{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,4,17]],"date-time":"2026-04-17T03:56:23Z","timestamp":1776398183948,"version":"3.51.2"},"reference-count":23,"publisher":"Wiley","issue":"5","license":[{"start":{"date-parts":[[2020,4,28]],"date-time":"2020-04-28T00:00:00Z","timestamp":1588032000000},"content-version":"vor","delay-in-days":0,"URL":"http:\/\/creativecommons.org\/licenses\/by-nc\/4.0\/"}],"funder":[{"name":"F\u00f6rderverein zur Kontinenzforschung und Kontinenzaufkl\u00e4rung e. V.","award":["17\/001"],"award-info":[{"award-number":["17\/001"]}]}],"content-domain":{"domain":["onlinelibrary.wiley.com"],"crossmark-restriction":true},"short-container-title":["Neurourology and Urodynamics"],"published-print":{"date-parts":[[2020,6]]},"abstract":"<jats:title>Abstract<\/jats:title><jats:sec><jats:title>Aims<\/jats:title><jats:p>In late\u2010onset Pompe disease (LOPD), a lysosomal storage disorder with glycogen accumulation in several tissues, patients suffer from progressive skeletal muscle weakness. Lower urinary tract symptoms (LUTS) have rarely been reported. The aim of this study is to objectively assess LUTS in patients with LOPD for the first time using urodynamic studies and to determine differences between LOPD patients with and without LUTS.<\/jats:p><\/jats:sec><jats:sec><jats:title>Methods<\/jats:title><jats:p>Eighteen patients with LOPD were recruited, of whom seven patients (38.9%) reported LUTS (both voiding and storage symptoms). Six of these patients underwent urodynamic studies. Medical histories and motor function tests were compared between the 7 patients with LUTS and the 11 patients without LUTS. The Student <jats:italic>t<\/jats:italic> test was used to determine an association between the two cohorts.<\/jats:p><\/jats:sec><jats:sec><jats:title>Results<\/jats:title><jats:p>In the seven LOPD patients with LUTS urodynamics revealed neurogenic dysfunction, underactive detrusor, and bladder outlet obstruction. These patients had suffered from clinical symptoms for a longer period of time before starting enzyme replacement therapy (<jats:italic>P<\/jats:italic>\u2009=\u2009.017) than patients without LUTS. They also scored more poorly on muscle function tests. Urodynamic results point to neurogenic causes for LUTS in LOPD, that is, neurogenic reflex bladder or impaired filling sensation. This could be due to glycogen accumulation in the urothelium and central nervous system. Patients with LUTS also seem to be more severely affected by LOPD than patients without LUTS.<\/jats:p><\/jats:sec><jats:sec><jats:title>Conclusion<\/jats:title><jats:p>LUTS in LOPD requires early and specific treatment to limit the development of severe health problems. Urodynamic studies should be considered in assessing LUTS.<\/jats:p><\/jats:sec>","DOI":"10.1002\/nau.24369","type":"journal-article","created":{"date-parts":[[2020,4,28]],"date-time":"2020-04-28T10:21:10Z","timestamp":1588069270000},"page":"1437-1446","update-policy":"https:\/\/doi.org\/10.1002\/crossmark_policy","source":"Crossref","is-referenced-by-count":4,"title":["Urodynamic and clinical studies in patients with late\u2010onset Pompe disease and lower urinary tract symptoms"],"prefix":"10.1002","volume":"39","author":[{"ORCID":"https:\/\/orcid.org\/0000-0001-9041-858X","authenticated-orcid":false,"given":"Katharina S.","family":"Kuchenbecker","sequence":"first","affiliation":[{"name":"Neuro\u2010Urologie\/Klinik f\u00fcr Urologie und Kinderurologie Universit\u00e4tsklinikum Bonn  Bonn Germany"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"ORCID":"https:\/\/orcid.org\/0000-0001-6332-916X","authenticated-orcid":false,"given":"Ruth","family":"Kirschner\u2010Hermanns","sequence":"additional","affiliation":[{"name":"Neuro\u2010Urologie\/Klinik f\u00fcr Urologie und Kinderurologie Universit\u00e4tsklinikum Bonn  Bonn Germany"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Cornelia","family":"Kornblum","sequence":"additional","affiliation":[{"name":"Neuro\u2010Urologie\/Klinik f\u00fcr Urologie und Kinderurologie Universit\u00e4tsklinikum Bonn  Bonn Germany"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Anke","family":"Jaekel","sequence":"additional","affiliation":[{"name":"Neuro\u2010Urologie\/Klinik f\u00fcr Urologie und Kinderurologie Universit\u00e4tsklinikum Bonn  Bonn Germany"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"ORCID":"https:\/\/orcid.org\/0000-0001-5020-3445","authenticated-orcid":false,"given":"Ralf","family":"Anding","sequence":"additional","affiliation":[{"name":"Neuro\u2010Urologie\/Klinik f\u00fcr Urologie und Kinderurologie Universit\u00e4tsklinikum Bonn  Bonn Germany"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Annette","family":"Kohler","sequence":"additional","affiliation":[{"name":"Neuro\u2010Urologie\/Klinik f\u00fcr Urologie und Kinderurologie Universit\u00e4tsklinikum Bonn  Bonn Germany"}],"role":[{"role":"author","vocabulary":"crossref"}]}],"member":"311","published-online":{"date-parts":[[2020,4,28]]},"reference":[{"key":"e_1_2_9_2_1","doi-asserted-by":"publisher","DOI":"10.1016\/j.ymgme.2012.05.007"},{"key":"e_1_2_9_3_1","first-page":"42","article-title":"Glycogen storage disease type II: clinical overview","volume":"26","author":"Di Rocco MD","year":"2007","journal-title":"Acta Myol"},{"key":"e_1_2_9_4_1","first-page":"73","article-title":"Extensive genetic heterogeneity in patients with acid alpha glucosidase deficiency as detected by abnormalities of DNA and mRNA","volume":"47","author":"Martiniuk F","year":"1990","journal-title":"Am J Hum Genet"},{"key":"e_1_2_9_5_1","doi-asserted-by":"publisher","DOI":"10.1038\/sj.ejhg.5200367"},{"key":"e_1_2_9_6_1","doi-asserted-by":"publisher","DOI":"10.1097\/GIM.0b013e318211a7e1"},{"key":"e_1_2_9_7_1","doi-asserted-by":"publisher","DOI":"10.1016\/j.nmd.2007.06.002"},{"key":"e_1_2_9_8_1","doi-asserted-by":"publisher","DOI":"10.1212\/WNL.0000000000008441"},{"key":"e_1_2_9_9_1","doi-asserted-by":"publisher","DOI":"10.1007\/s00415-016-8219-8"},{"key":"e_1_2_9_10_1","doi-asserted-by":"publisher","DOI":"10.1007\/8904_2014_334"},{"key":"e_1_2_9_11_1","doi-asserted-by":"publisher","DOI":"10.1038\/labinvest.3700484"},{"key":"e_1_2_9_12_1","doi-asserted-by":"publisher","DOI":"10.1016\/j.urology.2004.08.063"},{"key":"e_1_2_9_13_1","doi-asserted-by":"publisher","DOI":"10.1016\/j.juro.2016.07.071"},{"key":"e_1_2_9_14_1","doi-asserted-by":"publisher","DOI":"10.1111\/dmcn.13740"},{"key":"e_1_2_9_15_1","first-page":"191","article-title":"The epidemiology and pathophysiology of neurogenic bladder","volume":"19","author":"Ginsberg D","year":"2013","journal-title":"Am J Manag Care"},{"key":"e_1_2_9_16_1","first-page":"127","article-title":"Detrusor sphincter dyssynergia: a review of physiology, diagnosis and treatment strategies","volume":"5","author":"Stoffel JT","year":"2016","journal-title":"Transl Androl Urol"},{"key":"e_1_2_9_17_1","doi-asserted-by":"publisher","DOI":"10.1007\/s00192-012-1682-6"},{"key":"e_1_2_9_18_1","doi-asserted-by":"publisher","DOI":"10.1159\/000338776"},{"key":"e_1_2_9_19_1","first-page":"42","volume-title":"Pelvic Floor Re\u2010Education","author":"Laycock J","year":"1994"},{"key":"e_1_2_9_20_1","doi-asserted-by":"publisher","DOI":"10.1007\/BF00257163"},{"key":"e_1_2_9_21_1","doi-asserted-by":"publisher","DOI":"10.1002\/nau.22653"},{"key":"e_1_2_9_22_1","doi-asserted-by":"publisher","DOI":"10.1016\/S0022-5347(17)49698-4"},{"key":"e_1_2_9_23_1","first-page":"122","article-title":"BPH: epidemiology and comorbidities","volume":"12","author":"McVary KT","year":"2006","journal-title":"Am J Manag Care"},{"key":"e_1_2_9_24_1","doi-asserted-by":"publisher","DOI":"10.2174\/138161208784705504"}],"container-title":["Neurourology and Urodynamics"],"original-title":[],"language":"en","link":[{"URL":"https:\/\/api.wiley.com\/onlinelibrary\/tdm\/v1\/articles\/10.1002%2Fnau.24369","content-type":"application\/pdf","content-version":"vor","intended-application":"text-mining"},{"URL":"https:\/\/onlinelibrary.wiley.com\/doi\/pdf\/10.1002\/nau.24369","content-type":"application\/pdf","content-version":"vor","intended-application":"text-mining"},{"URL":"https:\/\/onlinelibrary.wiley.com\/doi\/full-xml\/10.1002\/nau.24369","content-type":"application\/xml","content-version":"vor","intended-application":"text-mining"},{"URL":"https:\/\/onlinelibrary.wiley.com\/doi\/pdf\/10.1002\/nau.24369","content-type":"unspecified","content-version":"vor","intended-application":"similarity-checking"}],"deposited":{"date-parts":[[2023,9,5]],"date-time":"2023-09-05T21:48:04Z","timestamp":1693950484000},"score":1,"resource":{"primary":{"URL":"https:\/\/onlinelibrary.wiley.com\/doi\/10.1002\/nau.24369"}},"subtitle":[],"short-title":[],"issued":{"date-parts":[[2020,4,28]]},"references-count":23,"journal-issue":{"issue":"5","published-print":{"date-parts":[[2020,6]]}},"alternative-id":["10.1002\/nau.24369"],"URL":"https:\/\/doi.org\/10.1002\/nau.24369","archive":["Portico"],"relation":{},"ISSN":["0733-2467","1520-6777"],"issn-type":[{"value":"0733-2467","type":"print"},{"value":"1520-6777","type":"electronic"}],"subject":[],"published":{"date-parts":[[2020,4,28]]},"assertion":[{"value":"2019-12-16","order":0,"name":"received","label":"Received","group":{"name":"publication_history","label":"Publication History"}},{"value":"2020-04-14","order":1,"name":"accepted","label":"Accepted","group":{"name":"publication_history","label":"Publication History"}},{"value":"2020-04-28","order":2,"name":"published","label":"Published","group":{"name":"publication_history","label":"Publication History"}}]}}