A RARE CASE OF HARLEQUIN ICHTHYOSIS SUCCESSFULLY TREATED WITH ACITRETIN: A CASE REPORT AND LITERATURE REVIEW

Harlequin Ichthyosis is the most serious congenital keratinization disorder. When the children are born, they are enveloped in thick horn armor. They are thick yellow horn plates that tear deeply when they dry out. In the most severe form, the children often die in the first few weeks of life. But there are also many milder courses, whereby there are obviously flowing transitions from collodion baby to harlequin ichthyosis. The skin condition later corresponds to that of a child with severe congenital ichthyosis (ARCI). Similar to the collodion baby, cases of harlequin ichthyosis should initially be cared for in the intensive care unit for newborns and require interdisciplinary therapy. Harlequin ichthyosis is caused by very special mutations in the ABCA12 gene. These mutations also have an impact on survival. If homozygous mutations are present, the prospects are worse than if the parents have heterozygous mutations. Homozygous mutations are often present when the parents are consanguineous.

Finding Out Suitable Index for Wetland Mapping in Barind Plain of India and Predicting Dynamics of Its Area and Depth

Remote Sensing and GIS play an important role in mapping and monitoring natural resources and their management. The present study attempts to delineate wetland in the lower Tangon river basin in the Barind flood plain region using suitable water body extraction indices. The main objectives of this present study are mapping and monitoring the flood plains wetlands along with the future status of wetland areas of 2028 and 2038 using the advanced Artificial Neural Network-based Cellular Automata (ANN-CA) model. Apart from wetland area prediction, wetland depth simulation and prediction are also carried out using statistical (Adaptive Exponential Smoothing) as well as advanced machine learning algorithms such as Bagging, Random subspace, Random forest, Support vector machine, etc. for the year 2028. The result shows a remarkable change in the overall wetland area in the upcoming two decades. The small wetland patches away from the master stream are expected to dry out during the forecast period, where the major wetland patches nearer to the master stream with greater depth are rather sustainable but their depth of water may be reduced in the next decades. All models show satisfactory performance for wetland depth mapping, but the Random subspace model was identified as the best-suited depth predicting method and machine learning models explored better results that adaptive exponential smoothing. This recent study will definitely be very helpful for the policymakers for managing wetland landscape as well as the natural environment.

Hygrothermal Monitoring of Two Pilot Prefabricated Exterior Energy Retrofit Panel Designs

NRCan undertook a proof-of-concept project to retrofit a small building with prefabricated wall panels in 2017 in Ottawa, Canada. The retrofit used two wall panel designs: nailbase and woodframe. The Nailbase panel consisted of fiberglass batt, an expanded polystyrene (EPS) core, oriented strand board (OSB) sheathing, a rainscreen, and cladding. The Woodframe panel also featured OSB sheathing and included a 90 mm stand-off gap filled with dense-packed, fibrous insulation. A side-by-side comparison of cost, constructability, and performance was performed. The wall assemblies were instrumented to monitor the temperature, relative humidity, and moisture content of sensitive layers. The data was used to evaluate the hygrothermal performance, moisture accumulation, and risk of associated problems such as mould growth. This paper presents the monitored hygrothermal data from 2017 to 2021, compares the two approaches and assesses their feasibility. During construction, some of the fibrous insulation may have been wetted by wind-driven snow before completion. The data showed that this moisture was able to dissipate without significant risk. The sheathing of the Woodframe panel experienced a higher peak moisture content during the dry-out period. Otherwise, both panel designs showed limited potential for mould growth on monitored surfaces over the monitored period.

Experimental analysis of moisture uptake and dry-out in CLT end-grain exposed to free water

This paper presents the results of a series of laboratory tests of CLT end-grain moisture uptake and dry-out. We put CLT test details (TDs) in direct water contact from the end-grain edge and then left the TDs to dry for two weeks in the laboratory and in an outside shelter. Half of the TDs had their wet sides attached to another CLT detail. Fibre saturation point was quickly reached in the bottom part of the TDs during the seven-day water contact. A tendency of increasing moisture content (MC) was up to 90 mm from the wet edges, but we did not record MC levels above the critical level at that height. However, MC exceeded critical levels at 60 mm from the water level. The measured water absorption coefficient Aw was 3.51×10−3 kg/m2-s0’5. Drying was negligible for the TDs which were in contact with another CLT detail. Thus, moisture dry-out is very complicated in joints where the CLT end-grain is covered, such as the exterior wall to foundation or intermediate ceiling connection. The dry-out of CLT is not expected in a cold and humid outdoor environment once the CLT end-grain has absorbed moisture even with wet edges exposed to air.

The use of an internal airtight membrane in CLT external wall in terms of hygrothermal performance

This study focused on the dry-out capacity of the vapor-permeable CLT (cross-laminated timber) external wall and the impact of using an internal airtight membrane. The results of the work were obtained first from the field measurements, after which the simulation model was created and validated, and the hygrothermal performance of the wall was analyzed by a stochastic approach. The results of this showed that the CLT dries out quickly and safely in a wall assembly with a high water vapor permeability, even with the large range of initial CLT MC (13-25%). When an additional airtight layer with high vapor diffusion resistance (Sd of 244 m) is added between the insulation and the CLT, the dry-out capacity of the CLT decreases significantly and there is a high probability of mold growth on the CLT surface. The risk of mold growth can be prevented when the vapor resistance (Sd) of the airtight layer is reduced to 1.5 m in a case where initial CLT MC is up to 25% and in a case where initial MC is up to 20%, the vapor resistance of an airtight layer must be reduced to 3 m.

Anthecological features of Lonicera japonica Thunb. in the environments of Southern Uzbekistan

Background. Identification of spectacular ornamental perennial lianas for urban landscaping in southern cities is important for modern urban greening programs. The aim of this work was to study flowering peculiarities of Japanese honeysuckle (Lonicera japonica Thunb.).Materials and methods. The target material was honeysuckle plants growing in the environments of Southern Uzbekistan. Observations were conducted in 2019–2020 using conventional approaches.Results and conclusion. The inflorescence of L. japonica is an open dibotryoid, with flowers arranged along the lateral axes of the first order. Flowers are bisexual, zygomorphic. Blossoming of flowers in inflorescences occurs acropetally. The flowering period is almost 150–170 days (starts in April and lasts until the end of August). Dichogamy in the form of proterandry is observed in honeysuckle flowers. The male flowering phase comes the first. It starts 1.5–2.0 hours after the opening of the corolla and ends with the drying of the stamens. The corolla turns yellow 36 hours after the blooming of the flower and the stamens begin to dry out. This is the female phase. Over the next 84 hours, the pistil begins to dry out slowly, but the corolla of the flower lasts up to 96 hours. The duration of the female flowering phase is 60 hours. L. japonica is of considerable interest for vertical landscaping of urban communities. Plants remain ornamental for a long time. Studying this species as an essential oil plant is promising, since it contains a significant amount of germacrene D.

Projecting the impacts of end of century climate extremes on the hydrology in California

In California, it is essential to understand the evolution of water resources in response to a changing climate to sustain its economy and agriculture and build resilient communities. Although extreme conditions have characterized the historical hydroclimate of California, climate change will likely intensify hydroclimatic extremes by the End of Century (EoC). However, few studies have investigated the impacts of EoC extremes on watershed hydrology. We use cutting-edge global climate and integrated hydrologic models to simulate EoC extremes and their effects on the water-energy balance. We assess the impacts of projected driest, median, and wettest water years under a Representative Concentration Pathway (RCP) 8.5 on the hydrodynamics of the Cosumnes river basin. High temperatures (> 2.5 °C) and precipitation (> 38 %) will characterize the EoC extreme water years compared to their historical counterparts. Also, precipitation, mostly in the form of rain, is projected to fall earlier. This change reduces snowpack by more than 90 %, increases peak surface water and groundwater storages up to 75 % and 23 %, respectively, and makes these peak storages occur earlier in the year. Because EoC temperatures and soil moisture are high, both potential and actual evapotranspiration (ET) increase. The latter, along with the lack of snowmelt in the warm EoC, cause surface water and groundwater storages to significantly decrease in summer, with groundwater showing the highest rates of decrease. Besides, the changes in the precipitation phase lead the lower-order streams to dry out in EoC summer whereas the mainstream experiences an increase in storage.