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We examined the long\u2010term survival and cardiopulmonary outcome in 54 children undergoing haematopoietic stem cell transplantation (HSCT) at the Royal Manchester Children's Hospital from 1985 to 2008. The median age at first HSCT was 15.1 months. Eighteen had graft failure and nine died after first HSCT. Of 18 patients with graft failure, 17 underwent second HSCT and the remaining one was lost to follow\u2010up (LOF). Twelve were alive\u2010and\u2010engrafted after second HSCT. The overall survival at one year and 20\u2010years was the same at 73.7%. Six children were followed up at the referral centers and excluded from cardiopulmonary endpoint review. Of the 33 evaluable children for the cardiopulmonary endpoints, nine (27.3%) had normal cardiac assessment. Of the four children on angiotensin\u2010converting\u2010enzyme inhibitors, two had mild cardiomyopathy and two had aortic valvular replacement. Twenty (60%) had mild\/moderate mitral and\/or aortic insufficiencies. Two had overnight hypoxia needing nocturnal non\u2010invasive support. Enzyme level and donor chimerism are important predictors of long\u2010term cardiac outcome.<\/jats:p>","DOI":"10.1007\/s10545-017-0034-6","type":"journal-article","created":{"date-parts":[[2017,3,10]],"date-time":"2017-03-10T12:52:13Z","timestamp":1489150333000},"page":"455-460","update-policy":"https:\/\/doi.org\/10.1007\/springer_crossmark_policy","source":"Crossref","is-referenced-by-count":36,"title":["Long term survival and cardiopulmonary outcome in children with Hurler syndrome after haematopoietic stem cell transplantation"],"prefix":"10.1002","volume":"40","author":[{"given":"Su Han","family":"Lum","sequence":"first","affiliation":[{"name":"Department of Paediatric Blood and Marrow Transplant Royal Manchester Children's Hospital Oxford Rd Manchester M13 9WL UK"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Karolina M.","family":"Stepien","sequence":"additional","affiliation":[{"name":"Mark Holland Metabolic Unit, Adult Inherited Metabolic Disorders Salford 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