title: Esomeprazole therapy for gastric acid hypersecretion in children with cystinosis.
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.
authors.affiliation: UCSD Medical Center, San Diego, CA 92103-8450, USA. rdohil@ucsd.edu
authors.forename: Ranjan
authors.initials: R
authors.lastname: Dohil
authors.forename: Meredith
authors.initials: M
authors.lastname: Fidler
authors.forename: Bruce
authors.initials: B
authors.lastname: Barshop
authors.forename: Robert
authors.initials: R
authors.lastname: Newbury
authors.forename: Zachary
authors.initials: Z
authors.lastname: Sellers
authors.forename: Reena
authors.initials: R
authors.lastname: Deutsch
authors.forename: Jerry
authors.initials: J
authors.lastname: Schneider
mesh_terms: D002648:Child; D002675:Child, Preschool; D003543:Cysteamine / Q000009:adverse effects / Q000097:blood; D003554:Cystinosis / Q000188:drug therapy*; D004791:Enzyme Inhibitors / Q000009:adverse effects / Q000627:therapeutic use*; D064098:Esomeprazole / Q000009:adverse effects / Q000627:therapeutic use*; D005260:Female; D005744:Gastric Acid / Q000378:metabolism*; D005755:Gastrins / Q000097:blood; D005767:Gastrointestinal Diseases / Q000139:chemically induced / Q000188:drug therapy / Q000503:physiopathology*; D005773:Gastroscopy; D006801:Humans; D007413:Intestinal Mucosa / Q000473:pathology; D008297:Male; D012189:Retrospective Studies; D012720:Severity of Illness Index
chemical_list: D004791:Enzyme Inhibitors; D005755:Gastrins; D003543:Cysteamine; D064098:Esomeprazole