Isolated noncompaction of the left ventricular myocardium (INVM) is characterized by the presence of numerous prominent trabeculations and deep intertrabecular recesses within the left ventricle, sometimes also affecting the right ventricle and interventricular septum. Familial occurrence of this disorder was described previously. We present a family in which 6 affected individuals demonstrated X-linked recessive inheritance of this trait. Affected relatives presented postnatally with left ventricular failure and arrhythmias, associated with the pathognomonic echocardiographic findings of INVM. The usual findings of Barth syndrome (neutropenia, growth retardation, elevated urinary organic acids, low carnitine levels, and mitochondrial abnormalities) were either absent or found inconsistently. Fetal echocardiograms obtained between 24-30 weeks of gestation in 3 of the affected males showed a dilated left ventricle in one heart, but were not otherwise diagnostic of INVM in any of the cases. Four of the affected individuals died during infancy, one is in cardiac failure at age 8 months, and one is alive following cardiac transplant at age 9 months. The hearts from infants who died or underwent transplantation appeared, on gross examination, to be enlarged, with coarse, deep ventricular trabeculations and prominent endocardial fibroelastosis. Histologically, there were loosely organized fascicles of myocytes in subepicardial and midmyocardial zones of both ventricles, and the myocytes showed thin, often angulated fibers with prominent central clearing and reduced numbers of filaments. Markedly elongated mitochondria were present in some ventricular myocytes from one specimen, but this finding was not reproducible. Genetic linkage analysis has localized INVM to the Xq28 region, where other myopathies with cardiac involvement have been located.

No description available.

No description available.

No description available.

No description available.

No description available.

This dissertation investigates the evangelical ethics of Prof. Oliver O’Donovan in order to explore the implications of his “evangelical realism” for theological anthropology, moral knowledge and the concept of moral order. The Barth-Brunner debate regarding natural theology provides a lens onto these issues. Theological case studies are used to test our findings. Chapter 1 provides an overture to these issues, paying attention to current ideas about human nature and morality, and the growing influence of neuroscience and evolutionary psychology. Chapter 2 focuses on Resurrection and Moral Order, elucidating the salient factors in its outline for evangelical ethics. Chapter 3 diagnoses the challenges which a dialectical epistemology presents to the development of a doctrine of evangelical ethics. Chapter 4 delves into O’Donovan’s treatment of the Barth-Brunner debate over natural theology, and discovers therein an illuminating correspondence between O’Donovan’s ethics and the concept of a human “capacity for revelation” (Offenbarungsmächtigkeit), which became a hinge issue in the debate. This provides a helpful lens onto O’Donovan’s concept of moral order. Chapter 5 examines the intrinsic connection between the concept of moral order and the epistemic role of faith. Kierkegaard’s treatment of the paradoxical aspects of faith as an event of epistemic access figures prominently in this analysis. Chapter 6 brings together the results of our analysis and applies them to the thesis that: the transformation of persons lies at the heart of evangelical ethics. The cosmology of faith emerges as a critical hermeneutical factor in the development of a doctrine of evangelical ethics. We explore here the doctrinal implications for Trinitarian theology. Chapter 7 draws out practical implications of our thesis. We see the central place of prayer and worship in evangelical ethics, and point out implications for teaching. Lastly, we show practical applications of our thesis by examining the bio-ethical issues of human reproductive technologies, with special attention to O’Donovan’s work, Begotten or Made?

This study offers an ecumenical exploration of human transformation through the examination of this topic in the thought of Karl Barth (1888-1968), a Swiss Reformed theologian; Joseph Ratzinger (b. 1927), a Roman Catholic theologian; and John Zizioulas (b. 1931), a Greek Orthodox theologian. Describing and understanding human transformation stands as a crucial task for theology because no one is simply born a Christian—in order to be a Christian one must become a Christian. The first chapter introduces this topic, the three theologians (highlighting their commonalities), and the three questions that guide the analysis of each theologian and the thesis as a whole: What is the goal of human transformation? What is the basis of human transformation? How are humans transformed? Chapters 2, 3, and 4 treat the topic of human transformation in the theology of Barth, Ratzinger, and Zizioulas, respectively. All three understand the goal of human transformation to be the prayer of the children of God, and locate its basis in God’s reconciling act in Jesus Christ—an act itself based in the primordial divine decision to be God pro nobis. Even within this broad agreement, however, differences are evident, especially with regard to eschatology. Consideration of how this transformation occurs reveals significant differences concerning the agency of Jesus Christ in relation to the Holy Spirit and the church. The final chapter explores 1) the convergences and divergences between Barth, Ratzinger, and Zizioulas regarding human transformation; 2) the contributions of this study to the interpretation of Barth, Ratzinger, and Zizioulas; and 3) the relationship between human transformation and participation in God. Throughout, attention is given to the relationship between Jesus Christ, the Holy Spirit, the church, the eschaton, and the triunity of God and human transformation. All three accounts of human transformation point beyond the transition between sinful and redeemed humanity to a dynamic anthropology in which the constant asking, receiving, thanking, and asking again is the very “ontological location” of the eschatological life of humanity: our being is in becoming.

No description available.

New, old novels, contemporary fictions that parody the forms, conventions, and devices of eighteenth- and nineteenth-century novels, form a significant and increasingly popular subclass of postmodernist fiction. Paradoxically combining realistic and metafictional conventions, these works establish an ironic dialogue with the past, employing yet simultaneously subverting traditional fictional techniques. / In this dissertation, I subject five new, old novels--John Barth's The Sot-Weed Factor and LETTERS, Erica Jong's Fanny, T. Coraghessan Boyle's Water Music, and John Fowles's The French Lieutenant's Woman--to a detailed analysis, which compares the parodic role of archaic devices in each contemporary novel to the serious use made of such devices in the past. I argue that new, old novels, by juxtaposing old and new world views, foreground the ontological concerns of fiction and suggest that literary representation is constitutive rather than imitative of reality. Their examination of the relationship between fiction and reality places them at the centre of contemporary concern.