{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2022,9,7]],"date-time":"2022-09-07T04:14:59Z","timestamp":1662524099331},"reference-count":6,"publisher":"Springer Science and Business Media LLC","issue":"1","license":[{"start":{"date-parts":[[2022,9,6]],"date-time":"2022-09-06T00:00:00Z","timestamp":1662422400000},"content-version":"tdm","delay-in-days":0,"URL":"https:\/\/creativecommons.org\/licenses\/by\/4.0"},{"start":{"date-parts":[[2022,9,6]],"date-time":"2022-09-06T00:00:00Z","timestamp":1662422400000},"content-version":"vor","delay-in-days":0,"URL":"https:\/\/creativecommons.org\/licenses\/by\/4.0"}],"funder":[{"name":"Proveca Pharma Limited"}],"content-domain":{"domain":["link.springer.com"],"crossmark-restriction":false},"short-container-title":["Ital J Pediatr"],"abstract":"<jats:title>Abstract<\/jats:title><jats:sec>\n                <jats:title>Background<\/jats:title>\n                <jats:p>Goldenhar syndrome (GS) is a rare congenital disease characterized by impaired development of different facial structures and deformations of the teeth structures. Sialorrhea, which can cause difficulties in breathing and language impairment, is very common in GS and often difficult to treat. This case report highlights the short- and long-term importance of the therapeutic choice \u2013 glycopyrronium in oral solution - for the treatment of sialorrhea in children with poly-malformative syndrome, complicated by outcomes of post-hemorrhagic hydrocephalus.<\/jats:p>\n              <\/jats:sec><jats:sec>\n                <jats:title>Case presentation<\/jats:title>\n                <jats:p>We report the case of a 6-year-old child with GS, carrying a percutaneous endoscopic gastrostomy after tracheostomy. The child also presented developmental dysfunction of oral motor skills of feeding, complicated by severe sialorrhea, related to the maxillo-facial dysmorphism. Sialorrhea caused several respiratory tract infections and led to an increase in the care burden. Both the inoculations of botulinum toxin and the treatment with scopolamine transdermal patch have shown mild and transient efficacy. The therapeutic choice of glycopyrronium in oral solution was the most suitable for this patient, leading to long-term sialorrhea control.<\/jats:p>\n              <\/jats:sec><jats:sec>\n                <jats:title>Conclusions<\/jats:title>\n                <jats:p>This clinical experience represents the first long-term efficacy and tolerability evaluation in using glycopyrrolate oral solution in treating drooling in children with GS. The reduction of drooling over time and the lack of clinically relevant adverse events have contributed to the decrease of respiratory tract infections, the development of oral motor skills, and determining a positive psycho-social impact on the patient's quality of life and her family.<\/jats:p>\n              <\/jats:sec>","DOI":"10.1186\/s13052-022-01320-8","type":"journal-article","created":{"date-parts":[[2022,9,6]],"date-time":"2022-09-06T21:05:58Z","timestamp":1662498358000},"update-policy":"http:\/\/dx.doi.org\/10.1007\/springer_crossmark_policy","source":"Crossref","is-referenced-by-count":0,"title":["Long-term efficacy of glycopyrrolate on sialorrhea in Goldenhar syndrome: a case report"],"prefix":"10.1186","volume":"48","author":[{"given":"Gessica","family":"Della Bella","sequence":"first","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Enrico","family":"Castelli","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Federico","family":"Vigevano","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]}],"member":"297","published-online":{"date-parts":[[2022,9,6]]},"reference":[{"issue":"5","key":"1320_CR1","doi-asserted-by":"publisher","first-page":"405","DOI":"10.1007\/s12519-017-0048-z","volume":"13","author":"K Bogusiak","year":"2017","unstructured":"Bogusiak K, Puch A, Arkuszewski P. Goldenhar syndrome: current perspectives. World J Pediatr. 2017;13(5):405\u201315. https:\/\/doi.org\/10.1007\/s12519-017-0048-z.","journal-title":"World J Pediatr."},{"issue":"4","key":"1320_CR2","doi-asserted-by":"publisher","first-page":"397","DOI":"10.2174\/1871530318666180328122035","volume":"18","author":"R Marvulli","year":"2018","unstructured":"Marvulli R, Gallo GA, Mastromauro L, et al. Efficacy and non invasive treatment of sialorrhea in the Goldenhar syndrome. Endocr Metab Immune Disord Drug Targets. 2018;18(4):397\u2013400. https:\/\/doi.org\/10.2174\/1871530318666180328122035.","journal-title":"Endocr Metab Immune Disord Drug Targets."},{"issue":"2","key":"1320_CR3","doi-asserted-by":"publisher","first-page":"117","DOI":"10.1272\/jnms.JNMS.2019_86-207","volume":"86","author":"Y Yamamura","year":"2019","unstructured":"Yamamura Y, Nonaka M. Sialorrhea successfully treated by the combined use of selective M1 and M3 muscarinic acetylcholine receptor antagonists. J Nippon Med Sch. 2019;86(2):117\u201321. https:\/\/doi.org\/10.1272\/jnms.JNMS.2019_86-207.","journal-title":"J Nippon Med Sch."},{"key":"1320_CR4","unstructured":"Summary of Product Characteristics. Sialanar 320 micrograms\/ml Glycopyrronium (400 micrograms\/ml Glycopyrronium Bromide) Oral Solution. Proveca Limited. Last updated on eMC 4\/1\/2017. Accessed 30 Mar 2017. http:\/\/www.medicines.org.uk\/emc\/medicine\/32715"},{"issue":"2","key":"1320_CR5","doi-asserted-by":"publisher","first-page":"e23","DOI":"10.1111\/j.1469-8749.2009.03519.x","volume":"52","author":"SM Reid","year":"2010","unstructured":"Reid SM, Johnson HM, Reddihough DS. The Drooling Impact Scale: a measure of the impact of drooling in children with developmental disabilities. Dev Med Child Neurol. 2010;52(2):e23\u20138. https:\/\/doi.org\/10.1111\/j.1469-8749.2009.03519.x.","journal-title":"Dev Med Child Neurol."},{"key":"1320_CR6","unstructured":"Guideline for managing Guideline on Management of Drooling in Children 2132-53709.doc D https:\/\/www.shropscommunityhealth.nhs.uk\/content\/doclib\/13593.pdf"}],"container-title":["Italian Journal of Pediatrics"],"original-title":[],"language":"en","link":[{"URL":"https:\/\/link.springer.com\/content\/pdf\/10.1186\/s13052-022-01320-8.pdf","content-type":"application\/pdf","content-version":"vor","intended-application":"text-mining"},{"URL":"https:\/\/link.springer.com\/article\/10.1186\/s13052-022-01320-8\/fulltext.html","content-type":"text\/html","content-version":"vor","intended-application":"text-mining"},{"URL":"https:\/\/link.springer.com\/content\/pdf\/10.1186\/s13052-022-01320-8.pdf","content-type":"application\/pdf","content-version":"vor","intended-application":"similarity-checking"}],"deposited":{"date-parts":[[2022,9,6]],"date-time":"2022-09-06T21:07:09Z","timestamp":1662498429000},"score":1,"resource":{"primary":{"URL":"https:\/\/ijponline.biomedcentral.com\/articles\/10.1186\/s13052-022-01320-8"}},"subtitle":[],"short-title":[],"issued":{"date-parts":[[2022,9,6]]},"references-count":6,"journal-issue":{"issue":"1","published-online":{"date-parts":[[2022,12]]}},"alternative-id":["1320"],"URL":"https:\/\/doi.org\/10.1186\/s13052-022-01320-8","relation":{},"ISSN":["1824-7288"],"issn-type":[{"value":"1824-7288","type":"electronic"}],"subject":[],"published":{"date-parts":[[2022,9,6]]},"assertion":[{"value":"18 November 2021","order":1,"name":"received","label":"Received","group":{"name":"ArticleHistory","label":"Article History"}},{"value":"8 July 2022","order":2,"name":"accepted","label":"Accepted","group":{"name":"ArticleHistory","label":"Article History"}},{"value":"6 September 2022","order":3,"name":"first_online","label":"First Online","group":{"name":"ArticleHistory","label":"Article History"}},{"order":1,"name":"Ethics","group":{"name":"EthicsHeading","label":"Declarations"}},{"value":"All methods were performed in accordance with the ethical standards as laid down in the Declaration of Helsinki and its later amendments or comparable ethical standards. Ethics approval was obtained by Hospital Scientific Direction. Written informed consent was obtained from a parent and\/or legal guardian","order":2,"name":"Ethics","group":{"name":"EthicsHeading","label":"Ethics approval and consent to participate"}},{"value":"Written informed consent was obtained from a parent and\/or legal guardian for publication of this case report and accompanying images","order":3,"name":"Ethics","group":{"name":"EthicsHeading","label":"Consent for publication"}},{"value":"The authors declare that they have no competing interests.","order":4,"name":"Ethics","group":{"name":"EthicsHeading","label":"Competing interests"}}],"article-number":"166"}}