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Aim Method Results Interpretation To determine the proportion of persons with cerebral palsy (CP) with major congenital anomalies, factors associated with the presence of anomalies, body systems involved, potential contribution to CP aetiology, and causal pathway subgroups implicated.This population‐based, observational study involved a cohort of 2238 persons born in one Australian state between 1999 and 2017. Major congenital anomalies were classified as affecting cerebral, cardiac, or other body systems, with further categorization as single or multisystem. We determined the potential for anomalies to contribute to the development of CP across causal pathway subgroups that were broadly categorized as developmental or involving destructive brain insults.Of persons with CP, 23% had major congenital anomalies and 17% of the cohort had anomalies that potentially contributed to the development of CP. Consistent with higher odds of parental consanguinity, maternal grand multiparity, and dysmorphic features in the group with anomalies, 82% of pathogenic anomalies, present in 14% of the cohort, were cerebral and involved developmental causal pathways. Only 3% (predominantly severe cardiac anomalies) were related to destructive brain insults.The study provides context for the impact on rates of CP of preventive measures or other changes in incidence or management of congenital anomalies. [ABSTRACT FROM AUTHOR]

Aim: To describe the distribution of neuroimaging patterns in a term/late preterm population‐based cohort with cerebral palsy (CP), ascertain associations between neuroimaging patterns and neonatal well‐being, estimate the proportion with antenatal or perinatal timing of neuropathology, and apply this information to the understanding of common mechanisms of brain injury and causal pathways. Method: The cohort for this observational study comprised 1348 persons born between 1999 and 2017 in Victoria, Australia. Using algorithms designed for the study, neonatal well‐being and timing of brain injury were tabulated for the whole cohort and across neuroimaging patterns and birth epochs. Results: Clinical and demographic profiles, neonatal well‐being, and timing of brain injury differed across neuroimaging patterns. An estimated 57% of the cohort had a complicated neonatal period. Timing of brain injury was antenatal in 57% and perinatal in 41%. A decrease in the relative proportions of perinatal timing of brain injury was observed over a period when the rates of CP in live births at term decreased. Interpretation: This study begins to bridge the knowledge gap about causation in CP, moving towards better description of the main mechanisms of brain injury and their contribution within CP cohorts, and facilitating the ability to monitor changes over time and the success of preventive measures. What this paper adds: In a population‐based, term/late preterm cohort with cerebral palsy, 57% had a complicated neonatal period.In the same cohort, 57% had presumed antenatal timing of brain injury.The relative proportion with perinatal injury decreased over time. What this paper adds: In a population‐based, term/late preterm cohort with cerebral palsy, 57% had a complicated neonatal period.In the same cohort, 57% had presumed antenatal timing of brain injury.The relative proportion with perinatal injury decreased over time. This original article is commented on by O'Shea on pages 829–830 of this issue. [ABSTRACT FROM AUTHOR]

Aim: To better understand parents' beliefs about causation in cerebral palsy (CP) and the emotions related to those beliefs. Method: We surveyed 226 parents of children with CP aged 1 to 18 years, recruited from the Victorian Cerebral Palsy Register, to evaluate their beliefs about the causes of CP, including genetic causes, causes specific to their own child, and their attitudes and emotions in relation to these. Results: Although 92% of participants reported that understanding the causes of their child's CP was important, uncertainty about the cause was expressed by 13%. The most frequently endorsed causal factors, in general and in their own child respectively, were intrapartum hypoxia (81%, 36%) or brain damage (69%, 22%), brain damage during pregnancy (73%, 28%), and preterm birth (66%, 28%). Genetic causes were deemed relevant by 13% of participants and hospital or professional error by 16%. Parents shared related feelings of anger (59%), sadness (80%), guilt (61%), and confusion (53%); parental anger was more likely when their child's CP was attributed to intrapartum events. Interpretation: Substantial parental interest in understanding the causes of CP, together with uncertainty about the causes, parents' causal attributions, and significant emotional sequelae, highlight a strong need for provision of information and support for families of children recently diagnosed with CP. What this paper adds: Understanding the causes of their child's cerebral palsy (CP) was important to parents.Parents most often endorsed intrapartum factors as a cause of CP.Parents reported experiencing strong emotions about the causes of their child's CP. What this paper adds: Understanding the causes of their child's cerebral palsy (CP) was important to parents.Parents most often endorsed intrapartum factors as a cause of CP.Parents reported experiencing strong emotions about the causes of their child's CP. This original article is commented on by Vasyutin on page 148 of this issue. [ABSTRACT FROM AUTHOR]

Aim: To provide an updated description of the rates, trends, and predictors of mortality of individuals with cerebral palsy (CP), born in the Australian state of Victoria between 1970 and 2012. Method: Data were extracted for 4807 individuals (2091 females; 2716 males). The probability of survival to 30th June 2017 was calculated using the Kaplan–Meier method. Mortality rates were calculated per 1000 person‐years using age strata and compared with population mortality rates to produce mortality ratios. Cox proportional hazards regression was used to calculate hazard ratios for selected demographic and clinical characteristics and to estimate the effect of birth epoch on 15‐year survival. Results: There were 666 recorded deaths. Compared to the general population, mortality was higher for all persons with CP and highest for children aged 1 to 15 years (45–62 times). We observed 35% improvement in the probability of survival to 15 years for births in the 2000s relative to the 1970s (hazard ratio 0.65, 95% confidence interval [CI] 0.49, 0.86), but only 4% improvement for the subgroup with complex CP (hazard ratio 0.96, 95% CI 0.69, 1.33). Interpretation: The observed improvements in survival for those born in the 2000s is likely related predominantly to a proportional reduction in complex CP within the cohort. What this paper adds: Length of survival improved for Australians with cerebral palsy (CP) born this millennium.Improved survival was mainly because of a proportional reduction in complex CP.A small improvement in length of survival was seen for children with complex CP. [ABSTRACT FROM AUTHOR]

Aim: To describe post‐neonatally acquired (PNN) cerebral palsy (CP) in terms of temporal trends in prevalence, clinical and sociodemographic profiles, known causes and associations between causes, and sociodemographic variables. Method: Numerator data, a count of children with PNN‐CP confirmed at 5 years of age (n = 523), was drawn from two Australian state CP registers (birth years 1973–2012). Poisson regression was used to investigate temporal trends in the prevalence of PNN‐CP by 5‐year intervals, calculated per 10 000 live births. Using data from all state and territory Australian CP registers (n = 469), distributions of clinical characteristics, PNN‐CP causes, and sociodemographic factors were tabulated (birth years 1995–2012). χ2 and logistic regression analyses were used to assess associations between sociodemographic profile, Australian reference data, and known causes. Results: A significant temporal decline in PNN‐CP in Victoria (p = 0.047) and Western Australia (p = 0.033) was observed. The most common proximal causes of PNN‐CP were cerebrovascular accidents (34%, n = 158), infection (25%, n = 117), and non‐accidental injuries (12%, n = 58). Children born to teenage mothers, Aboriginal and/or Torres Strait Islander mothers, or children born in remote areas were over‐represented in this cohort compared with reference data (all p ≤ 0.001). Infectious causes were strongly associated with teenage motherhood (odds ratio 3.0 [95% confidence interval 1.1–8.2], p = 0.028) and remote living (odds ratio 4.5 [95% confidence interval 2.0–10.2], p < 0.001). Interpretation: Although prevalence of PNN‐CP has declined, the over‐representation of priority populations, and the relative severity of a condition that is largely preventable, suggest the need for more specific primary preventive measures and support. What this paper adds: Prevalence of post‐neonatally acquired (PNN) cerebral palsy (CP) in Australia significantly declined between 1973 and 2012.Cerebrovascular accidents are the most common proximal cause of PNN‐CP.Children born in remote areas are at greater risk of PNN‐CP. [ABSTRACT FROM AUTHOR]

Aim: This 10-year follow-up study examined cognitive change in a cohort of children with cerebral palsy from preschool to adolescence at the group and individual levels.Methods: The Wechsler Preschool and Primary Scale of Intelligence was administered to 80 children with cerebral palsy (mean = 4 years 6 months, standard deviation = 7 months) at baseline (Time 1). At 10-year follow-up (Time 2), 28 adolescents (mean = 14 years 6 months, standard deviation = 9 months) returned for assessment with the Wechsler Intelligence Scale for Children. Motor-free intelligence quotient (IQ) scores were calculated and paired-samples t-tests and the Reliable Change Index (RCI) were used to investigate change in IQ over time.Results: At the group level, nonverbal IQ scores declined significantly. At the individual level, RCI indicated nine and 11 children showed a clinically significant decline in Full Scale IQ (FSIQ) and nonverbal IQ scores, respectively. Decline in FSIQ was related to a history of seizures whereas decline in nonverbal IQ was associated with higher initial IQ.Conclusion: Cognitive abilities in children with cerebral palsy evolve over time and selective deficits may not be observable until a later age, highlighting the importance of repeated cognitive assessment throughout childhood and adolescence. [ABSTRACT FROM AUTHOR]

Aims: To investigate whether second trimester maternal serum screening (2TMSS) biomarkers are associated with cerebral palsy (CP) and identify CP characteristics associated with abnormal biomarker levels.Method: In this retrospective case-control data linkage study, we linked mothers of 129 singleton CP cases from a population register to their 2TMSS records and selected 10 singleton pregnancy controls per case (n = 1290). We compared mean and abnormal levels of alpha-fetoprotein (AFP), beta subunit of human chorionic gonadotrophin (β-hCG), unconjugated estriol (uE3), and inhibin between cases and controls and within CP subgroups.Results: Compared to control pregnancies, CP pregnancies had higher mean levels of AFP (1.10 vs. 1.01 multiple of the population median [MoM], p = 0.01) and inhibin (1.10 vs. 0.98 MoM, p ≤ 0.01). CP pregnancies were 2.5 times more likely to be associated with high levels of AFP (OR 2.52 [95% confidence interval [CI] 1.30, 4.65]; p < 0.01) and 2.6 times for inhibin (OR 2.63 [95% CI 1.37, 4.77]; p < 0.01), and 6.8 times when AFP and inhibin were both elevated (OR 6.75 [95% CI 2.41, 18.94]; p < 0.01). In CP cases, high AFP and high inhibin levels were associated with preterm birth and low birthweight.Interpretation: Abnormal second-trimester biomarker levels suggest abnormal placentation plays a role in the causal pathway of some CP cases. [ABSTRACT FROM AUTHOR]

Aim: To describe the major congenital anomalies present in children with postneonatally acquired cerebral palsy (CP), and to compare clinical outcomes and cause of postneonatally acquired CP between children with and without anomalies. Method: Data were linked between total population CP and congenital anomaly registers in five European and three Australian regions for children born 1991 to 2009 (n=468 children with postneonatally acquired CP; 255 males, 213 females). Data were pooled and children classified into mutually exclusive categories based on type of congenital anomaly. The proportion of children with congenital anomalies was calculated. Clinical outcomes and cause of postneonatally acquired CP were compared between children with and without anomalies. Results: Major congenital anomalies were reported in 25.6% (95% confidence interval [CI] 21.7–29.9) of children with postneonatally acquired CP. Cardiac anomalies, often severe, were common and present in 14.5% of children with postneonatally acquired CP. Clinical outcomes were not more severe in children with congenital anomalies than those without anomalies. Cause of postneonatally acquired CP differed with the presence of congenital anomalies, with cerebrovascular accidents predominating in the anomaly group. Congenital anomalies were likely associated with cause of postneonatally acquired CP in 77% of children with anomalies. Interpretation: In this large, international study of children with postneonatally acquired CP, congenital anomalies (particularly cardiac anomalies) were common. Future research should determine specific causal pathways to postneonatally acquired CP that include congenital anomalies to identify opportunities for prevention. What this paper adds: One‐quarter of children with postneonatally acquired cerebral palsy (CP) have a major congenital anomaly.Cardiac anomalies, often severe, are the most common anomalies.Causes of postneonatally acquired CP differ between children with and without congenital anomalies. What this paper adds: One‐quarter of children with postneonatally acquired cerebral palsy (CP) have a major congenital anomaly.Cardiac anomalies, often severe, are the most common anomalies.Causes of postneonatally acquired CP differ between children with and without congenital anomalies. [ABSTRACT FROM AUTHOR]

Background: Progressive musculoskeletal pathology is ubiquitous among children with cerebral palsy (CP). Corrective surgery places them at risk of major blood loss and red blood cell (RBC) transfusion. Significant variability exists in uptake of perioperative patient blood management (PBM) strategies. This study aimed to examine factors contributing to RBC transfusion and assist in future development of care pathways. Methods: A retrospective review at a tertiary paediatric hospital was undertaken to identify patients with CP undergoing either primary spinal fusion or single event, multilevel surgery (SEMLS) between 2010 and 2015. Solely soft tissue procedures were excluded. Data collected included demographics, Gross Motor Function Classification System level, surgical details, perioperative PBM and transfusion rates. Univariable analysis was performed to assess contributing factors to RBC transfusion. Results: A total of 36 spinal fusion and 98 SEMLS patients were included. Preoperatively, 12% were anaemic, but only 19% had a ferritin checked. Overall, 49 patients (37%) received RBC transfusions. Intraoperative usage of tranexamic acid and cell salvage was 89% and 81%, respectively, for the spine cohort, and 22% and 3% for the SEMLS cohort. Successively higher Gross Motor Function Classification System levels, sodium valproate usage, longer surgical times, spinal fusion, pelvis instrumentation and more osteotomies were associated with RBC transfusion. Conclusion: More than one‐third of CP patients who underwent major orthopaedic surgery received RBC transfusion. As expected, the more severely affected patients undergoing longer procedures were at highest risk. Significant improvements can be made in PBM to help optimize patients for surgery and minimize the need for transfusion. [ABSTRACT FROM AUTHOR]

CP subgroups To explore whether specific aetiological and clinical variables, or combinations thereof, were related to extreme biomarker values in CP cases, additional variables were extracted from the Victorian Cerebral Palsy Register. To assess generalizability to the wider CP population, Table S1 (online supporting information) shows characteristics of the 435 matched CP singleton cases and compares them to the 941 records from the eligible CP sample that were unable to be matched to a cFTS record. Nuchal translucency and CP There was little difference in the mean nuchal translucency MoMs between CP cases (geometric mean 0.96 [95% CI 0.94-0.98]) and non-CP controls (geometric mean 0.95 [95% CI 0.95-0.96]; I p i =0.62 for difference; Table 1). When PAPP-A and -hCG levels were considered together, CP pregnancies were 2.6 times more likely than non-CP pregnancies to have low levels of both PAPP-A and -hCG. [Extracted from the article]

Aims: To describe the prevalence of, and factors associated with, anxiety in 6-18-year-old children with cerebral palsy (CP) and determine how often clinicians screen for and manage anxiety in this group.Methods: Using a population CP register as the sampling pool, 569 families were approached by email, and 172 (mean age of children 12 years 7 months [SD 3 years 5 months]; 96 males) participated. Parents and, where able, children completed the Screen for Child Anxiety Related Emotional Disorders (SCARED). Parents also completed the Strengths and Difficulties Questionnaire. Children's medical records were searched for previous anxiety diagnoses and treatments.Results: Clinically significant anxiety was identified in 38% of children on parent reports and 46% on child reports. Girls were twice as likely to have anxiety (p = 0.02). Parent- and child-reported scores were strongly correlated (r = 0.853). Fewer parents of children with intellectual and communication impairments completed the survey. Based on the SCARED parent reports, anxiety was not identified by a clinician in 16 children (43%) with clinically significant anxiety.Conclusion: Anxiety symptoms are prominent among children with CP, indicating a need for routine screening. Available screening tools are unsuitable for children with more severe limitations in cognition and communication; further research is needed to address this gap. [ABSTRACT FROM AUTHOR]

Aim: To determine: the effectiveness of three anticholinergic medications in reducing drooling in children with developmental disabilities (such as cerebral palsy, intellectual disability, and autism spectrum disorder), the frequency and nature of side effects, and their impact on treatment discontinuation.Method: After prescription of benzhexol hydrochloride, glycopyrrolate, or scopolamine patches at a tertiary saliva control clinic, all carers of 110 consecutive, eligible patients were recruited over a 5-year period. They provided data for 52 weeks, or until drug discontinuation, on compliance, drooling, adverse effects, and reasons for cessation. We evaluated and compared best drooling response, side effects, and drug cessation rates using survival analysis, and the effect of baseline variables on the discontinuation rate using proportional hazards regression.Results: Among 110 participants (71 males, 39 females; mean age 8y 5mo [SD 4y 3mo], range 1y 11mo-18y 11mo), benzhexol, glycopyrrolate, and scopolamine were prescribed 81, 62, and 17 times respectively, with respective response rates of 85%, 75%, and 65%. Poor head control and poor oromotor function were predictive of poor response. Side effects frequently prompted drug cessation in males more than females (hazard ratio 1.8 [95% confidence interval 1.0-3.2], p=0.048). Glycopyrrolate had the fewest side effects.Interpretation: Benzhexol, glycopyrrolate, and scopolamine reduce drooling, but improvement is offset by adverse side effects. Overall, glycopyrrolate performs best.What This Paper Adds: In drooling, glycopyrrolate produced the greatest improvement with fewer side effects compared with benzhexol and scopolamine. Poor head control and poor oromotor function were associated with poor response. Medication side effects were common and often led to treatment discontinuation. Behavioural issues instigated cessation of benzhexol more often in males than females. [ABSTRACT FROM AUTHOR]

Background: Direct oral anticoagulant drugs (DOACs) are known to improve clinical outcomes and decrease bleeding rates compared with warfarin. The effect of the introduction of DOACs into Australian general prescribing practice on anticoagulant‐related hospital presentations related to bleeding is unknown. Aim: This study compared the rate of anticoagulant‐related hospital presentations related to bleeding for DOACs and warfarin in South Australia. Methods: A retrospective observational cohort study across metropolitan tertiary hospitals identified hospital presentations related directly to an anticoagulant bleeding event. Presentation rates were calculated against prescribing quantities from Pharmaceutical Benefits Scheme data. Data were collected across two periods: January–June 2013 (warfarin‐only prescribing) and January–June 2015 (combined warfarin and DOAC prescribing). The main outcome measure was the number of hospital presentations per 100 patient‐years of prescribed anticoagulant therapy. Results: For the total study period, the overall hospital presentation rate for any DOAC use was 0.52 per 100 patient‐years, compared with 1.51 per 100 patient‐years for warfarin (incidence rate ratio 2.89; 95% confidence interval 2.02–4.12; p < 0.001). One hospital presentation was associated with each 67 years of warfarin use, and 192 years of DOAC use. The mean cost associated with warfarin and DOAC hospital presentations was similar ($3705 and $3671 respectively; p = 0.69). There were a further 0.28 presentations per 100 patient‐years for the management of supratherapeutic International Normalised Ratios in warfarin users, at a cost of $3233 per episode. Conclusion: The use of DOACs in general practice in the Australian setting is associated with decreased anticoagulant‐related hospital presentations for bleeding. [ABSTRACT FROM AUTHOR]

Aim: To describe the characteristics of emergency department (ED) presentations due to complications from gastrostomy or gastrojejunal feeding tubes among children with cerebral palsy (CP), the complexity of complications and the management approaches taken.Methods: The Victorian CP Register was linked to the ED databases of Victoria's two tertiary paediatric hospitals, and data on presentations due to feeding tube complications were identified based on discharge diagnosis codes. Additional data on presentations were extracted from medical records.Results: Over 5 years, there were 234 ED presentations due to feeding tube-related complaints among a CP cohort (n = 2183). ED notes were located for 183 of the 234 presentations. The majority of presentations (90%) involved children with severe gross motor impairment. A total of 46% of presentations (n = 84) was triaged as lower urgency, and 68% (n = 124) took place between 08:00 am and 06:00 pm. The most common presenting complaint was tube dislodgement (n = 105; 70%). No investigations were recorded in the majority of cases, and in almost 90% of cases, the feeding tube was successfully replaced in the ED, usually by an ED physician (n = 74) and less frequently by a surgeon (n = 9), gastroenterologist (n = 2) or nurse (n = 8); 9% (n = 17) resulted in a hospital admission.Conclusions: Most ED presentations due to feeding tube complaints in children with CP are in children with severe gross motor impairment but are able to be managed in the ED. As such, it is likely that care givers and other health professionals could manage some of the complications experienced in primary health-care settings closer to home. [ABSTRACT FROM AUTHOR]

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.)

Aim: A population-based observational study design was used to describe the epidemiology of intellectual disability in cerebral palsy (CP) in terms of clinical and neuroimaging associations, and to report the impact of intellectual disability on utilization of health services and length of survival.Method: Population CP registry data were used to retrospectively assess the frequency of intellectual disability and strength of associations between intellectual disability and mobility, epilepsy, vision, hearing, communication, and neuroimaging patterns (n=1141). Data linkage was undertaken to assess usage of hospital inpatient and emergency department services. Survival analysis was performed in a 30-year birth cohort (n=3248).Results: Intellectual disability, present in 45% of the cohort, was associated with non-ambulation (47% vs 8%), later walking (mean 2y 7mo vs 1y 9mo), hypotonic (8% vs 1%) or dyskinetic (9% vs 5%) CP, a quadriplegic pattern of motor impairment (42% vs 5%), epilepsy (52% vs 12%), more emergency and multi-day hospital admissions, and reduced 35-year survival (96% vs 71%). Grey matter injuries (13% vs 6%), malformations (18% vs 6%), and miscellaneous neuroimaging patterns (12% vs 4%) were more common in people with intellectual disability.Interpretation: Intellectual disability adds substantially to the overall medical complexity in CP and may increase health and mortality disparities.What This Study Adds: Cerebral maldevelopments and grey matter injuries are associated with higher intellectual disability rates. Health care is more 'crisis-driven' and 'reactive' in children with co-occurring intellectual disability. Length of survival is reduced in individuals with CP and co-occurring intellectual disability. [ABSTRACT FROM AUTHOR]

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.)

Still life occupied a position in the Socialist Realist canon so marginal that it could barely be called Socialist Realism at all. Although some artists attempted, in the Stalin era, to prove the genre's credentials at least as a component of Socialist Realist visual culture, a number of its genre characteristics rendered it ill-suited and even antithetical to the mandatory tasks of 'depicting reality in its revolutionary development' and demonstrating the role of the party-state and its leaders in achieving the radiant future. The paper focuses on the work of Robert Fal'k (1886-1958), an artist multiply marginalized in the Soviet art establishment-both as a person and through his work in the lowly, liminal genre of still life-yet nevertheless central to the story of Soviet art. It examines, from different perspectives, the quiet challenge his work seemed to present both to the vainglory of Soviet power and the chiliasm of Socialist Realism. In his Potatoes (1955) the genre characteristics of still life which placed it in the basement of Soviet public culture are so hypertrophied as to become a kind of unspoken worm's eye critique of Socialist Realism and the faith in state-led progress that it represented. It argues that, in the context of destalinization, when the modernist assertion of autonomy of art and artist presented a perceived challenge to party control over the arts, Fal'k's work alluded to the absence of the state and its powerlessness when faced with the ultimate projects of existence and of painting. Turning the tables on the Soviet state authorities that had marginalized it, his still life marginalized the state as irrelevant to art and life. [ABSTRACT FROM AUTHOR]

Aim: The overall aim was to investigate the feasibility and utility of linking a cerebral palsy (CP) register to an administrative data set for health services research purposes. We sought to compare CP hospital admissions to general childhood population admissions, and identify factors associated with type and frequency of admissions in a CP cohort.Method: The CP register for Victoria, Australia was linked to the state's hospital admissions database. Data pertaining to the admissions of a CP cohort (n=1748) that took place between 2007 and 2014 were extracted. Population data were also obtained.Results: Overall, 80% of the CP cohort (n=1401) had at least admission between 2007 and 2014, accounting for 11 012 admissions or 1.5% of all admissions in their age group. Compared to general population admissions, CP admissions were more costly and more likely to be elective (66% vs 57%; p<0.001), medical (71% vs 57%; p<0.001), and to take place in metropolitan hospitals (92% vs 78%; p<0.001). Increased CP severity and complexity were associated with having more admissions and a higher proportion of admissions attributable to respiratory illness.Interpretation: By linking with administrative data sets, CP registers may be useful for health services research and inform health service delivery. [ABSTRACT FROM AUTHOR]

Aim: Limited information exists on the ability of children with cerebral palsy (CP) to ride a two-wheel bicycle, an activity that may improve health and participation. We aimed to describe bicycle-riding ability and variables associated with ability to ride in children with CP (Gross Motor Functional Classification System [GMFCS] levels I-II) compared with children with typical development.Method: This case-control study surveyed parents of 114 children with CP and 87 children with typical development aged 6 to 15 years (115 males, mean age 9y 11mo, standard deviation [SD] 2y 10mo). Kaplan-Meier methods were used to compare proportions able to ride at any given age between the two groups. Logistic regression was used to assess variables associated with ability to ride for children with CP and typical development separately.Results: The proportion of children with CP able to ride at each level of bicycle-riding ability was substantially lower at each age than peers with typical development (p<0.001). While most children with typical development were able to ride independently by 10 years of age, 51% of children with CP classified as GMFCS level I and 3% of those classified as GMFCS level II had obtained independent riding in the community by 15 years of age. Variables associated with ability to ride for children classified as GMFCS level I were age and parent-rated importance of their child being able to ride.Interpretation: Some independently ambulant children with CP can learn to ride a bicycle, in particular if they are classified as GMFCS level I. Variables associated with ability to ride deserve consideration in shaping future efforts for the majority of this population who are not yet able to ride. [ABSTRACT FROM AUTHOR]

Aim: The aim of the study was to investigate the patterns of medical service use in children with cerebral palsy (CP), taking into account child and family characteristics.Methods: Nine hundred and one parents and carers of children registered with the Victorian CP Register were invited to complete a survey. Participants were asked about their child's appointments with general practitioners and public and private paediatric medical specialists over the preceding 12 months. Information on family characteristics and finances was also collected. Data on CP severity and complexity were extracted from the CP Register.Results: Three hundred and fifty parents and carers (39%) participated. Of these, 83% reported that their child had ≥1 appointment with a general practitioner over the preceding 12 months, while 84% had ≥1 appointment with a public or private paediatric medical specialist. Overall, 58% of children saw 2-5 different paediatric medical specialists, while 9% had appointments with ≥6 clinicians. Children with severe and complex CP were more likely to have had ≥1 appointment with a publically funded paediatric medical specialist and had seen a greater number of different clinicians over the study period. Family characteristics were not associated with service use.Conclusions: Children with CP are managed by a number of paediatric medical specialists, and they continue to see a range of specialists throughout adolescence. In Victoria, differences in service use are not based on family characteristics; instead the highest service users are those with severe and complex CP. For this group, care co-ordination and information sharing between treating clinicians are important, if gaps in care are to be avoided. [ABSTRACT FROM AUTHOR]

Aim: The aim of this study was to describe the patterns of therapy service use for a sample of children and adolescents with cerebral palsy over a 1 year period and to identify factors associated with frequency of therapy and parental satisfaction with therapy frequency.Methods: Parents of 83 children completed a survey on their child's use of occupational therapy, physiotherapy and speech and language pathology services over the previous year. Participants were randomly selected from a sample stratified by age and Gross Motor Function Classification System (GMFCS) level.Results: During the year prior to survey completion, 83% of children had received occupational therapy, 88% had received physiotherapy and 60% had received speech and language pathology services. Frequency of therapy was higher for younger children (P < 0.01), those classified at GMFCS levels IV-V (P < 0.05) and those attending schools specifically for children with disabilities.Conclusions: Current structures for therapy service delivery for children with cerebral palsy are systems-based, and age-based funding systems and the organisation of services around the education system are preventing the delivery of needs-based therapy. Paediatricians that care for children and young people with cerebral palsy need to pay particular attention to those that may miss out on therapy due to age or school type, and support these families in accessing appropriate therapy. [ABSTRACT FROM AUTHOR]

Aim: Males typically outnumber females in cerebral palsy (CP) cohorts. To better understand this 'male disadvantage' and provide insight into causal pathways to CP, this study used 1983 to 2009 Australian CP and population birth cohorts to identify associations and trends with respect to biological sex and CP.Method: Within birth gestation groups, sex ratios were calculated to evaluate any male excess in the CP cohort compared with livebirths, neonatal deaths, neonatal mortality and survival rates, neonatal survivors, and CP rates in survivors. Sex- and gestation-specific trends in neonatal mortality, CP rates, and CP sex ratios were assessed by plotting their annual frequencies and fitting quadratic curves.Results: Over-representation of males in preterm live births partly explained the male excess in the CP cohort after preterm birth, especially at 28 to 31 weeks. Higher CP rates in male neonatal survivors also contributed to the male excess in CP, particularly at <28 and 37+ weeks. Higher neonatal mortality rates in males at all gestations had little impact on the CP sex ratio. There was no clearly discernible change over time in the CP sex ratio.Interpretation: Gestation-specific associations between sex and CP provide insight into causal pathways to CP and suggest sex-specific differences in response to neuroprotective strategies. [ABSTRACT FROM AUTHOR]

Aim: To describe the distribution of impairments among persons with cerebral palsy (CP) in a large Australian cohort.Method: Records of persons on the Australian Cerebral Palsy Register (ACPR) (n=3466) born from 1996 to 2005 were reviewed to extract year of birth, sex, CP subtype, Gross Motor Function Classification System (GMFCS) level, and impairments in vision, hearing, speech, intellect, and epilepsy. The distributions of GMFCS levels and CP subtype were plotted, and the proportions of each level of impairment were tabulated and presented as stacked graphs within the GMFCS and CP subtype distributions.Results: The proportions of persons with CP with each associated impairment increased with increasing GMFCS level. Compared with other spastic CP subtypes, individuals with spastic quadriplegia had higher frequencies of all associated impairments. Other than epilepsy, which was most prevalent in persons with spastic quadriplegia (53%), all impairments were most frequent in non-spastic CP subtypes. Hearing impairment was recorded for 21% of persons with dyskinesia whereas the hypotonic subtype had the highest prevalence of visual impairment (57%), intellectual impairment (90%), and speech impairment (95%).Interpretation: Distributions of associated impairments across all GMFCS levels and CP subtypes in a large cohort are presented in formats suitable for clinical use and discussion with families. [ABSTRACT FROM AUTHOR]

Aim: Our aim was to build on previous research indicating that rates of cerebral palsy (CP) in the Australian state of Victoria are declining, and examine whether severity of impairments is also decreasing.Method: Data on individuals with CP were extracted from the Victorian Cerebral Palsy Register for birth years 1983 to 2009. The yearly rates of dichotomized categories for gross motor function, motor laterality, intellectual impairment, and epilepsy per 1000 neonatal survivors and proportions in the CP cohort were tabulated and plotted by birth gestation. Linear regression modelling was used to fit prediction curves; likelihood ratio tests were used to test for differences in trends between impairment severity groups.Results: Since the mid-1990s, CP rates declined in neonatal survivors of birth at all gestations. Our data suggest that the decreasing CP rates were associated with relatively greater decreases in the rates of Gross Motor Function Classification System levels III to V, bilateral CP, epilepsy, and intellectual impairment (all p<0.005). Some variation was seen between birth gestation groups.Interpretation: Declines in rates of CP of all levels of severity and complexity from the mid-1990s provides 'real-world' support for the effectiveness of concurrent neuroprotective strategies and continual innovation in perinatal practices. [ABSTRACT FROM AUTHOR]

Aims: In a population cohort of children with grey matter injury (GMI) and cerebral palsy (CP), we aimed to describe and classify magnetic resonance imaging characteristics specific to GMI, and to identify key structure-function associations that serve as a basis for rating GMI in clinically relevant ways. Method: Symmetry, extent of cerebral injury, and pathological pattern for 54 children (37 males, 17 females) with CP and a predominant GMI pattern on chronic-phase magnetic resonance imaging were related to gross motor function, motor type and topography, epilepsy, intellectual disability, blindness, and deafness. Results: Relative to mild GMI where there was no pallidal abnormality, severe GMI, comprising pallidal abnormality alone or in conjunction with other deep nuclear and generalized cortical-subcortical involvement, was strongly associated with Gross Motor Function Classification System levels IV to V (OR 35.7 [95% CI 3.5, 368.8]). Involvement of the basal ganglia was associated with non-spastic/mixed motor types, but predominantly where cortical-subcortical grey and white matter involvement was not extensive. The prevalence of epilepsy was highest where there was diffuse cortical-subcortical involvement and white matter loss. Interpretation: Better understanding of structure-function relationships in CP and GMI, and how to rate the severity of GMI, will be helpful in the clinical context and also as a basis for investigation of causal pathways in CP. [ABSTRACT FROM AUTHOR]

Aims: The aim of this study was to describe the pattern of tertiary paediatric emergency department (ED) use in children and young people with cerebral palsy (CP).Methods: A retrospective analysis of ED data routinely collected at the two tertiary paediatric hospitals in Victoria, Australia, cross-matched with the Victorian Cerebral Palsy Register. Data pertaining to the ED presentations of 2183 registered individuals born 1993-2008 were obtained.Results: Between 2008 and 2012, 37% (n = 814) of the CP cohort had 3631 tertiary paediatric ED presentations. Overall, 40% (n = 332) of presenters were residing in inner metropolitan Melbourne; 44% (n = 356) in outer Melbourne; and 13% (n = 108) in regional Victoria. Presenters were more likely than non-presenters to be younger, non-ambulant and have epilepsy. In total, 71% of presentations were triaged as Australasian Triage Scale 1-3 (urgent), and 44% resulted in a hospital admission. Disorders of the respiratory, neurological and gastrointestinal systems, and medical device problems were responsible for 72% of presentations.Conclusion: Many of the tertiary paediatric ED presentations in this group were appropriate based on the high admission rate and the large proportion triaged as urgent. However, there is evidence that some families are bypassing local services and travelling long distances to attend the tertiary paediatric ED, even for less urgent complaints that do not require hospital admission. Alternative pathways of care delivery, and strategies to promote the management of common problems experienced by children and young people with CP in non-paediatric EDs or primary care settings, may go some way towards reducing unnecessary tertiary paediatric ED use in this group. [ABSTRACT FROM AUTHOR]

For firms involved with the very early stages of emergent radical innovation, technical goals are often held in the mind(s) of only one or a few individuals. The way these individuals mentally imagine or visualize such goals, or 'technology visions,' provides an important looking glass for understanding a firm's progression along the path of involvement from a technical discontinuity toward project-level and organizational-level involvement with a given technology. Utilizing a large sample of firms engaged in radical innovation in North America and the United Kingdom, this empirical study examines the impact of five dimensions of technology vision on early success: benefits goals, efficiency goals, magnetism, specificity, and infrastructure clarity. Technology vision is found to have a significant positive impact on technical competitive advantage, early success with customers, and ability to attract capital, as measures of early success. [ABSTRACT FROM AUTHOR]

The overall question that this research seeks to answer is the following: Are there individual and/or organizational resources related to 'divergent thinking' that enhance or inhibit the firm's market visioning competence in the case of radical innovation? In order to answer this question, in this paper, the key focus is on the realm of potential divergent thinking-related resources that the firm may possess and access to aid in the creation and development of an effective market visioning competence. In particular, individual-level factors related to divergent thinking capabilities and, at the organizational level, encouragement of such capabilities, are investigated. Specifically, we propose that two organizational-level factors related to organizational encouragement of divergent thinking ('encouraging ideas' and 'encouraging diversity'), two individual-level divergent thinking attitudes factors ('openness' and 'ability to move from divergent thinking to convergent thinking efficiently and effectively'), one individual-level ideational behavior factor ('ability to generate new ideas'), and a further individual cognitive factor ('need for cognition') have direct impacts on market visioning competence. Scales are then developed and tested for measuring these potential antecedents, and they are included in a measurement model with market visioning competence to enable further research. [ABSTRACT FROM AUTHOR]

Perceived temporal trends in recognition and diagnosis of Prader-Willi syndrome served as the rationale for an updated epidemiological profile of individuals with this syndrome. Data from the Victorian Prader-Willi Syndrome Register were used to explore birth prevalence, birth characteristics, timing of diagnosis, and molecular mechanism, and to identify trends over time. Maternal age, birth gestation, small for gestational age, and sex were compared across molecular mechanisms. Between 1951 and 2012 there were 160 individuals with Prader-Willi syndrome, known to the Victorian Prader-Willi Syndrome Register, who were born in the Australian state of Victoria. The birth prevalence for individuals with a molecular diagnosis of Prader-Willi syndrome was estimated to be 1:15,830 for 2003-2012. Compared to 1973-1981, the decade 2003-2012 saw an increase in the rate of molecular diagnosis from 58% to 96%, more complete identification of the molecular mechanism (42% vs. 83%), earlier molecular diagnosis (1.3 years vs. 8.6 weeks), and a rise in the relative proportion of maternal uniparental disomy from 0% to 45%. One quarter of infants was born preterm and 53% were small for gestational age. This study confirms a temporal change in diagnostic patterns, suggests a greater relative contribution of maternal uniparental disomy as a molecular mechanism, provides a more robust estimate of birth prevalence and provides evidence of in utero growth restriction for this group. These findings have important clinical and health service delivery implications and pave the way for further research in Prader-Willi syndrome. © 2014 Wiley Periodicals, Inc. [ABSTRACT FROM AUTHOR]

Aim The aim of this study was to review the distribution of neuroimaging findings from a contemporary population cohort of individuals with cerebral palsy ( CP) and to facilitate standardization of imaging classification. Method Publications from 1995 to 2012 reporting imaging findings in population cohorts were selected through a literature search, and review of the titles, abstracts, and content of studies. Relevant data were extracted, including unpublished data from Victoria, Australia. The proportions for each imaging pattern were tabulated, and heterogeneity was assessed for all individuals with CP, and for subgroups based on gestational age, CP subtype, and Gross Motor Function Classification System level. Results Studies from three geographic regions met the inclusion criteria for individuals with CP, and two additional studies reported on specific CP subtypes. Brain abnormalities were observed in 86% of scans, but were observed least often in children with ataxia (24-57%). White matter injury was the most common imaging pattern (19-45%), although the proportions showed high heterogeneity. Additional patterns were grey matter injury (21%), focal vascular insults (10%), malformations (11%), and miscellaneous findings (4-22%). Interpretation This review suggests areas where further dialogue will facilitate progress towards standardization of neuroimaging classification. Standardization will enable future collaborations aimed at exploring the relationships among magnetic resonance imaging patterns, risk factors, and clinical outcomes, and, ultimately, lead to better understanding of causal pathways and opportunities for prevention. [ABSTRACT FROM AUTHOR]

Aim The aim of this study was to describe the distribution of magnetic resonance imaging ( MRI) patterns in a large population sample of children with cerebral palsy ( CP) and to examine associations between MRI patterns, and antenatal and perinatal variables. Method Data were retrieved from the Victorian CP Register for 884 children (527 males, 357 females) born between 1999 and 2006. Postneonatal MRI was classified for 594 children. For 563 children (329 males, 234 females) for whom classification was to a single MRI pattern, the frequency of each variable was compared between patterns and with the population frequency. Results White matter injury was the most common MRI pattern (45%), followed by grey matter injury (14%), normal imaging (13%), malformations (10%), focal vascular insults (9%), and miscellaneous patterns (7%). Parity, birth gestation, level of neonatal care, Apgar score, and time to established respiration varied between MRI patterns ( p<0.01). Nulliparity was most strongly associated with focal vascular insults, whereas multiparity was associated only with malformations. Grey matter injury was not associated with birth in a tertiary unit, but was strongly associated with severe perinatal compromise. The frequency of neonatal seizures and of nursery admissions was lowest among children with malformations. Interpretation As known risk factors for CP are differentially associated with specific MRI patterns, future exploration of causal pathways might be facilitated when performed in pathogenically defined groups. [ABSTRACT FROM AUTHOR]

Aim: To assess the recording of weight and height in children with disabilities and identify subgroups at risk of being either underweight or overweight. Methods: A retrospective clinical audit was undertaken within the Department of Developmental Medicine at The Royal Children's Hospital, Melbourne. Most recent weight and height measurements and data about the child's underlying condition were collected from the medical records of children attending a clinical appointment over a 3-month period. Primary medical diagnosis, age, gender, mode of feeding and ability to ambulate were recorded. Body mass index was calculated and corrected for age and gender. Results: Study sample size was 583 (356 males), mean age was 8.25 years (range 2.8-17.4 years). Body mass index could be calculated for 48%. Height was less commonly measured in those with severe physical impairments. A survey of barriers to weight and height measurement showed that the main barrier to measuring height was practical difficulties. The percentage of children classified as overweight/obese was 26.6% and this was associated with intellectual disabilities, moderately impaired ambulation, older age and female gender; 13.5% of the study group was underweight, the majority of whom were non-ambulant. Conclusions: Although significant rates of underweight and overweight were identified in children with a range of disabilities within our service, monitoring of weight status was suboptimal. Despite this, data indicate that specific subgroups of children with disability appear at risk of being either overweight or underweight. Where height cannot be measured, other anthropometric measures such as waist circumference may be useful in the assessment of children with disabilities. [ABSTRACT FROM AUTHOR]

'Market vision' is a mental model that helps focus the organization on a new market application for an advanced technology during the fuzzy front end of the new product development process. Previous research demonstrates that firms involved in the development of radically new, high-tech products need to develop a market visioning competence ( MVC) in order to develop an effective market vision ( MV), and these capabilities, in turn, have been found to have a positive effect on key aspects of the early performance ( EP) of these firms-specifically, the ability to attract capital and early success with customers. Based on a major empirical study of the nanotechnology sector, the research described in this paper takes an important step forward by focusing on factors in both the external and internal environment of the firm, and their moderating impact on the paths that link MVC, MV, and EP. External structural factors relevant to the firm's competitive environment as well as internal factors, including firm resources, size, incumbency, and technology, are shown to have significant moderating effects both on the way in which MV unfolds and on its capacity for affecting positive returns for the firm when undertaking radical innovation. Five of seven hypotheses were supported by the research. Both level of incumbency (the extent to which the firm has taken part in previous generations of a given technology) and resource availability are shown to positively impact the link between MVC and MV. Also, appropriability (i.e., protection for innovations) and reputation of the firm were found to positively impact the path to EP. Finally, a low level of industry concentration-that is, a large number of small firms-were found to have a positive effect on the path to EP. In sum, the findings support the structure of the model and the majority of the hypothesized moderating relationships, suggesting important implications for management. [ABSTRACT FROM AUTHOR]

Aim To establish a prevalence estimate for drooling and explore factors associated with drooling in a population sample of children with cerebral palsy (CP) aged 7 to 14 years living in Victoria, Australia. Method A self-report questionnaire was used to collect data on drooling from parents of children born between 1996 and 2001, and registered with the Victorian Cerebral Palsy Register. Results A total of 385 children (231 males, 154 females; mean age 10y 9mo [SD 1y 7mo], range 8-14y) were studied. The clinical type and distribution of CP were spastic (341), ataxic (16), dyskinetic (17), hypotonic (10), and unknown (1). Distribution in Gross Motor Function Classification System (GMFCS) levels was I (103), II (98), III (52), IV (63), V (61), and unknown (8). After adjustment for topographical pattern of motor impairment and GMFCS level, 40% were reported to have experienced drooling between 4 years of age and the time of completing the questionnaire. A significantly higher prevalence of drooling was found in children with poor gross motor function and in those with more severe presentations of CP, including poor head control, difficulty with eating, and inability to sustain lip closure ( p<0.001 for each). Drooling was shown to be significantly associated with both intellectual disability and epilepsy in this group of children ( p<0.001 for both). Interpretation With a prevalence of 40%, drooling is an important comorbidity in CP. It was considered severe in 15% of children. Poor oromotor function was associated with drooling and could be the target of interventions for this under-researched problem. [ABSTRACT FROM AUTHOR]

Aim: The aim of this study was to describe the rates, predictors and causes of mortality in a population sample of individuals with Prader-Willi syndrome (PWS). Methods: One hundred sixty-three individuals with PWS (90 males and 73 females, ages: 3 weeks to 60 years) were identified from the Victorian PWS Register. Information on demographics, age at diagnosis, genetic mechanism, age at which obesity developed and last known body mass index measurement were extracted. Notification and causes of death were obtained through linkage with Australian national and state of Victoria death indexes. Survival analysis was used to estimate the probability of survival and the effect of obesity on survival. Mortality rate ratios were calculated to investigate the effect of the factors listed above on mortality. Results: Fifteen deaths were recorded (nine males and six females), corresponding to an 87% probability of survival to 35 years. The probability of survival was significantly lower for individuals with known obesity ( P= 0.03), but there was no strong evidence for an effect on survival for the other factors studied. Cardiac or respiratory conditions were common causes of death after the age of 15 years. Conclusions: The effect of known obesity on the probability of survival and the causes of death reported in this and other studies suggest an important association between obesity and early death in adults with PWS. This finding highlights the critical nature of preventative and intervention strategies aimed at minimising the effects of hyperphagia in individuals with PWS. [ABSTRACT FROM AUTHOR]

Aim This study used data collected prospectively since 1986 from a population-based cerebral palsy registry to explore the rates, predictors, trends, and causes of mortality for individuals born in Victoria, Australia, between 1970 and 2004. Method Data were extracted for 3507 individuals (1972 males; 1535 females). The probability of survival before 31 May 2010 was determined using the Kaplan-Meier method; age-specific mortality rates were calculated per 1000 person-years and related to population rates. Using Cox proportional hazards regression, relative risks of mortality were estimated for different categories of chosen demographic and clinical variables. Causes were tabulated according to the direct cause of death. Results There were 418 deaths. Crude mortality was 20% at the age of 40 years. Relative to the population, mortality was highest in children aged under 15 years and decreased to twice the population rate at the age of 35 years. The strongest independent predictor of mortality was no independent ambulation (adjusted hazard ratio 6.2 [95% confidence interval 3.3-11.8]); additional predictors were severe intellectual impairment (3.0 [1.7-5.2]), epilepsy (1.4 [1.1-1.9]), deafness (2.6 [1.4-4.7]), and term birth (1.8 [1.3-2.4]). No improvement in survival was seen over time (unadjusted hazard ratio 1.00 [95% CI 0.99-1.01]). Respiratory causes were the most common direct causes of death. Interpretation Rates, predictors, and causes of death for individuals with cerebral palsy in Victoria were similar to those found in other population cohorts. Lack of improvement in survival since 1970 was an unexpected finding that warrants further investigation. [ABSTRACT FROM AUTHOR]

In an article by Reid and de Brentani, a theoretical model of the process and structure for the fuzzy front-end ( FFE) of new product development ( NPD) for discontinuous innovations was proposed. Its basic premise is that information flow in the early development of such innovations moves from the environment into the firm, facilitated by individuals playing three key roles at three decision-making interfaces: (1) the boundary spanner at the boundary interface, (2) the gatekeeper at the gatekeeping interface, and (3) what is identified in this paper as the 'project broker' at the project interface. The current paper builds on and augments the ideas presented in this theoretical model with the primary objective of formulating a set of propositions detailing factors affecting the flow of information, and thus role effectiveness at each of these interfaces for discontinuous innovations. The focus is on radically new innovations both because this type of innovation has the highest level of uncertainty during the FFE and because the development of products resulting from such innovations entails the greatest lack of understanding and the fewest strategies for effective management. To achieve this objective, individual, social system, and environmental factors, which promote and/or inhibit the effectiveness of the roles played during the three FFE phases, are examined in terms of both the speed and the quality of information flow. This is done with the goal of substantially improving NPD information as it proceeds through the FFE. In turn, it can help researchers, managers, and team players to better anticipate and meet the navigational challenges of this intrinsically complex, risky, but high potential, NPD scenario. [ABSTRACT FROM AUTHOR]

Aim The aim of this study was to describe the distribution of motor severity levels and temporal trends in an Australian population cohort and to review the distribution of Gross Motor Function Classification System (GMFCS) levels across cerebral palsy (CP) registries worldwide. Methods Data were extracted from the Victorian Cerebral Palsy Register for 3312 individuals (1852 males, 1460 females; mean age 21y 2mo [SD 9y 6mo]) with non-postneonatally acquired CP, born between 1970 and 2003. The proportions of each motor severity level were calculated and logistic regression analyses were used to assess trends over time. A systematic review of the literature was undertaken and GMFCS data were extracted based on previously devised criteria. The proportions were plotted and the degree of heterogeneity was assessed for each level. Results Population data from Victoria suggested a proportional increase in mild motor impairment (GMFCS levels I/II) from 54% of all cases of CP in the 1970s to 61% in the 2000s. For nine CP registries worldwide, the mean proportions of each GMFCS level, from level I to V, were 34.2%, 25.6%, 11.5%, 13.7%, and 15.6% respectively. There was substantial heterogeneity between registries for all levels except level III. Interpretation Despite the usefulness and reported reliability of the GMFCS, substantial variability was found in the distribution of GMFCS levels between population registries, particularly between levels I and II, suggesting greater classification uncertainty between these levels. Further research would be useful to determine whether routine collection of extra clinical information may facilitate more reliable classification. [ABSTRACT FROM AUTHOR]

Aim The aims of this study were to estimate the frequency of hearing loss in children with cerebral palsy (CP), to examine factors associated with hearing loss, and to describe aspects of hearing in a population sample of children with CP and hearing loss. Method A systematic review of the international literature was undertaken, and data on the frequency of hearing loss or severe hearing loss were extracted from 14 data sets based on previously devised criteria. Six hundred and eight-five children with CP (406 males, 279 females) born in Victoria, Australia, between 1999 and 2004 were identified from the Victorian Cerebral Palsy Register. Children were included if they had an established post neonatal cause for their CP before the age of 2 years. Additional information was collected on 48 children with documented hearing loss based on a four-tone pure tone average in the better ear. Results There was considerable variation in the definitions and proportions of hearing loss (range 4-13%) and severe hearing loss (range 2-12%) reported by CP registries in developed countries. In Victoria, 7% of individuals with CP had bilateral hearing loss of a moderate to profound degree, whereas the subgroup with a severe-profound degree of loss constituted 3% to 4% of the CP population. Interpretation These population-based data are likely to more accurately reflect the true frequency of defined hearing loss in children with CP than previous reviews. [ABSTRACT FROM AUTHOR]

Researchers have cited significant gaps in our knowledge regarding the early stages of vision formation in the radical innovation context and have emphasised the importance of further investigation in this area. As such, this paper aims first to build on the extant literature on organizational, project and Market Vision in order to construct a measure for Technology Vision through theory construction, scale development and modeling. The second goal is to help firms to better understand what the underlying components of Technology Vision are in order to offer themselves the best possible chance of success with the development of radically new, high-tech products. Based on samples of firms involved with radical innovation research and development in high-tech sectors in North America and the United Kingdom, conceptual and measurement studies conducted herewith suggest there are five factors related to Technology Vision: Technology Vision benefits, Technology Vision efficiency, Technology Vision magnetism, Technology Vision specificity, and infrastructure clarity. The paper concludes with an examination of the implications of these components of Technology Vision and discusses the need to understand its relationship with Market Vision and the performance of the firm. [ABSTRACT FROM AUTHOR]

Aim The aim of this study was to assess overall and gestational age-specific trends in the rate of cerebral palsy (CP) in Victoria, Australia, and to compare these findings with other population data. Method Individuals born in Victoria from 1970 to 2004 with non-postneonatally acquired CP were identified from a population register; 3491 were included in the study (1963 males, 1528 females). After a literature review, comparison data were extracted from publications using previously devised inclusion criteria. Rates were calculated per 1000 live births for all CP and by gestational age group: these were tabulated and plotted by year of birth. Results Data from nine registries, including the Victorian register, showed an increase in the rates of CP over the 1970s and 1980s, consistently seen in extremely preterm (<28wks) survivors but also in those born at term (≥37wks). Since the early 1990s, CP rates either stabilized or decreased, particularly for children born extremely preterm. Interpretation Increases in the rates of CP during the 1970s and 1980s are in part because of the increasing survival of extremely preterm infants that occurred without a concomitant improvement in neurological outcomes. Evidence from population samples now suggests that this trend has been reversed since the mid- to late 1990s. [ABSTRACT FROM AUTHOR]

The aim of this study was to describe the distribution of types of motor disorder (motor type) in a population-based registry of children with cerebral palsy (CP), to examine any trends in motor type distribution over time, and to make comparisons with other populations. Individuals born in Victoria, Australia, between 1970 and 2003 with congenital CP were identified from the Victorian Cerebral Palsy Register, 3297 of whom were included in the study (1840 males, 1457 females). Those who had a hypotonic motor type, those whose brain abnormality was believed to have developed after 28 days of life, and those with missing data were excluded from the study. The proportions of each motor type were calculated and plotted by year of birth. Logistic regression analyses were used to assess the mean change in odds per year of birth. A systematic review of the international literature was undertaken and comparison data were extracted based on previously devised criteria. The degree of heterogeneity was assessed and comparisons were made according to geographical region and reporting period. In Victoria, the predominant motor types for individuals with CP born between 1970 and 2003 were spasticity (91%), ataxia (5%), and dyskinesia (4%). There was no clear trend for any motor type over time. The mean proportions from two other large data sets were similar but there was considerable heterogeneity between 29 individual CP registries, mainly because of differences in the classification of mixed motor types. A more objective method for determining the predominant motor type is required to make the classification of motor type in CP more consistent and reliable. [ABSTRACT FROM AUTHOR]

For the 9% to 16% of children with cerebral palsy (CP) who have normal brain imaging, further testing for metabolic and/or genetic conditions has been recommended. This study aimed to identify a cohort of children with CP with normal magnetic resonance imaging (MRI), clinically review and describe the cases, and assess the value of testing for inherited metabolic disorders in these children. Children with congenital CP born from 1999 to 2005 were selected from a population register. Normal MRI reports were identified and the scans reassessed. Children whose scans were performed before 18 months were excluded, as were children with spastic CP (Gross Motor Function Classification System [GMFCS] level I). The remainder were reviewed clinically and offered investigations. Of 730 children identified, 515 had available imaging and 54 were confirmed as normal. Cases with non-spastic CP and those with milder clinical severity were more likely to have normal imaging. Twenty-three children (17 males, six females; mean age 6y 11mo, SD 1y 10mo, range 3y 0mo to 10y 0mo) were reviewed clinically and offered investigations. Twelve children had spasticity (11 with diplegia, one quadriplegia), three had dyskinesia, five ataxia, and three hypotonia. Two children functioned in GMFCS level I, 11 in level II, seven in level III and three in level IV. Four children with spasticity had unusual features. No alternative diagnoses were made. Although important to consider in individual cases, comprehensive metabolic testing failed to clarify the aetiology of CP further in this large cohort of children with normal MRIs, even those with atypical features. [ABSTRACT FROM AUTHOR]

Aim The aim of this study was to describe the frequency, risk factors, manifestations, and outcome of epilepsy in children with hemiplegic cerebral palsy (CP) due to perinatal arterial ischaemic stroke (AIS). Method The study group comprised 63 participants (41 males, 22 females) from a population-based CP register whose brain imaging showed perinatal AIS. Information collected included occurrence of neonatal seizures, family history of epilepsy, motor function and epilepsy onset, treatment, and outcome. Electroclinical findings were classified according to seizure semiology, seizure type, and epilepsy syndrome. Results Mean age of participants at the time of study was 10 years 6 months (SD 4y 7mo, range 4-20y). Gross Motor Function Classification System levels I and II were reported in 96% of participants, and Manual Ability Classification System levels I and II were reported in 79% of children. Thirty-four children (54%) developed epilepsy. Term delivery and more severe motor impairment were associated with epilepsy, but neonatal seizures and family history of epilepsy were not. Initial seizures were epileptic spasms, focal seizures, or myoclonic seizures. Focal seizure semiology suggested Rolandic or occipital seizure origin in the majority of children. Focal epileptic discharges in children with focal seizures had features of idiopathic partial epilepsy. Only 15% of children had active epilepsy 10 years after onset. Interpretation Despite a high incidence of epilepsy in children with hemiplegic CP due to AIS, the prognosis for seizure remission is good. Many children have clinical features, electroencephalography findings, and remission typical of idiopathic partial epilepsy. [ABSTRACT FROM AUTHOR]

Aim To determine whether assisted reproductive technologies (ART) were more likely to be the method of conception in singletons with cerebral palsy (CP) than in those without CP. Method Singletons with CP born between 1991 and 2004 were selected from the Victorian Cerebral Palsy Register and matched for birth year to two singletons randomly selected from the Victorian Perinatal Data Collection Unit. Data from both sources were linked to records from three ART centres. Conditional logistic regression was used to assess the association between CP and aspects of conception using ART. Multivariate models were adjusted for parity, previous miscarriages, sex, gestational age, birthweight, and weight for gestational age. Results We identified 1241 singletons with CP (males n=721 [58%], females n=420 [42/100]; motor type: spastic [87%; unilateral 37%; bilateral 63%], ataxic n=60 [5/100], dyskinetic n=46 [4/100], hypotonic n=29 [2/100], unknown n=25 [2/100]. Gross Motor Function Classification System levels were I n=363 [29/100], II n=297 [24/100], III n=137 [11/100], IV n=160 [13/100], V n=192 [15/100], and unknown n=92 [7/100]). Sixteen (1.3/100) of the children with CP and 25 (1.0/100) of 2482 children without CP were conceived using ART. There was no significant increase in the odds of children with CP being conceived using ART (adjusted odds ratio 1.19, 95% confidence interval (CI) 0.63, 2.24) nor in the odds of them being conceived by a subfertile couple without ART (adjusted odds ratio 2.7, 95% CI 0.87, 8.36). Interpretation Singleton conception using ART is not strongly associated with an increased risk of CP. [ABSTRACT FROM AUTHOR]

Having the “right” market vision (MV) in new product scenarios involving high degrees of uncertainty has been shown to help firms achieve a significant competitive advantage, which can ultimately lead to superior financial results. Despite today's increased rate of radical innovation, and hence the importance of effective vision, relatively little research has been undertaken to improve our understanding of this phenomenon. The exploratory and empirical investigation undertaken herewith responds to this research gap by focusing on MV and its precursor, market visioning competence (MVC), for radically new, high-tech products. MV is a clear and specific mental model/image that organizational members have of a desired and important product-market for a new advanced technology, and MVC is a set of individual and organizational capabilities that enable the linking of advanced technologies to a future market opportunity. Based on samples of high-tech firms involved in early technology developments, the measurement study indicates that five factors comprise MV (i.e., clarity, magnetism, specificity, form, and scope) and that four factors underlie MVC (i.e., networking, idea driving, proactive market orientation, and market learning tools). Structural equation modeling is used to demonstrate that MVC significantly and positively impacts MV and that each of these constructs significantly and positively influences certain aspects of early performance (EP) in new product development. This is the first empirical study to develop a comprehensive set of scales to measure these constructs and then to combine them in a model by which to examine their interrelationships. [ABSTRACT FROM AUTHOR]

Aim To describe the development and clinimetric properties of a new scale to evaluate changes in the impact of drooling in children with developmental disabilities. Method After examining the properties of potential items, 10 items were retained for inclusion in the final Drooling Impact Scale. The clinimetric properties of the scale were evaluated using data from two convenience samples of children attending a saliva-control clinic: a stable group ( n=31, 22 males, nine females; mean age 10y 7mo, SD 4y 5mo, range 3y 6mo–18y 3mo; cerebral palsy [CP] n=17, intellectual disability n=10; non-ambulatory n=13, nonverbal n=12) and an intervention group ( n=49, 29 males, 20 females; mean age 11y, SD 3y 6mo, range 3y 4mo–16y 10mo; CP n=31, intellectual disability n=15; non-ambulatory n=27, nonverbal n=28). To assess validity, changes in scores on the Drooling Impact Scale over time were compared with a carer’s global rating of change using Pearson’s correlations and t-tests. A concordance correlation coefficient was used to compute the level of agreement between assessments 1 month apart in stable children. Effect size, standardized response mean, Guyatt responsiveness statistic, and an unpaired t-test were used to estimate responsiveness. Results The correlation between the global rating and change in Drooling Impact Scale scores was 0.69 ( p<0.001). The concordance correlation coefficient was 0.85. An effect size of 1.8, standardized response mean of 1.5, Guyatt responsiveness statistic of 1.4, and mean group difference of 23.5 (95% confidence interval 17.4–29.6) were obtained. Interpretation The Drooling Impact Scale is a valid and reliable subjective measure that is responsive to change. [ABSTRACT FROM AUTHOR]