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87 Efficacy of hematopoietic cell transplantation in metachromatic leukodystrophy 5 each follow-up and regarded as normal if within the range of normal according to the institutional reference range, and low if below the lower limit of normal but not within disease range. RESULTS At latest assessment all patients were full chimeras. ASA activity at latest assessment was in the normal range for 11/13 patients. In 2 patients, ASA activity was low (36 and 38 nmol/mg/17h), but substantially higher than the disease range (0–11 nmol/h/mg). The sibling donor for both patients was heterozygous for the pseudodeficiency allele, resulting in this low activity, as no matched unrelated donor could be found. REFERENCES 1 Kehrer C, Blumenstock G, Raabe C, Krageloh-Mann I. Development and reliability of a classificationsystemforgrossmotorfunctioninchildrenwithmetachromaticleucodystrophy. Dev Med Child Neurol. 2011;53:156–160. 2 Royden Jones H, Bolton CF, Harper CM. Pediatric Clinical Electromyography. Philadelphia, Lippincott-Raven Publishers; 1995. 3 Puksa L, Stalberg E, Falck B. Reference values of F wave parameters in healthy subjects. Clin Neurophysiol. 2003;114:1079–1090. 4 Wechsler D. Wechsler Intelligence Scale for Children- Fourth Edition Edition (WISC-IV). San Antonio,TX: Psychological Corporation; 2003. 5 Bode MM, D'Eugenio DB, Mettelman BB, Gross SJ. Predictive validity of the Bayley, Third Edition at 2 years for intelligence quotient at 4 years in preterm infants. J Dev Behav Pediatr. 2014;35:570–575. 6 Eichler F, Grodd W, Grant E et al. Metachromatic leukodystrophy: a scoring system for brain MR imaging observations. AJNR Am J Neuroradiol. 2009;30:1893–1897. 7 Glucksberg H, Storb R, Fefer A et al. Clinical manifestations of graft-versus-host disease in human recipients of marrow from HL-A-matched sibling donors. Transplantation. 1974;18:295–304.