Mortality from gastrointestinal congenital anomalies at 264 hospitals in 74 low-income, middle-income, and high-income countries: a multicentre, international, prospective cohort study

Summary Background Congenital anomalies are the fifth leading cause of mortality in children younger than 5 years globally. Many gastrointestinal congenital anomalies are fatal without timely access to neonatal surgical care, but few studies have been done on these conditions in low-income and middle-income countries (LMICs). We compared outcomes of the seven most common gastrointestinal congenital anomalies in low-income, middle-income, and high-income countries globally, and identified factors associated with mortality. Methods We did a multicentre, international prospective cohort study of patients younger than 16 years, presenting to hospital for the first time with oesophageal atresia, congenital diaphragmatic hernia, intestinal atresia, gastroschisis, exomphalos, anorectal malformation, and Hirschsprung’s disease. Recruitment was of consecutive patients for a minimum of 1 month between October, 2018, and April, 2019. We collected data on patient demographics, clinical status, interventions, and outcomes using the REDCap platform. Patients were followed up for 30 days after primary intervention, or 30 days after admission if they did not receive an intervention. The primary outcome was all-cause, in-hospital mortality for all conditions combined and each condition individually, stratified by country income status. We did a complete case analysis. Findings We included 3849 patients with 3975 study conditions (560 with oesophageal atresia, 448 with congenital diaphragmatic hernia, 681 with intestinal atresia, 453 with gastroschisis, 325 with exomphalos, 991 with anorectal malformation, and 517 with Hirschsprung’s disease) from 264 hospitals (89 in high-income countries, 166 in middleincome countries, and nine in low-income countries) in 74 countries. Of the 3849 patients, 2231 (58·0%) were male. Median gestational age at birth was 38 weeks (IQR 36–39) and median bodyweight at presentation was 2·8 kg (2·3–3·3). Mortality among all patients was 37 (39·8%) of 93 in low-income countries, 583 (20·4%) of 2860 in middle-income countries, and 50 (5·6%) of 896 in high-income countries (p<0·0001 between all country income groups). Gastroschisis had the greatest difference in mortality between country income strata (nine [90·0%] of ten in lowincome countries, 97 [31·9%] of 304 in middle-income countries, and two [1·4%] of 139 in high-income countries; p≤0·0001 between all country income groups). Factors significantly associated with higher mortality for all patients combined included country income status (low-income vs high-income countries, risk ratio 2·78 [95% CI 1·88–4·11], p<0·0001; middle-income vs high-income countries, 2·11 [1·59–2·79], p<0·0001), sepsis at presentation (1·20 [1·04–1·40], p=0·016), higher American Society of Anesthesiologists (ASA) score at primary intervention (ASA 4–5 vs ASA 1–2, 1·82 [1·40–2·35], p<0·0001; ASA 3 vs ASA 1–2, 1·58, [1·30–1·92], p<0·0001]), surgical safety checklist not used (1·39 [1·02–1·90], p=0·035), and ventilation or parenteral nutrition unavailable when needed (ventilation 1·96, [1·41–2·71], p=0·0001; parenteral nutrition 1·35, [1·05–1·74], p=0·018). Administration of parenteral nutrition (0·61, [0·47–0·79], p=0·0002) and use of a peripherally inserted central catheter (0·65 [0·50–0·86], p=0·0024) or percutaneous central line (0·69 [0·48–1·00], p=0·049) were associated with lower mortality. Interpretation Unacceptable differences in mortality exist for gastrointestinal congenital anomalies between lowincome, middle-income, and high-income countries. Improving access to quality neonatal surgical care in LMICs will be vital to achieve Sustainable Development Goal 3.2 of ending preventable deaths in neonates and children younger than 5 years by 2030

Tumeurs frontières de l’ovaire. Recommandations pour la pratique clinique du CNGOF – Fertilité

International audienceObjectivesBorderline ovarian tumors (BOT) represent around 15% of all ovarian neoplasms and are more likely tobe diagnosed in women of reproductive age. Overall, given the epidemiological profile of BOT and theirfavourable prognosis, ovarian function and fertility preservation should be systematically considered in patientspresenting these lesions.MethodsThe research strategy was based on the following terms: borderline ovarian tumor, fertility, fertility preservation,infertility, fertility-sparing surgery, in vitro fertilization, ovarian stimulation, oocyte cryopreservation,using PubMed, in English and French.Results and conclusionsFertility counselling should become an integral part of the clinical management of women with BOT. Patientswith BOT should be informed that surgical management of BOT may cause damage ovarian reserveand/or peritoneal adhesions. Nomogram to predict recurrence, ovarian reserve markers and fertility explorationsshould be used to provide a clear and relevant information about the risk of infertility in patients withBOT. Fertility-sparing surgery should be considered for young women who wish preserving their fertilitywhen possible. There is insufficient evidence to claim a causal relation between controlled ovarian stimulation(COS) and BOT. However, in case of poor prognosis factors, the use of COS should be consideredcautiously through a multidisciplinary approach. In case of infertility after surgery for BOT, COS can be performedwithout delay, once histopathological diagnosis of BOT is confirmed. There is insufficient consistentevidence that fertility drugs and COS increase the risk of recurrence of BOT after conservative management.The conservative surgical treatment can be associated to oocyte cryopreservation considering the high riskof recurrence of the disease. In women with BOT recurrence in a single ovary and in women with bilateralovarian involvement when the conservative management is not possible, other fertility preservation strategiesare available, but still experimental.ObjectifsLes tumeurs frontières de l’ovaire (TFO) représentent 10 à 20 % des tumeurs séreuses de l’ovaire et surviennentdans près d’un tiers des cas chez des femmes âgées de moins de 40 ans, n’ayant pas toujours accomplileur projet conceptionnel. Ainsi, la problématique de la fertilité dans la prise en charge des TFO doit être miseau premier plan.MéthodesUne sélection bibliographique a été réalisée dans PubMed de 1988 à mai 2019 inclus, sur les thématiques :infertilité et TFO, préservation de la fertilité et TFO.Résultats et conclusionsIl est recommandé de proposer une consultation spécialisée de Médecine de la reproduction lors du diagnosticde TFO chez une patiente en âge de procréer. Il est recommandé de délivrer une information complèteaux patientes, sur le risque de baisse de réserve ovarienne faisant suite à un traitement chirurgical de TFO. Ilest recommandé de s’appuyer sur les scores d’évaluation du risque de récidive, l’étude des paramètres d’infertilitéet de réserve ovarienne pour délivrer une information complète quant au risque d’infertilité des patientesprésentant une TFO (grade C). Lorsqu’elle est possible, une stratégie chirurgicale conservatrice est recommandéepour préserver la fertilité des femmes en âge de procréer en cas de TFO (grade C). Après traitementoptimal d’une TFO, il n’existe pas dans la littérature de donnée contre-indiquant formellement le recours àune stimulation ovarienne. Néanmoins, en cas de facteurs pronostics histologiques péjoratifs (implants), lerecours à une stimulation ovarienne sera discuté au cas par cas dans le cadre d’une RCP. En cas d’infertilitéaprès traitement conservateur d’une TFO, il n’existe pas de données justifiant un délai entre le traitementchirurgical et la prise en charge en assistance médicale à la procréation. La stimulation ovarienne dans le cadred’une AMP chez les femmes ayant été traitées de façon conservatrice pour TFO ne semble pas augmenter lerisque de récidive (grade C). En cas de traitement conservateur (chirurgie complète et stadification), il n’existepas de contre-indication dans la littérature à réaliser une stimulation ovarienne en vue d’une vitrificationovocytaire pour préservation de fertilité (PF). En présence de critères histologiques péjoratifs (implants), lapossibilité d’une stimulation ovarienne sera à discuter au cas par cas en RCP avant PF. Pour les femmes dontle traitement chirurgical ne peut être conservateur sur les annexes ou pour les patientes présentant une récidivede TFO sur ovaire unique, plusieurs techniques de préservation de la fertilité sont décrites dans la littératuremais avec des niveaux de preuves insuffisants pour pouvoir les recommander

Testing two competitive models of empathic communication in cancer care encounters: A factorial analysis of the CARE measure

International audienceObjective: The mechanisms associating physician empathy (PE) with patient outcomes remain unclear. PE can be considered as a whole (one process) or three subcomponents can be identified (an establishing rapport process; an emotional process; a cognitive process). The objective was to test two competitive models of PE in cancer care: a three-process model adapted from Neumann's model versus a one-process model, with the use of the Consultation and Relational Empathy measure (CARE). Methods: The CARE was completed by 488 oesogastric cancer patients from the national French database FREGAT. A confirmatory factor analysis (CFA) and a bifactor model were performed to test the two competitive models. Results: The CFA revealed that the one-factor structure showed a moderate fit to the data whereas the three-factor structure showed a good fit. However, the bifactor model favoured unidimensionality. Conclusion: We cannot provide a clear-cut conclusion about whether PE should be considered as on unique process or not. Further work is still needed. Meanwhile, one should not preclude the use of three subscores in cancer care if specific elements of the encounter need to be assessed

Should Preimplantation Genetic Testing (PGT) Systematically Be Proposed to BRCA Pathogenic Variant Carriers?

Over the past years, BRCA genes pathogenic variants have been associated to reproductive issues. Indeed, evidence indicate that BRCA-mutated patients are not only at higher risk of developing malignancies, but may also present a reduction of the follicular stockpile. Given these characteristics, BRCA patients may be candidates to fertility preservation (FP) techniques or preimplantation genetic testing (PGT) to avoid the transmission of this inherited situation. Since the success rates of both procedures are highly related to the number of oocytes that could be recovered after ovarian stimulation, predicted by ovarian reserve tests, they are ideally performed before the diagnosis of cancer and its treatment. Despite the specific reproductive challenges related to BRCA status, no international guidelines for the application of PGT and FP in this subgroup of patients is currently available. The present article aims to review the available data regarding BRCA carriers&rsquo; ovarian reserve and PGT success rates in oncologic and non-oncologic contexts, to determine the actual indication of PGT and further to improve patients&rsquo; care pathway