Identifying information needs among children and teens living with haemophilia.

Transitioning from one life stage to the next can be difficult, but for those living with a chronic condition, it can be even more challenging. Children and adolescents with haemophilia need help to manage transitions while dealing with the complications of their disorder. The National Haemophilia Foundation (NHF), headquartered in New York City, has an extensive information centre on bleeding disorders, but it was not clear how much material existed on the topic of transition. The objectives of this project were to (i) assess the availability of literature about transition for children and adolescents living with haemophilia, (ii) determine which transition issues were the most relevant and (iii) develop and test information products that would address those transition issues. An inventory of NHF's resources and an environmental scan over the Internet was performed. Focus groups were conducted to determine messaging. Video prototypes containing messages were created, tested by focus groups and revised. The literature search yielded limited information available on transition for children and adolescents with haemophilia. Results of the formative research indicated that adolescents wanted more information on sports participation and disclosure of their condition (e.g. to peers, teachers, coaches, health care providers). Video was found to be the preferred delivery format. Children and adolescents living with haemophilia need information to help them transition through life. As a result of this study, two educational products were produced, but several more are recommended to guide these individuals in making healthy transitions into adulthood.

Gonadal dysgenesis determinants in a natural population of Drosophila melanogaster.

Three populations of Drosophila melanogaster from northern California were surveyed for the ability to produce and resist gonadal dysgenesis in the P-M system of hybrid dysgenesis. Males from all three populations produced low to moderate levels of gonadal dysgenesis in crosses to Oregon-R M females. Most females had the P cytotype, but the M cytotype occurred occasionally. The three populations could not be statistically differentiated from one another, but were easily distinguished from populations from Australia and Wisconsin on the basis of gonadal dysgenesis potential. The California populations had higher levels of M cytotype than did the Wisconsin population. Thirteen X chromosomes and 11 pairs of autosomes were extracted from one of the California populations, using a modification of the standard balancer chromosome technique to suppress hybrid dysgenesis during extraction. All lines produced strongly skewed sterility distributions in crosses to M-strain females, and mean levels of sterility were less than 50%. There was evidence of nonadditive interactions between the autosomes. Most extraction lines had the P cytotype, but M and intermediate cytotypes were observed. Some of the intermediate cytotypes were stable over time. Lines were tested at two different times after extraction. Some lines evolved higher sterility potential as they were kept in the laboratory, even in the presence of P cytotype. The results point out a number of deficiencies in current genetic and population genetic models of hybrid dysgenesis and imply that gonadal dysgenesis is unlikely to be an important evolutionary force in this population.

Cryoconite holes on glaciers.

Cryoconite holes are water-filled depressions on the surface of glaciers. They contain microbial communities and may contribute to glacial wastage and biological colonization of ice-free areas.

Successful pregnancy outcome in association with lipoatrophic diabetes mellitus.

Lipoatrophic diabetes mellitus is a rare syndrome characterized by lipoatrophy and insulin-resistant diabetes mellitus. Partial lipodystrophy without clinical diabetes mellitus has been associated with intrauterine growth retardation and fetal death. We report successful pregnancy outcomes in two women with lipoatrophic diabetes mellitus.

Maternal brain death and prolonged fetal survival.

A 30-year-old woman suffered massive brain injuries after a motor vehicle accident at 15 weeks' gestation. The patient was diagnosed as brain-dead on her tenth hospital day. She was supported with intensive care for 107 days after this diagnosis, and a normal 1555-g male infant was delivered at approximately 32 weeks' gestation by repeat cesarean section. The child is developing normally at 11 months of age. This represents the longest reported case of prolongation of pregnancy after brain death.

Oxygen budget of a perennially ice-covered Antarctic lake.

A bulk O2 budget for Lake Hoare, Antarctica, is presented. Five years of seasonal data show the lake to be persistently supersaturated with O2. Oxygen is carried into the lake in glacial meltstreams and is left behind when this water is removed as ice by ablation and sublimation. A diffusive loss of O2 from the lake through the summer moat is suggested. Measured values of the total O2 in the water column indicate that the time scale of O2 turnover is much longer than a year. Based on these results we suggest that the amount of O2 in the water does not change significantly throughout the year and that the lake is also supersaturated with N2.

Sediment oxygen profiles in a super-oxygenated antarctic lake.

Perennially ice-covered lakes are found in the McMurdo Dry Valleys of southern Victoria Land, Antarctica. In contrast to temperate lakes that have diurnal photic periods, antarctic (and arctic) lakes have a yearly photic period. An unusual feature of the antarctic lakes is the occurrence of O2 at supersaturated levels in certain portions of the water column. Here we report the first sediment O2 profiles obtained using a microelectrode from a perennially ice-covered antarctic lake. Sediment cores collected in January and October 1987 from Lake Hoare in Taylor Valley show oxygenation down to 15, and in some cases, 25 cm. The oxygenation of sediments several centimeters below the sediment-water interface is atypical for lake sediments and may be characteristic of perennially ice-covered lakes. There is a significant difference between the observed January and October sediment O2 profiles. Several explanations may account for the difference, including seasonality. A time-dependent model is presented which tests the feasibility of a seasonal cycle resulting from the long photoperiod and benthic primary production in sediments overlain by a highly oxygenated water column.

Treatment of calcified cephalohematoma.

This is a case report concerning the surgical treatment of a calcified cephalohematoma, which was possibly caused by an intrauterine fetal monitor. This is the first report of this particular entity as a complication of an intrauterine fetal monitor. As far as we can determine, there is no detailed information available about surgical decision making or surgical techniques for removing such lesions. We operated because of the size and persistence of the lesion. We developed a procedure in which we used the bony cap of the cephalohematoma for a cranioplasty, securing it with microplates.

Changes in ice cover thickness and lake level of Lake Hoare, Antarctica: implications for local climatic change.

We report results from 10 years of ice thickness measurements at perennially ice-covered Lake Hoare in southern Victoria Land, Antarctica. The ice cover of this lake had been thinning steadily at a rate exceeding 20 cm yr-1 during the last decade but seems to have recently stabilized at a thickness of 3.3 m. Data concerning lake level and degree-days above freezing are presented to show the relationship between peak summer temperatures and the volume of glacier-derived meltwater entering Lake Hoare each summer. From these latter data we infer that peak summer temperatures have been above 0 degrees C for a progressively longer period of time each year since 1972. We also consider possible explanations for the thinning of the lake ice. The thickness of the ice cover is determined by the balance between freezing during the winter and ablation that occurs all year but maximizes in summer. We suggest that the term most likely responsible for the change in the ice cover thickness at Lake Hoare is the extent of summer melting, consistent with the rising lake levels.

Early martian environments: the Antarctic and other terrestrial analogs.

The comparability of the early environments of Mars and Earth, and the biological evolution which occurred on early earth, motivates serious consideration of the possibility of an early martian biota. Environments which could have contained this early martian life and which may presently contain evidence of this former life include aquatic, ice, soil, and rock habitats. Several analogs of these potential early martian environments, which can provide useful information in searching for extinct life on Mars, are currently available for study on Earth. These terrestrial analogs include the perennially ice-covered lakes and sandstone rocks in the Polar Deserts of Antarctica, surface of snowfields and glaciers, desert soils, geothermal springs, and deep subsurface environments.

Horizontal transfer of hobo transposable elements within the Drosophila melanogaster species complex: evidence from DNA sequencing.

The hobo family of transposable elements, one of three transposable-element families that cause hybrid dysgenesis in Drosophila melanogaster, appears to be present in all members of the D. melanogaster species complex: D. melanogaster, D. simulans, D. mauritiana, and D. sechellia. Some hobo-hybridizing sequences are also found in the other members of the melanogaster subgroup and in many members of the related montium subgroup. Surveys of older isofemale lines of D. melanogaster suggest that complete hobo elements were absent prior to 50 years ago and that hobo has recently been introduced into the species by horizontal transfer. To test the horizontal transfer hypothesis, the 2.6-kb XhoI fragments of hobo elements from D. melanogaster, D. simulans, and D. mauritiana were cloned and sequenced. The DNA sequences reveal an extremely low level of divergence and support the conclusion that the active hobo element has been horizontally transferred into or among these species in the recent past.

Perennially ice-covered Lake Hoare, Antarctica: physical environment, biology and sedimentation.

Lake Hoare (77 degrees 38' S, 162 degrees 53' E) is a perennially ice-covered lake at the eastern end of Taylor Valley in southern Victoria Land, Antarctica. The environment of this lake is controlled by the relatively thick ice cover (3-5 m) which eliminates wind generated currents, restricts gas exchange and sediment deposition, and reduces light penetration. The ice cover is in turn largely controlled by the extreme seasonality of Antarctica and local climate. Lake Hoare and other dry valley lakes may be sensitive indicators of short term (< 100 yr) climatic and/or anthropogenic changes in the dry valleys since the onset of intensive exploration over 30 years ago. The time constants for turnover of the water column and lake ice are 50 and 10 years, respectively. The turnover time for atmospheric gases in the lake is 30-60 years. Therefore, the lake environment responds to changes on a 10-100 year timescale. Because the ice cover has a controlling influence on the lake (e.g. light penetration, gas content of water, and sediment deposition), it is probable that small changes in ice ablation, sediment loading on the ice cover, or glacial meltwater (or groundwater) inflow will affect ice cover dynamics and will have a major impact on the lake environment and biota.

Response of an antarctic lake heterotrophic community to high dissolved oxygen.

The upper waters of Lake Hoare, Antarctica, contain dissolved oxygen at about three times the normal saturation (>/=42 mg liter). The response of the heterotrophic plankton community to this high dissolved oxygen was evaluated by the criteria of CFU and d-[U-C]glucose assimilated-respired. High dissolved oxygen was not inhibitory to d-[U-C]glucose assimilation-respiration compared with normal atmospheric dissolved oxygen in Lake Hoare water. The d-[U-C]glucose was assimilated and respired optimally at 12 degrees C in Lake Hoare. The d-[U-C]glucose assimilated-respired in the upper saturated atmospheric dissolved oxygen waters of Mountain Lake, Va., was inhibited in contrast to Lake Hoare (P < 0.05). CFU formation was inhibited in both lakes. CFU represent <1% of the fluorochrome-stained direct counts in Lake Hoare. Lake Hoare planktobacteria are smaller than the planktobacteria in Mountain Lake. ATP size fractionation revealed that 39% of the ATP biomass was <0.5 mum in Lake Hoare.

Polymorphism and divergence at the prune locus in Drosophila melanogaster and D. simulans.

The prune locus of Drosophila melanogaster lies at the tip of the X chromosome, in a region of reduced recombination in which nearby loci show reduced variation relative to evolutionary divergence from D. simulans. DNA sequencing of prune alleles from D. melanogaster and D. simulans reveals extremely low variation in D. melanogaster but greater variation in D. simulans. Divergence between the two species is not reduced. This pattern may be explained by either positive selection leading to hitchhiking of neutral variation or background selection against deleterious mutations. The pattern of silent versus replacement polymorphism and divergence at prune is consistent with either a model of weakly deleterious selection against amino acid substitutions or balancing selection.

Sensitivity of an oligotrophic lake planktonic bacterial community to oxygen stress.

Dissolved oxygen at approximately four times normal saturation (42 mg liter) inhibited the growth and metabolism of summer planktonic bacteria in the surface water of alpine oligotrophic Mountain Lake (Giles County, Va.). Data were derived from growth of CFU on membrane filters, d-[U-C]glucose incorporation into the extractable lipid of these CFU, and respiration and assimilation of d-[U-C]glucose by lake water samples. Statistically significant (alpha < 0.05) differences were not detected in either CFU or C incorporation in lipid when superoxide dismutase (30 U ml) or catalase (130 U ml) was added to the medium. Thus, exogenous oxygen by-products apparently are not responsible for the observed inhibition of growth and metabolism.

Climatological observations and predicted sublimation rates at Lake Hoare, Antarctica.

In December 1985, an automated meteorological station was established at Lake Hoare in the dry valley region of Antarctica. Here, we report on the first year-round observations available for any site in Taylor Valley. This dataset augments the year-round data obtained at Lake Vanda (Wright Valley) by winter-over crews during the late 1960s and early 1970s. The mean annual solar flux at Lake Hoare was 92 W m-2 during 1986, the mean air temperature -17.3 degrees C, and the mean 3-m wind speed 3.3 m s-1. The local climate is controlled by the wind regime during the 4-month sunless winter and by seasonal and diurnal variations in the incident solar flux during the remainder of the year. Temperature increases of 20 degrees-30 degrees C are frequently observed during the winter due to strong föhn winds descending from the Polar Plateau. A model incorporating nonsteady molecular diffusion into Kolmogorov-scale eddies in the interfacial layer and similarity-theory flux-profiles in the surface sublayer, is used to determine the rate of ice sublimation from the acquired meteorological data. Despite the frequent occurrence of strong winter föhns, the bulk of the annual ablation occurs during the summer due to elevated temperatures and persistent moderate winds. The annual ablation from Lake Hoare is estimated to have been 35.0 +/- 6.3 cm for 1986.

Enhanced visualization of soft tissues in the study of aborted fetuses through the use of xeroradiography.

As part of the diagnostic workup following an episode of fetal loss, it is generally recommended that fetal tissue be submitted for chromosome analysis and that the fetus be photographed and radiographed. Our recent clinical experience has suggested that, in those fetuses where size is compatible, xeroradiography may be superior to standard radiography. Xeroradiography utilizes principles similar to those in film radiology, with low-energy photon beams and relatively long exposure times. The physical characteristics of the beam and imaging system provide optimal soft tissue visualization. We have found this technique to be of use in studying a broad variety of abortuses with abnormalities. Examples of fetal abnormalities in which we have used this technique include acardia, neural tube defects, nuchal cystic hygroma, and arthrogryposis. In fetuses weighing 500-1,000 gm, the exposure settings are 25 MA, 1 second, 40 KVP. For larger fetuses the KVP should be increased by 1 or 2. This technique has not been useful for a complete body view of large fetuses due to the size limitations of the xeroradiographic cassette itself.

Perennial N2 supersaturation in an Antarctic lake.

The dry valleys of southern Victoria Land in Antarctica contain several closed basins in which perennially ice-covered lakes are found. One of the most unusual features of these lakes is the occurrence of high O2 concentrations in the water column; values ranging from slightly more than saturation to more than four times saturation have been reported. Recently, we considered a bulk O2 budget for Lake Hoare, Antarctica, which led us to suggest that biological processes alone were not sufficient to explain the observed elevated oxygen levels. Consequently, there must be a non-biological source of O2. We suggested that this source results from the exclusion of O2 during the freezing of aerated meltstream water at the bottom of the ice cover, and predicted that this physical mechanism should also enhance the other atmospheric gases. Here we report the results of a study which, for the first time, documents the supersaturation of N2 in a lake. Dissolved N2 levels of 145% and 163% were determined from samples taken just below the ice cover and at a depth of 12 m, respectively. The relatively importance of biological and abiological sources is reflected in the ratio of N2 concentration to O2 concentration. In Lake Hoare this ratio was 1.20 at ice/water interface and 1.05 at 12 m; considerably different from the ratio in equilibrium with air (approximately 1.8). Based on these results, we have determined that about half of the net O2 production in the lake is the result of biological processes.

Characteristics of the Drosophila paulistorum Male Sterility Agent in a Secondary Host, Ephestia kuehniella.

Crosses among the six semispecies of Drosophila paulistorum produce sterile male hybrids. This sterility is caused by an agent which has characteristics of a microorganism. It is pathogenic in a secondary host, the larvae of the Mediterranean meal moth, Ephestia kuehniella, and can be serially passaged in Ephestia, where it is lethal. The agent was passaged back into D. paulistorum, where it induced sterility in males of a semispecies different from that of origin of the agent. Infectious particles were obtained from an extract of infected Ephestia by ultracentrifugation in a sucrose-Ficoll-metrizamide gradient. Both crude and purified extracts were lyophilized and stored indefinitely without loss of killing power. The agent was destroyed by low pH, lipid solvents, ultraviolet light, and exposure to a temperature of 56 degrees C for 30 min. It appeared to be sensitive to tetracycline and insensitive to penicillin, suggesting that the agent is not a virus, but more likely a cell wall-deficient bacterium or mycoplasma-like organism.