Displaying publications 1 - 20 of 127 in total

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  1. Smith JD
    Math Biosci, 1998 Nov;153(2):151-61.
    PMID: 9825637
    The Gibbs canonical ensemble of statistical mechanics is used to describe the probability distribution of the age classes of mothers of new-borns in an age-structured population. The Malthusian parameter emerges as a Lagrange multiplier corresponding to a generation time constraint, while a new perturbation parameter appears as the Lagrange multiplier corresponding to a maternity constraint. Classical Lotka stability reduces to the unperturbed case of the more general canonical ensemble model. The model is used in a case study of the female (peninsular) Malaysian population of 1970. The Malthusian parameter and perturbation are calculated easily by linear regression. Use of the model identifies an anomaly in the population due to the effects of World War II.
  2. Smith JD
    J Math Biol, 2004 Jan;48(1):105-18.
    PMID: 14685774
    A canonical/lognormal model for human demography is established, specifying the net maternity function and the age distribution for mothers of new-borns using a single macroscopic parameter vector of dimension five. The age distribution of mothers is canonical, while the net maternity function normalizes to a lognormal density. Comparison of an actual population with the model serves to identify anomalies in the population which may be indicative of phase transitions or influences from levels outside the demographic. Tracking the time development of the parameter vector may be used to predict the future state of a population, or to interpolate for data missing from the record. In accordance with classical theoretical considerations of Backman, Prigogine, et al., it emerges that the logarithm of a mother's age is the most fundamental time variable for demographic purposes.
  3. Subramaniam V, Stewart MW, Smith JF
    J Pain Symptom Manage, 1999 May;17(5):376-83.
    PMID: 10355217
    To study the process of establishment, and evaluate the outcome of participation, in a self-help support group for people with chronic nonmalignant pain, members of a newly-established, consumer-led group participated in two interviews 5 months apart and a researcher observed group meetings. Participants reported significant benefits from participating in the group. They had a significant increase in functional ability and activity, and reported decreased recourse to health professionals, particularly family physicians. This study indicates that pain support groups can play a valuable role for people in pain, assisting with support and rehabilitation, and meeting needs that health professionals are often not appropriate or able to provide.
  4. Layton R, Smith J, Macdonald P, Letchumanan R, Keese P, Lema M
    PMID: 26301217 DOI: 10.3389/fbioe.2015.00110
    Risk assessment is a reasoned, structured approach to address uncertainty based on scientific and technical evidence. It forms the foundation for regulatory decision-making, which is bound by legislative and policy requirements, as well as the need for making timely decisions using available resources. In order to be most useful, environmental risk assessments (ERAs) for genetically modified (GM) crops should provide consistent, reliable, and transparent results across all types of GM crops, traits, and environments. The assessments must also separate essential information from scientific or agronomic data of marginal relevance or value for evaluating risk and complete the assessment in a timely fashion. Challenges in conducting ERAs differ across regulatory systems - examples are presented from Canada, Malaysia, and Argentina. One challenge faced across the globe is the conduct of risk assessments with limited resources. This challenge can be overcome by clarifying risk concepts, placing greater emphasis on data critical to assess environmental risk (for example, phenotypic and plant performance data rather than molecular data), and adapting advances in risk analysis from other relevant disciplines.
  5. Lee WS, Beattie RM, Meadows N, Walker-Smith JA
    J Paediatr Child Health, 1999 Dec;35(6):568-71.
    PMID: 10634985
    OBJECTIVES: To assess the clinical features, investigations and outcome of 69 children (40 males, 29 females) with gastro-oesophageal reflux (GOER) referred to a tertiary referral centre in paediatric gastroenterology.

    METHODS: A study of all patients with significant GOER seen at the Paediatric Gastroenterology Unit, Queen Elizabeth Hospital for Children, Hackney Road, London, between December 1994 and August 1995.

    RESULTS: The median age at referral was 16 months. Presenting symptoms were recurrent vomiting (72%), epigastric and abdominal pain (36%), feeding difficulties (29%), failure to thrive (28%) and irritability (19%). Continuous 24-h lower oesophageal pH studies performed in 57 children showed 20 (35%) had a reflux index of between 10% to 20%, 14 (25%) had a index > 20%, and six (11%) had a postprandial reflux index > 10%. Reflux was shown in 38 (62%) of 62 children who underwent barium studies. None had significant anatomical abnormalities, but in the 22 children who had a negative barium studies, six had severe reflux (reflux index > 20%). Upper gastrointestinal endoscopy performed in 47 children showed reflux oesophagitis in 29 (62%), oesophageal ulceration in three, and Barrett's oesophagus in one. All of the children were treated with standard medical therapy. Sixty-six per cent were able to discontinue medication within 12 months and remained well. Four children (6%) required Nissen's fundoplication for failure to respond to medical therapy.

    CONCLUSIONS: Most infants with GOER have an uncomplicated course. False negative results were noted in both pH monitoring and barium meal. Up to 80% of children, with therapy, will improve within 12 months.

  6. Linkie M, Guillera-Arroita G, Smith J, Rayan DM
    Integr Zool, 2010 Dec;5(4):342-350.
    PMID: 21392352 DOI: 10.1111/j.1749-4877.2010.00215.x
    With only 5% of the world's wild tigers (Panthera tigris Linnaeus, 1758) remaining since the last century, conservationists urgently need to know whether or not the management strategies currently being employed are effectively protecting these tigers. This knowledge is contingent on the ability to reliably monitor tiger populations, or subsets, over space and time. In the this paper, we focus on the 2 seminal methodologies (camera trap and occupancy surveys) that have enabled the monitoring of tiger populations with greater confidence. Specifically, we: (i) describe their statistical theory and application in the field; (ii) discuss issues associated with their survey designs and state variable modeling; and, (iii) discuss their future directions. These methods have had an unprecedented influence on increasing statistical rigor within tiger surveys and, also, surveys of other carnivore species. Nevertheless, only 2 published camera trap studies have gone beyond single baseline assessments and actually monitored population trends. For low density tiger populations (e.g. <1 adult tiger/100 km(2)) obtaining sufficient precision for state variable estimates from camera trapping remains a challenge because of insufficient detection probabilities and/or sample sizes. Occupancy surveys have overcome this problem by redefining the sampling unit (e.g. grid cells and not individual tigers). Current research is focusing on developing spatially explicit capture-mark-recapture models and estimating abundance indices from landscape-scale occupancy surveys, as well as the use of genetic information for identifying and monitoring tigers. The widespread application of these monitoring methods in the field now enables complementary studies on the impact of the different threats to tiger populations and their response to varying management intervention.
  7. Anuar A, Marwan NF, Smith J, Siriyanun S, Sharif A
    Environ Sci Pollut Res Int, 2022 Feb;29(9):13729-13741.
    PMID: 34599441 DOI: 10.1007/s11356-021-16470-1
    The aim of this paper is to examine immigration and environmental degradation using bibliometric analysis. This paper also analyzes sources of publication, authorship, citations, distributions publications and other bibliometric indicators. The study focuses on a total of 1372 articles published from 2000 to 2020. These articles were collected through an automated process from the Scopus database and later analyzed using techniques such as bibliometric indicators analysis, VOSviewer, and Perish or Publish. The research identified 991 articles from varieties of published sources. The topic of immigrants and environmental degradation has been an emerging topic since 1981. Starting in 2000, most of the scholars actively producing an articles pertinent to this topic. Most of the articles were published in journals, and English is the primary language of research. United States is the leading country in contributing the publications. Meanwhile, the most significant fields in which the sources were produced were environmental science, agricultural and biological sciences, arts and humanities and earth and planetary sciences. However, some limitations has been found. It has been suggested for future research, to lengthen this work to other databases, as well as bibliometric analyses of immigration and environmental degradation in developed and developing countries by adding a new keyword such as energy consumption and climate change. This paper aims to assess recent trends in the expansion of academic literature on immigration and environmental degradation using the bibliometric analysis method. Network visualization and bibliometric indicators are used in this paper to present the results.
  8. Mohammed S, Savage T, Smith J, Shepley MM, White RD
    J Perinatol, 2023 Dec;43(Suppl 1):40-44.
    PMID: 38086966 DOI: 10.1038/s41372-023-01794-2
    Design charettes have been utilized in architectural and design practice to generate innovative ideas. The Reimagining Workshop is a version that combines practical and blue-sky thinking to improve healthcare facility design. The workshop engages diverse stakeholders who follow a human-centered design framework. The Reimagining the Neonatal Intensive Care Unit workshop sought to generate ideas for the future, optimal NICU without specific site or client constraints. Key themes include family-centered care, technology-enabled care, neighborhood and village design and investing in the care team. Recommendations include a supportive physical environment, celebrating milestones, complementary and alternative medicine, enhancing the transition of care, aiding the transition period, and leveraging technology. The workshop showcased the potential for transformative change in NICU design and provided a roadmap for future advancements. These findings can inform regulatory standards for NICU design and drive improvements in family-centered care, patient experiences, and outcomes within the NICU environment.
  9. Sideek MA, Smith J, Menz C, Adams JRJ, Cowin AJ, Gibson MA
    Int J Mol Sci, 2017 Oct 09;18(10).
    PMID: 28991210 DOI: 10.3390/ijms18102114
    Latent transforming growth factor-β-1 binding protein-2 (LTBP-2) belongs to the LTBP-fibrillin superfamily of extracellular proteins. Unlike other LTBPs, LTBP-2 does not covalently bind transforming growth factor-β1 (TGF-β1) but appears to be implicated in the regulation of TGF-β1 bioactivity, although the mechanisms are largely unknown. In experiments originally designed to study the displacement of latent TGF-β1 complexes from matrix storage, we found that the addition of exogenous LTBP-2 to cultured human MSU-1.1 fibroblasts caused an increase in TGF-β1 levels in the medium. However, the TGF-β1 increase was due to an upregulation of TGF-β1 expression and secretion rather than a displacement of matrix-stored TGF-β1. The secreted TGF-β1 was mainly in an inactive form, and its concentration peaked around 15 h after addition of LTBP-2. Using a series of recombinant LTBP-2 fragments, the bioactivity was identified to a small region of LTBP-2 consisting of an 8-Cys motif flanked by four epidermal growth factor (EGF)-like repeats. The LTBP-2 stimulation of TGF-β expression involved the phosphorylation of both Akt and p38 mitogen-activated protein kinase (MAPK) signalling proteins, and specific inactivation of each protein individually blocked TGF-β1 increase. The search for the cell surface receptor mediating this LTBP-2 activity proved inconclusive. Inhibitory antibodies to integrins β1 and αVβ5 showed no reduction of LTBP-2 stimulation of TGF-β1. However, TGF-β1 upregulation was partially inhibited by anti-αVβ3 integrin antibodies, suggestive of a direct or indirect role for this integrin. Overall, the study indicates that LTBP-2 can directly upregulate cellular TGF-β1 expression and secretion by interaction with cells via a short central bioactive region. This may be significant in connective tissue disorders involving aberrant TGF-β1 signalling.
  10. Musa AF, Yasin MSM, Smith J, Yakub MA, Nordin RB
    Health Qual Life Outcomes, 2021 Feb 09;19(1):50.
    PMID: 33563262 DOI: 10.1186/s12955-020-01658-9
    BACKGROUND: The Short Form 36 (SF-36) is a scoring system comprising of 36 items categorized into eight constructs corresponding to patients' health-related quality of life. It has been used extensively in various countries on different sub-populations and used to indicate the health status and help to ascertain the effect of clinical interventions on the particular population.

    OBJECTIVE: To examine the psychometric properties of the Malay version of SF-36 (Malay SF-36) summated rating scales and validate the scale among post-coronary artery bypass grafting surgery (CABG) patients at the National Heart Institute (IJN), Kuala Lumpur.

    METHODS: Five hundred and nine post-CABG patients at the IJN, Malaysia completed the questionnaires between 1 July and 31 December 2017. Psychometric tests endorsed by the "International Quality of Life Assessment Project" were utilised.

    RESULTS: The data quality was excellent with a high questionnaire completion rate (100%). As hypothesized, the ordering of item means within scales was clustered. In unison, scaling assumptions were satisfied. Good discriminant validity was shown between subsets of patients with various levels of health status. Notwithstanding, there were probably translation issues of the Physical Functioning scale which showed small ceiling effects. We clearly observed high ceiling and floor effects in both Role Physical and Role Emotional scale most probably attributed to the dichotomous style of their choice of responses. Cronbach alpha values of the eight scales ranged from 0.73 to 0.90, showing good internal consistency reliability. Confirmatory Factor Analysis (CFA) confirmed the 8-factor solution and Composite Reliability revealed internal consistency reliability except for Vitality and Social Functioning. Based on the Average Variance Extracted (AVE), convergent validity was adequate except for two domains. Discriminant Validity is good for the eight constructs as the √AVE are generally higher than the correlation coefficients between the latent constructs.

    CONCLUSION: The scoring for the Malay SF-36 based on the summated ratings method was proven to be valid to be applied in our local clinical population. The CFA, fitness estimates, reliability and validity assessments suggest that the Malay version of SF36 is a valid and reliable instrument. However, further work is warranted to further refine the convergent validity and reliability of some scales.

  11. Ab Lah R, Smith J, Savins D, Dowell A, Bucher D, Benkendorff K
    Food Sci Nutr, 2017 Jan;5(1):14-30.
    PMID: 28070312 DOI: 10.1002/fsn3.360
    Turban snails (family Turbinidae) are gastropod molluscs that are harvested for human consumption yet little is known about the nutritional properties of these snails, particularly from Australian waters. This study compares the proximate composition (ash, moisture, protein, and lipid content), fatty acid profiles, mineral, and trace element content of three species of turbinid snails; Turbo militaris, Lunella undulata, and Lunella torquata from northern New South Wales, Australia. They were all found to have relatively high protein in their flesh (16.0% to 18.5% of the fresh weight). L. torquata had a significantly higher lipid content (8.5% w/w) than L. undulata (5.2% w/w), whereas T. militaris (5.6% w/w) was not significantly different to either. Analysis with gas chromatography showed there was no significant difference in monounsaturated fatty acid (MUFA) content, with an average of approximately 14% of the total fatty acids in all three species. However, saturated fatty acids (SFA) were significantly higher in T. militaris (41%), whereas polyunsaturated fatty acids (PUFA) were significantly higher in L. undulata (46%). The ratio of n-3/n-6 fatty acids ranged from 1.1 in T. militaris to 1.4 in L. torquata, which is good for human health and comparable to other high value gastropods. The results indicate that T. militaris, L. undulata, and L. torquata provide a good source of essential elements such as zinc, selenium, and iron. At the location studied, toxic metals and metalloids were below safe recommended standards for human consumption. Overall, this study confirms the suitability of turban snails as a nutritional food for human consumption.
  12. Azizam NA, Hussain M, Nauenberg E, Ang WC, Azzeri A, Smith J
    PLoS One, 2024;19(9):e0307234.
    PMID: 39240834 DOI: 10.1371/journal.pone.0307234
    OBJECTIVE: In Malaysia, there is now a dearth of recommendations pertaining to the priority of biologic treatments for the effective management of psoriasis, given the multitude of available therapeutic alternatives. Present analysis reports results of a cost-effectiveness model that determines the most optimal arrangement of biologic treatments, with a particular focus of adding biosimilars to the existing treatment pathway for psoriasis in Malaysia.

    METHODS: A Markov model was developed to compare the cost effectiveness of various biologic sequential treatments in a hypothetical cohort of moderate to severe psoriasis patient in Malaysia over a lifetime horizon. The model simulated the progression of patients through three lines of active biologic therapy, before transitioning to best supportive care. Costs and effects were discounted annually at a rate of 3%.

    RESULTS: First line secukinumab has produced lowest incremental cost effectiveness ratios (ICERs) when compared to first line systemic [ICERs value; US$152,474 (first set analysis) and US$110,572 (second set analysis)] and first line phototherapy [ICERs value; US$147,057 (first set analysis) and US$107,616 (second set analysis)]. However, these values were slightly higher than the Malaysian based threshold of three times gross domestic product per capita, US$104,337. A 40% reduction in the unit costs of reference biologics renders most of the evaluated treatment sequences cost-effective.

    CONCLUSION: Adding biosimilar to the current treatment sequence could achieve cost savings ranging from 4.3% to 10.8% without significant loss of effectiveness. Given the significant impact of comorbidities and the resulting decline in quality of life among individuals with psoriasis, it may be justifiable to establish a threshold of up to US$184,000 per quality-adjusted life year (QALY) for the provision of therapies in the context of Malaysia.

  13. Smalley S, Bruza-Augatis M, Colletti T, Heistermann P, Mahmud A, Song D, et al.
    J Physician Assist Educ, 2024 Mar 01;35(1):108-115.
    PMID: 38251929 DOI: 10.1097/JPA.0000000000000571
    Given the international growth of physician associate/physician assistant (PA) and PA-comparable professions, it is crucial to understand the educational curricula of PA/PA-comparable professions worldwide. This article provides an overview of the process and development of the PA/PA-comparable curricula mapping design applying an international framework. It uses an innovative web-based tool, the Learning Opportunities, Objectives, and Outcomes Platform (LOOOP), to map and evaluate course content, teaching strategies, assessment formats, and learning outcomes. It highlights the project's rationale, challenges, and future implications.
  14. Rajagopal BS, Yates N, Smith J, Paradisi A, Tétard-Jones C, Willats WGT, et al.
    IUCrJ, 2024 Mar 01;11(Pt 2):260-274.
    PMID: 38446458 DOI: 10.1107/S2052252524001386
    The discovery of lytic polysaccharide monooxygenases (LPMOs), a family of copper-dependent enzymes that play a major role in polysaccharide degradation, has revealed the importance of oxidoreductases in the biological utilization of biomass. In fungi, a range of redox proteins have been implicated as working in harness with LPMOs to bring about polysaccharide oxidation. In bacteria, less is known about the interplay between redox proteins and LPMOs, or how the interaction between the two contributes to polysaccharide degradation. We therefore set out to characterize two previously unstudied proteins from the shipworm symbiont Teredinibacter turnerae that were initially identified by the presence of carbohydrate binding domains appended to uncharacterized domains with probable redox functions. Here, X-ray crystal structures of several domains from these proteins are presented together with initial efforts to characterize their functions. The analysis suggests that the target proteins are unlikely to function as LPMO electron donors, raising new questions as to the potential redox functions that these large extracellular multi-haem-containing c-type cytochromes may perform in these bacteria.
  15. Reijnders MRF, Ansor NM, Kousi M, Yue WW, Tan PL, Clarkson K, et al.
    Am J Hum Genet, 2017 Sep 07;101(3):466-477.
    PMID: 28886345 DOI: 10.1016/j.ajhg.2017.08.007
    RAC1 is a widely studied Rho GTPase, a class of molecules that modulate numerous cellular functions essential for normal development. RAC1 is highly conserved across species and is under strict mutational constraint. We report seven individuals with distinct de novo missense RAC1 mutations and varying degrees of developmental delay, brain malformations, and additional phenotypes. Four individuals, each harboring one of c.53G>A (p.Cys18Tyr), c.116A>G (p.Asn39Ser), c.218C>T (p.Pro73Leu), and c.470G>A (p.Cys157Tyr) variants, were microcephalic, with head circumferences between -2.5 to -5 SD. In contrast, two individuals with c.151G>A (p.Val51Met) and c.151G>C (p.Val51Leu) alleles were macrocephalic with head circumferences of +4.16 and +4.5 SD. One individual harboring a c.190T>G (p.Tyr64Asp) allele had head circumference in the normal range. Collectively, we observed an extraordinary spread of ∼10 SD of head circumferences orchestrated by distinct mutations in the same gene. In silico modeling, mouse fibroblasts spreading assays, and in vivo overexpression assays using zebrafish as a surrogate model demonstrated that the p.Cys18Tyr and p.Asn39Ser RAC1 variants function as dominant-negative alleles and result in microcephaly, reduced neuronal proliferation, and cerebellar abnormalities in vivo. Conversely, the p.Tyr64Asp substitution is constitutively active. The remaining mutations are probably weakly dominant negative or their effects are context dependent. These findings highlight the importance of RAC1 in neuronal development. Along with TRIO and HACE1, a sub-category of rare developmental disorders is emerging with RAC1 as the central player. We show that ultra-rare disorders caused by private, non-recurrent missense mutations that result in varying phenotypes are challenging to dissect, but can be delineated through focused international collaboration.
  16. Shepherdson JL, Hutchison K, Don DW, McGillivray G, Choi TI, Allan CA, et al.
    Am J Hum Genet, 2024 Mar 07;111(3):487-508.
    PMID: 38325380 DOI: 10.1016/j.ajhg.2024.01.007
    Pathogenic variants in multiple genes on the X chromosome have been implicated in syndromic and non-syndromic intellectual disability disorders. ZFX on Xp22.11 encodes a transcription factor that has been linked to diverse processes including oncogenesis and development, but germline variants have not been characterized in association with disease. Here, we present clinical and molecular characterization of 18 individuals with germline ZFX variants. Exome or genome sequencing revealed 11 variants in 18 subjects (14 males and 4 females) from 16 unrelated families. Four missense variants were identified in 11 subjects, with seven truncation variants in the remaining individuals. Clinical findings included developmental delay/intellectual disability, behavioral abnormalities, hypotonia, and congenital anomalies. Overlapping and recurrent facial features were identified in all subjects, including thickening and medial broadening of eyebrows, variations in the shape of the face, external eye abnormalities, smooth and/or long philtrum, and ear abnormalities. Hyperparathyroidism was found in four families with missense variants, and enrichment of different tumor types was observed. In molecular studies, DNA-binding domain variants elicited differential expression of a small set of target genes relative to wild-type ZFX in cultured cells, suggesting a gain or loss of transcriptional activity. Additionally, a zebrafish model of ZFX loss displayed an altered behavioral phenotype, providing additional evidence for the functional significance of ZFX. Our clinical and experimental data support that variants in ZFX are associated with an X-linked intellectual disability syndrome characterized by a recurrent facial gestalt, neurocognitive and behavioral abnormalities, and an increased risk for congenital anomalies and hyperparathyroidism.
  17. Agrawal R, Agarwal A, Jabs DA, Kee A, Testi I, Mahajan S, et al.
    Ocul Immunol Inflamm, 2019 Dec 10.
    PMID: 31821096 DOI: 10.1080/09273948.2019.1653933
    Purpose: To standardize a nomenclature system for defining clinical phenotypes, and outcome measures for reporting clinical and research data in patients with ocular tuberculosis (OTB).Methods: Uveitis experts initially administered and further deliberated the survey in an open meeting to determine and propose the preferred nomenclature for terms related to the OTB, terms describing the clinical phenotypes and treatment and reporting outcomes.Results: The group of experts reached a consensus on terming uveitis attributable to tuberculosis (TB) as tubercular uveitis. The working group introduced a SUN-compatible nomenclature that also defines disease "remission" and "cure", both of which are relevant for reporting treatment outcomes.Conclusion: A consensus nomenclature system has been adopted by a large group of international uveitis experts for OTB. The working group recommends the use of standardized nomenclature to prevent ambiguity in communication and to achieve the goal of spreading awareness of this blinding uveitis entity.
  18. Zheleva B, Verstappen A, Overman DM, Ahmad F, Ali SKM, Al Halees ZY, et al.
    Cardiol Young, 2023 Aug;33(8):1277-1287.
    PMID: 37615116 DOI: 10.1017/S1047951123002688
    The Eighth World Congress of Pediatric Cardiology and Cardiac Surgery (WCPCCS) will be held in Washington DC, USA, from Saturday, 26 August, 2023 to Friday, 1 September, 2023, inclusive. The Eighth World Congress of Pediatric Cardiology and Cardiac Surgery will be the largest and most comprehensive scientific meeting dedicated to paediatric and congenital cardiac care ever held. At the time of the writing of this manuscript, The Eighth World Congress of Pediatric Cardiology and Cardiac Surgery has 5,037 registered attendees (and rising) from 117 countries, a truly diverse and international faculty of over 925 individuals from 89 countries, over 2,000 individual abstracts and poster presenters from 101 countries, and a Best Abstract Competition featuring 153 oral abstracts from 34 countries. For information about the Eighth World Congress of Pediatric Cardiology and Cardiac Surgery, please visit the following website: [www.WCPCCS2023.org]. The purpose of this manuscript is to review the activities related to global health and advocacy that will occur at the Eighth World Congress of Pediatric Cardiology and Cardiac Surgery.Acknowledging the need for urgent change, we wanted to take the opportunity to bring a common voice to the global community and issue the Washington DC WCPCCS Call to Action on Addressing the Global Burden of Pediatric and Congenital Heart Diseases. A copy of this Washington DC WCPCCS Call to Action is provided in the Appendix of this manuscript. This Washington DC WCPCCS Call to Action is an initiative aimed at increasing awareness of the global burden, promoting the development of sustainable care systems, and improving access to high quality and equitable healthcare for children with heart disease as well as adults with congenital heart disease worldwide.
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