Clinical characteristics at presentation
| Patient | Sex/Age (y) | Presenting symptoms | Duration of symptoms (months) | Weight loss (% pre-illness weight) | Stool volume (g/24 h) | Stool fat (g/24 h) | α1-antitrypsin clearance (ml/min) | Gut segments involved | Extra-digestive involvement | Stage |
|---|---|---|---|---|---|---|---|---|---|---|
| 1 | M/43 | Diarrhoea, weight loss | 24 | 10 | 700 | 36 | 195 | Duodenum, jejunum, ileum | MLN | IE |
| 2 | M/28 | Diarrhoea, weight loss, small bowel volvulus | 84 | 15 | 870 | 39 | 165 | Duodenum, jejunum | Peripheral blood, MLN, liver | IV |
| 3 | F/59 | Diarrhoea, weight loss, night sweating | 6 | 15 | 355 | 34 | 81 | Duodenum, jejunum, ileum | Liver | IV |
| 4 | M/57 | Diarrhoea, weight loss | 14 | 20 | 1000 | 30 | 46 | Stomach, duodenum, jejunum, ileum | None | IE |
MLN, mesenteric lymph nodes.