Feasibility and Effectiveness of an Individualized 12-Week "Uptime" Participation (U-PART) Intervention in Girls and Women With Rett Syndrome.

Background: Girls and women with Rett Syndrome (RTT) have low levels of daily physical activity and high levels of sedentary time. Reducing sedentary time and enhancing "uptime" activities, such as standing and walking, could be an important focus for interventions to address long-term health and quality of life in RTT.

Objective: The aim of the study was to evaluate the feasibility and health-related effects of an individualized 12-week uptime participation (U-PART) intervention in girls and women with RTT.

Facilitators and Barriers of Participation in "Uptime" Activities in Girls and Women With Rett Syndrome: Perspectives From Parents and Professionals.

Rett syndrome (RTT) is a rare neurodevelopmental disorder usually affecting females. It is associated with intellectual and multiple disabilities leading to a high level of dependency in all aspects of daily living including participation in physical activities. This study explored facilitators and barriers to "uptime" (non-sedentary) activities in Danish girls and women with RTT as perceived by parents and professionals using focus groups.

Functional profiles of children with cerebral palsy in Jordan based on the association between gross motor function and manual ability.

Background: Cerebral palsy (CP) is the most common cause of physical disability in childhood. A major challenge for delivering effective services for children with CP is the heterogeneity of the medical condition. Categorizing children into homogeneous groups based on functional profiles is expected to improve service planning.

Participation in physical activities for children with cerebral palsy: feasibility and effectiveness of physical activity on prescription.

Background: Children with cerebral palsy (CP) are less physically active and more sedentary than other children which implies risk factors for their physical and mental health. Physical activity on prescription (PAP) is an effective intervention to promote a lifestyle change towards increased physical activity in adults in general. Knowledge is lacking about the use of PAP in children with CP.

Development of a Cerebral Palsy Follow-up Registry in Jordan (CPUP-Jordan).

Aims: This study aims to describe the development of a Cerebral Palsy Follow-up Registry in Jordan (CPUP-Jordan) and to provide a baseline child and parent demographic information, birth history of the child participants, and distribution of the participants based on topographical distribution of cerebral palsy (CP) and functional classification systems.

Methods: The CPUP-Jordan was developed using a similar framework of a follow-up surveillance programme for persons with CP in Sweden (CPUP). Standard assessment forms were utilized to collect data related to child and family demographics, child birth history, and functional classifications and physiotherapy and occupational therapy assessments and interventions.

Assessments of pain in children and adolescents with cerebral palsy: a retrospective population-based registry study.

Aim: To explore pain screening in CPUP, a follow-up surveillance programme for people with cerebral palsy (CP), specifically to describe reported pain prevalence, localizations, patterns of distribution; to compare with studies using psychometrically sound assessment instruments; and to assess agreement between pain documented in CPUP and medical records.

Method: Registry study of a population with CP, born 1993 to 2008, living in Skåne, Sweden in 2013. Descriptive data, cross-tabulations, and chi-square tests to characterize and compare the study groups.

Parents' experiences of participation in physical activities for children with cerebral palsy - protecting and pushing towards independence.

Purpose: To explore how parents of children with cerebral palsy (CP) experience their child's participation in physical activities and to identify facilitators and barriers for being physically active and reducing sedentary behaviour.

Methods: Twenty-five parents of sixteen children, aged 8-11 years old with CP, with varying gross motor, cognitive and communicative functions and with different cultural backgrounds, participated in focus group or individual interviews. Content analysis was used for analysis.

Stability of the Gross Motor Function Classification System in children and adolescents with cerebral palsy: a retrospective cohort registry study.

Aim: To investigate the stability and to determine factors that affect change in the Gross Motor Function Classification System (GMFCS) in a sample from the total population with cerebral palsy (CP) in two regions of Sweden.

Method: Retrospective cohort registry study based on the follow-up programme for CP. Children with CP and a minimum of two GMFCS ratings were included.

Building the repertoire of measures of walking in Rett syndrome.

Background: The repertoire of measures of walking in Rett syndrome is limited. This study aimed to determine measurement properties of a modified two-minute walk test (2MWT) and a modified Rett syndrome-specific functional mobility scale (FMS-RS) in Rett syndrome.

Methods: Forty-two girls and women with Rett syndrome (median 18.

A combined surveillance program and quality register improves management of childhood disability.

Purpose: To describe a concept for prevention of secondary conditions in individuals with chronic neuromuscular disabilities by using two Swedish developed follow-up-programmes for cerebral palsy (CP; CPUP) and myelomeningocele (MMC; MMCUP) respectively as examples.

Method: This paper describes and outlines the rationale, development and implementation of CPUP and MMCUP.

Results: Both programmes are multidisciplinary longitudinal follow-up programmes that simultaneously serve as national registries.

Parents' Experiences of Health and Needs When Supporting Their Adolescents With Cerebral Palsy During Transition to Adulthood.

Aims: Parents are the primary support providers for adolescents with disabilities, their health and wellbeing is therefore of great importance when planning for youths' transition into adulthood. The aim of this study was to gain a deeper understanding of how parents of adolescents with cerebral palsy (CP) experience their own health and wellbeing and their needs for support during the adolescent's transition to adulthood.

Methods: An inductive qualitative approach was used, including interviews with 15 mothers and fathers to 10 adolescents with CP aged 17-18 years.

Functional performance in self-care and mobility after selective dorsal rhizotomy: a 10-year practice-based follow-up study.

Aim: To explore changes in performance in daily activities (self-care and mobility) 10 years after selective dorsal rhizotomy (SDR).

Method: Twenty-four children with bilateral spastic cerebral palsy were followed; the median age at SDR was 4 years 1 month (range 2y 5mo-6y 4mo) and at 10-year follow-up was 14 years 6 months (range 12y 3mo-16y 9mo). The preoperative Gross Motor Function Classification System (GMFCS) levels were: I (n=1), II (n=7), III (n=4), IV (n=11), and V (n=1).

Living in transition - experiences of health and well-being and the needs of adolescents with cerebral palsy.

Background: Transition to adulthood for adolescents in general is a multifaceted process, and for adolescents with cerebral palsy (CP) it also involves transition from child- to adult-oriented support. CP entails a variety and combination of disabilities, which in association with external factors may make the transition to adult health services difficult. The aim of this study was to gain a deeper understanding of how adolescents with CP experience their own health, well-being and need of support during their transition to adulthood.

"It's fun, but …" Children with cerebral palsy and their experiences of participation in physical activities.

Purpose: To explore the experiences of children with cerebral palsy (CP) regarding participation in physical activities, and to describe facilitators and barriers.

Methods: Sixteen children with CP 8-11 years old who varied in gross motor, cognitive and communicative function participated in either an individual interview or a focus group.

Results: Two categories and 10 sub-categories emerged from the content analysis.

Fractures in children with cerebral palsy: a total population study.

Aim: To analyse factors associated with fractures in children with cerebral palsy (CP) in different levels of Gross Motor Function Classification System (GMFCS).

Method: This was an epidemiological retrospective study of a total population of 536 children (214 females, 322 males) with CP born between 1990 and 2005. CP type was unilateral spastic (n=159), bilateral spastic (n=225), ataxic (n=60), dyskinetic (n=80), and mixed type (n=12); 384 children were in Gross Motor Function Classification Scale (GMFCS) levels I-III and 152 children were in GMFCS levels IV-V.

Physical activity in a total population of children and adolescents with cerebral palsy.

The aims of this study were to describe the participation in physical activity of children with cerebral palsy (CP) at school and during leisure time and to identify characteristics associated with physical activity. The frequency of receiving physiotherapeutic interventions were described as a variable of interest. A total population of 364 children with verified CP aged 7-17 years living in the Skåne region in Sweden was studied using cross-sectional data from the CP follow-up programme (CPUP).

Motor function after selective dorsal rhizotomy: a 10-year practice-based follow-up study.

Aim: The aim of this study was to explore changes in motor function up to 10 years after selective dorsal rhizotomy (SDR).

Method: The participants comprised 29 children (20 males, nine females) with bilateral spastic diplegia who were consecutively operated on at a median age of 4 years and 3 months and followed until a median age of 15 years. SDR was combined with physiotherapy and regular follow-up visits.

Survival at 19 years of age in a total population of children and young people with cerebral palsy.

Aim: The aims were to investigate survival of children with cerebral palsy (CP) and to search for modifiable factors that influence survival in CP.

Method: The total population of children with CP in southern Sweden born between 1990 and 2005, and followed from 1994 to 2010 comprised 718 children. The study included 708 of these children (297 females, 411 males) participating in a secondary prevention programme.

Growth in children with cerebral palsy during five years after selective dorsal rhizotomy: a practice-based study.

Background: Overweight is reported as a side effect of SDR. The aims were to study the development of weight, height and body mass index (BMI) during five years after SDR.

Methods: This prospective, longitudinal and practice-based study included all 56 children with CP spastic diplegia undergoing SDR from the start in March 1993 to April 2003 in our hospital.

Development of lower limb range of motion from early childhood to adolescence in cerebral palsy: a population-based study.

Background: The decreasing range of joint motion caused by insufficient muscle length is a common problem in children with cerebral palsy (CP), often worsening with age. In 1994 a CP register and health care programme for children with CP was initiated in southern Sweden. The aim of this study was to analyse the development of the passive range of motion (ROM) in the lower limbs during all the growth periods in relation to gross motor function and CP subtype in the total population of children with CP.

Longitudinal construct validity of the GMFM-88 total score and goal total score and the GMFM-66 score in a 5-year follow-up study.

Background: The Gross Motor Function Measure (GMFM) is the instrument most commonly used to measure gross motor function in children with cerebral palsy (CP). Different scoring options have been developed, and their measurement properties have been assessed. Limited information is available regarding longitudinal construct validity.

Long-term outcomes five years after selective dorsal rhizotomy.

Background: Selective dorsal rhizotomy (SDR) is a well accepted neurosurgical procedure performed for the relief of spasticity interfering with motor function in children with spastic cerebral palsy (CP). The goal is to improve function, but long-term outcome studies are rare. The aims of this study were to evaluate long-term functional outcomes, safety and side effects during five postoperative years in all children with diplegia undergoing SDR combined with physiotherapy.

Cerebral palsy in a total population of 4-11 year olds in southern Sweden. Prevalence and distribution according to different CP classification systems.

Background: The aim of this study was to investigate the prevalence of cerebral palsy (CP) as well as to characterize the CP population, its participation in a secondary prevention programme (CPUP) and to validate the CPUP database.

Methods: The study population was born 1990-1997 and resident in Skåne/Blekinge on Jan 1st 2002. Multiple sources were used.

Interaction of a Salmonella enteritidis O-antigen octasaccharide with the phage P22 tailspike protein by NMR spectroscopy and docking studies.

The tailspike protein P22 recognizes an octasaccharide derived from the O-antigen polysaccharide of Salmonella enteritidis in a shallow groove and molecular docking successfully identifies this binding region on the protein surface. Analysis by 2D (1)H,(1)H-T-ROESY and transferred NOESY NMR experiments indicate that the bound octasaccharide ligand has a conformation similar to that observed in solution. The results from a saturation transfer difference NMR experiment show that a large number of protons in the octasaccharide are in close contact with the protein as a result of binding.