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Context: 4H or POLR3-related leukodystrophy is an autosomal recessive disorder typically characterized by hypomyelination, hypodontia, and hypogonadotropic hypogonadism, caused by biallelic pathogenic variants in POLR3A, POLR3B, POLR1C, and POLR3K. The endocrine and growth abnormalities associated with this disorder have not been thoroughly investigated to date. Objective: To systematically characterize endocrine abnormalities of patients with 4H leukodystrophy. Design: An international cross-sectional study was performed on 150 patients with genetically confirmed 4H leukodystrophy between 2015 and 2016. Endocrine and growth abnormalities were evaluated, and neurological and other non-neurological features were reviewed. Potential genotype/phenotype associations were also investigated. Setting: This was a multicenter retrospective study using information collected from 3 predominant centers. Patients: A total of 150 patients with 4H leukodystrophy and pathogenic variants in POLR3A, POLR3B, or POLR1C were included. Main Outcome Measures: Variables used to evaluate endocrine and growth abnormalities included pubertal history, hormone levels (estradiol, testosterone, stimulated LH and FSH, stimulated GH, IGF-I, prolactin, ACTH, cortisol, TSH, and T4), and height and head circumference charts. Results: The most common endocrine abnormalities were delayed puberty (57/74; 77% overall, 64% in males, 89% in females) and short stature (57/93; 61%), when evaluated according to physician assessment. Abnormal thyroid function was reported in 22% (13/59) of patients. Conclusions: Our results confirm pubertal abnormalities and short stature are the most common endocrine features seen in 4H leukodystrophy. However, we noted that endocrine abnormalities are typically underinvestigated in this patient population. A prospective study is required to formulate evidence-based recommendations for management of the endocrine manifestations of this disorder. © 2020 The Author(s) 2020. Published by Oxford University Press on behalf of the Endocrine Society.

In 1981, the National Research Council of Canada (NRC) commenced research on the development of 3D laser scanner imaging technology. Initially it was thought that development would be driven primarily by industrial applications. However, in the past decade, the cultural sector has become one of the main driving forces. The purpose of this paper is to present an overview of some of the cultural applications developed during the past decade that has helped drive the development of the technology., Electronic Imaging and the Visual Arts, The Fifth International Conference, EVA 2002 Moscow, July 25 2002, Farnborough, England

Depuis son ouverture en 1989, le Centre de bouturage de la Pépinière de Saint-Modeste ne cesse d'augmenter et de diversifier sa production, qui se chiffre maintenant à environ quatre millions de plants issus de boutures livrés annuellement. Les recherches menées au Ministère des Ressources Naturelles du Québec ont permis de développer des techniques uniques de bouturage pour la multiplication en vrac, à grande échelle, de plusieurs espèces d'arbres forestiers concernées par des programmes d'amélioration génétique. Les espèces multipliées par bouturage sont l'épinette noire (Picea mariana), l'épinette blanche (Picea glauca), l'épinette de Norvège (Picea abies) et depuis peu, les mélèzes hybrides (Larix x eurolepis et autres). Chacune possède des particularités propres qui déterminent le choix du calendrier de production et du système de multiplication à mettre en place. Un souci constant d'amélioration et une collaboration étroite entre la Pépinière de Saint-Modeste et la Direction de la Recherche Forestière sont parmi les clés de ce succès. Nous résumons ici nos techniques, ainsi que la démarche de recherche qui nous a permis d'atteindre les niveaux actuels de production.

This paper presents our activities on smart VLSI opto-sensors for 3D vision. A description of the integrated devices jointly developed for industrial and scientific applications is given. All the sensors presented here have been fabricated using standard CMOS technology that allows the monolithic integration of photo-sensors, together with readout circuits, and digital signal processors., The 18th IEEE Instrumentation and Measurement Technology Conference. Rediscovering Measurement in the Age of Informatics, 21-23 May 2001, Budapest, Hungary, Series: IEEE Instrumentation and Measurement Technology Conference Proceedings