Uusia ja uudistettuja katseita

Kirjallisuusarvostelu

[Clinton] Larsen, saut en hauteur [vainqueur des Jeux interalliés avec 1m86, stade Pershing, le 4 juillet 1919] : [photographie de presse] / [Agence Rol]

Référence bibliographique : Rol, 54703

Humanismo e Técnica segundo o Princípio Responsabilidade de Hans Jonas

Segundo Hans Jonas,a acção humana adquiriu um novo sentido. Ela tornou-se técnica, ou seja, a própria existência humana, como afirmara Heidegger, tornou-se uma dessiminação de relações técnicas: vivemos com a técnica, mas não da técnica. Este pensamento, que encontramos em Hans Jonas estava já presente na Meditação sobre a Técnica de Ortega y Gasset, cujas origens do pensamento sobre a técnica remontam a Oswald Spengler. A técnica como a táctica vital que visa diminuir os nossos esforços, mas será que nesse esforço não estamos a correr o risco de pôr em causa a própria humanidade?

Congenital joint dislocations caused by carbohydrate sulfotransferase 3 deficiency in recessive Larsen syndrome and humero-spinal dysostosis

Deficiency of carbohydrate sulfotransferase 3 (CHST3; also known as chondroitin-6-sulfotransferase) has been reported in a single kindred so far and in association with a phenotype of severe chondrodysplasia with progressive spinal involvement. We report eight CHST3 mutations in six unrelated individuals who presented at birth with congenital joint dislocations. These patients had been given a diagnosis of either Larsen syndrome (three individuals) or humero-spinal dysostosis (three individuals), and their clinical features included congenital dislocation of the knees, elbow joint dysplasia with subluxation and limited extension, hip dysplasia or dislocation, clubfoot, short stature, and kyphoscoliosis developing in late childhood. Analysis of chondroitin sulfate proteoglycans in dermal fibroblasts showed markedly decreased 6-O-sulfation but enhanced 4-O-sulfation, confirming functional impairment of CHST3 and distinguishing them from diastrophic dysplasia sulphate transporter (DTDST)-deficient cells. These observations provide a molecular basis for recessive Larsen syndrome and indicate that recessive Larsen syndrome, humero-spinal dysostosis, and spondyloepiphyseal dysplasia Omani type form a phenotypic spectrum.

Institution de Jonas, év. d'Orléans.

Ex-libris : f.1 : « Sti Germani a Pratis »

Espaces et sociétés : revue critique internationale de l'aménagement, de l'architecture et de l'urbanisation / dir. publ. Serge Jonas

1977/03 (N20)-1977/06 (N21).

Espaces et sociétés : revue critique internationale de l'aménagement, de l'architecture et de l'urbanisation / dir. publ. Serge Jonas

1998 (N95,T1).