PMID- 16249283 OWN - NLM STAT- MEDLINE DCOM- 20060131 LR - 20120910 IS - 0021-972X (Print) IS - 0021-972X (Linking) VI - 91 IP - 1 DP - 2006 Jan TI - Patients with multiple endocrine neoplasia type 1 with gastrinomas have an increased risk of severe esophageal disease including stricture and the premalignant condition, Barrett's esophagus. PG - 204-12 AB - CONTEXT: Multiple endocrine neoplasia type 1 (MEN1) patients frequently develop Zollinger-Ellison syndrome (MEN1/ZES). Although esophageal reflux symptoms are common in these patients, little is known about long-term occurrence of severe peptic esophageal disease including strictures and Barrett's esophagus (BE). OBJECTIVE: The objective of the study was to prospectively analyze the frequency of severe peptic esophageal disease in ZES patients with and without MEN1. SETTING: The study was conducted at a tertiary care research center. PATIENTS: Two hundred ninety-five patients (80 = MEN1/ZES, 215 = sporadic ZES) participated in a prospective study. INTERVENTIONS AND OUTCOME MEASURES: Assessment of MEN1, acid hypersecretion, upper gastrointestinal endoscopy/biopsies, and tumor status were measured initially and at each follow-up. Esophageal manometry was performed in 89 patients. Frequency and type of esophageal disease were correlated with clinical/laboratory/tumoral features of ZES/MEN1. RESULTS: In MEN1/ZES patients, esophageal stricture was 3-fold higher, BE 5-fold higher, and dysplasia 8-fold higher, and one patient died of esophageal adenocarcinoma. Esophageal symptoms were more frequent or severe in MEN1/ZES, but known risk factors for severe esophageal disease and ZES-specific features did not differ between MEN1/ZES and sporadic ZES. In MEN1/ZES, the onset of ZES was 10 yr earlier, and H2-antagonists were used longer and at lower doses. MEN1/ZES patients with esophageal disease differed from those without in that ZES diagnosis was delayed longer, esophageal symptoms were more frequent or severe, hiatal hernias were more frequent, esophagitis or pyloric scarring was more common, basal acid output was higher, and hyperparathyroidism was underdiagnosed. CONCLUSIONS: This study shows that MEN1/ZES patients have a higher incidence of severe esophageal disease including the premalignant condition BE and identifies factors important for their pathogenesis that need to be incorporated into their long-term treatment. FAU - Hoffmann, K Martin AU - Hoffmann KM AD - Digestive National Institutes of Health/National Institute of Diabetes and Digestive and Kidney Diseases/Digestive Diseases Branch, Bethesda, Maryland 20892-1804, USA. FAU - Gibril, Fathia AU - Gibril F FAU - Entsuah, Laurence K AU - Entsuah LK FAU - Serrano, Jose AU - Serrano J FAU - Jensen, Robert T AU - Jensen RT LA - eng PT - Journal Article DEP - 20051025 PL - United States TA - J Clin Endocrinol Metab JT - The Journal of clinical endocrinology and metabolism JID - 0375362 RN - 0 (Histamine H2 Antagonists) SB - IM MH - Adult MH - Barrett Esophagus/*epidemiology/*etiology MH - Esophageal Diseases/*epidemiology/*etiology MH - Esophageal Sphincter, Lower/physiopathology MH - Esophageal Stenosis/*epidemiology/*etiology MH - Esophagoscopy MH - Female MH - Gastric Acidity Determination MH - Gastrinoma/*complications MH - Histamine H2 Antagonists/pharmacology MH - Humans MH - Male MH - Multiple Endocrine Neoplasia Type 1/*complications MH - Prospective Studies MH - Risk MH - Sex Factors MH - Zollinger-Ellison Syndrome/complications EDAT- 2005/10/27 09:00 MHDA- 2006/02/01 09:00 CRDT- 2005/10/27 09:00 PHST- 2005/10/27 09:00 [pubmed] PHST- 2006/02/01 09:00 [medline] PHST- 2005/10/27 09:00 [entrez] AID - jc.2005-1349 [pii] AID - 10.1210/jc.2005-1349 [doi] PST - ppublish SO - J Clin Endocrinol Metab. 2006 Jan;91(1):204-12. doi: 10.1210/jc.2005-1349. Epub 2005 Oct 25.