caa a22 4500 475784928 CHVBK 20180406123655.0 cr unu---uuuuu 170329e20001101xx s 000 0 eng 10.1007/PL00008361 doi (NATIONALLICENCE)springer-10.1007/PL00008361 Xerostomia in patients with triple A syndrome - a newly recognised finding [Elektronische Daten] [Miroslav Dumić, Marinka Mravak-Stipetić, Zvonimir Kaić, Jasenka Ille, Vesna Plavšić, Stipe Batinica, Marijana Cvitanović] 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. Springer-Verlag Berlin Heidelberg, 2000 Key words Achalasia nationallicence Adrenal insufficiency nationallicence Alacrima nationallicence Autonomic neuropathy nationallicence Xerostomia nationallicence Dumić Miroslav Department of Paediatrics, University Hospital Zagreb-Rebro, Kišpatićeva 12, 10000 Zagreb, Croatia e-mail: maricvit@mef.hr Tel.: +385-1-213087; Fax: +385-1-2421894, HR aut Mravak-Stipetić Marinka Department of Oral Medicine, School of Dental Medicine, University of Zagreb, Zagreb, Croatia, HR aut Kaić Zvonimir Department of Dental Anthropology, School of Dental Medicine, University of Zagreb, Zagreb, Croatia, HR aut Ille Jasenka Department of Paediatrics, University Hospital Zagreb-Rebro, Kišpatićeva 12, 10000 Zagreb, Croatia e-mail: maricvit@mef.hr Tel.: +385-1-213087; Fax: +385-1-2421894, HR aut Plavšić Vesna Endocrinological Laboratory, University Hospital Zagreb-Rebro, Zagreb, Croatia, HR aut Batinica Stipe Department of Child Surgery, University Hospital Zagreb-Rebro, Zagreb, Croatia, HR aut Cvitanović Marijana Department of Paediatrics, University Hospital Zagreb-Rebro, Kišpatićeva 12, 10000 Zagreb, Croatia e-mail: maricvit@mef.hr Tel.: +385-1-213087; Fax: +385-1-2421894, HR aut https://doi.org/10.1007/PL00008361 text/html Onlinezugriff via DOI 1 research-article jats NATIONALLICENCE 856 40 https://doi.org/10.1007/PL00008361 text/html Onlinezugriff via DOI NATIONALLICENCE 700 1- Dumić Miroslav Department of Paediatrics, University Hospital Zagreb-Rebro, Kišpatićeva 12, 10000 Zagreb, Croatia e-mail: maricvit@mef.hr Tel.: +385-1-213087; Fax: +385-1-2421894, HR aut NATIONALLICENCE 700 1- Mravak-Stipetić Marinka Department of Oral Medicine, School of Dental Medicine, University of Zagreb, Zagreb, Croatia, HR aut NATIONALLICENCE 700 1- Kaić Zvonimir Department of Dental Anthropology, School of Dental Medicine, University of Zagreb, Zagreb, Croatia, HR aut NATIONALLICENCE 700 1- Ille Jasenka Department of Paediatrics, University Hospital Zagreb-Rebro, Kišpatićeva 12, 10000 Zagreb, Croatia e-mail: maricvit@mef.hr Tel.: +385-1-213087; Fax: +385-1-2421894, HR aut NATIONALLICENCE 700 1- Plavšić Vesna Endocrinological Laboratory, University Hospital Zagreb-Rebro, Zagreb, Croatia, HR aut NATIONALLICENCE 700 1- Batinica Stipe Department of Child Surgery, University Hospital Zagreb-Rebro, Zagreb, Croatia, HR aut NATIONALLICENCE 700 1- Cvitanović Marijana Department of Paediatrics, University Hospital Zagreb-Rebro, Kišpatićeva 12, 10000 Zagreb, Croatia e-mail: maricvit@mef.hr Tel.: +385-1-213087; Fax: +385-1-2421894, HR aut Metadata rights reserved Springer special CC-BY-NC licence nationallicence BK010053 XK010053 XK010000 NATIONALLICENCE NATIONALLICENCE NL-springer