This is the first pilot study to examine the Australian adaptation of the Strengthening Families Program (SFP), a manualised family intervention recommended internationally in evidence reviews to ...improve family functioning and child mental health. The study compared two versions (8 vs. 14-session) and longitudinally evaluated outcomes for child emotional and behavioural difficulties, and parental psychological distress.
Fifty-eight families from disadvantaged primary schools in regional Victoria with children 8-12-years (80.6% of initially enrolled families, 62 parents and 74 children) completed the program and evaluation measures. Measures were repeated at pre-, post-, and 3-month follow-up and included the Kessler 6, the Strengths and Difficulties Questionnaire, and subscales adapted from the Longitudinal Study of Australian Children and Communities that Care Youth Survey.
The program showed significant reductions in child difficulties and parental psychological distress from pre- to post-measurements that were sustained at follow-up. Reductions in parental psychological distress were significantly associated with reductions in child difficulties at follow-up. The 8- and 14-session formats were not found to be significantly different in reducing child difficulties or parental psychological distress. Effect sizes for the Australian version were similar to those reported in previous U.S. trials.
Findings support the feasibility and effectiveness of an Australian adaptation of the SFP. The current study is unique in identifying similar outcomes for shorter and longer versions of the intervention. It is recommended that the 8-session Australian version is examined in a larger randomised controlled trial where children present with behavioural and emotional problems.
Weight loss and anorexia are commonly reported symptoms in people with advanced cancer. Little is known about patient experience of these phenomena, in particular whether they find them of concern. ...In this study, the prevalence of weight loss and eating-related concern was evaluated in patients with advanced cancer receiving specialist palliative homecare. The survey was a component of a larger study exploring the potential for helping patients and their families live with weight loss and change in eating habits. Patients were under the care of two specialist palliative homecare teams in the south of England in 2003. The questionnaire was distributed to the total eligible caseload of 233 patients with advanced cancer. The response rate was 85%. More than three-quarters of the 199 patients who returned the questionnaires reported weight loss (79%) and/or eating less (76%). Excluding the 32 patients (16%) who had sought help from a family member or friend to complete the questionnaires, more than half (52%) reported concern about weight loss and/or eating. Concern about weight loss or eating was found irrespective of proximity to death. Weight loss and eating-related concerns are commonly experienced by people with advanced cancer receiving palliative homecare. Further work is needed to establish if concerns are amenable to interventions that translate into meaningful outcomes for patients and their families.
Intercellular communication via gap junctions is thought to play an important role in embryonic cell survival and differentiation. Classical studies demonstrated both dye and electrical coupling of ...cells in the inner cell mass of mouse embryos, as well as the development of restrictions against coupling between cells of the inner cell mass and surrounding trophectoderm. Here we demonstrate extensive gap junctional communication between human embryonic stem (ES) cells, the pluripotent cells isolated from the inner cell mass of preimplantation blastocysts. Human ES cells maintained in vitro expressed RNA for 18 of the 20 known connexins; only connexin 40.1 (Cx40.1) and Cx50 were not detected by reverse transcription-polymerase chain reaction. Cx40, Cx43, and Cx45 were visualized by immunofluorescence at points of contact between adjacent cells. Electron microscopy confirmed that neighboring cells formed zones of tight membrane apposition characteristic of gap junctions. Fluorescent dye injections demonstrated extensive coupling within human ES cell colonies growing on mouse embryonic fibroblast (MEF) feeder cells, whereas dye coupling between human ES cells and adjacent MEFs was extremely rare. Physiological recordings demonstrated electrical and dye coupling between human ES cells in feeder-free monolayers and between isolated human ES cell pairs. Octanol, 18-alpha-glycyrrhetinic acid, and arylaminobenzoates inhibited transjunctional currents. Dye uptake studies on human ES cell monolayers and recordings from solitary human ES cells gave evidence for the surface expression of connexon hemichannels. Human ES cells provide a unique system for the study of human connexin proteins and their potential functions in cellular differentiation and the maintenance of pluripotency.
Dialysis withdrawal (DW) in patients with ESRD is increasing in importance. This study assessed causes of death and risk factors for DW in Australia and New Zealand in the first year of dialysis.
...This retrospective observational cohort study included all adult Australians and New Zealanders beginning renal replacement therapy in 1999-2008.
A total of 24,884 patients with 10,073 deaths were included. Deaths from cardiac and social causes (predominantly DW) accounted for 38% and 28% of all deaths, respectively. Cumulative incidence of DW was 3.5% at 1 year (95% confidence interval CI, 3.3%-3.8%), 9.0% at 3 years (95% CI, 8.6%-9.4%), and 13.4% at 5 years (95% CI, 12.8%-13.9%). In multivariate analysis, predictors for DW in the first year were older age (subhazard ratio SHR, 1.70 per decade 95% CI, 1.59-1.83; P<0.001), late referral (SHR, 1.83 95% CI, 1.59-2.11; P<0.001), comorbid conditions (SHR, 1.33 per each additional comorbid condition 95% CI, 1.25-1.41; P<0.001), and diabetes (SHR, 1.16 95% CI, 1.00-1.34; P=0.05). Negative predictors for DW included male sex (SHR, 0.75 95% CI, 0.66-0.87; P<0.001), indigenous ethnicity (SHR, 0.74 95% CI, 0.58-0.95; P=0.02), other nonwhite race (SHR, 0.66 95% CI, 0.48-0.91; P=0.01), and peritoneal dialysis user (SHR, 0.59 95% CI, 0.49-0.72; P<0.001).
DW is common among dialysis patients in Australia and New Zealand. Risk factors include older age, female sex, white race, diabetes, higher comorbidity burden, hemodialysis user, and late referral to nephrologist.
Axon demyelination contributes to the loss of sensory and motor function following injury or disease in the central nervous system. Numerous reports have demonstrated that myelination can be achieved ...in neuron/oligodendrocyte co-cultures. However, the ability to selectively treat neuron or oligodendrocyte (OL) cell bodies in co-cultures improves the value of these systems when designing mechanism-based therapeutics. We have developed a microfluidic-based compartmentalized culture system to achieve segregation of neuron and OL cell bodies while simultaneously allowing the formation of myelin sheaths. Our microfluidic platform allows for a high replicate number, minimal leakage, and high flexibility. Using a custom built lid, fit with platinum electrodes for electrical stimulation (10-Hz pulses at a constant 3 V with ~190 kΩ impedance), we employed the microfluidic platform to achieve activity-dependent myelin segment formation. Electrical stimulation of dorsal root ganglia resulted in a fivefold increase in the number of myelinated segments/mm
2
when compared to unstimulated controls (19.6 ± 3.0 vs. 3.6 ± 2.3 MBP
+
segments/mm
2
). This work describes the modification of a microfluidic, multi-chamber system so that electrical stimulation can be used to achieve increased levels of myelination while maintaining control of the cell culture microenvironment.
ABSTRACT
Introduction: Outcomes sensitive to change over time in non‐ambulatory boys/men with Duchenne muscular dystrophy (DMD) are not well‐established. Methods: Subjects (n = 91; 16.8 ± 4.5 years ...old) were assessed at baseline and 6‐month intervals for 2 years. We analyzed all subjects using an intent‐to‐treat model and a subset of stronger subjects with Brooke Scale score ≤4, using repeated measures. Results: Eight patients (12–33 years old) died during the study. Sixty‐six completed 12‐month follow‐up, and 51 completed 24‐month follow‐up. Those taking corticosteroids performed better at baseline, but rates of decline were similar. Forced vital capacity percent predicted (FVC% predicted) declined significantly only after 2 years. However, Brooke and Egen Klassifikation (EK) Scale scores, elbow flexion, and grip strength declined significantly over both 1 and 2 years. Conclusion: Brooke and EK Scale scores, elbow flexion, and grip strength were outcomes most responsive to change. FVC% predicted was responsive to change over 2 years. Corticosteroids benefited non‐ambulatory DMD subjects but did not affect decline rates of measures tested here. Muscle Nerve 54: 681–689, 2016
Premise of research. Rain forest ecosystems globally are synonymous with biodiversity, yet these vegetation communities vary widely in structure and composition. Historical biogeography and ...environment are thought to have significantly impacted Australian rain forest diversity and composition. Rain forest in Australia is extensively fragmented, and detailed vegetation mapping is used as a tool for conservation and land management planning. In Queensland, a regional ecosystem (RE) vegetation classification scheme is used by land managers. However, there has been no assessment to date of how evenly rain forest RE types are conserved at a regional scale, whether those conserved are the best representatives of biodiversity, and whether there are significantly distinctive RE types that are relatively poorly conserved or not recognized for their significance to biodiversity conservation.
Methodology. Our phylogeny for South East Queensland (SEQ) rain forest species, which encompasses 95% of rain forest species based on a unified three-marker DNA barcode, along with species lists were used for assessment of both species richness and phylogenetic diversity of RE types. We mapped the distribution of the most diverse and the most distinctive rain forest ecosystem types.
Pivotal results. We found that there is enormous variation in phylogenetic diversity within and among rain forest RE types in SEQ, with some depauperate in species richness. We identified the rain forest RE types in SEQ containing the highest diversity as well as those significantly sampling a wide part of the SEQ rain forest species pool compared with other RE types with similar species richness.
Conclusions. This study confirms that different RE types are not equivalent and so should not be considered as such in any conservation planning processes. Conservation assessment of ecosystems needs to take into account distinctiveness and diversity among types in order to gain better representativeness and complementarity across all RE types.
Immunodysregulation, Polyendocrinopathy, Enteropathy, X‐linked (IPEX) syndrome is a rare, X‐linked recessive disease that affects regulatory T cells (Tregs) resulting in diarrhea, enteropathy, ...eczema, and insulin‐dependent diabetes mellitus. IPEX syndrome is caused by pathogenic alterations in FOXP3 located at Xp11.23. FOXP3 encodes a transcription factor that interacts with several partners, including NFAT and NF‐κB, and is necessary for the proper cellular differentiation of Tregs. Although variable, the vast majority of IPEX syndrome patients have onset of disease during infancy with severe enteropathy. Only five families with prenatal presentation of IPEX syndrome have been reported. Here, we present two additional prenatal onset cases with novel inherited frameshift pathogenic variants in FOXP3 that generate premature stop codons. Ultrasound findings in the first patient identified echogenic bowel, echogenic debris, scalp edema, and hydrops. In the second patient, ultrasound findings included polyhydramnios with echogenic debris, prominent fluid‐filled loops of bowel, and echogenic bowel. These cases further broaden the phenotypic spectrum of IPEX syndrome by describing previously unappreciated prenatal ultrasound findings associated with the disease.
Climate change and associated disturbances are expected to exacerbate forest root diseases because of altered distributions of existing and emerging forest pathogens and predisposition of trees due ...to climatic maladaptation and other disturbances. Predictions of suitable climate space (potential geographic distribution) for forest pathogens and host trees under contemporary and future climate scenarios will guide the selection of appropriate management practices by forest managers to minimize adverse impacts of forest disease within forest ecosystems. A native pathogen (
Armillaria solidipes
) that causes Armillaria root disease of conifers in North America is used to demonstrate bioclimatic models (maps) that predict suitable climate space for both pathogen and a primary host (
Pseudotsuga menziesii
, Douglas-fir) under contemporary and future climate scenarios. Armillaria root disease caused by
A. solidipes
is a primary cause of lost productivity and reduced carbon sequestration in coniferous forests of North America, and its impact is expected to increase under climate change due to tree maladaptation. Contemporary prediction models of suitable climate space were produced using Maximum Entropy algorithms that integrate climatic data with 382 georeferenced occurrence locations for DNA sequence-confirmed
A. solidipes
. A similar approach was used for visually identified
P. menziesii
from 11,826 georeferenced locations to predict its climatic requirements. From the contemporary models, data were extrapolated through future climate scenarios to forecast changes in geographic areas where native
A. solidipes
and
P. menziesii
will be climatically adapted. Armillaria root disease is expected to increase in geographic areas where predictions suggest
A
.
solidipes
is well adapted and
P. menziesii
is maladapted within its current range. By predicting areas at risk for Armillaria root disease, forest managers can deploy suitable strategies to reduce damage from the disease.
Nonsense mutations are usually predicted to function as null alleles due to premature termination of protein translation. However, nonsense mutations in the DMD gene, encoding the dystrophin protein, ...have been associated with both the severe Duchenne Muscular Dystrophy (DMD) and milder Becker Muscular Dystrophy (BMD) phenotypes. In a large survey, we identified 243 unique nonsense mutations in the DMD gene, and for 210 of these we could establish definitive phenotypes. We analyzed the reading frame predicted by exons flanking those in which nonsense mutations were found, and present evidence that nonsense mutations resulting in BMD likely do so by inducing exon skipping, confirming that exonic point mutations affecting exon definition have played a significant role in determining phenotype. We present a new model based on the combination of exon definition and intronic splicing regulatory elements for the selective association of BMD nonsense mutations with a subset of DMD exons prone to mutation-induced exon skipping. Hum Mutat 32:299-308, 2011.
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