Related Experiment Videos
Severe short-bowel syndrome in children. Clinical experience
J C de Agustin1, J J Vázquez, D Rodríguez-Arnao
1Department of Pediatric Surgery, Hospital General Universitario Gregorio Marañón, Universidad Complutense de Madrid, Spain.
Insights
Home parenteral nutrition (HPN) improves child behavior in short-bowel syndrome (SBS). Growth hormone (GH) enhances enteral feeding tolerance, offering hope for improved outcomes in pediatric patients with SBS.
Area of Science:
- Pediatric Surgery
- Gastroenterology
- Nutritional Support
Background:
- Short-bowel syndrome (SBS) presents a significant challenge in pediatric care.
- Advancements in surgical and pharmacological treatments are improving patient prognoses.
- This study focuses on recent experiences with home parenteral nutrition (HPN) and growth hormone (GH) in SBS management.
Purpose of the Study:
- To evaluate the impact of HPN on the quality of life in children with SBS.
- To assess the efficacy of growth hormone (GH) in improving enteral feeding tolerance.
- To present recent treatment experiences for severe SBS.
Main Methods:
- A retrospective study of 8 pediatric patients with severe SBS.
- Inclusion criteria: intestinal length <25% of normal.
- Treatment modalities included HPN, GH administration, and surgical interventions.
Main Results:
- Etiologies of SBS included Hirschsprung's disease, midgut volvulus, gastroschisis, omphalocele, and Crohn's disease.
- Survival rate was 60% among the studied patients.
- GH administration increased enteral feeding tolerance; HPN improved child behavior and facilitated school attendance.
Conclusions:
- Growth hormone (GH) can enhance enteral feeding tolerance in SBS patients.
- Home parenteral nutrition (HPN) positively impacts child behavior and daily life.
- Intestinal transplantation should be considered when other treatments fail.
Introduction:
Innovative surgical and pharmacological therapeutic measures in short-bowel syndrome (SBS) are constantly changing the prognosis of this devastating condition. The aim of this paper is to present our most recent experience in the treatment of this disease, with particular emphasis on the impact of home parenteral nutrition (HPN) and the use of growth hormone (GH).
Methods:
A group of 8 patients with severe SBS have been studied for the past 4 years. Intestinal length of less than 25% normal at the time of bowel resection was the criterion for inclusion in this study.
Results:
Mean age at the time of diagnosis was 2 years (ranging from 1 day to 9 years). The etiology of the SBS was Hirschsprung's disease (n = 3), midgut volvulus (n = 2), gastroschisis (n = 1), omphalocele with ileal atresia and necrotizing enterocolitis (n = 1) and Crohn's disease (n = 1). Length of the residual bowel was 8 and 50 cm with ileocecal valve (ICV) preservation and 23, 27, 30, 50, 70, 100 cm without ICV. Sixty percent of the patients survived. Two patients died due to fulminant gram-negative sepsis and one due to cardiac malformation. Two patients are still on parenteral nutrition (PN) providing 30 and 60% of total calories. Human GH (0.3 U/kg/day) was used in two patients over a period of 28 days. In these patients, an increased tolerance to enteral feeding was observed. HPN was provided in 5 cases, allowing regular school attendance in 3 patients. In 3 cases, discontinuation of the PN was achieved at 24, 25 and 35 months respectively.
Conclusions:
Human GH can improve tolerance of enteral feeding. HPN has a beneficial effect on child behaviour. Intestinal transplantation must be considered when no other surgical or medical measures are available.