Laboratory-based measures of executive function and daily living skills in young children with Down syndrome: informing future interventions.

BACKGROUND: Adaptive behaviour refers to the practical skills necessary for independence and is considered a high-priority intervention target for children with neurogenetic conditions associated with intellectual disability, like Down syndrome (DS). Daily living skills (DLS) are a critical aspect of adaptive behaviour, but they have received little intervention attention, possibly because they involve a wide variety of skills across many settings. The present study aimed to advance DLS intervention science by examining the concurrent and longitudinal association between DLS performances and a cognitive skillset hypothesised to support DLS skill acquisition, executive function (EF). METHODS: Participants were 71 children with DS between the ages of 2.5 and 8.7 years (M = 5.23 years; standard deviation = 1.65) who completed a battery of adapted EF tasks and a primary caregiver who completed the Vineland Adaptive Behavior Scales 3rd Edition Parent/Caregiver Comprehensive Report Form. A subset of caregivers also provided 6- and 12-month follow-up adaptive behaviour information. RESULTS: Results demonstrated a positive association between EF task performance and DLS standard scores and v-scores both concurrently and longitudinally. CONCLUSIONS: The findings have implications for potential future intervention approaches that aim to strengthen DLS performances by advancing EF skills in this population.

Genetic evaluation of living kidney donor candidates: A review and recommendations for best practices.

The growing accessibility and falling costs of genetic sequencing techniques has expanded the utilization of genetic testing in clinical practice. For living kidney donation, genetic evaluation has been increasingly used to identify genetic kidney disease in potential candidates, especially in those of younger ages. However, genetic testing on asymptomatic living kidney donors remains fraught with many challenges and uncertainties. Not all transplant practitioners are aware of the limitations of genetic testing, are comfortable with selecting testing methods, comprehending test results, or providing counsel, and many do not have access to a renal genetic counselor or a clinical geneticist. Although genetic testing can be a valuable tool in living kidney donor evaluation, its overall benefit in donor evaluation has not been demonstrated and it can also lead to confusion, inappropriate donor exclusion, or misleading reassurance. Until more published data become available, this practice resource should provide guidance for centers and transplant practitioners on the responsible use of genetic testing in the evaluation of living kidney donor candidates.

Microresonator-based spectral translation of a gain-switched semiconductor laser comb.

Gain-switched semiconductor laser technology provides a simple and low-cost method to generate optical frequency combs. However, the spectral coverage of these compact comb sources has been limited to the near-infrared range. Here, we combine a gain-switched laser comb with a continuous-wave translation laser within a periodically poled lithium niobate microresonator and demonstrate efficient and broadband sum-frequency conversion, spectrally translating the near-infrared comb to the visible domain. The broadband nature of the nonlinear conversion arises from a chirping of the domain inversion grating period along the microresonator circumference. We also validate the coherence of the visible-wavelength comb teeth which underlines the general applicability of this spectral translation approach.

Spatiotemporal analysis of the effect of global development indicators on child mortality.

BACKGROUND: Child mortality continue to be a major public health issue in most developing countries; albeit there has been a decline in global under-five deaths. The differences in child mortality can best be explained by socioeconomic and environmental inequalities among countries. In this study, we explore the effect of country-level development indicators on under-five mortality rates. Specifically, we examine potential spatio-temporal heterogeneity in the association between major world development indicators on under-five mortality, as well as, visualize the global differential time trend of under-five mortality rates. METHODS: The data from 195 countries were curated from the World Bank's World Development Indicators (WDI) spanning from 2000 to 2017 and national estimates for under-five mortality from the UN Inter-agency Group for Child Mortality Estimation (UN IGME).We built parametric and non-parametric Bayesian space-time interaction models to examine the effect of development indicators on under-five mortality rates. We also used employed Bayesian spatio-temporal varying coefficient models to assess the spatial and temporal variations in the effect of development indicators on under-five mortality rates. RESULTS: In both parametric and non-parametric models, the results show indicators of good socioeconomic development were associated with a reduction in under-five mortality rates while poor indicators were associated with an increase in under-five mortality rates. For instance, the parametric model shows that gross domestic product (GDP) (ß = - 1.26, [CI - 1.51; - 1.01]), current healthcare expenditure (ß = - 0.40, [CI - 0.55; - 0.26]) and access to basic sanitation (ß = - 0.03, [CI - 0.05; - 0.01]) were associated with a reduction under-five mortality. An increase in the proportion practising open defecation (ß = 0.14, [CI 0.08; 0.20]) an increase under-five mortality rate. The result of the spatial components spatial variation in the effect of the development indicators on under-five mortality rates. The spatial patterns of the effect also change over time for some indicators, such as PM2.5. CONCLUSION: The findings show that the burden of under-five mortality rates was considerably higher among sub-Saharan African countries and some southern Asian countries. The findings also reveal the trend in reduction in the sub-Saharan African region has been slower than the global trend.

Early developmental profiles among infants with Down syndrome.

BACKGROUND: Down syndrome (DS) generally predisposes children to a pattern of relative developmental strengths and challenges, but within-syndrome heterogeneity is also commonly observed across many dimensions. The present research examines whether heterogeneity in developmental presentation can be detected during infancy in DS and whether factors associated with differing profiles can be identified. METHODS: Infants with DS (n = 75; age range: 3.9-17.6 months) were administered the Bayley Scales of Infant Development III (Bayley-III). A primary caregiver provided information regarding developmental history and family demographics. Latent profile analysis was conducted to identify whether early profiles were present across the five Bayley-III domains. RESULTS: Three developmental profiles were observable within the sample: a 'Mild Delay' Profile, an 'Moderate Delay' Profile and a 'Pronounced Delay' Profile. In addition, chronological age, having received heart surgery and having received occupational therapy were associated with probability of profile membership. CONCLUSIONS: Findings from this study contribute to the growing knowledgebase regarding heterogenous presentations associated with DS and can inform early intervention planning.

Evaluating an adapted reverse categorisation task to assess cognitive flexibility in young children with Down syndrome.

BACKGROUND: Accurate measurement of cognitive skills is necessary to advance both developmental and intervention science for individuals with Down syndrome (DS). This study evaluated the feasibility, developmental sensitivity and preliminary reliability of a reverse categorisation measure designed to assess cognitive flexibility in young children with DS. METHODS: Seventy-two children with DS ages 2.5-8 years completed an adapted version of a reverse categorisation task. Twenty-eight of the participants were assessed again 2 weeks later for retest reliability. RESULTS: This adapted measure demonstrated adequate feasibility and developmental sensitivity, and preliminary evidence for test-retest reliability when administered to children with DS in this age range. CONCLUSIONS: This adapted reverse categorisation measure may be useful for future developmental and treatment studies that target early foundations of cognitive flexibility in young children with DS. Additional recommendations for use of this measure are discussed.

Effect of age and rural residency on perceptions about SARS-CoV-2 pandemic and vaccination in kidney transplant recipients.

BACKGROUND: Transplant patients have poor outcomes in coronavirus-disease 2019 (COVID-19). The pandemic's effects on rural patients' overall care experience, attitudes to telemedicine, and vaccination are poorly understood. METHODS: We administered a cross-sectional survey to adult kidney transplant recipients in central Pennsylvania across four clinical sites between March 29, 2021 and June 2, 2021. We assessed the pandemic's impact on care access, telemedicine experience, attitudes toward preventive measures, vaccination, and variation by sociodemographic variables. RESULTS: Survey completion rate was 51% (303/594). Of these, 52.8% were rural residents. The most common impact was use of telemedicine (79.2%). Predominant barriers to telemedicine were lack of video devices (10.9%), perceived complexity (5.6%), and technical issues (5.3%). On a 0-10 Likert scale, the mean positive impression for telemedicine was 7.7; lower for patients with telephone-only versus video visits (7.0 vs. 8.2; p < .001), and age ≥60 years (7.4 vs. 8.1; p = .01) on univariate analyses. Time/travel savings were commonly identified (115/241, 47.7%) best parts of telemedicine and lack of personal connection (70/166, 42.2%) the worst. Only 68.9% had received any dose of COVID vaccination. The vaccinated group members were older (58.4 vs. 53.5 years; p = .007), and less likely rural (47.8% vs. 65.2%; p = .005). Common themes associated with vaccine hesitancy included concerns about safety (27/59, 46%), perceived lack of data (19/59, 32%), and distrust (17/59, 29%). At least one misconception about the vaccines or COVID-19 was quoted by 29% of vaccine-hesitant patients. CONCLUSIONS: Among respondents, the pandemic significantly impacted healthcare experience, especially in older patients in underserved communities. COVID-19 vaccination rate was relatively low, driven by misconceptions and lack of trust.

Infant precursors of executive function in Down syndrome.

BACKGROUND: Although early features of infant cognition are predictive of executive function (EF) in typically developing (TD) children, there is little information regarding the developmental origins of EF in neurogenetic conditions, such as Down syndrome (DS). METHODS: The current study compared the performance of infants with and without DS on three dimensions that are hypothesised EF precursors: visual engagement, attention shifting and action planning. Additionally, the relationship between these EF precursors at Time 1 and EF performance at Time 2 (6 months later) was examined in the DS group. Participants were 36 infants with DS, M chronological age = 12.65 months, SD = 2.11; M developmental age = 8.84 months, SD = 2.22, and 36 TD infants, M chronological age = 8.62, SD = 3.06; M developmental age = 8.64 months, SD = 3.40. RESULTS: Infants with DS visually engaged with objects for longer durations and demonstrated challenges with action planning compared with TD infants at Time 1. Attention shifting at Time 1 significantly predicted EF performance at Time 2 in the DS group. CONCLUSIONS: This study provides evidence that an early atypical presentation of EF precursors is detectable during infancy in DS and is predictive of subsequent EF performance. These findings contribute to the identification of areas of early cognitive risk in DS and can inform future interventions in this population.

Latent profiles of autism symptoms in children and adolescents with Down syndrome.

BACKGROUND: Down syndrome (DS) is associated with elevated rates of autism spectrum disorder (ASD) and autism symptomatology. To better characterise heterogeneity in ASD symptomatology in DS, profiles of caregiver-reported ASD symptoms were modelled for children and adolescents with DS. METHODS: Participants (n = 125) were recruited through several multi-site research studies on cognition and language in DS. Using the Social Responsiveness Scale-2 (SRS-2; Constantino and Gruber 2012), two latent profile analyses (LPA) were performed, one on the broad composite scores of social communication and interaction and restricted interests and repetitive behaviour, and a second on the four social dimensions of social communication, social motivation, social awareness, and social cognition. RESULTS: A three-profile model was the best fit for both analyses, with each analysis yielding a low ASD symptom profile, an elevated or mixed ASD symptom profile and a high ASD symptom profile. Associations were observed between profile probability scores and IQ, the number of co-occurring biomedical conditions reported, sex, and SRS-2 form. CONCLUSIONS: Characterising heterogeneity in ASD symptom profiles can inform more personalised supports in this population, and implications for potential therapeutic approaches for individuals with DS are discussed.

Perceptions of COVID-19 shocks and adoption of sustainable agricultural practices in Ghana.

Most studies on the novel COVID-19 pandemic have focused mainly on human health, food systems, and employment with limited studies on how farmers implement sustainable agricultural practices (SAPs) in response to the pandemic. This study examines how perceptions of COVID-19 shocks influence the adoption of SAPs among smallholder farmers in Ghana. We find that perceptions of COVID-19 shocks influence the probability and intensity of SAPs adoption. Secondly, households who anticipated COVID-19 shocks recorded heterogeneity effects in the combinations (complementarity and substitutability) of SAPs. Farmers who anticipated an increase in input prices and loss of income due to COVID-19 recorded the highest complementarity association between pesticide and zero tillage while farmers who expected limited market access reported the highest complementarity between mixed cropping and mulching. Farmers who projected a decrease in output prices complements pesticides with mixed cropping. The findings suggest that understanding the heterogeneity effects in the combinations of SAPs due to COVID-19 shocks is critical to effectively design, target and disseminate sustainable intensification programs in a post-pandemic period.

Absolute distance measurement with a gain-switched dual optical frequency comb.

The measurement of distance plays an important role in many aspects of modern societies. In this paper, an absolute distance measurement method for arbitrary distance is proposed and demonstrated using mode-resolved spectral interferometry with a gain-switched dual comb. An accuracy of 12 µm, when compared to a He-Ne fringe counting laser interferometer, for a displacement up to 2.5 m is demonstrated by tuning the repetition frequency of the dual comb from 1.1 GHz to 1.4 GHz. The compact measurement system based on a gain-switched dual comb breaks the constraint of periodic ambiguity. The simplification and improvements are significant for further industrial applications.

Feasibility of a syndrome-informed micro-intervention for infants with Down syndrome.

BACKGROUND: Infants with Down syndrome (DS) are at risk for a range of phenotypic outcomes, including delays in the onset of reaching behaviour, a critical skill that facilitates early learning. This parallel-group feasibility and pilot study presents findings from a parent-mediated micro-intervention that aimed to support the development of reaching behaviour in a sample of infants with DS. METHODS: Participants were 73 infants with DS and their caregivers. Infants who qualified for the home-based intervention (based on manual skill performance on Bayley Scales of Infant and Toddler Development, Third Edition items) were randomly assigned individually or by geographical region to a treatment or an alternative treatment condition that involved toy-based interactions with caregivers. Infants in the treatment condition experienced facilitated reaching during the toy-based interactions through the use of Velcro-affixed mittens and toys. RESULTS: Forty-two infants met criteria to participate in the intervention, and 37 participated in both baseline and post-treatment visits. At post-treatment, infants in the treatment condition demonstrated shorter latencies to make contact with objects and showed higher frequencies of reach attempts and swats at objects than infants in the alternative treatment group. These findings were more pronounced when examining a chronological age-restricted subgroup of infants 5 to 10 months. CONCLUSIONS: Findings suggest that a syndrome-informed approach to targeted intervention may be a promising application of phenotyping science in DS and other neurogenetic conditions associated with intellectual disability.

Climate-smart cowpea adoption and welfare effects of comprehensive agricultural training programs.

Agricultural training programs remain one of the primary mechanisms for disseminating modern and climate-smart technologies with the aim to improve the welfare outcomes of smallholder farmers. With persistent low agricultural productivity in Sub Saharan Africa (SSA), the content, effectiveness, and mode of delivery of training programs remain a debate. In this paper, we examine the adoption, productivity, and income effects of participating in a novel comprehensive agricultural training program (CATP) involving cowpea farmers in northern Ghana by using the endogenous switching regression (ESR) model. The CATP requires farmers to complete a set of modules on good agronomic practices to gain informal certification. The results indicate that participating in the CATP increases the adoption of climate-smart cowpea varieties, productivity, and cowpea income by 75, 15, and 24% points, respectively, compared to their mean levels. These positive welfare effects of participating in the CATPs confirm the need to increase capacity-enhancing activities in agricultural development projects, and design mechanisms to eliminate barriers to participation among rural farm households.

Predictors and Prevalence of Nodal Disease in Salvage Oropharyngectomy.

BACKGROUND: Patients with recurrent oropharyngeal cancer often require extensive salvage surgery. For patients with clinically N0 necks, the indication for concurrent neck dissection remains unclear. This study aimed to determine predictors, prevalence, and distribution of nodal disease in patients treated with salvage oropharyngectomy. METHODS: In a case series with data collection at a single tertiary academic National Cancer Institute (NCI)-designated comprehensive cancer center, this study analyzed patients treated with prior radiation or chemoradiation who had persistent, recurrent, or second primary squamous cell carcinoma of the oropharynx requiring oropharyngeal resection between 1998 and 2017 (n = 95). Clinical and oncologic characteristics and treatment outcomes were collected, and statistical analyses were performed. RESULTS: The overall rate of nodal positivity was 21% (24/95), and the rate of occult nodal disease was 6% (4/65). Ipsilateral and contralateral level 2 were the most common areas harboring positive nodes. Bivariate analysis showed female sex (p = 0.01), initial overall stage (p = 0.02), and N status (p = 0.03), as well as recurrent overall and T stage (p = 0.05) to be predictors of nodal disease. In the multivariate analysis, recurrent T stage continued to be significantly predictive of pathologic nodal disease. Both computed tomography (CT) and positron emission tomography-CT were moderately accurate in predicting nodal disease in the salvage setting (area under the curve, 0.79 and 0.80, respectively). CONCLUSION: Occult nodal disease is observed in few patients undergoing salvage oropharyngeal resection. This study identified factors predictive of nodal disease in patients undergoing salvage oropharyngectomy and appropriate diagnostic tests in this setting.

Integrated dual optical frequency comb source.

A monolithically integrated dual-channel optical frequency comb source is demonstrated in this paper. Three lasers are integrated on a single chip using a regrowth-free fabrication process in a master-slave-slave configuration. The master laser's power is split equally using a 1x2 multimode interference coupler and injection locks the two slave lasers. The slave lasers are gain-switched to produce dual optical frequency combs at 4.1 GHz and 5 GHz. To the best of our knowledge, this is the first demonstration of a dual optical frequency comb source with all light sources monolithically integrated in a photonic integrated circuit (PIC).

Active demultiplexer enabled mmW ARoF transmission of directly modulated 64-QAM UF-OFDM signals.

In this Letter, we experimentally demonstrate an unamplified analog RoF distribution of 60 GHz 5G signals. The system entails the heterodyning of two optical tones from an externally injected gain switched laser (EI-GSL) based optical frequency comb to generate a millimeter wave (mmW) signal. A fixed frequency separation and a high level of phase correlation, between the EI-GSL comb lines, results in the generation of a high-quality signal. An active demultiplexer is used to filter and amplify two comb tones, thus alleviating the need for an external optical amplifier to boost the low power comb tones. Furthermore, the same demultiplexer is also used to modulate one of the tones with a 64-QAM UF-OFDM signal. Such an approach enables the remote generation of a mmW downlink data signal as well as an unmodulated RF carrier that could be used to downconvert the mmW signals to an intermediate frequency. Using the abovementioned scheme, we demonstrate the distribution of the downlink signal over 25 km of fiber, achieving a BER of 2.4e-3 (below the HD-FEC limit of 3.8e-3) and only a 0.5 dB penalty at the FEC limit in comparison to the BtB case.

Characteristics associated with significantly worse quality of life in mycosis fungoides/Sézary syndrome from the Prospective Cutaneous Lymphoma International Prognostic Index (PROCLIPI) study.

BACKGROUND: Mycosis fungoides (MF) and Sézary Syndrome (SS) are the most common cutaneous T-cell lymphomas. MF/SS is accompanied by considerable morbidity from pain, itching and disfigurement. AIM: To identify factors associated with poorer health-related quality of life (HRQoL) in patients newly diagnosed with MF/SS. METHODS: Patients enrolled into Prospective Cutaneous Lymphoma International Prognostic Index (PROCLIPI; an international observational study in MF/SS) had their HRQoL assessed using the Skindex-29 questionnaire. Skindex-29 scores were analysed in relation to patient- and disease-specific characteristics. RESULTS: The study population consisted of 237 patients [60·3% male; median age 60 years, (interquartile range 49-70)], of whom 179 had early MF and 58 had advanced MF/SS. In univariate analysis, HRQoL, as measured by Skindex-29, was worse in women, SS, late-stage MF, those with elevated lactate dehydrogenase, alopecia, high modified Severity Weighted Assessment Tool and confluent erythema. Linear regression models only identified female gender (ß = 8·61; P = 0·003) and alopecia (ß = 9·71, P = 0·02) as independent predictors of worse global HRQoL. Item-level analysis showed that the severe impairment in symptoms [odds ratio (OR) 2·14, 95% confidence interval (CI) 1·19-3·89] and emotions (OR 1·88, 95% CI 1·09-3·27) subscale scores seen in women was caused by more burning/stinging, pruritus, irritation and greater feelings of depression, shame, embarrassment and annoyance with their diagnosis of MF/SS. CONCLUSIONS: HRQoL is significantly more impaired in newly diagnosed women with MF/SS and in those with alopecia. As Skindex-29 does not include existential questions on cancer, which may cause additional worry and distress, a comprehensive validated cutaneous T-cell lymphoma-specific questionnaire is urgently needed to more accurately assess disease-specific HRQoL in these patients. What's already known about this topic? Cross-sectional studies of mixed populations of known and newly diagnosed patients with mycosis fungoides (MF)/Sézary syndrome (SS) have shown significant impairment in health-related quality of life (HRQoL). Previous studies on assessing gender-specific differences in HRQoL in MF/SS are conflicting. More advanced-stage disease and pruritus is associated with poorer HRQoL in patients with MF/SS. What does this study add? This is the first prospective study to investigate HRQoL in a homogenous group of newly diagnosed patients with MF/SS. In patients newly diagnosed with MF/SS, HRQoL is worse in women and in those with alopecia and confluent erythema. MF/SS diagnosis has a multidimensional impact on patient HRQoL, including a large burden of cutaneous symptoms, as well as a negative impact on emotional well-being.

Rationale for the advancement of PI3K pathway inhibitors for personalized chordoma therapy.

PURPOSE: Chordomas are rare and serious tumors with few effective treatments outside of aggressive surgery and radiation. Targeted therapies may present a more effective option for a subset of patients with lesions possessing certain genetic biomarkers. METHODS: A small molecule inhibitor library was tested in patient-derived UM-Chor1 cells to identify targeted therapies with potential efficacy. Targeted exome sequencing of UM-Chor1 and UM-Chor2 cells was performed to investigate genetic aberrations in relevant pathways. Chordoma cell lines were treated with inhibitors of the phosphotidylinositol 3-kinase (PI3K), epidermal growth factor receptor (EGFR), and cyclin dependent kinase (CDK) pathways, and responses were determined using resazurin cell viability assays, Annexin V apoptosis assays, and western blotting. Pan-PI3K inhibitor BKM120 was also tested in five chordoma xenograft models. RESULTS: Unbiased small molecule profiling nominated PI3K-AKT-mTOR pathway inhibitors as a promising therapy in chordoma, and genetic analyses of UM-Chor1 and UM-Chor2 cell lines revealed aberrations in PTEN, EGFR, and CDKN2A. Treatment of UM-Chor1 and UM-Chor2 with targeted PI3K, EGFR, and CDK inhibitors inhibited growth and proliferation and induced apoptosis more robustly than imatinib, a currently used chordoma therapy. Furthermore, BKM120 significantly inhibited tumor growth in a subset of the xenograft models tested. CONCLUSION: Targeted therapies, especially those inhibiting PI3K, display promising effects in multiple chordoma cell line and xenograft models. Nevertheless, the limited effects of PI3K, EGFR, and CDK targeting agents in other models reveal the presence of resistance mechanisms, which motivates future research to both identify biomarkers of response and develop combination therapies.

Differing Resuscitation With Aortic Occlusion in a Swine Junctional Hemorrhage Polytrauma Model.

BACKGROUND: Resuscitative endovascular balloon occlusion of the aorta (REBOA) and Abdominal Aortic and Junctional Tourniquet (AAJT) have received much attention in recent as methods for temporary control of junctional hemorrhage. Previous studies typically used the animal's shed blood for resuscitation. With current interest in moving REBOA to prehospital environment, this study aimed to evaluate the hemodynamic and metabolic responses to different resuscitation fluids used with these devices. METHODS: In swine (Sus scrofa), shock was induced using a controlled hemorrhage, femur fracture, and uncontrolled hemorrhage from the femoral artery. Infrarenal REBOA or AAJT was deployed for 60 min during which the arterial injury was repaired. Animals were resuscitated with 15 mL/kg of shed whole blood (SWB) or fresh frozen plasma (FFP) or 30 mL/kg of a balanced crystalloid (PlasmaLyte). RESULTS: Animals in the AAJT and REBOA groups did not show any measurable differences in hemodynamics, metabolic responses, or survival with AAJT or REBOA treatment; hence, the data are pooled and analyzed among the three resuscitative fluids. SWB, FFP, and PlasmaLyte groups did not have a difference in survival time or overall survival. The animals in the SWB and FFP groups maintained higher blood pressure after resuscitation, (P < 0.001) and required significantly less norepinephrine to maintain blood pressure than those in the PlasmaLyte group (P < 0.001). The PlasmaLyte resuscitation prolonged prothrombin time and decreased thromboelastography maximum amplitude. CONCLUSIONS: After 60 min, infrarenal REBOA or AAJT aortic occlusion SWB and FFP resuscitation provided better blood pressure support with half of the resuscitative volume of PlasmaLyte. Swine resuscitated with SWB and FFP also had a more favorable coagulation profile. These data suggest that whole blood or component therapy should be used for resuscitation in conjunction with REBOA or AAJT, and administration of these fluids should be considered if prehospital device use is pursued.

Goal-directed action planning in infants with Down syndrome.

BACKGROUND: Down syndrome (DS) is a neurogenetic disorder associated with risk for executive dysfunction, or difficulties with the cognitive processes required for planning volitional, goal-directed behaviour. This study examines the developmental origins of difficulties with goal-directed action planning in infants with DS to inform our understanding of the cognitive phenotype associated with DS and its implications for intervention. METHOD: First, the study compared the performance of infants with DS (n = 44, mean chronological age = 7.5 months, SD = 2.3) and typically developing infants (n = 31, mean chronological age = 7.5 months, SD = 2.9) on plan production and planning efficiency during an early planning task. Next, potential sources of variability in planning behaviour (motor performance and sensory processing) within the DS sample were examined. All infants completed an early planning laboratory task and the Bayley Scales of Infant Development-III Cognitive Scale. The motor and sensory skills of infants with DS were assessed by the motor scales of the Bayley Scales of Infant Development-III and the Infant Sensory Profile-2. DS-related biomedical history information was provided by caregivers for the infants with DS. RESULTS: Between-group differences in planning were observed on the dimensions of strategy production and completion, such that infants with DS were less efficient in their strategy execution than typically developing infants. In the DS group, motor skills and sensory processing were associated with planning efficiency on all components of the early planning task. CONCLUSIONS: Less efficient action planning in infants with DS may disrupt the shaping of goal-directed behaviour, and the identification of early risk factors associated with planning efficiency has important implications for early intervention.