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Contains fulltext : 293193.pdf (Publisher’s version ) (Open Access)

Item does not contain fulltext, Congenital adrenal hyperplasia (CAH) is a group of autosomal recessive disorders affecting cortisol biosynthesis. Reduced activity of an enzyme required for cortisol production leads to chronic overstimulation of the adrenal cortex and accumulation of precursors proximal to the blocked enzymatic step. The most common form of CAH is caused by steroid 21- hydroxylase deficiency due to mutations in CYP21A2. Since the last publication summarizing CAH in Endocrine Reviews in 2000 there have been numerous new developments. These include more detailed understanding of steroidogenic pathways, refinements in neonatal screening, improved diagnostic measurements utilizing chromatography and mass spectrometry coupled with steroid profiling, and improved genotyping methods. Clinical trials of alternative medications and modes of delivery have been recently completed or are under way. Genetic and cell-based treatments are being explored. A large body of data concerning long-term outcomes in patients affected by CAH, including psychosexual well-being, has been enhanced by the establishment of disease registries. This review provides the reader with current insights in congenital adrenal hyperplasia with special attention to these new developments.

Contains fulltext : 253204.pdf (Publisher’s version ) (Open Access)

Contains fulltext : 283126.pdf (Publisher’s version ) (Open Access), OBJECTIVE: To assess the current medical practice in Europe regarding prenatal dexamethasone (Pdex) treatment of congenital adrenal hyperplasia (CAH) due to 21-hydroxylase deficiency. DESIGN AND METHODS: A questionnaire was designed and distributed, including 17 questions collecting quantitative and qualitative data. Thirty-six medical centres from 14 European countries responded and 30 out of 36 centres were reference centres of the European Reference Network on Rare Endocrine Conditions, EndoERN. RESULTS: Pdex treatment is currently provided by 36% of the surveyed centres. The treatment is initiated by different specialties, that is paediatricians, endocrinologists, gynaecologists or geneticists. Regarding the starting point of Pdex, 23% stated to initiate therapy at 4-5 weeks postconception (wpc), 31% at 6 wpc and 46 % as early as pregnancy is confirmed and before 7 wpc at the latest. A dose of 20 µg/kg/day is used. Dose distribution among the centres varies from once to thrice daily. Prenatal diagnostics for treated cases are conducted in 72% of the responding centres. Cases treated per country and year vary between 0.5 and 8.25. Registries for long-term follow-up are only available at 46% of the centres that are using Pdex treatment. National registries are only available in Sweden and France. CONCLUSIONS: This study reveals a high international variability and discrepancy in the use of Pdex treatment across Europe. It highlights the importance of a European cooperation initiative for a joint international prospective trial to establish evidence-based guidelines on prenatal diagnostics, treatment and follow-up of pregnancies at risk for CAH.

Item does not contain fulltext, INTRODUCTION: There is wide variation in reported rates of acute adrenal insufficiency (AI)-related adverse events (sick day episodes and adrenal crises) between centres. This study aimed to evaluate the level of consensus on criteria considered essential for defining and managing these events in children with Congenital Adrenal Hyperplasia. METHODS: Active users of the International Congenital Adrenal Hyperplasia and International Disorders of Sex Development (I-CAH/I-DSD) Registries (n = 66), non-active users of I-CAH/I-DSD (n = 35), and the EuRRECa e-Reporting Registry (n = 10) were approached to complete an online survey. RESULTS: Fifty-six centres from 27 countries responded to the survey; the response rates for the three groups were 42 (65%), 11 (31%), and 3 (30%), respectively. Steroid management plans, one to one patient education, and contact details of health care staff were provided by over 90% of centres in high-income countries. All 56 centres advised glucocorticoid stress dosing in the event of fever. Less common indications for sick day dosing included vaccination and mild afebrile intercurrent illness, recommended by 17 (30%) and 9 (16%) centres, respectively. The most frequently reported stress dosing regimens were tripling the total daily dose of hydrocortisone and administering 3 times daily and doubling or tripling the largest daily hydrocortisone dose depending on the nature of the trigger and administering 3 times daily, recommended by 24 (43%) and 21 (38%) centres, respectively. Vomiting was the most common indication for intramuscular hydrocortisone injection, reported by 34 (61%) centres. Over 50% of respondents indicated that essential clinical criteria for adrenal crisis should include fatigue and nausea or vomiting and over 60% indicated that hypotension, hyponatraemia, hyperkalaemia, and clinical improvement following parenteral glucocorticoids were essential criteria. In the event of an adrenal crisis, 47 (84%) reported that the majorit

Phase change materials (PCMs) have garnered significant attention as low-cost thermal energy storage systems that efficiently capture and store solar energy. Recent review works have largely focused only on thermal conductivity enhancement techniques, and/or applications of PCMs, while others have mainly discussed the performance enhancement of either heating, cooling, or clean energy storage systems integrating with PCMs. However, not enough studies recently reviewed all of these techniques/systems comprehensively to provide insights into them. This paper thus comprehensively reviews the integration of PCMs as an enhancement to most types of heating, cooling, and clean energy storage system performance, and the techniques to enhance thermal conductivity. The integration of PCMs with these systems has shown promising performance. For instance, an improvement of 13.5% is found in the efficiency of photovoltaic (PV) system when it is integrated with PCM/Al2O3 nanoparticles. In addition, the solar air heater's daily energy efficiency reaches 17% on its own, but when combined with PCM, it reaches 33%. However, the major drawback of using PCM–TES (thermal energy storage) for cooling is that PCM does not entirely solidify at night. The literature also shows that the issues related to PCMs' low thermal conductivity, phase separation, and subcooling/supercooling, their poor compatibility with other materials, and the environmental hazards they pose hinder their application on a large scale. It is necessary to implement international standards for assessing the thermophysical properties of PCMs and compile data to better facilitate the utilization of PCMs by end-users.

Wastewater containing toxic substances is a major threat to the health of both aquatic and terrestrial ecosystems. In order to treat wastewater, nanomaterials are currently being studied intensively due to their unprecedented properties. The unique features of nanoparticles are prompting an increasing number of studies into their use in wastewater treatment. Although several studies have been undertaken in recent years, most of them did not focus on some of the nanomaterials that are now often utilized for wastewater treatment. It is essential to investigate the most recent advances in all the types of nanomaterials that are now frequently employed for wastewater treatment. The recent advancements in common nanomaterials used for sustainable wastewater treatment is comprehensively reviewed in this paper. This paper also thoroughly assesses unique features, proper utilization, future prospects, and current limitations of green nanotechnology in wastewater treatment. Zero-valent metal and metal oxide nanoparticles, especially iron oxides were shown to be more effective than traditional carbon nanotubes (CNTs) for recovering heavy metals in wastewater. Iron oxide achieved 75.9% COD (chemical oxygen demand) removal efficiency while titanium oxide (TiO2) achieved 75.5% COD. Iron nanoparticles attained 72.1% methyl blue removal efficiency. However, since only a few types of nanomaterials have been commercialized, it is important to also focus on the economic feasibility of each nanomaterial. This study found that the large surface area, high reactivity, and strong mechanical properties of nanoparticles means they can be considered as a promising option for successful wastewater treatment.