GASTROESOPHAGEAL REFLUX AND SEVERE COMBINED IMMUNODEFICIENCY

Background: Gastrointestinal and respiratory symptoms and failure to t hrive not associated with infections or medications were noted in pati ents with severe combined immunodeficiency. Objective: The aim of our study was to determine the frequency of gastroesophageal reflux in pat ients with severe combined immunodeficiency. Methods: We studied the c ase histories of 73 pediatric patients who had been treated at Duke Un iversity Medical Center for severe combined immunodeficiency between 1 982 and 1995. Charts were reviewed for documentation of gastroesophage al reflux on the basis of clinical course and results of barium swallo w esophageal pH probe monitoring, or endoscopy. To compare the inciden ce of gastroesophageal reflux in patients with severe combined immunod eficiency to known high-risk populations, we additionally tabulated th e underlying diagnoses in an age-matched group of patients who underwe nt Nissen fundoplication from 1990 to 1995. Results: We found clinical ly significant gastroesophageal reflux in 15 of the 73 patients (20.5% ), much higher than has been reported in the normal population (0.1% t o 0.3%, p < 0.001). Of patients treated between 1990 and the present, 10 of 36 (27.7%) had significant gastroesophageal reflux compared with five of 37 patients (13.5%) in the previous years. Thus with greater recognition and improved methods for diagnosis, the observed incidence of gastroesophageal reflux has increased greatly. The clinical presen tations were not different from those of patients with other well-docu mented underlying diagnoses. Seven of the 15 patients (46.6%) did not respond to medical treatment with antacids, H-2-blockers, and prokinet ic agents and underwent surgical treatment. Indications for surgery in cluded persistent esophagitis, vomiting, pneumonia, and growth failure . Conclusions: The reason for the high incidence of gastroesophageal r eflux in patients with severe T-cell disorders remains unclear. Consid ering the frequency of this association, early recognition and treatme nt is important to enable adequate nutrition and prevent damage to the esophagus and lungs.