[Therapeutic Embolization of a Pulmonary Arteriovenous-fistula in a Child]

Background. - Pulmonary arteriovenous fistula is a rare vascular abnormality in children that is usually treated by lobectomy. This report describes a case that was improved by coil embolization. Case report. - A thoracic X-ray was routinely taken before anygdalectomy in a 6 year-old girl. It showed a consolidation area in the left superior lobe that persisted despite treatment with erythromycin for 3 weeks. Bronchoscopy was normal, but study of blood gases showed a refractory hypoxemia cyanosis and hemoglobin at 16/2 g/dl. Right-heat catheterization and angiography showed a pulmonary arteriovenous fistula that was treated by coil embolization. A control investigation 3 months later showed normal arterial oxygen saturation, hemoglobin at 14.5 g/dl and reduced pulmonary condensation. Conclusion. - Pulmonary arteriovenous fistula may show few symptoms. Coil embolization appears to be a useful initial non-aggressive treatment

Embolisation percutanée d'une fistule artérioveineuse pulmonaire chez un enfant.

BACKGROUND--Pulmonary arteriovenous fistula is a rare vascular abnormality in children that is usually treated by lobectomy. This report describes a case that was improved by coil embolization. CASE REPORT--A thoracic X-ray was routinely taken before amygdalectomy in a 6 year-old girl. It showed a consolidation area in the left superior lobe that persisted despite treatment with erythromycin for 3 weeks. Bronchoscopy was normal, but study of blood gases showed a refractory hypoxemia with moderate cyanosis and hemoglobin at 16.2 g/dl. Right-heart catheterization and angiography showed a pulmonary arteriovenous fistula that was treated by coil embolization. A control investigation 3 months later showed normal arterial oxygen saturation, hemoglobin at 14.5 g/dl and reduced pulmonary condensation. CONCLUSION--Pulmonary arteriovenous fistula may show few symptoms. Coil embolization appears to be a useful initial non-aggressive treatment

An Rpb4/Rpb7-Like Complex in Yeast RNA Polymerase III Contains the Orthologue of Mammalian CGRP-RCP

The essential C17 subunit of yeast RNA polymerase (Pol) III interacts with Brf1, a component of TFIIIB, suggesting a role for C17 in the initiation step of transcription. The protein sequence of C17 (encoded by RPC17) is conserved from yeasts to humans. However, mammalian homologues of C17 (named CGRP-RCP) are known to be involved in a signal transduction pathway related to G protein-coupled receptors, not in transcription. In the present work, we first establish that human CGRP-RCP is the genuine orthologue of C17. CGRP-RCP was found to functionally replace C17 in Δrpc17 yeast cells; the purified mutant Pol III contained CGRP-RCP and had a decreased specific activity but initiated faithfully. Furthermore, CGRP-RCP was identified by mass spectrometry in a highly purified human Pol III preparation. These results suggest that CGRP-RCP has a dual function in mammals. Next, we demonstrate by genetic and biochemical approaches that C17 forms with C25 (encoded by RPC25) a heterodimer akin to Rpb4/Rpb7 in Pol II. C17 and C25 were found to interact genetically in suppression screens and physically in coimmunopurification and two-hybrid experiments. Sequence analysis and molecular modeling indicated that the C17/C25 heterodimer likely adopts a structure similar to that of the archaeal RpoE/RpoF counterpart of the Rpb4/Rpb7 complex. These RNA polymerase subunits appear to have evolved to meet the distinct requirements of the multiple forms of RNA polymerases

An in vivo genetic reversion highlights the crucial role of Myb-Like, SWIRM, and MPN domains 1 (MYSM1) in human hematopoiesis and lymphocyte differentiation

International audienceMyb-Like, SWIRM, and MPN domains 1(MYSM1) is a metalloprotease that deubiquitinates the K119-monoubiquitinated form of histone 2A (H2A), a chromatinmarker associated with gene transcription silencing. Likewise, ithas been reported that murine Mysm1 participates intranscription derepression of genes, among which aretranscription factors involved in hematopoietic stem cellhomeostasis, hematopoiesis, and lymphocyte differentiation.However, whether MYSM1 has a similar function in humansubjects remains unclear. Here we describe a patient presentingwith a complete lack of B lymphocytes, T-cell lymphopenia,defective hematopoiesis, and developmental abnormalities.Objectives: We sought to characterize the underlying geneticcause of this syndrome.Methods: We performed genome-wide homozygosity mapping,followed by whole-exome sequencing.Results: Genetic analysis revealed that this novel disorder iscaused by a homozygous MYSM1 missense mutation affectingthe catalytic site within the deubiquitinase JAB1/MPN/Mov34(JAMM)/MPN domain. Remarkably, during the course of ourstudy, the patient recovered a normal immunohematologicphenotype. Genetic analysis indicated that this improvementoriginated from a spontaneous genetic reversion of the MYSM1mutation in a hematopoietic stem cell.Conclusions: We here define a novel human immunodeficiencyand provide evidence that MYSM1 is essential for properimmunohematopoietic development in human subjects. Inaddition, we describe one of the few examples of spontaneousin vivo genetic cure of a human immunodeficiency

Prédication en chapitre général et réforme pontificale de la vita religiosa au XIIIe siècle. À propos des sermons aux chapitres généraux des moines noirs d’Eudes de Châteauroux et Jacques de Furnes

International audienceÀ partir de l’analyse de sermons respectivement donnés par Eudes de Châteauroux et Jacques de Furnes aux chapitres généraux des moines noirs des provinces de Sens et de Reims, l’occasion est offerte de confronter le point de vue de deux acteurs (un gradué séculier ayant la faveur de la papauté et un abbé bénédictin au cœur de la mêlée) sur les réformes monastiques du pape Grégoire IX (1235-1237) et de tenter de cerner les stratégies discursives et les méthodes de gouvernement mises en œuvre par la papauté dans son dessein global de contrôle centralisé de tous les membres de l’Ecclesia. Un sermon de chaque prédicateur est édité en annex