Smith-Magenis syndrome (SMS; OMIM 182290) is a neurodevelopmental disorder characterized by a well-defined pattern of anomalies. The majority of cases are due to a common deletion in chromosome 17p11.2 that includes the RAI1 gene. In children with SMS, autistic-like behaviors and symptoms start to emerge around 18 months of age. This study included 26 individuals (15 females and 11 males), with a confirmed deletion (del 17p11.2). Parents/caregivers were asked to complete the Social Responsiveness Scale (SRS) and the Social Communication Questionnaire (SCQ) both current and lifetime versions. The results suggest that 90% of the sample had SRS scores consistent with autism spectrum disorders. Moreover, females showed more impairment in total T-scores (p=0.02) and in the social cognition (p=0.01) and autistic mannerisms (p=0.002) subscales. The SCQ scores are consistent to show that a majority of individuals may meet criteria for autism spectrum disorders at some point in their lifetime. These results suggest that SMS needs to be considered in the differential diagnosis of autism spectrum disorders but also that therapeutic interventions for autism are likely to benefit individuals with SMS. The mechanisms by which the deletion of RAI1 and contiguous genes cause psychopathology remain unknown but they provide a solid starting point for further studies of gene-brain-behavior interactions in SMS and autism spectrum disorders.
Summary
This is the translation of the Mizar article containing readable Mizar proofs of some axiomatic geometry theorems formulated by the great Polish mathematician Alfred Tarski [8], and we hope to continue this work.
The article is an extension and upgrading of the source code written by the first author with the help of miz3 tool; his primary goal was to use proof checkers to help teach rigorous axiomatic geometry in high school using Hilbert’s axioms.
This is largely a Mizar port of Julien Narboux’s Coq pseudo-code [6]. We partially prove the theorem of [7] that Tarski’s (extremely weak!) plane geometry axioms imply Hilbert’s axioms. Specifically, we obtain Gupta’s amazing proof which implies Hilbert’s axiom I1 that two points determine a line.
The primary Mizar coding was heavily influenced by [9] on axioms of incidence geometry. The original development was much improved using Mizar adjectives instead of predicates only, and to use this machinery in full extent, we have to construct some models of Tarski geometry. These are listed in the second section, together with appropriate registrations of clusters. Also models of Tarski’s geometry related to real planes were constructed.
Enormous increases in international travelby public sector employees and others, along with incidents of terrorism, accidents, and disease, raise a variety of ethical issues not normally covered in the training of public personnel administrators or in the standard administrative ethics course. Issues of accessibility for individuals with disabilities may be familiar to personnel administrators and students of ethics, but take on vast new dimensions when those individuals travel abroad. Travel-related ethics issues involved in health, safety, and accessibility may include identification of individual and institutional responsibilities, informed consent, contingency planning, emergency response mechanisms, fairness, and equal treatment.This study provides an overview of trends and issues, explores their ethical dimen sions, and identifies relevant strategies to prepare public administrators to deal appropriately with these concerns. The study treats both tourist and educational travel abroad, and considers risks to host societies as well as to travelers.
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