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- Title
Xerostomia in patients with triple A syndrome--a newly recognised finding.
- Authors
Dumić, Miroslav; Mravak-Stipetić, Marinka; Kaić, Zvonimir; Ille, Jasenka; Plavsić, Vesna; Batinica, Stipe; Cvitanović, Marijana; Dumić, M; Mravak-Stipetić, M; Kaić, Z; Ille, J; Plavsić, V; Batinica, S; Cvitanović, M
- 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.
- Subjects
NERVOUS system abnormalities; DYSAUTONOMIA; SJOGREN'S syndrome
- Publication
European Journal of Pediatrics, 2000, Vol 159, Issue 12, p885
- ISSN
0340-6199
- Publication type
journal article
- DOI
10.1007/PL00008361