Abstract
Children with Hurler syndrome (mucopolysaccharidosis type IH (MPSIH)) have skeletal, joint and soft tissue abnormalities that may persist or progress after hematopoietic stem cell transplantation (HSCT). We report our single center experience with development of carpal tunnel syndrome (CTS) in 43 children with MPSIH after HSCT. Twenty-three children (59%) developed CTS following HSCT; 19 of the 39 children with enzyme activity in the normal or heterozygous range developed CTS (49%), whereas all four children with low heterozygous or absent enzyme activity developed CTS after HSCT. Fourteen of 19 related donor marrow recipients, eight of 19 of those receiving an unrelated donor graft and one of five unrelated cord blood recipients developed CTS. The mean age at surgical release was 4.8 years. With each year increase in age at HSCT, there was a 55% increased risk. Age and enzyme activity after HSCT were significant factors in the development of CTS. Transplantation by 2 years of age reduced the risk of developing CTS by 46%; higher enzyme activity led to a 78% reduction in the risk of developing CTS. However, children transplanted for MPSIH remain at risk for the development of CTS, and should be monitored on an ongoing basis by nerve conduction velocity testing.
This is a preview of subscription content, access via your institution
Access options
Subscribe to this journal
Receive 12 print issues and online access
$259.00 per year
only $21.58 per issue
Buy this article
- Purchase on Springer Link
- Instant access to full article PDF
Prices may be subject to local taxes which are calculated during checkout
Similar content being viewed by others
References
Neufeld EF, Muenzer J . The Mucopolysaccharidoses. In: Scriver CR, Beaudet AL, Sly WS, Valle D (eds). The Metabolic and Molecular Bases of Inherited Disease, 7th edn. McGraw-Hill: New York, 1995, pp 2465–2494.
Guffon N, Souillet G, Maire I, Straczek J, Guibaud P . Follow-up of nine patients with Hurler syndrome after bone marrow transplantation. J Pediatr 1998; 133: 119–125.
Peters C, Balthazor M, Shapiro EG, King RJ, Kollman C, Hegland JD et al. Outcome of unrelated donor bone marrow transplantation in 40 children with Hurler syndrome. Blood 1996; 87: 4894–4902.
Peters C, Shapiro EG, Anderson J, Henslee-Downey PJ, Klemperer MR, Cowan MJ et al. Hurler syndrome: II. outcome of HLA-genotypically identical sibling and HLA-haploidentical related donor bone marrow transplantation in fifty-four children. The Storage Disease Collaborative Study Group. Blood 1998; 91: 2601–2608.
Peters C, Shapiro EG, Krivit W . Neuropsychological development in children with Hurler syndrome following hematopoietic stem cell transplantation. Pediatr Transplant 1998; 2: 250–253.
Vellodi A, Young EP, Cooper A, Wraith JE, Winchester B, Meaney C et al. Bone marrow transplantation for mucopolysaccharidosis type I: experience of two British centres. Arch Dis Child 1997; 76: 92–99.
Phipps S, Mulhern R . Developmental outcome of unrelated donor bone marrow transplantation in children with Hurler syndrome. Blood 1997; 89: 732–734.
Shapiro EG, Lockman LA, Balthazor M, Krivit W . Neuropsychological outcomes of several storage diseases with and without bone marrow transplantation. J Inherit Metab Dis 1995; 18: 413–429.
Braunlin EA, Stauffer NR, Peters CH, Bass JI, Berry JM, Hopwood JJ et al. Usefulness of bone marrow transplantation in the Hurler syndrome. Am J Cardiol 2003; 92: 882–886.
Field RE, Buchanan JA, Copplemans MG, Aichroth PM . Bone-marrow transplantation in Hurler's syndrome. Effect on skeletal development. J Bone Joint Surg Br 1994; 76: 975–981.
Odunusi E, Peters C, Krivit W, Ogilvie J . Genu valgum deformity in Hurler syndrome after hematopoietic stem cell transplantation: correction by surgical intervention. J Pediatr Orthop 1999; 19: 270–274.
Van Heest AE, House J, Krivit W, Walker K . Surgical treatment of carpal tunnel syndrome and trigger digits in children with mucopolysaccharide storage disorders. J Hand Surg (Am) 1998; 23: 236–243.
Wraith JE, Alani SM . Carpal tunnel syndrome in the mucopolysaccharidoses and related disorders. Arch Dis Child 1990; 65: 962–963.
Cruz Martinez A, Arpa J . Carpal tunnel syndrome in childhood: study of 6 cases. Electroencephalogr Clin Neurophysiol 1998; 109: 304–308.
Fisher RC, Horner RL, Wood VE . The hand in mucopolysaccharide disorders. Clin Orthop Relat Res 1974, 191–199.
Gschwind C, Tonkin MA . Carpal tunnel syndrome in children with mucopolysaccharidosis and related disorders. J Hand Surg (Am) 1992; 17: 44–47.
Haddad FS, Hill RA, Jones DH . Triggering in the mucopolysaccharidoses. J Pediatr Orthop B 1998; 7: 138–140.
MacDougal B, Weeks PM, Wray Jr RC . Median nerve compression and trigger finger in the mucopolysaccharidoses and related diseases. Plast Reconstr Surg 1977; 59: 260–263.
Hall CW, Neufeld EF . Alpha-L-iduronidase activity in cultured skin fibroblasts and amniotic fluid cells. Arch Biochem Biophys 1973; 158: 817–821.
Wenger D, Williams C . Screening for lysosomal disorders. In: Hommes F (ed). Techniques in Diagnostic Human Biochemical Genetics: A Laboratory Manual. Wiley-Liss: New York, 1991, pp 587–661.
Acknowledgements
This work was supported by the Children's Cancer Research Fund.
Author information
Authors and Affiliations
Corresponding author
Rights and permissions
About this article
Cite this article
Khanna, G., Heest, A., Agel, J. et al. Analysis of factors affecting development of carpal tunnel syndrome in patients with Hurler syndrome after hematopoietic cell transplantation. Bone Marrow Transplant 39, 331–334 (2007). https://doi.org/10.1038/sj.bmt.1705586
Received:
Accepted:
Published:
Issue Date:
DOI: https://doi.org/10.1038/sj.bmt.1705586
Keywords
This article is cited by
-
Early detection of median nerve compression by Electroneurography can improve outcome in children with Mucopolysaccharidoses
Orphanet Journal of Rare Diseases (2018)
-
Open issues in Mucopolysaccharidosis type I-Hurler
Orphanet Journal of Rare Diseases (2017)
-
Musculoskeletal manifestations in mucopolysaccharidosis type I (Hurler syndrome) following hematopoietic stem cell transplantation
Orphanet Journal of Rare Diseases (2016)
-
The craniocervical junction following successful haematopoietic stem cell transplantation for mucopolysaccharidosis type I H (Hurler syndrome)
Journal of Inherited Metabolic Disease (2011)
-
Orthopaedic management of Hurler's disease after hematopoietic stem cell transplantation: a systematic review
Journal of Inherited Metabolic Disease (2011)