Table 1

Clinical characteristics at presentation

PatientSex/Age (y)Presenting symptomsDuration 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 involvedExtra-digestive involvementStage
1M/43Diarrhoea, weight loss2410 70036195Duodenum, jejunum, ileumMLNIE
2M/28Diarrhoea, weight loss, small bowel volvulus8415 87039165Duodenum, jejunumPeripheral blood, MLN, liverIV
3F/59Diarrhoea, weight loss, night sweating 615 35534 81Duodenum, jejunum, ileumLiverIV
4M/57Diarrhoea, weight loss1420100030 46Stomach, duodenum, jejunum, ileumNoneIE
  • MLN, mesenteric lymph nodes.