Table 2.
Clinical characteristics of chILD patients at initial presentation to reporting centre
| 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 |
||
| Sex: male/female | 59/45 (57/43) |
104 | 40/26 (61/39) |
66 | 19/18 (51/49) |
37 |
| Age at Diagnosis (years) | 0.01–18.00 | 0.01–1.92 | 2.00–18.00 | |||
| Median (range) | 0.50 | 8.20 | ||||
| Ethnic Origin | 57 | 27 | 30 | |||
| Caucasian | 41 (72.0) | 21 (77.8) | 20 (66.8) | |||
| Aboriginal | 2 (3.5) | 2 (7.4) | 0 | |||
| Maori | 6 (10.5) | 2 (7.4) | 4 (13.3) | |||
| Pacific Islander | 2 (3.5) | 0 | 2 (6.6) | |||
| Other | 6 (10.5) | 2 (7.4) | 4 (13.3) | |||
| Parental Consanguinity | 3 (5.8) | 51 | 0 | 26 | 3 (12.0) | 25 |
| Family History | ||||||
| Affected siblings | 7 (8.1) | 86 | 6 (12.0) | 50 | 1 (2.7) | 36 |
| Other family members affected | 3 (4.0) | 75 | 3 (7.5) | 40 | 0 | 35 |
| Symptoms | ||||||
| Cough | 51 (56.0) | 91 | 24 (47.0) | 51 | 26 (66.7) | 39 |
| Tachypnoea | 76 (76.7) | 99 | 54 (91.5) | 59 | 22 (55.0) | 40 |
| Failure to thrive | 35 (40.2) | 87 | 26 (53.1) | 49 | 9 (23.7) | 38 |
| Fever | 12 (13.8) | 87 | 7 (14.6) | 48 | 5 (12.8) | 39 |
| Exertion dyspnoea | 42 (56.0) | 75 | 16 (41.0) | 39 | 26 (72.2) | 36 |
| Dyspnoea at rest | 43 (50.0) | 86 | 26 (53.1) | 49 | 17 (45.9) | 37 |
| Physical Findings | ||||||
| Cyanosis | 22 (22.7) | 97 | 11 (19.3) | 57 | 11 (28.2) | 39 |
| Clubbing | 13 (14.3) | 91 | 3 (5.9) | 51 | 10 (25.6) | 39 |
| Crackles | 56 (58.3) | 96 | 29 (51.8) | 56 | 26 (66.7) | 39 |
| Wheeze | 20 (22.7) | 88 | 12 (23.5) | 51 | 8 (21.6) | 37 |
| Birth History | ||||||
| Mode of delivery | ||||||
| Spontaneous vaginal birth | 61 (77.2) | 79 | 40 (74.1) | 54 | 20 (83.3) | 24 |
| Gestation | ||||||
| < 37 weeks | 18 (21.4) | 84 | 15 (25.4) | 59 | 3 (12.0) | 25 |
| ≥ 37 weeks | 66 (78.6) | 84 | 44 (74.6) | 59 | 22 (88.0) | 25 |
| Respiratory distress at birth | 31 (41.3) | 75 | 26 (57.8) | 45 | 4 (13.8) | 29 |
| Intubation at birth | 20 (24.7) | 81 | 18 (38.3) | 47 | 2 (6.1) | 33 |
| Surfactant at birth | 7 (9.2) | 76 | 7 (15.9) | 44 | 0 | 32 |
a Age at diagnosis was not documented for 1 subject