Psychometric evaluation before and after BMT. A comparison of pre-BMT and most recent results of the psychometric evaluation of 10 children with MPS-I
| Patient No | Pre-BMT results | Most recent results | Discrepancy in SD | |||||||
|---|---|---|---|---|---|---|---|---|---|---|
| Age (years) | Test | GQ | SD2-150 | Age (years) | Test | GQ/IQ | SD2-150 | |||
| 12-151 | 0.7 | GMDS | 77 (68) | −1.5 (−2.1) | 15.7 | BAS | 77 (70) | −1.5 (−2.0) | < ±1.0 (< ±1.0) | |
| 2 | 1.2 | GMDS | 76 (67) | −1.6 (−2.2) | 15.5 | BAS | 122 (115) | +1.5 (+1.0) | +3.7 (+3.2) | |
| 32-151 | 1.9 | GMDS | 82 (72) | −1.0 (−1.8) | 16.2 | WISC | <50 | > 3.30 | >−2.3 (−1.5) | |
| 4 | 2.0 | GMDS | 70 (63) | −2.0 (−2.6) | 11.1 | BAS | 82 (75) | −1.2 (−1.6) | < ±1.0 (< ±1.0) | |
| 5 | 1.0 | GMDS | 118 (107) | +1.2 (+0.5) | 13.5 | WISC | 68 | −2.1 | −3.3 (−2.6) | |
| 62-152 | 2.2 | GMDS | 74 (63) | −1.8 (−2.5) | 13.8 | GMDS | 16 (1) | −5.6 (−6.6) | −3.8 (−4.1) | |
| 72-151 | 2.8 | GMDS | 48 (36) | −3.5 (−4.2) | 5.2 | GMDS | 48 (35) | −3.5 (−4.3) | < ±1.0 (< ±1.0) | |
| 82-151 | 0.4 | GMDS | 79 (67) | −1.4 (−2.2) | 8.4 | BAS | 88 (81) | −0.8 (−1.3) | < ±1.0 (< ±1.0) | |
| 9 | 1.7 | GMDS | 67 (55) | −2.2 (−3.0) | 8.3 | BAS | <62 (<55) | > −2.5 (−3.0) | < ±1.0 (< ±1.0) | |
| 10 | 0.8 | GMDS | 89 (76) | −0.8 (−1.6) | 5.7 | GMDS | 65 (50) | −2.4 (−3.4) | −1.6 (−1.8) | |
↵2-150 SD from mean, that is from GQ/IQ = 100. Corrected GQ/IQ is given in parentheses. Discrepancies between pre-BMT GQ and most recent GQ/IQ are given. The pre-BMT ages refer to age at testing, not at BMT.
↵2-151 These four children underwent BMT twice. The age at testing pre-BMT is the age before second BMT.
↵2-152 GMDS was used in this 13.8 year old child post-BMT, although the GMDS is only standardised for children up to the age of 8.