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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Received: 29 May 2000 and in revised form: 19 July and 27 July 2000 / Accepted: 28 July 2000
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Dumić, M., Mravak-Stipetić, M., Kaić, Z. et al. Xerostomia in patients with triple A syndrome – a newly recognised finding. Eur J Pediatr 159, 885–888 (2000). https://doi.org/10.1007/PL00008361
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DOI: https://doi.org/10.1007/PL00008361