PatientsCystinosis patients, ages 1 to 18 years, with GIsymptoms were asked to participate in this study. All patientsreceived regular cysteamine bitartrate (Cystagon, Mylan,Morgantown, WV) at a free-base dose of approximately 60mg/kg/day. Acid suppressants, antibiotics, nonsteroidal antiinflammatorydrugs, prokinetic agents, and antihistamineswere discontinued one month before admission. A meanleukocyte cystine level of <2.0 nmol half-cystine per mg proteinover the past year was required for inclusion in the study.Serum GastrinAfter subjects underwent an eight-hour overnight fast,except for water, 3-mL blood samples were taken at baselineand at 30, 60, 90, and 120 minutes after ingestion of thepatient’s regular dose of cysteamine. The cysteamine was administeredin solution with water. Serum gastrin levels weremeasured in pg/mL using theDiagnostic Products Corporation(Los Angeles, Calif ) gastrin125 radioimmunoassay kit.13Gastric Acid SecretionOn the second study day, and after an overnight fast foreverything except water, a multiport 8 French nasogastric tubewas inserted into children who did not have a pre-existinggastrostomy tube. To assure correct tube placement, 50 mL ofwater was instilled into the stomach, aspirated, and discardedwith the overnight gastric contents. The hydrogen ion contentwithin aspirated gastric contents was measured to provide basalacid output (BAO)/maximum acid output (MAO)/peak acidoutput (PAO) levels. The BAO was estimated from gastriccontents collected over 60 minutes in four 15-minute aliquots.The patient’s regular dose of cysteamine was then administered,the feeding tube was clamped for 30 minutes, and the gastriccontents were again collected and discarded. Over the next twohours, gastric contents were collected in eight consecutive 15-minute aliquots. The MAO was the acid content in the first 60minutes. The PAO was the highest two consecutive 15-minutecollection periods multiplied by a factor of two to express acidsecretion in the usual way, ie, lEq/kg/hr. Gastric acid wasmeasured by standard titration method14 by using a digital pHmeter (PHM 82 Radiometer, Copenhagen, Denmark).
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