{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,9,20]],"date-time":"2026-09-20T18:21:59Z","timestamp":1789928519809,"version":"4.0.1"},"reference-count":45,"publisher":"Springer Science and Business Media LLC","issue":"1","license":[{"start":{"date-parts":[[2018,8,15]],"date-time":"2018-08-15T00:00:00Z","timestamp":1534291200000},"content-version":"tdm","delay-in-days":0,"URL":"http:\/\/creativecommons.org\/licenses\/by\/4.0\/"}],"content-domain":{"domain":["link.springer.com"],"crossmark-restriction":false},"short-container-title":["Orphanet J Rare Dis"],"published-print":{"date-parts":[[2018,12]]},"DOI":"10.1186\/s13023-018-0882-7","type":"journal-article","created":{"date-parts":[[2018,8,15]],"date-time":"2018-08-15T08:18:59Z","timestamp":1534321139000},"update-policy":"https:\/\/doi.org\/10.1007\/springer_crossmark_policy","source":"Crossref","is-referenced-by-count":23,"title":["Characterization of vertigo and hearing loss in patients with Fabry disease"],"prefix":"10.1186","volume":"13","author":[{"ORCID":"https:\/\/orcid.org\/0000-0003-2431-2511","authenticated-orcid":false,"given":"Maria","family":"K\u00f6ping","sequence":"first","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Wafaa","family":"Shehata-Dieler","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Dieter","family":"Schneider","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Mario","family":"Cebulla","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Daniel","family":"Oder","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Jonas","family":"M\u00fcntze","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Peter","family":"Nordbeck","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Christoph","family":"Wanner","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Rudolf","family":"Hagen","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Sebastian P.","family":"Schraven","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"297","published-online":{"date-parts":[[2018,8,15]]},"reference":[{"issue":"4","key":"882_CR1","doi-asserted-by":"publisher","first-page":"338","DOI":"10.7326\/0003-4819-138-4-200302180-00014","volume":"138","author":"RJ Desnick","year":"2003","unstructured":"Desnick RJ, Brady RO, Barranger J, Collins AJ, Germain DP, Goldman M, et al. Fabry disease, an under-recognized multisystemic disorder: expert recommendations for diagnosis, management, and enzyme replacement therapy. Ann Intern Med. 2003;138(4):338\u201346.","journal-title":"Ann Intern Med"},{"key":"882_CR2","unstructured":"Arbeitsgemeinschaft der Wissenschaftlichen Medizinischen Fachgesellschaften (AWMF) (2013): S2k-Leitlinie: Interdisziplin\u00e4re Leitlinie f\u00fcr die Diagnose und Therapie des Morbus Fabry. Online verf\u00fcgbar unter. \n                    http:\/\/www.awmf.org\/uploads\/tx_szleitlinien\/030-134l_S2k_Morbus_Fabry_Diagnose_Therapie_2013-abgelaufen.pdf\n                    \n                  . Accessed 12 Aug 2018."},{"issue":"1","key":"882_CR3","doi-asserted-by":"publisher","first-page":"1","DOI":"10.1186\/s13023-015-0253-6","volume":"10","author":"M Biegstraaten","year":"2015","unstructured":"Biegstraaten M, Arngr\u00edmsson R, Barbey F, Boks L, Cecchi F, Deegan PB, et al. Recommendations for initiation and cessation of enzyme replacement therapy in patients with Fabry disease: the European Fabry working group consensus document. Orphanet J Rare Dis. 2015;10(1):1\u201310.","journal-title":"Orphanet J Rare Dis"},{"issue":"1\u20132","key":"882_CR4","doi-asserted-by":"publisher","first-page":"5","DOI":"10.1016\/j.jns.2014.06.029","volume":"344","author":"R El-Abassi","year":"2014","unstructured":"El-Abassi R, Singhal D, England JD. Fabry\u2019s disease. J Neurol Sci. 2014;344(1\u20132):5\u201319.","journal-title":"J Neurol Sci"},{"issue":"3","key":"882_CR5","doi-asserted-by":"publisher","first-page":"249","DOI":"10.1001\/jama.281.3.249","volume":"281","author":"PJ Meikle","year":"1999","unstructured":"Meikle PJ, Hopwood JJ, Clague AE, Carey WF. Prevalence of lysosomal storage disorders. J Am Med Assoc. 1999;281(3):249\u201354.","journal-title":"J Am Med Assoc"},{"issue":"26","key":"882_CR6","first-page":"440","volume":"106","author":"B Hoffmann","year":"2009","unstructured":"Hoffmann B, Mayatepek E. Fabry disease-often seen, rarely diagnosed. Dtsch Arztebl Int. 2009;106(26):440\u20137.","journal-title":"Dtsch Arztebl Int"},{"issue":"1","key":"882_CR7","doi-asserted-by":"publisher","first-page":"31","DOI":"10.1086\/504601","volume":"79","author":"M Spada","year":"2006","unstructured":"Spada M, Pagliardini S, Yasuda M, Tukel T, Thiagarajan G, Sakuraba H, et al. High incidence of later-onset Fabry disease revealed by newborn screening. Am J Hum Genet. 2006;79(1):31\u201340.","journal-title":"Am J Hum Genet"},{"issue":"10","key":"882_CR8","doi-asserted-by":"publisher","first-page":"1397","DOI":"10.1002\/humu.21074","volume":"30","author":"WL Hwu","year":"2009","unstructured":"Hwu WL, Chien YH, Lee NC, Chiang SC, Dobrovolny R, Huang AC, et al. Newborn screening for Fabry disease in Taiwan reveals a high incidence of the later-onset GLA mutation c.936+919G>A (IVS4+919G>A). Hum Mutat. 2009;30(10):1397\u2013405.","journal-title":"Hum Mutat"},{"issue":"5","key":"882_CR9","doi-asserted-by":"publisher","first-page":"450","DOI":"10.1161\/CIRCGENETICS.109.862920","volume":"2","author":"HY Lin","year":"2009","unstructured":"Lin HY, Chong KW, Hsu JH, Yu HC, Shih CC, Huang CH, et al. High incidence of the cardiac variant of Fabry disease revealed by newborn screening in the Taiwan Chinese population. Circ Cardiovasc Genet. 2009;2(5):450\u20136.","journal-title":"Circ Cardiovasc Genet"},{"issue":"11","key":"882_CR10","doi-asserted-by":"publisher","first-page":"769","DOI":"10.1136\/jmg.38.11.769","volume":"38","author":"KD MacDermot","year":"2001","unstructured":"MacDermot KD, Holmes A, Miners AH. Anderson-Fabry disease: clinical manifestations and impact of disease in a cohort of 60 obligate carrier females. J Med Genet. 2001a;38(11):769\u201375.","journal-title":"J Med Genet"},{"issue":"11","key":"882_CR11","doi-asserted-by":"publisher","first-page":"750","DOI":"10.1136\/jmg.38.11.750","volume":"38","author":"KD MacDermot","year":"2001","unstructured":"MacDermot KD, Holmes A, Miners AH. Anderson-Fabry disease: clinical manifestations and impact of disease in a cohort of 98 hemizygous males. J Med Genet. 2001b;38(11):750\u201360.","journal-title":"J Med Genet"},{"issue":"1","key":"882_CR12","doi-asserted-by":"publisher","first-page":"65","DOI":"10.1016\/j.pharmthera.2009.01.003","volume":"122","author":"R Schiffmann","year":"2009","unstructured":"Schiffmann R. Fabry disease. Pharmacol Ther. 2009;122(1):65\u201377.","journal-title":"Pharmacol Ther"},{"issue":"1","key":"882_CR13","doi-asserted-by":"publisher","first-page":"86","DOI":"10.1080\/08035250500275022","volume":"95","author":"U Ramaswami","year":"2006","unstructured":"Ramaswami U, Whybra C, Parini R, Pintos-Morell G, Mehta A, Sunder-Plassmann G, et al. Clinical manifestations of Fabry disease in children: data from the Fabry outcome survey. Acta Paediatr. 2006;95(1):86\u201392.","journal-title":"Acta Paediatr"},{"issue":"3","key":"882_CR14","doi-asserted-by":"publisher","first-page":"236","DOI":"10.1111\/j.1365-2362.2004.01309.x","volume":"34","author":"A Mehta","year":"2004","unstructured":"Mehta A, Ricci R, Widmer U, Dehout F, Garcia de Lorenzo A, Kampmann C, et al. Fabry disease defined: baseline clinical manifestations of 366 patients in the Fabry outcome survey. Eur J Clin Investig. 2004;34(3):236\u201342.","journal-title":"Eur J Clin Investig"},{"key":"882_CR15","doi-asserted-by":"publisher","first-page":"1","DOI":"10.1186\/1471-2350-3-10","volume":"3","author":"DP Germain","year":"2002","unstructured":"Germain DP, Avan P, Chassaing A, Bonfils P. Patients affected with Fabry disease have an increased incidence of progressive hearing loss and sudden deafness: an investigation of twenty-two hemizygous male patients. BMC Med Genet. 2002;3:1\u201310.","journal-title":"BMC Med Genet"},{"issue":"0","key":"882_CR16","doi-asserted-by":"publisher","first-page":"33","DOI":"10.1111\/j.1651-2227.2003.tb00219.x","volume":"92","author":"G Conti","year":"2003","unstructured":"Conti G, Sergi B. Auditory and vestibular findings in Fabry disease: a study of hemizygous males and heterozygous females. Acta Paediatr. 2003;92(0):33\u20137.","journal-title":"Acta Paediatr"},{"issue":"10","key":"882_CR17","doi-asserted-by":"publisher","first-page":"1433","DOI":"10.1007\/s00415-007-0575-y","volume":"254","author":"A Palla","year":"2007","unstructured":"Palla A, Hegemann S, Widmer U, Straumann D. Vestibular and auditory deficits in Fabry disease and their response to enzyme replacement therapy. J Neurol. 2007;254(10):1433\u201342.","journal-title":"J Neurol"},{"issue":"5 Pt 1","key":"882_CR18","doi-asserted-by":"publisher","first-page":"359","DOI":"10.1177\/000348948909800509","volume":"98","author":"PA Schachern","year":"1989","unstructured":"Schachern PA, Shea DA, Paparella MM, Yoon TH. Otologic histopathology of Fabry\u2019s disease. Ann Otol Rhinol and Laryngol. 1989;98(5 Pt 1):359\u201363.","journal-title":"Ann Otol Rhinol and Laryngol"},{"issue":"2","key":"882_CR19","doi-asserted-by":"publisher","first-page":"345","DOI":"10.1093\/ndt\/gfi152","volume":"21","author":"R Schiffmann","year":"2006","unstructured":"Schiffmann R, Ries M, Timmons M, Flaherty JT, Brady RO. Long-term therapy with agalsidase alfa for Fabry disease: safety and effects on renal function in a home infusion setting. Nephrol Dial Transplant. 2006;21(2):345\u201354.","journal-title":"Nephrol Dial Transplant"},{"issue":"4","key":"882_CR20","doi-asserted-by":"publisher","first-page":"563","DOI":"10.1016\/j.jpeds.2007.09.007","volume":"152","author":"JE Wraith","year":"2008","unstructured":"Wraith JE, Tylki-Szymanska A, Guffon N, Lien YH, Tsimaratos M, Vellodi A, Germain DP. Safety and efficacy of enzyme replacement therapy with agalsidase beta: an international, open-label study in pediatric patients with Fabry disease. J Pediatr. 2008;152(4):563\u201370.","journal-title":"J Pediatr"},{"issue":"5","key":"882_CR21","doi-asserted-by":"publisher","first-page":"1547","DOI":"10.1681\/ASN.2006080816","volume":"18","author":"DP Germain","year":"2007","unstructured":"Germain DP, Waldek S, Banikazemi M, Bushinsky DA, Charrow J, Desnick RJ, et al. Sustained, long-term renal stabilization after 54 months of agalsidase beta therapy in patients with Fabry disease. J Am Soc Nephrol. 2007;18(5):1547\u201357.","journal-title":"J Am Soc Nephrol"},{"issue":"2","key":"882_CR22","doi-asserted-by":"publisher","first-page":"153","DOI":"10.1136\/hrt.2006.104026","volume":"94","author":"DA Hughes","year":"2008","unstructured":"Hughes DA, Elliott P, Shah J, Zuckerman J, Coghlan G, Brookes J, Mehta A. Effects of enzyme replacement therapy on the cardiomyopathy of Anderson-Fabry disease: a randomised, double-blind, placebo-controlled clinical trial of agalsidase alfa. Heart. 2008;94(2):153\u20138.","journal-title":"Heart"},{"issue":"0","key":"882_CR23","doi-asserted-by":"publisher","first-page":"28","DOI":"10.1111\/j.1651-2227.2003.tb00217.x","volume":"92","author":"D Hajioff","year":"2003","unstructured":"Hajioff D, Goodwin S, Quiney R, Zuckerman J, MacDermot KD, Mehta A. Hearing improvement in patients with Fabry disease treated with agalsidase alfa. Acta Paediatr. 2003;92(0):28\u201330.","journal-title":"Acta Paediatr"},{"issue":"2 Suppl 1","key":"882_CR24","first-page":"1","volume":"39","author":"KDOQI","year":"2002","unstructured":"KDOQI. KDOQI clinical practice guidelines for chronic kidney disease: evaluation, classification, and stratification. Am J Kidney Dis. 2002;39(2 Suppl 1):1\u2013266.","journal-title":"Am J Kidney Dis"},{"key":"882_CR25","volume-title":"Nomenclature and criteria for diagnosis of diseases of the heart and great vessels","author":"The Criteria Committee of the New York Heart Association","year":"1994","unstructured":"The Criteria Committee of the New York Heart Association. Nomenclature and criteria for diagnosis of diseases of the heart and great vessels. 9th ed. Boston: Little and Brown Co.; 1994.","edition":"9"},{"issue":"1","key":"882_CR26","first-page":"8","volume":"7","author":"M Niemann","year":"2014","unstructured":"Niemann M, Rolfs A, St\u00f6rk S, Bijnens B, Breunig F, Beer M, et al. Gene mutations versus clinically relevant phenotypes. Lyso-Gb3 defines Fabry disease. Circ Genom Precis Med. 2014;7(1):8\u201316.","journal-title":"Circ Genom Precis Med"},{"issue":"1","key":"882_CR27","doi-asserted-by":"publisher","first-page":"48","DOI":"10.3109\/05384916709074230","volume":"6","author":"A Spoor","year":"2009","unstructured":"Spoor A. Presbycusis values in relation to noise induced hearing loss. Int J Audiol. 2009;6(1):48\u201357.","journal-title":"Int J Audiol"},{"issue":"12","key":"882_CR28","doi-asserted-by":"publisher","first-page":"2745","DOI":"10.1016\/j.clinph.2007.08.005","volume":"118","author":"Y Chihara","year":"2007","unstructured":"Chihara Y, Iwasaki S, Ushio M, Murofushi T. Vestibular-evoked extraocular potentials by air-conducted sound: another clinical test for vestibular function. Clin Neurophysiol. 2007;118(12):2745\u201351.","journal-title":"Clin Neurophysiol"},{"issue":"6","key":"882_CR29","doi-asserted-by":"publisher","first-page":"739","DOI":"10.1007\/s10545-009-1290-x","volume":"32","author":"A Keilmann","year":"2009","unstructured":"Keilmann A, Hajioff D, Ramaswami U. Ear symptoms in children with Fabry disease: data from the Fabry outcome survey. J Inherit Metab Dis. 2009;32(6):739\u201344.","journal-title":"J Inherit Metab Dis"},{"issue":"Pt 1","key":"882_CR30","first-page":"143","volume":"130","author":"M Ries","year":"2007","unstructured":"Ries M, Kim HJ, Zalewski CK, Mastroianni MA, Moore DF, Brady RO, et al. Neuropathic and cerebrovascular correlates of hearing loss in Fabry disease. Brain. 2007;130(Pt 1):143\u201350.","journal-title":"Brain"},{"key":"882_CR31","doi-asserted-by":"crossref","unstructured":"Germain DP. A new phenotype of Fabry disease with intermediate severity between the classical form and the cardiac variant. Contrib Nephrol. 2001;136:234\u201340.","DOI":"10.1159\/000060194"},{"issue":"11","key":"882_CR32","doi-asserted-by":"publisher","first-page":"e0188103","DOI":"10.1371\/journal.pone.0188103","volume":"12","author":"M K\u00f6ping","year":"2017","unstructured":"K\u00f6ping M, Shehata-Dieler W, Cebulla M, Rak K, Oder D, Schraven S, et al. Cardiac and renal dysfunction is associated with progressive hearing loss in patients with Fabry disease. PLoS One. 2017;12(11):e0188103.","journal-title":"PLoS One"},{"issue":"6","key":"882_CR33","doi-asserted-by":"publisher","first-page":"898","DOI":"10.1212\/01.wnl.0000175987.59991.3d","volume":"65","author":"HK Neuhauser","year":"2005","unstructured":"Neuhauser HK, von Brevern M, Radtke A, Lezius F, Feldmann M, Ziese T, Lempert T. Epidemiology of vestibular vertigo: a neurotologic survey of the general population. Neurology. 2005;65(6):898\u2013904.","journal-title":"Neurology"},{"issue":"6","key":"882_CR34","doi-asserted-by":"publisher","first-page":"412","DOI":"10.1177\/000348940611500603","volume":"115","author":"D Vibert","year":"2006","unstructured":"Vibert D, Blaser B, Ozdoba C, H\u00e4usler R. Fabry\u2019s disease: otoneurologic findings in twelve members of one family. Ann Otol Rhinol Laryngol. 2006;115(6):412\u20138.","journal-title":"Ann Otol Rhinol Laryngol"},{"issue":"3","key":"882_CR35","doi-asserted-by":"publisher","first-page":"185","DOI":"10.1007\/s00106-006-1454-z","volume":"55","author":"A Limberger","year":"2007","unstructured":"Limberger A, Beck M, Delgado-Sanchez S, Keilmann A. H\u00f6rst\u00f6rungen bei Morbus Fabry. HNO. 2007;55(3):185\u20139.","journal-title":"HNO"},{"issue":"2","key":"882_CR36","first-page":"87","volume":"30","author":"B Sergi","year":"2010","unstructured":"Sergi B, Conti G, Paludetti G. Inner ear involvement in Anderson-Fabry disease: long-term follow-up during enzyme replacement therapy. Acta Otorhinolaryngol Ital. 2010;30(2):87\u201393.","journal-title":"Acta Otorhinolaryngol Ital"},{"issue":"2","key":"882_CR37","doi-asserted-by":"publisher","first-page":"42","DOI":"10.4081\/audiores.2017.176","volume":"7","author":"S Carmona","year":"2017","unstructured":"Carmona S, Weinschelbaum R, Pardal A, Marchesoni C, Zuberbuhler P, Acosta P, et al. Neuro-otological and peripheral nerve involvement in Fabry disease. Audiol Res. 2017;7(2):42\u20136.","journal-title":"Audiol Res"},{"key":"882_CR38","doi-asserted-by":"publisher","first-page":"e010422","DOI":"10.1136\/bmjopen-2015-010422","volume":"6","author":"D Oder","year":"2016","unstructured":"Oder D, \u00dc\u00e7eyler N, Liu D, Hu K, Petritsch B, Sommer C, et al. Organ manifestations and long-term outcome of Fabry disease in patients with the GLA haplotype D313Y. BMJ Open. 2016;6:e010422.","journal-title":"BMJ Open"},{"key":"882_CR39","first-page":"S113","volume":"114","author":"A Thomas","year":"2015","unstructured":"Thomas A, Baker R, Mehta A, Hughes D. The N215S mutation results in a distinct subtype of Fabry disease. Mol Genet Metab. 2015;114:S113.","journal-title":"Mol Genet Metab"},{"issue":"12","key":"882_CR40","doi-asserted-by":"publisher","first-page":"2087","DOI":"10.1007\/s11606-008-0801-z","volume":"23","author":"DE Newman-Toker","year":"2008","unstructured":"Newman-Toker DE, Dy FJ, Stanton VA, Zee DS, Calkins H, Robinson KA. How often is dizziness from primary cardiovascular disease true Vertigo? A systematic review. J Gen Intern Med. 2008;23(12):2087\u201394.","journal-title":"J Gen Intern Med"},{"issue":"9","key":"882_CR41","doi-asserted-by":"publisher","first-page":"500","DOI":"10.3766\/jaaa.14.9.5","volume":"14","author":"FW Akin","year":"2003","unstructured":"Akin FW, Murnane OD, Proffitt TM. The effects of click and tone-burst stimulus parameters on the vestibular evoked myogenic potential (VEMP). J Am Acad Audiol. 2003;14(9):500\u20139.","journal-title":"J Am Acad Audiol"},{"issue":"9","key":"882_CR42","doi-asserted-by":"publisher","first-page":"2216","DOI":"10.1016\/j.clinph.2005.06.010","volume":"116","author":"D Basta","year":"2005","unstructured":"Basta D, Todt I, Ernst A. Normative data for P1\/N1-latencies of vestibular evoked myogenic potentials induced by air- or bone-conducted tone bursts. Clin Neurophysiol. 2005;116(9):2216\u20139.","journal-title":"Clin Neurophysiol"},{"issue":"5","key":"882_CR43","doi-asserted-by":"publisher","first-page":"636","DOI":"10.1016\/j.clinph.2009.10.016","volume":"121","author":"SM Rosengren","year":"2010","unstructured":"Rosengren SM, Welgampola MS, Colebatch JG. Vestibular evoked myogenic potentials: past, present and future. Clin Neurophysiol. 2010;121(5):636\u201351.","journal-title":"Clin Neurophysiol"},{"issue":"3","key":"882_CR44","doi-asserted-by":"publisher","first-page":"189","DOI":"10.1080\/010503901316914566","volume":"30","author":"K Brantberg","year":"2009","unstructured":"Brantberg K, Fransson PA. Symmetry measures of vestibular evoked myogenic potentials using objective detection criteria. Scand Audiol. 2009;30(3):189\u201396.","journal-title":"Scand Audiol"},{"issue":"9","key":"882_CR45","doi-asserted-by":"publisher","first-page":"2135","DOI":"10.1016\/j.clinph.2008.05.028","volume":"119","author":"S Iwasaki","year":"2008","unstructured":"Iwasaki S, Smulders YE, Burgess AM, McGarvie LA, MacDougall HG, Halmagyi GM, Curthoys IS. Ocular vestibular evoked myogenic potentials to bone conducted vibration of the midline forehead at Fz in healthy subjects. Clin Neurophysiol. 2008;119(9):2135\u201347.","journal-title":"Clin Neurophysiol"}],"container-title":["Orphanet Journal of Rare Diseases"],"original-title":[],"language":"en","link":[{"URL":"http:\/\/link.springer.com\/content\/pdf\/10.1186\/s13023-018-0882-7.pdf","content-type":"application\/pdf","content-version":"vor","intended-application":"text-mining"},{"URL":"http:\/\/link.springer.com\/article\/10.1186\/s13023-018-0882-7\/fulltext.html","content-type":"text\/html","content-version":"vor","intended-application":"text-mining"},{"URL":"http:\/\/link.springer.com\/content\/pdf\/10.1186\/s13023-018-0882-7.pdf","content-type":"application\/pdf","content-version":"vor","intended-application":"similarity-checking"}],"deposited":{"date-parts":[[2019,8,14]],"date-time":"2019-08-14T19:18:42Z","timestamp":1565810322000},"score":1,"resource":{"primary":{"URL":"https:\/\/ojrd.biomedcentral.com\/articles\/10.1186\/s13023-018-0882-7"}},"subtitle":[],"short-title":[],"issued":{"date-parts":[[2018,8,15]]},"references-count":45,"journal-issue":{"issue":"1","published-print":{"date-parts":[[2018,12]]}},"alternative-id":["882"],"URL":"https:\/\/doi.org\/10.1186\/s13023-018-0882-7","relation":{},"ISSN":["1750-1172"],"issn-type":[{"value":"1750-1172","type":"electronic"}],"subject":[],"published":{"date-parts":[[2018,8,15]]},"assertion":[{"value":"6 May 2018","order":1,"name":"received","label":"Received","group":{"name":"ArticleHistory","label":"Article History"}},{"value":"29 July 2018","order":2,"name":"accepted","label":"Accepted","group":{"name":"ArticleHistory","label":"Article History"}},{"value":"15 August 2018","order":3,"name":"first_online","label":"First Online","group":{"name":"ArticleHistory","label":"Article History"}},{"value":"The study was approved by the institutional review board of the medical department W\u00fcrzburg (20,170,904 01; 220\/15_z). Informed oral and written consent had been obtained of all patients.","order":1,"name":"Ethics","group":{"name":"EthicsHeading","label":"Ethics approval and consent to participate"}},{"value":"All patients gave written informed consent for participation in the study and publication of the study\u2019s results.","order":2,"name":"Ethics","group":{"name":"EthicsHeading","label":"Consent for publication"}},{"value":"DO received speaker\u2019s honoraria from Sanofi-Genzyme and travel assistance from Sanofi-Genzyme and Shire. JM received travel assistance from Sanofi-Genzyme, Shire and Amicus. PN received travel assistance, speaker\u2019s\/advisory board honoraria, and research support from Amicus, Sanofi-Genzyme and Shire. CW received travel assistance, speaker\u2019s\/advisory board honoraria, and research support from Actelion, Protalix, Sanofi-Genzyme and Shire. The other authors declare no conflicts of interest. The funders had no role in study design, data collection and analysis, decision to publish, or preparation of the manuscript.","order":3,"name":"Ethics","group":{"name":"EthicsHeading","label":"Competing interests"}},{"value":"Springer Nature remains neutral with regard to jurisdictional claims in published maps and institutional affiliations.","order":4,"name":"Ethics","group":{"name":"EthicsHeading","label":"Publisher\u2019s Note"}}],"article-number":"137"}}