Réduire les pertes sanguines et les besoins transfusionnels en chirurgie pédiatrique

Purpose: To summarize the physiology and pathophysiology relevant to perioperative blood loss in children. Strategies to reduce blood losses are reviewed. Methods: The literature was reviewed using the electronic library PUBMED and the Cochrane Database of Systematic Reviews. Relevant studies published in English or French with an English abstract are included. The following keywords were used: children, blood transfusion, surgical blood loss, erythropoietin, autologous blood, red blood cell saver, normovolemic hemodilution, desmopressin, aminocaproic acid, tranexamic acid, aprotinin, cardiac surgery, liver transplantation and scoliosis surgery. Main findings: For patients with idiopathic scoliosis, predonation with or without the addition of erythropoietin is a safe and effective way to avoid the use of allogenic blood products. For open heart procedures: whole blood of less than 48 hr is helpful for children of less than two years of age undergoing complex procedures; tranexamic acid may be helpful for cyanotic heart disease and, to a lesser degree, for reoperations; while antikallikrein blood levels of aprotinin may both reduce the need for allogenic blood transfusions and improve postoperative oxygenation in infants. Conclusion: Reducing perioperative allogenic blood transfusions is possible in pediatric patients provided that prophylactic measures are adapted to age, disease and type of surgery. Objectif: Revoir la littérature pertinente à la prise en charge des pertes sanguines péri-opératoires de ľenfant ainsi que les stratégies ďépargne sanguine. Méthode: La littérature a été revue à ľaide de la banque de données électronique PUBMED et du Cochrane Database of Systematic Reviews. Les études pertinentes publiées en langue française ou anglaise avec résumé en langue anglaise ont été revues. Les mots-clés suivants ont été utilisés: enfant, transfusion sanguine, pertes sanguines chirurgicales, érythropoïétine, transfusion autologue préprogrammée, récupérateur de globules rouges, hémodilution isovolémique, desmopressine, acide aminocaproïque, acide tranexamique, aprotinine, chirurgie cardiaque, transplantation hépatique et arthrodèse vertébrale. Constatations principales: Pour les scolioses idiopathiques, la transfusion autologue programmée avec ou sans ľajout ďérythropoïétine réduit ľadministration de sang homologue. Pour les chirurgies à cœur ouvert, le sang complet de moins de 48 h est utile pour les corrections de cardiopathies complexes avant ľâge de deux ans, ľacide tranexamique est utile pour les corrections de cardiopathies cyanogènes et à un degré moindre pour les réinterventions alors que ľaprotinine à dose anti-kallikréïne diminue les besoins transfusionnels et améliore ľoxygénation postopératoire en dessous de ľâge de un an. Conclusion: Il est possible de réduire les transfusions allogènes chez ľenfant si des mesures adaptées à ľâge, à la pathologie et au type de chirurgie sont appliquée

Viscoelastometric Testing to Assess Hemostasis of COVID-19: A Systematic Review

Infection by SARS-CoV-2 is associated with a high risk of thrombosis. The laboratory documentation of hypercoagulability and impaired fibrinolysis remains a challenge. Our aim was to assess the potential usefulness of viscoelastometric testing (VET) to predict thrombotic events in COVID-19 patients according to the literature. We also (i) analyzed the impact of anticoagulation and the methods used to neutralize heparin, (ii) analyzed whether maximal clot mechanical strength brings more information than Clauss fibrinogen, and (iii) critically scrutinized the diagnosis of hypofibrinolysis. We performed a systematic search in PubMed and Scopus databases until 31st December 2020. VET methods and parameters, and patients' features and outcomes were extracted. VET was performed for 1063 patients (893 intensive care unit (ICU) and 170 non-ICU, 44 studies). There was extensive heterogeneity concerning study design, VET device used (ROTEM, TEG, Quantra and ClotPro) and reagents (with non-systematic use of heparin neutralization), timing of assay, and definition of hypercoagulable state. Notably, only 4 out of 25 studies using ROTEM reported data with heparinase (HEPTEM). The common findings were increased clot mechanical strength mainly due to excessive fibrinogen component and impaired to absent fibrinolysis, more conspicuous in the presence of an added plasminogen activator. Only 4 studies out of the 16 that addressed the point found an association of VETs with thrombotic events. So-called functional fibrinogen assessed by VETs showed a variable correlation with Clauss fibrinogen. Abnormal VET pattern, often evidenced despite standard prophylactic anticoagulation, tended to normalize after increased dosing. VET studies reported heterogeneity, and small sample sizes do not support an association between the poorly defined prothrombotic phenotype of COVID-19 and thrombotic events

Type I interferon-mediated autoinflammation due to DNase II deficiency

Microbial nucleic acid recognition serves as the major stimulus to an antiviral response, implying a requirement to limit the misrepresentation of self nucleic acids as non-self and the induction of autoinflammation. By systematic screening using a panel of interferon-stimulated genes we identify two siblings and a singleton variably demonstrating severe neonatal anemia, membranoproliferative glomerulonephritis, liver fibrosis, deforming arthropathy and increased anti-DNA antibodies. In both families we identify biallelic mutations in DNASE2, associated with a loss of DNase II endonuclease activity. We record increased interferon alpha protein levels using digital ELISA, enhanced interferon signaling by RNA-Seq analysis and constitutive upregulation of phosphorylated STAT1 and STAT3 in patient lymphocytes and monocytes. A hematological disease transcriptomic signature and increased numbers of erythroblasts are recorded in patient peripheral blood, suggesting that interferon might have a particular effect on hematopoiesis. These data define a type I interferonopathy due to DNase II deficiency in humans

La thrombopénie immunoallergique induite par l'héparine (évaluation d'un test fonctionnel rapide)

PARIS-BIUP (751062107) / SudocSudocFranceF

Place d'un nouveau dosage automatisé de l'activité du facteur Willebrand dans la stratégie diagnostique de la maladie de Willebrand

PARIS-BIUP (751062107) / SudocSudocFranceF

TEG Platelet Mapping® (intérêt dans le suivi des traitements anti-plaquettaires chez les enfants sous assistance circulatoire de type Excor Berlin Heart®)

CHATENAY M.-PARIS 11-BU Pharma. (920192101) / SudocSudocFranceF

Implementation of the new EUR IVD regulation and relation with ISO15189 accreditation: Guidance is urgently required for haemostasis testing.

On May 26th 2017 the European Parliament and the Council of The European Union adopted the new regulation on in vitro diagnostic medical devices (IVDR)-Regulation EU 2017/746-planned to be applied from May 26th 2022 in substitution to the previous IVD directives (IVDD 98/79 EC). After several health and legal causes due to medical device malfunctions, the European Union (EU) extensively reviewed the previous regulatory, which had remained unchanged since 1998. Aim of the work is to analyse the effects of the new IVDR on the field of haemostasis and thrombosis testing with particular attention to specific clinical conditions. Clinical laboratories will mainly deal with three different situations: (1) Diagnostic test performed with IVDR products used according with clinical indication certified by manufacturers. (2) Diagnostic test performed with certified IVDR products without clinical validation. (3) Diagnostic test performed with reagents classified as Research Use Only (RUO). At present, only few clinical laboratories through different European countries have been prepared to the new IVDR, while many laboratories are not yet aware about crucial aspects of the new process that deeply involves laboratory medicine. In conclusion, each laboratory should be aware of the IVDR certification of the reagents/instruments used in its laboratory. There are several urgent needs regarding IVDR certification: studies about the clinical performance of haemostasis tests, guidelines for LDTs (definition and documentation), internal and external quality controls for the tests recommended/suggested in the guidance/guidelines and finally implementation and/or update of clinical and laboratory guidelines

Inflammation in deep vein thrombosis: a therapeutic target?

International audienceDeep vein thrombosis is a common disease associated with a variety of complications including post-thrombotic syndrome as a late complication. It is now clear that in addition to classical deep vein thrombosis triggers such as blood flow disturbance, hypercoagulability, and vessel wall changes, inflammation has a key role in the pathophysiology of deep vein thrombosis, and there is a close relationship between inflammation and coagulation. As attested by changes in several plasma biomarkers, inflammation may have a significant role in the development of post-thrombotic syndrome. Here, we review the link between inflammation and deep vein thrombosis and thus the potential value of anti-inflammatory and/or anticoagulant drugs in the treatment of deep vein thrombosis and the prevention of post-thrombotic syndrome