Oropharyngeal dysphagia and aspiration in patients with ataxia- telangiectasia

Maureen A. Lefton-Greif, Thomas O. Crawford, Jerry A. Winkelstein, Gerald M. Loughlin, Celide B. Koerner, Marianna Zahurak, Howard M. Lederman

Research output: Contribution to journalArticlepeer-review

87 Scopus citations


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.

Original languageEnglish (US)
Pages (from-to)225-231
Number of pages7
JournalJournal of Pediatrics
Issue number2
StatePublished - 2000

ASJC Scopus subject areas

  • Pediatrics, Perinatology, and Child Health


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