Johnson Barbara A, MacWilliams Bruce A, Carey John C, Viskochil David H, D'Astous Jacques L, Stevenson David A
Shriners Hospitals for Children Salt Lake City, Salt Lake City, Utah, USA.
Pediatr Phys Ther. 2010 Winter;22(4):344-8. doi: 10.1097/PEP.0b013e3181f9dbc8.
Neurofibromatosis type 1 (NF1) is a genetic disorder with associated musculoskeletal abnormalities, tumors, and developmental delays. The purpose of this study was to investigate and characterize the motor proficiency of children with NF1.
Children with NF1 were assessed using the Bruininks-Oseretsky Test (BOT 2) instrument. The NF1 group scores were compared with age and sex-matched test norms.
Twenty-six children participated in the study. The NF1 group had statistically significant lower scores (P < .05) for the total motor composite (Z = -1.62) and 7 of the 8 subtests. Nineteen percent (n = 5) scored in the average category, 54% (n = 14) scored in the below-average category, and 27% (n = 7) scored in the well-below-average category.
Children with NF1 have significantly lower motor proficiency than the BOT 2 normative scores. The results indicate the BOT 2 is useful in identifying and characterizing delays in motor proficiency for children with NF1.
1型神经纤维瘤病（NF1）是一种伴有肌肉骨骼异常、肿瘤和发育迟缓的遗传性疾病。本研究的目的是调查和描述NF1患儿的运动能力。
使用布鲁因inks-奥塞雷茨基测试（BOT 2）工具对NF1患儿进行评估。将NF1组的分数与年龄和性别匹配的测试常模进行比较。
26名儿童参与了该研究。NF1组在总运动综合得分（Z = -1.62）以及8个分测试中的7个分测试中，得分在统计学上显著较低（P <.05）。19%（n = 5）的儿童得分处于平均水平，54%（n = 14）的儿童得分处于低于平均水平，27%（n = 7）的儿童得分处于远低于平均水平。
NF1患儿的运动能力明显低于BOT 2常模分数。结果表明，BOT 2有助于识别和描述NF1患儿的运动能力延迟情况。
