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.
Pôle des maladies de l'appareil digestif, Service de gastroentérologie et d'assistance nutritive, Université Paris 7, AP-HP, Hôpital Beaujon, Clichy, France.
Aurelien
A
Amiot
Francisca
F
Joly
Arnaud
A
Alves
Yves
Y
Panis
Yoram
Y
Bouhnik
Bernard
B
Messing
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