{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,10,24]],"date-time":"2026-10-24T23:59:34Z","timestamp":1792886374505,"version":"4.5.0"},"reference-count":30,"publisher":"Wiley","issue":"2","license":[{"start":{"date-parts":[[2013,2,13]],"date-time":"2013-02-13T00:00:00Z","timestamp":1360713600000},"content-version":"vor","delay-in-days":0,"URL":"http:\/\/creativecommons.org\/licenses\/by-nc\/4.0\/"}],"content-domain":{"domain":[],"crossmark-restriction":false},"short-container-title":["J of Inher Metab Disea"],"published-print":{"date-parts":[[2013,3]]},"abstract":"<jats:title>Abstract<\/jats:title><jats:p>Nearly all children with MPS IVA develop skeletal deformities affecting the spine. At the atlanto\u2010axial spine, odontoid hypoplasia occurs. GAG deposition around the dens, leads to peri\u2010odontoid infiltration. Transverse\/alar ligament incompetence causes instability. Atlanto\u2010axial instability is associated with cord compression and myelopathy, leading to major morbidity and mortality. Intervention is often required. Does the presence of widened bullet shaped vertebra in platyspondily encroach on the spinal canal and cause spinal stenosis in MPS IVA? So far, there have been no standardised morphometric measurements of the paediatric MPS IVA cervical spine to evaluate whether there is pre\u2010existing spinal stenosis predisposing to compressive myelopathy or whether this is purely an acquired process secondary to instability and compression. This study provides the first radiological quantitative analysis of the cervical spine and spinal cord in a series of affected children. MRI morphometry indicates that the MPS IVA spine is narrower at C1\u20132 level giving an inverted funnel shape. There is no evidence of a reduction in the Torg ratio (canal\u2010body ratio) in the cervical spine. The spinal canal does not exceed 11 mm at any level, significantly smaller than normal historical cohorts (14 mm). The sagittal diameter and axial surface area of both spinal canal and cord are reduced. C1\u20132 level cord compression was evident in the canal\u2010cord ratio but the Torg ratio was not predictive of cord compression. In MPS IVA the reduction in the space available for the cord (SAC) is multifactorial rather than due to congenital spinal stenosis.<\/jats:p>","DOI":"10.1007\/s10545-013-9585-3","type":"journal-article","created":{"date-parts":[[2013,2,12]],"date-time":"2013-02-12T06:17:06Z","timestamp":1360649826000},"page":"329-337","source":"Crossref","is-referenced-by-count":26,"title":["MRI morphometric characterisation of the paediatric cervical spine and spinal cord in children with MPS IVA (Morquio\u2010Brailsford syndrome)"],"prefix":"10.1002","volume":"36","author":[{"given":"Guirish A.","family":"Solanki","sequence":"first","affiliation":[{"name":"Department of Paediatric Neurosurgery Birmingham Children's Hospital NHS Foundation Trust Steelhouse lane Birmingham B4 6NH UK"},{"name":"School of Clinical and Experimental Medicine, College of Medical and Dental Sciences University of Birmingham Birmingham UK"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"William B.","family":"Lo","sequence":"additional","affiliation":[{"name":"Birmingham Children's Hospital NHS Foundation Trust Birmingham UK"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Christian J.","family":"Hendriksz","sequence":"additional","affiliation":[{"name":"Manchester Academic Health Science Centre University of Manchester Manchester UK"}],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"311","published-online":{"date-parts":[[2013,2,13]]},"reference":[{"key":"e_1_2_9_2_1","doi-asserted-by":"publisher","DOI":"10.1302\/0301-620X.81B1.0810110"},{"key":"e_1_2_9_3_1","doi-asserted-by":"publisher","DOI":"10.1016\/S0002-9610(29)90496-7"},{"key":"e_1_2_9_4_1","doi-asserted-by":"publisher","DOI":"10.1177\/036354659702500503"},{"key":"e_1_2_9_5_1","first-page":"307","article-title":"Occipito\u2010atlanto\u2010axial fusion in Morquio\u2010Brailsford syndrome. A 10\u2010year experience.","volume":"78","author":"Crockard HA","year":"1996","journal-title":"J Bone Joint Surg Br"},{"key":"e_1_2_9_6_1","doi-asserted-by":"publisher","DOI":"10.1073\/pnas.43.6.443"},{"key":"e_1_2_9_7_1","doi-asserted-by":"publisher","DOI":"10.1302\/0301-620X.71B3.2722928"},{"key":"e_1_2_9_8_1","doi-asserted-by":"publisher","DOI":"10.1097\/00007632-199110001-00016"},{"key":"e_1_2_9_9_1","unstructured":"HendrikszCJ Al\u2010JawadM BergerKI HawleySM LawrenceR Mc ArdleC SummersCG et al (2012)Clinical overview and treatment options for non\u2010skeletal manifestations of mucopolysaccharidosis type IVA.J Inherit Metab Disdoi:10.1007\/s10545\u2010012\u20109459\u20100"},{"key":"e_1_2_9_10_1","doi-asserted-by":"publisher","DOI":"10.1097\/00007632-199106001-00001"},{"key":"e_1_2_9_11_1","doi-asserted-by":"publisher","DOI":"10.1148\/79.1.97"},{"key":"e_1_2_9_12_1","doi-asserted-by":"publisher","DOI":"10.1007\/s002340050429"},{"key":"e_1_2_9_13_1","doi-asserted-by":"publisher","DOI":"10.1097\/00007632-200202010-00009"},{"key":"e_1_2_9_14_1","doi-asserted-by":"publisher","DOI":"10.1038\/sj.sc.3101375"},{"key":"e_1_2_9_15_1","doi-asserted-by":"publisher","DOI":"10.1097\/00007632-199409150-00013"},{"key":"e_1_2_9_16_1","doi-asserted-by":"publisher","DOI":"10.1097\/00007632-199606010-00001"},{"key":"e_1_2_9_17_1","doi-asserted-by":"publisher","DOI":"10.1007\/s00586-012-2176-4"},{"key":"e_1_2_9_18_1","doi-asserted-by":"publisher","DOI":"10.1136\/jnnp.38.12.1218"},{"key":"e_1_2_9_19_1","doi-asserted-by":"publisher","DOI":"10.1097\/00007632-199310001-00006"},{"key":"e_1_2_9_20_1","doi-asserted-by":"publisher","DOI":"10.1097\/00007632-200003150-00005"},{"key":"e_1_2_9_21_1","first-page":"129","article-title":"Sur une forme de dystrophie osseuse familiale","volume":"32","author":"Morquio L","year":"1929","journal-title":"Arch Med Enfants"},{"key":"e_1_2_9_22_1","doi-asserted-by":"publisher","DOI":"10.1007\/BF01799267"},{"key":"e_1_2_9_23_1","doi-asserted-by":"publisher","DOI":"10.1148\/radiology.164.3.3615879"},{"key":"e_1_2_9_24_1","doi-asserted-by":"publisher","DOI":"10.1097\/00007632-199015100-00004"},{"key":"e_1_2_9_25_1","volume-title":"MPS VI Cervical Cord Compression Advisory Board Meeting","author":"Solanki G.","year":"2011"},{"key":"e_1_2_9_26_1","doi-asserted-by":"publisher","DOI":"10.1302\/0301-620X.73B5.1910048"},{"issue":"2","key":"e_1_2_9_27_1","first-page":"190","article-title":"Cervical spine stenosis measures in normal subjects","volume":"37","author":"Tierney RT","year":"2002","journal-title":"J Athl Train"},{"key":"e_1_2_9_28_1","doi-asserted-by":"publisher","DOI":"10.2174\/138920111795542615"},{"key":"e_1_2_9_29_1","doi-asserted-by":"publisher","DOI":"10.3171\/jns.1997.87.6.0843"},{"key":"e_1_2_9_30_1","doi-asserted-by":"publisher","DOI":"10.1136\/adc.72.3.263"},{"key":"e_1_2_9_31_1","doi-asserted-by":"publisher","DOI":"10.1097\/01.bsd.0000181294.67212.79"}],"container-title":["Journal of Inherited Metabolic Disease"],"original-title":[],"language":"en","link":[{"URL":"https:\/\/onlinelibrary.wiley.com\/doi\/pdf\/10.1007\/s10545-013-9585-3","content-type":"unspecified","content-version":"vor","intended-application":"similarity-checking"}],"deposited":{"date-parts":[[2023,9,15]],"date-time":"2023-09-15T08:00:43Z","timestamp":1694764843000},"score":1,"resource":{"primary":{"URL":"https:\/\/onlinelibrary.wiley.com\/doi\/10.1007\/s10545-013-9585-3"}},"subtitle":[],"short-title":[],"issued":{"date-parts":[[2013,2,13]]},"references-count":30,"journal-issue":{"issue":"2","published-print":{"date-parts":[[2013,3]]}},"alternative-id":["10.1007\/s10545-013-9585-3"],"URL":"https:\/\/doi.org\/10.1007\/s10545-013-9585-3","archive":["Portico"],"relation":{},"ISSN":["0141-8955","1573-2665"],"issn-type":[{"value":"0141-8955","type":"print"},{"value":"1573-2665","type":"electronic"}],"subject":[],"published":{"date-parts":[[2013,2,13]]}}}