Challenges in Using Parent-Reported Bed and Wake Times for Actigraphy Scoring in Rett-Related Syndromes.

Sleep problems are common in Rett syndrome and other neurogenetic syndromes. Actigraphy is a cost-effective, objective method for measuring sleep. Current guidelines require caregiver-reported bed and wake times to facilitate actigraphy data scoring.

Polyhydroxybutyrate synthesis in Camelina: Towards coproduction of renewable feedstocks for bioplastics and fuels.

Plant-based co-production of polyhydroxyalkanoates (PHAs) and seed oil has the potential to create a viable domestic source of feedstocks for renewable fuels and plastics. PHAs, a class of biodegradable polyesters, can replace conventional plastics in many applications while providing full degradation in all biologically active environments. Here we report the production of the PHA poly[(R)-3-hydroxybutyrate] (PHB) in the seed cytosol of the emerging bioenergy crop Camelina sativa engineered with a bacterial PHB biosynthetic pathway.

Caregiver Perspectives on Pain Sensitivity and Pain Experience in Rett Syndrome.

Background: Although delayed or decreased responses to pain are commonly reported among caregivers of individuals with Rett syndrome (RTT), previous studies in relatively small samples have documented that caregivers are concerned about pain, particularly due to gastrointestinal and musculoskeletal conditions.

Aims: The purpose of the current study was to investigate in detail caregivers' perceptions of pain sensitivity, as well as the types, severity, and effect of pain experienced by individuals with RTT in a larger sample than previous studies.

Methods: A total of 51 caregivers (mostly mothers) participated in the study, which involved standardized questionnaires and interviews.

Preliminary assessment of the reliability and validity of infrared skin temperature measurements in Rett syndrome.

Background: As clinical trials for Rett syndrome are underway, there is a need to validate potential supplemental outcome measures that reflect important signs and symptoms. Autonomic dysfunction, particularly vasomotor dysfunction, is one potential area for which biomarkers could be developed.

Methods: In the current study, infrared thermal images of hands and feet from 26 females with Rett syndrome (aged 62 months to 39 years), and 17 females without known intellectual, genetic or neurological disorders (aged 55 months to 39 years) were collected.

Modifying quantitative sensory testing to investigate behavioral reactivity in a pediatric global developmental delay sample: Relation to peripheral innervation and chronic pain outcomes.

Early tactile and nociceptive (pain) mechanisms in children with global developmental delay at risk for intellectual and developmental disability are not well understood. Sixteen children with global developmental delay (mean age = 5.1 years, SD = 1.

Pain burden in children with cerebral palsy (CPPain) survey: Study protocol.

Pain is a significant health concern for children living with cerebral palsy (CP). There are no population-level or large-scale multi-national datasets using common measures characterizing pain experience and interference (ie, pain burden) and management practices for children with CP. The aim of the CPPain survey is to generate a comprehensive understanding of pain burden and current management of pain to change clinical practice in CP.

Presence and predictors of pain after orthopedic surgery and associated orthopedic outcomes in children with cerebral palsy.

While children with cerebral palsy (CP) may undergo 8-22 orthopedic surgeries in their lifetime, little is known about the associated pain. We aimed to assess the pain presence before and one year after lower extremity orthopedic surgery, predictors of pain presence at follow-up, and the association between pain and orthopedic outcomes related to surgery. This retrospective study included 86 children with CP (M age = 10.

Parental Pain Catastrophizing, Communication Ability, and Post-surgical Pain Outcomes Following Intrathecal Baclofen Implant Surgery for Patients With Cerebral Palsy.

There is strong evidence that psychosocial variables, including pain catastrophizing, influence parental and child ratings of pain, pain expression, and long-term outcomes among children with chronic pain. The role of these factors among children who have communication deficits due to cerebral palsy (CP) and other intellectual and developmental disabilities is currently unclear. In this study, parental pain catastrophizing was assessed before intrathecal baclofen (ITB) pump implantation for spasticity management in 40 children and adolescents with CP, aged 4 to 24 years.

Expression of and correlational patterns among neuroinflammatory, neuropeptide, and neuroendocrine molecules from cerebrospinal fluid in cerebral palsy.

Background: The underlying pathogenesis of cerebral palsy (CP) remains poorly understood. The possibility of an early inflammatory response after acute insult is of increasing interest. Patterns of inflammatory and related biomarkers are emerging as potential early diagnostic markers for understanding the etiologic diversity of CP.

Caregiver-Reported Pain Management Practices for Individuals With Cerebral Palsy.

Objective: To investigate types and intensity of pain experienced by individuals with cerebral palsy (CP) and common pain-relieving approaches used by caregivers.

Design: The approach was cross-sectional, using standardized interviews.

Setting: Individuals with CP were recruited from a specialty health care hospital.

Camelina sativa, an oilseed at the nexus between model system and commercial crop.

The rapid assessment of metabolic engineering strategies in plants is aided by crops that provide simple, high throughput transformation systems, a sequenced genome, and the ability to evaluate the resulting plants in field trials. Camelina sativa provides all of these attributes in a robust oilseed platform. The ability to perform field evaluation of Camelina is a useful, and in some studies essential benefit that allows researchers to evaluate how traits perform outside the strictly controlled conditions of a greenhouse.

Production of high levels of poly-3-hydroxybutyrate in plastids of Camelina sativa seeds.

Poly-3-hydroxybutyrate (PHB) production in plastids of Camelina sativa seeds was investigated by comparing levels of polymer produced upon transformation of plants with five different binary vectors containing combinations of five seed-specific promoters for expression of transgenes. Genes encoding PHB biosynthetic enzymes were modified at the N-terminus to encode a plastid targeting signal. PHB levels of up to 15% of the mature seed weight were measured in single sacrificed T1 seeds with a genetic construct containing the oleosin and glycinin promoters.

Parental assessment of pain coping in individuals with intellectual and developmental disabilities.

Pain coping is thought to be the most significant behavioural contribution to the adjustment to pain. Little is known about how those with intellectual and developmental disabilities (IDD) cope with pain. We describe parental reported coping styles and how coping relates to individual factors.

Assessing pain in children with intellectual disabilities.

Children with intellectual and developmental disabilities suffer more often from pain than their typically developing peers. Their pain can be difficult to manage, and assessment is often complicated by their limited communication skills, multiple complex pain problems and the presence of maladaptive behaviours. However, current research does provide some guidance for assessing their pain.