Monogenic forms of hypertension

Arterial hypertension in childhood is less frequent as compared to adulthood but is more likely to be secondary to an underlying disorder. After ruling out more obvious causes, some patients still present with strongly suspected secondary hypertension of yet unknown etiology. A number of these children have hypertension due to single gene mutations inherited in an autosomal dominant or recessive fashion. The finding of abnormal potassium levels (low or high) in the presence of suppressed renin secretion, and metabolic alkalosis or acidosis should prompt consideration of these familial diseases. However, mild hypertension and the absence of electrolyte abnormalities do not exclude hereditary conditions. In monogenic hypertensive disorders, three distinct mechanisms leading to the common final pathway of increased sodium reabsorption, volume expansion, and low plasma renin activity are documented. The first mechanism relates to gain-of-function mutations with a subsequent hyperactivity of renal sodium and chloride reabsorption leading to plasma volume expansion (e.g., Liddle's syndrome, Gordon's syndrome). The second mechanism involves deficiencies of enzymes that regulate adrenal steroid hormone synthesis and deactivation (e.g., subtypes of congenital adrenal hyperplasia, apparent mineralocorticoid excess (AME)). The third mechanism is characterized by excessive aldosterone synthesis that escapes normal regulatory mechanisms and leading to volume-dependent hypertension in the presence of suppressed renin release (glucocorticoid remediable aldosteronism). Hormonal studies coupled with genetic testing can help in the early diagnosis of these disorder

Peripheral Facial Nerve Palsy in Severe Systemic Hypertension: A Systematic Review

BACKGROUND Signs of nervous system dysfunction such as headache or convulsions often occur in severe systemic hypertension. Less recognized is the association between severe hypertension and peripheral facial nerve palsy. The aim of this study was to systematically review the literature on the association of peripheral facial palsy with severe hypertension. METHODS Systematic review of Medline, Embase, Web of Science, and Google Scholar from 1960 through December 2011 and report of two cases. RESULTS The literature review revealed 24 cases to which we add two cases with severe hypertension and peripheral facial palsy. Twenty-three patients were children. Palsy was unilateral in 25 cases, bilateral in one case, and recurred in nine. The time between the first facial symptoms and diagnosis of hypertension was a median of 45 days (range, 0 days-2 years). In five case series addressing the complications of severe hypertension in children, 41 further cases of peripheral facial palsy were listed out of 860 patients (4.8%). CONCLUSIONS The association between severe hypertension and peripheral facial palsy is mainly described in children. Arterial hypertension is diagnosed with a substantial delay. Outcome is favorable with adequate antihypertensive treatment. The pathophysiology is still debate

Preliminary study of the hybrid solar DEC "nAC wall" system integration in building façades in urban context

A new concept of hybrid/natural air conditioning system, “NAC (Natural Air Conditioning) wall”, with a high level of architectural integration is presented. NAC wall concept is that of a solar DEC (Desiccant Evaporative Cooling) open cycle with very low pressure drops, thus drastically reducing, or even avoiding, the electricity consumption for driving fans. The supply air is dehumidified by an adsorption bed and is cooled indirectly by an evaporative cooler, through a low pressure drop heat exchanger. Adsorption bed is a finned coil heat exchanger coated with a SAPO-34 zeolite layer realizing both heat and mass transfer in a unique component. The assembling of NAC wall components is analysed in order to optimize architectural integration and performances. Experimental data carried out in different operation mode offered promising optimization suggestions to increase the specific cooling power for a better building integration. The integration at a building level would represent an architectural innovation, and the NAC wall production would not impact the supply chain with disruptive changes

A complex interprofessional intervention to improve the management of painful procedures in neonates

During hospitalization, neonates are exposed to a stressful environment and a high number of painful procedures. If pain is not treated adequately, short- and long-term complications may develop. Despite evidence about neonatal pain and available guidelines, procedural pain remains undertreated. This gap between research and practice is mostly due to limited implementation of evidence-based knowledge and time constraints. This study describes in detail the development process of a complex interprofessional intervention to improve the management of procedural pain in neonates called NEODOL© (NEOnato DOLore). The framework of the Medical Research Council (MRC) for the development and evaluation of complex interventions was used as a methodological guide for the design of the NEODOL© intervention. The development of the intervention is based on several steps and multiple methods. To report this process, we used the Criteria for Reporting the Development of Complex Interventions in Healthcare (CReDECI 2). Additionally, we evaluated the content of the intervention using a Delphi method to obtain consensus from experts, stakeholders, and parents. The complex interprofessional intervention, NEODOL©, is developed and designed for three groups: healthcare professionals, parents, and neonates for a level IIb neonatal unit at a regional hospital in southern Switzerland. A total of 16 panelists participated in the Delphi process. At the end of the Delphi process, the panelists endorsed the NEODOL© intervention as important and feasible. Following the MRC guidelines, a multimethod process was used to develop a complex interprofessional intervention to improve the management of painful procedures in newborns. Complex interprofessional interventions need theoretical bases, careful development, and integration of stakeholders to provide a comprehensive approach. The NEODOL intervention consists of promising components and has the potential to improve the management of painful procedures and should facilitate the knowledge translation into practice. KEYWORDS bundle of care, complex interventions, interprofessional relations, knowledge translation, neonate, procedural pai

A hybrid passive cooling wall system: concept and laboratory testing results

The research unit associated to the Laboratory Systems for Technology Innovation (LaSTIn) of the Department of Architecture and Design as well as the Department of Energy, Polytechnic University of Turin, has investigated the possibility of producing a modular wall system for hybrid/natural passive cooling. This system uses pressure differences typical of natural air movements and it is conceived as a mean to reach a quasi-zero-energy building as foreseen by Dir. 2010/31/EU by 2020 for new constructions. In addition, it realises a high level of technological and architectural integration in building constructions. The research focuses on passive and solar cooling techniques and studies the following possible systems, designed and tested separately in laboratory: a) latent heat adsorption cells including silica gel and zeolites for controlling the specific air humidity content, with heat regeneration by a vacuum water solar collectors system; b) a low-pressure heat exchanger, with crossing flows through ducts of rectangular section, to recover sensible heat/cold from return air; c) a passive evaporative cooling element. This paper present the design concept of the wall system as well as a first series of results from laboratory testing regarding the latent heat absorption component

15 What is the future of (remote) work?

Among the individuals who worked continuously since the start of the COVID-19 pandemic, around 22% of men and 30% of women were working remotely in both waves of the SHARE Corona survey. Only 10% of the workers in our sample were initially working remotely, and then moved back to their usual workplace. Remote work adoption varied depending on the technical feasibility of performing a job remotel