An evaluation of cancer survivorship activities across national comprehensive cancer control programs

Introduction

Centers for Disease Control and Prevention’s (CDC) National Comprehensive Cancer Control Program (NCCCP) funds states, the District of Columbia, tribal organizations, territories, and jurisdictions across the USA develop and implement jurisdiction-specific comprehensive cancer control (CCC) plans. The objective of this study was to analyze NCCCP action plan data for incorporation and appropriateness of cancer survivorship-specific goals and objectives.

Methods

In August 2013, NCCCP action plans maintained within CDC’s Chronic Disease Management Information System (CDMIS) from years 2010 to 2013 were reviewed to assess the inclusion of cancer survivorship objectives. We used the CDMIS search engine to identify “survivorship” within each plan and calculated the proportion of programs that incorporate cancer survivorship-related content during the study period and in each individual year. Cancer survivorship objectives were then categorized by compatibility with nationally accepted, recommended strategies from the report A National Action Plan for Cancer Survivorship: Advancing Public Health Strategies (NAP).

Results

From 2010 to 2013, 94 % (n?=?65) of NCCCP action plans contained survivorship content in at least 1 year during the time period and 38 % (n?=?26) of all NCCCP action plans addressed cancer survivorship every year during the study period. Nearly 64 % (n?=?44) of NCCCP action plans included cancer survivorship objectives recommended in NAP.

Conclusion

Nearly all NCCCP action plans addressed cancer survivorship from 2010 to 2013, and most programs implemented recommended cancer survivorship efforts during the time period.

Implications for Cancer Survivors

NCCCP grantees can improve cancer survivorship support by incorporating recommended efforts within each year of their plans.

     

Assessment of complication and functional outcome reporting in the minimally invasive prostatectomy literature from 2006 to the present

To query the minimally invasive urological literature from 2006 to the middle of 2010, focusing on complications and functional outcome reporting in laparoscopic radical prostatectomy (LRP) and robot-assisted LRP (RALP), to see if there has been an improvement in the overall reporting of complications. We performed a Medline search using the Medical Subject Heading terms 'prostatectomy', 'laparoscopy', 'robotics', and 'minimally invasive'. We then applied the Martin criteria for complications reporting to the selected articles. We identified 51 studies for a total of 32,680 patients. When excluding functional outcomes the outpatient complications reporting was 20/51 (39.2%). In all, 35% and 43% of papers did not list any method for recording continence and potency, respectively. A complication grading system was only used in 30 studies (58.8%). Of the 16 papers using a grading scale in 2006-2007, only 31.3% used the Clavien system, compared with 69% from 2008 to the first half of 2010. In all, 27% of papers used some form of risk-factor analysis for complications. Multivariate analysis was used in 43% of papers, 29% looked at body mass index, while one looked at prostate weight, and another age. There has been an overall improvement in complications reporting in the minimally invasive RP literature since 2005. However, most studies still do not fulfil many of the criteria necessary for standardised complication reporting. Functional outcome reporting remains poor and unstandardised. Given our current reliance on observational studies, increased efforts should be made to standardise all complication outcomes reporting.     

Development of a Genomic DNA Reference Material Panel for Rett Syndrome (MECP2-Related Disorders) Genetic Testing

Rett syndrome is a dominant X-linked disorder caused by point mutations (approximately 80%) or by deletions or insertions (approximately 15% to 18%) in the MECP2 gene. It is most common in females but lethal in males, with a distinctly different phenotype. Rett syndrome patients have severe neurological and behavioral problems. Clinical genetic testing laboratories commonly use characterized genomic DNA reference materials to assure the quality of the testing process; however, none are commercially available for MECP2 genetic testing. The Centers for Disease Control and Prevention’s Genetic Testing Reference Material Coordination Program, in collaboration with the genetic testing community and the Coriell Cell Repositories, established 27 new cell lines and characterized the MECP2 mutations in these and in 8 previously available cell lines. DNA samples from the 35 cell lines were tested by eight clinical genetic testing laboratories using DNA sequence analysis and methods to assess copy number (multiplex ligation-dependent probe amplification, semiquantitative PCR, or array-based comparative genomic hybridization). The eight common point mutations known to cause approximately 60% of Rett syndrome cases were identified, as were other MECP2 variants, including deletions, duplications, and frame shift and splice-site mutations. Two of the 35 samples were from males with MECP2 duplications. These MECP2 and other characterized genomic DNA samples are publicly available from the NIGMS Repository at the Coriell Cell Repositories.Rett syndrome is a dominant X-linked disorder usually caused by point mutations (approximately 80% in classical and 40% in atypical cases) and deletions or insertions (approximately 15% to 18% in classical and 3% in atypical cases) in the MECP2 gene, although patients with mutations in two other genes, CDKL5 and FOXG1, may also exhibit a Rett-like phenotype.^1,2 MECP2, located on Xq28 and comprising four exons, encodes methyl-CpG binding protein 2 (MeCP2). In males, Rett syndrome is usually lethal, because of abnormal MeCP2 function from the single X chromosome and severe neonatal encephalopathy, although Rett syndrome in males with an XXY karyotype has been reported.³ Duplications in MECP2 have also been observed, and these can cause severe neurodevelopmental disability in males.⁴ The prevalence of Rett syndrome in females is approximately 1:10,000.⁵Girls with classic Rett syndrome (OMIM #312750) exhibit a rapid decline in language and motor skills at approximately 1 year of age. These patients exhibit a loss of acquired purposeful hand use, loss of communication, gait ataxia and apraxia, and stereotypic hand movements. They may also exhibit additional symptoms, including bruxism, seizures, episodic apnea or hyperpnea, abnormal muscle tone, and often acquired microcephaly.^4,6,7Patients with Rett syndrome can present with various phenotypes. Those diagnosed with atypical Rett syndrome may have either more mild or more severe presentation than patients with the classical form. These patients share some of the same symptoms with classical Rett syndrome cases, but must also have some of the additional symptoms listed above.^4,6 Some patients present with only mild learning disabilities (females) or intellectual disability (males).^4,8Molecular diagnosis of Rett syndrome is performed by examination of the patient’s DNA for MECP2 mutations, using a variety of molecular diagnostic methods. Most MECP2 mutations are sporadic, and testing is performed on the proband, although predictive prenatal and preimplantation genetic testing may also be performed. DNA sequence analysis may detect point mutations and small insertions and deletions (indels). Larger deletions and duplications are detected using multiplex ligation-dependent probe amplification (MLPA), quantitative PCR, and array-based comparative genomic hybridization (CGH). To date, there are no US Food and Drug Administration (FDA)–approved assays for Rett syndrome. All testing is performed using laboratory-developed tests.Reference materials are needed by laboratories to comply with regulatory and accreditation requirements for assay development, assay validation, and quality control, and their use is recommended by professional guidelines for clinical laboratories [eg, http://www.acmg.net/Pages/ACMG_Activities/stds-2002/g.htm, last accessed October 16, 2013; Washington State Legislature, http://www.doh.wa.gov/hsqa/fsl/lqa_home.htm, last accessed January 11, 2013; College of American Pathologists http://www.cap.org/apps/cap.portal, last accessed January 11, 2013 (registration required); New York State Clinical Laboratory Evaluation Program, http://www.wadsworth.org/clep, last accessed January 11, 2013).^9–15 Clinical genetic testing laboratories commonly use characterized genomic DNA reference materials to assure the quality of the testing process. Ideally, these reference materials should closely resemble patient samples containing variants and types of variants common to the disorder and should be thoroughly characterized using methods different from those used in the user’s laboratory.¹⁵ For Rett syndrome, genomic DNA reference materials derived from females (and, if possible, males) containing common point mutations, indels, and larger deletions and duplications should be used. Careful use of a well-characterized and comprehensive set of reference materials helps to assure the proper design and function of a clinical assay. To date, there are no commercially available reference materials for Rett syndrome genetic testing.To address the need for characterized genomic DNA reference materials for Rett syndrome testing, the Centers for Disease Control and Prevention (CDC) based Genetic Testing Reference Material Coordination Program (GeT-RM), in collaboration with members of the genetic testing community and the National Institute of General Medical Sciences (NIGMS) Repository at the Coriell Cell Repositories, have characterized the MECP2 mutations in 35 publicly available cell lines. Twenty-seven of the 35 cell lines were generated as part of this project, using blood collected with informed consent from Rett syndrome patients with variants not previously represented in cell lines at the Coriell Repository. The availability of a renewable source of characterized reference materials for Rett syndrome helps to assure the accuracy of these genetic tests and facilitate research and test development.     

Remote intrapulmonary spread of recurrent respiratory papillomatosis with malignant transformation

Recurrent respiratory papillomatosis is the most common neoplasm of the larynx in childhood. Extension into lung parenchyma occurs in less than 1% of patients and has a low risk of malignant transformation. Treatment options for intrapulmonary spread have shown limited success. We describe a case of recurrent respiratory papillomatosis with extensive parenchymal involvement and adenosquamous carcinoma in a 14-year-old girl.     

Natural course of large spontaneous pneumothorax

A large pneumothorax was detected on chest x-ray film in a 41-year-old woman. She declined recommendations for evacuation of the air trapped in her right chest. The condition spontaneously resolved during 3 months after being detected.     

Reframing High-Quality Public Preschool as a Vehicle for Narrowing Child Health Disparities Based on Family Income

Past research shows that high-quality public preschool may disproportionately support low-income children's school readiness because low-income children tend to arrive at school with fewer of the academic skills needed for success. This suggests a compensatory process in human development in which the children who benefit most from a promotive factor are those who stand to gain the most. We propose that high-quality public preschool may similarly confer its greatest health rewards to low-income children, who are generally in poorer health than their peers. If that is true, preschool has the potential to narrow health disparities by income, which without intervention, persist into adulthood. To date, no one has articulated all the pathways through which high-quality public preschool may improve children's health, much less those that should disproportionately benefit those from low-income families. Drawing on the bioecological paradigm of human development, we propose a model identifying specific mechanisms likely to promote equity in child health. These mechanisms reflect core characteristics of high-quality public preschool that may disproportionately benefit low-income children's health. This model serves as a working template for a program of future research.