Long-term outcome of chronic intestinal pseudo-obstruction adult patients requiring home parenteral nutrition.

Abstract

OBJECTIVES:

Chronic intestinal pseudo-obstruction (CIPO) is a rare, disabling disorder responsible for motility-related intestinal failure. Because it induces malnutrition, CIPO is a significant indication for home parenteral nutrition (HPN). The objective of the study was to evaluate long-term outcome of CIPO patients requiring HPN during adulthood.

METHODS:

In total, 51 adult CIPO patients (18 men/33 women, median age at symptom occurrence 20 (0-74) years, 34/17 primary/secondary CIPO) followed up at our institution for HPN management between 1980 and 2006 were retrospectively studied for survival and HPN dependence rates using univariate and multivariate analysis.

RESULTS:

Follow-up after diagnosis was 8.3 (0-29) years. Surgery was required in 84% of patients. The number of interventions was 3 +/- 3 per patient (mean +/- s.d.), leading to short bowel syndrome in 19 (37%) patients. Actuarial survival probability was 94, 78, 75, and 68% at 1, 5, 10, and 15 years, respectively. Multivariate analysis showed that lower mortality was associated with the ability to restore oral feeding at baseline (hazard ratio (HR) = 0.2 (0.06-0.65), P = 0.008) and symptom occurrence before the age of 20 years (HR=0.18 (0.04-0.88), P = 0.03). Higher mortality was associated with systemic sclerosis (HR=10.4 (1.6-67.9), P = 0.01). Actuarial HPN dependence was 94, 75, and 72% at 1, 2, and 5 years, respectively.

CONCLUSIONS:

In this large cohort of CIPO adult patients with severe intestinal failure, i.e., those requiring HPN, we found a higher survival probability than previously reported. These results should be taken into account when considering intestinal transplantation.

• Authors: Aurelien Amiot, Francisca Joly, Arnaud Alves, Yves Panis, Yoram Bouhnik, Bernard Messing
• Journal: The American journal of gastroenterology (Am J Gastroenterol) Vol. 104 Issue 5 Pg. 1262-70 (May 2009) ISSN: 1572-0241 [Electronic] United States
• PMID: 19367271 (Publication Type: Evaluation Study, Journal Article)

Topics

• Adolescent
• Adult
• Aged
• Analysis of Variance
• Cause of Death
• Cohort Studies
• Digestive System Surgical Procedures (adverse effects, methods)
• Female
• Gastrointestinal Motility
• Humans
• Intestinal Pseudo-Obstruction (diagnosis, mortality, therapy)
• Kaplan-Meier Estimate
• Long-Term Care
• Male
• Manometry
• Middle Aged
• Multivariate Analysis
• Parenteral Nutrition, Home (methods, mortality)
• Probability
• Prognosis
• Proportional Hazards Models
• Retrospective Studies
• Risk Assessment
• Severity of Illness Index
• Short Bowel Syndrome (etiology, mortality, therapy)
• Survival Rate
• Time Factors
• Treatment Outcome
• Young Adult