<p>Wilson's disease (WD), bepatolenticular degeneration, is an autosomal recessive disorder caused by mutations in the ATP7B gene, and is characterised by abnormal metabolism and deposition of copper in the liver, brain and other organs. The main aim of this thesis was to investigate the occurrence of psychopathology, as well as personality traits and neuropsychological function in Swedish patients with treated WD. The research subjects were 29 patients with confirmed WD, investigated at the Department of Internal Medicine at Uppsala University Hospital between 1996 and 2000. </p><p>The treated WD patients showed prominent psychopathology as determined by the Comprehensive Psychopathological Rating Scale. The spectrum of psychopathological symptoms is not typical of classic psychiatric syndromes, and includes symptoms from Anxiety, Depression and Obsessive-Compulsive disorders as well as Negative Symptoms. In self-assessment, the WD patients tended to underestimate the presence of psychopathological symptoms. The treated WD patients differed in their sleep pattern from the control group, as measured with the Uppsala Sleep Inventory. The spectrum of self-reported symptoms suggests an altered REM sleep function. </p><p>The treated WD patients had significant deviations in personality traits, especially in aggressivity-hostility related scales and Psychic anxiety, compared to healthy controls, as measured with the Karolinska Scales of Personality. The deviations were not related to age, age at onset or duration of WD. The treated WD patients displayed a specific profile of moderate neuropsychological impairment, as determined by the Automated Psychological Test battery. Finally, an attempt was made to search for, possible genotype-phenotype relationships in some ATP7B mutations. </p>

Wilson's disease (WD), bepatolenticular degeneration, is an autosomal recessive disorder caused by mutations in the ATP7B gene, and is characterised by abnormal metabolism and deposition of copper in the liver, brain and other organs. The main aim of this thesis was to investigate the occurrence of psychopathology, as well as personality traits and neuropsychological function in Swedish patients with treated WD. The research subjects were 29 patients with confirmed WD, investigated at the Department of Internal Medicine at Uppsala University Hospital between 1996 and 2000. The treated WD patients showed prominent psychopathology as determined by the Comprehensive Psychopathological Rating Scale. The spectrum of psychopathological symptoms is not typical of classic psychiatric syndromes, and includes symptoms from Anxiety, Depression and Obsessive-Compulsive disorders as well as Negative Symptoms. In self-assessment, the WD patients tended to underestimate the presence of psychopathological symptoms. The treated WD patients differed in their sleep pattern from the control group, as measured with the Uppsala Sleep Inventory. The spectrum of self-reported symptoms suggests an altered REM sleep function. The treated WD patients had significant deviations in personality traits, especially in aggressivity-hostility related scales and Psychic anxiety, compared to healthy controls, as measured with the Karolinska Scales of Personality. The deviations were not related to age, age at onset or duration of WD. The treated WD patients displayed a specific profile of moderate neuropsychological impairment, as determined by the Automated Psychological Test battery. Finally, an attempt was made to search for, possible genotype-phenotype relationships in some ATP7B mutations.

Abstract available through the Developmental Psychobiology.

Environmental issues increasingly demonstrate local-global linkages in driving forces and effects. Policy responses are initiated at local, national and global levels. The successful management of such cross-level environmental issues involves co-ordinated and co-operative policies and action among stakeholders at several levels of governance. Pesticide use in the South-which is a potential driving force for environmental and health problems-has in this dissertation been analysed in relation to such multilayered governance. The theoretical framework from studies on common property resource (CPR) management is applied, facilitating the cross-level analysis of pesticide use in the South as being a global common. The study looks into problem structuring, risk reduction policies and decisionmaking with respect to pesticide use in the South at the local, national and global levels, with Kenya and Costa Rica as cases for the national and local levels. The degree of common understanding among stakeholders across governance levels on what the problems with pesticide use in the South are and how the problems should be addressed and why, is limited but not entirely absent. Mismatches ininformation flows and knowledge, institutions, and values between governance levels hamper the prospect of establishing multilayered governance. These mismatches can be addressed by giving more attention to the level at which institutions are functional, by involving more stakeholders in the generation of knowledge, and by adopting more inclusive values. One approach to achieve these required changes is to embrace a systems perspective on this issue as a global common, a global environmental concern.

No description available.

本研究由個人參與社區發展行動的動機與選擇決策作為研究的重心，以理性選擇行為模式為理論基礎，探討社區發展上如何才能克服集體行動的困境，建立永續性的自主治理機制。由於社區事務具有小規模共用資源（Common-Pool Resources；CPRs）的特性，在功能上及產權上無法分割，在使用上無排他性（non- exclusive）但具有某種程度的敵對性（rivalry），過度取用將產生資源耗竭的現象。因此本研究援引共用資源分析方法（Common-Pool Resources Approach）作為分析架構，從個人選擇與互動形式、外部環境與技術的變量以及組織與執行機制三方面，探討社區居民是否形成自主治理的集體行動及其影響變量。 本研究採用多重個案研究的複現方法，選取了三處遭遇特定事件觸發了社群共同議題，而由外力團隊協助建立治理組織之社區，作為實證研究之個案。包括：（1）南投縣中寮鄉的農村社區在歷經九二一大地震後，面對土地資源永續利用與產業轉型的議題；（2）桃園縣龍潭鄉百年大鎮大型住宅社區因社區組織不健全，導致社區環境出現安全問題；（3）台北縣鶯歌鎮陶瓷老街因政策獲選為形象商圈，店家面臨商圈環境管理與商圈行銷議題。 / Base on the theory of reasonable choice behavior mode, the study focus on the resident’s individual motive and decision making process trying to identify the solutions of breaking the bottlenecks of collective actions and build up the sustainable self-government mechanism. The community affairs are small scale common-pool resources CPRs) therefore we cannot clearly dive up their ownerships and functions. In the aspects of usage, the community affairs are non-exclusive but kind of rivalry. It will be decreased if being over-deprived. Thus, this study adopted the common-pool resources approach as the analytical structure to explore if the community residents have organized the collective actions of self-government and the influence variables form 3 major aspects of “individual choice vs. interactive form”, “external environment vs. technical variables” and “organization vs. enforcement mechanism ”. The study took 3 occasional events which touched off some common agenda in the community and have the residents established the organizations of governance by the assistance of external teams as the impractical cases. The three cases included: (1)The agenda faced by rural villages on the aspects of land sustainable usage and industrial transformation in Jhingliao Township of Nantou County, (2) The safety problems brought by the poor community organization of the big scale residential community—Netown in Longtan Township of Taoyuan County.(3) The marketing and business district environment management issues brought by being selected as the official model business district in the Yingge Township of Taipei County.