Criminal Law: Demise of “Status”—“Act” Distinction in Symptomatic Crimes of Narcotic Addiction

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On Aniridia in Sweden and Norway

Introduction Aniridia (iris more or less missing), is a congenital, dominant, inherited, serious and sight-threatening disease, most often appearing as an isolated eye disorder. It is caused by mutations in the PAX6 gene, the master gene of the development of the eye. Typically, Aniridia causes severe visual impairment. In addition to glare caused by the iris defect, underdevelopment of the macula and the adjacent retina contribute to a reduction of the visual function. A number of complications including lens luxation and/or cataract, glaucoma and keratodystrophy are often superimposed. Purpose To increase knowledge about Congenital Aniridia, an uncommon, serious and sight-threatening disease, which is not well known, neither by ophthalmologists nor by patients? Many of the treatment strategies used today have insufficient effects. Material and Methods We searched for patients with aniridia in the entire populations of two countries, Sweden and Norway, to be able to do a retrospective investigation.The material consists of 181 patients, 123 in Sweden and 58 in Norway. Of the 181 identified patients, a total of 124 (69%) were examined. Four patients with WAGR (Wilm´s tumor, Aniridia, Genitourinary abnormalities, mental Retardation) and four patients with Gillespie syndrome (aniridia, cerebellar ataxia and mental retardation) were included in the material. The examination included recordings of past medical and family history. Blood samples were taken for mutation analysis and pedigrees were established. The first part of the clinical examination was testing of visual acuity. Slit lamp examination and ophthalmoscopy were both supplemented by digital photographs, to enable re-evaluation of the results. Results 1)Our prevalence figures in children and teenagers with aniridia show that aniridia may be more common than previously estimated. 2)Complications seem to appear earlier in life than previously described in the literature. 3)We found a significant positive correlation between aniridic keratopathy and sensitivity. 4)In contrast to the literature, we found that almost all eyes with congenital aniridia had a normal corneal diameter. 5)Considering the fact that a study of aniridia in Sweden and Norway patients revealed four patients with the Gillespie syndrome compared to some 30 patients reported in the literature, the Gillespie syndrome seems to be less uncommon than previously supposed

Corneal Involvement in Congenital Aniridia.

PURPOSE:: The purpose of this research is 2-fold. First of all, the level of keratopathy development in patients with congenital aniridia is studied. In addition, a correlation between the effects of ocular surgery on the severity of keratopathy is made. METHODS:: A thorough search for the total number of patients with aniridia in Sweden and Norway was performed. One hundred eighty-one patients were identified and 124 (69%) of these were examined. Three artificial eyes, 16 eyes with corneal transplants, and 1 eye with a corneal limbal allograft were excluded from the study. All participating patients underwent clinical ophthalmologic examinations, including photographs, and their medical history was recorded. A slit lamp was used to examine the presence of keratopathy. RESULTS:: Visible keratopathy was found in 80% of the eyes. Keratopathy that caused impaired visual acuity was found in 26% of the eyes. The study displayed a significant correlation between the level of keratopathy and the patient's age. A significant correlation between the level of keratopathy and intraocular surgery exists. This is irrespective of the patient's age. The study also found that irrespective of the patient's age, a significant correlation between the level of keratopathy and impaired corneal sensitivity exists. CONCLUSIONS:: This research identified the presence of visible keratopathy in 80% of eyes. In addition, 26% of eyes had a keratopathy level that caused visual disturbances. The study showed that the prevalence and severity of keratopathy increased with the patient's age. Further conclusions are that intraocular surgery increases the severity of keratopathy and that the severity of keratopathy is correlated to reduced corneal sensitivity. Finally, extreme care should be taken when selecting patients for intraocular surgery because this procedure can trigger the development of keratopathy

Epidemiology of aniridia in Sweden and Norway.

Purpose: To investigate the epidemiology of aniridia in the populations of Sweden and Norway. Methods: A thorough search for aniridia patients has been performed in Sweden and Norway. All participants had a clinical ophthalmological examination documented through photography. Blood samples were taken for mutation analysis and pedigrees were established. Results: A total of 181 patients with aniridia were identified in the two countries. This gives an age-specific prevalence of 1:72 000 in the entire region, 1:70 000 in Sweden and 1:76 000 in Norway. A total of 124 individuals (69%) were examined. Male/female ratio was 0.94 (Sweden 0.85 and Norway 1.2). Mean age of the examined patients was 29 years and median age 25 years. We did not find any significant age difference between the two countries. The mean visual acuity (VA) was 0.19 (Sweden 0.19 and Norway 0.18).The number of families with more than one affected member was 31 and the number of sporadic cases was 40. Conclusion: We have done a thorough search of the literature, but we have found no earlier studies describing aniridia in an entire country and only a few reports from larger areas. We assume that most aniridia patients have been found and the aniridia prevalence of 1:72 000 can be regarded as well supported. Further studies on other aspects of aniridia are in progress, and information from these can contribute to guidelines for the care of patients with this rare but serious disease

Congenital Aniridia and the Ocular Surface

Aniridia is a congenital pan-ocular disorder caused by haplo-insufficiency of Pax6, a crucial gene for proper development of the eye. Aniridia affects a range of eye structures, including the cornea, iris, anterior chamber angle, lens, and fovea. The ocular surface, in particular, can be severely affected by a progressive pathology termed aniridia-associated keratopathy (AAK), markedly contributing to impaired vision. The purpose of this review is to provide an update of the current knowledge of the genetic, clinical, micro-morphological, and molecular aspects of AAK. We draw upon material presented in the literature and from our own observations in large aniridia cohorts. We summarize signs and symptoms of AAK, describe current options for management, and discuss the latest research findings that may lead to better diagnosis and new treatment or prevention strategies for this debilitating ocular surface condition.Funding Agencies|Ogonfonden; Swedish Research Council [2012-2472]; Country of Ostergotland; Kronprinsessan Margaretas Arbetsnamnd</p

Aniridia among children and teenagers in Sweden and Norway.

Purpose: To investigate patients under the age of 20 with aniridia in Sweden and Norway in order to estimate the prevalence of aniridia, to describe clinical signs and identify complications in the young, which will help improve diagnostic tools and treatment. Methods: A thorough search for patients with aniridia (of all ages) was performed. Sixty-two of the 181 patients were under the age of 20. Fifty-two of them were examined and they constituted the study population. Patient history was obtained and all participants underwent clinical ophthalmologic examination, including photography. Blood samples were taken for mutation analysis. Results: Epidemiological data are only based on the results in Sweden. The age-specific prevalence in Sweden was 1:47 000, male/female ratio was 0.57, mean age 12 years and median age 14 years. The proportion of sporadic cases including WAGR (Wilms tumour, Aniridia, Genitourinary abnormalities, Mental Retardation) and Gillespie syndrome (aniridia, cerebellar ataxia and mental retardation) was 48%. In the entire study population (Sweden and Norway), the mean visual acuity (VA) was 0.2 (range 0.04-0.9). We found VA < 0.3 in 80% and <0.1 in 18% of the patients. Twenty-two patients (42%) had one or more of the sight threatening complications such as cataract/lens luxation, corneal clouding or glaucoma. Conclusion: Descriptions of aniridia in the younger are rare. This study shows that aniridia seems to be more common than previously estimated and that some complications appear early in life. Watchfulness as regards these complications and regular examinations are essential even in the youngest

The Elusive Quest for a Constitutional Right to Liberty

Professor Michael S. Moore, Charles R. Walgreen, Jr. Chair and Co-Director, Program in Law and Philosophy at the University of Illinois College of Law, delivered Duke Law\u27s Annual Brainerd Currie Memorial Lecture entitled The Elusive Quest for a Constitutional Right to Liberty. One of the country\u27s most prominent authorities on the intersection of law and philosophy, he has published eight books and some 60 major articles, which have appeared in the country\u27s top law reviews and peer reviewed journals in philosophy and psychiatry. He is the author of Placing Blame, a General Theory of the Criminal Law (Oxford University Press, 1997), widely regarded as the leading modern statement of the retributivist theory of punishment and of that theory\u27s systematic application to criminal law doctrine. In Act and Crime: The Philosophy of Action and its Implications for Criminal Law (Oxford University Press, 1993), Moore provided a unified theory of action that underlies English and American criminal jurisprudence. Professor Moore has presented more than 150 lectures and papers in law, jurisprudence, political theory, legal philosophy, political science and economics, philosophy, psychology, and psychiatry. Sponsored by the Office of the Dean