Use of the Bruininks-Oseretsky test of motor proficiency (BOT-2) to assess efficacy of velmanase alfa as enzyme therapy for alpha-mannosidosis

Phillips, Dawn; Hennermann, Julia B.; Tylki‐Szymańska, Anna; Borgwardt, Line; Gil-Campos, Mercedes; Guffon, Nathalie; Amraoui, Yasmina; Geraci, Silvia; Ardigò, Diego; Cattaneo, Federica; Lund, Allan M.

doi:10.1016/j.ymgmr.2020.100586

Cited by 6 publications

(4 citation statements)

References 19 publications

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“…This study lacked normative data from the general population regarding the outcome of the BOT‐2 domains. Studies comparing results from patient populations (obesity, hypermobility syndrome, developmental disorder, sickle cell disease) and healthy children showed lower results for patients 42–45 . However, unlike the population in this study, these groups were less involved in sports/physical activity than the general population 6,7 .…”

Section: Discussioncontrasting

confidence: 72%

“…Studies comparing results from patient populations (obesity, hypermobility syndrome, developmental disorder, sickle cell disease) and healthy children showed lower results for patients. [42][43][44][45] However, unlike the population in this study, these groups were less involved in sports/physical activity than the general population. 6,7 As motor proficiency is associated with sports participation, 46 the present study population is expected to show similar results as the general population.…”

Section: Adults (Median Proportion (Iqr))mentioning

confidence: 65%

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Can motor proficiency testing predict sports injuries and sports‐induced bleeds in people with haemophilia?

et al. 2023

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Introduction:Predicting the risk of sports injuries and sports-induced bleeds (SIBs) in people with haemophilia (PWH) may support clinical counselling. Aim:To assess the association between motor proficiency testing and sports injuries and SIBs and to identify a specific set of tests for predicting injury risk in PWH. Methods:In a single centre, prospective study male PWH aged 6-49 playing sports ≥1x/week were tested for running speed and agility, balance, strength and endurance.Test results below −2Z were considered poor. Sports injuries and SIBs were collected for 12 months while 7 days of physical activity (PA) for each season was registered with accelerometers. Injury risk was analysed according to test results and type of physical activity (%time walking, cycling, running). Predictive values for sports injuries and SIBs were determined.Results: Data from 125 PWH (mean [± SD] age: 25 [± 12], 90% haemophilia A; 48% severe, 95% on prophylaxis, median factor level: 2.5 [IQR 0-15]IU/dl) were included.Few participants (n = 19, 15%) had poor scores. Eighty-seven sports injuries and 26 SIBs were reported. Poor scoring participants reported 11/87 sports injuries and 5/26 SIBs. The current tests were poor predictors of sports injuries (Range PPV: 0%-40%), or SIBs (PPV: 0%-20%). PA type was not associated with season (activity seasonal p values > .20) and type of PA was not associated with sports injuries or SIBs (Spearman's rho < .15). Conclusion:These motor proficiency-and endurance tests were unable to predict sports injuries or SIBs in PWH, potentially due to few PWH with poor results and low numbers of sports injuries and SIBs.

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Section: Discussioncontrasting

confidence: 72%

Section: Adults (Median Proportion (Iqr))mentioning

confidence: 65%

Can motor proficiency testing predict sports injuries and sports‐induced bleeds in people with haemophilia?

et al. 2023

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“…In addition to that, agility tests are also often used to measure physical function. The two-leg side hop test is a speed and agility test part of the BOT-2 ( Bruininks and Bruininks, 2005 ), that is widely used in children and young adults to assess functional performance ( Phillips et al, 2020 ; Marchese et al, 2021 ). A distinct characteristic of many dynamic stability tests, including the FSST, and speed and agility tests, such as the two-leg hop test, is that a better score (e.g., lower time and/or higher count of jumps) indicates better physical function.…”

Section: Introductionmentioning

confidence: 99%

Hip Abductor and Adductor Rate of Torque Development and Muscle Activation, but Not Muscle Size, Are Associated With Functional Performance

et al. 2021

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Understanding the physiological variables that contribute to a functional task provides important information for trainers and clinicians to improve functional performance. The hip abductors and adductors muscles appear to be important in determining the performance of some functional tasks; however, little is known about the relationship of the hip abductor/adductors muscle strength, activation, and size with functional performance. This study aimed to investigate the relationship of maximum torque, rate of torque development (RTD), rate of activation (RoA), and muscle thickness of the hip abductors [tensor fascia latae (TFL) and gluteus medius (GM)] and adductor magnus muscle with the Four Square Step Test (FSST) and the two-leg hop test in healthy young adults. Twenty participants (five males) attended one testing session that involved ultrasound image acquisition, maximal isometric voluntary contractions (hip abduction and hip adduction) while surface electromyography (EMG) was recorded, and two functional tests (FSST and two-leg side hop test). Bivariate correlations were performed between maximum voluntary torque (MVT), RTD at 50, 100, 200, and 300ms, RoA at 0–50, 0–100, 0–200, and 0–300, and muscle thickness with the dynamic stability tests. For the hip abduction, MVT (r=−0.455, p=0.044) and RTD300 (r=−0.494, p=0.027) was correlated with the FSST. GM RoA50 (r=−0.481, p=0.032) and RoA100 (r=−0.459, p=0.042) were significantly correlated with the two-leg side hop test. For the hip adduction, there was a significant correlation between the FSST and RTD300 (r=−0.500, p=0.025), while the two-leg side hop test was correlated with RTD200 (r=0.446, p=0.049) and RTD300 (r=0.594, p=0.006). Overall, the ability of the hip abductor and adductor muscles to produce torque quickly, GM rapid activation, and hip abductor MVT is important for better performance on the FSST and two-leg hop tests. However, muscle size appears not to influence the same tests.

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“…Велманаза альфа предназначена для лечения детей и взрослых с легкой и среднетяжелой формами альфа-маннозидоза, вводится внутривенно, медленно (в соответствии с разработанной схемой) 1 раз в неделю в дозе 1 мг на 1 кг массы тела. В работах, посвященных отдаленным (не менее 4 лет) результатам заместительной ферментной терапии, констатируется улучшение двигательной активности детей, состояния мелкой моторики, функциональных и биохимических параметров, а также иммунологических показателей [12,13] В России, по данным Медико-генетического научного центра им. академика Н.П.…”

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Alpha-mannosidosis in children: analysis of the observations and treatment options

Семячкина

Николаева

Воскобоева

et al. 2020

Ross. vestn. perinatol. pediatr.

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Б ольшая группа лизосомных болезней накопления (более 50) привлекает внимание клиницистов в связи с наличием выраженного фенотипического сходства отдельных заболеваний этой группы, затрудняющего постановку истинного диагноза, а также с разработкой и внедрением в практику патогенети-© Коллектив авторов, 2020 Адрес для корреспонденции: Семячкина Алла Николаевна-д.м.н., гл. науч. сотр. отдела клинической генетики Научно-исследовательского клинического института педиатрии им. академика Ю.Е. Вельтищева,

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Use of the Bruininks-Oseretsky test of motor proficiency (BOT-2) to assess efficacy of velmanase alfa as enzyme therapy for alpha-mannosidosis

Cited by 6 publications

References 19 publications

Can motor proficiency testing predict sports injuries and sports‐induced bleeds in people with haemophilia?

Can motor proficiency testing predict sports injuries and sports‐induced bleeds in people with haemophilia?

Hip Abductor and Adductor Rate of Torque Development and Muscle Activation, but Not Muscle Size, Are Associated With Functional Performance

Alpha-mannosidosis in children: analysis of the observations and treatment options

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