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She often said in the latter years of her life that she longed to leave this world and go to her heavenly home
just a few hours after a morning chat with her caregiver
God granted her that desire and she peacefully went home in her sleep
Where do you see the most exciting research/debates happening in your field
I’ve immersed myself in analyzing foreign policy within the broader scope of international studies
we now have various tools illuminating the intricate connections between foreign policy
An emerging body of literature brilliantly connects psychological foundations to foreign policy choices
This allows us to integrate individual factors and continually refine our analytical lens
there isn’t a singular overarching theory
which I believe offer the most clarity in understanding governments’ international actions
A pressing query that captivates me is: How do foreign policy preferences crystallize
and how do narratives and ideologies shape these preferences
we’re witnessing a fascinating shift: Foreign policy has increasingly intertwined with domestic politics
becoming a hotbed for influences like polarization
coupled with heightened geopolitical rivalries
the rapid pace of globalization and climate change
has muddied the waters for many governments
Striking a sustainable balance or consensus amidst these dynamics proves challenging
the study of foreign policy in our contemporary era is a tapestry woven from local and global threads
and more intricate landscape than ever before
How has the way you understand the world changed over time
and what (or who) prompted the most significant shifts in your thinking
emphasizing a problem-centric rather than a theory-centric approach
I can delve into how a coalition’s ideology shapes a government’s foreign policy and then seamlessly transition to analyzing a strategic scenario driven by tangible interests
“I’m a realist” or “I’m a constructivist,” I recognize the strong pull of confirmation bias that often accompanies such statements
One of your most recent publications analyses how China influences the liberal international order from the inside
How do your research findings enhance our understanding of China’s aspirations and actions in shaping the international order?
In our collaborative work with my colleagues Pauselli and Urdinez
we’ve observed that China’s presence in the UN Human Rights Council influences the voting patterns of other member countries
nations sharing Council membership with China tend to align their votes more closely with China’s preferences
This isn’t just about China’s sway over its allies; it signals a deliberate strategy to recalibrate the perception of human rights
China’s approach isn’t to dismantle the Human Rights Council but to promote a more Westphalian perspective
China primarily sees Human rights as domestic concerns
with international intervention warranted only in situations that jeopardize global stability
Our research underscores China’s inclination to redefine the “liberal” signifier in the international liberal order
the West has equated modernity with liberal democracy
advocating for a vision of modernity that doesn’t necessarily hinge on conventional democratic ideals
This dynamic is intriguing because China’s competition extends beyond strategic
or technological arenas into the realm of ideas
this ideological competition seems more pronounced in the Global South compared to the Global North
What are the most important implications of US-China competition for Latin America’s autonomy
Latin America is navigating a tumultuous phase marked by persistent economic inequality
and states often influenced by elite interests rather than genuine autonomy
China emerges as an appealing ally for leaders seeking alternatives to liberal democracy
They view China as a partner that respects non-interference in domestic matters
China’s governance ethos emphasizes the primacy of the state
While some regional leaders may not advocate for single-party dominance
they exhibit comfort with centralized power and suppressing dissent
China’s appeal resonates with sectors like agribusiness
China’s appeal has transcended ideological boundaries
Even though numerous Latin American governments may not share China’s communist ideology
they discover common ground in principles of non-intervention
This presents a nuanced challenge: striking a balance between economic interests with China while considering domestic institutions that align more closely with Western norms
What are some of the novel approaches and debates in the areas of IR and FPA in Latin America?
I find that four key approaches prove instrumental in deciphering the intricate web of international dynamics at play
delving into the study of domestic coalitions in power and their influence on foreign policy preferences offers valuable insights
While Andrew Moravcsik’s work on preferences is foundational
Etel Solingen’s extensive exploration of coalitions in Latin America
particularly the distinction between inward-looking and outward-looking coalitions
Given Latin America’s notable levels of inequality resulting in social and political polarization
comprehending the formation of these coalitions and how their preferences shape foreign policy emerges as a critical factor in understanding the region
The second perspective I find intriguing centers around the notion that the material dynamics of power
are not as pronounced in Latin America as in other regions of the global south
framing the region in terms of a balance of political identities
as opposed to the traditional concept of power comprising military and economic strength
the threat posed by a neighboring country stem not from its raw power but from its success and potential electoral ramifications within my own country
A crucial predictor for predicting the relations between two countries in the region lies in the ideological distance between them
Governments gauge the nature of the coalition in power in a neighboring country to infer its proximity to the United States
and its alignment with the demands of the global south
revolves around the idea that Latin America
Drawing inspiration from the English School of International Relations
this perspective identifies a strong Westphalian pluralistic ethos in the region
particularly concerning issues like democracy and human rights
the core remains anchored in traditional institutions of international society
A particularly fascinating aspect is the concept of “concertation” as a unique institution in Latin America
This entails leaders agreeing on minimal yet fundamental issues to foster continued coexistence and establish the groundwork for basic cooperation
not necessarily for providing public goods but rather to prevent “public bads” such as interstate wars
the fourth dimension that I think demands consideration when contemplating the region is the understanding that Latin America exists within a world characterized by uneven and combined development
with the enduring tension between the North and South as a fundamental element
Unlike other regions of the global south where geopolitics and international security dominate the regional discourse
our focus in Latin America gravitates more towards discussions on globalization and international well-being
that aligning such a perspective on a global scale proves challenging
given the current priority of global powers
which predominantly revolves around geopolitical competition—an area not necessarily at the forefront of the region’s concerns
What elements of foreign policy change and continuity do you expect to happen in Argentina during Javier Milei’s presidential term?
Certain trends are becoming evident in evaluating Milei’s foreign policy at this early juncture
there is a stated interest in seeking OECD membership
a commitment to negotiating a free trade agreement with the European Union
and a proactive stance in disengaging from UNASUR
accompanied by a distant approach in dealings with China
There is a distinct inclination toward a pro-North orientation
coupled with a notable indifference towards regional affiliations
The historical relationship with Brazil has experienced deterioration over time
and the likelihood of Milei prioritizing efforts to repair this connection seems low for me so far
it seems to me that Milei’s global outlook will embody dogmatism when conditions allows and pragmatism when circumstances require
This inclination towards dogmatism would manifest itself as a heightened receptivity to influences from the Global North and a wariness towards those from the Global South
his government may enhance global trade and align with international regulations across various domains by fostering a more open economy
More than an original libertarian approach
Milei’s foreign policy up to this point seems to be a classic liberal-right approach
albeit with more assertiveness than in previous governments
In a 2022 article
you discuss the increasing divergence in the Argentine-Brazilian relationship over recent decades
Could you elaborate on the factors contributing to this trend and how the bilateral divergence affects South America’s regional integration
we delve into three key factors explaining the deterioration of this bilateral relationship:
I am skeptical about any immediate changes
Milei and Lula seem to be concentrating on divergent agendas
and while there is potential for them to complement each other
I haven’t observed any indications from either side in that direction
It is a matter of foreign policy orientations and domestic policy considerations
Neither party seems willing to approach the other at the risk of losing support from their respective domestic bases
What is the most important advice you could give young International Relations scholars
My most fundamental advice would be to avoid theoretical pigeonholing
But there is more to it: Engage in the study of data analytics and acquire programming skills as they are indispensable tools for any research project
Ensure you incorporate the time variable in your analysis and explore the political economy of the foreign agenda
consider that every action a president takes in foreign policy serves purely domestic purposes
and you’ll uncover intriguing insights
To identify international sources of political risk
approach your analysis as if you were sitting on the board of a multinational company
always return to the classics for foundational insights
Copyright © — E-International Relations
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Treatment for congenital adrenal hyperplasia (CAH) was introduced in the 1950s following the discovery of the structure and function of adrenocortical hormones
Although major advances in molecular biology have delineated steroidogenic mechanisms and the genetics of CAH
management and treatment of this condition continue to present challenges
Management is complicated by a combination of comorbidities that arise from disease-related hormonal derangements and treatment-related adverse effects
The clinical outcomes of CAH can include life-threatening adrenal crises
Standard-of-care glucocorticoid formulations fall short of replicating the circadian rhythm of cortisol and controlling efficient adrenocorticotrophic hormone-driven adrenal androgen production
Adrenal-derived 11-oxygenated androgens have emerged as potential new biomarkers for CAH
as traditional biomarkers are subject to variability and are not adrenal-specific
Multiple alternative treatment approaches are being developed with the aim of tailoring therapy for improved patient outcomes
This Review focuses on challenges and advances in the management and treatment of CAH due to 21-hydroxylase deficiency
including treatments designed to replace cortisol in a physiological manner and adjunct agents intended to control excess androgens and thereby enable reductions in glucocorticoid doses
Challenges in the management of congenital adrenal hyperplasia (CAH) arise from multiple hormonal imbalances
the intrinsic tendency of the CAH-affected adrenal gland to overproduce androgens and limited treatment options
which often necessitate glucocorticoid excess
The relationship between glucocorticoid and mineralocorticoid actions should be considered in the management of replacement therapies in CAH
Patients are at risk of life-threatening adrenal crises with hypoglycaemia
most often triggered by infectious illnesses and exacerbated by adrenaline deficiency
Traditional biomarkers vary with glucocorticoid dose or time of day and are not adrenal-specific
reflecting the need for new biomarkers; for example
the biologically active 11-oxygenated androgens
Circadian glucocorticoid replacement and adjunct non-glucocorticoid therapies promise to enable glucocorticoid dose reduction; furthermore
the development of personalized gene and cellular therapies is under way
Prices may be subject to local taxes which are calculated during checkout
Congenital adrenal hyperplasia-current insights in pathophysiology
Neonatal screening for congenital adrenal hyperplasia
A missense mutation at Ile172–Asn or Arg356–Trp causes steroid 21-hydroxylase deficiency
Expression of human 21-hydroxylase (P450c21) in bacterial and mammalian cells: a system to characterize normal and mutant enzymes
Determination of functional effects of mutations in the steroid 21-hydroxylase gene (CYP21) using recombinant vaccinia virus
Revisiting the prevalence of nonclassic congenital adrenal hyperplasia in US Ashkenazi Jews and Caucasians
Prevalence of frequent recessive diseases in the Spanish population through DNA analyses on samples from the neonatal screening
High carrier frequency of 21-hydroxylase deficiency in Cyprus
Carrier frequency of congenital adrenal hyperplasia (21-hydroxylase deficiency) in a middle European population
150 Years of congenital adrenal hyperplasia: translation and commentary of De Crecchio’s classic paper from 1865
The suppression of androgen secretion by cortisone in a case of congenital adrenal hyperplasia
The hypothalamic-pituitary-adrenal axis: a brief history
The use of adrenalectomy as a treatment for congenital adrenal hyperplasia
and reduced hydrocortisone dose maintain normal growth velocity and bone maturation despite elevated androgen levels in children with congenital adrenal hyperplasia
Androgen excess is due to elevated 11-oxygenated androgens in treated children with congenital adrenal hyperplasia
Adrenomedullary dysplasia and hypofunction in patients with classic 21-hydroxylase deficiency
Adrenomedullary function may predict phenotype and genotype in classic 21-hydroxylase deficiency
Interaction between glucagon and human corticotropin-releasing hormone or vasopressin on ACTH and cortisol secretion in humans
Congenital adrenal hyperplasia due to 21-hydroxylase deficiency
Adrenal-derived 11-oxygenated 19-carbon steroids are the dominant androgens in classic 21-hydroxylase deficiency
Morphologic and molecular characterization of adrenals and adrenal rest affected by congenital adrenal hyperplasia
Health status of adults with congenital adrenal hyperplasia: a cohort study of 203 patients
Clinical characteristics of a cohort of 244 patients with congenital adrenal hyperplasia
Congenital adrenal hyperplasia due to steroid 21-hydroxylase deficiency: an endocrine society clinical practice guideline
Pharmacokinetic/pharmacodynamic evaluation of hydrocortisone therapy in pediatric patients with congenital adrenal hyperplasia
International practice of corticosteroid replacement therapy in congenital adrenal hyperplasia: data from the I-CAH registry
Glucocorticoid replacement regimens for treating congenital adrenal hyperplasia
Reduced final height outcome in congenital adrenal hyperplasia under prednisone treatment: deceleration of growth velocity during puberty
Hydrocortisone suspension and hydrocortisone tablets are not bioequivalent in the treatment of children with congenital adrenal hyperplasia
Iatrogenic cushing syndrome in a child with congenital adrenal hyperplasia: erroneous compounding of hydrocortisone
Quality of compounded hydrocortisone capsules used in the treatment of children
Absorption and tolerability of taste-masked hydrocortisone granules in neonates
infants and children under 6 years of age with adrenal insufficiency
A prospective study of children aged 0–8 years with CAH and adrenal insufficiency treated with hydrocortisone granules
Salt wasting in simple virilizing congenital adrenal hyperplasia
Aldosterone-to-renin ratio as a marker for disease severity in 21-hydroxylase deficiency congenital adrenal hyperplasia
Clinical review: adult height in patients with congenital adrenal hyperplasia: a systematic review and metaanalysis
in The Diagnosis and Treatment of Endocrine Disorders in Childhood and Adolescence Ch
Plasma renin measurements are unrelated to mineralocorticoid replacement dose in patients with primary adrenal insufficiency
Sodium chloride supplementation is not routinely performed in the majority of german and austrian infants with classic salt-wasting congenital adrenal hyperplasia and has no effect on linear growth and hydrocortisone or fludrocortisone dose
fludrocortisone dose and plasma renin activity in children with classic congenital adrenal hyperplasia due to 21-hydroxylase deficiency followed from birth to 4 years of age
Blood pressure in a large cohort of children and adolescents with classic adrenal hyperplasia (CAH) due to 21-hydroxylase deficiency
Cardiovascular disease risk factors and metabolic morbidity in a longitudinal study of congenital adrenal hyperplasia
Highly potent adrenal cortical steroids: structure and biologic activity
A practical guide to the monitoring and management of the complications of systemic corticosteroid therapy
progesterone and sex steroids on mineralocorticoid receptor transactivation in congenital adrenal hyperplasia
Hormonal circadian rhythms in patients with congenital adrenal hyperplasia: identifying optimal monitoring times and novel disease biomarkers
Steroid metabolome analysis in disorders of adrenal steroid biosynthesis and metabolism
11-Oxygenated androgens in health and disease
11-Oxygenated androgens are biomarkers of adrenal volume and testicular adrenal rest tumors in 21-hydroxylase deficiency
11-Oxygenated androgens useful in the setting of discrepant conventional biomarkers in 21-hydroxylase deficiency
11-ketotestosterone and 11-ketodihydrotestosterone in castration resistant prostate cancer: potent androgens which can no longer be ignored
11-Ketotestosterone is the dominant circulating bioactive androgen during normal and premature adrenarche
Glucocorticoid activity of adrenal steroid precursors in untreated patients with congenital adrenal hyperplasia
Adrenal steroid metabolites accumulating in congenital adrenal hyperplasia lead to transactivation of the glucocorticoid receptor
Real-world estimates of adrenal insufficiency-related adverse events in children with congenital adrenal hyperplasia
and illness sequelae in patients with congenital adrenal hyperplasia
Increased mortality in patients with congenital adrenal hyperplasia due to 21-hydroxylase deficiency
depression and healthcare costs in patients with congenital adrenal hyperplasia
Adrenal crises in children: perspectives and research directions
Frequency and causes of adrenal crises over lifetime in patients with 21-hydroxylase deficiency
High incidence of adrenal crisis in educated patients with chronic adrenal insufficiency: a prospective study
Epidemiology of adrenal crisis in chronic adrenal insufficiency: the need for new prevention strategies
Incidence of adrenal crisis in patients with adrenal insufficiency
Children with classic congenital adrenal hyperplasia experience salt loss and hypoglycemia: evaluation of adrenal crises during the first 6 years of life
Low adrenomedullary function predicts acute illness in infants with classical congenital adrenal hyperplasia
and learning difficulties in salt wasting 21-hydroxylase deficiency
Manifestations and characteristics of congenital adrenal hyperplasia-associated encephalopathy
Increased infection risk in addison’s disease and congenital adrenal hyperplasia
Arlt, W., Baldeweg, S. E., Pearce, S. H. S. & Simpson, H. L. Endocrinology in the time of COVID-19: management of adrenal insufficiency. Eur. J. Endocrinol. https://doi.org/10.1530/eje-20-0361 (2020)
Therapeutic management of adrenal insufficiency
A glucocorticoid education group meeting: an effective strategy for improving self-management to prevent adrenal crisis
Congenital adrenal hyperplasia due to 21-hydroxylase deficiency: alterations in cortisol pharmacokinetics at puberty
Management of adolescents with congenital adrenal hyperplasia
Growth inhibition by glucocorticoid treatment in salt wasting 21-hydroxylase deficiency: in early infancy and (pre)puberty
Growth hormone therapy alone or in combination with gonadotropin-releasing hormone analog therapy to improve the height deficit in children with congenital adrenal hyperplasia
Final adult height in children with congenital adrenal hyperplasia treated with growth hormone
Cardiovascular risk factors in children and adolescents with congenital adrenal hyperplasia due to 21-hydroxylase deficiency
Cardiovascular and metabolic outcomes in congenital adrenal hyperplasia: a systematic review and meta-analysis
Increased cardiovascular and metabolic morbidity in patients with 21-hydroxylase deficiency: a Swedish population-based national cohort study
Reproductive and perinatal outcomes in women with congenital adrenal hyperplasia: a population-based cohort study
Alterations in hydrocortisone pharmacokinetics in a patient with congenital adrenal hyperplasia following bariatric surgery
timing and monitoring hydrocortisone replacement therapy in patients with adrenal insufficiency
A switch in hepatic cortisol metabolism across the spectrum of non alcoholic fatty liver disease
Cortisol clearance and associations with insulin sensitivity
body fat and fatty liver in middle-aged men
Bone mineral density in adults with congenital adrenal hyperplasia: a systematic review and meta-analysis
Increased prevalence of fractures in congenital adrenal hyperplasia: a Swedish population-based national cohort study
in Management of Adrenal Masses in Children and Adults 207–224 (Springer
Prevalence and characteristics of adrenal tumors and myelolipomas in congenital adrenal hyperplasia: a systematic review and meta-analysis
Adrenal morphology and associated comorbidities in congenital adrenal hyperplasia
Total adrenal volume but not testicular adrenal rest tumor volume is associated with hormonal control in patients with 21-hydroxylase deficiency
and testicular function in 219 adult men born with classic 21-hydroxylase deficiency
Prevalence of testicular adrenal rest tumor and factors associated with its development in congenital adrenal hyperplasia
Testicular adrenal rest tumors: current insights on prevalence
Molecular characterization of testicular adrenal rest tumors in congenital adrenal hyperplasia: lesions with both adrenocortical and Leydig cell features
Two cases of reversible male infertility due to congenital adrenal hyperplasia combined with testicular adrenal rest tumor
Prevalence of ovarian adrenal rest tumours and polycystic ovaries in females with congenital adrenal hyperplasia: results of ultrasonography and MR imaging
Management of endocrine disease: gonadal dysfunction in congenital adrenal hyperplasia
Reduced frequency of biological and increased frequency of adopted children in males with 21-hydroxylase deficiency: a Swedish population-based national cohort study
Improving glucocorticoid replacement therapy using a novel modified-release hydrocortisone tablet: a pharmacokinetic study
Improved cortisol exposure-time profile and outcome in patients with adrenal insufficiency: a prospective randomized trial of a novel hydrocortisone dual-release formulation
Modified-release hydrocortisone for circadian therapy: a proof-of-principle study in dexamethasone-suppressed normal volunteers
hydrocortisone replacement therapy that provides physiological cortisol exposure
A pharmacokinetic and pharmacodynamic study of delayed- and extended-release hydrocortisone (Chronocort) vs
conventional hydrocortisone (Cortef) in the treatment of congenital adrenal hyperplasia
a modified-release formulation of hydrocortisone
in the treatment of adults with classic congenital adrenal hyperplasia
Modified-release and conventional glucocorticoids and diurnal androgen excretion in congenital adrenal hyperplasia
Modified-release hydrocortisone in congenital adrenal hyperplasia
Modified-release hydrocortisone to provide circadian cortisol profiles
A phase 2 study of continuous subcutaneous hydrocortisone infusion in adults with congenital adrenal hyperplasia
Long-term use of continuous subcutaneous hydrocortisone infusion therapy in patients with congenital adrenal hyperplasia
24-Hour profiles of 11-oxygenated C(19) steroids and Δ(5)-steroid sulfates during oral and continuous subcutaneous glucocorticoids in 21-hydroxylase deficiency
and physiology of human steroidogenesis and its disorders
ABCC1 confers tissue-specific sensitivity to cortisol versus corticosterone: a rationale for safer glucocorticoid replacement therapy
Kyle, C. et al. Comparison of acute effects of corticosterone versus cortisol (hydrocortisone) infusion in adults with congenital adrenal hyperplasia. Endocr. Abstr. https://doi.org/10.1530/endoabs.59.OC2.2 (2018)
US National Library of Medicine. ClinicalTrials.gov https://clinicaltrials.gov/ct2/show/NCT00001521 (1995)
Abiraterone and increased survival in metastatic prostate cancer
Abiraterone acetate to lower androgens in women with classic 21-hydroxylase deficiency
Abiraterone acetate treatment lowers 11-oxygenated androgens
US National Library of Medicine. ClinicalTrials.gov https://clinicaltrials.gov/ct2/show/NCT02574910 (2021)
multicenter study of nevanimibe for the treatment of congenital adrenal hyperplasia
a selective and potent inhibitor of acyl-CoA acyltransferase 1
induces apoptosis in H295R adrenocortical cells and in the adrenal cortex of dogs
Single-dose study of a corticotropin-releasing factor receptor-1 antagonist in women with 21-hydroxylase deficiency
Crinecerfont lowers elevated hormone markers in adults with 21-hydroxylase deficiency congenital adrenal hyperplasia
US National Library of Medicine. ClinicalTrials.gov https://clinicaltrials.gov/ct2/show/NCT04490915 (2021)
US National Library of Medicine. ClinicalTrials.gov https://clinicaltrials.gov/ct2/show/NCT04806451 (2021)
Tildacerfont in adults with classic congenital adrenal hyperplasia: results from two phase 2 studies
US National Library of Medicine. ClinicalTrials.gov https://clinicaltrials.gov/ct2/show/NCT04457336 (2021)
US National Library of Medicine. ClinicalTrials.gov https://clinicaltrials.gov/ct2/show/NCT04544410 (2021)
Spruce Biosciences. Pediatric Classic CAH, Pipeline https://sprucebiosciences.com/pipeline/indications/pediatric-classic-cah/ (2021)
Feldhaus, A. L. et al. ALD1613, a novel long-acting monoclonal antibody to control ACTH-driven pharmacology. Endocrinology https://doi.org/10.1210/en.2016-1455 (2016)
A long-acting neutralizing monoclonal ACTH antibody blocks corticosterone and adrenal gene responses in neonatal rats
Kusnetzow, A. et al. SAT-364 nonpeptide orally-bioavailable ACTH antagonists: suppression of ACTH-induced corticosterone secretion and adrenal hypertrophy in rats. J. Endocr. Soc. https://doi.org/10.1210/js.2019-SAT-364 (2019)
Kusnetzow, A. K. et al. MON-176 Discovery and Identification of Late Stage Selective Nonpeptide ACTH Antagonists for the Treatment of Cushing’s Disease, Ectopic ACTH secreting tumors, and congenital adrenal hyperplasia. J. Endocr. Soc. https://doi.org/10.1210/jendso/bvaa046.690 (2020)
Markison, S. et al. OR19-03 effects of nonpeptide orally bioavailable ACTH antagonists on adrenal gland size and function in rats. J. Endocr. Soc. https://doi.org/10.1210/jendso/bvaa046.699 (2020)
Effect of a melanocortin type 2 receptor (MC2R) antagonist on the corticosterone response to hypoxia and ACTH stimulation in the neonatal rat
Melanocortin 2 receptor antagonists in canine pituitary-dependent hypercortisolism: in vitro studies
Positive fertility outcomes in a female with classic congenital adrenal hyperplasia following bilateral adrenalectomy
The role of bilateral adrenalectomy in the treatment of congenital adrenal hyperplasia
Outcome of bilateral adrenalectomy in congenital adrenal hyperplasia: one unit’s experience
Bilateral adrenalectomy in congenital adrenal hyperplasia: a systematic review and meta-analysis
Use of PET/CT with cosyntropin stimulation to identify and localize adrenal rest tissue following adrenalectomy in a woman with congenital adrenal hyperplasia
11C-Metomidate PET/CT detected multiple ectopic adrenal rest tumors in a woman with congenital adrenal hyperplasia
Ovarian adrenal rest tumor in a congenital adrenal hyperplasia patient with adrenocorticotropin hypersecretion following adrenalectomy
American Association of Clinical Endocrinology disease state clinical review on the evaluation and management of adrenocortical carcinoma in an adult: a practical approach
Medical therapy for Cushing’s syndrome in the twenty-first century
Mitotane for 21-hydroxylase deficiency in an infertile man
Effects of mitotane on testicular adrenal rest tumors in congenital adrenal hyperplasia due to 21-hydroxylase deficiency: a retrospective series of five patients
U.S. Food and Drug Administration LYSODREN® (mitotane) Tablets https://www.accessdata.fda.gov/drugsatfda_docs/label/2009/016885s023lbl.pdf (2017)
Modeling congenital adrenal hyperplasia and testing interventions for adrenal insufficiency using donor-specific reprogrammed cells
Bornstein, S. R. et al. New horizons: novel adrenal regenerative therapies. J. Clin. Endocrinol. Metabol. https://doi.org/10.1210/clinem/dgaa438 (2020)
Transplantation of bovine adrenocortical cells encapsulated in alginate
Restoration of adrenal steroidogenesis by adenovirus-mediated transfer of human cytochromeP450 21-hydroxylase into the adrenal gland of21-hydroxylase-deficient mice
An AAVrh10-CAG-CYP21-HA vector allows persistent correction of 21-hydroxylase deficiency in a Cyp21(-/-) mouse model
Biology of the adrenal gland cortex obviates effective use of adeno-associated virus vectors to treat hereditary adrenal disorders
Extra-adrenal induction of Cyp21a1 ameliorates systemic steroid metabolism in a mouse model of congenital adrenal hyperplasia
Eclov, R. J. et al. OR25-01 Durable CYP21A2 gene therapy in non-human primates for treatment of congenital adrenal hyperplasia. J. Endocr. Soc. https://doi.org/10.1210/jendso/bvaa046.899 (2020)
dose-escalation study of the safety and efficacy of gene therapy in adults with classic congenital adrenal hyperplasia (CAH) due to 21-hydroxylase deficiency through administration of an adeno-associated virus (AAV) serotype 5-based recombinant vector encoding the human CYP21A2 Gene
US National Library of Medicine. ClinicalTrials.gov https://clinicaltrials.gov/ct2/show/NCT04783181 (2020)
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National Institutes of Health Clinical Center
Eunice Kennedy Shriver National Institute of Child Health and Human Development
The authors contributed equally to all aspects of the article
received research funds from Diurnal Limited through the National Institutes of Health Cooperative Research and Development Agreement
took up employment at AstraZeneca and is currently employed there
Nature Reviews Endocrinology thanks Antonio Balsamo
who co-reviewed the manuscript with Rita Ortolano
reviewer(s) for their contribution to the peer review of this work
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Gerald grew up on his parent’s farm in River Hills
He attended Winnipeg Falls South School in River Hills until Grade 9
Gerald was the eldest of 9 children: 4 Brothers
He was predeceased by his parents Otto & Hildegarde Merke
brother Victor and recently his brother-in-law Frank Behnke
with Dominion Catering first as an assistant manager and later as manager
he worked for the LGD of Pinawa and would run the projector for the LDB movie theater on weekends
Gerald started a new business venture as a Texaco Bulk Agent first in River Hills
Gerald and Elaine have done some travelling over the years
Their Honeymoon was spent travelling through the states to B.C
they travelled with his maternal grandmother to Germany to visit her sister
Gerald has been to NASCAR races in San Diego and has seen the Hoover Dam
49 of them in business together and raising a family
Love of the land never took him far from farming
he always came home with a smile on his face
He will be sadly missed by everyone in the whole family
the many friends he made over the years and especially by his tail wagging sidekick
Masks are optional when attending the service
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This week Moody’s changed ExxonMobil’s Triple-A rating from stable to negative
raising the prospect of the oil company being downgraded in 2020
Moody’s has red-flagged ExxonMobil’s rising capital expenditures and weak revenues
The oil giant is challenged by a low price environment ($60/barrel) and diminished refining and chemical earnings
The problems for ExxonMobil’s business model are fundamental — revenues have eroded and rebounding oil prices are proving inadequate to fund operating expenses
Moody’s negative outlook is based on its assessment of recent actions by the company in light of current market conditions:
“The company’s high level of capital investments cannot be funded with operating cash flow and asset sales at projected levels.”
Despite ExxonMobil’s efforts to reduce costs
Moody’s concludes: “The company’s high level of growth capital investments cannot be funded with operating cash flow and asset sales at projected levels given ExxonMobil’s dividend payout
absent meaningfully higher commodity prices and earnings from downstream and chemicals.”
These credit concerns are accompanied by a broader warning for ExxonMobil and other companies
“The negative outlook also reflects the emerging threat to oil and gas companies’ profitability and cash flow from growing efforts by many nations to mitigate the impacts of climate change through tax and regulatory policies that are intended to shift global demand towards other sources of energy and conservation.”
The company now sees the investment as a long-term proposition at a time when heavy capital investments and low earnings have undermined the company’s basic value proposition to investors
Moody’s suggests that the company could experience a turnaround if it successfully executes its capital program
increases production growth and adds to its chemical and refining capacity
IEEFA update: Fracking sector spills more red ink in Q3
IEEFA update: ExxonMobil abandons goal of “quick cash” from Permian fracking
IEEFA update: ExxonMobil’s slide from the Top Ten of the S&P 500 ‒ historic turning point for the company
Former IEEFA Financial Analyst Kathy Hipple is a founding partner of Noosphere Marketing and the finance professor at Bard’s MBA for Sustainability
Over 5% of recent Hubble Space Telescope images examined in the study contained Starlink satellite trails
The effect of satellites on our view of the universe is getting worse
an examination of images taken by the Hubble Space Telescope has revealed.
The findings may confirm the fears of astronomers who argue that satellite constellations such as SpaceX's fleet of over 3,500 Starlink spacecraft stand to severely impact astronomy.
These fears were initially confined to astronomers working with ground-based observatories
but as humanity's exploitation of the space around our planet has burgeoned and plans for so-called "mega-constellations" of satellites have progressed
those concerns have spread to colleagues working with space-based instruments.
To reach their findings, a team of researchers including over 11,000 citizen scientists from the Hubble Asteroid Hunter project scoured through images produced by the Hubble Space Telescope between 2002 and 2021
The team found that 2.7% of Hubble's images with a typical exposure time of 11 minutes were crossed by satellites
the probability of finding satellite trails in Hubble images has increased with time from 3.7% in 2002 to 5.9% in 2021.
Related: Megaconstellations like SpaceX's Starlink may interfere with search for life by world's largest radio telescope
The volunteer citizen scientist collaborators looked at Hubble images and highlighted those marred by bright satellite streaks
Two machine-learning algorithms were then used to examine the volunteer classifications
The examination revealed trails were consistent between images taken with Hubble over the two exposure times
— Megaconstellations becoming the new light pollution
— Light pollution damaging views of space for majority of large observatories, survey finds
— International Astronomical Union launches new center to fight satellite megaconstellation threat
coming before swarms of artificial satellites reach orbit
will hopefully help establish a baseline for future studies that will assist astronomers to better assess the impact of megaconstellations on astronomy
Follow us on Twitter @Spacedotcom or on Facebook
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Robert LeaSocial Links NavigationSenior WriterRobert Lea is a science journalist in the U.K
whose articles have been published in Physics World
He also writes about science communication for Elsevier and the European Journal of Physics
Rob holds a bachelor of science degree in physics and astronomy from the U.K.’s Open University
James Webb Space Telescope captures thousands of galaxies in a cosmic 'feast' (image)
Astronomers gaze into 'dark nebula' 60 times the size of the solar system (video)
What would it be like living on Tatooine from 'Star Wars'
This exoplanet orbiting twin suns could tell us
Richard Milne
selects her favourite stories in this weekly newsletter
Norway’s $1.4tn oil fund tests the logic and limits of some of the hype around responsible investing
Can an investor funded entirely from oil and gas revenues take a credible stance against climate change? Can a fund ultimately controlled by Norway’s parliament and government make decisions on what to invest in — and, more crucially, what not to — and not be thought of as acting politically
These questions and more have come to enliven a tepid national election campaign that looks set to see a return of the centre-left to power after eight unprecedented years of centre-right rule in Oslo. The oil fund, now run by a high-profile former hedge fund manager
is the world’s largest sovereign wealth fund
owning on average 1.4 per cent of every listed company in the world
Norwegian politicians have intoned ad nauseam that the fund is purely a financial investor
and is not a tool for foreign policy (or anything else) for whatever party holds power in Oslo
Various policies were formulated by the fund’s manager — Norges Bank Investment Management, an arm of the central bank — on everything from executive pay and board diversity to sustainability reporting and different share classes, all based on international standards rather than a particular Norwegian view
But alongside that are a number of product bans taken by the Norwegian parliament as a sign of what the people — the ultimate owners — are unwilling to hold
The original exclusions of tobacco and nuclear weapon producers have been added to over the years by parliament to include companies too dependent on coal and pure oil exploration and production groups
It was against this backdrop that Jonas Gahr Store
leader of the Labour party and favourite to become prime minister after the elections on September 13
said in late 2019 that “we have to get used to saying that the oil fund is a political tool”
with both the central bank governor and the current centre-right government intoning that the fund must never be used for political purposes
Many on the left though welcomed the stance as they are keen to use the fund to push green change
But for many his comments merely reflected the reality of the fund and its growing heft
a professor at NHH Norwegian School of Economics and a recent member of a government-appointed committee that looked at climate risk for the oil fund
says about the fund’s tens of thousands of votes at annual meetings: “There’s no such thing as non-political
The expert panel she was part of hinted that the oil fund
once seen as world-leading in ethical or responsible investing issues
Its mandate should be changed to include climate risk and a long-term goal that companies in its investment portfolio should have net zero emissions
Companies that fail to set a timetable for net zero could be barred
it has never been explicit in the mandate: what does it mean to be a responsible investor?” asks Thorburn
She argues this is less about any financial impact such a push would have and more about securing popular support for the fund
You really need political legitimacy for the fund to operate in Norway,” she says
reaction to the report was split on party political lines
Gahr Store promised to implement the recommendations if he came to power
while the populist rightwing Progress party argued that it would be “unwise to politicise the oil fund”
All this is probably a taster of what is to come for one of the rare sovereign wealth funds in a democracy as Norway grapples with its own questions around climate change and having the biggest petroleum industry in western Europe
The fund is now about four times larger than Norway’s annual GDP
its annual payments represent a quarter of the state budget
and it is worth more than the oil and gas left in the ground for Norway
Questions about the political use of the fund are only starting
Twitter: @rmilneNordic
www.soberingfuneralchapel.com
Offer Condolence for the family of Hildegarde Adelaide Merke
2022 at the age of 59 at his home in Beausejour
He was born the 4th child of Charlie and Iris (Johnson) Merke
He married Geraldine (Gerry) Mosquin-Smorang on July 6
Paul Lutheran Church in the small town of River Hills where he lived with his parents and siblings
He attended Whitemouth School until Grade 10 when he transferred to Lord Selkirk in Selkirk
he came home from school and parked his truck in its usual space by his Dad’s garage
forgot that Steven’s truck was in its spot and backed right into it
Steven was hired by a contractor who was building and erecting Hydro lines in Manitoba
Some of these lines were in remote areas and the men stayed in camps
He was supplied with a company truck to move men and equipment around where they were working
One day when he was working in the Hamoita area
Steven was travelling down one of the many construction roads when his brakes failed and into a huge rock went Steven’s truck
he worked as a bartender of Steve’s Sports Bar & Restaurant
He also worked in other hotels as a bartender
when working at Pappy’s Restaurant in Beausejour
They dated a bit and later they were married
As per Steven’s wishes cremation has taken place and no formal service will be held
www.soberingfuneralchapel.com
Offer Condolence for the family of Steven Merke
It is with deep sorrow that we announce the passing of our mother
great-grandmother and great great-grandmother
2010 at the Beausejour Hospital at the age of 93 years
Mom is survived by her children: Marge Aitkenhead (friend Elmer Voelpel)
and Iris (Archie) Aitkenhead; grandchildren: Rocky (Christel)
Kim (Scott) Zandbergen; great-grandchildren: Jovan
Emily and Katie; great great-grandchildren: Jayda
Pauline was predeceased by her husband Roy in 1992; parents August
Emma and Olga Schindel; brothers Adolf (Minnie) and William Schindel; son-in-law Norm Aitkenhead and daughter-in-law Diane Merke
She is survived by brothers Rudolf (Laura) and Fred (Anne) Schindel; sisters-in-law Margaret Schindel
Ella Klepatz and also many nieces and nephews
Friends may view at the church prior to the service
Interment will follow at the Zion Lutheran Church cemetery
donations may be made Pauline’s memory to the Zion Lutheran Memorial Fund
Condolences for the family may be offered at www.SoberingFuneralChapel.com
Offer Condolence for the family of Pauline (Schindel) Merke
It is with heavy hearts that we announce the passing of Victor Merke on January 5
He is survived by his mother Hildegarde Merke; companion Elisabete and her daughter Susie; grandchildren Ondreis and Lennox; his two children
and Kelly (Kallie); as well as many nieces
Victor was predeceased by his father Otto in 1975
Honorary Pallbearers will be Victor’s nieces and nephews
donations may be made in Victor’s memory to St
Offer Condolence for the family of Victor Larry Merke
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heat treated and ready for installation are produced in a matter of days,” said Warren M
“Customer demand for our additive manufacturing technology is growing to the point where we need significantly more space to assemble and test our MERKE IV RPD machines,” said Chris Bohlmann
The facility is under construction outside Oslo
Norway and scheduled for completion October 2016
For more information, visit the Norsk Titanium website
covering additive manufacturing and 3D printing
Ian holds bachelors and masters degrees in philosophy from McMaster University and spent six years pursuing a doctoral degree at York University before withdrawing in good standing
Advanced Manufacturing
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and insisted that she wants to continue the centre-right coalition she has led since 2009
German media have speculated that the country's first female chancellor
might retire before the end of a third term to pave the way for a successor from within her party
"I want to be chancellor for four years," Merkel said in an interview with public TV broadcaster ZDF five weeks before the vote
She even left open the possibility of standing again in 2017
also played down talk of reviving the grand coalition from her first term
when her conservative party ruled the country with the Social Democrats from 2005 to 2009
Surveys put her Christian Democrats far ahead of their leftist rivals in the runup to the 22 September ballot
have fared poorly in opinion polls and could complicate her re-election if they win less than the 5% share of the vote needed to enter parliament
Merkel could be forced to work with the Social Democrats
who are led by her first-term finance minister
"Nobody wants this," said Merkel
adding that in her view "the election will be very
and Governance Program is a leading source of independent policy research
It analyzes and seeks to improve international efforts to reduce democratic backsliding
and advance pro-democratic uses of new technologies
The Carnegie Rising Democracies Network is a research network of leading experts on democracy and foreign policy
dedicated to examining the growing role of non-Western democracies in international democracy support and conflict issues
The Rising Democracies Network is carried out in partnership with the Robert Bosch Stiftung
and with additional support from the Ford Foundation and the UK Foreign
Venezuela is being rocked by its worst political and economic crisis in more than a decade.1 A humanitarian crisis is taking shape in a country that has among the largest proven oil reserves in the world
With President Nicolás Maduro having neutralized the opposition-dominated National Assembly elected in December 2015 and decimated the judiciary’s independence
democratic solution to the crisis looks increasingly remote
Given that Venezuela seems unable to overcome its internal divisions alone
external actors will be vital in influencing how the crisis unfolds
Yet the crisis has erupted at a moment when Latin American governments’ interest and capacity to engage in Venezuela are limited
While the Maduro government has fewer regional allies than his predecessor Hugo Chávez could count on
governments in the region are doing little to defend democratic governance in Venezuela
Despite much pro-democracy rhetoric and some mediation efforts
they seem content to let Venezuela find its own way out of the crisis—whether this means an abrupt collapse of the authoritarian government or a prolongation of its increasingly heavy-handed rule
Latin American governments need to do more to help Venezuela overcome its impasse
The regional mechanisms established to preserve democratic governance in the Americas have dramatically underperformed in Venezuela
need to adopt a less equivocal position toward the crisis
Field research that we carried out in April in Caracas leads us to the conclusion that existing external efforts to build dialogue and mediation are unlikely to gain traction if they are not accompanied by clearer commitments to defend core democratic norms
President Maduro declared a state of emergency in Venezuela and argued that the country was under attack from imperialist forces led by the U.S
The political and economic crisis engulfing Venezuela is rapidly deteriorating and pushing the country into an abyss
In what for decades was one of Latin America’s most prosperous countries
and falling oil prices have produced an incipient humanitarian emergency
Venezuela’s GDP contracted 8.4 percent while inflation rose to an astonishing 400 percent a year
The budget deficit is running at 14 percent of the country’s GDP
The social consequences of this situation are severe
Poverty levels previously reduced by the Chavista revolution are now being wiped out by hyperinflation
Poverty levels rose from 27 percent of the population in 2013 to 73 percent in 2015.2 Industrial production has largely stopped
The government lacks hard currency to import even milk
A major drought has forced the government to ration energy
Falling budgets and energy shortages have forced the government to ask public employees to work only two days a week
particularly in major cities across the country
170 major looting incidents have been reported in San Cristóbal
Violent crime has grown to epidemic proportions
Venezuela now has the highest homicide rate in the world.3 The Economist ranks Caracas as the most dangerous city in the world.4
Venezuela’s long-simmering political standoff has become more acute
The Bolivarian revolution initiated by the late Hugo Chávez has profoundly polarized Venezuelans
transformed the country’s political system by diluting democratic checks and balances
Venezuela now retains few vestiges of a meaningful democracy
and the advantages of holding office have become overwhelming
Relations between the government and the opposition have become a highly confrontational
zero-sum game that lacks any spirit of accommodation or compromise
The ideological distance between the ruling government and the opposition now appears to be almost insurmountable
The situation has worsened as a result of the unpopular presidency of Nicolás Maduro
Polls indicate that 70 percent of Venezuelans want him out of office this year.5 Maduro lacks the charisma and political skills of Hugo Chávez
The president is surrounded by loyalists and sycophants and seems out of touch with the travails of ordinary Venezuelans
we were struck by how strongly Maduro is disliked and ridiculed not only by the opposition but also by many in the government
The political crisis has spiraled out of control since the December 2015 legislative elections
The ruling United Socialist Party of Venezuela (PSUV) lost its sixteen-year control of the National Assembly to the Democratic Unity Roundtable (MUD)
The opposition won 112 seats (two-thirds of the total) against the regime’s 56 seats
This supermajority granted the MUD significant powers
including the ability to block ministerial appointments
and—most significantly—initiate a recall referendum (revocatorio) against the president
The opposition’s triumph initiated a more conflictive phase in Venezuela’s political crisis
An emboldened opposition began to wield its new power in an effort to undermine the government
The Maduro administration brought in the courts to annul the opposition’s electoral gains
The government resorted to the Constitutional Court
to impugn the victory of six members of parliament in an effort to take away the opposition’s two-thirds supermajority
It also blocked a constitutional amendment introduced by the opposition to shorten Maduro’s term
The opposition initiated the process for a referendum to recall the president after it collected the necessary 1.85 million signatures in a petition
we heard widespread speculation that the government is planning to retaliate against state employees and citizens who depend on government programs if they sign the recall petition
Opposition leader Henrique Capriles told us that the recall referendum is the best tactic because it uses formal provisions in the chavistas’ own constitution
Maduro attacked the initiative as a plot by what he damned as “the oligarchy” to derail the revolution
He created a special council headed by Jorge Rodríguez
the mayor of a Caracas district and one of his most loyal supporters
many members of the opposition—in particular those who have been put in jail
institutional means are likely to be ineffective against the regime
There is thus a division in the opposition over what kind of strategy should be adopted
The crisis is a strongly ideological battle for power
The government says that the turmoil is necessary to bring about radical social change and overcome conservative opposition to change
It insists that the humanitarian crisis is the result of the opposition
By harking back to the highly elitist Second Republic
some opposition leaders can appear insensitive to Venezuela’s poor and marginalized sectors
The current impasse raises the stakes for both sides
An increasingly cornered government appears determined to cling to power and defend its revolutionary legacy—and to do so in ever more autocratic ways
The opposition has seized its rare electoral triumph to push forward a far-reaching reform agenda that entails removing Maduro and the PSUV from power entirely
Rather than the elections ushering in a search for compromise
both camps have adopted more rigid and radical positions
Our meetings in Caracas left us feeling that the prospects for negotiated agreements are increasingly slim
All of this puts the army in a potentially decisive role
The armed forces are ostensibly loyal to the revolution but have distanced themselves from Maduro and his disastrous record
This has engendered much speculation over what the army’s eventual stance might be on some form of negotiated transition
There are three potential scenarios for how the crisis may evolve
The first is that the opposition succeeds in ousting Maduro through a recall referendum by the end of 2016
The second scenario would see the president hang on to power at least until 2017
even as Venezuela’s economy continues its downturn spiral and social conflict and repression escalate
The third possibility is a military coup against Maduro
The chances of the first scenario occurring are slim
To succeed in the recall referendum the opposition would need to clear several
It would need to collect the signatures of 20 percent of the electorate (nearly 4 million people) to trigger the referendum
the opposition would then need to win a greater number of votes than the 7.5 million votes won by the government in 2013
the regime is already resorting to myriad means to prevent the referendum from taking place
Vice President Aristóbulo Istúriz has already stated that the opposition is too late to act and is guilty of procedural irregularities and even fraud.6 The National Electoral Council is supportive of the government and is aiming to delay the process until 2017
If the referendum is held (and won by the opposition) after December 2016
Maduro would have to leave office but there would not be new elections
The vice president would take over and the regime would survive—with Maduro effectively wielding influence from behind the scenes
The second scenario—Maduro remaining in power—is the most likely
The regime has control of all the main state bodies and institutional processes that it can use to derail any effort to remove the president
which ceased to be independent a long time ago and has openly pledged its support to defend the Bolivarian revolution and chavismo
Maduro recently filed a complaint against the National Assembly—and threatened to close the body down altogether—after the assembly backed moves to invoke the Organization of American States (OAS) Inter-American Democratic Charter against Venezuela (see below)
The government has also moved to imprison more opposition figures
and journalists and to break up social protests in an effort to undercut the opposition’s momentum
the economic and social situation is unlikely to improve
barring increases in the price of oil large enough to replenish state coffers
the economy will almost certainly further deteriorate
Living conditions for most of the population will descend to an even more critical position
A large-scale social explosion and mass outflow of Venezuelan citizens across Latin America cannot be discounted
This may bring the third scenario into play
institutional avenues for change virtually barred
the only scenario that would oust Maduro involves the military
There may be an internal coup or a rebellion in the military
For the moment this remains relatively improbable
but if the crisis worsens it cannot be completely ruled out
Maduro—like Chávez before him—has sought to tie the military strongly into the regime
handing key positions in government to leading military figures
The regime has also given the army responsibility for food distribution across the country—an opportunity to make virtually limitless gains on the black market
there will be a coup only if parts of the military—such as the middle- and lower-ranking members of the armed forces—believe the costs of repression and rising instability have begun to outweigh the benefits of supporting the Bolivarian regime
Venezuelan sources and diplomats we interviewed in Caracas spoke of growing divisions between high-ranking officers who have a stake in the survival of the regime and less senior officers who have not benefited directly from the existing order and seem worried about the country’s general loss of direction
the role that regional actors could play to address the crisis has become more critical
Other South American governments and regional intergovernmental organizations have not been able to temper Venezuela’s turmoil and have failed to read the nature or gravity of the crisis correctly
As these actors have increasingly committed themselves to defending democracy across Latin America
their failure to intervene decisively in Venezuela stands as a conspicuous failure for their democracy support policies
Brazil’s lack of clear backing for Venezuelan democracy has been often noted
the impeachment of Brazilian President Dilma Rousseff and the appointment of Michel Temer as interim president have now thrown Brazil into disarray and diverted the country from active foreign policy engagements
Brazil has for many years been a staunch supporter of the Venezuelan government
The transition of power in Brazil may change this stance and leave Venezuela more isolated
Brazil’s new government invited opposition leader Henrique Capriles to Brasília in a clear sign of its evolving stance on Venezuela
Brazil is unlikely to engage in any assertive support for Venezuela’s democratic reformers
is expected to run for president for a third time in 2018 and is unlikely to engage in topics that could produce negative headlines
Brazil can be expected to support a stronger regional reaction—such as Venezuela’s suspension from Mercosur—only if another state takes the lead
Argentine President Mauricio Macri challenged the reigning code of silence among Latin American countries by publicly condemning those left-wing governments that have eroded freedom of the press and other human rights
his government has gradually adopted a less principled position on Venezuela
This is in part related to the informal candidacy of Argentina’s foreign minister
to become the next secretary general of the United Nations
which reduces her willingness to confront Venezuela
Malcorra has advocated further attempts at dialogue with Caracas and rejected taking any punitive measures against the regime.7
Chile has become more vocal in its criticism of the Maduro government
but also without adopting a highly confrontational stance
The Chilean Supreme Court has pressed the Chilean government to get the OAS’s Inter-American Commission on Human Rights to visit Leopoldo López and another imprisoned opposition figure
The administration of President Michelle Bachelet has agreed to do this
This is probably the most forward-leaning measure taken anywhere in the region—which shows how low the hurdle has been set in democracy support measures and how strikingly cautious Latin American governments have been in trying to limit the extension of authoritarianism in Venezuela
the ruling administration of President Tabaré Vázquez of the center-left Frente Amplio (Broad Front) has so far adhered to a noninterventionist position
Former government figures including ex-president José Mujica and his foreign minister
Luis Almagro—who is now head of the OAS—have vocally criticized the Maduro administration for its handling of the economy and for its authoritarian turn.8 But the current Uruguayan government has maintained a hands-off approach
the agenda of democracy promotion in Venezuela has no regional champion
All of Latin America is suffering a hangover from the ongoing commodity bust
Venezuela still provides subsidized oil to at least ten countries in the Caribbean and retains traditional allies in Bolivia
Governments in the region apparently have no appetite to incur any risks by supporting Venezuelan democrats
Even those governments that extol pro-democracy rhetoric are in practice driven by realpolitik
None of the South American governments that profess support for democratic norms has offered any material support to Venezuelan civil society or any capacity building for pro-democracy activism
is still apparently anathema in Latin America—even in the case of such a repressive autocratic turn as witnessed in Venezuela
Venezuelan opposition leaders told us how disappointed they are with the lack of wholehearted support from Argentina
Various parliamentary organizations have visited Venezuela
Many of them have criticized Caracas for imprisoning opposition figures and curtailing the freedom of expression
been met with a hostile reception by Venezuelan authorities
A Brazilian delegation of center-right members of Congress was attacked by an angry mob on its way to visit opposition leaders in prison from Simón Bolívar International Airport and was forced to leave the country
and Uruguay have voiced concern over the erosion of democratic rule in Venezuela—but this has not pushed their respective governments into any more tangible pro-democracy support
Regional organizations have been equally ineffective in getting a grip on the crisis
The Bolivarian Alliance for the Peoples of Our America (ALBA) has been severely weakened by the Venezuelan crisis and the 2015 electoral upset in Argentina
ALBA now counts on the support of only a relatively small
less influential group of left-populist governments in Bolivia
The new diplomatic dialogue between Havana and Washington leaves Venezuela’s regime further exposed at a regional level
The Union of South American Nations (UNASUR)—long influenced most strongly by pro-chavista governments—is now less likely to be able to mediate in the Venezuelan crisis due to the rise of center-right governments in Buenos Aires
Colombia’s commitment to UNASUR has been limited from the very beginning
Policymakers in Buenos Aires and Brasília see the UNASUR secretary general
With ALBA and UNASUR sidelined and weakened
Venezuela has long worked to weaken the OAS by questioning its legitimacy and financing rival regional bodies
Many Latin American governments share this unease with the OAS and the role it accords to the United States in the region
This has prevented the OAS from playing any significant role in Venezuela
has recently been one of the most outspoken critics of the Venezuelan government
Maduro accused Almagro of being “a traitor,” and Almagro replied by calling Maduro “a petty dictator.”
Almagro invoked Article 20 of the Inter-American Democratic Charter and asked for a meeting of the OAS Permanent Council
submitting a comprehensive report that laid out the case against Venezuela’s violations of democracy and human rights
This is the first time that a secretary general of the OAS has taken such an initiative in support of democracy
Although the democratic charter was designed mainly as a mechanism to protect incumbents from coups
it makes it possible for the secretary general or any member state to call for a meeting of the Permanent Council where incumbent governments are themselves putting democracy at risk
This call was received with great enthusiasm by several organizations such as Human Rights Watch
and—as mentioned—the Venezuelan parliament.9 It certainly succeeded in generating a regionwide debate about the crisis in Venezuela
the (two-thirds majority) support needed to suspend Venezuela is lacking in the OAS
The new governments in Argentina and Brazil have both opposed any use of the Democratic Charter
OAS states declined to activate the charter when they met on June 23
If this charter is not invoked in the current context in Venezuela
it is difficult to imagine that it has any tangible utility at all
It remains unclear what the OAS can do in any concrete sense to foster democratic transition in Venezuela
to request a meeting of Mercosur foreign ministers to address the situation.10 Paraguayan government officials often express frustration that Brazil and Argentina
which were swift to punish Paraguay for a less clear-cut overturning of democracy four years ago
are today silent in the face of Venezuela’s massive violations of democratic governance
Paraguay complains that it was suspended from Mercosur for blocking Venezuela’s accession to the bloc
more than for reasons related to democracy
Although Mercosur is unlikely to suspend Venezuela or exert other forms of pressure on Maduro
on June 10 the foreign ministers of Argentina
and Uruguay signed a declaration condemning the violence in Caracas and reasserting that “the authorities are responsible for guaranteeing the right to peaceful demonstrations and to freedom of speech.” The ministers also urged that “disagreements be settled through peaceful dialogue and democratic methods.”11 Mercosur’s role is more likely to be played through critical persuasion of Venezuela than through punitive sanctions against it
Regional actors’ mediation efforts have failed to preserve democracy in Venezuela
While such bridge-building approaches should continue
they need to be backed up by a more assertive stance toward the Maduro government
The vast majority of Latin American countries seem unwilling to take an active stance on the crisis as they fear the diplomatic costs and the precedent such moves could entail
they are tacitly favoring the government’s position to the detriment of democracy and the Venezuelan people
The region should act more decisively in the defense of democracy
in particular given that the humanitarian crisis afflicting Venezuela poses real dangers for regional stability
The OAS should be one of the main vehicles of a new regional strategy
and Uruguay) should help put together a coalition of the two-thirds of OAS members needed to suspend Venezuela until the Maduro government restores judicial independence and the protection of fundamental rights
and Uruguay should suspend Venezuela from Mercosur
While some analysts argue that such initiatives would have little effect
the Maduro government cares far more about its international reputation than is generally appreciated
preserving an image of stability is so important that—insiders told us—the government sees servicing foreign debts as more pressing than maintaining social programs
Mediation efforts should continue alongside such critical measures
Former Spanish prime minister José Luis Rodríguez Zapatero has recently engaged in efforts to establish a meaningful dialogue between the government and the opposition
these efforts are laudable and should be maintained
remain the only response to the crisis in Venezuela
Brazil and Argentina have clear reasons to step in
Venezuela’s crisis damages the region’s reputation and feeds the notion that South America is adrift and incapable of solving its own problems
the leaders of other governments will look for cues from Brasília and Buenos Aires as they make up their minds on how to act
both Brazil and Argentina have a moral obligation to help
after their previous governments actively promoted economic cooperation with Caracas during a period when Maduro and Chávez worked to dismantle Venezuela’s democracy
Without diplomatic support and economic engagement from Brasília and Buenos Aires over the past decade—which generated ample economic rewards for Brazil and subsidized oil for Argentina—chavismo could not have kept itself afloat or gained such an uncompromising grip on power
Realpolitik thinking should be inverted: Venezuela’s opposition is bound to take over at some point and is unlikely to forget the lack of pro-democratic support from its neighbors
Our meetings in Caracas left us convinced of the urgent need for a more effective regional strategy for supporting democracy in Venezuela yet pessimistic about the likelihood that this will take shape
With Argentina’s government reluctant to lose votes for Malcorra’s campaign to become UN secretary general and Brazil’s government too unstable to take leadership in a meaningful way
It is far from clear whether Mercosur can unify to suspend Venezuela or whether two-thirds of the other 34 countries in the OAS are willing to invoke the Inter-American Democratic Charter
Action via the OAS or Mercosur would increase pressure on the Maduro government to cease its attacks against the legislature and scale down its meddling in the judiciary
This would not be an undue interference in the country’s internal affairs
nor would it be a signal that regional actors are biased toward the opposition
it would be a defense of Venezuelans’ rights to choose their leaders—and a sign that the region is capable of using the legal instruments that have been arduously established over the past two and a half decades in a very practical way to further democratic norms
Federico Merke is professor of international relations and director of undergraduate studies in political science and international relations at San Andrés University
Feldmann is an associate professor in the Departments of Latin American and Latino Studies and Political Science at the University of Illinois at Chicago
Oliver della Costa Stuenkel is an assistant professor of international relations at the Getúlio Vargas Foundation in São Paulo
He is also a nonresident fellow at the Global Public Policy Institute in Berlin
The Carnegie Endowment is grateful to the Robert Bosch Stiftung
and the UK Department for International Development for their support of the Rising Democracies Network
The opinions expressed in this article are the responsibility of the authors
1 We thank Richard Youngs for his insightful comments and help in the preparation of this essay
http://www.bbc.com/news/world-latin-america-34983467
3 “The World’s Most Dangerous Cities,” Economist,February 3
http://www.economist.com/blogs/graphicdetail/2016/02/daily-chart-3
6 “Venezuela’s President Maduro ‘Won’t Face Recall Referendum,’” BBC
2016, http://www.bbc.com/news/world-latin-america-36301742
“Venezuela da en la OEA un portazo a cualquier mediación en su crisis,” El Pais
http://internacional.elpais.com/internacional/2016/05/06/estados_unidos/1462492624_995606.html
8 “‘Pepe’ Mujica Said Maduro Is ‘Mad as a Hatter,’” La Nación, May 19, 2016, http://www.lanacion.com.ar/1900289-pepe-mujica-dijo-que-maduro-esta-loco-como-una-cabra
10 “Paraguay Requests Meeting of Mercosur Foreign Ministers to Address Venezuela Situation,” MercoPress
http://en.mercopress.com/2016/05/27/paraguay-requests-meeting-of-mercosur-foreign-ministers-to-address-venezuela-situation
11 Ministry of Foreign Affairs and Worship, “Press Release on Venezuela,” Argentine Republic, June 10, 2016, https://www.mrecic.gov.ar/en/press-release-venezuela.
Federico Merke is an associate professor of international relations at the Universidad de San Andrés, Argentina. He is also a researcher for the National Council for Scientific Research.
Andreas E. Feldmann is an associate professor in the departments of Latin American and Latino Studies and Political Science at the University of Illinois at Chicago.
Visiting Scholar, Democracy, Conflict, and Governance Program
Full Textprogram Democracy, Conflict, and GovernanceThe Democracy
Federico Merke is an associate professor of international relations at the Universidad de San Andrés
He is also a researcher for the National Council for Scientific Research
Feldmann is an associate professor in the departments of Latin American and Latino Studies and Political Science at the University of Illinois at Chicago
Our program examines emerging global challenges to democracy and produces policy-relevant research on international support to advance democracy
In a global political landscape marked by twenty years of widespread democratic backsliding
some good news exists: in a handful of countries where leaders significantly undercut democratic norms and institutions
elections have brought to power new leadership with a strong declared commitment to restoring democracy
As the United States steps back from diplomacy and aid aimed at supporting democracy abroad
new analytical tools are needed to assess how the new U.S
administration will impact democracy globally
Eastern Europe successfully ended its dependence on Russian natural gas
the EU must continue to diversify its supplies and invest in LNG infrastructure across the continent
The Trump administration’s effort to reshape the global trading system and reset overseas security commitments is creating an historic inflection point
Less clear is how far China will be able to capitalize on these dynamics
Home » Education » About Norsk’s Patented Rapid Plasma Deposition™ (RPD™) – A DED 3D Printing Technology
Norsk Titanium’s RPD™ is the world’s first FAA-approved
delivering substantial lead-time and cost savings for aerospace
In this article, we will dig deep into the Rapid Plasma Deposition™ technology to understand this patented 3D printing technology
Norsk Titanium is completely focused on metallurgy since the very beginning
The superior metallurgy expertise is clearly visible across part geometries
and Norsk Titanium sites in the United States and Europe
forging is considered as one of the major production technology to manufacture strong parts
Through Norsk Titanium’s patented Rapid Plasma Deposition™ (RPD™) process
titanium can be utilised to manufacture parts previously not possible
It also simplifies the entire process flow
titanium wire is precisely melted with an inert gas
This melted metal is then rapidly built up in subsequent layers
just like in case of any additive manufacturing method
The resulting product has greater strength and durability compared to regular forge-based approach
the product requires significantly less machining with the final part delivered in an on-demand environment
The MERKE IV is Norsk Titanium’s 4th generation 3D printing machine
It runs on the patented Rapid Plasma Deposition™ 3D printing technology
the MERKE IV can produce 10-20 metric tons annually
This machine is capable of taking forging to the next level by augmenting it to the on-demand environment to help aerospace companies rapidly produce complex structural components
the process is monitored more than 600 times per second for quality assurance
This sort of rapid additive manufacturing method delivers huge time and cost advantages to the entire forging industry
Reduced machining also leads to less tooling and energy usage
which are significant cost drivers for titanium parts. The end result is reduced production cost for finish titanium parts
ultimately benefiting the customer and also having an improved environmental footprint
Rapid Plasma Deposition™ technology mostly utilises titanium wire as it is heavily used in the aerospace industry but the technology works equally well with other metals like Nickel alloys
The Rapid Plasma Deposition™ technology can be used in three different methods as mentioned below:
This is the most basic method to manufacture parts in an Rapid Plasma Deposition™ 3D printing technology machine
metal parts can be additively manufactured to a near-net-shape and later machined to create the final usable product
Since the technology offers manufacturing flexibility
the Norsk Titanium RPD™ process allows the user to print the forging form that can then go into the forging die
and the capital of several of the forging dies
As the patented technology is capable of printing on any suitable surface
So users can directly forge the basic parts in a regular forge and then print the complicated features of the forgings later by printing them by Norsk Titanium Rapid Plasma Deposition™ 3D printing technology
a more simplified forging can be used to make a more complicated part
bosses on turbine engine cases can be added to ring-rolled forgings
Forge-then-print reduces the amount of material in the ring-rolled forging as well as the machining required
The technology is most suitable in the aerospace industry and it is very successful in the same industry
Norsk Titanium is a tier 1 supplier to Boeing and is committed to cost-reducing aero-structures and jet engines for the world’s premier aerospace manufacturers
Norsk Titanium’s technology also caters to the defence and space sector
Norsk Titanium has set its sights on the transportation industry
As municipalities levy green requirements on their light rail systems
and use of titanium is increasing in new designs
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Dawkins was on the programme yesterday to talk about a survey suggesting around two thirds of Christians don't know the title of the first book of the New Testament
Fraser retaliated with his own question about the full title of Origin
and when Dawkins couldn't reel it off he concluded: "If you ask people who believe in evolution that question
and you came back and said 2% got it right
it would be terribly easy for me to say they don't believe it after all."
the full title is On the Origin of Species by Means of Natural Selection or the Preservation of Favoured Races in the Struggle for Life
Crowing about Dawkins's inability to remember the full
tongue-twisting title of a 150-year-old book says more about the shallowness of anti-science arguments than his early morning memory lapse
when science was in its infancy (1986) I studied the history of the idea of evolution as part of my final year as an indifferent zoology student at Liverpool University
There were some fine budding biologists in that lecture theatre
many of whom had a hinterland of interests
in defiance of the stereotype of scientists
Which made it all the more telling when our professor
an eminent man from the days when zoology involved fewer genes and bleeping machines
asked how many of us had read On The Origin of Species by Means of Natural etc
Not one hand of the 30-odd strong honours class went up
because although it may get an honourable mention in textbooks and lessons
Origin does not represent the state of modern evolutionary biology
for all the affection it commands among scientisis
time and again as the evidence in support of evolution mounts
And the authors of sacred texts tend not to have doubts about their work
but the antipathy shown to it by some might have something to do with the wounds it inflicted on a whole herd of sacred cows
Peter McGrath is a director of the HMS Beagle Trust
787 Dreamliner to fly with FAA-certified parts made with additive manufacturing
Metal additive manufacturing in the aerospace industry isn’t just for fuel nozzles any more
Norsk Titanium AS
a supplier of additive-manufactured titanium components
recently announced that it has received a production purchase order for 3D-printed titanium structural components from Boeing
The parts are being produced using Norsk’s proprietary wire-based Rapid Plasma Deposition (RPD) process
collaborating with Norsk over the course of development
To certify these structural components on Boeing’s Dreamliner 787
the two companies undertook a rigorous testing program with FAA certification deliverables
Norsk Titanium is the first supplier for Boeing’s high deposition rate material specification
“We are proud to take this historic step with a great aerospace innovator like Boeing,” said Norsk Titanium’s president and CEO
“The Norsk Titanium team will continue to expand the portfolio of components supplied to Boeing meeting stringent certification requirements
It is an honor to earn FAA approval for these structural parts.”
“We are always looking at the latest technologies to drive cost reduction
performance and value to our customers,” said John Byrne
structures and supplier management at Boeing Commercial Airplanes
“Norsk Titanium’s RPD capability fits the bill in a new and creative way.”
The Dreamliner RPD components will be on display at the International Paris Airshow
along with a full-scale mock-up of the company’s MERKE IV Rapid Plasma Deposition machine
which produced the pioneering structural parts
For more information, visit the websites for Boeing and Norsk Titanium
Notices are posted by 10 am Monday through Saturday
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As published in Winnipeg Free Press on May 28
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it is their hope to be in a caring long-term care facility such as the New Hope Pioneer Lodge in Stoughton
She drives from Radville daily to do her job
Daenckaert is taking Daphne Merke-Opruk’ place as Merke-Opruk is presently on a maternity leave
The government-owned facility was built in 1986 and its home to 26 long-term residents
The LTC can house 30 residents and also has a homecare office
All are such important roles to keep this home comfortable for the residents and well cared for
Daenckaert explained that during the pandemic
staff followed all rules and regulations very closely to ensure the safety and wellbeing of the residents
they had their struggles through these tough times
but the staff worked extremely hard along with family and friends to make this pandemic an easier situation for all involved
especially during the challenging time of lockdowns
they made sure that family and friends could do virtual calls
regular phone calls and even window visits
The staff did their utmost to accommodate these visits
as this was an especially important part of the health and wellness of the residents
The hallway became a music room and dance hall
As staff would dance with their clients up and down the halls
Bingo was another exciting event for the home
The Stoughton Central School also did their part by painting the windows
Extra activities became part of the agenda to keep everyone busy and happy
“I’m so grateful for the hard-working
amazing staff that stayed educated and focused during that time.”
She went on to say they had daily communication so everyone could stay on top of the events happening with COVID
Church services are also a part of the lodge
With several of the mandates being removed
A new volunteer program is available for those who wish to help out
Volunteers are always in demand in most areas including the Meals on Wheels program
as residents may not have family or friends nearby
When a resident has the opportunity to interact with a caring volunteer
it can make all the difference in the world to them
Someone may just like a book read to them or to play a game
according to the Saskatchewan Health Authority
over 257,000 hours were logged through the volunteer program with the SHA
Approximately 30,000 volunteers are registered
A strong magnitude 5.0 earthquake hit 23 km (14 mi) away from Merke, Zhambyl Oblysy, Kazakhstan
2025 at 3.56 pm local time (Asia/Almaty GMT +5)
The depth of the quake could not be determined
but is assumed to be shallow.The earthquake was felt over a large region.
The shallow depth of the quake caused it to be felt more strongly near the epicenter than a deeper quake of similar magnitude would.