Objectives - This report presents the development, plan, and operation of the 2009-2010 National Survey of Children with Special Health Care Needs, a module of the State and Local Area Integrated Telephone Survey. The survey is conducted by the Centers for Disease Control and Prevention's National Center for Health Statistics. This survey was designed to produce national and state-specific prevalence estimates of children with special health care needs (CSHCN), to describe the types of services that they need and use, and to assess aspects of the system of care for CSHCN. Methods - A random-digit-dial sample of households with children under age 18 years was constructed for each of the 50 states and the District of Columbia. The sampling frame consisted of landline phone numbers and cellular(cell) phone numbers of households that reported a cell-phone-only or cell-phone-mainly status. Children in identified households were screened for special health care needs. If CSHCN were identified in the household, a detailed interview was conducted for one randomly selected child with special health care needs. Respondents were parents or guardians who knew about the children's health and health care. Results - A total of 196,159 household screening interviews were completed from July 2009 through March 2011, resulting in 40,242 completed special-needs interviews, including 2,991 from cell-phone interviews. The weighted overall response rate was 43.7% for the landline sample, 15.2% for the cell-phone sample, and 25.5% overall.
Chronic myeloid leukemia (CML) stem cells are not dependent on BCR-ABL kinase for their survival, suggesting that kinase-independent mechanisms must contribute to their persistence. We observed that CML stem/progenitor cells (SPCs) produce tumor necrosis factor-? (TNF-?) in a kinase-independent fashion and at higher levels relative to their normal counterparts. We therefore investigated the role of TNF-? and found that it supports survival of CML SPCs by promoting nuclear factor ?B/p65 pathway activity and expression of the interleukin 3 and granulocyte/macrophage-colony stimulating factor common ?-chain receptor. Furthermore, we demonstrate that in CML SPCs, inhibition of autocrine TNF-? signaling via a small-molecule TNF-? inhibitor induces apoptosis. Moreover TNF-? inhibition combined with nilotinib induces significantly more apoptosis relative to either treatment alone and a reduction in the absolute number of primitive quiescent CML stem cells. These results highlight a novel survival mechanism of CML SPCs and suggest a new putative therapeutic target for their eradication.
In childhood B-cell precursor acute lymphoblastic leukemia, cytogenetics is important in diagnosis and as an indicator of response to therapy, thus playing a key role in risk stratification of patients for treatment. Little is known of the relationship between different cytogenetic subtypes in B-cell precursor acute lymphoblastic leukemia and the recently reported copy number abnormalities affecting significant leukemia associated genes. In a consecutive series of 1427 childhood B-cell precursor acute lymphoblastic leukemia patients, we have determined the incidence and type of copy number abnormalities using multiplex ligation-dependent probe amplification. We have shown strong links between certain deletions and cytogenetic subtypes, including the novel association between RB1 deletions and intrachromosomal amplification of chromosome 21. In this study, we characterized the different copy number abnormalities and show heterogeneity of PAX5 and IKZF1 deletions and the recurrent nature of RB1 deletions. Whole gene losses are often indicative of larger deletions, visible by conventional cytogenetics. An increased number of copy number abnormalities is associated with NCI high risk, specifically deletions of IKZF1 and CDKN2A/B, which occur more frequently among these patients. IKZF1 deletions and rearrangements of CRLF2 among patients with undefined karyotypes may point to the poor risk BCR-ABL1-like group. In conclusion, this study has demonstrated in a large representative cohort of children with B-cell precursor acute lymphoblastic leukemia that the pattern of copy number abnormalities is highly variable according to the primary genetic abnormality.
The Laurentian Great Lakes region of North America contains substantial aquatic resources and mercury-contaminated landscapes, fish, and wildlife. This special issue emanated from a bi-national synthesis of data from monitoring programs and case studies of mercury in the region, here defined as including the Great Lakes, the eight U.S. states bordering the Great Lakes, the province of Ontario, and Lake Champlain. We provide a retrospective overview of the regional mercury problem and summarize new findings from the synthesis papers and case studies that follow. Papers in this issue examine the chronology of mercury accumulation in lakes, the importance of wet and dry atmospheric deposition and evasion to regional mercury budgets, the influence of land-water linkages on mercury contamination of surface waters, the bioaccumulation of methylmercury in aquatic foods webs; and ecological and health risks associated with methylmercury in a regionally important prey fish.
This special issue examines bioaccumulation and risks of methylmercury in food webs, fish and wildlife in the Laurentian Great Lakes region of North America, and explores mercury policy in the region and elsewhere in the United States and Canada. A total of 35 papers emanated from a bi-national synthesis of multi-media data from monitoring programs and research investigations on mercury in aquatic and terrestrial biota, a 3-year effort involving more than 170 scientists and decision-makers from 55 different universities, non-governmental organizations, and governmental agencies. Over 290,000 fish mercury data points were compiled from monitoring programs and research investigations. The findings from this scientific synthesis indicate that (1) mercury remains a pollutant of major concern in the Great Lakes region, (2) that the scope and intensity of the problem is greater than previously recognized and (3) that after decades of declining mercury levels in fish and wildlife concentrations are now increasing in some species and areas. While the reasons behind these shifting trends require further study, they also underscore the need to identify information gaps and expand monitoring efforts to better track progress. This will be particularly important as new pollution prevention measures are implemented, as global sources increase, and as the region faces changing environmental conditions.
The Clean Air Regulatory Agenda (CARA) Mercury Science Program was developed to provide scientific information to support regulatory activities and accountability pertaining to atmospheric emissions of mercury in Canada. The first phase of the science program, entitled "Setting-the-Baseline", sought to achieve the following: identify key indicators of the state-of-the-Canadian environment with respect to the transport, fate and effects of mercury; define these indicators; and, understand the processes that relate these indicators to anthropogenic emissions of mercury. To achieve these outcomes, a consultative process was used to identify the scientific needs of the agenda for mercury; understand Canadas scientific capacity; and, develop a plan to fulfill these scientific needs. The science plan that emerged from this process was structured around the themes of atmospheric monitoring, landscape-based risk assessment, ecological risk assessment, ecosystem modeling, and trends. Implementation of the science plan necessitated a multi-disciplinary and extensively partnered program. To date, the CARA Mercury Science Program is producing coordinated science at the national-scale that aims to directly assess the effectiveness of the CARA for mercury and for many of Canadas other mercury-related policies.
Intrachromosomal amplification of chromosome 21 (iAMP21) defines a distinct subgroup of childhood B-cell precursor acute lymphoblastic leukemia (BCP-ALL) that has a dismal outcome when treated with standard therapy. For improved diagnosis and risk stratification, the initiating genetic events need to be elucidated. To investigate the genetic basis of BCP-ALL, genomes of 94 iAMP21 patients were interrogated by arrays, FISH, and multiplex ligation-dependent probe amplification. Most copy number alterations targeted chromosome 21, reinforcing the complexity of this chromosome. The common region of amplification on chromosome 21 was refined to a 5.1-mb region that included RUNX1, miR-802, and genes mapping to the Down syndrome critical region. Recurrent abnormalities affecting genes in key pathways were identified: IKZF1 (22%), CDKN2A/B (17%), PAX5 (8%), ETV6 (19%), and RB1 (37%). Investigation of clonal architecture provided evidence that these abnormalities, and P2RY8-CRLF2, were secondary to chromosome 21 rearrangements. Patient outcome was uniformly poor with standard therapy irrespective of the presence or absence of these changes. This study has provided evidence that chromosome 21 instability is the only anomaly among those so far investigated that is common to all iAMP21 patients, and therefore the initiating event is likely to be found among the complex structural rearrangements of this abnormal chromosome.
We evaluated antecedent exercise for treating the automatically reinforced problem behavior of 4 individuals with autism. We conducted preference assessments to identify leisure and exercise items that were associated with high levels of engagement and low levels of problem behavior. Next, we conducted three 3-component multiple-schedule sequences: an antecedent-exercise test sequence, a noncontingent leisure-item control sequence, and a social-interaction control sequence. Within each sequence, we used a 3-component multiple schedule to evaluate preintervention, intervention, and postintervention effects. Problem behavior decreased during the postintervention component relative to the preintervention component for 3 of the 4 participants during the exercise-item assessment; however, the effects could not be attributed solely to exercise for 1 of these participants.
Deregulated expression of CRLF2 (CRLF2-d) arises via its juxtaposition to the IGH@ enhancer or P2RY8 promoter. Among 865 BCP-ALL children treated on MRC ALL97, 52 (6%) had CRLF2-d, but it was more prevalent among Down syndrome patients (54%). P2RY8-CRLF2 (n = 43) was more frequent than IGH@-CRLF2 (n = 9). CRLF2-d was not associated with age, sex, or white cell count, but IGH@-CRLF2 patients were older than P2RY8-CRLF2 patients (median 8 vs 4 years, P = .0017). Patients with CRLF2-d were more likely to present with enlarged livers and spleens (38% vs 18%, P < .001). CRLF2-d was not seen in conjunction with established chromosomal translocations but 6 (12%) cases had high hyperdiploidy, and 5 (10%) had iAMP21. Univariate analysis suggested that CRLF2-d was associated with an inferior outcome: (event-free survival [EFS] hazard ratio 2.27 [95% confidence interval 1.48-3.47], P < .001; OS 3.69 [2.34-5.84], P < .001). However, multivariate analysis indicated that its effect was mediated by other risk factors such as cytogenetics and DS status (EFS 1.45 [0.88-2.39], P = .140; OS 1.90 [1.08-3.36], P = .027). Although the outcome of IGH@-CRLF2 patients appeared inferior compared with P2RY8-CRLF2 patients, the result was not significant (EFS 2.69 [1.15-6.31], P = .023; OS 2.86 [1.15-6.79], P = .021). Therefore, we concluded that patients with CRLF2-d should be classified into the intermediate cytogenetic risk group.
This report presents the development, plan, and operation of the National Survey of Adoptive Parents (NSAP), a module of the State and Local Area Integrated Telephone Survey conducted by the Centers for Disease Control and Preventions National Center for Health Statistics. NSAP was designed to produce national estimates of the characteristics, health, and well-being of adopted children and their families, the preadoption experiences of the adoptive parents, and their access to and utilization of postadoption supports and services. Funding for the survey was provided by the Office of the Assistant Secretary for Planning and Evaluation and the Administration for Children and Families, both of the Department of Health and Human Services.
This report presents the development, plan, and operation of the National Survey of Adoptive Parents of Children with Special Health Care Needs (NSAP-SN), a module of the State and Local Area Integrated Telephone Survey conducted by the Centers for Disease Control and Preventions National Center for Health Statistics. The survey was designed to produce national estimates of the characteristics, health, and well-being of adopted children with special health care needs (CSHCN) and their families, the preadoption experiences of the adoptive parents, and their access to and utilization of postadoption services. Funding was provided by the Office of the Assistant Secretary for Planning and Evaluation and the Administration for Children and Families, both of the U.S. Department of Health and Human Services.
We report 2 novel, cryptic chromosomal abnormalities in precursor B-cell acute lymphoblastic leukemia (BCP-ALL): a translocation, either t(X;14)(p22;q32) or t(Y;14)(p11;q32), in 33 patients and an interstitial deletion, either del(X)(p22.33p22.33) or del(Y)(p11.32p11.32), in 64 patients, involving the pseudoautosomal region (PAR1) of the sex chromosomes. The incidence of these abnormalities was 5% in childhood ALL (0.8% with the translocation, 4.2% with the deletion). Patients with the translocation were older (median age, 16 years), whereas the patients with the deletion were younger (median age, 4 years). The 2 abnormalities result in deregulated expression of the cytokine receptor, cytokine receptor-like factor 2, CRLF2 (also known as thymic stromal-derived lymphopoietin receptor, TSLPR). Overexpression of CRLF2 was associated with activation of the JAK-STAT pathway in cell lines and transduced primary B-cell progenitors, sustaining their proliferation and indicating a causal role of CRLF2 overexpression in lymphoid transformation. In Down syndrome (DS) ALL and 2 non-DS BCP-ALL cell lines, CRLF2 deregulation was associated with mutations of the JAK2 pseudokinase domain, suggesting oncogenic cooperation as well as highlighting a link between non-DS ALL and JAK2 mutations.
To determine the prevalence and prognostic impact of significant acute lymphoblastic leukemia (ALL) -related genes: CRLF2 deregulation (CRLF2-d), IGH@ translocations (IGH@-t), and deletions of CDKN2A/B, IKZF1, PAX5, ETV6, RB1, BTG1, and EBF1 in adolescents and adults.
This report presents the development, plan, and operation of the 2007 National Survey of Childrens Health, a module of the State and Local Area Integrated Telephone Survey, conducted by the Centers for Disease Control and Preventions National Center for Health Statistics. This survey was designed to produce national and state-specific prevalence estimates for a variety of physical, emotional, and behavioral health indicators and measures of childrens experiences with the health care system. The survey also includes questions about the family (for example, parents health status, stress and coping behaviors, family activities) and about respondents perceptions of the neighborhoods where their children live. Funding and direction for this survey was provided by the Maternal and Child Health Bureau of the Health Resources and Services Administration.
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