Long term outcomes of no gut syndrome in children - A multicentre study.
No Thumbnail Available
All Authors
Cernat, E.
Hill, S.
Wood, R.
Butt, A.
Buxton, S.
Cheng, J.
Goldthorpe, J.
Hind, J.
Koglmeier, J.
Onyeador, N.
LTHT Author
Cernat, Elena
Goldthorpe, Jenny
Goldthorpe, Jenny
LTHT Department
Leeds Children's Hospital
Paediatric Gastroenterology
Children's Services
Children's Nephrology, Hepatology & Gastroenterology
Paediatric Gastroenterology
Children's Services
Children's Nephrology, Hepatology & Gastroenterology
Non Medic
Children's Nutrition & Ibd Nurse Specialist Team Manager
Publication Date
2025
Item Type
Journal Article
Language
Subject
GASTROINTESTINAL DISEASES , PAEDIATRICS , PARENTERAL NUTRITION , SHORT BOWEL SYNDROME
Subject Headings
Abstract
Background: Children with extreme short bowel syndrome, including those who have undergone resection of the entire jejunum and ileum-termed "No Gut Syndrome" (NGS) in the adult literature-are now surviving due to advancements in parenteral nutrition (PN) and medical care. This study aims to describe the population characteristics and long-term outcomes of children with NGS.
Material and methods: A questionnaire was distributed to the paediatric intestinal failure (IF) centres in the UK to identify patients with NGS discharged home on PN. Inclusion criteria: infants/children <18 years with duodenum plus up to 5cm jejunum and colon (total/partial). Follow-up data was collected two years after the initial recruitment. A multicentre Health Research Authority approval was obtained.
Results: 19/20 (95%) IF centres managing 379 HPN patients responded - 27/379 (7%) had ultrashort-bowel syndrome (U-SBS) and an additional 14/379 (4%) NGS. Aetiology of NGS was - midgut volvulus (64%), bowel ischaemia (22%), necrotising enterocolitis (7%), jejuno-ileal atresia (7%). Nine patients had a colonic remnant in continuity. Thirteen/14 patients (93%) remained stable on PN with one weaned off following small bowel transplant. Nine/13 (69%) patients received >80% of EAR as PN and 4/13 (31%), 30-80%. Eight/13 (61%) tolerated some form of enteral nutrition. There were no deaths during the follow-up period. Educational outcomes were positive showing that 11/14 (79%) are attending school, 2/14 (14%) nurseries with 1 awaiting enrollment (7%).
Conclusion: Advances in PN and health care have improved the long-term survival of patients with NGS. In addition, these children are attending school and participating in daily activities without significant limitation.
Journal
Intestinal Failure (New York, N.Y.)