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Living related transplantation for MSUD--caution, or a new path forward?
Soltys KA, Mazariegos GV, Strauss KA. Soltys KA, et al. Among authors: strauss ka. Pediatr Transplant. 2015 May;19(3):247-8. doi: 10.1111/petr.12423. Pediatr Transplant. 2015. PMID: 25808910 No abstract available.
Liver transplantation for treatment of severe S-adenosylhomocysteine hydrolase deficiency.
Strauss KA, Ferreira C, Bottiglieri T, Zhao X, Arning E, Zhang S, Zeisel SH, Escolar ML, Presnick N, Puffenberger EG, Vugrek O, Kovacevic L, Wagner C, Mazariegos GV, Mudd SH, Soltys K. Strauss KA, et al. Mol Genet Metab. 2015 Sep-Oct;116(1-2):44-52. doi: 10.1016/j.ymgme.2015.06.005. Epub 2015 Jun 19. Mol Genet Metab. 2015. PMID: 26095522
Hepatic Parenchymal Injury in Crigler-Najjar Type I.
Mitchell E, Ranganathan S, McKiernan P, Squires RH, Strauss K, Soltys K, Mazariegos G, Squires JE. Mitchell E, et al. Among authors: strauss k. J Pediatr Gastroenterol Nutr. 2018 Apr;66(4):588-594. doi: 10.1097/MPG.0000000000001843. J Pediatr Gastroenterol Nutr. 2018. PMID: 29176474
Living related versus deceased donor liver transplantation for maple syrup urine disease.
Feier F, Schwartz IV, Benkert AR, Seda Neto J, Miura I, Chapchap P, da Fonseca EA, Vieira S, Zanotelli ML, Pinto e Vairo F, Camelo JS Jr, Margutti AV, Mazariegos GV, Puffenberger EG, Strauss KA. Feier F, et al. Among authors: strauss ka. Mol Genet Metab. 2016 Mar;117(3):336-43. doi: 10.1016/j.ymgme.2016.01.005. Epub 2016 Jan 12. Mol Genet Metab. 2016. PMID: 26786177
Liver transplantation for classical maple syrup urine disease: long-term follow-up in 37 patients and comparative United Network for Organ Sharing experience.
Mazariegos GV, Morton DH, Sindhi R, Soltys K, Nayyar N, Bond G, Shellmer D, Shneider B, Vockley J, Strauss KA. Mazariegos GV, et al. Among authors: strauss ka. J Pediatr. 2012 Jan;160(1):116-21.e1. doi: 10.1016/j.jpeds.2011.06.033. Epub 2011 Aug 11. J Pediatr. 2012. PMID: 21839471 Free PMC article.
Elective liver transplantation for the treatment of classical maple syrup urine disease.
Strauss KA, Mazariegos GV, Sindhi R, Squires R, Finegold DN, Vockley G, Robinson DL, Hendrickson C, Virji M, Cropcho L, Puffenberger EG, McGhee W, Seward LM, Morton DH. Strauss KA, et al. Am J Transplant. 2006 Mar;6(3):557-64. doi: 10.1111/j.1600-6143.2005.01209.x. Am J Transplant. 2006. PMID: 16468966
Cognitive and adaptive functioning after liver transplantation for maple syrup urine disease: a case series.
Shellmer DA, DeVito Dabbs A, Dew MA, Noll RB, Feldman H, Strauss KA, Morton DH, Vockley J, Mazariegos GV. Shellmer DA, et al. Among authors: strauss ka. Pediatr Transplant. 2011 Feb;15(1):58-64. doi: 10.1111/j.1399-3046.2010.01411.x. Epub 2010 Oct 8. Pediatr Transplant. 2011. PMID: 20946191 Free PMC article.
Classical maple syrup urine disease and brain development: principles of management and formula design.
Strauss KA, Wardley B, Robinson D, Hendrickson C, Rider NL, Puffenberger EG, Shellmer D, Moser AB, Morton DH. Strauss KA, et al. Mol Genet Metab. 2010 Apr;99(4):333-45. doi: 10.1016/j.ymgme.2009.12.007. Epub 2010 Jan 12. Mol Genet Metab. 2010. PMID: 20061171 Free PMC article. Clinical Trial.
Recessive GM3 synthase deficiency: Natural history, biochemistry, and therapeutic frontier.
Bowser LE, Young M, Wenger OK, Ammous Z, Brigatti KW, Carson VJ, Moser T, Deline J, Aoki K, Morlet T, Scott EM, Puffenberger EG, Robinson DL, Hendrickson C, Salvin J, Gottlieb S, Heaps AD, Tiemeyer M, Strauss KA. Bowser LE, et al. Among authors: strauss ka. Mol Genet Metab. 2019 Apr;126(4):475-488. doi: 10.1016/j.ymgme.2019.01.013. Epub 2019 Jan 21. Mol Genet Metab. 2019. PMID: 30691927
Prevention of brain disease from severe 5,10-methylenetetrahydrofolate reductase deficiency.
Strauss KA, Morton DH, Puffenberger EG, Hendrickson C, Robinson DL, Wagner C, Stabler SP, Allen RH, Chwatko G, Jakubowski H, Niculescu MD, Mudd SH. Strauss KA, et al. Mol Genet Metab. 2007 Jun;91(2):165-75. doi: 10.1016/j.ymgme.2007.02.012. Epub 2007 Apr 3. Mol Genet Metab. 2007. PMID: 17409006
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