Xerostomia in patients with triple A syndrome--a newly recognised finding.
Eur J Pediatr
; 159(12): 885-8, 2000 Dec.
Article
in En
| MEDLINE
| ID: mdl-11131343
ABSTRACT
Triple A syndrome is characterised by achalasia, alacrima, adrenal insufficiency and progressive neurological abnormalities including impaired autonomic nervous function. We present five patients with triple A syndrome in whom we describe xerostomia for the first time, a symptom which was presumed to be practically exclusive to Sjøgren syndrome and familial dysautonomia. Conclusion We recommend the investigation of salivation in all patients with triple A syndrome and treatment of xerostomia in order to ease swallowing. Further, our results corroborate earlier doubts that some patients with Sjøgren syndrome, especially those with the so-called "achalasia sicca" syndrome and adrenocortical insufficiency, actually had triple A syndrome. Therefore, adrenocortical function should be assessed in all patients with Sjøgren syndrome, particularly in those with difficulties in swallowing, because even latent adrenocortical insufficiency could be life-threatening for these patients in stressful situations.
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Database:
MEDLINE
Main subject:
Xerostomia
/
Esophageal Achalasia
/
Adrenal Gland Diseases
/
Lacrimal Apparatus Diseases
Type of study:
Diagnostic_studies
Limits:
Adolescent
/
Adult
/
Child
/
Female
/
Humans
Language:
En
Journal:
Eur J Pediatr
Year:
2000
Type:
Article
Affiliation country:
Croatia