Xerostomia in patients with triple A syndrome--a newly recognised finding.
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.
Dumić
M
M
Department of Paediatrics, University Hospital Zagreb-Rebro, Croatia. maricvit@mef.hr
Mravak-Stipetić
M
M
Kaić
Z
Z
Ille
J
J
Plavsić
V
V
Batinica
S
S
Cvitanović
M
M
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*