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1.  The ImmProve Project: Leveraging electronic health record data to promote immunization delivery 
Academic pediatrics  2013;13(5):458-465.
Objective
Though an essential pediatric preventive service, immunizations are challenging to deliver reliably. Our objective was to measure the impact on pediatric immunization rates of providing clinicians with electronic health record-derived immunization prompting.
Methods
Operating in a large, urban, hospital-based pediatric primary care clinic, we evaluated 2 interventions to improve immunization delivery to children ages 2, 6, and 13 years: point-of-care, patient-specific electronic clinical decision support (CDS) when children overdue for immunizations presented for care and provider-specific bulletins listing children overdue for immunizations.
Results
Overall, the proportion of children up-to-date for a composite of recommended immunizations at ages 2, 6, and 13 years was not different in the intervention (CDS active) and historical control (CDS not active) periods; historical immunization rates were high. The proportion of children receiving 2 doses of hepatitis A immunization prior to their second birthday was significantly improved during the intervention period. Human papilloma virus (HPV) immunization delivery was low during both control and intervention periods and was unchanged for 13-year-olds. For 14-year-olds, however, 4 of the 5 highest quarterly rates of complete HPV immunization occurred in the final year of the intervention. Provider-specific bulletins listing children overdue for immunizations increased the likelihood of identified children receiving catch-up hepatitis A immunizations (hazard ratio: 1.32 [95% confidence interval (CI): 1.12–1.56]); results for HPV and the composite of recommended immunizations were of a similar magnitude but not statistically significant.
Conclusions
In our patient population, with high baseline uptake of recommended immunizations, electronic health record-derived immunization prompting had a limited effect on immunization delivery. Benefit was more clearly demonstrated for newer immunizations with lower baseline uptake.
doi:10.1016/j.acap.2013.03.004
PMCID: PMC3769502  PMID: 23726754
immunizations; clinical decision support systems; quality improvement
2.  Epidemiological Evidence for Possible Radiation Hormesis from Radon Exposure: A Case-Control Study Conducted in Worcester, MA. 
Dose-Response  2010;9(1):59-75.
Data from a case-control study of lung cancer and residential radon exposure conducted in Worcester County, Massachusetts, are presented. Lung cancer risk was estimated using conditional logistic regression models that controlled for demographic, smoking, and occupational exposure covariates. Preliminary exploratory analyses using lowess smoothing revealed a non-linear association between exposure and the log odds of lung cancer. Radon exposure was considered by using linear spline terms in order to model this nonlinearity. The best fit of this linear spline model to these data predicted a shift from a positive to a negative slope in the log-odds of lung cancer at a radon concentration of 70 Bq m−3. A statistically significant decrease in cancer risk with increased exposure was found for values ≤ 157 Bq m−3 normalized to the reference exposure of 4.4 Bq m−3, the lowest radon concentration measured(adjusted odds ratio (AOR) [95% CI] = 0.42 [0.180, 1.00], p = 0.049). This result is consistent with those reported elsewhere that considered radon exposure with cubic spline terms (Thompson, RE et al. 2008). Furthermore, this model predicts an AOR that is numerically less than 1.0 for radon exposures up to 545 Bq m−3 versus the above baseline, reference exposure.
doi:10.2203/dose-response.10-026.Thompson
PMCID: PMC3057636  PMID: 21431078
Radon; Adaptive Response; Hormesis; Lung Cancer
3.  Factors Associated with Second Hand Smoke Exposure In Young Inner City Children with Asthma 
Objectives
To examine the association of social and environmental factors with levels of second hand smoke (SHS) exposure, as measured by salivary cotinine, in young inner city children with asthma.
Methods
We used data drawn from a home-based behavioral intervention for young high risk children with persistent asthma post emergency department (ED) treatment (N=198). SHS exposure was measured by salivary cotinine and caregiver report. Caregiver demographic and psychological functioning, household smoking behavior and asthma morbidity were compared with child cotinine concentrations. Chi-square and ANOVA tests and multivariate regression models were used to determine the association between cotinine concentrations with household smoking behavior and asthma morbidity.
Results
Over half (53%) of the children had cotinine levels compatible with SHS exposure and mean cotinine concentrations were high at 2.42 ng/ml (SD 3.2). The caregiver was the predominant smoker in the home (57%) and (63%) reported a total home smoking ban. Preschool age children, and those with caregivers reporting depressive symptoms and high stress had higher cotinine concentrations than their counterparts. Among children living in a home with a total home smoking ban, younger children had significantly higher mean cotinine concentration than older children (Cotinine: 3–5 year olds, 2.24 ng/ml (SD 3.5); 6–10 year olds, 0.63 ng/ml (SD 1.0); p <0.05). In multivariate models, the factors most strongly associated with high child cotinine concentrations were increased number of household smokers (β = 0.24) and younger child age (3–5 years) (β = 0.23; P <0.001, R2 = 0.35).
Conclusion
Over half of young inner-city children with asthma were exposed to second hand smoke and caregivers are the predominant household smoker. Younger children and children with depressed and stressed caregivers are at significant risk of smoke exposures, even when a household smoking ban is reported. Further advocacy for these high-risk children is needed to help caregivers quit and to mitigate smoke exposure.
doi:10.3109/02770903.2011.576742
PMCID: PMC3113681  PMID: 21545248
asthma; children; cotinine; second hand smoke
4.  Precise determination of the erythema response of human skin to ultraviolet radiation and quantification of effects of protectors 
Summary
Background
We describe highly reproducible methods for quantifying the erythema response of precisely selected areas (spots) of human skin to graded doses of ultraviolet radiation (UVR). These methods have permitted evaluation of the efficacy of protectors, such as sulforaphane from crucifers, that defend cells through induction of cytoprotective (phase 2) genes.
Methods
Spots on the back were precisely located by opaque, adhesive, vinyl templates provided with 16 circular, 2.0 cm diameter occludable windows. Doses (100–800 mJ/cm2) of narrow-band (311 nm) UVR were administered, and the erythema index (a*) was measured with a chromometer on treated and control areas, before and after radiation.
Results
Daily variations in basal a* values of each spot were much smaller than the differences of a* values among spots of one individual, or those of corresponding spots among different individuals. The increments in erythema responses to UVR (Δa*) were similar despite large variations of basal a* of spots. The most appropriate measure of UVR-evoked erythema is therefore the Δa* value for each spot, which is an independent observational entity. Δa* was proportional to UVR dose, and independent of spot location. To evaluate effectiveness of protectors against UVR damage we paired horizontally adjacent spots for treatment and controls. Vertical or random spot pairing did not provide significantly higher consistency. Protective efficacy against UVR erythema is appropriately expressed as percent reduction in Δa* values upon treatment with inducers.
Conclusions
The protection of skin against UVR damage can be quantified precisely from changes in erythema index (Δa*) obtained with a chromometer.
doi:10.1111/j.1600-0781.2009.00404.x
PMCID: PMC3236561  PMID: 19152516
analysis of variance (ANOVA); erythema index; sulforaphane
5.  Renal Injury Is Associated with Operative Mortality after Cardiac Surgery for Women and Men 
Objectives
The purpose of this study was to determine whether acute renal injury develops more frequently in women than in men after cardiac surgery and whether this complication is associated with operative mortality in women.
Methods
Prospectively collected data were evaluated from 9461 coronary artery bypass graft and/or cardiac valve surgery patients (3080 women) not on preoperative dialysis. Glomerular filtration rate (eGFR) was estimated by the Modification of Diet in Renal Disease equations using the last plasma creatinine before surgery (baseline) and the highest level of the first postoperative week. The primary renal injury outcome was the composite endpoint of renal injury according to RIFLE criteria (eGFR decrease > 50% from baseline) or failure.
Results
Thirty-day operative mortality and renal injury were more common in women than in men (5.9% vs 2.8%, P=0.01; 5.1% vs 3.6%, P<0.001, respectively). Nonetheless, patient sex was not independently associated with risk for renal injury when baseline eGFR was included in multivariate modeling. Perioperative complications, ICU length of stay, and mortality were more frequent for patients with than without renal injury (women, 20.6% vs 3.2%, P<0.0001; men, 18.3% vs 2.2%, P<0.001). Renal injury was independently associated with 30-day mortality for women (OR, 3.96; 95% CI, 1.86–8.44, P<0.0001) and men (OR, 4.05; 95% CI, 2.19–7.48, P<0.0001).
Conclusions
Postoperative renal injury is independently associated with 30-day mortality regardless of patient sex. Higher rates of renal injury in women compared with men might be explained in part by a higher prevalence of low eGFR before surgery.
Ultra-Mini Abstract
Renal injury is more common in women than in men after cardiac surgery. Regardless of patient sex, the development of this complication is independently associated with elevated 30-day mortality.
doi:10.1016/j.jtcvs.2010.02.021
PMCID: PMC2904436  PMID: 20381074
6.  Lead Exposure during Synaptogenesis Alters Vesicular Proteins and Impairs Vesicular Release: Potential Role of NMDA Receptor–Dependent BDNF Signaling 
Toxicological Sciences  2010;116(1):249-263.
Lead (Pb2+) exposure is known to affect presynaptic neurotransmitter release in both in vivo and cell culture models. However, the precise mechanism by which Pb2+ impairs neurotransmitter release remains unknown. In the current study, we show that Pb2+ exposure during synaptogenesis in cultured hippocampal neurons produces the loss of synaptophysin (Syn) and synaptobrevin (Syb), two proteins involved in vesicular release. Pb2+ exposure also increased the number of presynaptic contact sites. However, many of these putative presynaptic contact sites lack Soluble NSF attachment protein receptor complex proteins involved in vesicular exocytosis. Analysis of vesicular release using FM 1-43 dye confirmed that Pb2+ exposure impaired vesicular release and reduced the number of fast-releasing sites. Because Pb2+ is a potent N-methyl-D-aspartate receptor (NMDAR) antagonist, we tested the hypothesis that NMDAR inhibition may be producing the presynaptic effects. We show that NMDAR inhibition by aminophosphonovaleric acid mimics the presynaptic effects of Pb2+ exposure. NMDAR activity has been linked to the signaling of the transsynaptic neurotrophin brain-derived neurotrophic factor (BDNF), and we observed that both the cellular expression of proBDNF and release of BDNF were decreased during the same period of Pb2+ exposure. Furthermore, exogenous addition of BDNF rescued the presynaptic effects of Pb2+. We suggest that the presynaptic deficits resulting from Pb2+ exposure during synaptogenesis are mediated by disruption of NMDAR-dependent BDNF signaling.
doi:10.1093/toxsci/kfq111
PMCID: PMC2886862  PMID: 20375082
lead; BDNF; vesicular release; synaptophysin; synaptobrevin; FM 1-43
7.  DNA promoter hypermethylation of p16 and APC predicts neoplastic progression in Barrett’s esophagus 
Introduction
Prediction of progression to cancer in patients with Barrett’s esophagus is difficult using current techniques. We determined whether DNA promoter hypermethylation of genes frequently methylated in esophageal adenocarcinoma (p16 and APC) could be used as predictors of progression in Barrett’s esophagus.
Methods
We first performed a cross-sectional study to evaluate the prevalence of gene hypermethylation in biopsies from patients with normal esophagus (n=17), Barrett’s esophagus (n=102), and adenocarcinoma (n=42). We then performed a nested case-control study comparing gene hypermethylation in Barrett’s esophagus patients who progressed from baseline pathology to high-grade dysplasia or cancer (n=7) versus patients who did not progress (n=50).
Results
None of the patients with normal esophagus had p16 or APC hypermethylation. Hypermethylation was prevalent in Barrett’s esophagus without dysplasia or low-grade dysplasia (p16=31% and APC=50%; p<0.01) and high-grade dysplasia or adenocarcinoma (p16=54% and APC=68%; p<0.001) compared to normal esophagus (not detected). Patients who progressed from baseline pathology to high-grade dysplasia or cancer had higher prevalence of hypermethylation in their initial esophagus biopsies compared to those who did not progress for both p16 (100% vs. 33%; p=0.008) and APC (86% vs. 40%; p=0.02). Hypermethylation of both p16 and APC was a strong predictor of subsequent progression to cancer during a mean follow-up time of 4.1 years (adjusted OR [95% CI]=14.97 [1.73,inf], p=0.01). Among patients who were negative for both p16 and APC hypermethylation, none progressed from baseline pathology to high-grade dysplasia or cancer.
Conclusions
Hypermethylation of both p16 and APC strongly predicts progression to high-grade dysplasia or cancer in patients with Barrett’s esophagus. Absence of p16 and APC hypermethylation is associated with a benign course.
doi:10.1038/ajg.2009.300
PMCID: PMC3090447  PMID: 19584833
8.  Human Lung Cancer Risks from Radon – Part III - Evidence of Influence of Combined Bystander and Adaptive Response Effects on Radon Case-Control Studies - A Microdose Analysis 
Dose-Response  2010;10(3):415-461.
Since the publication of the BEIR VI (1999) report on health risks from radon, a significant amount of new data has been published showing various mechanisms that may affect the ultimate assessment of radon as a carcinogen, in particular the potentially deleterious Bystander Effect (BE) and the potentially beneficial Adaptive Response radio-protection (AR). The case-control radon lung cancer risk data of the pooled 13 European countries radon study (Darby et al 2005, 2006) and the 8 North American pooled study (Krewski et al 2005, 2006) have been evaluated. The large variation in the odds ratios of lung cancer from radon risk is reconciled, based on the large variation in geological and ecological conditions and variation in the degree of adaptive response radio-protection against the bystander effect induced lung damage. The analysis clearly shows Bystander Effect radon lung cancer induction and Adaptive Response reduction in lung cancer in some geographical regions. It is estimated that for radon levels up to about 400 Bq m−3 there is about a 30% probability that no human lung cancer risk from radon will be experienced and a 20% probability that the risk is below the zero-radon, endogenic spontaneous or perhaps even genetically inheritable lung cancer risk rate. The BEIR VI (1999) and EPA (2003) estimates of human lung cancer deaths from radon are most likely significantly excessive. The assumption of linearity of risk, by the Linear No-Threshold Model, with increasing radon exposure is invalid.
doi:10.2203/dose-response.09-059.Leonard
PMCID: PMC3430402  PMID: 22942874
Radon Case-Control Studies; Non-Linearity Adaptive Response Bystander Effects
9.  Human Lung Cancer Risks from Radon – Part I - Influence from Bystander Effects - A Microdose Analysis 
Dose-Response  2010;9(2):243-292.
Since the publication of the BEIR VI report in 1999 on health risks from radon, a significant amount of new data has been published showing various mechanisms that may affect the ultimate assessment of radon as a carcinogen, at low domestic and workplace radon levels, in particular the Bystander Effect (BE) and the Adaptive Response radio-protection (AR). We analyzed the microbeam and broadbeam alpha particle data of Miller et al. (1995, 1999), Zhou et al. (2001, 2003, 2004), Nagasawa and Little (1999, 2002), Hei et al. (1999), Sawant et al. (2001a) and found that the shape of the cellular response to alphas is relatively independent of cell species and LET of the alphas. The same alpha particle traversal dose response behavior should be true for human lung tissue exposure to radon progeny alpha particles. In the Bystander Damage Region of the alpha particle response, there is a variation of RBE from about 10 to 35. There is a transition region between the Bystander Damage Region and Direct Damage Region of between one and two microdose alpha particle traversals indicating that perhaps two alpha particle “hits” are necessary to produce the direct damage. Extrapolation of underground miners lung cancer risks to human risks at domestic and workplace levels may not be valid.
doi:10.2203/dose-response.09-057.Leonard
PMCID: PMC3118770  PMID: 21731539
Radon Lung cancer; Bystander; Adaptive Response; Case-control Studies
10.  Development and validation of the Family Decision-Making Self-Efficacy Scale 
Objective
Several studies have reported high levels of distress in family members who have made health care decisions for loved ones at the end of life. A method is needed to assess the readiness of family members to take on this important role. Therefore, the purpose of this study was to develop and validate a scale to measure family member confidence in making decisions with (conscious patient scenario) and for (unconscious patient scenario) a terminally ill loved one.
Methods
On the basis of a survey of family members of patients with amyotrophic lateral sclerosis (ALS) enriched by in-depth interviews guided by Self-Efficacy Theory, we developed six themes within family decision making self-efficacy. We then created items reflecting these themes that were refined by a panel of end-of-life research experts. With 30 family members of patients in an outpatient ALS and a pancreatic cancer clinic, we tested the tool for internal consistency using Cronbach’s alpha and for consistency from one administration to another using the test–retest reliability assessment in a subset of 10 family members. Items with item to total scale score correlations of less than .40 were eliminated.
Results
A 26-item scale with two 13-item scenarios resulted, measuring family self-efficacy in decision making for a conscious or unconscious patient with a Cronbach’s alphas of .91 and .95, respectively. Test–retest reliability was r = .96, p = .002 in the conscious senario and r = .92, p = .009 in the unconscious scenario.
Significance of results
The Family Decision-Making Self-Efficacy Scale is valid, reliable, and easily completed in the clinic setting. It may be used in research and clinical care to assess the confidence of family members in their ability to make decisions with or for a terminally ill loved one.
doi:10.1017/S1478951509990241
PMCID: PMC2814174  PMID: 19788773
Decision making; Self-efficacy; End of life; Family; Scale
11.  Human Lung Cancer Risks from Radon – Part II – Influence from Combined Adaptive Response and Bystander Effects – A Microdose Analysis 
Dose-Response  2010;9(4):502-553.
In the prior Part I, the potential influence of the low level alpha radiation induced bystander effect (BE) on human lung cancer risks was examined. Recent analysis of adaptive response (AR) research results with a Microdose Model has shown that single low LET radiation induced charged particles traversals through the cell nucleus activates AR. We have here conducted an analysis based on what is presently known about adaptive response and the bystander effect (BE) and what new research is needed that can assist in the further evaluation human cancer risks from radon. We find that, at the UNSCEAR (2000) worldwide average human exposures from natural background and man-made radiations, the human lung receives about a 25% adaptive response protection against the radon alpha bystander damage. At the UNSCEAR (2000) minimum range of background exposure levels, the lung receives minimal AR protection but at higher background levels, in the high UNSCEAR (2000) range, the lung receives essentially 100% protection from both the radon alpha damage and also the endogenic, spontaneously occurring, potentially carcinogenic, lung cellular damage.
doi:10.2203/dose-response.09-058.Leonard
PMCID: PMC3315171  PMID: 22461760
12.  Kindergarten Predictors of Math Learning Disability 
The aim of the present study was to address how to effectively predict mathematics learning disability (MLD). Specifically, we addressed whether cognitive data obtained during kindergarten can effectively predict which children will have MLD in third grade, whether an abbreviated test battery could be as effective as a standard psychoeducational assessment at predicting MLD, and whether the abbreviated battery corresponded to the literature on MLD characteristics. Participants were 226 children who enrolled in a 4-year prospective longitudinal study during kindergarten. We administered measures of mathematics achievement, formal and informal mathematics ability, visual-spatial reasoning, and rapid automatized naming and examined which test scores and test items from kindergarten best predicted MLD at grades 2 and 3. Statistical models using standardized scores from the entire test battery correctly classified ~80–83 percent of the participants as having, or not having, MLD. Regression models using scores from only individual test items were less predictive than models containing the standard scores, except for models using a specific subset of test items that dealt with reading numerals, number constancy, magnitude judgments of one-digit numbers, or mental addition of one-digit numbers. These models were as accurate in predicting MLD as was the model including the entire set of standard scores from the battery of tests examined. Our findings indicate that it is possible to effectively predict which kindergartners are at risk for MLD, and thus the findings have implications for early screening of MLD.
doi:10.1111/j.1540-5826.2005.00129.x
PMCID: PMC2806680  PMID: 20084182
13.  Risk Factors for Neurocognitive Dysfunction After Cardiac Surgery in Postmenopausal Women 
The Annals of thoracic surgery  2008;86(2):511-516.
Background
Women are at higher risk than men for neurologic complications from cardiac operations. This study identified risk factors for neurocognitive dysfunction after cardiac operations in elderly women.
Methods
One hundred thirteen postmenopausal women undergoing primary coronary artery bypass grafting, with or without valve operation, underwent psychometric testing and neurologic evaluation the day before operation and 4 to 6 weeks postoperatively. Risk factors assessed for neurologic complications included atherosclerosis of the ascending aorta and apolipoprotein ε4 genotype. Postoperative neurocognitive dysfunction was defined as the composite end point of a one standard deviation decrement from baseline on two or more psychometric tests or a new neurologic deficit.
Results
Neurocognitive dysfunction was present in 25% of the women 4 to 6 weeks postoperatively. Women with a neurocognitive deficit tended to be older than those without a deficit (72.1 ± 8.1 vs 69.4 ± 8.9 years, p = 0.144) and were more likely to have mild atherosclerosis of the ascending aorta, a history of congestive heart failure, longer duration of cardiopulmonary bypass (CPB) and aortic cross-clamping, lower nadir blood pressure during CPB, higher rates of postoperative atrial fibrillation, and longer postoperative hospitalization. Mild atherosclerosis of the ascending aorta, duration of CPB, duration of aortic cross-clamping (p = 0.051), and length of postsurgical hospitalization were independently associated with postoperative neurocognitive dysfunction.
Conclusions
Mild atherosclerosis of the ascending aorta, duration of CPB, aortic cross-clamping time, and length of hospitalization, but not apolipoprotein ε4 genotype, identified risk for neurocognitive dysfunction after cardiac operation in postmenopausal women.
doi:10.1016/j.athoracsur.2008.04.058
PMCID: PMC2784735  PMID: 18640325
14.  Autism Spectrum Disorder in Fragile X Syndrome: A Longitudinal Evaluation 
The present study extends our previous work on characterizing the autistic behavior profile of boys with fragile X syndrome (FXS) who meet Diagnostic and Statistical Manual for Mental Disorders, 4th Edition criteria for autism spectrum disorder (ASD) into a longitudinal evaluation of ASD in FXS (FXS+ASD). Specifically, we aimed to determine the stability of the diagnosis and profile of ASD in FXS over time. Through regression models, we also evaluated which autistic and social behaviors and skills were correlates of diagnosis and autistic behavior severity (i.e., Autism Diagnostic Interview-Revised total scores). Finally, we assessed the evolution of cognitive parameters in FXS+ASD. A population of 56 boys (30–88 months at baseline) with FXS was evaluated using measures of autistic, social, and cognitive behaviors and skills at three yearly evaluations. We found that the diagnosis of ASD in FXS was relatively stable over time. Further emphasizing this stability, we found a set of behaviors and skills, particularly those related to peer relationships and adaptive socialization, that differentiated FXS+ASD from the rest of the FXS cohort (FXS+None) and contributed to autistic severity at all time points. Nevertheless, the general improvement in autistic behavior observed in FXS+ASD coupled with the concurrent worsening in FXS+None resulted in less differentiation between the groups over time. Surprisingly, FXS+ASD IQ scores were stable while FXS+None non-verbal IQ scores declined. Our findings indicate that ASD is a distinctive subphenotype in FXS characterized by deficits in complex social interaction, with similarities to ASD in the general population.
doi:10.1002/ajmg.a.32848
PMCID: PMC2734278  PMID: 19441123
Fragile X; autism; longitudinal; Autism Diagnostic Interview; adaptive socialization
15.  When Patients Lack Capacity 
Current approaches to end-of-life decision making are widely considered inadequate. We explored these complexities by examining how patients with terminal diagnoses would choose to involve their physicians and loved ones in making medical decisions, assuming they were able and unable to participate. Cross-sectional interviews of 130 patients recently diagnosed with fatal conditions were conducted. Patients were recruited from two academic medical centers using a modification of the Decision Control Preferences Scale, ranging from independent decision making to decision making that relies upon others. Patients were asked how they would balance their own wishes relative to the input of physician and loved ones in making medical decisions, and to weigh the input of loved ones relative to physician. Most patients (52%), assuming they had the capacity, would opt to share decision making with their physicians, but 15% would defer to their physicians and 34% would make decisions independently. Similarly, 44% would share decision making with their loved ones, but fewer (6%) would defer to their loved ones. Thirty-nine percent would rely upon their physicians’ judgments about what would be best for them rather than their own wishes if they became unconscious, compared with 15% who would do so if they were conscious (P < 0.001). Nonetheless, patients were more likely to weigh their loved ones’ input more heavily than their physicians’ input if they were unconscious (33%) than if they were conscious (7%, P = 0.05). Race, religion, gender, diagnosis, and health status were largely unassociated with patients’ decision control preferences. Patients with terminal diagnoses report a wide diversity of decision control preferences, but most would opt to share decision making with their physicians and loved ones. If unable to decide for themselves, they shift toward greater reliance on physician input relative to their own wishes but would weigh loved ones’ input more heavily than physician input. Deciding for patients who cannot speak for themselves may be more complex than has previously been reflected in law, policy, or clinical ethics.
doi:10.1016/j.jpainsymman.2005.04.010
PMCID: PMC2604910  PMID: 16256898
Ethics; end of life; decision making; advance care planning
16.  How Would Terminally Ill Patients Have Others Make Decisions for Them in the Event of Decisional Incapacity? A Longitudinal Study 
OBJECTIVES
To determine the role terminally ill patients would opt to have their loved ones and physicians play in healthcare decisions should they lose decision-making capacity and how this changes over time.
DESIGN
Serial interviews.
SETTING
The study institutions were The Johns Hopkins Medical Institutions in Baltimore, Maryland, and St. Vincent’s Hospital, in New York.
PARTICIPANTS
One hundred forty-seven patients with cancer, amyotrophic lateral sclerosis, or heart failure, at baseline and 3 and 6 months.
RESULTS
Patients’ baseline decision control preferences varied widely, but most opted for shared decision-making, leaning slightly toward independence from their loved ones. This did not change significantly at 3 or 6 months. Fiftyseven percent opted for the same degree of decision control at 3 months as at baseline. In a generalized estimating equation model adjusted for time, more-independent decision-making was associated with college education (P =.046) and being female (P =.01), whereas more-reliant decision-making was associated with age (P<.001). Patients leaned toward more reliance upon physicians to make best-interest determinations at diagnosis but opted for physicians to decide based upon their own independent wishes (substituted judgment) over time, especially if college educated.
CONCLUSION
Terminally ill patients vary in how much they wish their own preferences to control decisions made on their behalf, but most would opt for shared decisionmaking with loved ones and physicians. Control preferences are stable over time with respect to loved ones, but as they live longer with their illnesses, patients prefer somewhat less reliance upon physicians.
doi:10.1111/j.1532-5415.2007.01473.x
PMCID: PMC2583169  PMID: 18031490
decision-making; end of life; decisional incapacity; surrogates; ethics
17.  Family health care decision making and self-efficacy with patients with ALS at the end of life 
Palliative & supportive care  2008;6(3):273-280.
Objective:
Persons with ALS differ from those with other terminal illnesses in that they commonly retain capacity for decision making close to death. The role patients would opt to have their families play in decision making at the end of life may therefore be unique. This study compared the preferences of patients with ALS for involving family in health care decisions at the end of life with the actual involvement reported by the family after death.
Methods:
A descriptive correlational design with 16 patient–family member dyads was used. Quantitative findings were enriched with in-depth interviews of a subset of five family members following the patient's death.
Results:
Eighty-seven percent of patients had issued an advance directive. Patients who would opt to make health care decisions independently (i.e., according to the patient's preferences alone) were most likely to have their families report that decisions were made in the style that the patient preferred. Those who preferred shared decision making with family or decision making that relied upon the family were more likely to have their families report that decisions were made in a style that was more independent than preferred. When interviewed in depth, some family members described shared decision making although they had reported on the survey that the patient made independent decisions.
Significance of results:
The structure of advance directives may suggest to families that independent decision making is the ideal, causing them to avoid or underreport shared decision making. Fear of family recriminations may also cause family members to avoid or underreport shared decision making. Findings from this study might be used to guide clinicians in their discussions of treatments and health care decision making with persons with ALS and their families.
doi:10.1017/S1478951508000412
PMCID: PMC2572768  PMID: 18662421
Decision making; End of life; Family; Ethics
18.  Abnormalities of Cholesterol Metabolism in Autism Spectrum Disorders 
Although Smith-Lemli-Opitz Syndrome (SLOS), a genetic condition of impaired cholesterol biosynthesis, is associated with autism [Tierney et al., 2001], the incidence of SLOS and other sterol disorders among individuals with autism spectrum disorders (ASD) is unknown. This study investigated 1) the incidence of biochemically diagnosed SLOS in blood samples from a cohort of subjects with ASD from families in which more than one individual had ASD and 2) the type and incidence of other sterol disorders in the same group. Using gas chromatography/mass spectrometry, cholesterol and its precursor sterols were quantified in one hundred samples from subjects with ASD obtained from the Autism Genetic Resource Exchange (AGRE) specimen repository. Although no sample had sterol levels consistent with SLOS, 19 samples had total cholesterol levels lower than 100 mg/dL, which is below the 5th centile for children over age 2 years. These findings suggest that, in addition to SLOS, there may be other disorders of sterol metabolism or homeostasis associated with ASD.
doi:10.1002/ajmg.b.30368
PMCID: PMC2553243  PMID: 16874769
Smith-Lemli-Opitz syndrome; hypocholesterolemia; Autism Genetic Resource Exchange; Asperger disorder; pervasive developmental disorder
19.  Effectiveness of Nebulizer Use–Targeted Asthma Education on Underserved Children With Asthma 
Objective
To determine the effectiveness of a home-based asthma education intervention in increasing appropriate nebulizer use and reducing symptom frequency, emergency department (ED) visits, and hospitalizations over 12 months.
Design
A randomized clinical trial.
Settings
Pediatric primary care, pulmonary/allergy, and ED practices associated with the University of Maryland Medical System and The Johns Hopkins Hospital, Baltimore.
Participants
Children with persistent asthma, aged 2 to 9 years, with regular nebulizer use and an ED visit or hospitalization within the past 12 months. Children were randomized into the intervention (n=110) or control (n=111) group. Follow-up data were available for 95 intervention and 86 control children.
Intervention
Home-based asthma education, including symptom recognition, home treatment of acute symptoms, appropriate asthma medication, and nebulizer practice.
Main Outcome Measures
Estimates of mean differences in asthma symptom frequency, number of ED visits and hospitalizations and appropriate quick relief, controller medication, and nebulizer practice over 12 months.
Results
Of the 221 children, 181 (81.9%) completed the study. There were no significant differences in home nebulizer practice, asthma morbidity, ED visits, or hospitalizations between groups (P range, .11–.79). Although most children received appropriate nonurgent asthma care (mean, 2 visits per 6 months), more than one third of all children received at least 6 quick-relief medication prescriptions during 12 months, with no difference by group.
Conclusions
A nebulizer education intervention had no effect on asthma severity or health care use. Of concern is the high quick-relief and low controller medication use in young children with asthma seen nearly every 3 months for nonurgent care.
doi:10.1001/archpedi.160.6.622
PMCID: PMC2269706  PMID: 16754825

Results 1-19 (19)