Gorlin syndrome in a patient with skin type VI

Gorlin syndrome, also known as nevoid basal cell carcinoma syndrome, is a rare autosomal dominant disorder that is characterized by multiple basal cell carcinomas developing at a young age, keratocystic odontogenic tumors of the jaw, palmar or plantar pits, calcification of the falx cerebri, and skeletal abnormalities. Nevoid basal cell carcinoma syndrome is caused by mutations in the PTCH1 or SUFU genes. Our patient with Fitzpatrick skin type VI was diagnosed with Gorlin syndrome based on the presentation of multiple major diagnostic characteristics. Although he is 33 years old, he has not developed any multiple basal cell carcinomas to date

Stage-specific vertical distribution of Alaska plaice (Pleuronectes quadrituberculatus) eggs in the eastern Bering Sea

The stage-specific distribution of Alaska plaice (Pleuronectes quadrituberculatus) eggs in the southeastern Bering Sea was examined with collections made in mid-May in 2002, 2003, 2005, and 2006. Eggs in the early stages of development were found primarily offshore of the 40-m isobath. Eggs in the middle and late stages of development were found inshore and offshore of the 40-m isobath. There was some evidence that early-stage eggs occur deeper in the water column than late-stage eggs, although year-to-year variability in that trend was observed. Most eggs were in the later stages of development; therefore the majority of spawning is estimated to have occurred a few weeks before collection—probably April—and may be highly synchronized among local spawning areas. Results indicate that sampling with continuous underway fish egg collectors(CUFES) should be supplemented with sampling of the entire water column to ensure adequate samples of all egg stages of Alaska plaice. Data presented offer new information on the stage-dependent horizontal and vertical distribution of Alaska plaice eggs in the Bering Sea and provide further evidence that the early life history stages of this species are vulnerable to near-surface variations in hydrographical conditions and climate forcing

The Flanagan Quality of Life Scale: Evidence of Construct Validity

BACKGROUND: The Quality of Life Scale (QOLS), developed originally by John Flanagan in the 1970's, has been adapted for use in chronic illness groups. Evidence for reliability and validity has been published over the years for both English and translations. This paper presents further evidence of construct validity for persons with chronic conditions as well as across two languages, and gender. METHODS: A sample of 1241 chronically ill and healthy adults from American and Swedish databases was used to generate factor analyses for both the 15-item original QOLS and the 16-item chronic illness adaptation. RESULTS: Analysis of the data suggested that the QOLS has three factors in the healthy sample and across chronic conditions, two languages and gender. Factors that could be labeled (1) Relationships and Material Well-Being, (2) Health and Functioning, and (3) Personal, Social and Community Commitment were identified. CONCLUSIONS: The QOLS is a valid instrument for measuring domains of quality of life across diverse patient groups

Awareness of the Red Dress Symbol and Heart Disease among College Women

The purpose of this study was to determine the level of concern and knowledge about heart disease among college women and to explore the relationship between awareness of the Red Dress symbol and knowledge of risk factors for heart disease. Female college students (n = 475) completed a survey. While 63% believed they are informed about heart disease, only 43% identified heart disease as the leading cause of death in women. Breast cancer (36%) was listed as the one greatest health concern facing women, followed by heart disease (29%). Chi-square analysis revealed that a significantly greater proportion of participants familiar with the Red Dress symbol (n = 157) identified heart disease as the greatest health problem facing women and the leading cause of death of women than participants unfamiliar with the symbol (n = 318). Participants familiar with the symbol identified 0.4-0.5 more correct choices on two questions related to causes of and activities to prevent heart disease than women unfamiliar with the symbol. College women are a prime target for a Red Dress campaign due to their low level of awareness. Young women can reduce their risk of developing heart disease through prevention

Tufted angioma presenting with subclinical coagulopathy

Tufted angioma is a rare, benign vascular tumor of uncertain pathogenesis, characterized histopathologically by "tufts" of capillaries within the dermis. A life-threatening coagulopathy, Kasabach-Merritt phenomenon, occurs in approximately 10% of cases of tufted angioma and is characterized by profound thrombocytopenia and fibrinogen consumption. We present an asymptomatic 10-month-old boy who presented with an erythematous patch of the right upper extremity and subsequently was diagnosed by biopsy with tufted angioma. Baseline laboratory workup of our patient revealed mildly decreased fibrinogen and elevated D-dimer levels without thrombocytopenia or elevated partial thromboplastin time. This suggests that asymptomatic patients with tufted angioma may present with coagulopathy in the absence of Kasabach-Merritt phenomenon. This also highlights the importance of obtaining baseline laboratory workup in patients presenting with tufted angioma

The Effects of Fire on the Function of the 200-BP-1 Engineered Surface Barrier

A critical unknown in use of barrier technology for long-term waste isolation is performance after a major disturbance especially when institutional controls are intact, but there are no resources to implement corrective actions. The objective of this study was to quantify the effects of wild fire on alterations the function of an engineered barrier. A controlled burn September 26, 2008 was used to remove all the vegetation from the north side of the barrier. Flame heights exceeded 9 m and temperatures ranged from 250 oC at 1.5 cm below the surface to over 700 oC at 1 m above the surface. Post-fire analysis of soil properties show significant decreases in wettability, hydraulic conductivity, air entry pressure, organic matter, and porosity relative to pre-fire conditions whereas dry bulk density increased. Decreases in hydraulic conductivity and wettabilty immediately after the fire are implicated in a surface runoff event that occurred in January 2009, the first in 13 years. There was a significant increase in macro-nutrients, pH, and electrical conductivity. After one year, hydrophobicity has returned to pre-burn levels with only 16% of samples still showing signs of decreased wettability. Over the same period, hydraulic conductivity and air entry pressure returned to pre-burn levels at one third of the locations but remained identical to values recorded immediately after the fire at the other two thirds. Soil nutrients, pH, and electrical conductivity remain elevated after 1 year. Species composition on the burned surface changed markedly from prior years and relative to the unburned surface and two analog sites. An increase in the proportion of annuals and biennials is characteristic of burned surfaces that have become dominated by ruderal species. Greenhouse seedling emergence tests conducted to assess the seed bank of pre- and post-burn soils and of two analog sites at the McGee Ranch show no difference in the number of species emerging from soils collected before and after the fire. However, there were fewer species emerging from the seed bank on the side slopes and more species emerging from two analog sites. Leaf area index measures confirmed the substantial differences in plant communities after fire. Xylem pressure potential were considerably higher on the burned half of the barrier in September 2009 suggesting that not all the water in the soil profile will be removed before the fall rains begin. The results of this study are expected to contribute to a better understanding of barrier performance after major disturbances in a post-institutional control environment. Such an understanding is needed to enhance stakeholder acceptance regarding the long-term efficacy of engineered barriers. This study will also support improvements in the design of evapotranspiration (ET) and hybrid (ET + capacitive) barriers and the performance monitoring systems

Using Decision Analysis to Improve Malaria Control Policy Making

Malaria and other vector-borne diseases represent a significant and growing burden in many tropical countries. Successfully addressing these threats will require policies that expand access to and use of existing control methods, such as insecticide-treated bed nets (ITNs) and artemesinin combination therapies (ACTs) for malaria, while weighing the costs and benefits of alternative approaches over time. This paper argues that decision analysis provides a valuable framework for formulating such policies and combating the emergence and re-emergence of malaria and other diseases. We outline five challenges that policy makers and practitioners face in the struggle against malaria, and demonstrate how decision analysis can help to address and overcome these challenges. A prototype decision analysis framework for malaria control in Tanzania is presented, highlighting the key components that a decision support tool should include. Developing and applying such a framework can promote stronger and more effective linkages between research and policy, ultimately helping to reduce the burden of malaria and other vector-borne diseases

200-BP-1 Prototype Hanford Barrier - 15 Years of Performance Monitoring

Engineered surface barriers are recognized as a remedial alternative to the removal, treatment and disposal of near-surface contaminants at a variety of waste sites within the DOE complex. One issue impacting their acceptance by stakeholders the use of limited data to predict long-term performance. In 1994, a 2-ha multi-component barrier was constructed over an existing waste disposal site at Hanford using natural materials. Monitoring has been almost continuous for the last 15 yrs and has focused on barrier stability, vegetative cover, plant and animal intrusion, and the components of the water balance, including precipitation, runoff, storage, drainage, and percolation. The total precipitation received from October 1994 through August 2008 was 3311 mm on the northern half (formerly irrigated), and 2638 mm on the southern, non-irrigated half. Water storage in the fine-soil layer shows a cyclic pattern, increasing in the winter and decreasing in the spring and summer to a lower limit of around 100 mm, regardless of precipitation, in response to evapotranspiration. Topographic surveys show the barrier and side slopes to be stable and the pea-gravel admix has proven effective in minimizing erosion through the creation of a desert pavement during deflationary periods. Three runoff events have been observed but the 600-mm design storage capacity has never been exceeded. Total percolation ranged from near zero amounts under the soil-covered plots to over 600 mm under the side slopes. The asphaltic concrete prevented any of this water from reaching the buried waste thereby eliminating the driving force for the contaminant remobilization. Plant surveys show a relatively high coverage of native plants still persists after the initial revegetation although the number of species decreased from 35 in 1994 to 10 in 2009. Ample evidence of insect and small mammal use suggests that the barrier is behaving like a recovering ecosystem. In September 2008, the north half of the barrier was burned to remove vegetation and study the effects of fire on barrier performance. The most immediate effects has been on water storage patterns with the bare surface showing a slower accumulation of water, a smaller peak storage and a delayed release relative to the unburned side due to evaporation . Nonetheless the residual storage at the end of the year was similar for the burned and unburned sides