Prader-Willi syndrome (PWS) is an unusual genetic disorder characterized by short stature, obesity, hypogonadism, hypotonia, cognitive impairment, and dysmorphic facies. There is an interstitial deletion of the proximal long arm of chromosome 15 in about 70 % of patients. Some of these clinical features suggest a central hypothalamic/pituitary dysfunction, and recent investigations have demonstrated a marked impairment in spontaneous growth hormone (GH) secretion. We studied 15 GH-deficient PWS patients by magnetic resonance imaging (MRI) to determine whether there was a diminution in the gross morphological size of the anterior pituitary gland, the site of GH synthesis. We also set out to catalog the pertinent imaging findings in this patient population. Our results indicate that this is the first report documenting pituitary size by MRI in PWS patients. No statistically significant difference was found in the height of the anterior pituitary gland in PWS patients compared with either normal children or children with isolated GH deficiency. An interesting imaging finding is that three of 15 patients (20 %) demonstrated complete absence of the posterior pituitary bright spot (PPBS), and a fourth patient demonstrated a small PPBS. These observations reflect an objective physiologic disturbance in the hypothalamus. The clinical and radiologic implications of these findings are discussed.
Le tourisme cultural amérindien est un secteur de L'industrie touristique canadienne potentiellement en forte croissance, qui connaît un vif succès auprès des visiteurs européens, surtout les Allemands. Le présent article recourt à L'analyse du discours pour examiner les rencontres touristiques qui se déroulent dans les différents lieux touristiques amérindiens du sud de L'Alberta. Il analyse la construction de L'«indianite» et de la culture amérindienne par les guides amérindiens et les visiteurs étrangers. Il appert que ces constructions sont façonnées par le discours primitiviste qui, ironiquement, renforce la notion de «noble sauvage» héritée des Lumières. Nous discutons L'idée selon laquelle le discours primitiviste, malgré ses aspects colonialistes et essentialistes, peut représenter une stratégie de résistance envers un système social perçu comme une source d'oppression par plusieurs Premières Nations.
Aboriginal cultural tourism is a potentially high‐growth segment of the Canadian tourism industry that is currently enjoying widespread demand among Europeans, especially German visitors. This paper uses a discourse analysis approach to examine the tourist encounter at various Aboriginal tourist sites in southern Alberta. It analyses the negotiation of “Indianness” and Indian culture by both Native interpreters and foreign visitors. These negotiations are shown to be informed by the primitivist discourse that, ironically, reinforces the Enlightenment notion of the “noble savage.” We argue that, despite its colonialist and essentialist aspects, the primitivist discourse can nevertheless function as a strategy of resistance to a social system viewed by many First Nations as politically oppressive.
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