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Cisapride Improves Enteral Tolerance in Pediatric Short-bowel Syndrome With Dysmotility

Raphael, Bram P*; Nurko, Samuel; Jiang, Hongyu; Hart, Kristen; Kamin, Daniel S*; Jaksic, Tom*; Duggan, Christopher*

Journal of Pediatric Gastroenterology & Nutrition:
doi: 10.1097/MPG.0b013e3181fe2d7a
Original Articles: Gastroenterology

Background and Objectives: Gastrointestinal dysmotility is common in pediatric short-bowel syndrome, leading to prolonged parenteral nutrition dependence. There is limited literature regarding the safety and efficacy of cisapride for this indication. The aim of the study was to describe the safety and efficacy of cisapride for enteral intolerance in pediatric short-bowel syndrome.

Methods: Open-labeled pilot study in a limited access program for cisapride. Indications were short-bowel syndrome with underlying dysmotility and difficulty advancing enteral feeds despite standard therapies and without evidence of anatomic obstruction. Patients received cisapride 0.1 to 0.2 mg/kg per dose for 3 to 4 doses per day. We collected electrocardiogram, nutrition, and anthropometric data prospectively at study visits.

Results: Ten patients with mean (SD) age of 30.3 (30.5) months were enrolled in our multidisciplinary pediatric intestinal rehabilitation program. Median (interquartile range [IQR]) duration of follow-up was 8.7 (3.1–14.3) months. Median (IQR) residual bowel length was 102 (85–130) cm. Median (IQR) citrulline level was 14.5 (10.5–31.3) μmol/L. Diagnoses included isolated gastroschisis (n = 3), gastroschisis with intestinal atresia (n = 4), necrotizing enterocolitis (n = 2), and long-segment Hirschsprung disease (n = 1). Six subjects had at least 1 prior bowel-lengthening procedure. Median (IQR) change in percentage enteral energy intake was 19.9% (15.4%–29.8%) during follow-up (P = 0.01). Seven patients improved in enteral tolerance during treatment and 2 were weaned completely from parenteral nutrition. Complications during therapy were prolonged corrected QT interval (n = 2), gastrointestinal bleeding (n = 2), D-lactic acidosis (n = 1), and death due to presumed sepsis (n = 1). Longitudinal analysis (general estimating equation model) showed a strong positive association between cisapride duration and improved enteral tolerance. Mean percentage of enteral intake increased by 2.9% for every month of cisapride treatment (P < 0.0001).

Conclusions: Cisapride is a potentially useful therapy in patients with pediatric short-bowel syndrome with gastrointestinal dysmotility. We observed modest improvement in feeding tolerance where prior treatments failed; however, patients treated with cisapride require careful cardiac monitoring because corrected QT prolongation occurred in 20% of our cohort.

Author Information

*Center for Advanced Intestinal Rehabilitation, USA

Center for Motility and Functional Gastrointestinal Disorders, USA

Clinical Research Program, Children's Hospital Boston, Harvard Medical School, Boston, MA, USA.

Received 6 May, 2010

Accepted 30 August, 2010

Address correspondence and reprint requests to Christopher Duggan, MD, MPH, Division of Gastroenterology and Nutrition, Children's Hospital Boston, 300 Longwood Ave, Hunnewell Ground Floor, Boston, MA 02115 (e-mail:

This study was supported by NIH grants T32DK007477-25 (Dr Raphael), K24DK082792A (Dr Nurko), and 1K24HD058795 (Dr Duggan).

The authors report no conflicts of interest.

Copyright 2011 by ESPGHAN and NASPGHAN