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Predicting the clinical response to cisapride in children with chronic intestinal pseudo-obstruction
P E Hyman1, C Di Lorenzo, L McAdams
1Department of Pediatrics, Harbor-UCLA Medical Center, Torrance.
Insights
Children with chronic intestinal pseudo-obstruction (CIP) and absent migrating motor complex (MMC) required more nutritional support and responded less to cisapride. However, MMCs and normal bowel diameter predicted better cisapride response in these pediatric patients.
Area of Science:
- Pediatric Gastroenterology
- Gastrointestinal Motility Disorders
- Pharmacology
Background:
- Chronic intestinal pseudo-obstruction (CIP) is a severe motility disorder in children.
- Nutritional support is often required, and treatment options are limited.
- Understanding manometric patterns can guide therapeutic strategies.
Purpose of the Study:
- To evaluate upper gastrointestinal anatomy and function in children with CIP.
- To assess the efficacy of cisapride in pediatric CIP patients.
- To identify predictors of cisapride response in this population.
Main Methods:
- Contrast radiology and antroduodenal manometry were performed in 51 children with CIP.
- Patients underwent prolonged fasting and postprandial manometric recordings.
- An open-label, outpatient trial of oral cisapride was conducted for up to one year.
Main Results:
- Manometric abnormalities included absent migrating motor complex (MMC) in 27 patients.
- Absence of MMC was associated with a higher need for parenteral nutrition (p < 0.001).
- Cisapride response was better in children with MMCs (13/18) versus without MMCs (11/31, p < 0.02) and normal bowel diameter (p < 0.004).
Conclusions:
- Absence of MMC in pediatric CIP correlates with increased nutritional support needs.
- The presence of MMC and normal bowel diameter are positive predictors of cisapride response.
- Cisapride efficacy in pediatric CIP is variable but can be predicted by manometric findings and bowel caliber.
Abstract:
We assessed upper gastrointestinal anatomy and function with contrast radiology and antroduodenal manometry in 51 children with chronic intestinal pseudo-obstruction (CIP) prior to entering these patients into an open-label outpatient trial of cisapride. The diagnosis of CIP was based on characteristic symptoms requiring special nutritional support (parenteral in 30, tube feeding in 12) or interfering with daily activities (documented by diary in nine). At a time the subjects were not acutely ill, antroduodenal pressures were recorded for > 4 h fasting and > 1 h after a complex liquid meal. Results were categorized by the most prominent manometric abnormality as myopathy (n = 6), absent migrating motor complex (MMC) (n = 27), failure to induce fed pattern (n = 7), MMC plus discrete abnormalities (n = 7), and postprandial duodenal hypomotility (n = 4). Patients in the first two categories did not have effective MMCs, but those in the last three categories did. Compared to children without MMCs, those with MMCs rarely required parenteral nutrition (p < 0.001). All children were treated with oral cisapride 0.2 mg/kg/dose t.i.d., and evaluated every 2 months for up to 1 yr. Of 49 evaluable subjects, the final global assessment was unchanged in 25, fair (improved symptom score) in 17, or excellent (change from TPN to tube feeding or tube feeding to oral feeding) in seven. Children with MMCs (13 of 18) responded more often to cisapride than those without MMCs (11 of 31), p < 0.02. All four subjects with postprandial duodenal hypomotility had excellent responses. Children with normal diameter bowel responded more often than those with dilated bowel, p < 0.004. To summarize, in children with CIP, absence of the MMC was associated with need for greater intensity of nutritional support and decreased response rate to cisapride. The response to cisapride was highly variable within the study group, but often could be predicted by the presence or absence of bowel dilation and MMCs.