Le mystère de la Passion / d'Arnoul Greban ; publié d'après les manuscrits de Paris, avec une introduction et un glossaire, par Gaston Paris et Gaston Raynaud

[Mystère de la Passion (français moyen). 1878]

Interplay between keratinocytes and immune cells--recent insights into psoriasis pathogenesis.

Psoriasis is one of the most common human inflammatory skin diseases characterised by hyperproliferation and aberrant differentiation of keratinocytes. The trigger of the typical epidermal changes seen in psoriasis was considered to be a dysregulated immune response with Th-1/Tc1 cells playing a central role. Recent studies have provided new insights into psoriasis pathogenesis in defining intraepidermal alpha(1)beta(1)+ T cells as key effectors driving keratinocyte changes. Critical roles for IFN-alpha secreted by plasmacytoid dendritic cells and the IL-23/Th-17 axis were postulated. Initially, these subsequent stages are at least partially driven by the endogenous antimicrobial peptide LL37 that converts inert self-DNA into a potent trigger of interferon production by binding and delivering the DNA into plasmacytoid dendritic cells to trigger toll-like receptor 9. As LL37 is expressed by keratinocytes upon various stimuli, keratinocytes might regain momentum as instigators of an aberrant immune response which then precedes the characteristic changes in the epidermis. Data from these new studies indicate a complex interplay between keratinocytes overexpressing antimicrobial peptides and immune cells driving epidermal hyperproliferation and aberrant keratinocyte differentiation in the pathogenesis of psoriasis.

Fourteen new cases contribute to the characterization of the 7q11.23 microduplication syndrome.

Interstitial deletions of 7q11.23 cause Williams-Beuren syndrome, one of the best characterized microdeletion syndromes. The clinical phenotype associated with the reciprocal duplication however is not well defined, though speech delay is often mentioned. We present 14 new 7q11.23 patients with the reciprocal duplication of the Williams-Beuren syndrome critical region, nine familial and five de novo. These were identified by either array-based MLPA or by array-CGH/oligonucleotide analysis in a series of patients with idiopathic mental retardation with an estimated population frequency of 1:13,000-1:20,000. Variable speech delay is a constant finding in our patient group, confirming previous reports. Cognitive abilities range from normal to moderate mental retardation. The association with autism is present in five patients and in one father who also carries the duplication. There is an increased incidence of hypotonia and congenital anomalies: heart defects (PDA), diaphragmatic hernia, cryptorchidism and non-specific brain abnormalities on MRI. Specific dysmorphic features were noted in our patients, including a short philtrum, thin lips and straight eyebrows. Our patient collection demonstrates that the 7q11.23 microduplication not only causes language delay, but is also associated with congenital anomalies and a recognizable face.

[Georges] Thibout, [Louis] Lajarrige, [Constant] Pilate, députés de Paris : [photographie de presse] / [Agence Rol]

Référence bibliographique : Rol, 56972

De Radbruch a Alexy: derecho injusto y racionalidad

Aquest treball analitza la connexió entre Gustav RADBRUCH i Robert ALEXY des de la perspectiva de la doctrina del dret injust del primer d’aquets autors, exposada sintèticament en el que s’ha anomenat “fórmula de RADBRUCH”. Nogensmenys, els dos autors pertanyen a corrents iusfilosofiques certament diferenciables. Per això, aquet treball pretén trobar en el significat profund de la fórmula un canvi cognitiu respecte de lo jurídic que permeti vehicular la relació entre els dos autors. Serveix de suport tant la teoria filosòfica en la que fonamenti el sistema de cadascú dels autors, com les circumstàncies sociopolítiques en las que es ofereix cadascú dels sistemes.

2q34-qter duplication and 4q34.2-qter deletion in a patient with developmental delay.

The 2q3 duplication and 4q3 deletion syndromes are two conditions with variable phenotypes including Pierre-Robin sequence (PRS), limb anomalies, congenital heart defects (CHD), developmental delays and intellectual disabilities. We describe a patient born to a mother with a balanced t(2; 4) translocation who combines both a 2q34-qter duplication and a 4q34.2-qter deletion through inheritance of the derivative chromosome 4 (der(4)). He showed developmental delay, growth retardation, hearing problems, minor facial and non-facial anomalies, such as bilateral fifth finger shortness and clinodactyly, but no PRS or CHD. The comparison of his features with those of 46 and 65 published cases of 2q3 duplication and 4q3 deletion, respectively, allows us to further restrict the size of the proposed critical intervals for PRS and CHD on chromosome 4.

Moro-Giafferi, [Jean] Erlich [16-1-20, cour du Sénat, vote préliminaire pour l'élection du président de la République] : [photographie de presse] / [Agence Rol]

Référence bibliographique : Rol, 57661

Première réunion de la ligue des nations à Londres, Albert Thomas et M. [Léon] Bourgeois au Palais St James : [photographie de presse] / [Agence Rol]

Référence bibliographique : Rol, 57999

17-1-20, Congrès de Versailles, M. Albert Thomas : [photographie de presse] / [Agence Rol]

Référence bibliographique : Rol, 57686