Table.
Clinical Characteristics.
| Patient 1 | Patient 2 | Patient 3 | Patient 4 | |
|---|---|---|---|---|
| Diagnosis | DPB | DPB | PCD | PCD |
| Age, years/sex | 70/man | 71/man | 52/man | 40/man |
| Smoking status | Ex-smoker (SI 50) | Ex-smoker (SI 100) | Ex-smoker (SI 50) | Never |
| Chronic sinusitis | + | + | + | + |
| Childhood episodes of respiratory infection | - | - | - | - |
| Other information | - | - | Infertility, visceral inversion | Infertility |
| Laboratory findings | ||||
| Cold agglutinin reaction (titer) | +(256) | - (32) | - (32) | - (32) |
| Autoantibodies | - | - | - | - |
| HTLV-1 and HIV antibodies | - | - | - | - |
| HLA | A11/A24/B52/B60 | A2/A24/B51/B61 | A26/A33/B44/B61 | A24/B54/B59 |
| Sputum or broncho-lavage culture | Haemophilus influenzae | Non-significant | Pseudomonas sp. Staphylococcus aureus | Haemophilus influenzae Streptococcus pneumoniae |
| Pulmonary function | ||||
| VC, % predicted | 88 % | 122 % | 103 % | 102 % |
| FEV1/FVC ratio (%) | 61 % | 64 % | 82 % | 68 % |
| DLco, % predicted | 152 % | 104 % | 84 % | 67 % |
| Ciliary structure under electron microscope | Normal | Normal | Complete defects of inner dynein arm | Partial defects of outer and inner dynein arm |
| Pathological findings by a cryobiopsy | Respiratory bronchioles with various inflammation cells and foamy macrophages | Bronchiolitis with high infiltration and peribronchiolar fibrosis | Mild respiratory bronchiolitis | Bronchiolitis obliterans involving terminal and respiratory bronchioles |
| Effects of macrolide therapy for six months | Good | Slightly worsened | Slightly improved | No change |
DPB: diffuse panbronchiolitis, PCD: primary ciliary dyskinesia, SI: smoking index, HTLV-1: human T-cell leukemia virus type 1, HIV: human immunodeficiency virus, HLA: human leukocyte antigen, VC: vital capacity, FVC: forced vital capacity, FEV1: forced expiratory volume in 1 second, DLco, diffusing capacity of carbon monoxide