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