title: Xerostomia in patients with triple A syndrome--a newly recognised finding.
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.
authors.affiliation: Department of Paediatrics, University Hospital Zagreb-Rebro, Croatia. maricvit@mef.hr
authors.forename: M
authors.initials: M
authors.lastname: Dumić
authors.forename: M
authors.initials: M
authors.lastname: Mravak-Stipetić
authors.forename: Z
authors.initials: Z
authors.lastname: Kaić
authors.forename: J
authors.initials: J
authors.lastname: Ille
authors.forename: V
authors.initials: V
authors.lastname: Plavsić
authors.forename: S
authors.initials: S
authors.lastname: Batinica
authors.forename: M
authors.initials: M
authors.lastname: Cvitanović
mesh_terms: D000293:Adolescent; D000307:Adrenal Gland Diseases / Q000150:complications*; D000328:Adult; D002648:Child; D004931:Esophageal Achalasia / Q000150:complications*; D005260:Female; D006801:Humans; D007766:Lacrimal Apparatus Diseases / Q000150:complications*; D013577:Syndrome; D014987:Xerostomia / Q000150:complications*