Oropharyngeal dysphagia and aspiration in patients with ataxia- telangiectasia

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

Research output: Contribution to journalArticle

Abstract

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
Volume136
Issue number2
StatePublished - 2000

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Ataxia Telangiectasia
Deglutition Disorders
Deglutition
Nutritional Status
Weights and Measures
Cough
Drinking

ASJC Scopus subject areas

  • Pediatrics, Perinatology, and Child Health

Cite this

Oropharyngeal dysphagia and aspiration in patients with ataxia- telangiectasia. / Lefton-Greif, Maureen; Crawford, Thomas Owen; Winkelstein, Jerry A.; Loughlin, Gerald M.; Koerner, Celide B.; Zahurak, Marianna; Lederman, Howard M.

In: Journal of Pediatrics, Vol. 136, No. 2, 2000, p. 225-231.

Research output: Contribution to journalArticle

Lefton-Greif, M, Crawford, TO, Winkelstein, JA, Loughlin, GM, Koerner, CB, Zahurak, M & Lederman, HM 2000, 'Oropharyngeal dysphagia and aspiration in patients with ataxia- telangiectasia', Journal of Pediatrics, vol. 136, no. 2, pp. 225-231.
Lefton-Greif M, Crawford TO, Winkelstein JA, Loughlin GM, Koerner CB, Zahurak M et al. Oropharyngeal dysphagia and aspiration in patients with ataxia- telangiectasia. Journal of Pediatrics. 2000;136(2):225-231.
Lefton-Greif, Maureen ; Crawford, Thomas Owen ; Winkelstein, Jerry A. ; Loughlin, Gerald M. ; Koerner, Celide B. ; Zahurak, Marianna ; Lederman, Howard M. / Oropharyngeal dysphagia and aspiration in patients with ataxia- telangiectasia. In: Journal of Pediatrics. 2000 ; Vol. 136, No. 2. pp. 225-231.
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