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Long-Term Outcome of Chronic Intestinal Pseudo-Obstruction Adult Patients Requiring Home Parenteral Nutrition
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Long-Term Outcome of Chronic Intestinal Pseudo-Obstruction Adult Patients Requiring Home Parenteral Nutrition
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Journal Article
Long-Term Outcome of Chronic Intestinal Pseudo-Obstruction Adult Patients Requiring Home Parenteral Nutrition
2009
Overview
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.
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.
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.
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.
Publisher
Nature Publishing Group,Wolters Kluwer Health Medical Research, Lippincott Williams & Wilkins
Subject
/ Adult
/ Aged
/ Anesthesia. Intensive care medicine. Transfusions. Cell therapy and gene therapy
/ Biological and medical sciences
/ Digestive System Surgical Procedures - adverse effects
/ Digestive System Surgical Procedures - methods
/ Emergency and intensive care: metabolism and nutrition disorders. Enteral and parenteral nutrition
/ Female
/ Gastroenterology. Liver. Pancreas. Abdomen
/ Humans
/ Intestinal Pseudo-Obstruction - diagnosis
/ Intestinal Pseudo-Obstruction - mortality
/ Intestinal Pseudo-Obstruction - therapy
/ Male
/ Parenteral Nutrition, Home - methods
/ Parenteral Nutrition, Home - mortality
/ Short Bowel Syndrome - etiology
/ Short Bowel Syndrome - mortality
/ Short Bowel Syndrome - therapy
