Bigger BW - Search Results

1

Enzyme replacement therapy prior to haematopoietic stem cell transplantation in Mucopolysaccharidosis Type I: 10 year combined experience of 2 centres.

Ghosh A, Miller W, Orchard PJ, Jones SA, Mercer J, Church HJ, Tylee K, Lund T, Bigger BW, Tolar J, Wynn RF. Ghosh A, et al. Among authors: bigger bw. Mol Genet Metab. 2016 Mar;117(3):373-7. doi: 10.1016/j.ymgme.2016.01.011. Epub 2016 Jan 27. Mol Genet Metab. 2016. PMID: 26832957 Free PMC article.

2

Improved metabolic correction in patients with lysosomal storage disease treated with hematopoietic stem cell transplant compared with enzyme replacement therapy.

Wynn RF, Wraith JE, Mercer J, O'Meara A, Tylee K, Thornley M, Church HJ, Bigger BW. Wynn RF, et al. Among authors: bigger bw. J Pediatr. 2009 Apr;154(4):609-11. doi: 10.1016/j.jpeds.2008.11.005. J Pediatr. 2009. PMID: 19324223 Clinical Trial.

3

Evaluation of heparin cofactor II-thrombin complex as a biomarker on blood spots from mucopolysaccharidosis I, IIIA and IIIB mice.

Langford-Smith K, Arasaradnam M, Wraith JE, Wynn R, Bigger BW. Langford-Smith K, et al. Among authors: bigger bw. Mol Genet Metab. 2010 Mar;99(3):269-74. doi: 10.1016/j.ymgme.2009.10.175. Epub 2009 Oct 23. Mol Genet Metab. 2010. PMID: 19926322

4

Successful allogeneic bone marrow transplant for Niemann-Pick disease type C2 is likely to be associated with a severe 'graft versus substrate' effect.

Bonney DK, O'Meara A, Shabani A, Imrie J, Bigger BW, Jones S, Wraith JE, Wynn RF. Bonney DK, et al. Among authors: bigger bw. J Inherit Metab Dis. 2010 Dec;33 Suppl 3:S171-3. doi: 10.1007/s10545-010-9060-3. J Inherit Metab Dis. 2010. PMID: 20393800

5

Heparin cofactor II-thrombin complex and dermatan sulphate:chondroitin sulphate ratio are biomarkers of short- and long-term treatment effects in mucopolysaccharide diseases.

Langford-Smith KJ, Mercer J, Petty J, Tylee K, Church H, Roberts J, Moss G, Jones S, Wynn R, Wraith JE, Bigger BW. Langford-Smith KJ, et al. Among authors: bigger bw. J Inherit Metab Dis. 2011 Apr;34(2):499-508. doi: 10.1007/s10545-010-9254-8. Epub 2010 Dec 18. J Inherit Metab Dis. 2011. PMID: 21170681 Free PMC article.

6

Chronic norovirus infection in pediatric hematopoietic stem cell transplant recipients: a cause of prolonged intestinal failure requiring intensive nutritional support.

Saif MA, Bonney DK, Bigger B, Forsythe L, Williams N, Page J, Babiker ZO, Guiver M, Turner AJ, Hughes S, Wynn RF. Saif MA, et al. Pediatr Transplant. 2011 Aug;15(5):505-9. doi: 10.1111/j.1399-3046.2011.01500.x. Epub 2011 Apr 19. Pediatr Transplant. 2011. PMID: 21504523

7

Hyperactive behaviour in the mouse model of mucopolysaccharidosis IIIB in the open field and home cage environments.

Langford-Smith A, Malinowska M, Langford-Smith KJ, Wegrzyn G, Jones S, Wynn R, Wraith JE, Wilkinson FL, Bigger BW. Langford-Smith A, et al. Among authors: bigger bw. Genes Brain Behav. 2011 Aug;10(6):673-82. doi: 10.1111/j.1601-183X.2011.00706.x. Epub 2011 Jun 29. Genes Brain Behav. 2011. PMID: 21635693 Free article.

8

Female mucopolysaccharidosis IIIA mice exhibit hyperactivity and a reduced sense of danger in the open field test.

Langford-Smith A, Langford-Smith KJ, Jones SA, Wynn RF, Wraith JE, Wilkinson FL, Bigger BW. Langford-Smith A, et al. Among authors: bigger bw. PLoS One. 2011;6(10):e25717. doi: 10.1371/journal.pone.0025717. Epub 2011 Oct 18. PLoS One. 2011. PMID: 22028789 Free PMC article.

9

Hematopoietic stem cell transplantation improves the high incidence of neutralizing allo-antibodies observed in Hurler's syndrome after pharmacological enzyme replacement therapy.

Saif MA, Bigger BW, Brookes KE, Mercer J, Tylee KL, Church HJ, Bonney DK, Jones S, Wraith JE, Wynn RF. Saif MA, et al. Among authors: bigger bw. Haematologica. 2012 Sep;97(9):1320-8. doi: 10.3324/haematol.2011.058644. Epub 2012 Feb 27. Haematologica. 2012. PMID: 22371174 Free PMC article.

10

Hematopoietic stem cell and gene therapy corrects primary neuropathology and behavior in mucopolysaccharidosis IIIA mice.

Langford-Smith A, Wilkinson FL, Langford-Smith KJ, Holley RJ, Sergijenko A, Howe SJ, Bennett WR, Jones SA, Wraith J, Merry CL, Wynn RF, Bigger BW. Langford-Smith A, et al. Among authors: bigger bw. Mol Ther. 2012 Aug;20(8):1610-21. doi: 10.1038/mt.2012.82. Epub 2012 May 1. Mol Ther. 2012. PMID: 22547151 Free PMC article.

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