OBJECTIVES: To determine whether patients with ataxia-telangiectasia exhibit oropharyngeal dysphagia with concomitant aspiration and to examine the relationships among swallowing function, age, and nutritional status. STUDY DESIGN: Seventy patients (mean age, 10.7 years; range, 1.8 to 30 years) had feeding/swallowing and nutritional evaluations. Fifty-one patients, in whom there were concerns about swallowing safety, were examined with a standardized videofluoroscopic swallow study. RESULTS: Fourteen of the 51 patients (27%) with histories suggestive of dysphagia demonstrated aspiration. Of these, silent aspiration (aspiration without a cough) occurred in 10 (71%) patients. Aspirators were significantly older than non-aspirators (mean age, 16.9 vs 10.8 years; P =.002). Advancing age was the strongest factor associated with aspiration during continuous drinking (P =.01). In patients with ataxia-telangiectasia, weight and weight/height were abnormally low at all ages and most compromised in older patients. Patients who aspirated had significantly lower mean weight (P <.002) and weight/height z scores (P <.001) than did patients who did not aspirate. CONCLUSIONS: Oropharyngeal dysphagia is common and appears to be progressive in patients with ataxia-telangiectasia. Older patients also have a higher incidence of poorer nutritional status. The relationship between dysphagia and nutritional status deserves further investigation.
OBJECTIVES: To determine whether patients with ataxia-telangiectasia exhibit oropharyngeal dysphagia with concomitant aspiration and to examine the relationships among swallowing function, age, and nutritional status. STUDY DESIGN: Seventy patients (mean age, 10.7 years; range, 1.8 to 30 years) had feeding/swallowing and nutritional evaluations. Fifty-one patients, in whom there were concerns about swallowing safety, were examined with a standardized videofluoroscopic swallow study. RESULTS: Fourteen of the 51 patients (27%) with histories suggestive of dysphagia demonstrated aspiration. Of these, silent aspiration (aspiration without a cough) occurred in 10 (71%) patients. Aspirators were significantly older than non-aspirators (mean age, 16.9 vs 10.8 years; P =.002). Advancing age was the strongest factor associated with aspiration during continuous drinking (P =.01). In patients with ataxia-telangiectasia, weight and weight/height were abnormally low at all ages and most compromised in older patients. Patients who aspirated had significantly lower mean weight (P <.002) and weight/height z scores (P <.001) than did patients who did not aspirate. CONCLUSIONS:Oropharyngeal dysphagia is common and appears to be progressive in patients with ataxia-telangiectasia. Older patients also have a higher incidence of poorer nutritional status. The relationship between dysphagia and nutritional status deserves further investigation.
Authors: Sharon A McGrath-Morrow; W Adam Gower; Cynthia Rothblum-Oviatt; Alan S Brody; Claire Langston; Leland L Fan; Maureen A Lefton-Greif; Thomas O Crawford; Michelle Troche; John T Sandlund; Paul G Auwaerter; Blaine Easley; Gerald M Loughlin; John L Carroll; Howard M Lederman Journal: Pediatr Pulmonol Date: 2010-09
Authors: Maureen A Lefton-Greif; Thomas O Crawford; Sharon McGrath-Morrow; Kathryn A Carson; Howard M Lederman Journal: Orphanet J Rare Dis Date: 2011-05-15 Impact factor: 4.123
Authors: Kelly A Weir; Sandra M McMahon; Gillian Long; Judith A Bunch; Nirmala Pandeya; Kerry S Coakley; Anne B Chang Journal: Pediatr Radiol Date: 2007-01-10
Authors: Sharon A McGrath-Morrow; Howard M Lederman; Angela D Aherrera; Maureen A Lefton-Greif; Thomas O Crawford; Timothy Ryan; Jennifer Wright; Joseph M Collaco Journal: Pediatr Pulmonol Date: 2013-02-08