Pediatric rheumatology: addressing the transition to adult-orientated health care.

The transition from pediatric to adult health care is often a challenging process due to multiple interwoven complexities, especially for children with chronic medical conditions. Health care transition (HCT) is a process of moving from a pediatric to an adult model of health care with or without a transfer to a new clinician. This paper focuses on what is known about HCT for youth and young adults (Y/YA) with rheumatic diseases within a larger context of HCT recommendations. HCT barriers for youth, families, and providers and current evidence for a structured HCT processes are reviewed. Practical advice is offered on how to approach transition for Y/YA, what tools are available to assist in a successful transition process, and what are the areas of future research that are needed to improve the HCT evidence base

"Do Not Go Out Alone at Night": Law and Demonic Discourse in the Babylonian Talmud

This dissertation focuses on the modes of controlling, avoiding, and appropriating demons in the Babylonian Talmud, with particular attention to rabbinic legal discourse. Though scholars have largely overlooked demons as a source of information about rabbinic legal discourse, cross-cultural interaction, and theology, this dissertation has asked how the inclusion of rabbinic demonology enriches our picture of rabbinic discourse and thought in Late Antique Sasanian Babylonia. I analyze rabbinic legal passages relating to demons within their larger textual, redactional, and cultural – Zoroastrian, Christian, and ancient Near Eastern – contexts, in order to uncover and highlight the discursive choices made by Babylonian rabbis in their legislation regarding demons, and in their constructions of the demonic. I argue that the rabbis constructed demons as subjects of rabbinic law in ways that adopt, adapt, and reject particular cultural options available to them. This act of cultural bricolage results in the creation of a uniquely rabbinic perspective. The first chapter reviews previous scholarship on demons in ancient Judaism and religious studies more broadly and lays out the theoretical model for my study of those ancient texts which deal with demons. The second chapter examines one extended passage in the Babylonian Talmud (b. Pesahim 109b-112a) using source criticism, form criticism, and redaction criticism, in order to create a basic model of Babylonian rabbinic demonic discourse. I argue that the Babylonian rabbis neutralize demons by turning them into subjects, informants, and teachers of rabbinic law, thus subjugating them to the legal system. The third chapter highlights those areas where the Babylonian rabbis differed from the Jewish traditions they inherited, by comparing demonic discourse in the law and narratives of the Babylonian Talmud with those of Second Temple literature and the Palestinian Talmud. I suggest that this differentiation was a crucial element of Babylonian rabbinic self-formation as an elite distinct from their Palestinian confreres. The fourth chapter contextualizes Babylonian rabbinic demonic discourse within early Mesopotamian, Ugaritic, Zoroastrian, Armenian, and Syriac Christian literatures, as well as the Babylonian incantation bowls. I show that Babylonian rabbinic demonic discourse aligns in content with ancient Sumerian and Akkadian understandings of the demonic, and adopts only the form of legal discourse from the contemporaneous Zoroastrian elite. My conclusion advocates for a more nuanced understanding of rabbinic interaction with non-Jews and non-rabbinic Jews which takes into account both past and present cultural traditions, as part of the construction of rabbinic identity as an elite group empowered over and against other Jews. My understanding of the role of demons in Talmudic law also suggests one way for scholars of monotheistic religion more generally to understand the historical attitude towards and construction of semi-divine beings within monotheistic systems as part of a broader holistic theological and legal worldview. My work also contributes to a refinement of broader theories of demons, magic, and religion by situating demonological concerns within the realm of normative religion and not that of non-normative magic

Overconfident people are more exposed to “black swan” events: a case study of avalanche risk

Overconfidence is a well-established bias in which someone’s subjective confidence in their own judgment is systematically greater than their objective accuracy. There is abundant anecdotal evidence that overconfident people increase their exposure to risk. In this paper, we test whether overconfident backcountry skiers underestimate the probability of incurring a snow avalanche accident. An avalanche accident is a typical “black swan” event as defined by Taleb (The black swan: the impact of the highly improbable, Random House, New York, 2007) because it has a very low probability of occurring but with potentially dramatic consequences. To consider black swan events when studying overconfidence is particularly important, in light of previous findings on the role of overconfidence when feedbacks on tasks previously performed are inconclusive and infrequent. We run our test by measuring individual overconfidence using standard tools from the literature and then use a random effect logit model to measure its effect on the probability to take the ski route. We show that (1) overconfidence is widespread in our sample; (2) practitioners who are more prone to overestimate their knowledge are also more likely to take risks associated with a ski trip under the threat of avalanche danger, a result robust to a set of specification tests we perform. This suggests that overconfident people are more exposed to black swan events, by taking a risky decision that can bring about fatal consequences

Distinct interferon signatures and cytokine patterns define additional systemic autoinflammatory diseases

BACKGROUND. Undifferentiated systemic autoinflammatory diseases (USAIDs) present diagnostic and therapeutic challenges. Chronic interferon (IFN) signaling and cytokine dysregulation may identify diseases with available targeted treatments. METHODS. Sixty-six consecutively referred USAID patients underwent underwent screening for the presence of an interferon signature using a standardized type-I IFN-response-gene score (IRG-S), cytokine profiling, and genetic evaluation by next-generation sequencing. RESULTS. Thirty-six USAID patients (55%) had elevated IRG-S. Neutrophilic panniculitis (40% vs. 0%), basal ganglia calcifications (46% vs. 0%), interstitial lung disease (47% vs. 5%), and myositis (60% vs. 10%) were more prevalent in patients with elevated IRG-S. Moderate IRG-S elevation and highly elevated serum IL-18 distinguished 8 patients with pulmonary alveolar proteinosis (PAP) and recurrent macrophage activation syndrome (MAS). Among patients with panniculitis and progressive cytopenias, 2 patients were compound heterozygous for potentially novel LRBA mutations, 4 patients harbored potentially novel splice variants in IKBKG (which encodes NF-κB essential modulator [NEMO]), and 6 patients had de novo frameshift mutations in SAMD9L. Of additional 12 patients with elevated IRG-S and CANDLE-, SAVI- or Aicardi-Goutières syndrome–like (AGS-like) phenotypes, 5 patients carried mutations in either SAMHD1, TREX1, PSMB8, or PSMG2. Two patients had anti-MDA5 autoantibody–positive juvenile dermatomyositis, and 7 could not be classified. Patients with LRBA, IKBKG, and SAMD9L mutations showed a pattern of IRG elevation that suggests prominent NF-κB activation different from the canonical interferonopathies CANDLE, SAVI, and AGS. CONCLUSIONS. In patients with elevated IRG-S, we identified characteristic clinical features and 3 additional autoinflammatory diseases: IL-18–mediated PAP and recurrent MAS (IL-18PAP-MAS), NEMO deleted exon 5–autoinflammatory syndrome (NEMO-NDAS), and SAMD9L-associated autoinflammatory disease (SAMD9L-SAAD). The IRG-S expands the diagnostic armamentarium in evaluating USAIDs and points to different pathways regulating IRG expression