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Copyright of Developmental Medicine & Child Neurology is the property of Wiley-Blackwell and its content may not be copied or emailed to multiple sites or posted to a listserv without the copyright holder's express written permission. However, users may print, download, or email articles for individual use. This abstract may be abridged. No warranty is given about the accuracy of the copy. Users should refer to the original published version of the material for the full abstract. (Copyright applies to all Abstracts.)

Copyright of Developmental Medicine & Child Neurology is the property of Wiley-Blackwell and its content may not be copied or emailed to multiple sites or posted to a listserv without the copyright holder's express written permission. However, users may print, download, or email articles for individual use. This abstract may be abridged. No warranty is given about the accuracy of the copy. Users should refer to the original published version of the material for the full abstract. (Copyright applies to all Abstracts.)

Purpose. The study aimed to establish whether the manual ability classification system (MACS), a valid classification system for manual ability in children with cerebral palsy (CP), is applicable in young adults with CP and normal intelligence. Subjects. The participants ( n = 83) were young adults with CP and normal intelligence and had a mean age of 19.9 years. Method. In this study, inter observer reliability of the MACS was determined. We investigated relationships between the MACS level and patient characteristics (such as the gross motor function classification system (GMFCS) level, limb distribution of the spastic paresis and educational level) and with functional activities of the upper extremity (assessed with the Melbourne assessment, the Abilhand questionnaire and the domain self-care of the functional independence measure (FIM)). Furthermore, with a linear regression analysis it was determined whether the MACS is a significant determinant of activity limitations and participation restrictions. Results. The reliability was good (intraclass correlation coefficient 0.83). The Spearman correlation coefficients with GMFCS level, limb distribution of the spastic paresis and educational level were 0.53, 0.46, and 0.26, respectively. MACS level correlated moderately with outcome measures of functional activities (correlations ranging from −0.38 to −0.55). MACS level is, in addition to the GMFCS level, an important determinant for limitations in activities and restrictions in participation. Conclusion. We conclude that the MACS is a feasible method to classify manual ability in young adults with CP and normal intelligence with a good manual ability. [ABSTRACT FROM AUTHOR]

Purpose. To assess functional activities of the upper extremity of young adults with cerebral palsy (CP) and to determine their relations with participant characteristics and participation. Method. Assessment of functional activities of the upper extremity was performed on 103 participants (aged 16 - 20 years) with the Melbourne assessment and the Abilhand Questionnaire. Participation was measured with the Life Habits Questionnaire. Participant characteristics included age, gender, limb distribution of the spastic paresis, educational level and gross and fine motor function. Relations among these variables were studied by means of correlation coefficients and linear regression analysis. Results. Limitations in functional activities of the upper extremity were related to the limb distribution of the spastic paresis and were especially present in quadriplegic participants. Significant correlations between participant characteristics and measures of functional activities were present. Limitations in functional activities of the upper extremity, measured with the Abilhand Questionnaire, were an important determinant for participation, in addition to the gross motor function and educational level. Conclusion. Limitations in functional activities of the upper extremity are an important determinant for restrictions in participation in young adults with CP. It is recommended to include assessment of functional activities of the upper extremity in patients with CP. [ABSTRACT FROM AUTHOR]

Test-retest reliability is important for long-term follow-up; however, data on the reliability of isokinetic dynamometry of the shoulder are scarce. Twenty subjects (50% male) were measured; 10 with asymmetrical use of the arms (mean age 27 years) and 10 used their arms symmetrically (mean age 32 years). A Biodex dynamometer (Multi joint system 2) was used. Abduction/adduction and external/internal rotation were measured following a standardized protocol. Performed scheme: two sessions with a 2-week interval, all measurements were done with 60 degrees/second (5 repetitions) and respectively 120 degrees/second and 180 degrees/second (10 repetitions). Differences in the mean peak torques, split for muscle group and gender were significant. Intraclass correlation coefficients ranged from 0.69 to 0.92. This implies good to excellent reliability in research on groups. To determine test-retest reliability of two consecutive individual measurements smallest detectable differences (SDD) were computed and ranged from 21% to 43%. It is questionable whether the SDDs are small enough to detect real changes in muscle strength. [ABSTRACT FROM AUTHOR]

Surgical left atrial appendage (LAA) occlusion with an AtriClip (AtriCure, West Chester, Ohio) is frequently performed for stroke prophylaxis in patients with atrial fibrillation (AF). We conducted a retrospective analysis of all patients with long-standing persistent AF who underwent hybrid convergent ablation and LAA clipping. Contrast-enhanced cardiac computed tomography was performed at 3 to 6 months after LAA clipping to assess the degree of complete closure and the residual LAA stump. A total of 78 patients (64 ± 10 years, 72% male) underwent LAA clipping as part of hybrid convergent AF ablation, from 2019 to 2020. Median size of AtriClip used was 45 mm. Mean LA size was 4.6 ± 1 cm. At 3-to-6 months follow-up computed tomography, 46.2% of patients (n = 36) had a residual stump proximal to the deployed LAA clip. Mean depth of residual stump was 3.95 ± 5.5 mm, with 19% of patients (n = 15) having a stump depth of ≥10 mm and 1 patient requiring more endocardial LAA closure owing to large stump depth. During 1-year follow-up, 3 patients developed stroke; device leak of 6 mm was noted in 1 patient; and none of the patients had a thrombus proximal to the clip. In conclusion, high incidence of residual LAA stump was observed with AtriClip. Larger studies with long-term follow-up are needed to better assess the thromboembolic implications of a residual stump after AtriClip placement., Competing Interests: Declaration of Competing Interest Dr. Pothenini reports speaking honorarium from Boston Scientific. Dr. Singh reports research grant—American Society of Nuclear Cardiology and Pfizer; Advisory Board—Pfizer; and speaker bureau—Pfizer. Dr. Lakkireddy reports being consultant to Bionsense Webster, Boston Scientific, Abbott, AtriCure, Alta Thera, Phillips, and Acutus. Dr. Gopinathannair reports consultancy/honoraria—Abbott Medical and Sanofi; and being on the advisory board of Pacemate. The remaining authors have no conflicts of interest to declare., (Copyright © 2023 Elsevier Inc. All rights reserved.)

Objective: Ten years ago, the authors reported on the outcome of their study investigating the degree of discomfort and pain in newborns with myelomeningocele (MMC), using the parameters of unbearable and hopeless suffering. In the current study, they investigated the quality of life, daily functioning, pain and fatigue, ability to communicate, and number of surgeries in the same cohort of patients. They subdivided their study population into severe (Lorber) and less severe (non-Lorber) cases and compared these cases with a healthy population (non-MMC group) and with each other., Methods: The parents of 22 of 28 patients gave informed consent for this study. The KIDSCREEN-27 and PEDI-CAT (Pediatric Evaluation of Disability Inventory) were used to assess quality of life and daily functioning. Pain and fatigue were self-reported on a 10-point numeric rating scale. Communication and ambulation levels were determined using the Communication Function Classification System (CFCS) and the Hoffer ambulation scale. Using reference data from the KIDSCREEN-27 and PEDI-CAT, the authors created a healthy population comparison group., Results: There was no significant difference in health-related quality-of-life (HRQOL) scores between Lorber and non-Lorber patients, except that school environment domain scores were lower in the Lorber group. When comparing the HRQOL of MMC patients with that of the non-MMC group, the physical well-being and parent relations and autonomy domains scored significantly lower. The daily functioning of MMC patients was lower on all domains of the PEDI-CAT compared with the non-MMC group. Lorber MMC patients scored lower on all domains of the PEDI-CAT when compared with non-Lorber patients. All patients were capable of communicating effectively; most patients (n = 18) were considered CFCS level I, and 4 patients were considered CFCS level II., Conclusions: This study shows that MMC is a severe, lifelong condition that affects patients' lives in many domains. All the patients in this study are capable of effective communication, irrespective of severity of MMC. Overall, the data show that in newborn MMC patients, future unbearable suffering with respect to pain, mobility, cognition, and communication is hard to predict and may not always occur.

Background: The ideal treatment of inappropriate sinus tachycardia (IST) and postural orthostatic tachycardia syndrome (POTS) still needs to be defined. Medical treatments yield suboptimal results. Endocardial catheter ablation of the sinus node (SN) may risk phrenic nerve damage and open-heart surgery may be accompanied by unjustified invasive risks., Methods: We describe our first multicenter experience of 255 consecutive patients (235 females, 25.94 ± 3.84 years) having undergone a novel SN sparing hybrid thoracoscopic ablation for drug-resistant IST (n = 204, 80%) or POTS (n = 51, 20%). As previously described, the SN was identified with 3D mapping. Surgery was performed through three 5-mm ports from the right side. A minimally invasive approach with a bipolar radiofrequency clamp was used to ablate targeted areas while sparing the SN region. The targeted areas included isolation of the superior and the inferior caval veins, and a crista terminalis line was made. All lines were interconnected., Results: Normal sinus rhythm (SR) was restored in all patients at the end of the procedure. All patients discontinued medication during the follow-up. After a blanking period of 6 months, all patients presented stable SR. At a mean of 4.07 ± 1.8 years, normal SN reduction and chronotropic response to exercise were present. In the 51 patients initially diagnosed with POTS, no syncope occurred. During follow-up, pericarditis was the most common complication (121 patients: 47%), with complete resolution in all cases. Pneumothorax was observed in 5 patients (1.9%), only 3 (1.1%) required surgical drainage. Five patients (1.9%) required a dual-chamber pacemaker due to sinus arrest > 5 s., Conclusions: Preliminary results of this multicenter experience with a novel SN sparing hybrid ablation of IST/POTS, using surgical thoracoscopic video-assisted epicardial ablation combined with simultaneous endocardial 3D mapping may prove to be an efficient and safe therapeutic option in patients with symptomatic drug-resistant IST and POTS. Importantly, in our study, all patients had a complete resolution of the symptoms and restored normal SN activity., (© 2021. The Author(s).)

Purpose: Employment of young adults with chronic physical conditions entering the labor market after finishing post-secondary education remains behind compared to typically developing peers. The aim of this study is to evaluate changes in their paid employment levels after following a vocational rehabilitation intervention ('At Work')., Materials and Methods: Participants aged between 16 and 27 years ( n  = 90) were recruited via rehabilitation physicians and a jobcoach agency and participated in a vocational rehabilitation program. Cochran's Q and McNemar tests served to test the development of intervention participants' paid employment over time. Chi-square tests were used to compare intervention participants' paid employment level with national reference data selected on age and having a self-reported chronic physical condition., Results: Paid employment level of the intervention cohort significantly increased from 10.0% at baseline to 42.4% at 2-years follow-up ( p  < 0.001). At 2-years follow-up, their employment rates approached the employment rates of national reference data (42.4% versus 52.9%, p  = 0.17)., Conclusion: Starting from a disadvantaged position, the paid employment rate of the intervention cohort substantially increased over time, approaching the employment rate of reference data. 'At Work' seems to be appropriate for supporting this specific group who face obstacles to enter the labor market, to find competitive employment.IMPLICATIONS FOR REHABILITATION'At Work' is a vocational rehabilitation intervention for young adults with chronic physical conditions who experience problems with finding and maintaining competitive employment after finishing post-secondary education.The 'At Work' intervention entails a combination of group sessions and individual coaching sessions based on the supported employment methodology.Paid employment rates of the intervention cohort substantially increased on the short- and long term, and approached employment rates of reference data of persons with chronic physical conditions.The 'At Work' intervention seems appropriate to support young adults with chronic physical conditions who experience barriers for work participation, to enter the labor market and find competitive and sustainable employment.

Fatigue related to Multiple Sclerosis (MS) is considered a multidimensional symptom, manifesting in several dimensions such as physical, cognitive, and psychosocial fatigue. This study investigated in 264 patients with severe primary MS-related fatigue (median MS duration 6.8 years, mean age 48.1 years, 75% women) whether subgroups can be distinguished based on these dimensions. Subsequently, we tested whether MS-related fatigue consists of a single common unidimensional factor. Subscale scores on four self-reported fatigue questionnaires, including the Checklist of Individual Strength, the Modified Fatigue Impact Scale, the Fatigue Severity Scale and the SF36 vitality, were used in a cluster analysis to identify patients with similar fatigue characteristics. Next, all 54 items were included in exploratory factor analysis to test unidimensionality. Study results show that in patients with a treatment indication for primary MS-related fatigue, fatigue profiles are based on severity and not on the various dimensions of fatigue. The three profiles found, suggested one underlying fatigue dimension, but this could not be confirmed. Factor analysis of all 54 items resulted in 8 factors, confirming the multidimensional construct of the included fatigue questionnaires.

Background: Adults with cerebral palsy (CP) may experience problems with participation in domestic life and interpersonal relationships., Aims: To identify teenage predictors of adult participation in domestic life and interpersonal relationships., Methods and Procedures: This 13-year follow-up of the PERRIN 16-24 cohort included 53 adults with CP without intellectual disability [current age 31.7 (SD = 1.4) years]. Participation performance was assessed as attendance (Vineland Adaptive Behavior Scales), and difficulty/assistance with participation (Life Habits questionnaire). 56 teenage factors were categorized in ICF components. Stepwise multiple linear regression analyses explored predictors of participation., Outcomes and Results: Lower gross motor capacity, following special education, having protective parents and a rigid personality predicted less participation in domestic life. Having rejective parents, receiving little daily support, having a socially avoidant personality or coping style and the male gender predicted less participation in interpersonal relationships. Lower activity and participation levels as a teenager predicted less participation in both domestic life and interpersonal relationships of adults with CP., Conclusions and Implications: Environmental and personal factors, gross motor capacity and teenage participation were predictors of participation of adults with CP. These factors help identify subgroups at risk for suboptimal adult participation and provide targets for rehabilitation., (Copyright © 2019. Published by Elsevier Ltd.)

Objective: Myelomeningocele (MMC) is the most common form of spina bifida, with a lifelong impact on the quality of life for infants born with this condition. In recent decades, fetal surgery has evolved from an experimental therapy to standard of care for many centers in the world. In this study, the authors aimed to provide an overview of the current management and outcomes for infants with MMC managed at their institution. This then provides a center-specific historical cohort for comparison with future antenatal-treated MMC cases., Methods: This is a retrospective, single-institution cohort study including all consecutive MMC cases between January 1, 2000, and June 1, 2018, at Erasmus MC. Outcome data included closure of the defect (location, timing, and surgical parameters), hydrocephalus management, Chiari malformation type II (CMTII) management, incidence of spinal cord tethering and outcome, motor outcomes, and continence., Results: A total of 93 patients were included with predominantly lumbosacral lesions. Two patients died during follow-up. Hydrocephalus was present in 84%, with a 71% ventriculoperitoneal shunt reoperation rate. Surgery was performed in 12% for a tethered spinal cord at a mean age of 8 years. Decompression surgery was performed in 3 patients for CMTII. Special education in 63% was significantly associated with hydrocephalus (p < 0.015). Nineteen percent of patients were able to walk independently, and 47% were nonambulators. Social continence for urine was obtained in 75% of patients, 4% had fecal incontinence., Conclusions: This study provides an overview of current MMC outcomes at the authors' center and will serve as a historical cohort for comparison with future fetal surgery cases operated on at the center in the coming years. Apart from a relatively low surgical untethering rate, the authors' outcome data are comparable to those in the literature. Hydrocephalus is highly prevalent in postnatally treated MMC patients; in this study as in much of the literature, hydrocephalus is correlated with a low cognitive function. Fetal surgery for MMC halves the need for shunt treatment in a select group of MMC pregnancies, constituting a major indication for us to undergo the transition to a fetal surgery center. The fetal benefits of open antenatal surgery for MMC are well established, yet long-term data on especially tethered spinal cord are eagerly awaited.

Objective: We investigated whether demographic, disease-related, or personal baseline determinants can predict a positive response to energy conservation management (ECM)., Method: We conducted a secondary analysis of a single-blind, two-parallel-arms randomized controlled trial that included ambulatory adults with severe MS-related fatigue. Therapy responders and nonresponders were categorized by Checklist Individual Strength fatigue change scores between baseline and end of treatment. Logistic regression analyses were used to assess the determinants of response., Results: Sixty-nine participants were included (ECM group, n = 34; control group, n = 35). In the ECM group, fatigue severity, perception of fatigue, illness cognitions about MS, and social support discrepancies were related to the probability of being a responder., Conclusion: The results suggest that people with MS-related fatigue who had a less negative perception of fatigue and who perceived fewer disease benefits and a higher discrepancy in social support had the best response to ECM treatment., (Copyright © 2019 by the American Occupational Therapy Association, Inc.)

Background: Fatigue is a frequently reported and disabling symptom in multiple sclerosis (MS)., Objective: To investigate the effectiveness of an individual energy conservation management (ECM) intervention on fatigue and participation in persons with primary MS-related fatigue., Methods: A total of 86 severely fatigued and ambulatory adults with a definite diagnosis of MS were randomized in a single-blind, two-parallel-arm randomized clinical trial to the ECM group or the information-only control group in outpatient rehabilitation departments. Blinded assessments were carried out at baseline and at 8, 16, 26 and 52 weeks after randomization. Primary outcomes were fatigue (fatigue subscale of Checklist Individual Strength - CIS20r) and participation (Impact on Participation and Autonomy scale - IPA)., Results: Modified intention-to-treat analysis was based on 76 randomized patients (ECM, n = 36; MS nurse, n=40). No significant ECM effects were found for fatigue (overall difference CIS20r between the groups = -0.81; 95% confidence interval (CI), -3.71 to 2.11) or for four out of five IPA domains. An overall unfavourable effect was found in the ECM group for the IPA domain social relations (difference between the groups = 0.19; 95% CI, 0.03 to 0.35)., Conclusion: The individual ECM format used in this study did not reduce MS-related fatigue and restrictions in participation more than an information-only control condition.