title: Oropharyngeal dysphagia and aspiration in patients with ataxia-telangiectasia.
abstract: OBJECTIVE
  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.
  
  
  METHODS
  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.affiliation: Ataxia-Telangiectasia Clinical Center, John's Hopkins Children's Center, Baltimore, MD 21287-3923, USA.
authors.forename: M A
authors.initials: MA
authors.lastname: Lefton-Greif
authors.forename: T O
authors.initials: TO
authors.lastname: Crawford
authors.forename: J A
authors.initials: JA
authors.lastname: Winkelstein
authors.forename: G M
authors.initials: GM
authors.lastname: Loughlin
authors.forename: C B
authors.initials: CB
authors.lastname: Koerner
authors.forename: M
authors.initials: M
authors.lastname: Zahurak
authors.forename: H M
authors.initials: HM
authors.lastname: Lederman
mesh_terms: D000367:Age Factors; D001260:Ataxia Telangiectasia / Q000150:complications* / Q000503:physiopathology; D002648:Child; D003371:Cough / Q000209:etiology; D003679:Deglutition / Q000502:physiology; D003680:Deglutition Disorders / Q000209:etiology* / Q000503:physiopathology; D005260:Female; D006801:Humans; D008297:Male; D009752:Nutritional Status; D011015:Pneumonia, Aspiration / Q000209:etiology*; D014743:Videotape Recording