Patient No (sex) | Age at onset (months) | Symptoms and signs | Associated disorders | Age at lung biopsy (months) |
---|---|---|---|---|
1 (F) | 2 | Poor feeding and tachypnoea; at 3 months rib retraction, inspiratory crackles, and cyanosis | None | 3 |
2 (M) | Birth | Tachypnoea; at 3 months poor weight gain, rib retraction, inspiratory crackles, and cyanosis | 35 weeks’ gestation; birth weight 1690 g, required oxygen for 24 hours only (max 28%) | 5 |
3 (F) | Birth | Tachypnoea; at 5 months rib retration and inspiratory crackles | None | 13 |
4 (F) | Birth | Poor feeding, poor weight gain ,and tachypnoea; at 4 months rib retraction, inspiratory crackles, and cyanosis | None | 4 |
5 (M) | 2 | Poor weight gain and tachypnoea; at 14 months rib retraction, inspiratory crackles, and cyanosis | None | 17 |
6 (F) | 4 | Poor weight gain, tachypnoea, and inspiratory crackles | None | 10 |
7 (M) | 7 | Tachypnoea, rib retraction, generalised wheeze, and cyanosis | Congenital pelviureteric junction obstruction, left pyeloplasty age 2 months | 8 |
8 (M) | Birth | Tachypnoea, rib retraction, generalised wheeze, and cyanosis; at 3 months inspiratory crackles | None | 3 |