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Severe short-bowel syndrome in children. Clinical experience.
Eur J Pediatr Surg. 1999 Aug; 9(4):236-41.EJ

Abstract

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.

Authors+Show Affiliations

Department of Pediatric Surgery, Hospital General Universitario Gregorio Marañón, Universidad Complutense de Madrid, Spain.No affiliation info availableNo affiliation info availableNo affiliation info availableNo affiliation info availableNo affiliation info available

Pub Type(s)

Journal Article

Language

eng

PubMed ID

10532265

Citation

de Agustin, J C., et al. "Severe Short-bowel Syndrome in Children. Clinical Experience." European Journal of Pediatric Surgery : Official Journal of Austrian Association of Pediatric Surgery ... [et Al] = Zeitschrift Fur Kinderchirurgie, vol. 9, no. 4, 1999, pp. 236-41.
de Agustin JC, Vázquez JJ, Rodríguez-Arnao D, et al. Severe short-bowel syndrome in children. Clinical experience. Eur J Pediatr Surg. 1999;9(4):236-41.
de Agustin, J. C., Vázquez, J. J., Rodríguez-Arnao, D., Canals, M. J., Soler, J., & Alvarez, E. (1999). Severe short-bowel syndrome in children. Clinical experience. European Journal of Pediatric Surgery : Official Journal of Austrian Association of Pediatric Surgery ... [et Al] = Zeitschrift Fur Kinderchirurgie, 9(4), 236-41.
de Agustin JC, et al. Severe Short-bowel Syndrome in Children. Clinical Experience. Eur J Pediatr Surg. 1999;9(4):236-41. PubMed PMID: 10532265.
* Article titles in AMA citation format should be in sentence-case
TY - JOUR T1 - Severe short-bowel syndrome in children. Clinical experience. AU - de Agustin,J C, AU - Vázquez,J J, AU - Rodríguez-Arnao,D, AU - Canals,M J, AU - Soler,J, AU - Alvarez,E, PY - 1999/10/26/pubmed PY - 1999/10/26/medline PY - 1999/10/26/entrez SP - 236 EP - 41 JF - European journal of pediatric surgery : official journal of Austrian Association of Pediatric Surgery ... [et al] = Zeitschrift fur Kinderchirurgie JO - Eur J Pediatr Surg VL - 9 IS - 4 N2 - 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. SN - 0939-7248 UR - https://www.unboundmedicine.com/medline/citation/10532265/Severe_short_bowel_syndrome_in_children__Clinical_experience_ L2 - http://www.thieme-connect.com/DOI/DOI?10.1055/s-2008-1072252 DB - PRIME DP - Unbound Medicine ER -