Pour l'étude de la guerre 1936-1939 : initiatives récentes

Broue Pierre. Pour l'étude de la guerre 1936-1939 : initiatives récentes. In: Matériaux pour l'histoire de notre temps, n°3-4, 1985. L'Espagne, 1900-1985, sous la direction de Stéphane Courtois. pp. 64-68

Diagnostic et devenir du syndrome d'Alagille (étude rétrospective à partir de 17 cas)

TOULOUSE3-BU Santé-Centrale (315552105) / SudocPARIS-BIUM (751062103) / SudocSudocFranceF

Traitement anténatal de l'hémochromatose périnatale par perfusions maternelles d'immunoglobulines polyvalentes (l'expérience française en 2008)

L'hémochromatose périnatale (HP) est un syndrome associant une insuffisance hépatocellulaire néonatale et une surcharge en fer extrahépatique. Un décès ou une transplantation hépatique néonatale surviennent dans la majorité des cas. En 2004, Whitington montre l'amélioration du pronostic vital des nouveau-nés nés de mères ayant des antécédents obstétricaux de grossesses compliquées d'HP et traitées par perfusions hebdomadaires d'immunoglobulines polyvalentes (IgP) à partir de la 20e semaine d'aménorrhée (SA). Ce travail reprend les huit grossesses traitées en France depuis 2004 et confirme l'amélioration considérable du pronostic vital puisque tous les nouveau-nés ont survécu sans transplantation hépatique. Le recours au collège national d'experts pour sélectionner les grossesses à traiter est recommandé. La validation de l'indication des IgP par l'agence française de sécurité sanitaire des produits de santé (AFSSAPS) permettrait une meilleure prise en charge des patientes.TOULOUSE3-BU Santé-Centrale (315552105) / SudocPARIS-BIUM (751062103) / SudocSudocFranceF

Impact of age at Kasai operation on its results in late childhood and adolescence: a rational basis for biliary atresia screening

BACKGROUND: Increased age at surgery has a negative impact on results of the Kasai operation for biliary atresia in infancy and early childhood. It remained unclear if an age threshold exists and if this effect persists with extended follow-up. In this study we examined the relationship between increased age at surgery and its results in adolescence. METHODS: All patients with biliary atresia who were living in France and born between 1986 and 2002 were included. Median follow-up in survivors was 7 years. RESULTS: Included in the study were 743 patients with biliary atresia, 695 of whom underwent a Kasai operation; 2-, 5-, 10-, and 15-year survival rates with native liver were 57.1%, 37.9%, 32.4%, and 28.5%, respectively. Median age at Kasai operation was 60 days and was stable over the study period. Whatever the follow-up (2, 5, 10, or 15 years), survival rates with native liver decreased when age at surgery increased ( ≤ 30, 31-45, 46-60, 61-75, and 76-90 days). Accordingly, we estimated that if every patient with biliary atresia underwent the Kasai operation before 46 days of age, 5.7% of all liver transplantations performed annually in France in patients younger than 16 years could be spared. CONCLUSIONS: Increased age at surgery had a progressive and sustained deleterious effect on the results of the Kasai operation until adolescence. These findings indicate a rational basis for biliary atresia screening to reduce the need for liver transplantations in infancy and childhood

Heterogeneity of follow-up procedures in French and Belgian patients with treated hereditary tyrosinemia type 1: results of a questionnaire and proposed guidelines.

The 1991 introduction of 2-(2-nitro-4-trifluoro-methylbenzyol)-1,3 cyclohexanedione (NTBC) as a treatment for hereditary tyrosinemia type 1 (HT-1), a disorder of tyrosine catabolism, has radically modified the natural history of this disorder. Despite the dramatic improvements in survival, outcomes and quality of life seen with NTBC treatment, HT-1 remains a chronic disorder with several long-term complications, including, a persistent (albeit low) risk of hepatocellular carcinoma and suboptimal neuropsychological outcomes. There remain unsolved key-questions concerning the long-term outcomes of patients with HT-1, which closely depend on the quality of follow-up in these patients. In the absence of published guidelines, we investigated the follow-up methods used for French and Belgian patients with HT-1. A simple questionnaire providing a rapid overview of follow-up procedures was sent to the 19 physicians in charge of HT-1 patients treated with NTBC and low-tyrosine diet in France and Belgium. Several areas of heterogeneity (especially liver imaging, slit lamp examination, neuropsychological evaluation and maximal plasma tyrosine level accepted) were observed. In an attempt to improve long-term management and outcome of patients with HT-1, we proposed follow-up recommendations

Building blocks for a Ka band switch matrix using MEMS and printed circuit board technology

International audienceThe key objectives of the SMARTIS (SMART thin film on alumina Substrate) project are presented in this paper. A focus is made on the design of the DPDT switches and on the optimization of the elementary building blocks necessary for the development of a Ka band switch matrix on Printed Circuit Board

Heterogeneity of follow-up procedures in French and Belgian patients with treated hereditary tyrosinemia type 1: Results of a questionnaire and proposed guidelines

The 1991 introduction of 2-(2-nitro-4-trifluoromethylbenzyol)-1, 3 cyclohexanedione (NTBC) as a treatment for hereditary tyrosinemia type 1 (HT-1), a disorder of tyrosine catabolism, has radically modified the natural history of this disorder. Despite the dramatic improvements in survival, outcomes and quality of life seen with NTBC treatment, HT-1 remains a chronic disorder with several long-term complications, including, a persistent (albeit low) risk of hepatocellular carcinoma and suboptimal neuropsychological outcomes. There remain unsolved keyquestions concerning the long-term outcomes of patients with HT-1, which closely depend on the quality of followup in these patients. In the absence of published guidelines, we investigated the follow-up methods used for French and Belgian patients with HT-1. A simple questionnaire providing a rapid overview of follow-up procedures was sent to the 19 physicians in charge of HT-1 patients treated with NTBC and low-tyrosine diet in France and Belgium. Several areas of heterogeneity (especially liver imaging, slit lamp examination, neuropsychological evaluation and maximal plasma tyrosine level accepted) were observed. In an attempt to improve long-term management and outcome of patients with HT-1, we proposed follow-up recommendations. © SSIEM and Springer 2011.SCOPUS: ar.jinfo:eu-repo/semantics/publishe

Targeted-Capture Next-Generation Sequencing in Diagnosis Approach of Pediatric Cholestasis

BACKGROUND: Cholestasis is a frequent and severe condition during childhood. Genetic cholestatic diseases represent up to 25% of pediatric cholestasis. Molecular analysis by targeted-capture next generation sequencing (NGS) has recently emerged as an efficient diagnostic tool. The objective of this study is to evaluate the use of NGS in children with cholestasis. METHODS: Children presenting cholestasis were included between 2015 and 2020. Molecular sequencing was performed by targeted capture of a panel of 34 genes involved in cholestasis and jaundice. Patients were classified into three categories: certain diagnosis; suggested diagnosis (when genotype was consistent with phenotype for conditions without any available OMIM or ORPHANET-number); uncertain diagnosis (when clinical and para-clinical findings were not consistent enough with molecular findings). RESULTS: A certain diagnosis was established in 169 patients among the 602 included (28.1%). Molecular studies led to a suggested diagnosis in 40 patients (6.6%) and to an uncertain diagnosis in 21 patients (3.5%). In 372 children (61.7%), no molecular defect was identified. CONCLUSIONS: NGS is a useful diagnostic tool in pediatric cholestasis, providing a certain diagnosis in 28.1% of the patients included in this study. In the remaining patients, especially those with variants of uncertain significance, the imputability of the variants requires further investigations