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      • Record: found
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      Major salivary gland aplasia and hypoplasia in Down syndrome: review of the literature and report of a case

      case-report

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          Key Clinical Message

          Salivary gland aplasia and hypoplasia are rarely described in the medical literature. This article presents a case of aplasia and hypoplasia of the major salivary glands in a patient with Down syndrome. A literature review, as well as an overview of the diagnosis and management of this condition, is presented.

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          Most cited references20

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          The physiology of salivary secretion.

          Gordon Proctor (2016)
          Saliva in the mouth is a biofluid produced mainly by three pairs of major salivary glands--the submandibular, parotid and sublingual glands--along with secretions from many minor submucosal salivary glands. Salivary gland secretion is a nerve-mediated reflex and the volume of saliva secreted is dependent on the intensity and type of taste and on chemosensory, masticatory or tactile stimulation. Long periods of low (resting or unstimulated) flow are broken by short periods of high flow, which is stimulated by taste and mastication. The nerve-mediated salivary reflex is modulated by nerve signals from other centers in the central nervous system, which is most obvious as hyposalivation at times of anxiety. An example of other neurohormonal influences on the salivary reflex is the circadian rhythm, which affects salivary flow and ionic composition. Cholinergic parasympathetic and adrenergic sympathetic autonomic nerves evoke salivary secretion, signaling through muscarinic M3 and adrenoceptors on salivary acinar cells and leading to secretion of fluid and salivary proteins. Saliva gland acinar cells are chloride and sodium secreting, and the isotonic fluid produced is rendered hypotonic by salivary gland duct cells as it flows to the mouth. The major proteins present in saliva are secreted by salivary glands, creating viscoelasticity and enabling the coating of oral surfaces with saliva. Salivary films are essential for maintaining oral health and regulating the oral microbiome. Saliva in the mouth contains a range of validated and potential disease biomarkers derived from epithelial cells, neutrophils, the microbiome, gingival crevicular fluid and serum. For example, cortisol levels are used in the assessment of stress, matrix metalloproteinases-8 and -9 appear to be promising markers of caries and periodontal disease, and a panel of mRNA and proteins has been proposed as a marker of oral squamous cell carcinoma. Understanding the mechanisms by which components enter saliva is an important aspect of validating their use as biomarkers of health and disease.
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            Is salivary gland ultrasonography a useful tool in Sjögren's syndrome? A systematic review.

            Sandrine Jousse-Joulin, Vera Milic, Malin Jonsson … (2016)
            Ultrasonography (US) is a sensitive tool in the diagnosis of major salivary gland abnormalities in primary Sjögren's syndrome (pSS). The aim of this systematic review was to assess the metric properties of this technique.
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              Major salivary gland imaging.

              M Kraut, David M Yousem, A Chalian (2000)
              The algorithm for imaging the salivary glands depends on the clinical scenario with which the patient presents to the clinician. Because of the importance of identifying small calculi in the gland or salivary duct as the cause of the symptom complex, nonenhanced computed tomography is often the best initial study for the evaluation of the painful gland. If an infiltrative neoplasm is highly suspected, nonenhanced and enhanced magnetic resonance (MR) imaging may be superior in demonstrating perineural, meningeal, and skull base invasion. Sialography is reserved for the evaluation of chronic sialadenitides unrelated to sialolithiasis. Thin-section MR techniques for MR sialography may soon replace conventional sialography.
              Article 0 comments Cited 40 times – based on 0 reviews     Review now
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                Author and article information

                Contributors
                Adel Kauzman: adel.kauzman@umontreal.ca
                Journal
                Journal ID (nlm-ta): Clin Case Rep
                Journal ID (iso-abbrev): Clin Case Rep
                Journal ID (doi): 10.1002/(ISSN)2050-0904
                Journal ID (publisher-id): CCR3
                Title: Clinical Case Reports
                Publisher: John Wiley and Sons Inc. (Hoboken )
                ISSN (Electronic): 2050-0904
                Publication date (Electronic): 04 May 2017
                Publication date Collection: June 2017
                Volume: 5
                Issue: 6 ( doiID: 10.1002/ccr3.2017.5.issue-6 )
                Pages: 939-944
                Affiliations
                [ 1 ] Faculty of DentistryUniversité de Montréal Montréal QuébecCanada
                [ 2 ]Private practice Laval QuébecCanada
                Author notes
                [*] [* ] Correspondence

                Adel Kauzman, Faculty of Dentistry, Université de Montréal, PO box 6128, Centre‐ville station, Montréal, Qc, H3C 3J7 Canada. Tel: +1 514‐343‐6081;

                Fax: +1 514‐343‐2233;

                E‐mail: adel.kauzman@ 123456umontreal.ca

                Author information
                http://orcid.org/0000-0002-1812-1412
                Article
                Publisher ID: CCR3975
                DOI: 10.1002/ccr3.975
                PMC ID: 5457992
                SO-VID: 37b61ce7-194a-4b65-bdaa-265215de55cd
                Copyright © © 2017 The Authors. Clinical Case Reports published by John Wiley & Sons Ltd.
                License:

                This is an open access article under the terms of the Creative Commons Attribution License, which permits use, distribution and reproduction in any medium, provided the original work is properly cited.

                History
                Date received : 11 October 2016
                Date revision received : 21 March 2017
                Date accepted : 22 March 2017
                Page count
                Figures: 4, Tables: 1, Pages: 6, Words: 3254
                Categories
                Subject: Case Report
                Subject: Case Reports
                Custom metadata
                source-schema-version-number 2.0
                component-id ccr3975
                cover-date June 2017
                details-of-publishers-convertor Converter:WILEY_ML3GV2_TO_NLMPMC version:5.1.0 mode:remove_FC converted:05.06.2017

                Keywords: congenital abnormalities,down syndrome,parotid gland,salivary glands,tooth wear,xerostomia
                Data availability:
                Keywords: congenital abnormalities, down syndrome, parotid gland, salivary glands, tooth wear, xerostomia

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