Long-term outcome of chronic intestinal pseudo-obstruction adult patients requiring home parenteral nutrition.
OBJECTIVE
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.
Amiot
Aurelien
A
Pôle des maladies de l'appareil digestif, Service de gastroentérologie et d'assistance nutritive, Université Paris 7, AP-HP, Hôpital Beaujon, Clichy, France.
Joly
Francisca
F
Alves
Arnaud
A
Panis
Yves
Y
Bouhnik
Yoram
Y
Messing
Bernard
B
D000293:Adolescent; D000328:Adult; D000368:Aged; D000704:Analysis of Variance; D002423:Cause of Death; D015331:Cohort Studies; D013505:Digestive System Surgical Procedures / Q000009:adverse effects / Q000379:methods; D005260:Female; D005769:Gastrointestinal Motility; D006801:Humans; D007418:Intestinal Pseudo-Obstruction / Q000175:diagnosis / Q000401:mortality* / Q000628:therapy*; D053208:Kaplan-Meier Estimate; D008134:Long-Term Care; D008297:Male; D008365:Manometry; D008875:Middle Aged; D015999:Multivariate Analysis; D016331:Parenteral Nutrition, Home / Q000379:methods / Q000401:mortality*; D011336:Probability; D011379:Prognosis; D016016:Proportional Hazards Models; D012189:Retrospective Studies; D018570:Risk Assessment; D012720:Severity of Illness Index; D012778:Short Bowel Syndrome / Q000209:etiology / Q000401:mortality* / Q000628:therapy*; D015996:Survival Rate; D013997:Time Factors; D016896:Treatment Outcome; D055815:Young Adult