Introduction: Medical cannabis (marijuana) use is legal in 33 U.S. states and the District of Columbia. Clinicians can play an important role in helping patients access and weigh potential benefits and risks of medicinal cannabis. Accordingly, this study aimed to assess clinician beliefs and practices related to cannabis. Methods: Data are from 1506 family practice doctors, internists, nurse practitioners, and oncologists who responded to the 2018 DocStyles, a web-based panel survey of clinicians. Questions assessed medicinal uses for and practices related to cannabis and assessed clinicians' knowledge of cannabis legality in their state. Logistic regression was used to assess multivariable correlates of asking about, assessing, and recommending cannabis. Results: Over two-thirds (68.9%) of clinicians surveyed believe that cannabis has medicinal uses and just over a quarter (26.6%) had ever recommended cannabis to a patient. Clinicians who believed cannabis had medicinal uses had 5.9 times the adjusted odds (95% confidence interval 3.9-8.9) of recommending cannabis to patients. Beliefs about conditions for medical cannabis use did not necessarily align with the current scientific evidence. Nearly two-thirds (60.0%) of clinicians surveyed incorrectly reported the legal status of cannabis in their state. Discussion: Findings suggest that while clinicians believe that cannabis has medicinal uses, they may not have a full understanding of the scientific evidence and may not accurately understand their state-based policies for cannabis legalization and use. Given that clinicians are responsible for recommending medicinal cannabis in most states that have legalized it, ongoing education about the health effects of cannabis is warranted.

Background Little is known about breastfeeding initiation and duration in the context of postpartum marijuana use and safety beliefs. Research Aims (1) To describe characteristics of women who used marijuana postpartum; (2) to evaluate the relationship between postpartum marijuana use and breastfeeding behaviors; and 3) to assess, among women who used marijuana postpartum, how safety perceptions are associated with breastfeeding behaviors. Methods Data from the cross-sectional Pregnancy Risk Assessment Monitoring System, a United States national governmental survey, 2017, were analyzed for participants with infants aged ≥ 12 weeks (seven states, unweighted N = 4604). Chi-square tests were used to compare characteristics and counseling for postpartum marijuana use. For participants with postpartum use, adjusted prevalence ratios (aPR) were calculated to evaluate relationships between safety perceptions and breastfeeding initiation and duration. Results Overall, 5.5% (95% CI [4.6, 6.6]) of participants reported postpartum marijuana use; among these women, 47.2% (CI [37.6, 56.9]) were breastfeeding at the time of the survey. Overall, 25.7% of participants indicated that they had been advised, by their prenatal care provider, against marijuana use while breastfeeding. Breastfeeding initiation or duration did not differ by postpartum marijuana use. Among participants with postpartum use, those who perceived marijuana was safe for breastfeeding women to use were more likely to have breastfed (aPR = 1.22, CI [1.04, 1.43]) and have a breastfeeding duration > 12 weeks (aPR = 1.57, CI [1.08, 2.27]) compared to those who perceived it to be unsafe. Conclusions Understanding maternal safety beliefs and provider education about the latest evidence and guidance related to postpartum marijuana use may improve clinical care.

Background: Research on prenatal cannabis use and adverse infant outcomes is inconsistent, and findings vary by frequency of use or cigarette use. We assess (1) the prevalence of high frequency (>once/week), low frequency (<once/week), and any cannabis use during pregnancy by maternal characteristics and adverse infant outcomes; (2) the prevalence of infant outcomes by cannabis use frequency, stratified by cigarette smoking; and (3) the association between cannabis use frequency and infant outcomes, stratified by cigarette smoking. Methods: Cross-sectional data from 8 states' 2017 Pregnancy Risk Assessment Monitoring System (n = 5548) were analyzed. We calculated adjusted prevalence ratios (aPR) between cannabis use frequency and infant outcomes with Modified Poisson regression. Results: Approximately 1.7 % and 2.6 % of women reported low and high frequency prenatal cannabis use, respectively. Prevalence of use was higher among women with small-for-gestational age (SGA) (10.2 %) and low birthweight (9.7 %) deliveries, and cigarette use during pregnancy (21.2 %). Among cigarette smokers (aPR: 1.8; 95 % CI: 1.1-3.0) and non-smokers (aPR: 2.1; 95 % CI: 1.1-3.9), high frequency cannabis use doubled the risk of low birthweight delivery but did not increase preterm or SGA risk. Regardless of cigarette use, low frequency cannabis use did not significantly increase infant outcome risk. Conclusions: Prenatal cannabis use was more common among women who smoked cigarettes during pregnancy. High frequency cannabis use was associated with low birthweight delivery, regardless of cigarette use. Healthcare providers can implement recommended substance use screening and provide evidence-based counseling and cessation services to help pregnant women avoid tobacco and cannabis use.

IntroductionAs policies legalizing nonmedical marijuana have increased in states, understanding the implications of marijuana use among adolescents is increasingly important. This study uses nationally representative data to assess behavioral risk factors among students with different patterns of marijuana use.MethodsData from the 2015 and 2017 Youth Risk Behavior Surveys, cross-sectional surveys conducted among a nationally representative sample of students in Grades 9–12 (n=30,389), were used to examine the association between self-reported current marijuana use status and self-report of 30 risk behaviors across 3 domains: substance use, injury/violence, and sexual health. Among current marijuana users, authors assessed differences between established (≥100 lifetime uses) and nonestablished (<100 uses) users. Multivariable models were used to calculate adjusted prevalence ratios. Data were analyzed in 2019.ResultsCurrent marijuana users (regardless of use pattern) had a significantly greater likelihood of engaging in 27 of the 30 behaviors assessed across the 3 domains than the noncurrent users. Those with established use patterns (versus nonestablished) had a greater risk of lifetime use of most other substances (licit and illicit, including tobacco, alcohol, heroin, misuse of opioids), some injury/violence behaviors (including driving while using marijuana and suicide ideation and attempt), and sexual risk behaviors.ConclusionsBoth established and nonestablished patterns of adolescent marijuana use are associated with a number of other risky behaviors. In addition to interventions focused on preventing youth initiation of marijuana, clinicians and public health professionals should consider interventions to help adolescents who have nonestablished use patterns to avoid continued, established use.

Marijuana is the most commonly used illicit substance under federal law in the United States (1); however, many states have legalized medical and adult nonmedical use. Evidence regarding the safety and health effects of cannabis use during pregnancy is largely inconclusive (2). Potential adverse health effects to exposed infants (e.g., lower birthweight) have been documented (2). To provide population-based estimates of use surrounding pregnancy, identify reasons for and mode of use, and understand characteristics of women who continue versus cease marijuana use during pregnancy, CDC analyzed data from eight states participating in the 2017 Pregnancy Risk Assessment Monitoring System (PRAMS) marijuana supplement. Overall, 9.8% of women self-reported marijuana use before pregnancy, 4.2% during pregnancy, and 5.5% after pregnancy. The most common reasons for use during pregnancy were to relieve stress or anxiety, nausea or vomiting, and pain. Smoking was the most common mode of use. In multivariable models that included age, race/ethnicity, marital status, education, insurance status, parity, trimester of entry into prenatal care, and cigarette and e-cigarette use during pregnancy, women who continued versus ceased marijuana use during pregnancy were more likely to be non-Hispanic white or other race/ethnicity than non-Hispanic black, be unmarried, have ≤12 years of education, and use cigarettes during pregnancy. The American College of Obstetricians and Gynecologists (ACOG) and the American Academy of Pediatrics (AAP) recommend refraining from marijuana use during pregnancy and lactation (3,4). Given the increasing number of states legalizing medical and adult nonmedical marijuana use, surveillance of perinatal marijuana use can inform clinical guidance, provider and patient education, and public health programs to support evidence-based approaches to addressing substance use.

Comprehensive public health surveillance of hemophilia and related disorders is important to monitor health indicators to inform prevention strategies. The United States (US) Centers for Disease Control and Prevention (CDC) Haemophilia Surveillance Study (HSS,1993–1998) was population based in six states to estimate occurrence rates, sources of care, complications, and outcomes.1 HSS reported 67% of the 16 960 persons with hemophilia A or B projected to be then living in the US were seen at hemophilia treatment centers (HTCs).1 HSS determined a case rate for intracranial hemorrhage of 0.0054 cases/patient year, with increased risk with the human immunodeficiency virus (HIV) coinfection.2 Persons with hemophilia and HIV or acquired immunodeficiency syndrome (AIDS) had an increased risk of death (5- and 33-fold risk as compared to uninfected persons, respectively); mortality was decreased by 40% in persons seen in HTCs.3 Informed by HSS, the Universal Data Collection (UDC) project (1998–2011) collected data on 27 368 individuals (19 023 with hemophilia A or B) seen within the 129 HTCs of the US HTC network (USHTCN). UDC focused on treatment-related infections and arthropathy.4 No new infections of HIV or hepatitis were linked to products used to treat bleeding disorders in 90 000 tested specimens.4 UDC documented the rapid penetration of prophylaxis and associated decreases in bleeding and arthropathy.5 In 2012, hemophilia surveillance was revamped as the Community Counts (CC) project to focus on contemporary outcomes and complications. With novel therapies, widespread prophylaxis, and highly effective HIV and hepatitis C treatments, persons with hemophilia now survive to be at risk for disorders of aging, and the development of inhibitory antibodies has emerged as a major cause of morbidity. CC builds upon previous work while addressing current priorities relevant to persons affected by bleeding disorders. CC represents an innovative and effective partnership among: (1) a healthcare network (the USHTCN); (2) a national data coordinating center [the American Thrombosis and Hemostasis Network (ATHN)]; (3) a national public health agency (CDC); and (4) community participants, to apply public health surveillance techniques to a set of rare chronic diseases. The USHTCN includes 139 HTCs located primarily in academic medical centers and partially funded by the Health Resources and Services Administration. The USHTCN is organized in 10 geographic regions covering the continental US, Alaska, Hawaii, Guam, and Puerto Rico with leadership through regional coordinators (with nursing, social work, or public health expertise) who function as information and support conduits and regional directors (mainly hematologists) who assume responsibility for the scientific direction. ATHN was established by USHTCN members to create and manage a secure data resource. ATHN, as the coordinating center, provides the electronic data platform, training and technical assistance. CDC provides funding, programmatic guidance, laboratory testing, technical assistance, and data management, analysis, and reports. The HTCs identify and enroll eligible participants, collect specimens, and analyze, present, and publish results. CC infrastructure includes five core committees. Each committee includes representatives of ATHN, CDC, and the USHTCN. The Executive Committee provides overall direction of the project. The Administrative Committee supports the administration of CC throughout the regions. The Science Committee develops and evaluates the science of the project. A Project Review Committee evaluates proposals for analysis based on feasibility and scientific validity. A Presentation and Publication Committee reviews all project products for scientific soundness and authorship. The HTC PP captures minimal data to estimate population size and characteristics of patients receiving care within the USHTCN. HTC PP is a Health Insurance Portability and Accountability Act of 1996 (HIPAA) compliant de-identified dataset and includes: year of birth, gender, race, ethnicity, 3-digit zip code, insurance status, primary diagnosis, laboratory assays for factor deficiency, and history of HIV and/or HCV infection. Persons with inherited bleeding disorders, Ehlers–Danlos syndrome and venous thromboembolism (VTE) are included. The Registry includes a HIPAA compliant limited dataset and a sample repository. VTE and Ehlers–Danlos syndrome are excluded. The outcomes and complications collected include: bleeding events; joint surgeries; inhibitor development; treatment products and regimens; blood-borne infections; diseases of aging (hypertension, cardiovascular disease, cancer, etc); functional impairment; pain and health service utilization. A central laboratory located at the CDC utilizes advanced technology to detect very low levels of inhibitory antibodies, even in the presence of exogenous factor, as well as non-neutralizing binding antibodies that may precede or contribute to inadequate treatment response.6 Risk-based testing is performed for blood-borne pathogens. Mortality Reporting collects causes of death of persons with bleeding disorders as a HIPAA compliant de-identified dataset. ATHN processes incorporate administrative, technical, and physical safeguards for the web-based data collection system to prevent inappropriate use or disclosure of data to ensure HIPAA/Health Information Technology for Economic and Clinical Health Act of 2009 compliance. Data are securely transmitted to the CDC daily. From September 30, 2011 through September 30, 2017, 168 644 visits were recorded on 70 137 individuals in HTC PP; the Registry recorded data on 13 198 persons, including 41% of patients in HTC PP with hemophilia A and B (Table 1). Mortality data were collected on 822 decedents. Public health surveillance of bleeding disorders is essential because complications are of high prevalence, economic impact, and morbidity, and are often preventable. A strength of CC is its representativeness, covering the entire US and territories; race and ethnicity approximate the general US population. By accruing baseline and longitudinal data spanning the entire health history of individuals, CC will yield invaluable information on key outcomes. Limitations to CC surveillance include cost, training, quality assurance, and reliance on self-assessment for patient reported outcomes. In spite of these limitations, CC is a model public health surveillance program for rare chronic diseases. With the proliferation of new treatment products and regimens, CC is likely to facilitate an exponential growth in data about hemophilia practices and outcomes to inform treatment recommendations. Community Counts is funded by the CDC through a cooperative agreement (DD001155) awarded to ATHN in partnership with the USHTCN. Additional support was supplied by the Maternal and Child Health Bureau, 340B program 2H30MC 24049. The authors would like to thank the USHTCN regional leadership, clinicians and staff for their efforts in the development and ongoing implementation of Community Counts, and especially the patients and families who contributed information comprising the database. The findings and conclusions in this report are those of the authors and do not necessarily represent the official position of the CDC. Marilyn J. Manco-Johnson has no conflicts to declare. Vanessa Byams has no conflicts to declare. Michael Recht has no conflicts to declare. Becky Dudley has no conflicts to declare. Brandi Dupervil has no conflicts to declare. Diane Aschman has no conflicts to declare. Meredith Oakley has no conflicts to declare. Suzanne Kapica has no conflicts to declare. Mariam Voutsis has no conflicts to declare. Steven Humes has no conflicts to declare. Roshni Kulkarni has no conflicts to declare. Althea Grant has no conflicts to declare. Marilyn Manco-Johnson designed research, performed research, analyzed data, and wrote the paper; Vanessa Byams designed research, performed research, and wrote the paper; Michael Recht designed research, performed research, edited and approved the manuscript; Becky Dudley coordinated the research project, performed research, reviewed and approved the manuscript; Brandi Dupervil analyzed data, reviewed and approved the paper; Diane Aschman performed research, reviewed and approved the paper; Meredith Oakley designed research, performed research, reviewed and approved the manuscript; Suzanne Kapica performed research, reviewed and approved the manuscript; Mariam Voutsis performed research, reviewed and approved the manuscript; Steven Humes performed research, reviewed and approved the manuscript; Roshni Kulkarni performed research, reviewed and approved the manuscript; Althea Grant designed research, performed research, reviewed and approved the manuscript.

BACKGROUND AND OBJECTIVES Data are limited on the behavioral risk correlates of synthetic cannabinoid use. The purpose of this study was to compare the behavioral risk correlates of synthetic cannabinoid use with those among marijuana users. METHODS Data from the 2015 Youth Risk Behavior Survey, a cross-sectional survey conducted in a nationally representative sample of students in grades 9 through 12 (N = 15 624), were used to examine the association between self-reported type of marijuana use (ie, never use of marijuana and synthetic cannabinoids, ever use of marijuana only, and ever use of synthetic cannabinoids) and self-report of 36 risk behaviors across 4 domains: substance use, injury/violence, mental health, and sexual health. Multivariable models were used to calculate adjusted prevalence ratios. RESULTS Students who ever used synthetic cannabinoids had a significantly greater likelihood of engaging in each of the behaviors in the substance use and sexual risk domains compared with students who ever used marijuana only. Students who ever used synthetic cannabinoids were more likely than students who ever used marijuana only to have used marijuana before age 13 years, to have used marijuana ≥1 times during the past 30 days, and to have used marijuana ≥20 times during the past 30 days. Several injury/violence behaviors were more prevalent among students who ever used synthetic cannabinoids compared with students who ever used marijuana only. CONCLUSIONS Health professionals and school-based substance use prevention programs should include strategies focused on the prevention of both synthetic cannabinoids and marijuana.

Objective To better understand the knowledge, attitudes and practices of obstetrician-gynecologists with respect to screening and treatment for iron deficiency anemia (IDA). Methods A total of 1,200 Fellows and Junior Fellows of the American College of Obstetricians and Gynecologists were invited to participate in a survey on blood disorders. Respondents completed a questionnaire regarding their patient population, screening and treatment practices for IDA, and general knowledge about IDA and its risk factors. Results Overall response rate was 42.4%. Thirty-eight percent of respondents screen non-pregnant patients regularly, based on risk factors; 30.5% screen only when symptoms of anemia are present. For pregnant patients, 50.0% of respondents screen patients at their initial visit, while 46.2% screen every trimester. Sixty-one percent of respondents supplement pregnant patients when there is laboratory evidence of anemia; 31.6% supplement all pregnant patients. Forty-two percent of respondents screen post-partum patients based on their risk factors for IDA. However, when asked to identify risk factors for post-partum anemia, slightly more than half of respondents correctly identified young age and income level as risk factors for post-partum anemia; only 18.9% correctly identified pre-pregnancy obesity as a risk factor. Conclusion There are opportunities for increased education on IDA for obstetrician-gynecologists, specifically with respect to risk factors. There also appears to be substantial practice variance regarding screening and supplementation for IDA, which may correspond to variability in professional guidelines. Increased education on IDA, especially the importance of sociodemographic factors, and further research and effort to standardize guidelines is needed.

The purpose of this study was to examine the patterns of marijuana and tobacco use and their associations with suboptimal self-rated health (SRH) among US adults who reported “ever, even once, using marijuana or hashish.” Data came from the 2009–2012 National Health and Nutrition Examination Survey, restricting to respondents aged 20 years and older who reported using marijuana at least once in their lifetime (n = 3,210). We assessed the age-adjusted prevalence of mutually exclusive groups of regular (at least once a month for more than one year) and non-regular marijuana smoking by current (serum cotinine ≥3.08 ng/mL) and not current use of tobacco. Suboptimal SRH status was defined as “fair” or “poor” in response to the question “Would you say that in general your health is excellent, very good, good, fair, or poor?” We produced prevalence ratios with multivariable log-linear regression models. Among ever users of marijuana, the age-adjusted prevalence of regular marijuana smoking with current tobacco use, non-regular marijuana smoking with current tobacco use, and regular marijuana smoking without current tobacco use was 24.7%, 15.2%, and 21.1%, respectively. When compared to non-regular marijuana smokers without current tobacco use, the adjusted prevalence ratio for reporting suboptimal SRH was 1.98 (95% CI: 1.50–2.61), 1.82 (95% CI: 1.40–2.37), and 1.34 (95% CI: 1.05–1.69), respectively. In conclusion, among adult ever users of marijuana, current tobacco use is high and strongly associated with suboptimal SRH; regular marijuana smoking with or without current tobacco use is significantly associated with suboptimal SRH.

Objectives Vitamin K deficiency bleeding (VKDB) in infants is a coagulopathy preventable with a single dose of injectable vitamin K at birth. The Tennessee Department of Health (TDH) and Centers for Disease Control and Prevention (CDC) investigated vitamin K refusal among parents in 2013 after learning of four cases of VKDB associated with prophylaxis refusal. Methods Chart reviews were conducted at Nashville-area hospitals for 2011-2013 and Tennessee birthing centers for 2013 to identify parents who had refused injectable vitamin K for their infants. Contact information was obtained for parents, and they were surveyed regarding their reasons for refusing. Results At hospitals, 3.0% of infants did not receive injectable vitamin K due to parental refusal in 2013, a frequency higher than in 2011 and 2012. This percentage was much higher at birthing centers, where 31% of infants did not receive injectable vitamin K. The most common responses for refusal were a belief that the injection was unnecessary (53%) and a desire for a natural birthing process (36%). Refusal of other preventive services was common, with 66% of families refusing vitamin K, newborn eye care with erythromycin, and the neonatal dose of hepatitis B vaccine. Conclusions for Practice Refusal of injectable vitamin K was more common among families choosing to give birth at birthing centers than at hospitals, and was related to refusal of other preventive services in our study. Surveillance of vitamin K refusal rates could assist in further understanding this occurrence and tailoring effective strategies for mitigation.

PROBLEM/CONDITIONIn the United States, marijuana is the most commonly used illicit drug. In 2013, 7.5% (19.8 million) of the U.S. population aged ≥12 years reported using marijuana during the preceding month. Because of certain state-level policies that have legalized marijuana for medical or recreational use, population-based data on marijuana use and other related indicators are needed to help monitor behavioral health changes in the United States.PERIOD COVERED2002-2014.DESCRIPTION OF SYSTEMThe National Survey on Drug Use and Health (NSDUH) is a national- and state-level survey of a representative sample of the civilian, noninstitutionalized U.S. population aged ≥12 years. NSDUH collects information about the use of illicit drugs, alcohol, and tobacco; initiation of substance use; frequency of substance use; substance dependence and abuse; perception of substance harm risk or no risk; and other related behavioral health indicators. This report describes national trends for selected marijuana use and related indicators, including prevalence of marijuana use; initiation; perception of harm risk, approval, and attitudes; perception of availability and mode of acquisition; dependence and abuse; and perception of legal penalty for marijuana possession.RESULTSIn 2014, a total of 2.5 million persons aged ≥12 years had used marijuana for the first time during the preceding 12 months, an average of approximately 7,000 new users each day. During 2002-2014, the prevalence of marijuana use during the past month, past year, and daily or almost daily increased among persons aged ≥18 years, but not among those aged 12-17 years. Among persons aged ≥12 years, the prevalence of perceived great risk from smoking marijuana once or twice a week and once a month decreased and the prevalence of perceived no risk increased. The prevalence of past year marijuana dependence and abuse decreased, except among persons aged ≥26 years. Among persons aged ≥12 years, the percentage reporting that marijuana was fairly easy or very easy to obtain increased. The percentage of persons aged ≥12 reporting the mode of acquisition of marijuana was buying it and growing it increased versus getting it for free and sharing it. The percentage of persons aged ≥12 years reporting that the perceived maximum legal penalty for the possession of an ounce or less of marijuana in their state is a fine and no penalty increased versus probation, community service, possible prison sentence, and mandatory prison sentence.INTERPRETATIONSince 2002, marijuana use in the United States has increased among persons aged ≥18 years, but not among those aged 12-17 years. A decrease in the perception of great risk from smoking marijuana combined with increases in the perception of availability (i.e., fairly easy or very easy to obtain marijuana) and fewer punitive legal penalties (e.g., no penalty) for the possession of marijuana for personal use might play a role in increased use among adults.PUBLIC HEALTH ACTIONNational- and state-level data can help federal, state, and local public health officials develop targeted prevention activities to reduce youth initiation of marijuana use, prevent marijuana dependence and abuse, and prevent adverse health effects. As state-level laws on medical and recreational marijuana use change, modifications might be needed to national- and state-level surveys and more timely and comprehensive surveillance systems might be necessary to provide these data. Marijuana use in younger age groups is a particular public health concern, and changing the perception of harm risk from smoking marijuana is needed.

Sickle cell disease (SCD) and sickle cell trait (SCT) are highly prevalent in Africa. Despite public health implications, there is limited understanding of community issues for implementing newborn screening and appropriate family counseling. We conducted a 3-day workshop in Kumasi, Ghana, with community leaders as lay program development advisors to assist the development and implementation of a Sickle Cell Counselor Training and Certification Program. We employed qualitative methods to understand cultural, religious, and psychosocial dimensions of SCD and SCT, including the advisors' attitudes and beliefs in relation to developing a culturally sensitive approach to family education and counseling that is maximally suited to diverse communities in Ghana. We collated advisors' discussions and observations in order to understand community issues and potential challenges and guide strategies for advocacy in SCD family education and counseling. Results from the workshop revealed that community leaders representing diverse communities in Ghana were engaged constructively in discussions about developing a culturally sensitive counselor training program. Key findings included the importance of improved knowledge about SCD among the public and youth in particular, the value of stakeholders such as elders and religious and traditional leaders, and government expectations of reduced SCD births. We submitted a report to the Ministry of Health in Ghana with recommendations for the next steps in developing a national sickle cell counselor training program. We named the program "Genetic Education and Counseling for Sickle Cell Conditions in Ghana" (GENECIS-Ghana). The first GENECIS-Ghana Training and Certification Program Workshop was conducted from June 8 to 12, 2015.

Objective. Population-based surveillance data from California and Georgia for years 2004 through 2008 were linked to state death record files to determine the all-cause death rate among 12,143 patients identified with sickle cell disease (SCD). Methods. All-cause death rates, by age, among these SCD patients were compared with all-cause death rates among both African Americans and the total population in the two states. All-cause death rates were also compared with death rates for SCD derived from publicly available death records: the compressed mortality files and multiple cause of death files. Results. Of 12,143 patients identified with SCD, 615 patients died. The all-cause mortality rate for the SCD population was lower than the all-cause mortality rate among African Americans and similar to the total population all-cause mortality rates from birth through age 4 years, but the rate was higher among those with SCD than both the African American and total population rates from ages 5 through 74 years. The count of deceased patients identified by using population-based surveillance data ( n=5615) was more than twice as high as the count identified in compressed mortality files using SCD as the underlying cause of death alone ( n=5297). Conclusion. Accurate assessment of all-cause mortality and age at death requires long-term surveillance via population-based registries of patients with accurately diagnosed SCD.

Introduction Recurrent venous thromboembolism (VTE) occurs infrequently following a provoked event but occurs in up to 30% of individuals following an initial unprovoked event. There is limited understanding of the biological mechanisms that predispose patients to recurrent VTE. Objectives To identify whole blood gene expression profiles that distinguished patients with clinically distinct patterns of VTE. Patients/Methods We studied 107 patients with VTE separated into 3 groups: (1) 'low-risk' patients had one or more provoked VTE; (2) 'moderate-risk' patients had a single unprovoked VTE; (3) 'high-risk' patients had ≥2 unprovoked VTE. Each patient group was also compared to twenty-five individuals with no personal history of VTE. Total RNA from whole blood was isolated and hybridized to Illumina HT-12 V4 Beadchips to assay whole genome expression. Results Using class prediction analysis, we distinguished high-risk patients from low-risk patients and healthy controls with good receiver operating curve characteristics (AUC = 0.81 and 0.84, respectively). We also distinguished moderate-risk individuals and low-risk individuals from healthy controls with AUC's of 0.69 and 0.80, respectively. Using differential expression analysis, we identified several genes previously implicated in thrombotic disorders by genetic analyses, including SELP, KLKB1, ANXA5, and CD46. Protein levels for several of the identified genes were not significantly different between the different groups. Conclusion Gene expression profiles are capable of distinguishing patients with different clinical presentations of VTE, and genes relevant to VTE risk are frequently differentially expressed in these comparisons.

Sickle cell disease (SCD), sickle cell trait (SCT) and related conditions are highly prevalent in sub-Saharan Africa. Despite the public health implications, there is limited understanding of the unique needs regarding establishing and implementing extensive screening for newborns and appropriate family counseling. We sought to gain understanding of community attitudes and beliefs about SCD/SCT from counselors and potential counselors in Ghana; obtain their input about goals for counseling following newborn screening; and obtain guidance about developing effective counselor education. Five focus groups with 32 health care providers and health educators from 9 of 10 regions in Ghana were conducted by trained facilitators according to a structured protocol. Qualitative data were coded and categorized to reflect common themes. Saturation was achieved in themes related to genetics/inheritance; common complications of SCD; potential for stigmatization; marital strain; and emotional stress. Misconceptions about SCT as a form of SCD were prevalent as were cultural and spiritual beliefs about the causes of SCD/SCT. Potential positive aspects included affected children's academic achievement as compensation for physical limitations, and family cohesion. This data informed recommendations for content and structure of a counselor training program that was provided to the Ministry of Health in Ghana.

Background. Approximately 10-20% of children with sickle cell disease (SCD) develop stroke, but few consistent national estimates of the stroke burden for children with SCD exist. The purpose of this study is to determine the proportion of diagnosed stroke among African-American pediatric discharges with and without SCD. Procedure. Records for African-Americans aged 1-18 years in the Kids' Inpatient Database (KID) 1997-2012 with >= 1 ICD-9-CM diagnosis code for stroke were included. Data were weighted to provide national estimates. A total of 2,994 stroke cases among African-American children were identified. Diagnoses co-existing with ischemic or hemorrhagic stroke were frequency ranked separately. Results. From 1997 through 2012, SCD was present in 24% of stroke discharges, with 89% being ischemic stroke. For hospital discharges of African-American children, SCD is the highest co-existing risk factor for ischemic stroke (29%). Stroke in children with SCD occurred predominantly in children aged 5-9 years, older than previously reported. The trend of stroke discharges significantly decreased for children with SCD from 1997 to 2012 for children aged 10-14 years. Conclusions. SCD is a leading risk factor to pediatric stroke in African-American children. Reducing the number of strokes among children with SCD would have a significant impact on the rate of strokes among African-American children. Preventative intervention may be modifying initial age of presentation of stroke in children with SCD. (C) 2015 Wiley Periodicals, Inc.

BACKGROUND:Venous thromboembolism (VTE) is a significant clinical and public health concern. We evaluated a variety of multilevel factors--demographics, clinical and insurance status, preexisting comorbid conditions, and hospital characteristics--for VTE diagnosis among hospitalizations of US adults. METHODS:We generated adjusted odds ratios with 95% confidence intervals (CIs) and determined sources of outcome variation by conducting multilevel logistic regression analysis of data from the 2011 Nationwide Inpatient Sample that included 6,710,066 hospitalizations of US adults nested within 1,039 hospitals. RESULTS:Among hospitalizations of adults, age, sex, race or ethnicity, total days of hospital stay, status of health insurance, and operating room procedure were important determinants of VTE diagnosis; each of the following preexisting comorbid conditions--acquired immune deficiency syndrome, anemia, arthritis, congestive heart failure, coagulopathy, hypertension, lymphoma, metastatic cancer, other neurological disorders, obesity, paralysis, pulmonary circulation disorders, renal failure, solid tumor without metastasis, and weight loss--was associated independently with 1.04 (95% CI: 1.02-1.06) to 2.91 (95% CI: 2.81-3.00) times increased likelihood of VTE diagnosis than among hospitalizations of adults without any of these corresponding conditions. The presence of 2 or more of such conditions was associated a 180%-450% increased likelihood of a VTE diagnosis. Hospitalizations of adults who were treated in urban hospitals were associated with a 14%-15% increased likelihood of having a VTE diagnosis than those treated in rural hospitals. Approximately 7.4% of the total variation in VTE diagnosis occurred between hospitals. CONCLUSION:The presence of certain comorbidities and hospital contextual factors is associated with significantly elevated likelihood of VTE diagnosis among hospitalizations of adults. The findings of this study underscore the importance of clinical risk assessment and adherence to evidence-based clinical practice guidelines in preventing VTE, as well as the need to evaluate potential contextual factors that might modify the risk of VTE among hospitalized patients.

Abstract On-going public health surveillance efforts in sickle cell disease (SCD) are critical for understanding the course and outcomes of this disease over time. Once nearly universally fatal by adolescence, many patients are living well into adulthood and sometimes into retirement years. Previous SCD mortality estimates have relied on data from death certificates alone or from deaths of patients receiving care in high volume hematology clinics, resulting in gaps in reporting and potentially biased conclusions. The Registry and Surveillance System for Hemoglobinopathies (RuSH) project collected and linked population-based surveillance data on SCD in California and Georgia from a variety of sources for years 2004-2008. These data sources included administrative records, newborn screening reports and health insurance claims as well as case reports of adult and pediatric patients receiving care in the following large specialty treatment centers: Georgia Comprehensive Sickle Cell Center, Georgia Regents University, Georgia Comprehensive Sickle Cell Center at Grady Health Systems and Children's Healthcare of Atlanta in Georgia, and Children's Hospital Los Angeles and UCSF Benioff Children's Hospital Oakland in California. Cases identified from these combined data sources were linked to death certificates in CA and GA for the same years. Among 12,143 identified SCD cases, 640 were linked to death certificates. Combined SCD mortality rates by age group at time of death are compared to combined mortality rates for all African Americans living in CA and GA. (Figure 1). SCD death rates among children up to age 14 and among adults 65 and older were very similar to those of the overall African American population. In contrast, death rates from young adulthood to midlife were substantially higher in the SCD population. Overall, only 55% of death certificates linked to the SCD cases had SCD listed in any of the cause of death fields. Thirty-four percent (CA) and 37% (GA) had SCD as the underlying cause of death. An additional 22% and 20% (CA and GA, respectively) had underlying causes of death that were not unexpected for SCD patients, including related infections such as septicemia, pulmonary/cardiac causes of death, renal failure and stroke. The remaining 44% (CA) and 43% (GA) had underlying causes of death that were either not related to SCD (e.g., malignancies, trauma) or too vague to be associated with SCD (e.g., generalized pulmonary or cardiac causes of death. Figure 2 shows the number of deaths by state, age group at death and whether the underlying cause of death was SCD specific, potentially related to SCD or not clearly related to SCD. While the number of deaths was too small to use for life expectancy calculations, there were more deaths over age 40 than under age 40 during this five year period. This effort represents a novel, population-based approach to examine mortality in SCD patients. These data suggest that the use of death certificates alone to identify deceased cases may not capture all-cause mortality among all SCD patients. Additional years of surveillance are needed to provide better estimates of current life expectancy and the ability to track and monitor changes in mortality over time. On-going surveillance of the SCD population is required to monitor changes in mortality and other outcomes in response to changes in treatments, standards of care and healthcare policy and inform advocacy efforts. This work was supported by the US Centers for Disease Control and Prevention and the National Heart, Lung and Blood Institute, cooperative agreement numbers U50DD000568 and U50DD001008. Figure 1: SCD-Specific & Overall African American Mortality Rates in CA and GA, 2004 – 2008. Figure 1:. SCD-Specific & Overall African American Mortality Rates in CA and GA, 2004 – 2008. Figure 2: Deaths (Count) Among Individuals with SCD in CA and GA, by Age Group and Underlying Cause of Death, 2004-2008 (N=615) Figure 2:. Deaths (Count) Among Individuals with SCD in CA and GA, by Age Group and Underlying Cause of Death, 2004-2008 (N=615) Disclosures No relevant conflicts of interest to declare.

PurposeLong-term follow-up of newborn screening for conditions such as sickle cell disease can be conducted using linkages to population-based data. We sought to estimate childhood sickle cell disease mortality and risk factors among a statewide birth cohort with sickle cell disease identified through newborn screening.MethodsChildren with sickle cell disease identified by newborn screening and born to New York residents in 2000–2008 were matched to birth and death certificates. Mortality rates were calculated (using numbers of deaths and observed person-years at risk) and compared with mortality rates for all New York children by maternal race/ethnicity. Stratified analyses were conducted to examine associations between selected factors and mortality.ResultsAmong 1,911 infants with sickle cell disease matched to birth certificates, 21 deaths were identified. All-cause mortality following diagnosis was 3.8 per 1,000 person-years in the first 2 years of life and 1.0 per 1,000 person-years at ages 2–9 years. The mortality rate was significantly lower among children of foreign-born mothers and was significantly higher among preterm infants with low birth weight. The mortality rates were not significantly higher for infants after 28 days with sickle cell disease than for all New York births, but they were 2.7–8.4 times higher for children 1 through 9 years old with homozygous sickle cell disease than for those of all non-Hispanic black or Hispanic children born to New York residents.ConclusionEstimated mortality risk in children with homozygous sickle cell disease remains elevated even after adjustment for maternal race/ethnicity. These results provide evidence regarding the current burden of child mortality among children with sickle cell disease despite newborn screening.Genet Med17 6, 452–459.