Self-reported responsiveness to direct-to-consumer drug advertising and medication use: results of a national survey.

ABSTRACT: BACKGROUND: Direct-to-consumer (DTC) marketing of pharmaceuticals is controversial, yet effective. Little is known relating patterns of medication use to patient responsiveness to DTC. METHODS: We conducted a secondary analysis of data collected in national telephone survey on knowledge of and attitudes toward DTC advertisements. The survey of 1081 U.S. adults (response rate = 65%) was conducted by the Food and Drug Administration (FDA). Responsiveness to DTC was defined as an affirmative response to the item: "Has an advertisement for a prescription drug ever caused you to ask a doctor about a medical condition or illness of your own that you had not talked to a doctor about before?" Patients reported number of prescription and over-the-counter (OTC) medicines taken as well as demographic and personal health information. RESULTS: Of 771 respondents who met study criteria, 195 (25%) were responsive to DTC. Only 7% respondents taking no prescription were responsive, whereas 45% of respondents taking 5 or more prescription medications were responsive. This trend remained significant (p trend .0009) even when controlling for age, gender, race, educational attainment, income, self-reported health status, and whether respondents "liked" DTC advertising. There was no relationship between the number of OTC medications taken and the propensity to discuss health-related problems in response to DTC advertisements (p = .4). CONCLUSION: There is a strong cross-sectional relationship between the number of prescription, but not OTC, drugs used and responsiveness to DTC advertising. Although this relationship could be explained by physician compliance with patient requests for medications, it is also plausible that DTC advertisements have a particular appeal to patients prone to taking multiple medications. Outpatients motivated to discuss medical conditions based on their exposure to DTC advertising may require a careful medication history to evaluate for therapeutic duplication or overmedication.

Cardiovascular abnormalities, interventions, and long-term outcomes in infantile Williams syndrome.

OBJECTIVE: To determine the prevalence of cardiovascular abnormalities (CVA) and outcomes in patients with Williams syndrome presenting before 1 year of age. STUDY DESIGN: A retrospective review was undertaken of consecutive patients with WS at our institution from January 1, 1980, through December 31, 2007. WS was diagnosed by an experienced medical geneticist and/or by fluorescence in situ hybridization. CVA were diagnosed with the use of echocardiography, cardiac catheterization, or computerized tomographic angiography. Freedom from intervention was determined using Kaplan-Meier analysis. RESULTS: The study group was 129 patients with CVA. Age at presentation was 127 +/- 116 days, with follow-up of 8.0 +/- 7.5 years (0 to 42 years). The most common lesions were peripheral pulmonary artery stenosis (62%) and supravalvar aortic stenosis (57%). Other CVA were common. CV interventions were performed in 29%, with 58% of those before 1 year. Freedom from intervention was 85%, 73%, and 66% at 1, 5, and 25 years, respectively. Four patients died. CONCLUSIONS: CVA are the most common manifestations of infantile Williams syndrome and occur with greater frequency than previously reported. In those with CVA, interventions are common and usually occur by 5 years of age. Most of these patients do not require intervention on long-term follow-up, and overall mortality is low.

Surfactant protein A2 polymorphisms and disease severity in a respiratory syncytial virus-infected population.

OBJECTIVE: To examine whether genetic variations within the surfactant protein A2 (SP-A2) gene are associated with respiratory syncytial virus (RSV) disease severity in infected children. STUDY DESIGN: Naturally infected children aged < or =24 months were prospectively enrolled in 3 RSV seasons. SP-A2 genotyping was performed. Independent clinical predictors of disease severity were analyzed. The association of SP-A2 genetic diversity and disease severity was tested by using multivariate logistic regression models and 4 levels of disease gradation as outcome measures. RESULTS: Homozygosity of the 1A(0) allele was protective against hospitalization (odds ratio [OR] = 0.15, P = .0010). This remained significant in African American patients (OR = 0.24, P = .042) and Caucasian patients (OR = 0.05, P = .021) after adjustment for other co-variates. Hospitalized children with the 1A(2) allele demonstrated significant protection from severe disease with univariate analyses, but only a trend for protection with multivariate analyses. Patients homozygous or heterozygous for an asparagine at amino acid position 9 were twice or more likely to need intensive care unit admission (OR = 2.15, P = .022), require intubation (OR = 3.04, P = .005), and have a hospitalization lasting > or =4 days (OR = 1.89, P = .02) compared with children homozygous for a threonine at this position. CONCLUSIONS: SP-A2 polymorphisms are associated with the severity of RSV infection in infants.

Association of birth weight with polymorphisms in the IGF2, H19, and IGF2R genes.

There is a substantial genetic component for birth weight variation. We tested 18 single nucleotide polymorphisms (SNPs) in the IGF2, H19, and IGF2R genes for associations with birth weight variation in 342 mother-newborn pairs (birth weight 2.1-4.7 kg at term) and 527 parent-newborn trios (birth weight 2.1-5.1 kg) across three localities. SNPs in the IGF2R (rs8191754; maternal genotype), IGF2 (rs3741205; newborn genotype), and 5' region of the H19 (rs2067051, rs2251375, and rs4929984) genes were associated with birth weight. Detailed analyses to distinguish direct maternal, direct newborn, and parent of origin effects for the most strongly associated H19 SNP (rs4929984) determined that the association of maternal genotype with newborn birth weight was due to parent of origin effects not direct maternal effects. That SNP is located near the CTCF binding sites that influence expression of the maternally imprinted IGF2 and paternally imprinted H19 locus, and there are statistically significant and independent opposite effects of the same rs4929984 allele, depending on the parent from which it was inherited.

Arterial stiffness is increased in American adolescents compared to Japanese counterparts.

Cardiovascular disease is increased in US groups versus Japanese counterparts. Increased arterial stiffness is an important predictor of cardiovascular risk. Pulse wave velocity correlates well with arterial stiffness. Gender and ethnic differences in biracial US adolescent groups have been described. No data are available evaluating differences in arterial stiffness between US and Japanese subjects. Previously published data from an adolescent (12-17 years of age) Japanese cohort were used as an historical control and were compared to an adolescent cohort from the United States. The same simple noninvasive oscillometric technique was used in each cohort to measure brachial-ankle pulse wave velocity (baPWV) as an index of arterial stiffness. The US group was a cross-sectional, biracial (64% African American, 56% female) sample of 162 subjects. The Japanese group was a cross-sectional (48% female) sample of 820 Japanese subjects. All subjects in both cohorts were normotensive (BP < 95% for gender, height, and age) adolescents (12-17 years of age). Subjects were analyzed in four groups on the basis of gender and age (12-14 and 15-17 years of age). In both individual cohorts, the mean baPWV was higher in males versus females and the baPWV increased with age. The mean baPWV was higher in all US groups versus Japanese counterparts (p < 0.0001). The mean systolic and diastolic blood pressures were higher in all Japanese groups versus US counterparts (p < 0.005). Differences in arterial stiffness are present and detectable between normotensive US and Japanese adolescent subjects. Increased arterial stiffness among these adolescent groups correlates with known adult risk for cardiovascular events among the same ethnic and gender groups.

Ambulatory blood pressure and increased left ventricular mass in children at risk for hypertension.

OBJECTIVE: To relate ambulatory blood pressure (ABP) to cardiac target organ measurement in children at risk for primary hypertension (HTN). STUDY DESIGN: Left ventricular mass index (LVMI) and ABP were measured concomitantly in children (6 to 18 years) at risk for hypertension using a cross-sectional study design. RESULTS: LVMI showed a significant positive correlation with 24-hour systolic blood pressure (SBP) load, SBP index (SBPI), and standard deviation score (SDS). When subjects were stratified by LVMI percentile, there were significant differences in SBP load, 24-hour SBPI, and 24-hour SSDS. The odds ratio (OR) of having elevated LVMI increased by 54% for each incremental increase of SDS in 24-hour SSDS after controlling for race and BMI (OR = 1.54, unit = 1 SDS, CI = 1.1, 2.15, P = .011) and increased by 88% for each increase of 0.1 in BPI (OR = 1.88, CI = 1.03, 3.45, P = .04). Subjects with stage 3 HTN had significantly greater mean LVMI compared with normal subjects (P = .002 by ANOVA; LMVI, 31.6 +/- 7.9 versus 39.5 +/- 10.4). CONCLUSIONS: As systolic ABP variables increase, there is greater likelihood for increased LVMI. Staging based on ABPM allows assessment of cardiovascular risk in children with primary hypertension.

Gatifloxacin produces both hypoglycemia and hyperglycemia: a retrospective study.

BACKGROUND: Gatifloxacin, until recently one of the most commonly prescribed antibiotics, has been shown to produce hypoglycemia. METHODS: To further examine the effects of Gatifloxicin (G) on blood glucose (BS), we conducted a retrospective chart review on 264 inpatients, examining for both hypoglycemia and hyperglycemia, comparing G with another quinolone, Ciproflaxin (C), and nonquinolone, Ceftriaxone (R). RESULTS: We found that of 292 patient encounters, 28 hypoglycemia and 48 hyperglycemic events occurred. Patients given G were 5 times as likely to become hypoglycemic as C (P < 0.01) and 9 times as likely as those given R (P < 0.02). Patients given G were 5.6 times more likely to develop hypoglycemia (P < 0.001) than the combined group, R+C. Conversely, patients treated with G were 3.8 times as likely to become hyperglycemic as those give C (P < 0.01) and 9.8 times as those given R (P < 0.01). With C and R combined, those given G were 5.2 times as likely to develop hyperglycemia (P < 0.01). Looking at patient encounters where G was given, we found that having preexisting diabetes mellitus (DM) was positively associated with hypoglycemia (21/144, P < 0.001). Steroid use (P < 0.05) and being in the ICU (P < 0.01) were also positively associated with hyperglycemia (38/144, P < 0.01). CONCLUSIONS: In summary, G was clearly associated with both hypoglycemia and hyperglycemia compared with C and R. The risk of hyperglycemia increased in the presence of DM, steroid use, and "sick enough" to be in the intensive care unit.

Interleukin-1 receptor antagonist intron 2 variable number of tandem repeats polymorphism and respiratory failure in children with community-acquired pneumonia.

OBJECTIVE: To determine whether the variable nucleotide tandem repeat polymorphism in intron 2 of the interleukin-1 receptor antagonist gene is associated with lung injury in children with community-acquired pneumonia. DESIGN: A prospective cohort of children diagnosed with community-acquired pneumonia. SETTING: Two pediatric hospitals. PATIENTS: Eight hundred fifty pediatric patients with community-acquired pneumonia were enrolled. INTERVENTIONS: Genotyping of the variable nucleotide tandem repeat polymorphism in intron 2 of the interleukin-1 receptor antagonist gene was performed on DNA isolated from whole blood. MEASUREMENTS AND MAIN RESULTS: The requirement for positive pressure ventilation or the diagnosis of acute lung injury or acute respiratory distress syndrome were the main outcomes of the study. Children (14 days-19 yrs) with community-acquired pneumonia (850) were enrolled; analysis was limited to African American (515) and Caucasian (232) patients. Of the 82 patients requiring positive pressure ventilation, 44 were diagnosed with acute lung injury or acute respiratory distress syndrome. Multivariate logistic regression analyses indicated that children without a copy of the A1 allele of the variable nucleotide tandem repeat polymorphism in intron 2 of the interleukin-1 receptor antagonist gene were more likely to need positive pressure ventilation compared to those with one or two copies of this allele (odds ratio = 2.65, confidence interval, 1.02-6.90). In addition, the absence of the A1 allele also appeared to be associated with the development of community-acquired pneumonia-induced acute lung injury/acute respiratory distress syndrome (odds ratio = 3.1, confidence interval, 0.99-9.67). CONCLUSIONS: In children with community-acquired pneumonia, absence of the A1 allele at the interleukin-1 receptor antagonist intron 2 polymorphic site is associated with increased risk for more severe lung injury, as measured by the need for positive pressure ventilation or the development of acute lung injury or acute respiratory distress syndrome. Conversely, presence of the A1 allele is associated with decreased risk for more severe lung injury in this patient population.

Longitudinal relationship of parental hypertension with body mass index, blood pressure, and cardiovascular reactivity in children.

OBJECTIVE: To investigate whether parental hypertension (HTN) affects children's body mass index (BMI) and cardiovascular reactivity (CVR) over time. STUDY DESIGN: A longitudinal study of 315 students (black: 23 females, 19 males; white: 142 females, 131 males) was conducted in the public schools of Obion County, Tennessee, between 1987 and 1992. BMI and BMI z scores were calculated. The CVR task was a series of video games (taking approximately 10 minutes to play) given to the same students in their third-, fourth-, fifth-, seventh-, and eighth-grade years. CVR was defined as the change in blood pressure (delta_BP) or heart rate (delta_HR) between before playing and while playing the video game. Positive parental history of HTN (27.6%) was defined as at least 1 parent with HTN. Multivariable regression analyses were performed to estimate the effects of parental HTN on children's BMI and CVR over time. RESULTS: Children with parental HTN had significant higher BMI, BMI z score, and R_BP than did children without parental HTN (BMI: 21.6 vs 19.9, P = .001; BMI z score: 1.6 vs 1.1, P = .003; R_SBP: 112.6 vs 110.4 mm Hg, P = .01; R_DBP 62.7 vs 60.6 mm Hg, P = .003) after adjustment for covariates. Increased CVR was observed in children with parental HTN compared with children without parental HTN but was statistically significant only for SBP (delta_SBP: 17.2 vs 14.9 mm Hg; P = .01) after adjustment for covariates. CONCLUSIONS: Parental HTN independently predicted children's BMI, BMI z score, resting BP, and BP reactivity.

Influence of comorbid conditions on long-term mortality after pneumonia in older people.

OBJECTIVES: To test the hypothesis that increased long-term mortality after hospitalization for community-acquired pneumonia (CAP) is independent of comorbid conditions. DESIGN: Prospective observational cohort study in metropolitan areas. SETTING: Memphis, Tennessee, and Pittsburgh, Pennsylvania. PARTICIPANTS: Three thousand seventy-five subjects aged 70 to 79 over 5.2 years. MEASUREMENTS: Unadjusted and adjusted mortality from an initial hospitalization for CAP were compared with mortality from different causes of hospitalization, including cancer, fracture, congestive heart failure (CHF), cerebrovascular accident (CVA), and other causes. Demographics, smoking, nutritional markers, functional status, inflammatory markers, and chronic health conditions were adjusted for. RESULTS: Of the 106 subjects hospitalized for CAP, 22 (20.8%) and 38 (35.8%) died at 1 and 5 years. Subjects hospitalized with CAP had higher mortality than nonhospitalized subjects (adjusted odds ratio (OR)=7.8, 95% confidence interval (CI)=4.2-14.4). One- and 5-year mortality after CAP hospitalization were higher than mortality from other causes requiring hospitalization and remained unchanged in multivariable analysis (adjusted OR=3.5, 95% CI=1.5-8.1; adjusted OR=5.6, 95% CI=2.8-11.2, respectively). One- and 5-year mortality after hospitalization for CAP were similar to or higher than mortality after an initial hospitalization for CHF, CVA, or fracture. Rehospitalization was common in subjects hospitalized for CAP and may explain greater long-term mortality. CONCLUSION: In this high-functioning cohort of older persons, an initial hospitalization for CAP was associated with greater long-term mortality, independent of prehospitalization comorbid conditions. Hospitalization for CAP has as serious a prognosis as hospitalization for CHF, stroke, or major fracture.

Does it really take longer not to prescribe antibiotics for viral respiratory tract infections in children?

BACKGROUND: Although overuse of antibiotics in children has been well documented, few studies have evaluated if the visit time for viral infections varies when antibiotics are or are not prescribed. OBJECTIVE: To examine the relationship between physician visit time and antibiotic prescribing for children with viral respiratory tract infection (RTI). METHODS: Data obtained from the National Ambulatory Medical Care Survey (NAMCS) 1993-2003 were surveyed for children < or = 18 years who were seen by a primary care physician and given a primary diagnosis suggestive of viral RTI (cold, upper respiratory infection (URI), bronchiolitis, or brochitis). We excluded visits of children given a comorbid diagnosis justifying antibiotics or a prolonged visit time and those with implausible physician visit times (0 minutes or > 40 minutes). Using univariate and multivariate analysis, we compared self-reported physician visit time when antibiotics were and were not prescribed for viral RTIs. RESULTS: 2739 visits from the NAMCS database, representing 119,926 visits nationally, met study criteria. Antibiotics were prescribed at 46,949 (39%) visits-75% with a diagnosis of bronchitis, 54% with bronchiolitis, and 30% with cold or URI. After adjusting for factors related to physician visit time, there was no difference in visit duration when antibiotics were or were not prescribed (13.6 +/- 8.4 and 13.3 +/- 9.6 minutes, respectively, P = 0.24). CONCLUSION: While antibiotics prescribing for viral RTI in children occurred frequently, our findings do not support the contention that it takes longer 'not to prescribe' antibiotics for children with viral RTIs.

Fosinopril versus amlodipine comparative treatments study: a randomized trial to assess effects on plasminogen activator inhibitor-1.

BACKGROUND: ACE inhibitors and calcium antagonists may modulate fibrinolysis. We conducted a randomized controlled trial to assess the effects of these drugs on plasminogen activator inhibitor-1 (PAI-1) antigen, an inhibitor of fibrinolysis. METHODS AND RESULTS: Participants with hypertension and type 2 diabetes mellitus (n=96, 51% black) were randomized after an initial 4 weeks of placebo to double-blind 20 or 40 mg fosinopril or 5 or 10 mg amlodipine daily for 4 weeks in a fixed-dose regimen. After 4 weeks of placebo washout, the patients received 4 weeks of crossover treatments. After treatment with placebo, systolic and diastolic blood pressure were 143+/-2 and 86+/-1 mm Hg and plasma PAI-1 was 43.4+/-2.3 ng/mL. Amlodipine achieved a greater systolic and diastolic blood pressure reduction than fosinopril (10 mm Hg versus 8 mm Hg, P=0.029, and 5 mm Hg versus 3 mm Hg, P=0.040, respectively) but tended to increase PAI-1, whereas fosinopril tended to decrease PAI-1 (5.4+/-3.6 versus -3.8+/-2.5 ng/mL, P=0.045). The PAI-1 changes depended on drug dose (6.5+/-6.1 and 3.4+/-3.9 ng/mL with amlodipine 10 and 5 mg, respectively, and -0.4+/-3.1 and -7.4+/-4.0 ng/mL with fosinopril 20 and 40 mg, respectively, P for trend 0.024). No significant differences between fosinopril and amlodipine were found for short-term changes in tissue plasminogen activator antigen, fibrinogen, C-reactive protein, and interleukin-6. The findings were similar in black and white participants. CONCLUSIONS: Short-term treatment with fosinopril significantly reduced PAI-1 compared with amlodipine in a dose-dependent fashion. This effect, which was independent of blood pressure reduction, may account for the improved clinical outcomes achieved with ACE inhibitors compared with calcium antagonists.

Kinetics of visual field loss in Usher syndrome Type II.

PURPOSE: To characterize the kinetics of visual field decay in Usher syndrome type II. METHODS: The area of 137 Goldmann visual fields (GVFs) delimited with the I4e and V4e targets was measured in each eye of 19 patients with an established diagnosis of Usher syndrome type II, and the average interocular GVF area for each patient at each time point was calculated. The average follow-up was 5.58 years. Symptomatic disease duration was defined as years elapsed after symptoms were first noted. The data set (n = 67 for the I4e target; n = 70 for the V4e target) was analyzed with a random coefficient mixed model to identify the best-fit model describing the decay of visual field size over time. The half-life of the residual visual field area (t(0.5)) was also calculated. RESULTS: The variable that best explained the decay of the GVF area was the duration of symptomatic disease. In an exponential model, the slope estimate for the natural log of the GVF area was -0.172 for the I4e target and -0.136 for the V4e target for each year of symptomatic disease. Accordingly, t(0.5) was approximately 4 years for the I4e target and 5 years for the V4e target. These estimates are very similar to those in previous studies of nonsyndromic retinitis pigmentosa (RP). CONCLUSIONS: This study suggests that the kinetics of GVF decline in Usher syndrome type II are, on average, very similar to other forms of RP and that, once the disease becomes symptomatic, GVF deterioration follows stereotyped kinetics, even in patients with late-onset retinal disease.

Access of over-the-counter nicotine replacement therapy products to minors.

BACKGROUND: Public health policy guidelines recommend that health care providers (eg, physicians, nurses, others) counsel adolescent smokers to quit and that nicotine replacement therapy (NRT) may be considered to aid in smoking cessation for nicotine-dependent youth. This recommendation is discrepant with Food and Drug Administration-approved labeling of NRT products, stating that they not be sold to persons younger than 18 years. It is not clear how easily minors are able to purchase NRT products in retail markets. OBJECTIVE: To explore youth access to NRT by conducting the first study, to our knowledge, to determine the ability of minors to purchase over-the-counter NRT products. DESIGN: Observational case series of NRT purchase attempts and survey description of store characteristics. SETTING: Retail businesses in Memphis, Tenn. PARTICIPANTS: Population-based sample of 165 stores that sold over-the-counter medications. MAIN OUTCOME MEASURE: Successfully completed purchase attempts of NRT by the minor buyer. RESULTS: In most stores that stocked NRT products, the age of the minor was not queried at any time during the purchase attempt (79%) and the minor was able to successfully purchase the product (81%). If the minor was asked her age, the store was much less likely to sell the NRT product. Stores in which a cash register gave an age query prompt or in which alcohol was sold were more likely to inquire about the minor's age and less likely to sell NRT products. CONCLUSIONS: Nicotine replacement therapy products were successfully obtained in most purchases by a minor buyer without proof of age. While ease of purchasing NRT products is potentially beneficial to young smokers attempting to quit, these purchases are discrepant with Food and Drug Administration labeling regarding the sale of NRT products to minors.

Use of nicotine replacement therapy in adolescent smokers and nonsmokers.

BACKGROUND: Assessing whether and how adolescents use nicotine replacement therapy (NRT) will be important given recent recommendations to make NRT more accessible by lowering its price, increasing its distribution, and advising health care professionals to suggest its use for smoking cessation. OBJECTIVES: To report the prevalence, ease of access, and reasons for NRT use and describe inappropriate use in adolescent smokers and nonsmokers. DESIGN: Cross-sectional survey of 4078 high school students during the school term of 1998. SETTING: City schools in Memphis, Tenn. MAIN OUTCOME MEASURES: Community-based self-reported prevalence of NRT use and characteristics of those using NRT. RESULTS: Approximately 5% of adolescents reported trying or using nicotine gum or patches. Females were less likely than males and African Americans were less likely than others to use NRT. For African American smokers, NRT use was highest at lower smoking levels, while other smokers showed the opposite pattern. Almost 40% of former smokers reported using NRT to try to quit smoking; however, 75% of current smokers endorsed using NRT for reasons other than trying to quit smoking. Other inappropriate use of NRT was reported; 18% of NRT users reported themselves as never smokers. More than 50% of students reported that it would be easy for them to get NRT. CONCLUSIONS: Nicotine replacement therapy is used by adolescent smokers and nonsmokers, is easily accessible, and is used for reasons other than trying to quit smoking. Efforts are needed to discourage NRT use in nonsmoking youth and to encourage appropriate use of NRT in young smokers to maximize its potential for successful cessation.

Impact of a statewide Medicaid managed care system on healthcare utilization and outcomes for people living with HIV.

BACKGROUND: Medicaid managed care (MMC) systems provide insurance for many persons living with HIV and AIDS (PLWH). This study sought to assess the impact of a statewide MMC system (TennCare) on healthcare utilization and outcomes for PLWH. METHODS: A retrospective longitudinal analysis of trends in population characteristics was performed. The study population included all Tennessee PLWH identified by State Health Department, enrolled for 1 year or longer in Medicaid (1992-1993) or TennCare (1994-1997). Main outcome measures included health care utilization, incidence of opportunistic infections, and mortality. RESULTS: From 1992 to 1997, the following decreased: average number of hospitalizations for HIV (0.72 to 0.37) and AIDS (1.27 to 0.52); emergency visits for HIV (1.70 to 1.12) and AIDS (1.65 to 1.02); outpatient visits for HIV (5.94 to 5.00) and AIDS (8.37 to 7.35), percentage of persons diagnosed with Pneumocystis carinii pneumonia for AIDS (10% to 6%) and percentage of persons diagnosed with community-acquired pneumonia for HIV (14% to 9%) and AIDS (27% to 12%), annual incidence of AIDS in the HIV population (33% to 10%), and annual mortality for HIV (3% to 1%) and AIDS (16% to 3%). The average number of antiretroviral medication prescriptions filled increased for HIV (1.27 to 2.45) and AIDS (1.31 to 3.34). CONCLUSIONS: This study documents improvements in utilization patterns, morbidity, and mortality in a statewide MMC system. These findings suggest that MMC patients are benefiting from recent advances in therapy.

The effect of developmentally-at-risk status on the reliability of the iScreen® photorefractive device in young children.

INTRODUCTION AND PURPOSE: We evaluated the impact of developmentally-at-risk status on the results of photorefractive screening with iScreen®. PATIENTS AND METHODS: We sequentially recruited 169 children (aged 2-5 years) to participate in a blinded, prospective study of a photoscreening device. The principle investigator examined the children after photoscreening. Using established standards for amblyogenic factors, the principle investigator and photoscreen interpreters separately made a determination of "normal" or "needs referral" for each child. Sensitivity, specificity, positive predictive value, and negative predictive value for the photoscreening device were determined. RESULTS: Overall, sensitivity was 85% with a positive predictive value of 98%, and specificity was 87% with a negative predictive value of 47%. Developmentally-at-risk status in 34 children (three refused imaging) did not reduce sensitivity (89%) or specificity (100%) when compared with 130 children (two refused imaging) who did not have developmentally-at-risk factors (sensitivity = 84% and specificity = 80%). CONCLUSION: The efficacy of photorefractive screening in young children with developmentally-at-risk status is comparable to results found in normal children.

Long-term outcomes of patients with cardiovascular abnormalities and williams syndrome.

Williams syndrome (WS) is a congenital disorder affecting the vascular, connective tissue, and central nervous systems of 1 in 8,000 live births. Previous reports have reported high frequencies of cardiovascular abnormalities (CVAs) in small numbers of patients with WS. A retrospective review was undertaken of patients with WS evaluated at our institution from January 1, 1980 through December 31, 2007. WS was diagnosed by an experienced medical geneticist and/or by fluorescence in situ hybridization. CVAs were diagnosed using echocardiography, cardiac catheterization, or computed tomographic angiography. Freedom from intervention was determined using Kaplan-Meier analysis. The study group was 270 patients with WS. The age at presentation was 3.3 +/- 5.9 years with follow-up of 8.9 +/- 9.0 years (range 0 to 56.9). CVAs were present in 82% of the patients. The most common lesions were supravalvar aortic stenosis in 45% and peripheral pulmonary stenosis in 37%; 20% had both. Other common lesions included mitral valve prolapse and regurgitation in 15%, ventricular septal defect in 13%, and supravalvar pulmonary stenosis in 12%. Surgical or catheter-based interventions were performed in 21%. The rate of freedom from intervention was 91%, 81%, 78%, 72%, and 62% at 1, 5, 10, 20, and 40 years. Eight patients died. In conclusion, CVAs are common in patients with WS, but supravalvar aortic stenosis and peripheral pulmonary stenosis occurred less frequently in this large cohort than previously reported. In patients with WS and CVAs, interventions are common and usually occur by 5 years of age. Most patients with WS do not require intervention during long-term follow-up, and the overall mortality has been low.

Gender, smoking status, and risk behavior attitudes explain adolescents' patterns of nicotine replacement therapy use.

Treatment studies provide minimal support for nicotine replacement therapy (NRT) with youth; however, survey studies suggest that adolescents use NRT, and may engage in inappropriate use. The current study sought to examine patterns of NRT use and risk factors for use to further aid smoking cessation efforts including prevention of potential misuse. In-school surveys assessing socio-demographic and behavioral factors associated with NRT use, gum or patch, were completed by 4078, predominantly African American, high school students. Approximately 5% of students reported former or current use of NRT products: 42% gum, 29% patch, and 29% both gum and patch. Among smokers, 5.4% reported use of both NRT gum and patch, with exclusive use of gum twice as likely as exclusive use of the patch. Those with high-risk-taking attitudes were more likely than low-risk takers (3% vs. 1%) to report use of both products, with exclusive gum use more prevalent than patch use. A cumulative logit model revealed males, risk takers, and/or smokers were at greatest odds for NRT use. Among this adolescent sample, NRT gum was used more often than the patch. Adolescent males, risk takers, and/or smokers appear more likely to use NRT (gum and/or patch) compared to their counterparts, despite limited empirical support for effective use of these products as cessation aids among adolescents. Smoking cessation and prevention programs may emphasize appropriate NRT use, specifically within these populations.