[CHARACTERISTICS AND TRENDS OF CONGENITAL AND ACQUIRED LOWER LIMB DEFICIENCY IN CHILDREN AND ADOLESCENTS AT THE SHEBA MEDICAL CENTER].

INTRODUCTION: Lower limb deficiency in childhood has an impact on acquisition of motor skills. Information regarding the characteristics of this population was examined in several countries but not in Israel. AIMS: To provide demographics, clinical and functional characteristics of children with lower limb deficiency in a pediatric rehabilitation department. METHODS: Children with lower limb deficiency participated in this study. The study variables included demographics, and clinical and functional characteristics. The statistical analysis included calculations of frequency, chi-squared tests and correlations. RESULTS: During the years 1998-2015 fifty-eight children with lower extremity deformity were treated/examined in the department (mean age: 6.46+4.70 years; girls, n=21; congenital deformity, n=23; acquired deformity, n=35). The most common congenital and acquired injury was unilateral leg deformity (31% and 35%, respectively). In congenital injury, multi-limb deformity (including an involvement in the upper limb) is more prevalent than bilateral lower limb deformity (p<0.01). In children with congenital deformity, longitudinal deformity is more prevalent than transverse deformity (p<0.03). Among children with acquired injury, in 40% the etiology was sickness-related and in the rest traumatic. Among the traumatic group, 57% of the injuries were terror-related. More than 50% percent of the children underwent a complex surgical procedure (34% and 22% among congenital and acquired injury, respectively). The ambulation level of the sample was lower than expected. CONCLUSIONS: Children who received treatment due to lower limb deformity presented high variability in their characteristics and low ambulation level. Consequently, it is important to create and manage a register for pediatric lower limb deformity.

Gross motor proficiency deficits among children and adolescents post posterior fossa brain tumor removal vs. traumatic brain injury in the chronic phase of recovery: a cross-sectional study.

Introduction: Acquired brain injury (ABI) is a prevalent diagnosis in pediatric rehabilitation. Gross motor skills are often affected by ABI and limit the ability to participate in various physical activities. However, as ABI injury location is diverse, children and adolescents (youth) with localized ABI, such as ABI in the posterior fossa (ABI-PF) may present unique and different motor disabilities than youth with ABI on account of traumatic brain injury (TBI). Aims: The aims of the study were: (1) to compare gross motor deficits in youth with TBI vs. ABI-PF; and (2) to compare two methods on scoring BOT2 to determine which is better for identifying motor deficits. Methods: Participated in this study youth with TBI (N = 50) and ABI-PF (N = 30). Participants were tested on Bruininks-Oseretsky Test of Motor Proficiency-2nd Edition (BOT2) Upper-Limb Coordination, Balance, Strength, Running Speed and Agility, and Bilateral-Coordination subtests. Motor performance deficits were established using two-standard deviations (2SD) and age-equivalent methods. Between-group differences were assessed via independent t-tests and receiver operating characteristic curves (ROC). Results: According to the 2SD method, motor deficits in the ABI-PF group ranged from 20% to 66.66%, whereas in the TBI group 8%-16%. According to the age-equivalent method, in the TBI and ABI-PF groups 40%-66.0% and 46.66%-76.66% of the youth presented motor deficits, respectively. Moreover, ROC analysis showed that motor performance deficits of both groups in all sub-scales except for Bilateral Coordination differed enough to result in medium area under the curve. Conclusions: Motor deficits post-pediatric ABI are prevalent. In comparison to the TBI group, deficits are greater in the ABI-PF group. Moreover, compared to the 2SD method, the extent of motor deficiency is greater in the age-equivalent method. Therefore, using the later might provide a more valid classification of deficits in gross motor proficiency for youth post-ABI.

Functional neurological symptom disorder: Preliminary findings of factors associated with walking ability post integrative pediatric rehabilitation.

PURPOSE: The current study's aims were to (1) examine long-distance walking ability (6-minute walking test [6MWT]) and walking hemodynamic responses (i.e., heart rate) among youth with functional neurological symptom disorder (FNSD) before and after an integrative pediatric rehabilitation (IPR) program; and (2) explore factors predicting improvement in walking ability. METHODS: Thirty-one youth with FNSD participating in an IPR program were recruited. The IPR program was activated biweekly in an ambulatory format. Study measures included the 6MWT, hemodynamic characteristics, rate of perceived exertion (RPE), pain-intensity perception, and Child's Somatization Inventory-24 (CSI-24). All measures were conducted upon admission to the IPR and discharge. RESULTS: Findings indicated a significant increase in 6MWT distance, as well as decreases in heart rate, RPE, and pain-intensity perception from admission to discharge. At admission, CSI-24 and pain intensity predicted 6MWT distance (adjusted R2 = 0.68). Pain intensity predicted discharge 6MWT distance (adjusted R2 = 0.18). Initial 6MWT predicted changes in 6MWT distance from admission to discharge (adjusted R2 = 0.33). CONCLUSION: Using an integrative rehabilitation approach increases walking distance and decreases pain-related symptoms in youth with FNSD, emphasizing the need for collaboration between physical therapists and pediatric psychologists. Moreover, changes in walking ability may increase participation and thus should be the focus of IPR.

Factors Associated With Gains in Performance During Rehabilitation After Pediatric Brain Injury: Growth Curve Analysis.

OBJECTIVE: Predicting recovery of functional performance within pediatric rehabilitation after brain injury is important for health professionals and families, but information regarding factors associated with change in functional skills ("what the child can do") and functional independence ("what the child does") is limited. The aim of the study was to examine change in functional skills and independence over time in children with moderate-severe brain injury during prolonged inpatient rehabilitation. DESIGN: This study used a retrospective cohort design. Longitudinal data from 139 children (age 1.6-20.6 yrs), hospitalized for 115.4 ± 72 days, were examined. Growth curve analysis was used to examine factors associated with change in the Pediatric Evaluation of Disability Inventory (functional skills and caregiver assistance, ie, functional independence) in mobility and self-care. Typical estimates for change per hospitalization day were obtained. RESULTS: Traumatic brain injury and older age at injury, but not sex, were associated with faster recovery. Length of stay was associated with rate of change in functional skills but not in functional independence, suggesting that improvement in functional independence during rehabilitation may be associated with other factors. CONCLUSIONS: Identifying the factors associated with individual profiles of functional improvement can provide valuable information for clinicians and decision-makers to optimize performance after prolonged inpatient rehabilitation.

Correction: Barak, S. et al. "Long-Term Outcomes of Early Enzyme Replacement Therapy for Mucopolysaccharidosis IV: Clinical Case Studies of Two Siblings". Diagnostics 2020, 10, 108.

The authors wish to make the following correction to this paper [...].

Long-Term Outcomes of Early Enzyme Replacement Therapy for Mucopolysaccharidosis IV: Clinical Case Studies of Two Siblings.

Enzyme replacement therapy (ERT) is one of the available therapies for mucopolysaccharidosis (MPS). This study presents a follow-up of two siblings with MPS IVA (Morquio A disease) that received ERT. Both siblings received weekly intravenous infusions of elosulfase alfa for 4.5 years. One sibling (patient 1, P1; male) started therapy at 54 months of age, and the other sibling (patient 2, P2; female) started at 11 months of age. ERT was well-tolerated. In comparison to P1, P2's growth curves deviated less from the norm. The orthopedic deformities of P1 were more severe than those of P2 and required several surgical corrections. P1's sleep test at 48 months revealed obstructive sleep apnea, while by the age of 102 months, parameters were normal. P2 never had sleep apnea. Only P1 demonstrated ear, nose, and throat clinical illnesses. In comparison to P1, P2's physical function was better maintained. In conclusion, ERT was safe in both patients during a 4.5-year follow-up. Although the typical characteristics of this disease were similar in both patients, P1 had a complex clinical course in comparison to P2, which influenced function and quality of life. Therefore, in order to make the most of ERT, it may be more beneficial when initiated at a relatively young age.