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We report a case of hypoplastic left heart syndrome and with subsequent aortopathy and then found to have hereditary haemorrhagic telangiectasia/juvenile polyposis syndrome due to a germline SMAD4 pathologic variant. The patient’s staged palliation was complicated by the development of neoaortic aneurysms, arteriovenous malformations, and gastrointestinal bleeding thought to be secondary to Fontan circulation, but workup revealed a SMAD4 variant consistent with hereditary haemorrhagic telangiectasia/juvenile polyposis syndrome. This case underscores the importance of genetic modifiers in CHD, especially those with Fontan physiology.
Wortupaite (IMA2022–107) is a new hydrated magnesium nickel tellurite mineral with a zemannite-like structure, described from the Wortupa gold mine, South Australia, Australia. Wortupaite forms needles up to 25 μm in length, generally clustered and sometimes in blocky masses of shorter (10‒15 μm) crystals. Wortupaite is found growing on melonite, from which the component nickel and tellurium are derived, and is associated with calcite. The strongest powder diffraction lines are [dobsÅ(Iobs)(hkl)]: 8.059 (93) (100), 4.034 (92) (200), 2.832 (43) (211 and 121), 2.769 (100) (202) and 1.920 (45) (213 and 123). The empirical formula of wortupaite as determined by electron probe microanalysis is (Mg0.57Ni0.39Mn0.04)Σ1(Ni2+1.87Fe3+0.13)Σ2(Te4+O3)3⋅3H2O, simplified to the ideal formula of MgNi2+2(Te4+O3)3⋅3H2O with H2O content calculated from the crystal structure. The average crystal structure of wortupaite was determined by single-crystal X-ray diffraction with synchrotron radiation (R1 = 0.0558 for 100 independent reflections). Wortupaite is hexagonal, crystallising in the space group P63/m, with a = 9.2215(13) Å, c = 7.5150(15) Å, V = 553.43(19) Å3 and Z = 2. Wortupaite has a microporous structure, with the negatively charged zemannite-like framework formed by Te4+O3 trigonal pyramids and Ni2+O6 octahedra. For charge balance, Mg2+ and Ni2+ dominant sites are assumed to be located on central sites in the channels, coordinated by 6 H2O groups. An OW site was refined around the Mg2+ dominant site, but OW position(s) were not locatable around the Ni2+ dominant site. A discussion of the different models for crystallographic arrangement of channel species is provided, taking into account possible Fourier truncation effects. Unlike the other four minerals with zemannite-like structures which have a near 50% split of divalent and trivalent framework cations, wortupaite is the first natural phase to have only divalent cations in the framework sites.
This article presents texts recovered by post-processing of multispectral images from the fifth- or sixth-century underwriting of the palimpsest Codex Climaci Rescriptus. Texts identified include the Anonymous II Proemium to Aratus’ Phaenomena, parts of Eratosthenes’ Catasterisms, Aratus’ Phaenomena lines 71–4 and 282–99 and previously unknown text, including some of the earliest astronomical measurements to survive in any Greek manuscript. Codex Climaci Rescriptus also contains at least three astronomical drawings. These appear to form part of an illustrated manuscript, with considerable textual value not merely on the basis of its age but also of its readings. The manuscript undertexts show significant overlap with the Φ Edition, postulated as ancestor of the various Latin Aratea.
Shade coffee is a well-studied cultivation strategy that creates habitat for tropical birds while also maintaining agricultural yield. Although there is a general consensus that shade coffee is more “bird-friendly” than a sun coffee monoculture, little work has investigated the effects of specific shade tree species on insectivorous bird diversity. This study involved avian foraging observations, mist-netting data, temperature loggers, and arthropod sampling to investigate bottom-up effects of two shade tree taxa - native Cordia sp. and introduced Grevillea robusta - on insectivorous bird communities in central Kenya. Results indicate that foliage-dwelling arthropod abundance, and the richness and overall abundance of foraging birds were all higher on Cordia than on Grevillea. Furthermore, multivariate analyses of the bird community indicate a significant difference in community composition between the canopies of the two tree species, though the communities of birds using the coffee understorey under these shade trees were similar. In addition, both shade trees buffered temperatures in coffee, and temperatures under Cordia were marginally cooler than under Grevillea. These results suggest that native Cordia trees on East African shade coffee farms may be better at mitigating habitat loss and attracting insectivorous birds that could promote ecosystem services. Identifying differences in prey abundance and preferences in bird foraging behaviour not only fills basic gaps in our understanding of the ecology of East African coffee farms, it also aids in developing region-specific information to optimize functional diversity, ecosystem services, and the conservation of birds in agricultural landscapes.
Studying phenotypic and genetic characteristics of age at onset (AAO) and polarity at onset (PAO) in bipolar disorder can provide new insights into disease pathology and facilitate the development of screening tools.
To examine the genetic architecture of AAO and PAO and their association with bipolar disorder disease characteristics.
Genome-wide association studies (GWASs) and polygenic score (PGS) analyses of AAO (n = 12 977) and PAO (n = 6773) were conducted in patients with bipolar disorder from 34 cohorts and a replication sample (n = 2237). The association of onset with disease characteristics was investigated in two of these cohorts.
Earlier AAO was associated with a higher probability of psychotic symptoms, suicidality, lower educational attainment, not living together and fewer episodes. Depressive onset correlated with suicidality and manic onset correlated with delusions and manic episodes. Systematic differences in AAO between cohorts and continents of origin were observed. This was also reflected in single-nucleotide variant-based heritability estimates, with higher heritabilities for stricter onset definitions. Increased PGS for autism spectrum disorder (β = −0.34 years, s.e. = 0.08), major depression (β = −0.34 years, s.e. = 0.08), schizophrenia (β = −0.39 years, s.e. = 0.08), and educational attainment (β = −0.31 years, s.e. = 0.08) were associated with an earlier AAO. The AAO GWAS identified one significant locus, but this finding did not replicate. Neither GWAS nor PGS analyses yielded significant associations with PAO.
AAO and PAO are associated with indicators of bipolar disorder severity. Individuals with an earlier onset show an increased polygenic liability for a broad spectrum of psychiatric traits. Systematic differences in AAO across cohorts, continents and phenotype definitions introduce significant heterogeneity, affecting analyses.
We provide an overview of the monetary policy failures that resulted in the 2007–2008 financial crisis and ensuing Great Recession, focusing on the United States. Before the crisis, monetary policy was too loose, which fueled the bubble. After the bubble burst, monetary policy became too tight, hindering the recovery. These failures are fundamentally due to the Federal Reserve’s discretionary monetary policy. Furthermore, the popular approach of “constrained discretion” is really just discretion. Hence, it is sensitive to all the usual problems with discretionary monetary policy. Only firm monetary rules, ones that actually bind, can maintain macroeconomic stability and prevent crises.
Orthodox monetary policy scholarship assumes that central bankers act to maximize the public welfare. If imperfect incentives enter the model, it is on the part of the public. We challenge this assumption. Monetary policymakers are just as prone to incentive problems, which cause them to act according to their own self-interest. Furthermore, the self-interest of policymakers is not always the same thing as the public welfare. The two diverge frequently, in fact. We survey the history of the Federal Reserve and show the numerous ways discretionary central bankers have been compromised. These incentive problems are an inherent feature of discretion. They can only be eliminated by embracing true monetary rules.
We analyze the information problems inherent in discretionary monetary policy. Discretionary central bankers confront immense informational burdens. Some of these are technical problems only, and can in principle be overcome. But there is also a genuine knowledge problem involved in discretionary monetary policy: reacting in real time to changes in the demand for money. This problem is unsolvable. It renders discretionary central banking systematically unlikely to achieve macroeconomic stability. In contrast, rules-based policy does not confront a knowledge problem.
We conclude by situating the theory and practice of monetary policy within liberal political economy more generally. As we have seen, there are significant tensions between existing monetary institutions (discretionary central banking) and liberal ideals. This has been made even clearer by the Federal Reserve’s response to COVID-19. In brief, the Fed is now engaging in not only monetary policy but fiscal policy as well. This represents an immense expansion in its mandate, one that poses serious challenges for general and predictable monetary policy. The way out of this mess is embracing a comparative institutions approach to monetary policy. We cannot be satisfied with technical refinements to existing models and data. We need to explore alternative monetary policy rules, ones that are effective at providing macroeconomic stability while also respecting the requirements of democracy.
At root, the problems with the Federal Reserve (and many other central banks) are institutional. The repeated recessions and crises in the era of the Fed show that we need a radical reimagination of the basic institutions of monetary policy. In this chapter, we survey the work of the three great classically liberal Nobel laureates of the twentieth century – James Buchanan, F. A. Hayek, and Milton Friedman – to show that each of them gave serious consideration to monetary-institutional fundamentals. Our focus is not on their particular conclusions, but on how they thought about the problems of monetary institutional design. This represents a very different style of scholarship than macroeconomists and monetary economists currently practice. Unless scholars engage the research projects of Buchanan, Hayek, and Friedman, research in monetary economics will not be of much help in achieving lasting macroeconomic stability.
Financial crises are widely perceived to be the reason monetary rules cannot work. The extraordinary challenges posed by crises require policymakers to act discretionarily. We show that this argument is not only wrong but backward: It is more important than ever to have true rules for monetary policy, which actually bind, to cope with financial crises. We show how the Fed failed to respond appropriately to the 2007–2008 crisis. Contrary to the then chairman Bernanke’s public statements, the Fed did not behave as an orthodox lender of last resort. Instead, it experimented with dubious policies that further entrenched moral hazard in the financial system. We criticize these policies, as well as an approach to economics, which we call “triage economics,” that mistakenly supposes the basic rules of price theory provided no guidance in crafting policy responses to crises. A rules-based approach to monetary policy is thus consistent with extreme market turbulence. In fact, rules are how such turbulence is pacified.
In this chapter, we focus on the idea of the rule of law in the classical liberal tradition. The rule of law is a basic jurisprudential norm that undergirds liberal democracies. We show that discretionary central banking is inconsistent with the rule of law. Discretionary central banking fails the test of generality: It benefits special interests, but not the public as a whole. Also, discretionary banking fails the test of predictability: It does not create an environment conducive to reliable public expectations of future policy. For these reasons, it is unlikely that discretionary central banking can be reconciled with self-governance. We reaffirm the imperative of liberal democracy, as well as uncovering monetary institutions that are compatible with liberal democracy. Until we do so, we fail to meet the basic challenge of self-governance.
Contemporary monetary institutions are flawed at a foundational level. The reigning paradigm in monetary policy holds up constrained discretion as the preferred operating framework for central banks. But no matter how smart or well-intentioned are central bankers, discretionary policy contains information and incentive problems that make macroeconomic stability systematically unlikely. Furthermore, central bank discretion implicitly violates the basic jurisprudential norms of liberal democracy. Drawing on a wide body of scholarship, this volume presents a novel argument in favor of embedding monetary institutions into a rule of law framework. The authors argue for general, predictable rules to provide a sturdier foundation for economic growth and prosperity. A rule of law approach to monetary policy would remedy the flaws that resulted in misguided monetary responses to the 2007-8 financial crisis and the COVID-19 pandemic. Understanding the case for true monetary rules is the first step toward creating more stable monetary institutions.