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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.
J Clin Endocrinol Metab. 2006 Jan; 91(1):204-12.JC

Abstract

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.

Authors+Show Affiliations

Digestive National Institutes of Health/National Institute of Diabetes and Digestive and Kidney Diseases/Digestive Diseases Branch, Bethesda, Maryland 20892-1804, USA.No affiliation info availableNo affiliation info availableNo affiliation info availableNo affiliation info available

Pub Type(s)

Journal Article

Language

eng

PubMed ID

16249283

Citation

Hoffmann, K Martin, et al. "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." The Journal of Clinical Endocrinology and Metabolism, vol. 91, no. 1, 2006, pp. 204-12.
Hoffmann KM, Gibril F, Entsuah LK, et al. 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. J Clin Endocrinol Metab. 2006;91(1):204-12.
Hoffmann, K. M., Gibril, F., Entsuah, L. K., Serrano, J., & Jensen, R. T. (2006). 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. The Journal of Clinical Endocrinology and Metabolism, 91(1), 204-12.
Hoffmann KM, et al. 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. J Clin Endocrinol Metab. 2006;91(1):204-12. PubMed PMID: 16249283.
* Article titles in AMA citation format should be in sentence-case
TY - JOUR T1 - 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. AU - Hoffmann,K Martin, AU - Gibril,Fathia, AU - Entsuah,Laurence K, AU - Serrano,Jose, AU - Jensen,Robert T, Y1 - 2005/10/25/ PY - 2005/10/27/pubmed PY - 2006/2/1/medline PY - 2005/10/27/entrez SP - 204 EP - 12 JF - The Journal of clinical endocrinology and metabolism JO - J. Clin. Endocrinol. Metab. VL - 91 IS - 1 N2 - 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. SN - 0021-972X UR - https://www.unboundmedicine.com/medline/citation/16249283/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_ L2 - https://academic.oup.com/jcem/article-lookup/doi/10.1210/jc.2005-1349 DB - PRIME DP - Unbound Medicine ER -