Table 3.
Investigations performed at time of diagnosis
| N (%) | All cases | Diagnosis < 2 years | Diagnosis ≥ 2 years | |||
|---|---|---|---|---|---|---|
| No.reported (Max 107) |
N (%) | No. reported (Max 66)a | N (%) | No. reported (Max 40)a | ||
| Investigations performed | ||||||
| Chest X-Ray | 99 (100.0) | 99 | 63 (100.0) | 63 | 35 (100.0) | 35 |
| Interstitial infiltrates | 41 (55.4) | 74 | 27 (54.0) | 50 | 14 (58.3) | 24 |
| Alveolar infiltrates | 22 (35.5) | 62 | 13 (29.5) | 44 | 9 (50.0) | 18 |
| CT Scan | 95 (95.0) | 100 | 55 (93.2) | 59 | 39 (97.5) | 40 |
| with controlled ventilation | 40 (51.3) | 78 | 30 (69.8) | 43 | 10 (28.6) | 35 |
| expiratory views | 57 (79.1) | 72 | 32 (76.2) | 42 | 25 (83.3) | 30 |
| CT scan findings | ||||||
| Reticular nodular infiltrates | 16 (30.7) | 52 | 6 (18.7) | 32 | 10 (50.0) | 20 |
| Ground glass pattern | 52 (71.2) | 73 | 32 (71.1) | 45 | 20 (71.4) | 28 |
| Honey comb pattern | 6 (13.1) | 46 | 1 (3.4) | 29 | 5 (29.4) | 17 |
| Lung Biopsy | 79 (75.2) | 105 | 52 (81.3) | 64 | 26 (65.0) | 40 |
| Biopsy Method | ||||||
| Open lung biopsy | 23 (36.5) | 63 | 18 (43.9) | 41 | 5 (22.7) | 22 |
| Video assisted thoracoscopic | 40 (63.5) | 63 | 23 (56.1) | 41 | 17 (77.3) | 22 |
| Transbronchial | 0 | 63 | 0 | 41 | 0 | 22 |
| Echocardiogram | 79 (78.2) | 101 | 47 (77.0) | 61 | 32 (80.0) | 40 |
| Pulmonary HT | 17 (22.1) | 77 | 12 (26.1) | 46 | 5 (16.1) | 31 |
| Pulmonary Function Test | 28 (26.7) | 105 | 2 (3.1) | 65 | 26 (65.0) | 40 |
| Infant pulmonary function test | 2 (1.9) | 102 | 2 (3.2) | 63 | - | - |
| Sleep Study | 44 (64.7) | 68 | 31 (65.9) | 47 | 1 2 (60.0) | 20 |
| BAL | 54 (65) | 83 | 27 (57.4) | 47 | 26 (74.3) | 35 |
| GORD | 10 (10.2) | 98 | 5 (7.6) | 66 | 5 (15.6) | 32 |
| Genetics Investigations | 25 (25.0) | 100 | 21 (33.3) | 63 | 4 (10.8) | 37 |
| Histological Second Opinion Sought | 30 (49.2) | 61 | 19 (50) | 38 | 11 (47.8) | 23 |
BAL Bronchoalveolar lavage; GORD Gastro-oesophageal reflux disease; CT Computed tomography; HT, Hypertension
a Age at diagnosis was not documented for 1 subject