Long-term outcomes of patients with Wilson disease in a large Austrian cohort

BACKGROUND & AIMS Wilson disease is an autosomal recessive disorder that affects copper metabolism, leading to copper accumulation in liver, central nervous system, and kidneys. There are few data on long-term outcomes and survival from large cohorts; we studied these features in a well-characterized Austrian cohort of patients with Wilson disease. METHODS We analyzed data from 229 patients diagnosed with Wilson disease from 1961 through 2013; 175 regularly attended a Wilson disease outpatient clinic and/or their physicians were contacted for information on disease and treatment status and outcomes. For 53 patients lost during the follow-up period, those that died and reasons for their death were identified from the Austrian death registry. RESULTS The mean observation period was 14.8 ± 11.4 years (range, 0.5-52.0 years), resulting in 3116 patient-years. Of the patients, 61% presented with hepatic disease, 27% with neurologic symptoms, and 10% were diagnosed by family screening at presymptomatic stages. Patients with a hepatic presentation were diagnosed younger (21.2 ± 12.0 years) than patients with neurologic disease (28.8 ± 12.0; P < .001). In 2% of patients, neither symptoms nor onset of symptoms could be determined with certainty. Most patients stabilized (35%) or improved on chelation therapy (26% fully recovered, 24% improved), but 15% deteriorated; 8% required a liver transplant, and 7.4% died within the observation period (71% of deaths were related to Wilson disease). A lower proportion of patients with Wilson disease survived for 20 years (92%) than healthy Austrians (97%), adjusted for age and sex (P = .03). Cirrhosis at diagnosis was the best predictor of death (odds ratio, 6.8; 95% confidence interval, 1.5-31.03; P = .013) and need for a liver transplant (odds ratio, 07; 95% confidence interval, 0.016-0.307; P < .001). Only 84% of patients with cirrhosis survived 20 years after diagnosis (compared with healthy Austrians, P =.008). CONCLUSION Overall, patients who receive adequate care for Wilson disease have a good long-term prognosis. However, cirrhosis increases the risk of death and liver disease. Early diagnosis, at a precirrhotic stage, might increase survival times and reduce the need for a liver transplant.

Multi-grade teaching practices in Austrian and Finnish primary schools

This article describes the teaching strategies used in multi-grade classes in five small rural primary schools in Austria and Finland on the basis of the content analysis of transcribed teacher interviews. Two main types of strategies were identified: practices that (1) aim to reduce or (2) capitalize on students’ heterogeneity. The results illustrate how differently multi-grade teaching can be realized and how it can effectively support individual student learning. The findings are discussed with regard to teacher education with the intention of increasing the awareness of the professional skills required in high-quality teaching practices in multi-grade teaching.

The impact of a cooking and gardening summer camp experience on the fruit and vegetable consumption of elementary school-aged children

Childhood obesity is a significant public health problem. Over 15 percent of children in the United States are obese, and about 25 percent of children in Texas are overweight (CDC NHANES). Furthermore, about 30 percent of elementary school aged children in Harris County, Texas are overweight or obese (Children at Risk Institute 2010). In addition to actions such as increasing physical activity, decreasing television watching and video game time, decreasing snacking on low nutrient calorie dense foods and sugar sweetened beverages, children need to consume more fruits and vegetables. According to the National Health and Nutrition Examination Survey (NHANES) from 2002, about 26 percent of U.S. children are meeting the recommendations for daily fruit intake and about 16 percent are meeting the recommendations for daily vegetable intake (CDC NHANES). In 2004, the average total intake of vegetables was 0.9 cups per day and 1.1 cups of fruit per day by children ages four to nine years old in the U.S. (CDC NHANES). Not only do children need effective nutrition education to learn about fruits and vegetables, they also need access and repeated exposure to fruits and vegetables (Anderson 2009, Briefel 2009). Nutrition education interventions that provide a structured, hands-on curriculum such as school gardens have produced significant changes in child fruit and vegetable intake (Blair 2009, McAleese 2007). To prevent childhood obesity from continuing into adolescence and adulthood, effective nutrition education interventions need to be implemented immediately and for the long-term. However, research has shown short-term nutrition education interventions such as summer camps to be effective for significant changes in child fruit and vegetable intake, preferences, and knowledge (Heim 2009). ^ A four week summer camp based on cooking and gardening was implemented at 6 Multi-Service centers in a large, urban city. The participants included children ranging in age from 7 to 14 years old (n=64). The purpose of the camp was to introduce children to their food from the seed to the plate through the utilization of gardening and culinary exercises. The summer camp activities were aimed at increasing the children's exposure, willingness to try, preferences, knowledge, and intake of fruits and vegetables. A survey was given on the first day of camp and again on the last day of camp that measured the pre- and post differences in knowledge, intake, willingness to try, and preferences of fruits and vegetables. The present study examined the short-term effectiveness of a cooking and garden-based nutrition education program on the knowledge, willingness, preferences, and intake among children aged 8 to 13 years old (n=40). The final sample of participants (n=40) was controlled for those who completed pre- and post-test surveys and who were in or above the third grade level. Results showed a statistically significant increase in the reported intake of vegetables and preferences for vegetables, specifically green beans, and fruits. There was also a significant increase in preferences for fruits among boys and participants ages 11 to 13 years. The results showed a change in the expected direction of willingness to try, preferences for vegetables, and intake of fruit, however these were not statistically significant. Interestingly, the results also showed a decrease in the intake of low nutrient calorie dense foods such as sweets and candy.^

The Austrian Glacier Inventories GI 1 (1969), GI 2 (1998), GI 3 (2006), and GI LIA in ArcGIS (shapefile) format

Glacier inventories provide the basis for further studies on mass balance and volume change, relevant for local hydrological issues as well as for global calculation of sea level rise. In this study, a new Austrian glacier inventory has been compiled, updating data from 1969 (GI 1) and 1998 (GI 2) based on high-resolution lidar digital elevation models (DEMs) and orthophotos dating from 2004 to 2012 (GI 3). To expand the time series of digital glacier inventories in the past, the glacier outlines of the Little Ice Age maximum state (LIA) have been digitalized based on the lidar DEM and orthophotos. The resulting glacier area for GI 3 of 415.11 ± 11.18 km**2 is 44% of the LIA area. The annual relative area losses are 0.3%/yr for the ~119-year period GI LIA to GI 1 with one period with major glacier advances in the 1920s. From GI 1 to GI 2 (29 years, one advance period of variable length in the 1980s) glacier area decreased by 0.6% yr?1 and from GI 2 to GI 3 (10 years, no advance period) by 1.2%/yr. Regional variability of the annual relative area loss is highest in the latest period, ranging from 0.3 to 6.19%/yr. The mean glacier size decreased from 0.69 km**2 (GI 1) to 0.46 km**2 (GI 3), with 47% of the glaciers being smaller than 0.1 km**2 in GI 3 (22%).