Oropharyngeal dysphagia and aspiration in patients with ataxia-telangiectasia.
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.
Lefton-Greif
M A
MA
Ataxia-Telangiectasia Clinical Center, John's Hopkins Children's Center, Baltimore, MD 21287-3923, USA.
Crawford
T O
TO
Winkelstein
J A
JA
Loughlin
G M
GM
Koerner
C B
CB
Zahurak
M
M
Lederman
H M
HM
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