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Esomeprazole therapy for gastric acid hypersecretion in children with cystinosis

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Abstract

Oral cysteamine therapy prevents natural disease progression in children with cystinosis, but it may cause severe gastrointestinal (GI) symptoms through gastric acid-hypersecretion. The purpose of this study was to assess the value of esomeprazole in controlling cysteamine-induced acid-hypersecretion and GI symptoms in children with cystinosis. Subjects underwent upper GI endoscopy and biopsy, serum gastrin and cysteamine measurements as well as acid secretion studies (basal, maximal and peak acid output, BAO, MAO, PAO) before and during esomeprazole therapy. A symptom score (maximum 14 points) was devised to monitor symptoms. Twelve children (mean age 5.8 years) were studied. Cysteamine ingestion resulted in mean MAO and PAO significantly higher than mean BAO, both before and during esomeprazole therapy. PAO was usually within 60 min of cysteamine ingestion. Esomeprazole therapy significantly reduced MAO (P<0.01) and PAO (P<0.01). The mean symptom score fell from 6.4 to 0.7 (P<0.0001) during esomeprazole therapy. The mean final dose of esomeprazole was 1.7 mg/kg per day (range 0.7 mg/kg per day to 2.75 mg/kg per day). Plasma cysteamine levels were not affected by acid-suppression therapy. One child had multi-nucleated parietal cells. Cysteamine-induced gastric acid-hypersecretion and GI symptoms are dramatically reduced with esomeprazole therapy. Esomeprazole does not alter cysteamine absorption and is very well tolerated in children.

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Acknowledgments

Support was provided by AstraZeneca, Wilmington, Delaware, USA and National Institute of Health grant MO1RR00827.

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Authors and Affiliations

  1. UCSD Medical Center, Hillcrest, 200 West Arbor Drive, San Diego, CA 92103-8450, USA

    Ranjan Dohil

  2. Department of Pediatrics, University of California, San Diego, California, USA

    Ranjan Dohil, Meredith Fidler, Bruce Barshop, Reena Deutsch & Jerry Schneider

  3. Department of Pathology, Children’s Hospital and Health Center, San Diego, California, USA

    Robert Newbury

  4. Department of Medicine, University of California, San Diego, California, USA

    Zachary Sellers

  5. Department of Family and Preventive Medicine, University of California, San Diego, California, USA

    Reena Deutsch

Authors
  1. Ranjan Dohil
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  2. Meredith Fidler
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  3. Bruce Barshop
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  4. Robert Newbury
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  5. Zachary Sellers
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  6. Reena Deutsch
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  7. Jerry Schneider
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Correspondence to Ranjan Dohil.

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Dohil, R., Fidler, M., Barshop, B. et al. Esomeprazole therapy for gastric acid hypersecretion in children with cystinosis. Pediatr Nephrol 20, 1786–1793 (2005). https://doi.org/10.1007/s00467-005-2027-1

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  • DOI: https://doi.org/10.1007/s00467-005-2027-1

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