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1. Moving the Dial on Airway Inflammation in Response to Trikafta in Adolescents with Cystic Fibrosis

Author

Agathe, Lepissier, Anne Sophie, Bonnel, Nathalie, Wizla, Laurence, Weiss, Marie, Mittaine, Katia, Bessaci, Eitan, Kerem, Véronique, Houdouin, Philippe, Reix, Christophe, Marguet, Isabelle, Sermet-Gaudelus, Institut Necker Enfants-Malades (INEM - UM 111 (UMR 8253 / U1151)), Institut National de la Santé et de la Recherche Médicale (INSERM)-Centre National de la Recherche Scientifique (CNRS)-Université Paris Cité (UPCité), Centre de Référence Maladies Rares, Mucoviscidose et maladies apparentées (CRMR2MA / CHU Necker - Enfants Malades [AP-HP]), CHU Necker - Enfants Malades [AP-HP], Assistance publique - Hôpitaux de Paris (AP-HP) (AP-HP)-Assistance publique - Hôpitaux de Paris (AP-HP) (AP-HP), Hôpital Jeanne de Flandres, Université de Lille, Droit et Santé-Centre Hospitalier Régional Universitaire [Lille] (CHRU Lille), Hôpital de Hautepierre [Strasbourg], Service Pneumologie et allergologie pédiatrique [CHU Toulouse], Pôle Enfants [CHU Toulouse], Centre Hospitalier Universitaire de Toulouse (CHU Toulouse)-Centre Hospitalier Universitaire de Toulouse (CHU Toulouse), Service de pédiatrie générale et spécialisée [CHU de Reims - American Memorial Hospital] (SPGS), Centre Hospitalier Universitaire de Reims (CHU Reims)-American Memorial Hospital (Reims), Hadassah Hebrew University Medical Center [Jerusalem], Centre de ressources et de compétences pour la mucoviscidose [Debré], AP-HP Hôpital universitaire Robert-Debré [Paris], Université Paris Cité (UPCité), Hospices Civils de Lyon (HCL), Laboratoire de Biométrie et Biologie Evolutive - UMR 5558 (LBBE), Université Claude Bernard Lyon 1 (UCBL), Université de Lyon-Université de Lyon-Institut National de Recherche en Informatique et en Automatique (Inria)-VetAgro Sup - Institut national d'enseignement supérieur et de recherche en alimentation, santé animale, sciences agronomiques et de l'environnement (VAS)-Centre National de la Recherche Scientifique (CNRS), Groupe de Recherche sur le Handicap Ventilatoire (GRHV), CHU Rouen, Normandie Université (NU)-Normandie Université (NU)-Université de Rouen Normandie (UNIROUEN), Normandie Université (NU)-Institute for Research and Innovation in Biomedicine (IRIB), Université de Rouen Normandie (UNIROUEN), Normandie Université (NU)-Normandie Université (NU)-Institut National de la Santé et de la Recherche Médicale (INSERM)-Centre National de la Recherche Scientifique (CNRS)-Université de Rouen Normandie (UNIROUEN), Normandie Université (NU)-Institut National de la Santé et de la Recherche Médicale (INSERM)-Centre National de la Recherche Scientifique (CNRS), Dynamique Microbienne associée aux Infections Urinaires et Respiratoires (DYNAMICURE), Université de Caen Normandie (UNICAEN), Normandie Université (NU)-Institut National de la Santé et de la Recherche Médicale (INSERM), Goethe-University Frankfurt am Main, and dormoy, valerian

Subjects
[SDV] Life Sciences [q-bio], Pulmonary and Respiratory Medicine, Cystic Fibrosis, [SDV]Life Sciences [q-bio], Biomarker, Inflammation, Critical Care and Intensive Care Medicine, CFTR modulator
Abstract

International audience; No abstract available

Published
2023
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2. Tranexamic acid dose–response relationship for antifibrinolysis in postpartum haemorrhage during Caesarean delivery: TRACES, a double-blind, placebo-controlled, multicentre, dose-ranging biomarker study

Author

Anne-Sophie Ducloy-Bouthors, Sixtine Gilliot, Maeva Kyheng, David Faraoni, Alexandre Turbelin, Hawa Keita-Meyer, Agnès Rigouzzo, Gabriela Moyanotidou, Benjamin Constant, Francoise Broisin, Agnès L. Gouez, Rémi Favier, Edith Peynaud, Louise Ghesquiere, Gilles Lebuffe, Alain Duhamel, Delphine Allorge, Sophie Susen, Benjamin Hennart, Emmanuelle Jeanpierre, Pascal Odou, Cyril Huissoud, Charles Garabedian, Fanny Lassalle, Frederic J. Mercier, Catherine Barre-Drouard, Max Gonzalez Estevez, Julien Corouge, Anne-Sophie Baptiste, Anne-Frédérique Dalmas, Pierre Richart, Groupe de Recherche sur les formes Injectables et les Technologies Associées - ULR 7365 (GRITA), Université de Lille-Centre Hospitalier Régional Universitaire [Lille] (CHRU Lille), Hôpital Jeanne de Flandre [Lille], Service de Biostatistiques [CHRU Lille], Centre Hospitalier Régional Universitaire [Lille] (CHRU Lille), Evaluation des technologies de santé et des pratiques médicales - ULR 2694 (METRICS), Hôpital Jeanne de Flandres, Université de Lille, Droit et Santé-Centre Hospitalier Régional Universitaire [Lille] (CHRU Lille), Toxicologie et Génopathies [CHRU Lille], Pôle de Biologie Pathologie Génétique [CHU Lille], Centre Hospitalier Régional Universitaire [Lille] (CHRU Lille)-Centre Hospitalier Régional Universitaire [Lille] (CHRU Lille), Université de Lille, CHU Lille, Groupe de Recherche sur les formes Injectables et les Technologies Associées - ULR 7365 [GRITA], Evaluation des technologies de santé et des pratiques médicales - ULR 2694 [METRICS], Groupe de Recherche sur les formes Injectables et les Technologies Associées (GRITA) - ULR 7365, Récepteurs nucléaires, Maladies Cardiovasculaires et Diabète (EGID) - U1011, Centre Hospitalier Régional Universitaire [Lille] [CHRU Lille], and Récepteurs nucléaires, maladies cardiovasculaires et diabète - U 1011 [RNMCD]

Subjects
Anesthesiology and Pain Medicine, Tranexamic Acid, Double-Blind Method, Pregnancy, Cesarean Section, [SDV]Life Sciences [q-bio], Postpartum Hemorrhage, Humans, Female, Fibrinolysin, Blood Coagulation Disorders, Antifibrinolytic Agents, Biomarkers
Abstract

International audience; BackgroundThe optimal dose of tranexamic acid to inhibit hyperfibrinolysis in postpartum haemorrhage is unclear. Tranexamic Acid to Reduce Blood Loss in Hemorrhagic Cesarean Delivery (TRACES) was a double-blind, placebo-controlled, randomised, multicentre dose-ranging study to determine the dose–effect relationship for two regimens of intravenous tranexamic acid vs placebo.MethodsWomen experiencing postpartum haemorrhage during Caesarean delivery were randomised to receive placebo (n=60), tranexamic acid 0.5 g (n=57), or tranexamic acid 1 g i.v. (n=58). Biomarkers of fibrinolytic activation were assayed at five time points, with inhibition of hyperfibrinolysis defined as reductions in the increase over baseline in D-dimer and plasmin–antiplasmin levels and in the plasmin peak time.ResultsIn the placebo group, hyperfibrinolysis was evidenced by a mean increase over baseline [95% confidence interval] of 93% [68–118] for D-dimer level at 120 min and 56% [25–87] for the plasmin–antiplasmin level at 30 min. A dose of tranexamic acid 1 g was associated with smaller increases over baseline (D-dimers: 38% [13–63] [P=0.003 vs placebo]; plasmin–antiplasmin: –2% [–32 to 28] [P=0.009 vs placebo]). A dose of tranexamic acid 0.5 g was less potent, with non-significant reductions (D-dimers: 58% [32–84] [P=0.06 vs placebo]; plasmin–antiplasmin: 13% [18–43] [P=0.051]). Although both tranexamic acid doses reduced the plasmin peak, reduction in plasmin peak time was significant only for the 1 g dose of tranexamic acid.ConclusionsFibrinolytic activation was significantly inhibited by a dose of intravenous tranexamic acid 1 g but not 0.5 g. Pharmacokinetic–pharmacodynamic modelling of these data might identify the best pharmacodynamic monitoring criteria and the optimal tranexamic acid dosing regimen for treatment of postpartum haemorrhage.

Published
2022
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3. Do Surgeons Anticipate Women’s Hopes and Fears Associated with Prolapse Repair? A Qualitative Analysis in the PROSPERE Trial

Author

Fritel, Xavier, Ravit, Marion, Pizzoferrato, Anne-Cécile, Campagne-Loiseau, Sandrine, Bader, Georges, Capmas, Perrine, Cosson, Michel, Debodinance, Philippe, Deffieux, Xavier, Fernandez, Hervé, Ferry, Philippe, Garbin, Olivier, Jacquetin, Bernard, Legendre, Guillaume, Saussine, Christian, Tayrac, Renaud de, Wagner, Laurent, Lucot, Jean-Philippe, Fauconnier, Arnaud, team, the PROSPERE team the PROSPERE, CIC - Poitiers, Université de Poitiers-Centre hospitalier universitaire de Poitiers (CHU Poitiers)-Direction Générale de l'Organisation des Soins (DGOS)-Institut National de la Santé et de la Recherche Médicale (INSERM), Centre hospitalier universitaire de Poitiers (CHU Poitiers), Liverpool School of Tropical Medicine (LSTM), CHU Estaing [Clermont-Ferrand], CHU Clermont-Ferrand, CMC Ambroise Paré [Neuilly-sur-Seine], Centre de recherche en épidémiologie et santé des populations (CESP), Université de Versailles Saint-Quentin-en-Yvelines (UVSQ)-Assistance publique - Hôpitaux de Paris (AP-HP) (AP-HP)-Hôpital Paul Brousse-Institut National de la Santé et de la Recherche Médicale (INSERM)-Université Paris-Saclay, AP-HP Hôpital Bicêtre (Le Kremlin-Bicêtre), Université de Lille, Hôpital Jeanne de Flandres, Université de Lille, Droit et Santé-Centre Hospitalier Régional Universitaire [Lille] (CHRU Lille), CH Dunkerque, AP-HP - Hôpital Antoine Béclère [Clamart], Assistance publique - Hôpitaux de Paris (AP-HP) (AP-HP), Université Paris-Sud - Paris 11 (UP11), Groupe hospitalier de La Rochelle, CHU Strasbourg, Université de Strasbourg (UNISTRA), Centre Hospitalier Universitaire d'Angers (CHU Angers), PRES Université Nantes Angers Le Mans (UNAM), Institut de recherche en santé, environnement et travail (Irset), Université d'Angers (UA)-Université de Rennes (UR)-École des Hautes Études en Santé Publique [EHESP] (EHESP)-Institut National de la Santé et de la Recherche Médicale (INSERM)-Structure Fédérative de Recherche en Biologie et Santé de Rennes ( Biosit : Biologie - Santé - Innovation Technologique ), Hôpital Universitaire Carémeau [Nîmes] (CHU Nîmes), Centre Hospitalier Universitaire de Nîmes (CHU Nîmes), Université de Montpellier (UM), Hôpital Saint Vincent de Paul de Lille, Groupement des Hôpitaux de l'Institut Catholique de Lille (GHICL), Université catholique de Lille (UCL)-Université catholique de Lille (UCL), Université Paris-Saclay, and CHI Poissy-Saint-Germain

Subjects
surgery, [SDV]Life Sciences [q-bio], hope, fear, General Medicine, pelvic organ prolapse, expectations
Abstract

Women’s preoperative perceptions of pelvic-floor disorders may differ from those of their physicians. Our objective was to specify women’s hopes and fears before cystocele repair, and to compare them to those that surgeons anticipate. We performed a secondary qualitative analysis of data from the PROSPERE trial. Among the 265 women included, 98% reported at least one hope and 86% one fear before surgery. Sixteen surgeons also completed the free expectations-questionnaire as a typical patient would. Women’s hopes covered seven themes, and women’s fears eleven. Women’s hopes were concerning prolapse repair (60%), improvement of urinary function (39%), capacity for physical activities (28%), sexual function (27%), well-being (25%), and end of pain or heaviness (19%). Women’s fears were concerning prolapse relapse (38%), perioperative concerns (28%), urinary disorders (26%), pain (19%), sexual problems (10%), and physical impairment (6%). Surgeons anticipated typical hopes and fears which were very similar to those the majority of women reported. However, only 60% of the women reported prolapse repair as an expectation. Women’s expectations appear reasonable and consistent with the scientific literature on the improvement and the risk of relapse or complication related to cystocele repair. Our analysis encourages surgeons to consider individual woman’s expectations before pelvic-floor repair.

Published
2023
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4. Comparison of Endoscopic Dilatation and Heller's Myotomy for Treating Esophageal Achalasia in Children: A Multicenter Study

Author

Audrey Nicolas, Madeleine Aumar, Léa Chantal Tran, Alice Tiret, Rémi Duclaux-Loras, Laure Bridoux-Henno, Florence Campeotto, Alexandre Fabre, Anne Breton, Jeanne Languepin, Maéva Kyheng, Jérôme Viala, Stéphanie Coopman, Frédéric Gottrand, CHU Limoges, Centre Hospitalier Régional Universitaire [Lille] (CHRU Lille), Institute for Translational Research in Inflammation - U 1286 (INFINITE (Ex-Liric)), Institut National de la Santé et de la Recherche Médicale (INSERM)-Université de Lille-Centre Hospitalier Régional Universitaire [Lille] (CHRU Lille), AP-HP Hôpital universitaire Robert-Debré [Paris], Assistance publique - Hôpitaux de Paris (AP-HP) (AP-HP), Hospices Civils de Lyon (HCL), Hôpital Sud [CHU Rennes], CHU Pontchaillou [Rennes], CHU Necker - Enfants Malades [AP-HP], Hôpital de la Timone [CHU - APHM] (TIMONE), Génétique Médicale et Génomique Fonctionnelle (GMGF), Aix Marseille Université (AMU)-Assistance Publique - Hôpitaux de Marseille (APHM)- Hôpital de la Timone [CHU - APHM] (TIMONE)-Institut National de la Santé et de la Recherche Médicale (INSERM)-Centre National de la Recherche Scientifique (CNRS), Université Toulouse III Paul Sabatier - Faculté de médecine Purpan (UTPS), Université Toulouse III - Paul Sabatier (UT3), Université Fédérale Toulouse Midi-Pyrénées-Université Fédérale Toulouse Midi-Pyrénées, Evaluation des technologies de santé et des pratiques médicales - ULR 2694 (METRICS), Université de Lille-Centre Hospitalier Régional Universitaire [Lille] (CHRU Lille), Leroy, Mélanie, Université de Toulouse (UT)-Université de Toulouse (UT), Université de Lille, Inserm, CHU Lille, Centre Hospitalier Régional Universitaire [Lille] [CHRU Lille], Institut de Recherche Translationnelle sur l'Inflammation (INFINITE) - U1286, Institute for Translational Research in Inflammation - U 1286 [INFINITE (Ex-Liric)], Service d'Hépatologie et de Gastroentérologie [Lyon], Hôpital de la Timone [CHU - APHM] [TIMONE], Génétique Médicale et Génomique Fonctionnelle [GMGF], Université Toulouse III - Paul Sabatier [UT3], Evaluation des technologies de santé et des pratiques médicales - ULR 2694 [METRICS], and Hôpital Jeanne de Flandres

Subjects
Male, [SDV.MHEP.PED]Life Sciences [q-bio]/Human health and pathology/Pediatrics, Achalasia, Endoscopy, Achalasia Endoscopic dilatation Heller's myotomy Children, [SDV.MHEP.HEG]Life Sciences [q-bio]/Human health and pathology/Hépatology and Gastroenterology, Heller Myotomy, Endoscopic dilatation, Dilatation, Child, Humans, Female, Esophageal Achalasia, Retrospective Studies, Heller's myotomy, achalasia, children, endoscopic dilatation, [SDV.MHEP.HEG] Life Sciences [q-bio]/Human health and pathology/Hépatology and Gastroenterology, [SDV.MHEP.PED] Life Sciences [q-bio]/Human health and pathology/Pediatrics, Pediatrics, Perinatology and Child Health, Children
Abstract

International audience; Objective: To compare the efficacy of, and complications from, the 2 main treatments for achalasia: endoscopic dilatation and surgical cardiomyotomy (Heller's myotomy).Study design: We retrospectively collected data on children treated for achalasia over an 11-year period from 8 tertiary pediatric centers. A line of treatment was defined as performing either Heller's myotomy or 1-3 sessions of endoscopy dilatation over 3 months. Treatment success was a priori defined as clinical improvement and no need for new treatment.Results: Ninety-seven children (median age, 12 years; 57% boys) were included. The median time to diagnosis was 10.5 months, and the median follow-up period was 27 months. Thirty-seven children were treated by Heller's myotomy and 60 by endoscopy dilatation as the first-line treatment. After adjustment for potentially confounding factors, Heller's myotomy was significantly more successful than endoscopy dilatation (hazard ratio, 3.93 [1.74; 8.88]; P = .001), with a median survival without failure of 49 and 7 months, respectively, and with no significant difference in the occurrence of complications (35.2% for Heller's myotomy, 29.7% for endoscopy dilatation, P = .56). Hydrostatic dilatation was as successful as pneumatic dilatation (hazard ratio, 1.35 [0.56; 3.23]; P = .50).Conclusions: Heller's myotomy is more successful than endoscopy dilatation, with no significant difference in the occurrence of serious complications. This raises the potential role of peroral endoscopic myotomy as an alternative treatment to Heller's myotomy.

Published
2022
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5. Nutri-Score: its benefits and limitations in children's feeding

Author

Jean-Pierre, Chouraqui, Christophe, Dupont, André, Briend, Dominique, Darmaun, Noel, Peretti, Alain, Bocquet, Martin, Chalumeau, Arnaud, De Luca, François, Feillet, Marie-Laure, Frelut, Dominique, Guimber, Alexandre, Lapillonne, Agnès, Linglart, Jean-Christophe, Rozé, Umberto, Simeoni, Dominique, Turck, CHU Grenoble, Centre Hospitalier Universitaire Vaudois [Lausanne] (CHUV), Clinique Marcel Sembat (CCBB) - Ramsay Santé [Boulogne Billancourt], IT University of Copenhagen (ITU), University of Tampere [Finland], Physiopathologie des Adaptations Nutritionnelles (PhAN), Institut National de Recherche pour l’Agriculture, l’Alimentation et l’Environnement (INRAE)-Nantes Université - UFR de Médecine et des Techniques Médicales (Nantes Univ - UFR MEDECINE), Nantes Université - pôle Santé, Nantes Université (Nantes Univ)-Nantes Université (Nantes Univ)-Nantes Université - pôle Santé, Nantes Université (Nantes Univ)-Nantes Université (Nantes Univ), Cardiovasculaire, métabolisme, diabétologie et nutrition (CarMeN), Université Claude Bernard Lyon 1 (UCBL), Université de Lyon-Université de Lyon-Hospices Civils de Lyon (HCL)-Institut National de la Santé et de la Recherche Médicale (INSERM)-Institut National de Recherche pour l’Agriculture, l’Alimentation et l’Environnement (INRAE), Hôpital Femme Mère Enfant [CHU - HCL] (HFME), Hospices Civils de Lyon (HCL), Association Française de Pédiatrie Ambulatoire (AFPA), Equipe 1 : EPOPé - Épidémiologie Obstétricale, Périnatale et Pédiatrique (CRESS - U1153), Centre de Recherche Épidémiologie et Statistique Sorbonne Paris Cité (CRESS (U1153 / UMR_A_1125 / UMR_S_1153)), Conservatoire National des Arts et Métiers [CNAM] (CNAM), HESAM Université - Communauté d'universités et d'établissements Hautes écoles Sorbonne Arts et métiers université (HESAM)-HESAM Université - Communauté d'universités et d'établissements Hautes écoles Sorbonne Arts et métiers université (HESAM)-Université Sorbonne Paris Cité (USPC)-Institut National de la Santé et de la Recherche Médicale (INSERM)-Université Paris Cité (UPCité)-Institut National de Recherche pour l’Agriculture, l’Alimentation et l’Environnement (INRAE)-Conservatoire National des Arts et Métiers [CNAM] (CNAM), HESAM Université - Communauté d'universités et d'établissements Hautes écoles Sorbonne Arts et métiers université (HESAM)-HESAM Université - Communauté d'universités et d'établissements Hautes écoles Sorbonne Arts et métiers université (HESAM)-Université Sorbonne Paris Cité (USPC)-Institut National de la Santé et de la Recherche Médicale (INSERM)-Université Paris Cité (UPCité)-Institut National de Recherche pour l’Agriculture, l’Alimentation et l’Environnement (INRAE), CHU Necker - Enfants Malades [AP-HP], Assistance publique - Hôpitaux de Paris (AP-HP) (AP-HP), Nutrition, croissance et cancer (U 1069) (N2C), Université de Tours (UT)-Institut National de la Santé et de la Recherche Médicale (INSERM), Nutrition-Génétique et Exposition aux Risques Environnementaux (NGERE), Institut National de la Santé et de la Recherche Médicale (INSERM)-Université de Lorraine (UL), Centre Hospitalier Régional Universitaire de Nancy (CHRU Nancy), Centre Hospitalier d'Albi, Centre Hospitalier Régional Universitaire [Lille] (CHRU Lille), Hôpital Jeanne de Flandres, Université de Lille, Droit et Santé-Centre Hospitalier Régional Universitaire [Lille] (CHRU Lille), Fédération pour la recherche en explorations et thérapeutiques innovantes in utéro (FETUS (URP 7328)), Université Paris Cité (UPCité), Centre de Référence des Maladies Rares du Métabolisme du Phosphore et du Calcium and Filière de Santé Maladies Rares (OSCAR), Hôpital Bicêtre, Centre d'Investigation Clinique [Nantes] (CIC004), Centre hospitalier universitaire de Nantes (CHU Nantes), Université de Lausanne = University of Lausanne (UNIL), Institute for Translational Research in Inflammation - U 1286 (INFINITE (Ex-Liric)), Institut National de la Santé et de la Recherche Médicale (INSERM)-Université de Lille-Centre Hospitalier Régional Universitaire [Lille] (CHRU Lille), and CarMeN, laboratoire

Subjects
[SDV.BA] Life Sciences [q-bio]/Animal biology, [SDV.BA]Life Sciences [q-bio]/Animal biology, Pediatrics, Perinatology and Child Health, Gastroenterology
Abstract

International audience; Reducing the burden of non-communicable diseases (NCDs) is one of the top priorities of public health policies worldwide. One of the recognized means of achieving this objective is to improve the diet quality. The Nutri-Score (N-S) is a [five-color - A, B, C, D, E letters] front-of-pack labelling logo intended to help consumers quickly identify the healthier prepackaged foods within a food category. Available studies have shown that the N-S is an efficient tool to achieve this aim in terms of consumers' awareness, perception, understanding and purchasing and that its use may help to reduce the prevalence of NCDs. The N-S is currently implemented on a voluntary basis in seven European countries and a discussion is underway within the European Commission to achieve a harmonized mandatory label. However, no study on the putative impact of the N-S on children's dietary patterns and health is available. The N-S is not applicable to infants' and young children's formulas and to specific baby foods, the compositions of which are already laid down in European Union regulations. The N-S does not replace age-appropriate dietary guidelines. As children consume an increasing number of adult type and processed foods, the relevance of the N-S for children should be evaluated considering the children's high specific requirements, especially in younger children. This is especially necessary for fitting fat and iron requirements, whereas protein-rich foods should be better framed. Moreover, efforts should be made to inform on how to use the N-S and in education on healthy diets.

Published
2022
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6. Barriers to Physical Activity in Children and Adults Living With Type 1 Diabetes: A Complex Link With Real-life Glycemic Excursions

Author

Cassandra Parent, Elodie Lespagnol, Serge Berthoin, Sémah Tagougui, Joris Heyman, Chantal Stuckens, Iva Gueorguieva, Costantino Balestra, Cajsa Tonoli, Bérengère Kozon, Pierre Fontaine, Jacques Weill, Rémi Rabasa-Lhoret, Elsa Heyman, Unité de Recherche Pluridisciplinaire Sport, Santé, Société (URePSSS) - ULR 7369 - ULR 4488 (URePSSS), Université d'Artois (UA)-Université du Littoral Côte d'Opale (ULCO)-Université de Lille, Université de Rennes (UR), Hôpital Jeanne de Flandres, Université de Lille, Droit et Santé-Centre Hospitalier Régional Universitaire [Lille] (CHRU Lille), Haute Ecole de Bruxelles (HE2B), Vrije Universiteit Brussel [Bruxelles] (VUB), CIC CHU ( Lille)/inserm, Institut National de la Santé et de la Recherche Médicale (INSERM)-Université de Lille, Droit et Santé, Centre Hospitalier Régional Universitaire [Lille] (CHRU Lille), Université du Québec à Montréal = University of Québec in Montréal (UQAM), Université de Lille, Univ. Artois, Univ. Littoral Côte d’Opale, Unité de Recherche Pluridisciplinaire Sport, Santé, Société (URePSSS) - ULR 7369, Unité de Recherche Pluridisciplinaire Sport, Santé, Société (URePSSS) - ULR 7369 - ULR 4488 [URePSSS], Université de Rennes [UR], Haute Ecole de Bruxelles [HE2B], Vrije Universiteit Brussel [Bruxelles] [VUB], Génomique Intégrative et Modélisation des Maladies Métaboliques (EGID) - UMR 8199, Université du Québec à Montréal = University of Québec in Montréal [UQAM], Physiotherapy, Human Physiology and Anatomy, Anatomical Research and Clinical Studies, Brussels Heritage Lab, Human Physiology and Sports Physiotherapy Research Group, and Faculty of Physical Education and Physical Therapy

Subjects
barriers to physical activity, diabète de type 1, exercise, étude portant sur des personnes non hospitalisées, type 1 diabetes, Endocrinology, Diabetes and Metabolism, [SDV]Life Sciences [q-bio], General Medicine, exercice, hyperglycemia, hyperglycémie, hypoglycemia, hypoglycémie, outpatient study, sobstacle à l’activité physique, étude ambulatoire, Endocrinology, Internal Medicine
Abstract

OBJECTIVES: Ever since the first research on barriers to physical activity (PA) highlighting fear of hypoglycemia as a major barrier, many studies have attempted to understand their demographic and behavioural determinants. However, no research has been conducted on whether these perceived barriers toward PA are based on real-life-experienced adverse glycemic effects of exercise. METHODS: Sixty-two adults and 53 children/adolescents living with type 1 diabetes along with their parents completed the Barriers to Physical Activity in Type 1 Diabetes-1 (BAPAD-1) questionnaire on barriers to PA. Continuous glucose-monitoring data were collected during 1 week of everyday life for 26 adults and 33 children/adolescents. Multiple linear regressions were used to explore links between BAPAD-1 scores and glycemic excursions experienced during and after everyday-life self-reported PA sessions, controlling for behavioural (accelerometry) and demographic confounders. RESULTS: In children/adolescents, the more time spent in hypoglycemia on nights after PA sessions, the more they reported hypoglycemic risk as a barrier (ß=+0.365, p=0.034). Conversely, in adults, the higher the proportion of PA sessions accompanied by a drop in blood glucose, the less hypoglycemia was a barrier (ß=-0.046, p=0.004). In parents, BAPAD-1 scores were unrelated to children/adolescents' everyday-life exercise-induced hypo/hyperglycemia. CONCLUSIONS: In children/adolescents, fear of hypoglycemia was predominant in those exposed to nocturnal hypoglycemia associated with PA sessions. In adults, fewer barriers may mean they accept a bigger drop in their glycemia during PA. This shows the importance of finding and promoting age-specific solutions to prevent exercise-induced hypoglycemia.

Published
2022
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7. NOS1 mutations cause hypogonadotropic hypogonadism with sensory and cognitive deficits that can be reversed in infantile mice

Author

Konstantina Chachlaki, Andrea Messina, Virginia Delli, Valerie Leysen, Csilla Maurnyi, Chieko Huber, Gaëtan Ternier, Katalin Skrapits, Georgios Papadakis, Sonal Shruti, Maria Kapanidou, Xu Cheng, James Acierno, Jesse Rademaker, Sowmyalakshmi Rasika, Richard Quinton, Marek Niedziela, Dagmar L’Allemand, Duarte Pignatelli, Mirjam Dirlewander, Mariarosaria Lang-Muritano, Patrick Kempf, Sophie Catteau-Jonard, Nicolas J. Niederländer, Philippe Ciofi, Manuel Tena-Sempere, John Garthwaite, Laurent Storme, Paul Avan, Erik Hrabovszky, Alan Carleton, Federico Santoni, Paolo Giacobini, Nelly Pitteloud, Vincent Prevot, CHU Lille, Lille Neurosciences & Cognition - U 1172 (LilNCog), Institut National de la Santé et de la Recherche Médicale (INSERM)-Université de Lille-Centre Hospitalier Régional Universitaire [Lille] (CHRU Lille), FHU 1,000 Days for Health [Lille], Université de Lille, Université de Lausanne = University of Lausanne (UNIL), National and Kapodistrian University of Athens (NKUA), Lausanne University Hospital, Institute of Experimental Medicine [Budapest] (KOKI), Hungarian Academy of Sciences (MTA), Université de Genève = University of Geneva (UNIGE), Oxford Brookes University, Newcastle University [Newcastle], Poznan University of Medical Sciences [Poland] (PUMS), University of Applied Sciences of Eastern Switzerland (FHO), Universidade do Porto = University of Porto, Instituto de Patologia e Imunologia Molecular da Universidade do Porto (IPATIMUP), Geneva University Hospitals and Geneva University, University Children’s Hospital Zurich, Bern University Hospital [Berne] (Inselspital), University of Bern, Hôpital Jeanne de Flandres, Université de Lille, Droit et Santé-Centre Hospitalier Régional Universitaire [Lille] (CHRU Lille), Neurocentre Magendie : Physiopathologie de la Plasticité Neuronale (U1215 Inserm - UB), Université de Bordeaux (UB)-Institut François Magendie-Institut National de la Santé et de la Recherche Médicale (INSERM), Universidad de Córdoba = University of Córdoba [Córdoba], Instituto Maimonides de Investigación Biomédica de Cordoba (IMIBIC), Universidad de Córdoba = University of Córdoba [Córdoba]-Hospital Universitario Reina Sofía, Instituto de Salud Carlos III [Madrid] (ISC), University College of London [London] (UCL), Wolfson Institute for Biomedical Research (WIBR), Université de Clermont-Ferrand, ANR-17-CE16-0015,GRAND,Vieillissement et démence: un rôle hormonal?(2017), and Prevot, Vincent

Subjects
Hypogonadism, General Medicine, Nitric Oxide Synthase Type I, Nitric Oxide, Animals, Cognition, Gonadotropin-Releasing Hormone/genetics, Gonadotropin-Releasing Hormone/metabolism, Humans, Hypogonadism/complications, Hypogonadism/congenital, Hypogonadism/genetics, Mice, Mutant Proteins, Mutation/genetics, Nitric Oxide Synthase Type I/genetics, Nitrites, Gonadotropin-Releasing Hormone, Mutation, [SDV.NEU]Life Sciences [q-bio]/Neurons and Cognition [q-bio.NC], [SDV.NEU] Life Sciences [q-bio]/Neurons and Cognition [q-bio.NC], 610 Medicine & health
Abstract

The nitric oxide (NO) signaling pathway in hypothalamic neurons plays a key role in the regulation of the secretion of gonadotropin-releasing hormone (GnRH), which is crucial for reproduction. We hypothesized that a disruption of neuronal NO synthase (NOS1) activity underlies some forms of hypogonadotropic hypogonadism. Whole-exome sequencing was performed on a cohort of 341 probands with congenital hypogonadotropic hypogonadism to identify ultrarare variants in NOS1 . The activity of the identified NOS1 mutant proteins was assessed by their ability to promote nitrite and cGMP production in vitro. In addition, physiological and pharmacological characterization was carried out in a Nos1 -deficient mouse model. We identified five heterozygous NOS1 loss-of-function mutations in six probands with congenital hypogonadotropic hypogonadism (2%), who displayed additional phenotypes including anosmia, hearing loss, and intellectual disability. NOS1 was found to be transiently expressed by GnRH neurons in the nose of both humans and mice, and Nos1 deficiency in mice resulted in dose-dependent defects in sexual maturation as well as in olfaction, hearing, and cognition. The pharmacological inhibition of NO production in postnatal mice revealed a critical time window during which Nos1 activity shaped minipuberty and sexual maturation. Inhaled NO treatment at minipuberty rescued both reproductive and behavioral phenotypes in Nos1 -deficient mice. In summary, lack of NOS1 activity led to GnRH deficiency associated with sensory and intellectual comorbidities in humans and mice. NO treatment during minipuberty reversed deficits in sexual maturation, olfaction, and cognition in Nos1 mutant mice, suggesting a potential therapy for humans with NO deficiency.

Published
2022
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8. Risk Factors for Recurrence of Borderline Ovarian Tumours after Conservative Surgery and Impact on Fertility: A Multicentre Study by the Francogyn Group

Author

Adele Ozenne, Marion De Berti, Gilles Body, Xavier Carcopino, Olivier Graesslin, Yohan Kerbage, Cherif Akladios, Cyrille Huchon, Alexandre Bricou, Camille Mimoun, Emilie Raimond, Lobna Ouldamer, Service de Gynécologie-Obstétrique [CHRU Tours], Centre Hospitalier Régional Universitaire de Tours (CHRU Tours), Nutrition, croissance et cancer (U 1069) (N2C), Université de Tours (UT)-Institut National de la Santé et de la Recherche Médicale (INSERM), Institut méditerranéen de biodiversité et d'écologie marine et continentale (IMBE), Avignon Université (AU)-Aix Marseille Université (AMU)-Institut de recherche pour le développement [IRD] : UMR237-Centre National de la Recherche Scientifique (CNRS), Service de gynécologie-obstétrique [Hôpital Nord - APHM], Aix Marseille Université (AMU)-Assistance Publique - Hôpitaux de Marseille (APHM)- Hôpital Nord [CHU - APHM], Université de Reims Champagne-Ardenne (URCA), Hôpital Jeanne de Flandres, Université de Lille, Droit et Santé-Centre Hospitalier Régional Universitaire [Lille] (CHRU Lille), CHU Strasbourg, Risques cliniques et sécurité en santé des femmes et en santé périnatale (RISCQ), Université de Versailles Saint-Quentin-en-Yvelines (UVSQ), Service de gynécologie et obstétrique [CHI Poissy-Saint Germain], CHI Poissy-Saint-Germain, Service de gynécologie-obstétrique [Hôpital Jean Verdier, Bondy], Université Paris 13 (UP13)-Hôpital Jean Verdier [AP-HP], Assistance publique - Hôpitaux de Paris (AP-HP) (AP-HP)-Assistance publique - Hôpitaux de Paris (AP-HP) (AP-HP), Service de gynécologie et obstétrique [Hopital Lariboisière - Paris], Assistance publique - Hôpitaux de Paris (AP-HP) (AP-HP)-Hôpital Lariboisière-Fernand-Widal [APHP], Assistance publique - Hôpitaux de Paris (AP-HP) (AP-HP)-Université Paris Cité (UPCité), and HAL UVSQ, Équipe

Subjects
fertility, recurrence, [SDV.MHEP.CHI] Life Sciences [q-bio]/Human health and pathology/Surgery, ovarian tumours, conservative treatment, [SDV.CAN]Life Sciences [q-bio]/Cancer, General Medicine, [SDV.MHEP.CHI]Life Sciences [q-bio]/Human health and pathology/Surgery, [SDV.MHEP.GEO]Life Sciences [q-bio]/Human health and pathology/Gynecology and obstetrics, peritoneal staging, [SDV.MHEP.GEO] Life Sciences [q-bio]/Human health and pathology/Gynecology and obstetrics, [SDV.CAN] Life Sciences [q-bio]/Cancer, [SDV.SPEE] Life Sciences [q-bio]/Santé publique et épidémiologie, [SDV.SPEE]Life Sciences [q-bio]/Santé publique et épidémiologie
Abstract

Introduction: Borderline ovarian tumours (BOT) represent 10–20% of epithelial tumours of the ovary. Although their prognosis is excellent, the recurrence rate can be as high as 30%, and recurrence in the infiltrative form accounts for 3% to 5% of recurrences. Affecting, in one third of cases, women of childbearing age, the surgical strategy with ovarian conservation is now recommended despite a significant risk of recurrence. Few studies have focused exclusively on patients who have received ovarian conservative treatment in an attempt to identify factors predictive of recurrence and the impact on fertility. The objective of this study was to identify the risk factors for recurrence of BOT after conservative treatment and the impact on fertility. Material and methods: This was a retrospective, multicentre study of women who received conservative surgery for BOT between February 1997 and September 2020. We divided the patients into two groups, the “R group” with recurrence and the “NR group” without recurrence. Results: Of 175 patients included, 35 had a recurrence (R group, 20%) and 140 had no recurrence (NR group, 80%). With a mean follow-up of 30 months (IQ 8–62.5), the overall recurrence rate was 20%. Recurrence was BOT in 17.7% (31/175) and invasive in 2.3% (4/175). The mean time to recurrence was 29.5 months (IQ 16.5–52.5). Initial complete peritoneal staging (ICPS) was performed in 42.5% of patients (n = 75). In multivariate analysis, age at diagnosis, nulliparity, advanced FIGO stage, the presence of peritoneal implants, and the presence of a micropapillary component for serous tumours were factors influencing the occurrence of recurrence. The post-surgery fertility rate was 67%. Conclusion: This multicentre study is to date one of the largest studies analysing the risk factors for recurrence of BOT after conservative surgery. Five risk factors were found: age at diagnosis, nulliparity, advanced FIGO stage, the presence of implants, and a micropapillary component. Only 25% of the patients with recurrence underwent ICPS. These results reinforce the interest of initial peritoneal staging to avoid ignoring an advanced tumour stage.

Published
2022
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9. Metabolic hormones in human breast milk are preserved by high hydrostatic pressure processing but reduced by Holder pasteurization

Author

Lucie Marousez, Léa Tran, Edwina Micours, Marie De Lamballerie, Frédéric Gottrand, Véronique Pierrat, Delphine Eberlé, Delphine Ley, Jean Lesage, Université de Lille, Inserm, CHU Lille, Institut de Recherche Translationnelle sur l'Inflammation (INFINITE) - U1286, Institute for Translational Research in Inflammation - U 1286 [INFINITE (Ex-Liric)], Laboratoire de génie des procédés - environnement - agroalimentaire [GEPEA], Lille Inflammation Research International Center (LIRIC) - U995, Hôpital Jeanne de Flandres, Récepteurs nucléaires, Maladies Cardiovasculaires et Diabète (EGID) - U1011, Institute for Translational Research in Inflammation - U 1286 (INFINITE (Ex-Liric)), Institut National de la Santé et de la Recherche Médicale (INSERM)-Université de Lille-Centre Hospitalier Régional Universitaire [Lille] (CHRU Lille), Laboratoire de génie des procédés - environnement - agroalimentaire (GEPEA), École nationale vétérinaire, agroalimentaire et de l'alimentation Nantes-Atlantique (ONIRIS)-Centre National de la Recherche Scientifique (CNRS)-IMT Atlantique (IMT Atlantique), Institut Mines-Télécom [Paris] (IMT)-Institut Mines-Télécom [Paris] (IMT)-Nantes université - UFR des Sciences et des Techniques (Nantes univ - UFR ST), Nantes Université - pôle Sciences et technologie, Nantes Université (Nantes Univ)-Nantes Université (Nantes Univ)-Nantes Université - pôle Sciences et technologie, Nantes Université (Nantes Univ)-Nantes Université (Nantes Univ)-Nantes Université - Institut Universitaire de Technologie Saint-Nazaire (Nantes Univ - IUT Saint-Nazaire), Nantes Université (Nantes Univ)-Nantes Université (Nantes Univ), École nationale vétérinaire, agroalimentaire et de l'alimentation Nantes-Atlantique (ONIRIS), Université de Lille, Droit et Santé-Centre Hospitalier Régional Universitaire [Lille] (CHRU Lille), Récepteurs Nucléaires, Maladies Métaboliques et Cardiovasculaires - U1011 (RNMCD), Institut Pasteur de Lille, Réseau International des Instituts Pasteur (RIIP)-Réseau International des Instituts Pasteur (RIIP)-Institut National de la Santé et de la Recherche Médicale (INSERM)-Université de Lille-Centre Hospitalier Régional Universitaire [Lille] (CHRU Lille), ANR-18-CE21-0005,HHP-humanmilk,Le traitement du lait maternel par haute pression hydrostatique augmente ses qualités biologiques et intestinotrophiques : études expérimentales ex-vivo et chez le nouveau-né prématuré.(2018), Récepteurs Nucléaires, Maladies Métaboliques et Cardiovasculaires (RNMCD - U1011), lesage, jean, and APPEL À PROJETS GÉNÉRIQUE 2018 - Le traitement du lait maternel par haute pression hydrostatique augmente ses qualités biologiques et intestinotrophiques : études expérimentales ex-vivo et chez le nouveau-né prématuré. - - HHP-humanmilk2018 - ANR-18-CE21-0005 - AAPG2018 - VALID

Subjects
hormones, Milk, Human, Human milk, high hydrostatic pressure holder pasteurization human milk hormones, General Medicine, [SDV.IDA] Life Sciences [q-bio]/Food engineering, Analytical Chemistry, High hydrostatic pressure, Milk Banks, [CHIM.OTHE] Chemical Sciences/Other, [SDV.IDA]Life Sciences [q-bio]/Food engineering, Holder pasteurization, Hydrostatic Pressure, Humans, Insulin, Pasteurization, Female, [CHIM.OTHE]Chemical Sciences/Other, Food Science
Abstract

International audience; In human milk banks (HMBs), donor milk (DM) is commonly sterilized by Holder pasteurization (HoP). High hydrostatic pressure (HHP) processing is an innovative, alternative method for DM sterilization. We evaluated the impact of HHP processing on the concentration of seven metabolic milk hormones. Eight samples of raw DM were aliquoted. One aliquot was sterilized by HoP (62 • C for 30 min), and another was processed by HHP (350 MPa at 38 • C). Compared with raw DM, HoP milk displayed reduced concentrations of insulin, nesfatin-1, cortisol, leptin, apelin and GLP-1, though adiponectin levels were unchanged. HHP processing maintained the levels of insulin, nesfatin-1, cortisol and leptin at their initial levels in raw DM, reduced apelin and adiponectin levels, but increased GLP-1 level. Sterilization of DM by HHP thus preserves the main metabolic hormones in human milk, underlining the interest of this method for use in HMBs.

Published
2022
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10. Noninvasive Pediatric Liver Fibrosis Measurement:Two-Dimensional Shear Wave Elastography Compared With Transient Elastography

Author

Léa Chantal, Tran, Delphine, Ley, Gurvan, Bourdon, Stéphanie, Coopman, Héloïse, Lerisson, Céline, Tillaux, Hélène, Béhal, Frédéric, Gottrand, Madeleine, Aumar, Université de Lille, Inserm, CHU Lille, Institute for Translational Research in Inflammation - U 1286 [INFINITE (Ex-Liric)], Hôpital Jeanne de Flandre [Lille], Institut de Recherche Translationnelle sur l'Inflammation (INFINITE) - U1286, Hôpital Jeanne de Flandres, METRICS : Evaluation des technologies de santé et des pratiques médicales - ULR 2694, Institute for Translational Research in Inflammation - U 1286 (INFINITE (Ex-Liric)), Institut National de la Santé et de la Recherche Médicale (INSERM)-Université de Lille-Centre Hospitalier Régional Universitaire [Lille] (CHRU Lille), Université de Lille, Droit et Santé-Centre Hospitalier Régional Universitaire [Lille] (CHRU Lille), Evaluation des technologies de santé et des pratiques médicales - ULR 2694 (METRICS), and Université de Lille-Centre Hospitalier Régional Universitaire [Lille] (CHRU Lille)

Subjects
shear wave elastography, pediatric, ultrasound, cirrhosis, [SDV]Life Sciences [q-bio], Pediatrics, Perinatology and Child Health, liver disease
Abstract

ObjectivesAlthough transient elastography (TE) is the primary noninvasive method for assessing liver fibrosis, its use remains to be validated in children. This study aims to evaluate the agreement between two-dimensional ultrasound shear wave elastography (2D-SWE) and TE to assess pediatric liver stiffness method.MethodsDuring the 18-month study, we prospectively included 101 consecutive children (median age: 8.5 years, range: 1 month to 17 years) who required TE for medical reasons, and in whom 2D-SWE measurement was performed within a 3-month follow-up during a routine ultrasound. Liver elasticity values were classified according to the Metavir score using published pediatric norms for TE and according to the manufacturer's reference values for 2D-SWE. The Spearman's correlation coefficient was used to assess the relationship between the elasticity measured by the two techniques. Concordance was described by the Bland–Altman method.ResultsA strong correlation (rho = 0.70, p < 0.001) was found between 2D-SWE and TE for the elasticity measures. The strength of correlation was higher among patients older than 6 years (rho = 0.79, p < 0.001). Concordance between liver fibrosis stages assessed by these techniques was moderate [weighted kappa = 0.46, (95% CI: 0.35–0.57)]. When considering stages over F2, 2D-SWE diagnostic performances showed a sensitivity of 85% (95% CI: 74–92) and a specificity of 57% (95% CI: 42–70) compared with TE.ConclusionMeasurements of the liver stiffness using 2D-SWE and TE are strongly correlated. The moderate concordance between these techniques for assessing the liver fibrosis stage provides evidence against alternating between these methods during follow-up of patients with the chronic liver diseases.

Published
2022
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11. Peace, not war in Ukraine or anywhere else, please

Author

Jean-Yves Lefrant, Romain Pirracchio, Dan Benhamou, Marc-Olivier Fischer, Rosanna Njeim, Bernard Allaouchiche, Sophie Bastide, Matthieu Biais, Lionel Bouvet, Olivier Brissaud, Sorin J. Brull, Xavier Capdevila, Nicola Clausen, Philippe Cuvillon, Christophe Dadure, Jean-Stéphane David, Bin Du, Sharon Einav, Victoria Eley, Patrice Forget, Tomoko Fujii, Anne Godier, Dean P. Gopalan, Sophie Hamada, Ahmed Hasanin, Olivier Joannes-boyau, Sébastien Kerever, Éric Kipnis, Kerstin Kolodzie, Ruth Landau, Arthur Le Gall, Morgan Le Guen, Matthieu Legrand, Emmanuel Lorne, Frédéric J. Mercier, Nicolas Mongardon, Sheila Myatra, Armelle Nicolas-Robin, Mark John Peters, Hervé Quintard, Jordi Rello, Philippe Richebé, Jason Alexander Roberts, Antoine Rocquilly, Filippo Sanfilippo, Antoine Schneider, Mircea T. Sofonea, Francis Veyckemans, Paul Zetlaoui, Ahed Zeidan, Laurent Zieleskiewicz, Marzena Zielinska, Britta Von Ungern-Sternberg, Osama Abou Arab, Alice Blet, Fanny Bounes, Matthieu Boisson, Anaïs Caillard, Aude Carillion, Thomas Clavier, Denis Frasca, Arthur James, Stéphanie Sigaut, Sacha Rozencwajg, Pierre Albaladejo, Hervé Bouaziz, Centre Hospitalier Universitaire de Nîmes (CHU Nîmes), Initial MAnagement and prevention of acute orGan failures IN critically ill patiEnts (IMAGINE), Université de Montpellier (UM), Société française d'anesthésie et de réanimation (SFAR), SFAR, University of California [San Francisco] (UC San Francisco), University of California (UC), AP-HP Hôpital Bicêtre (Le Kremlin-Bicêtre), Département d'anesthésiologie [CHU Caen], Université de Caen Normandie (UNICAEN), Normandie Université (NU)-Normandie Université (NU)-CHU Caen, Normandie Université (NU)-Tumorothèque de Caen Basse-Normandie (TCBN)-Tumorothèque de Caen Basse-Normandie (TCBN), Hospices Civils de Lyon (HCL), CHU Bordeaux [Bordeaux], Mayo Clinic [Jacksonville], Centre Hospitalier Régional Universitaire [Montpellier] (CHRU Montpellier), Institut des Neurosciences de Montpellier (INM), Institut National de la Santé et de la Recherche Médicale (INSERM)-Université de Montpellier (UM), Odense University Hospital (OUH), Unité de réanimation médicale [CHU de Carémeau, Nîmes], Hôpital Universitaire Carémeau [Nîmes] (CHU Nîmes), Peking Union Medical College Hospital [Beijing] (PUMCH), The Hebrew University of Jerusalem (HUJ), University of Queensland [Brisbane], University of Aberdeen, The Jikei University School of Medicine, Hôpital Européen Georges Pompidou [APHP] (HEGP), Assistance publique - Hôpitaux de Paris (AP-HP) (AP-HP)-Hôpitaux Universitaires Paris Ouest - Hôpitaux Universitaires Île de France Ouest (HUPO), University of KwaZulu-Natal [Durban, Afrique du Sud] (UKZN), Cairo University - Faculty of Medicine, Service de Réanimation Médicale [CHU Bordeaux], CHU Bordeaux [Bordeaux]-Hôpital Pellegrin, Département d’Anesthésie-Réanimation-SMUR [Hôpital Lariboisière], Hôpitaux Universitaire Saint-Louis, Lariboisière, Fernand-Widal, Marqueurs cardiovasculaires en situation de stress (MASCOT (UMR_S_942 / U942)), Institut National de la Santé et de la Recherche Médicale (INSERM)-Groupe Hospitalier Saint Louis - Lariboisière - Fernand Widal [Paris], Assistance publique - Hôpitaux de Paris (AP-HP) (AP-HP)-Assistance publique - Hôpitaux de Paris (AP-HP) (AP-HP)-Centre National de la Recherche Scientifique (CNRS)-Université Paris Cité (UPCité)-Université Sorbonne Paris Nord, CHU Lille, Columbia University Medical Center (CUMC), Columbia University [New York], CHU Pontchaillou [Rennes], Hôpital Foch [Suresnes], Simplification des soins chez les patients complexes - UR UPJV 7518 (SSPC), Université de Picardie Jules Verne (UPJV), Clinique du Millénaire - Oc Santé [Montpellier], Oc Santé [Montpellier], AP-HP - Hôpital Antoine Béclère [Clamart], Assistance publique - Hôpitaux de Paris (AP-HP) (AP-HP), Hôpital Henri Mondor, Assistance publique - Hôpitaux de Paris (AP-HP) (AP-HP)-Hôpital Henri Mondor-Université Paris-Est Créteil Val-de-Marne - Paris 12 (UPEC UP12), Homi Bhabha National Institute (HBNI), Hôpital de la Fondation Ophtalmologique Adolphe de Rothschild [AP-HP], Great Ormond Street Hospital for Children NHS Foundation Trust [London, UK] (GOSHC), Centre Hospitalier Universitaire de Nice (CHU Nice), Universitat Internacional de Catalunya [Barcelona] (UIC), Hôpital Maisonneuve-Rosemont, Service des Urgences [CHU Nantes], Hôtel-Dieu de Nantes, Università degli studi di Catania = University of Catania (Unict), Centre Hospitalier Universitaire Vaudois [Lausanne] (CHUV), Maladies infectieuses et vecteurs : écologie, génétique, évolution et contrôle (MIVEGEC), Centre National de la Recherche Scientifique (CNRS)-Institut de Recherche pour le Développement (IRD [France-Sud])-Université de Montpellier (UM), Hôpital Jeanne de Flandres, Université de Lille, Droit et Santé-Centre Hospitalier Régional Universitaire [Lille] (CHRU Lille), King Faisal Specialist Hospital and Resarch Centre [Riyadh, Saudi Arabia] (KFSHRC), Service Anesthésie et Réanimation [Hôpital Nord - APHM], Aix Marseille Université (AMU)-Assistance Publique - Hôpitaux de Marseille (APHM)- Hôpital Nord [CHU - APHM], Wrocław Medical University, The University of Western Australia (UWA), CHU Amiens-Picardie, Mécanismes physiopathologiques et conséquences des calcifications vasculaires - UR UPJV 7517 (MP3CV), Université de Picardie Jules Verne (UPJV)-CHU Amiens-Picardie, Hôpital de la Croix-Rousse [CHU - HCL], Centre de Recherche en Cancérologie de Lyon (UNICANCER/CRCL), Centre Léon Bérard [Lyon]-Université Claude Bernard Lyon 1 (UCBL), Université de Lyon-Université de Lyon-Institut National de la Santé et de la Recherche Médicale (INSERM)-Centre National de la Recherche Scientifique (CNRS), Service SAMU-SMUR [CHU Toulouse], Pôle Médecine d'urgences [CHU Toulouse], Centre Hospitalier Universitaire de Toulouse (CHU Toulouse)-Centre Hospitalier Universitaire de Toulouse (CHU Toulouse), Pharmacologie des anti-infectieux et antibiorésistance (PHAR2), Université de Poitiers-Institut National de la Santé et de la Recherche Médicale (INSERM), Service des urgences [CHU Poitiers], Centre hospitalier universitaire de Poitiers (CHU Poitiers), Hôpital de la Cavale Blanche - CHRU Brest (CHU - BREST ), CHU Pitié-Salpêtrière [AP-HP], Assistance publique - Hôpitaux de Paris (AP-HP) (AP-HP)-Sorbonne Université (SU), Unité de Recherche sur les Maladies Cardiovasculaires, du Métabolisme et de la Nutrition = Research Unit on Cardiovascular and Metabolic Diseases (ICAN), Assistance publique - Hôpitaux de Paris (AP-HP) (AP-HP)-Institut National de la Santé et de la Recherche Médicale (INSERM)-Sorbonne Université (SU)-Institut de Cardiométabolisme et Nutrition = Institute of Cardiometabolism and Nutrition [CHU Pitié Salpêtrière] (IHU ICAN), Assistance publique - Hôpitaux de Paris (AP-HP) (AP-HP)-Sorbonne Université (SU)-Assistance publique - Hôpitaux de Paris (AP-HP) (AP-HP)-Sorbonne Université (SU)-CHU Pitié-Salpêtrière [AP-HP], Service de réanimation médicale [CHU Rouen], Hôpital Charles Nicolle [Rouen], CHU Rouen, Normandie Université (NU)-Normandie Université (NU)-CHU Rouen, Normandie Université (NU)-Normandie Université (NU)-Université de Rouen Normandie (UNIROUEN), Normandie Université (NU), Endothélium, valvulopathies et insuffisance cardiaque (EnVI), Université de Rouen Normandie (UNIROUEN), Normandie Université (NU)-Normandie Université (NU)-Institut National de la Santé et de la Recherche Médicale (INSERM), MethodS in Patients-centered outcomes and HEalth ResEarch (SPHERE), Université de Tours (UT)-Institut National de la Santé et de la Recherche Médicale (INSERM)-Nantes Université - UFR des Sciences Pharmaceutiques et Biologiques (Nantes Univ - UFR Pharmacie), Nantes Université - pôle Santé, Nantes Université (Nantes Univ)-Nantes Université (Nantes Univ)-Nantes Université - pôle Santé, Nantes Université (Nantes Univ)-Nantes Université (Nantes Univ), Hôpital Beaujon [AP-HP], AP-HP - Hôpital Bichat - Claude Bernard [Paris], Université Grenoble Alpes - UFR Médecine (UGA UFRM), Université Grenoble Alpes (UGA), Service de Réanimation Médicale [CHRU Nancy], and Centre Hospitalier Régional Universitaire de Nancy (CHRU Nancy)

Subjects
Warfare, Anesthesiology and Pain Medicine, MESH: Humans, MESH: Warfare, MESH: Ukraine, ARTICLE CLINIQUE, Humans, General Medicine, Critical Care and Intensive Care Medicine, Ukraine, [SDV.MHEP]Life Sciences [q-bio]/Human health and pathology
Abstract

International audience; War is back in Europe. With all its horrific pictures and live videos.In its report issued on the 24th of March 2022, the World Health Organization (WHO) [1] states that the Ukrainian conflict has involved 18 million persons so far, with 3.4 million refugees in bordering countries and 6.4 million persons internally displaced (Fig. 1). In this report, 1,035 deaths and 1,650 civilian injuries were reported in Ukraine without any reported data from Russia.

Published
2022
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12. Arabin pessary to prevent adverse perinatal outcomes in twin pregnancies with a short cervix: a multicenter randomized controlled trial (PESSARONE)

Author

Marion Groussolles, Norbert Winer, Loïc Sentilhes, Florence Biquart, Mona Massoud, Alexandre J. Vivanti, Hanane Bouchghoul, Patrick Rozenberg, Pascale Olivier, Raoul Desbriere, Celine Chauleur, Franck Perrotin, Frederic Coatleven, Florent Fuchs, Florence Bretelle, Vassilis Tsatsaris, Laurent J. Salomon, Nicolas Sananes, Gilles Kayem, Veronique Houflin-Debarge, Thomas Schmitz, Guillaume Benoist, Catherine Arnaud, Virginie Ehlinger, Christophe Vayssière, Fuchs, Florent, Centre Hospitalier Universitaire de Toulouse (CHU Toulouse), Centre d'Epidémiologie et de Recherche en santé des POPulations (CERPOP), Université Toulouse III - Paul Sabatier (UT3), Université Fédérale Toulouse Midi-Pyrénées-Université Fédérale Toulouse Midi-Pyrénées-Institut National de la Santé et de la Recherche Médicale (INSERM), Centre hospitalier universitaire de Nantes (CHU Nantes), Centre Hospitalier Universitaire d'Angers (CHU Angers), PRES Université Nantes Angers Le Mans (UNAM), Hôpital Femme Mère Enfant [CHU - HCL] (HFME), Hospices Civils de Lyon (HCL), AP-HP - Hôpital Antoine Béclère [Clamart], Assistance publique - Hôpitaux de Paris (AP-HP) (AP-HP), Université Paris-Sud - Paris 11 (UP11), Hôpital Bicêtre, Centre hospitalier intercommunal de Poissy/Saint-Germain-en-Laye - CHIPS [Poissy], Risques cliniques et sécurité en santé des femmes et en santé périnatale (RISCQ), Université de Versailles Saint-Quentin-en-Yvelines (UVSQ), Hôpital Saint-Joseph [Marseille], Centre Hospitalier Universitaire de Saint-Etienne (CHU de Saint-Etienne), Centre Hospitalier Régional Universitaire de Tours (CHRU Tours), CHU Bordeaux [Bordeaux], CHU Montpellier, Centre Hospitalier Régional Universitaire [Montpellier] (CHRU Montpellier), Hôpital Nord [CHU - APHM], Maternité Port-Royal [CHU Cochin], Assistance publique - Hôpitaux de Paris (AP-HP) (AP-HP)-Hôpital Cochin [AP-HP], CHU Necker - Enfants Malades [AP-HP], Université Paris Descartes - Paris 5 (UPD5), CHU Strasbourg, CHU Trousseau [APHP], Assistance publique - Hôpitaux de Paris (AP-HP) (AP-HP)-Sorbonne Université (SU), Hôpital Jeanne de Flandres, Université de Lille, Droit et Santé-Centre Hospitalier Régional Universitaire [Lille] (CHRU Lille), Hôpital Robert Debré, Université Paris Diderot - Paris 7 (UPD7), Service de Gynécologie-Obstétrique et Médecine de la Reproduction [CHU Caen], Université de Caen Normandie (UNICAEN), Normandie Université (NU)-Normandie Université (NU)-CHU Caen, Normandie Université (NU)-Tumorothèque de Caen Basse-Normandie (TCBN)-Tumorothèque de Caen Basse-Normandie (TCBN), and The French Ministry of Health (Programme Hospitalier de Recherche Clinique, AOM2013) supported this study,which was sponsored by the Department of Clinical Research of the Toulouse University Hospital Center, France

Subjects
adverse neonatal outcomes, pessary, Infant, Newborn, preterm birth, Obstetrics and Gynecology, Cervix Uteri, [SDV.MHEP.GEO]Life Sciences [q-bio]/Human health and pathology/Gynecology and obstetrics, Pessaries, twin pregnancies, [SDV.MHEP.GEO] Life Sciences [q-bio]/Human health and pathology/Gynecology and obstetrics, Cervical Length Measurement, Pregnancy, Pregnancy, Twin, Humans, Premature Birth, Female, short cervix, Progesterone
Abstract

International audience; Background: The number of twin pregnancies continues to increase worldwide as both the number of pregnancies obtained by medically assisted reproduction and age at first pregnancy keep rising. Preterm delivery is the major complication associated with twin pregnancies. The effectiveness of preventive treatments such as progesterone or cervical cerclage for women with a short cervix is doubtful in twin pregnancies. The effectivity of cervical pessaries in preventing preterm birth and its associated morbidity and mortality is also controversial.Objective: We sought to investigate if the Arabin pessary reduces adverse neonatal outcomes in twin pregnancies with a short cervix.Study design: This open-label, multicenter, randomized controlled trial on twin pregnancies with a cervical length of

Published
2022
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13. Lessons From Prospective Longitudinal Follow-up of a French APECED Cohort.

Author

Humbert L, Proust-Lemoine E, Dubucquoi S, Kemp EH, Saugier-Veber P, Fabien N, Raymond-Top I, Cardot-Bauters C, Carel JC, Cartigny M, Chabre O, Chanson P, Delemer B, Do Cao C, Guignat L, Kahn JE, Kerlan V, Lefebvre H, Linglart A, Mallone R, Reynaud R, Sendid B, Souchon PF, Touraine P, Wémeau JL, and Vantyghem MC

Subjects
Humans, Male, Female, France epidemiology, Adult, Prospective Studies, Adolescent, Child, Follow-Up Studies, Young Adult, Child, Preschool, Longitudinal Studies, Middle Aged, Mutation, Infant, Cohort Studies, Phenotype, Hypoparathyroidism epidemiology, Hypoparathyroidism genetics, Polyendocrinopathies, Autoimmune genetics, Polyendocrinopathies, Autoimmune epidemiology, Polyendocrinopathies, Autoimmune immunology, Polyendocrinopathies, Autoimmune diagnosis, AIRE Protein, Transcription Factors genetics, Transcription Factors immunology
Abstract

Background: Autoimmune polyendocrinopathy-candidiasis-ectodermal dystrophy syndrome is a rare disease caused by biallelic mutations of the AIRE gene, usually presenting with the triad hypoparathyroidism-adrenal failure-chronic mucocutaneous candidiasis (CMC) and nonendocrine manifestations. The aim of this study was to determine the molecular profile of the AIRE gene, the prevalence of rare manifestations, and to characterize immunological disturbances in a French cohort., Patients and Methods: A national, multicenter prospective observational study to collect genetic, clinical, biological, and immunological data (NCT03751683)., Results: Twenty-five patients (23 families) were enrolled. Eleven distinct AIRE variants were identified, 2 of which were not previously reported: an intronic variant, c.653-70G > A, and a c.1066del (p.Arg356GlyfsX22) variant (exon 9). The most common was the Finnish variant c.769C > T (16 alleles), followed by the variant c.967&#95;979del13 (15 alleles), which seemed associated with a less severe phenotype. Seventeen out of 25 patients were homozygote. The median number of clinical manifestations was 7; 19/25 patients presented with the hypoparathyroidism-adrenal failure-CMC triad, 8/13 showed pulmonary involvement, 20/25 had ectodermal dystrophy, 8/25 had malabsorption, and 6/23 had asplenia. Fifteen out of 19 patients had natural killer cell lymphopenia with an increase in CD4+ and CD8+ T lymphocytes and an age-dependent alteration of B lymphocyte homeostasis compared with matched controls (P < .001), related to the severity of the disease. All tested sera (n = 18) were positive for anti-interferon-α, 15/18 for anti-IL-22 antibodies, and 13/18 for anti-IL-17F antibodies, without clear phenotypic correlation other than with CMC., Conclusion: This first prospective cohort showed a high AIRE genotype variability, with 2 new gene variants. The prevalence of potentially life-threatening nonendocrine manifestations was higher with systematic screening. These manifestations could, along with age-dependent B-cell lymphopenia, contribute to disease severity. Systematic screening for all the manifestations of the syndrome would allow earlier diagnosis, supporting vaccination and targeted therapeutic approaches., (© The Author(s) 2024. Published by Oxford University Press on behalf of the Endocrine Society.)

Published
2025
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14. Chapter 7: The different forms of primary hyperparathyroidism at different ages of life: Childhood, pregnancy, lactation, old age.

Author

Lemaitre M, Picart C, Gueorguieva I, Charbit J, Edouard T, Linglart A, Luton D, and Chanson P

Subjects
Humans, Pregnancy, Female, Child, Adolescent, Infant, Newborn, Pregnancy Complications epidemiology, Pregnancy Complications diagnosis, Aged, Infant, Child, Preschool, Adult, Hyperparathyroidism, Primary diagnosis, Hyperparathyroidism, Primary complications, Hyperparathyroidism, Primary epidemiology, Hyperparathyroidism, Primary surgery, Lactation physiology
Abstract

Primary hyperparathyroidism is rare in children. A germline mutation is identified in half of all children with primary hyperparathyroidism (70% of newborns and infants, and 40% of children and adolescents). The clinical manifestations of primary hyperparathyroidism in children are highly variable (often absent in newborns, rather severe and symptomatic in children and adolescents) and depend on the genetic cause, as well as the severity, rapidity of onset and duration of hypercalcemia. Morphological investigation and treatment of children and adolescents follow the same rules as for adults. Surgery must be carried out by a surgeon expert in this pathology in children. Primary hyperparathyroidism is rarely diagnosed during pregnancy. Pregnancy-related changes in phosphocalcic homeostasis can sometimes mask its symptomatology, which explains why it is most often asymptomatic and/or undiagnosed due to low levels of hypercalcemia and/or the attribution of certain symptoms (e.g. vomiting) to pregnancy itself. Maternal-fetal morbidity associated with primary hyperparathyroidism during pregnancy, historically considered significant, is in fact rare and depends on maternal calcium levels. Treatment (conservative or surgical) must be adapted to the term of pregnancy, the severity of symptoms and maternal-fetal risks. Primary hyperparathyroidism is common in the elderly. Although the diagnostic approach is comparable to that in younger patients, some clinical specificities of this population need to be considered. In view of the high prevalence of osteoporotic fractures and their consequences for morbidity and mortality in the elderly, surgery is the preferred option in case of osteoporosis. In 2024, the neuropsychological symptoms and cardiovascular impairment associated with primary hyperparathyroidism do not justify parathyroidectomy. Parathyroidectomy is much less frequent than in the younger population, although it remains the first-line treatment, especially as its safety and efficacy have been widely demonstrated., (Copyright © 2025 Elsevier Masson SAS. All rights reserved.)

Published
2025
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15. Evaluating script concordance tests (SCTs) through the lens of Bayesian reasoning: Enhancing assessment in medical education.

Author

Dauchet L, Bentegeac R, Ghauss H, Hazzan M, Truffert P, Amouyel P, Gauthier V, and Hamroun A

Subjects
Humans, Clinical Competence standards, Clinical Reasoning, Bayes Theorem, Educational Measurement methods, Education, Medical methods
Abstract

Background: Script Concordance Tests (SCTs) represent an innovative assessment method which have been introduced in the 2024 French National Ranking Examinations (EDN). These tests compare a student's clinical reasoning with that of a panel of experts under conditions of uncertainty. Typically, the question involves the impact of new information on an initially proposed hypothesis, with answers given on a Likert scale., Main Findings: This article aims to didactically illustrate how SCTs are consistent with probabilistic reasoning as modeled by Bayes' theorem. In addition, by comparing SCT writing guidelines with Bayesian reasoning concepts, several ambiguities were identified: (1) What stage of clinical reasoning do SCTs evaluate? (2) What are the appropriate labels for Likert scale responses? (3) Does the expert panel provide a relevant reference for SCTs?, Conclusions: Currently, many of these questions remain unanswered in the literature, with recent data suggesting that experienced physicians' responses to SCTs are often biased. Beyond their use as an assessment tool in the EDN, SCTs offer a valuable opportunity to develop and deepen the teaching of probabilistic reasoning in medical education and serve as a potential area of research to improve clinical practice., Competing Interests: Conflict of interest The authors do not have any conflict of interest to declare., (Copyright © 2024 The Author(s). Published by Elsevier Masson SAS.. All rights reserved.)

Published
2025
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16. [The expert panel for Script Concordance Tests: A truly adequate reference?]

Author

Dauchet L, Bentegeac R, Ghauss H, Hazzan M, Truffert P, Amouyel P, Gauthier V, and Hamroun A

Subjects
Humans, Clinical Reasoning, France, Students, Medical statistics & numerical data, Uncertainty, Education, Medical standards, Education, Medical methods, Bayes Theorem, Educational Measurement methods, Educational Measurement standards, Clinical Competence standards
Abstract

The Script Concordance Tests (SCTs) are an examination modality introduced by decree in the French National Ranking Exam for medical students in 2024. Their objective is to evaluate clinical reasoning in situations of uncertainty. In practice, SCTs assess the impact of new information on the probability of a hypothesis formulated a priori based on an authentic clinical scenario. This approach resembles probabilistic (or Bayesian) reasoning. Due to the uncertainty associated with the explored clinical situation, SCTs do not compare the student's response to an expected one in a theoretical knowledge reference. Instead, the distribution of responses from a panel of experienced physicians is used to establish the question's scoring scale. Literature data suggest that physicians, even experienced ones, like most humans, often exhibit biased intuitive probabilistic reasoning. These biases raise questions about the relevance of using expert panel responses as scoring scales for SCTs., (Copyright © 2024 The Author(s). Published by Elsevier Masson SAS.. All rights reserved.)

Published
2024
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17. [Management of asthma attack in children aged 6 to 12 years].

Author

Marguet C, Benoist G, Werner A, Cracco O, L'excellent S, Rhagani J, Tamalet A, Vrignaud B, Schweitzer C, Lejeune S, Giovannini-Chami L, Mortamet G, and Houdouin V

Subjects
Child, Humans, Anti-Asthmatic Agents therapeutic use, Anti-Asthmatic Agents administration & dosage, Asthma therapy, Asthma diagnosis, Asthma epidemiology
Published
2024
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20. JAK inhibitors in difficult-to-treat adult-onset Still's disease and systemic-onset juvenile idiopathic arthritis.

Author

Gillard L, Pouchot J, Cohen-Aubart F, Koné-Paut I, Mouterde G, Michaud M, Reumaux H, Savey L, Belot A, Fautrel B, and Mitrovic S

Subjects
Adult, Child, Humans, Interleukin 1 Receptor Antagonist Protein therapeutic use, Retrospective Studies, Treatment Outcome, Cytokines, Arthritis, Juvenile drug therapy, Janus Kinase Inhibitors therapeutic use, Still's Disease, Adult-Onset drug therapy, Antirheumatic Agents therapeutic use
Abstract

Objectives: Excessive and inappropriate production of pro-inflammatory cytokines plays a key role in Still's disease. Janus kinase inhibitor (JAKi) agents mainly block pro-inflammatory cytokine pathways, notably IL-6 and IFN. The objective was to assess the efficacy and safety of JAKi agents in difficult-to-treat systemic JIA or adult-onset Still's disease (AOSD)., Methods: This retrospective study was based on a national survey conducted in the departments of rheumatology, paediatric rheumatology and internal medicine of French hospitals regarding systemic JIA and AOSD patients who received JAKi agents. The data were collected with a standardized questionnaire and analysed at different times (treatment initiation, months 1, 3 and 6 and the end of follow-up)., Results: Nine patients (seven adults) were included. All patients showed inadequate response to CS or conventional synthetic or biologic DMARDs. Baricitinib was used in five patients, ruxolitinib in two, tofacitinib in two and upadacitinib in one. A JAKi was used combined with CS in all but two patients. A JAKi was associated with anakinra and CS in one patient, and with MTX, anakinra and CS in another. The median (range) follow-up was 16 (1-33) months. Two cases out of nine showed complete remission, 3/9 partial response and 4/9 treatment failure. At the last visit, CS could be decreased but not stopped. Tolerance of the JAKi was acceptable (no severe adverse events)., Conclusion: JAKi agents may be a therapeutic option for some patients with difficult-to-treat Still's disease, especially those with partial response to medium- or high-dose CS or biologics., (© The Author(s) 2022. Published by Oxford University Press on behalf of the British Society for Rheumatology. All rights reserved. For permissions, please email: journals.permissions@oup.com.)

Published
2023
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21. Medium to long term functional and radiographic outcomes in patients with Madelung's deformity treated by isolated or combined radioulnar osteotomy.

Author

Guillaume PA, Mainard N, Canavese F, Baudoux M, Mézel A, Chantelot C, and Saab M

Subjects
Humans, Child, Adolescent, Young Adult, Adult, Retrospective Studies, Range of Motion, Articular, Radius diagnostic imaging, Radius surgery, Radius abnormalities, Wrist Joint diagnostic imaging, Wrist Joint surgery, Osteotomy methods, Activities of Daily Living, Ulna diagnostic imaging, Ulna surgery, Ulna abnormalities
Abstract

Introduction: Madelung's deformity is a rare congenital condition of the wrist that can cause pain, aesthetic concerns, reduced range of motion and grip strength. Currently, there is no consensus on the optimal age for surgery or whether operative procedures can be isolated or combined depending on the extent of the deformity. The main objective of our study was to analyze the postoperative functional clinical results at a minimum of 3-years follow-up in patients operated on for Madelung's deformity. The secondary objectives were (1) comparison of preoperative and postoperative radiographic parameters, and (2) to assess whether certain preoperative radiographic parameters influence the choice of surgical procedure., Hypothesis: The surgery offered at our center achieves clinical and radiological result necessary for long-term activities of daily living, and varies according to the severity of the distal radial deformity., Patients and Methods: We carried out a retrospective observational monocentric study including patients operated on between 2004 and 2016. Clinical (VAS, mobility), functional (PRWE score), and radiographic assessments were performed before and after the last follow-up., Results: The study included 11 patients (12 wrists) with a mean age of 17±7.3 years and a mean follow-up of 8.1 years (4-12.3). The mean VAS was 2.3 (0-6) and the mean PRWE score was 37 (0-108). The mean flexion-extension arc was 134° and that of pronation and supination was 142°. The mean grip strength was 25.8±11.8kg. Four out of 6 radiographic criteria were significantly improved. An isolated radial osteotomy or combined radioulnar osteotomy was performed when the sagittal radial tilt was greater than 30° and protrusion of the lunate greater than 5mm, otherwise below these values, an isolated ulnar osteotomy was performed., Discussion: Our center offers surgical management of Madelung's deformity by osteotomy which improves the majority of postoperative radiographic parameters and gives satisfactory clinical and radiographic results after a mean follow-up of 8.1 years. The surgery is influenced by the severity of the distal radial deformity, including protrusion of the lunate and sagittal radial tilt., Level of Evidence: IV, Retrospective study., (Copyright © 2022 Elsevier Masson SAS. All rights reserved.)

Published
2022
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22. Organ donation by Maastricht-III pediatric patients: Recommendations of the Groupe Francophone de Réanimation et Urgences Pédiatriques (GFRUP) and Association des Anesthésistes Réanimateurs Pédiatriques d'Expression Française (ADARPEF). Part II: Specific organizational and technical considerations.

Author

Dauger S, Blanot S, Deho A, Beaux J, Bonnin F, Bordet F, Cremer R, Dupont S, Klusiewicz A, Lafargue A, Lemains M, Michel F, Quéré R, Blanquat LS, Samyn M, Saulnier ML, Temper L, Merchaoui Z, Roux BG, Groupe-Francophone-de-Réanimation-Et-Urgences-Pédiatriques-Gfrup, and Association-des-Anesthésistes-Réanimateurs-Pédiatriques-d'Expression-Française-Adarpef

Subjects
Airway Extubation, Child, Death, Humans, Perfusion methods, Heart Arrest, Tissue and Organ Procurement
Abstract

A panel of pediatric experts met to develop recommendations on the technical requirements specific to pediatric controlled donation after planned withdrawal of life-sustaining therapies (Maastricht category III). The panel recommends following the withdrawal of life-sustaining therapies protocol usually applied in each unit, which may or may not include immediate extubation. The organ retrieval process should be halted if death does not occur within 3 h of life-support discontinuation. Circulatory arrest is defined as loss of pulsatile arterial pressure and should be followed by a 5-min no-touch observation period. Death is declared based on a list of clinical criteria assessed by two senior physicians. The no-flow time should be no longer than 30, 45, and 90 min for the liver, kidneys, and lungs, respectively. At present, the panel does not recommend pediatric heart donation after death by circulatory arrest. The mean arterial pressure cutoff that defines the start of the functional warm ischemia (FWI) phase is 45 mmHg in patients older than 5 years and/or weighing more than 20 kg. The panel recommends normothermic regional perfusion in these patients. The FWI phase should not exceed 30 and 45 min for retrieving the pancreas and liver, respectively. There is no time limit to the FWI phase for the lungs and kidneys. The panel recommends routine sharing of experience with Maastricht-III donation among all healthcare institutions involved in order to ensure optimal outcome assessment and continuous discussion on the potential difficulties, notably those related to the management of normothermic regional perfusion in small children., Competing Interests: Conflicts of interest None, (Copyright © 2022. Published by Elsevier Masson SAS.)

Published
2022
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23. Lessons from a large nationwide cohort of 350 children with ovarian mature teratoma: A study in favor of ovarian-sparing surgery.

Author

Delehaye F, Sarnacki S, Orbach D, Cheikhelard A, Rouger J, Parienti JJ, Faure-Conter C, Hameury F, Dijoud F, Aubry E, Wacrenier A, Habonimana E, Duchesne C, Joseph S, Alliot H, Scalabre A, Chaussy Y, Podevin G, Croue A, Haraux E, Guibal MP, Pommepuy I, Ballouhey Q, Lavrand F, Peycelon M, Irtan S, Guerin F, Dariel A, Borionne C, Galmiche L, and Rod J

Subjects
Adolescent, Child, Female, Humans, Ovariectomy, Retrospective Studies, Ovarian Neoplasms pathology, Teratoma pathology
Abstract

Background: Ovarian mature teratoma (OMT) is a common ovarian tumor found in the pediatric population. In 10%-20% of cases, OMT occurs as multiple synchronous or metachronous lesions on ipsi- or contralateral ovaries. Ovarian-sparing surgery (OSS) is recommended to preserve fertility, but total oophorectomy (TO) is still performed., Design: This study reviews the clinical data of patients with OMT, and analyzes risk factors for second events. A national retrospective review of girls under 18 years of age with OMTs was performed. Data on clinical features, imaging, laboratory studies, surgical reports, second events and their management were retrieved., Results: Overall, 350 children were included. Eighteen patients (5%) presented with a synchronous bilateral form at diagnosis. Surgery was performed by laparotomy (85%) and laparoscopy (15%). OSS and TO were performed in 59% and 41% of cases, respectively. Perioperative tumor rupture occurred in 23 cases, independently of the surgical approach. Twenty-nine second events occurred (8.3%) in a median time of 30.5 months from diagnosis (ipsilateral: eight cases including one malignant tumor; contralateral: 18 cases; both ovaries: three cases). A large palpable mass, bilateral forms, at diagnosis and perioperative rupture had a statistical impact on the risk of second event, whereas the type of surgery or approach did not., Conclusion: This study is a plea in favor of OSS as the first-choice treatment of OMT when possible. Close follow-up during the first 5 years is mandatory considering the risk of 8.3% of second events, especially in cases with risk factors., (© 2021 Wiley Periodicals LLC.)

Published
2022
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