Declining trends in birth prevalence and severity of singletons with cerebral palsy of prenatal or perinatal origin in Australia: A population-based observational study.

AIM: To investigate temporal trends in birth prevalence, disability severity, and motor type for singletons with prenatal or perinatally acquired cerebral palsy (CP). METHOD: Numerator data, number of children with CP born a singleton between 1995 and 2014, confirmed at 5 years of age, were drawn from three state registers with population-level ascertainment. Birth prevalence estimates and 95% confidence intervals (CI) were calculated per 1000 singleton live births for the three states combined, overall, by gestational age group, by dichotomized disability severity, and spastic laterality. Poisson regression models were used to analyse trends. Using data from all eight registers, trends in the proportional distribution of CP subtypes overall and stratified by gestational age were examined. RESULTS: Birth prevalence of CP declined from 1.8 (95% CI 1.6-2.0) in 1995 to 1996 to 1.2 (95% CI 1.1-1.4) in 2013 to 2014 (average 5% per 2-year epoch, p < 0.001). Declines in birth prevalence were observed across all gestational age groups with the largest decline in children born at <28 weeks (average 8% per epoch, p < 0.001). Prevalence of moderate-severe disability declined for children born at <28 and ≥37 weeks (average 11% and 7% per epoch respectively, p < 0.001). The proportions of bilateral spastic CP declined (p < 0.001) at <28 weeks (p = 0.014) and ≥37 weeks (p < 0.001). The proportion of children with dyskinesia increased (28-31 weeks: p = 0.021, 32-36 weeks: p = 0.001, and ≥37 weeks: p < 0.001). INTERPRETATION: Birth prevalence of CP and moderate-severe disability (<28 and ≥37 weeks) declined in Australian singletons between 1995 and 2014, reflecting changes in prenatal and perinatal care over time. WHAT THIS PAPER ADDS: Declines in birth prevalence of prenatal or perinatally acquired cerebral palsy were observed for singletons born in Australia between 1995 and 2014. These declines were evident across all gestational age groups. Declines in birth prevalence of moderate-severe disability were observed for children born at <28 weeks and ≥37 weeks.

Efficacy of mobilization with movement (MWM) for shoulder conditions: a systematic review and meta-analysis.

OBJECTIVE: To assess the effects of mobilization with movement (MWM) on pain, range of motion (ROM), and disability in the management of shoulder musculoskeletal disorders. METHODS: Six databases and Scopus, were searched for randomized control trials. The ROB 2.0 tool was used to determine risk-of-bias and GRADE used for quality of evidence. Meta-analyses were performed for the sub-category of frozen shoulder and shoulder pain with movement dysfunction to evaluate the effect of MWM in isolation or in addition to exercise therapy and/or electrotherapy when compared with other conservative interventions. RESULTS: Out of 25 studies, 21 were included in eight separate meta-analyses for pain, ROM, and disability in the two sub-categories. For frozen shoulder, the addition of MWM significantly improved pain (SMD -1.23, 95% CI -1.96, -0.51)), flexion ROM (MD -11.73, 95% CI -17.83, -5.64), abduction ROM (mean difference -13.14, 95% CI -19.42, -6.87), and disability (SMD -1.50, 95% CI (-2.30, -0.7). For shoulder pain with movement dysfunction, the addition of MWM significantly improved pain (SMD -1.07, 95% CI -1.87, -0.26), flexion ROM (mean difference -18.48, 95% CI- 32.43, -4.54), abduction ROM (MD -32.46, 95% CI - 69.76, 4.84), and disability (SMD -0.88, 95% CI -2.18, 0.43). The majority of studies were found to have a high risk of bias. DISCUSSION: MWM is associated with improved pain, mobility, and function in patients with a range of shoulder musculoskeletal disorders and the effects clinically meaningful. However, these findings need to be interpreted with caution due to the high levels of heterogeneity and risk of bias. LEVEL OF EVIDENCE: Treatment, level 1a.

Comorbidities and quality of life in children with intellectual disability.

BACKGROUND: Many children with intellectual disability live with medical comorbidities. This study examined the impacts of comorbidities on quality of life (QOL) of children with intellectual disabilities and whether impacts varied with caregiver perceptions that medical needs had been met. METHODS: Primary caregivers of 447 children (aged 5-19 years) with an intellectual disability reported on their child's medical comorbidities and the extent to which they perceived their child's medical needs had been met in a cross-sectional observational study. The Quality of Life Inventory-Disability was used to measure QOL on a 100-point scale. Linear regression models including interaction terms were used to evaluate their associations. RESULTS: Parent-reported recurrent child pain (-4.97, 95% CI -8.21, -1.72), night-time sleep disturbances (-4.98, 95% CI -7.23, -2.73), daytime somnolence (-8.71, 95% CI -11.30, -2.73), seizures that occurred at least weekly (-7.59, 95% CI -13.50, -1.68) and conservatively managed severe scoliosis (-7.39, 95% CI -12.97, -1.81) were negatively associated with child QOL. Despite the majority of parents (~70%) perceiving that their child's medical needs had been met to a great extent, this did not significantly moderate the association between any comorbidities and QOL. CONCLUSIONS: Comorbidities were common and had marked associations with QOL. Evaluation and management of pain and sleep disturbance continue to be high priorities in improving QOL of young people with intellectual disabilities. Further research on the optimal methods of managing these comorbidities is warranted.

Functioning, participation, and quality of life in children with intellectual disability: an observational study.

AIMS: To investigate associations between functioning, community participation, and quality of life (QoL) and identify whether participation mediates the effects of functioning on QoL. METHOD: The caregivers of 435 children (211 females, 224 males; mean age 12y; SD 3y 11mo; age range 5-18y) with intellectual disability and autism spectrum disorder, cerebral palsy, Down syndrome, or Rett syndrome reported on their child's functioning (dependence for managing personal needs, mobility, communication, eye contact when speaking), frequency of participation, and QoL. Linear regression and mediation analyses were used to evaluate the relationships between child functioning, participation, and QoL. RESULTS: Children with greater dependency for managing personal needs and limited eye contact when speaking experienced poorer QoL. Less impaired functioning was associated with more frequent participation, which, in turn, was associated with a 3-point gain in QoL for each additional point in frequency of participation (coefficient=2.67, 95% confidence interval 1.56-3.78). The effect of impaired functioning on QoL was partially mediated by participation in children with greater dependency in managing personal needs and those with mildly impaired communication. INTERPRETATION: Greater levels of impairments with poorer functioning, notably a high level of dependence, were associated with poorer QoL. Poorer QoL can be partly explained by less frequent community participation.

Impact of social disadvantage on cerebral palsy severity.

AIM: To investigate the impact of socio-economic disadvantage on indicators of cerebral palsy (CP) severity - motor impairment, intellectual disability, and the presence of severe comorbidities - in children with CP in Australia. METHOD: Data from the Australian Cerebral Palsy Register were analysed. Socio-economic disadvantage was assessed using maternal age, maternal country of birth, and a measure of neighbourhood socio-economic status (SES) at the time of the child's birth. Descriptive bivariate analysis, trend analysis, risk ratios, and mediation analysis were undertaken to examine the impact of disadvantage on the indicators of CP severity. RESULTS: A socio-economic gradient was demonstrated with an increasing proportion of children with non-ambulant status, at least moderate intellectual disability, and the presence of severe comorbidities (having epilepsy, functional blindness, bilateral deafness, and/or no verbal communication) with decreasing neighbourhood SES, adolescent motherhood, and maternal minority ethnicity. INTERPRETATION: In Australia, socio-economic disadvantage at birth impacts adversely on CP severity at age 5 years. By identifying that socio-economically disadvantaged children with CP are at greater risk of more severe functional outcomes, we can inform targeted interventions at the family and neighbourhood level to reduce these inequities for children with CP. WHAT THIS PAPER ADDS: Socio-economic disadvantage is associated with increased severity of cerebral palsy functional outcomes. This encompasses low neighbourhood socio-economic status, adolescent motherhood, and maternal minority ethnicity.

IMPACTO DE LA DESVENTAJA SOCIAL SOBRE LA SEVERIDAD DE LA PARÁLISIS CEREBRAL: OBJETIVO: Investigar el impacto de la desventaja socioeconómica en los indicadores de severidad de la parálisis cerebral (PC), definida como - deterioro motor, discapacidad intelectual y la presencia de severidad de las comorbilidades: en niños con PC en Australia. MÉTODO: Se analizaron los datos del Australian Cerebral Palsy Register. La desventaja socioeconómica se evaluó utilizando la edad materna, el país de nacimiento de la madre, y una medida de estado socioeconómico del vecindario (SES) en el momento del nacimiento del niño. Se realizaron análisis bivariados, análisis de tendencias, índices de riesgo y análisis de mediación para examinar el impacto de la desventaja en los indicadores de severidad PC. RESULTADOS: Se demostró un gradiente socioeconómico con una proporción creciente de niños con estado no ambulante, al menos discapacidad intelectual moderada, y la presencia de comorbilidades graves (con epilepsia, ceguera funcional, sordera bilateral y / o sin comunicación verbal) con SES vecinales, maternidad adolescente, y etnia de la minoría materna. INTERPRETACIÓN: En Australia, la desventaja socioeconómica al nacer tiene un impacto adverso en la severidad de PC a la edad de 5 años. Al identificar a los niños con desventajas socioeconómicas con PC tienen un mayor riesgo de resultados funcionales más severos. Con esta información podemos guiar intervenciones a nivel familiar y de vecindario para reducir estas inequidades en los niños con PC.

IMPACTO DA DESVANTAGEM SOCIAL NA SEVERIDADE DA PARALISIA CEREBRAL: OBJETIVO: Investigar o impacto da desvantagem sócio-econômica nos indicadores de severidade da paralisia (PC) - comprometimento motor, deficiência intelectual, e a presença de comorbidades graves - em crianças com PC na Austrália. MÉTODO: Dados do Registro Austrliano de Paralisia Cerebral foram analisados. Desvantagens sócio-econômicas foram avaliadas usando-se a idade materna, o país de Nascimento da mãe, e uma medida do estado sócio-econômico (ESE) da vizinhança no momento do nascimento da criança. Análise bivariada descritiva, análise de tendências, taxas de risco, e análise de mediação foram realizados para examinar o impacto da desvantagem nos indicadores da severidade da PC. RESULTADOS: Um gradiente sócio-econômico foi demonstrado com um aumento da proporção de crianças com situação não ambilante, deficiência intelectual pelo menos moderada, e presença de comorbidades severas (epilepsia, cegueira funcional, surdez bilateral, e/ou nenhuma comunicação verbal) quanto menor o ESE da vizinhança, a minoria étnica materna e maternidade na adolescência. INTERPRETAÇÃO: Na Austrália, a desvantagem sócio-econômica ao nascimento impacta adversamente a severidade da PC na idade de 5 anos. Ao identificar que crianças com PC em desvantagem sócio-econômica têm maior risco de resultados funcionais mais severos, podemos informar intervenções específicas voltadas para a família e para a vizinhança para reduzir o nível destas inequidades para crianças com PC.

Reliable classification of functional profiles and movement disorders of children with cerebral palsy.

OBJECTIVE: To examine the inter-rater reliability of the Communication Function Classification System (CFCS), Bimanual Fine Motor Function (BFMF), Surveillance of Cerebral Palsy in Europe (SCPE) classification tree, and Gross Motor Function Classification System (GMFCS) in children with cerebral palsy (CP) and periventricular white matter injury (PWMI) aged 4-11 years. METHOD: Twenty children were assessed by two raters using the four tools, in addition parents undertook ratings on the Manual Ability Classification System (MACS). Kappa statistics were used to calculate the level of agreement between raters' classifications. RESULTS: Participants comprised 12 males and 8 females with CP and PWMI, mean age 8 years 1 month (standard deviation 2 years 3 months). Inter-rater reliability across the four tools was 0.98 (CFCS, BFMF, and GMFCS) and 0.84 (SCPE). IMPLICATIONS: These findings suggest that these four tools are reasonably robust to inter-rater variability supporting their routine use along with the MACS in clinical and research applications.

Factor V Leiden mutation: a contributory factor for cerebral palsy?

Fifty-seven children with cerebral palsy (CP) and imaging evidence of vascular thrombosis (study group) and 167 children with CP and other imaging finds (control group)were selected. Sixty-one per cent of the study group were male and 53 (93%) had spastic hemiplegia compared with the control group, of whom 55% were male and 54 (32%) had a diagnosis of spastic hemiplegia. Mean age was 5 years 11 months (SD 5y 1mo) for the study group and 7 years 7 months (SD 4y 7mo) for the control group. Blood spots on Guthrie cards or buccal swabs were used to test both groups and their mothers for the factor V Leiden (fVL) mutation, which predisposes carriers to thrombophilia. Mothers were interviewed to gather antenatal, perinatal, demographic, and socio-economic data. The frequency of the fVL mutation in children with evidence of vascular thrombosis and their mothers was not statistically different from the frequency in children with CP with other imaging findings and their mothers. The frequency of the fVL mutation was significantly higher than the expected population frequency of 4% in the study group (10.5%, p=0.012) and in mothers of the control group (7.2%, p=0.036).

Cerebral palsy in Victoria: motor types, topography and gross motor function.

OBJECTIVES: To study the relationships between motor type, topographical distribution and gross motor function in a large, population-based cohort of children with cerebral palsy (CP), from the State of Victoria, and compare this cohort to similar cohorts from other countries. METHODS: An inception cohort was generated from the Victorian Cerebral Palsy Register (VCPR) for the birth years 1990-1992. Demographic information, motor types and topographical distribution were obtained from the register and supplemented by grading gross motor function according to the Gross Motor Function Classification System (GMFCS). RESULTS: Complete data were obtained on 323 (86%) of 374 children in the cohort. Gross motor function varied from GMFCS level I (35%) to GMFCS level V (18%) and was similar in distribution to a contemporaneous Swedish cohort. There was a fairly even distribution across the topographical distributions of hemiplegia (35%), diplegia (28%) and quadriplegia (37%) with a large majority of young people having the spastic motor type (86%). CONCLUSIONS: The VCPR is ideal for population-based studies of gross motor function in children with CP. Gross motor function is similar in populations of children with CP in developed countries but the comparison of motor types and topographical distribution is difficult because of lack of consensus with classification systems. Use of the GMFCS provides a valid and reproducible method for clinicians to describe gross motor function in children with CP using a universal language.

Inpatient care of children with cerebral palsy as perceived by their parents.

OBJECTIVES: Children with cerebral palsy (CP) have special health care needs. The aim of the study was to evaluate the inpatient care of children with CP, as perceived by their parents. METHODS: Forty parents of children with CP and 90 parents of able-bodied children completed a questionnaire designed to evaluate their perception of their child's hospital admission, including the care provided. The parents also completed the Perceived Stress Scale. RESULTS: Overall, parents of able-bodied children were more satisfied with the hospitalization than parents of children with CP (P < 0.0001). Significant differences were found in four of the five areas assessed: the admissions process (P = 0.0002); the care that their child received (P < 0.0001); their communication and confidence in doctors and nurses (P < 0.0001); and their personal experience of the hospital (P = 0.0076). Parents of disabled children displayed a much higher mean score on the Perceived Stress Scale, but no correlation was found between this scale and the satisfaction questionnaire for either group. CONCLUSION: The results of this study show that parents of children with CP were, as a group, less satisfied than parents of able-bodied children with their inpatient experience. Although these children have complex needs and often require more care than able-bodied children, it is imperative that their needs are met and that parents are satisfied with all aspects of the care they receive in hospital.

Addition of inhaled corticosteroids to systemic immunosuppression after lung transplantation: a double-blind, placebo-controlled trial.

BACKGROUND: It is postulated that bronchiolitis obliterans syndrome (BOS) is preceded by airway inflammation that has been described even in stable lung transplant recipients. Airway inflammation is known to be suppressed by inhaled steroids in other chronic inflammatory lung diseases, e.g., asthma and chronic obstructive pulmonary disease. BOS is the major cause of morbidity and mortality after lung transplantation. OBJECTIVE: To examine the effect of inhaled corticosteroids on the development of BOS in lung transplant recipients. METHODS: Thirty patients were recruited and randomized in a double-blind fashion to receive either 750 microg of fluticasone propionate (FP) or an identical-appearing placebo twice daily for 3 months; 20 of this group continued until 2 years posttransplantation. Detailed spirometry was performed regularly throughout the study. RESULTS: In the short-term study no differences were found in any examined parameters. In the long-term component of the study no differences were found in the development of neither BOS nor survival. There were minor differences in bronchoalveolar lavage (BAL) lymphocyte percentages. CONCLUSIONS: FP is ineffective for the prevention of BOS after lung transplantation despite the airway inflammation that characterizes this condition. Inadequate local delivery, timing of the therapy relative to transplantation and inherent steroid resistance of this condition may explain the negative finding of this study.