Characteristics of Fatal Agricultural Injuries by Production Type.

Although agriculture is recognized as a hazardous industry, it is unclear how fatal agricultural injuries differ by production type. The purpose of this study was to characterize fatal occupational injuries in agriculture, comparing crop and animal production, and determine which risk factors are specifically associated with each production type. A cross-sectional study was conducted among crop and animal pro ducers using data from the Census of Fatal Occupational Injuries in the Midwest region from 2005 to 2012. Rates offatal injury by production type were estimated. The frequency of fatal injury in each production type was also reported by demographic and injury characteristics. Finally, a logistic regression was performed to determine whether age, gender, injury timing, or injury event/exposure type were associated with crop or animal production. A total of 1,858 fatal agriculture-related injuries were identified, with 1,341 in crop production and 517 in animal production. The estimated rate of fatal injury was higher in crop production than in animal production (15.9 vs. 10.8 per 100,000 workers). Fatal injuries among young and elderly agricultural workers were significantly associated with crop production compared to animal production. Animal assaults, falls, and exposure to harmful substances or environments were significantly associated with animal production. Fatal agricultural injury is more common in crop production. However, the characteristics and risk factors of fatal injuries differ by production type. Intervention strategies may be guided by considering the production-specflc risk factors.

'It had to be done': genetic testing decisions for arrhythmogenic right ventricular cardiomyopathy.

Arrhythmogenic right ventricular cardiomyopathy (ARVC) is a heritable disease of the heart muscle, causing life-threatening ventricular arrhythmias, sudden cardiac death and/or biventricular heart failure. Little research examines ARVC genetic test decisions, despite the gravity of the condition. This qualitative study used semi-structured interviews to explore the testing decisions of 21 individuals across 15 families segregating a well-studied, particularly lethal form of ARVC caused by a p.S358L TMEM43 mutation. Genetic testing decisions were rarely described as 'decisions' per se, but rather 'something that had to be done'. This perception was attributed to personality type or personal suspicion of carrying the TMEM43 mutation, but most often was described in the context of testing for other family members, usually children. Participants related a strong need to rule out risk, more for children than for themselves, but lingering doubts remained about personal and children's risk for ARVC, even when gene test results were negative. Study findings highlight the interdependent nature of genetic test decisions and suggest that an individualistic conception of autonomy in genetic services may not meet the needs of affected families. Findings also suggest the need for follow-up support of families affected by ARVC, including for those individuals testing negative for the family mutation.

The natural history of a genetic subtype of arrhythmogenic right ventricular cardiomyopathy caused by a p.S358L mutation in TMEM43.

To determine the phenotype and natural history of a founder genetic subtype of autosomal dominant arrhythmogenic right ventricular cardiomyopathy (ARVC) caused by a p.S358L mutation in TMEM43. The age of onset of cardiac symptoms, clinical events and test abnormalities were studied in 412 subjects (258 affected and 154 unaffected), all of which occurred in affected males significantly earlier and more often than unaffected males. Affected males were hospitalized four times more often than affected females (p ≤ 0.0001) and died younger (p ≤ 0.001). The temporal sequence from symptoms onset to death was prolonged in affected females by 1-2 decades. The most prevalent electrocardiogram (ECG) manifestation was poor R wave progression (PRWP), with affected males twice as likely to develop PRWP as affected females (p ≤ 0.05). Left ventricular enlargement (LVE) occurred in 43% of affected subjects, with 11% fulfilling criteria for dilated cardiomyopathy. Ventricular ectopy on Holter monitor was common and occurred early: the most diagnostically useful clinical test. No symptom or test could rule out diagnosis. This ARVC subtype is a sex-influenced lethal arrhythmogenic cardiomyopathy, with a unique ECG finding, LV dilatation, heart failure and early death, where molecular pre-symptomatic diagnosis has the greatest clinical utility.

Pediatric cataract, myopic astigmatism, familial exudative vitreoretinopathy and primary open-angle glaucoma co-segregating in a family.

PURPOSE: To describe an Australian pedigree of European descent with a variable autosomal dominant phenotype of: pediatric cortical cataract (CC), asymmetric myopia with astigmatism, familial exudative vitreoretinopathy (FEVR), and primary open-angle glaucoma (POAG). METHODS: Probands with CC, FEVR, and POAG were enrolled in three independent genetic eye studies in Tasmania. Genealogy confirmed these individuals were closely related and subsequent examination revealed 11 other family members with some or all of the associated disorders. RESULTS: Twelve individuals had CC thought to be of childhood onset, with one child demonstrating progressive lenticular opacification. One individual had severe retinal detachment while five others had dragged retinal vessels. Seven individuals had POAG. Seven individuals had myopia in at least one eye ≤-3 Diopters. DNA testing excluded mutations in myocilin, trabecular meshwork inducible glucocorticoid response (MYOC) and tetraspanin 12 (TSPAN12). Haplotype analysis excluded frizzled family receptor 4 (FZD4) and low density lipoprotein receptor-related protein 5 (LRP5), but only partly excluded EVR3. Multipoint linkage analysis revealed multiple chromosomal single-nucleotide polymorphisms (SNPs) of interest, but no statistically significant focal localization. CONCLUSIONS: This unusual clustering of ophthalmic diseases suggests a possible single genetic cause for an apparently new cataract syndrome. This family's clinical ocular features may reflect the interplay between retinal disease with lenticular changes and axial length in the development of myopia and glaucoma.

Prevalence and causes of decreased visual acuity in Singaporean Chinese preschoolers.

AIMS: To describe the prevalence and causes of decreased visual acuity (VA) in Singaporean Chinese children. METHODS: A population-based survey of Singaporean Chinese children aged 6 to 72 months was conducted. Participants underwent an orthoptic evaluation, cycloplegic refraction and biometric measurements. A sub-group of children aged 30 to 72 months with presenting logMAR VA were included in this analysis. Retesting was performed on the same day or another day by predefined criteria with best refractive correction. Decreased VA was defined as worse than 20/50 (0.4 logMAR) for ages 30 to 47 months and worse than 20/40 (0.3 logMAR) for ages 48 to 72 months. RESULTS: The study examined 3009 children (participation rate 72.3%) of which 2017 children aged 30 to 72 months were eligible for VA testing and completed in 1684 (83.5%). In children aged 30-47 months, the prevalence of decreased presenting VA was 2.1%, and in children 48-72 months, it was 2.05%, with no significant difference between boys and girls in both age groups (p=0.15 and p=0.85). Causes for decreased presenting VA in those 30-47 months were refractive error (7/11, 63.6%), amblyopia (1/11, 9.1%) and "no explanation" (3/11, 27.3%), and 17/24 (70.8%), 5/24 (20.8%) and 2/24 (8.3%), respectively, for those aged 48-72 months. The types of refractive error were astigmatism (15/24, 62.5%), myopia (6/24, 25.0%), hyperopia (2/24, 8.3%) and hyperopia with astigmatism (1/24, 4.2%). CONCLUSIONS: The prevalence of decreased VA among Singaporean Chinese preschoolers is low, with uncorrected refractive error being the main cause in both children 30-47 and 48-72 months.

Outdoor activity and myopia in Singapore teenage children.

AIM: To investigate the relationship of outdoor activities and myopia in Singapore teenage children. METHODS: Teenage children (1249 participants), examined in the Singapore Cohort study Of Risk factors for Myopia (SCORM), during 2006 were included in analyses. Participants completed questionnaires that quantified total outdoor activity, and underwent an eye examination. RESULTS: The mean total time spent on outdoor activity was 3.24 h/day. The total outdoor activity (h/day) was significantly associated with myopia, odds ratio 0.90 (95% CI 0.84 to 0.96) (p = 0.004), after adjusting for age, gender, ethnicity, school type, books read per week, height, parental myopia, parental education and intelligence quotient. In addition, the total time spent outdoors was associated with significantly less myopic refraction (regression coefficient = 0.17; CI 0.10 to 0.25, p<0.001) and shorter axial length (regression coefficient -0.06 (CI -0.1 to -0.03, p<0.001). Total sports was also significantly negatively associated with myopia (p = 0.008) but not indoor sports (p = 0.16). CONCLUSIONS: Participants who spent more time outdoors were less likely to be myopic. Thus, outdoor activity may protect against development of myopia in children, supporting recent Australian data. As near work did not predict outdoor activity, this can be viewed as an independent factor and not merely the reciprocal of near work.

Familial variable expression of dilated cardiomyopathy in Alström syndrome: a report of four sibs.

Alström syndrome is an autosomal recessive disorder comprised of progressive vision loss (nystagmus, photophobia, and pigmentary retinopathy), progressive sensorineural hearing loss, morbid obesity, male hypogonadism, insulin resistant diabetes, renal failure, and dilated cardiomyopathy. We report on four sibs with Alström syndrome with intra-familial variability in onset, severity, and spectrum of manifestations; the most serious manifestation being dilated cardiomyopathy. This report emphasizes the difficulty of recognizing this constellation of symptoms as Alström syndrome at an early age, the seriousness of cardiac involvement, and the intra-familial variability of phenotypic expression.

The noncoding RNA taurine upregulated gene 1 is required for differentiation of the murine retina.

BACKGROUND: With the advent of genome-wide analyses, it is becoming evident that a large number of noncoding RNAs (ncRNAs) are expressed in vertebrates. However, of the thousands of ncRNAs identified, the functions of relatively few have been established. RESULTS: In a screen for genes upregulated by taurine in developing retinal cells, we identified a gene that appears to be a ncRNA. Taurine Upregulated Gene 1 (TUG1) is a spliced, polyadenylated RNA that does not encode any open reading frame greater than 82 amino acids in its full-length, 6.7 kilobase (kb) RNA sequence. Analyses of Northern blots and in situ hybridization revealed that TUG1 is expressed in the developing retina and brain, as well as in adult tissues. In the newborn retina, knockdown of TUG1 with RNA interference (RNAi) resulted in malformed or nonexistent outer segments of transfected photoreceptors. Immunofluorescent staining and microarray analyses suggested that this loss of proper photoreceptor differentiation is a result of the disregulation of photoreceptor gene expression. CONCLUSIONS: A function for a newly identified ncRNA, TUG1, has been established. TUG1 is necessary for the proper formation of photoreceptors in the developing rodent retina.

An analysis of the gene expression program of mammalian neural progenitor cells.

A diverse range of neural cell types is generated from a pool of dividing stem and progenitor cells in an orderly manner during development. Little is known of the molecular and cellular biology underpinning the intrinsic control of this process. We have used a nonbiased method to purify populations of neural progenitor cells from the murine CNS to characterize the gene expression program of mammalian retinal progenitor cells. Analysis of these data led to the identification of a core set of >800 transcripts enriched in retinal progenitor cells compared to both their immediate postmitotic progeny and to differentiated neurons. This core set was found to be shared by progenitors in other regions of the developing CNS, with important regional differences in key functional families. In addition to providing an expression fingerprint of this cell type, this set highlights several key aspects of progenitor biology.

Retinal neovascular markers in retinopathy of prematurity: aetiological implications.

AIM: (1) To determine if expression of the blood-tissue barrier associated glucose transporter GLUT1 is preserved by the neovasculature of retinopathy of prematurity (ROP), in contrast with the reported loss of GLUT1 expression in preretinal vessels of proliferative diabetic retinopathy. (2) To compare the vascular immunophenotype of ROP to juvenile haemangioma, another perinatal neovascular disorder that has recently been shown to express placental type vascular antigens, including GLUT1 and Lewis Y antigen. METHODS: A retrospective case report was carried out. Immunoreactivities for GLUT1 and Lewis Y antigen were assessed in a human eye with stage 3 ROP and compared with those in a control (paediatric) eye. The presence or absence of endothelial GLUT1 and Lewis Y immunoreactivity was determined in preretinal and intraretinal vessels. RESULTS: Immunoreactivity was positive for GLUT1 and negative for Lewis Y in the intraretinal and preretinal neovasculature of the ROP affected eye and in the normal retinal vessels of the control eye. CONCLUSIONS: Retention of immunoreactivity for GLUT1 distinguishes ROP from proliferative diabetic retinopathy. Furthermore, absence of Lewis Y antigen co-expression distinguishes ROP from juvenile haemangioma, a perinatal form of GLUT1 positive neovascularisation that has recently been linked to placental vasculature.

Non-syndromic progressive hearing loss DFNA38 is caused by heterozygous missense mutation in the Wolfram syndrome gene WFS1.

Dominantly inherited progressive hearing loss DFNA38 is caused by heterozygosity for a novel mutation in WFS1, the gene for recessively inherited Wolfram syndrome. Wolfram syndrome is defined by juvenile diabetes mellitus and optic atrophy and may include progressive hearing loss and other neurological symptoms. Heterozygotes for other Wolfram syndrome mutations generally have normal hearing. Dominant deafness defined by DFNA38 is more severe than deafness of Wolfram syndrome patients and lacks any syndromic features. In a six-generation kindred from Newfoundland, Canada, WFS1 Ala716Thr (2146 G-->A) was shared by all deaf members of the family and was specific to deaf individuals. The causal relationship between this missense mutation and deafness was supported by two observations based on haplotype and mutation analysis of the kindred. First, a relative homozygous for the mutation was diagnosed at age 3 years with insulin-dependent diabetes mellitus, the central feature of Wolfram syndrome. Second, two relatives with normal hearing had an identical haplotype to that defining DFNA38, with the exception of the base pair at position 2146. Other rare variants of WFS1 co-inherited with deafness in the family could be excluded as disease-causing mutations on the basis of this hearing-associated haplotype. The possibility that 'mild' mutations in WFS1 might be a cause of non-syndromic deafness in the general population should be explored.

Efficacy of inferior oblique anterior transposition placement grading for dissociated vertical deviation.

OBJECTIVE: To determine if graded anterior placement of a transposed inferior oblique muscle is beneficial for treating variable amounts of dissociated vertical deviation (DVD). DESIGN: Retrospective, consecutive, comparative case series. PARTICIPANTS: Patients who underwent inferior oblique muscle anterior transposition (IOAT) for DVD at one institution between 1991 and 1999. METHODS: Chart review. All patients had IOAT procedures of graded placement at 1, 2, or 3 mm anterior to the inferior rectus muscle insertion or standard placement at the level of the inferior rectus muscle insertion. MAIN OUTCOME MEASURES: The effect of graded and standard placement was assessed by measuring the difference between preoperative and postoperative DVD and was defined as DVD correction. The success of surgery was judged by the residual DVD at long-term follow-up of 6 months or more. Excellent, fair, and poor outcomes were defined as residual DVD of 0 to 5 prism diopters (PD), 6 to 12 PD, and 13 or more PD, respectively. RESULTS: Fifty-five patients (106 eyes) underwent IOAT for DVD. The comparison of DVD correction for the standard versus graded group yielded significance at long-term follow-up (P = 0.001). This result became nonsignificant after adjusting for preoperative DVD (P = 0.178). The power to detect a 5-PD difference between graded and standard placement was 90%. The surgical success was similar for patients receiving graded and standard IOAT. Patients with 0 to 15 PD of preoperative DVD fared better than those with more than 15 PD of preoperative DVD. CONCLUSIONS: This study does not demonstrate increased correction of DVD with graded IOAT versus standard IOAT. We do not recommend placement of the inferior oblique muscle anterior to the inferior rectus muscle insertion. Inferior oblique muscle anterior transposition for DVD was clinically more effective for smaller amounts of DVD.

Further refinement of the MYP2 locus for autosomal dominant high myopia by linkage disequilibrium analysis.

INTRODUCTION: High myopia (>-6.00 diopters) is a complex common disorder that predisposes individuals to retinal detachment, glaucoma, macular degeneration, and premature cataracts. A recent linkage analysis of seven families with autosomal dominant high myopia has identified one locus (MYP2) for high myopia on chromosome 18p11.31 (Young et al.: Am J Hum Genet 1998;63:109-119). Haplotype analysis revealed an initial interval of 7.6 centimorgans (cM). METHODS: Transmission disequilibrium tests (TDT) with both the Statistical Analysis for Genetic Epidemiology (SAGE) 3.1 TDTEX and GENEHUNTER 2 (GH2) programs were performed using chromosome 18p marker alleles for this interval. RESULTS: Using SAGE analysis, the following p values were obtained for markers in marker order in this region: D18S1146 (p = 0.227), D18S481 (p = 0.001), D18S63 (p = 0.062), D18S1138 (p = 0.0004), D18S52 (p = 1.79 x 10(-6)), and D18S62 (p = 0.141). GH2 TDT analysis revealed the following p values for the best allele for the markers: D18S1146 (p = 0.083), D18S481 (p = 0.108), D18S63 (p = 0.034), D18S1138 (p = 0.011), D18S52 (p = 0.007), and D18S62 (p = 0.479). CONCLUSION: These data suggest that the gene for 18p11.31-linked high myopia is most proximal to marker D18S52, with a likely interval of 0.8 cM between markers D18S63 and D18S52. Due to the contraction of the interval size by TDT, these results provide a basis for focused positional cloning and candidate gene analysis at the MYP2 locus.

Impact of a school-based health center on emergency department use by elementary school students.

While school-based health centers (SBHC) improve student access to health services, it remains unclear if use of the centers can reduce hospital emergency department visits. This study evaluated the impact of an elementary school SBHC on emergency department visits by children enrolled in the center. Major reasons for visits included trauma (32%), otitis media (15%), upper respiratory infections (9%), and gastroenteritis (6%). Implementation of an elementary SBHC resulted in a significant decrease (p < 0.03) in non-urgent emergency department visits. No difference existed in urgent emergency department visits. Medicaid-insured children were more likely to use the emergency department than privately insured or uninsured children. Reducing emergency department visits can decrease medical costs and support the cost effectiveness of SBHCs.

Organized sports for children and preadolescents.

Participation in organized sports provides an opportunity for young people to increase their physical activity and develop physical and social skills. However, when the demands and expectations of organized sports exceed the maturation and readiness of the participant, the positive aspects of participation can be negated. The nature of parental or adult involvement can also influence the degree to which participation in organized sports is a positive experience for preadolescents. This updates a previous policy statement on athletics for preadolescents and incorporates guidelines for sports participation for preschool children. Recommendations are offered on how pediatricians can help determine a child's readiness to participate, how risks can be minimized, and how child-oriented goals can be maximized.

Corn husk oil lowers plasma LDL cholesterol concentrations by decreasing cholesterol absorption and altering hepatic cholesterol metabolism in guinea pigs.

To test the hypocholesterolemic mechanisms of corn husk oil (CoHO), male Hartley guinea pigs were fed diets containing increasing doses of CoHO, either 0 (control), 5, 10, or 15 g/100 g, and 0.25 g/100 g cholesterol. A positive control group (LC) with low dietary cholesterol (0.04 g/100 g) was also included. Fat was adjusted to 15 g/100 g in all diets by the addition of regular corn oil. Plasma low density lipoprotein (LDL) cholesterol concentrations were 32, 55, and 57% (P < 0.0005) lower with increasing doses of CoHO. In addition, intake of CoHO resulted in 32 to 43% lower hepatic total and esterified cholesterol and 55 to 60% lower triacylglycerol concentrations compared with the control group (P < 0.01). CoHO intake resulted in plasma and hepatic cholesterol concentrations similar to those in guinea pigs from the LC group. The number of cholesteryl ester and free cholesterol molecules was higher in LDL from the control group than in LDL from the CoHO or the LC groups. Hepatic beta-hydroxy-beta-methylglutaryl-coenzyme A reductase activity was not modified by CoHO intake whereas cholesterol 7alpha-hydroxylase was up-regulated by 45 to 49% (P < 0.01) in the 10 and 15 g/100 g CoHO groups. Hepatic acyl coenzyme A cholesterol acyltransferase activity was down-regulated in a dose-dependent manner by 54, 58, and 63% with increasing doses of CoHO. CoHO intake resulted in increased fecal cholesterol excretion by 40 to 55% compared with the control and LC groups. Total fecal neutral sterol excretion was enhanced 42 to 55% by CoHO compared with the control group and by 59 to 68% compared with the LC group. The data from these studies suggest that CoHO has its hypocholesterolemic effect by decreasing cholesterol absorption and increasing bile acid output. These alterations in the intestinal lumen alter hepatic cholesterol metabolism and may affect the synthesis and catabolism of lipoproteins.

Economic model of sustained-release bupropion hydrochloride in health plan and work site smoking-cessation programs.

The development and application of an economic model designed to assess the specific costs and benefits of health plan coverage of smoking-cessation programs involving sustained-release bupropion hydrochloride are described. A cohort of 100,000 employees or health plan members and 60,000 adult dependents was followed from the start of the model to either retirement at age 65 or death at age 85. The model was used to compare outcomes for coverage versus no coverage of sustained-release bupropion hydrochloride as a component of a smoking-cessation benefit under four managed care plan scenarios and four employer scenarios. For the managed care scenarios involving coverage of bupropion sustained-release the overall decrease in health care costs over a 20-year period ranged from $7.9 million to $8.8 million; for every dollar spent covering smoking cessation, $4.10-$4.69 in health care costs was saved. For the employer scenarios, health care costs over 20 years decreased by $8.3 million to $14.0 million, and smoking-related indirect costs decreased an additional $5.1 million to $7.7 million; for every dollar spent covering smoking cessation, $5.04-$6.48 was saved. A model developed to assess the specific costs and benefits of covering sustained release bupropion hydrochloride as a component of a smoking-cessation benefit indicated cost savings for health plans and employers.

Anterior transposition of the superior oblique tendon in the treatment of oculomotor nerve palsy and its influence on postoperative hypertropia.

PURPOSE: To determine whether postoperative hypertropia after anterior transposition of the superior oblique tendon without trochleotomy could be avoided with a simplified surgical approach. METHODS: Eight patients with oculomotor nerve palsy (one patient was bilaterally affected) were retrospectively identified as having undergone anterior transposition of the superior oblique tendon without trochleotomy or vertical rectus muscle surgery between March 1992 and September 1998. The superior oblique tendon was cut at the medial border of the superior rectus muscle and placed 1-3.5 mm anterior to the medial insertion of the superior rectus muscle in each of these patients. Resection of the superior oblique tendon was not performed. The lateral rectus muscle was weakened, and no vertical rectus muscles were resected. RESULTS: Preoperative deviations with the uninvolved eye fixating in primary position ranged from 20-90 prism diopters (delta) of exotropia (mean: 49.3 delta) and from 0-20 delta of hypotropia (mean: 11.25 delta). Postoperative horizontal deviations in the primary gaze position ranged from 12 delta of exotropia to 20 delta of esotropia. Six cases were aligned within 10 delta of exotropia or esotropia. Postoperative vertical deviations in the primary gaze position ranged from 2 delta of hypertropia to 8 delta of hypotropia. Six cases were aligned within 2 delta of deviation. Significant postoperative restrictive hypertropia, or new postoperative paradoxical ocular movements, did not occur in any patient. Patients who underwent follow-up >4 months maintained stable eye alignment. CONCLUSION: Transposition of the superior oblique tendon without simultaneous resection or trochleotomy, or additional surgery to the vertical rectus muscle simplifies the surgical technique and eliminates subjective decision making regarding the amount of resection.