 1 mg/kg/d prednisone
Data of IPAI
HRCT scan finding Consolidation in left upper lobe and cavity in right upper lobe Multi-nodules bilaterally distributed along bronchi and part of multi-nodules fused into pieces more in upper lung A nodular consolidation with halo sign in left upper lobe
Bacterial/viral/fungal cultures A. fumigatus A. fumigatus A. fumigatus
Treatment Infusion of voriconazole for 2 months followed by oral voriconazole for 6 months Infusion of amphotericin liposome B for 2 months followed by oral voriconazole for one year Oral voriconazole for 4 months
  1. HP hypersensitivity pneumonitis, IPAI invasive pulmonary A.fumigatus infection, HRCT high-resolution computer tomography, BALF bronchoalveolar lavage fluid, AM Alveolar macrophages, Lym lymphocytes, Neu neutrophils, Eos eosinophils, FEV1 forced expiratory volume in one second, FVC forced vital capacity, DLCO decreased lung diffusion of carbon monoxide