Abstract Very early onset inflammatory bowel disease (VEOI BD) is a rare entity in pediatrics. Its association with pri mary immunodeficiencies of monogenic origin is known. We present the case of a patient diagnosed with VEOIBD who underwent massive paralleled exome sequencing. The result of the study showed a pathogenic variant in the RET proto-oncogene, associated with multiple endo crine neoplasia type 2A disease. There are no previous reports of association of RET proto-oncogene variants with VEOIBD. The presence of these two clinical entities cannot be attributed to a single genetic cause.

Resumen La enfermedad inflamatoria intestinal de inicio muy temprano (VEOIBD) es una entidad rara en pediatría. Es conocida su asociación con inmunodeficiencias prima rias de origen monogénico. Presentamos el caso de una paciente con diagnóstico de VEOIBD a quien se le realizó una secuenciación masiva del exoma. El resultado del estudio permitió identificar una variante patogénica en el proto oncogen RET, asociada con enfermedad neoplasia endocrina múltiple tipo 2A. No hay reportes de asociación de variantes en el proto oncogen RET con VEOIBD. No se puede adjudicar la presencia de estas dos entidades clínicas a una única causa genética.

Abstract Patient Blood Management (PBM) is a holistic approach to managing blood as a resource of each patient; it is a multimodal strategy that is implemented using a set of techniques that can be applied in individual cases. In fact, the overall result of the implementation of PBM cannot be fully appreciated or explained by simply summing up the effects of the individual strategies and techniques used, since they can only produce the expected ideal result if combined. Implementing a PBM program in healthcare offers several benefits including improved patient safety, better outcomes, cost savings, conservation of resources, evidence-based practice, transfusion alternatives, improved quality of care, compliance with accreditation standards, patient-centered care, and professional education and training. (PBM cases fact used combined safety outcomes savings resources evidencebased evidence based practice alternatives care standards patientcentered centered training

Abstract Managing the patient's blood and hematopoietic system is like managing any of the other organs and organ systems during patient care. Specialists control the heart, kidneys, endocrine system, etc. and the patient's blood requires similar clinical treatment. The hematopoietic system and its circulatory products are fundamental for the healthy functioning of the human body. In simple terms, Patient Blood Management (PBM) is an organized, patient-centered approach in which the entire healthcare team coordinates efforts to improve outcomes by managing and preserving the patient's own blood. By reducing dependence on blood transfusions, PBM seeks to improve clinical outcomes, reduce the risks and costs associated with transfusions, and improve the safety and quality of patient care. Essentially, the concept of PBM is about the holistic management and preservation of the patient's own blood in the medical and surgical context. patients s care heart kidneys etc treatment body terms (PBM organized patientcentered centered transfusions Essentially context

Abstract Autologous blood transfusion can be achieved through different techniques, including by the patient donating blood before surgery (pre-deposit), collecting blood from the patient immediately before the operation and replacing the volume with colloids or plasma expanders (acute normovolemic hemodilution) or through the salvage of lost blood, during or immediately after surgery, and its retransfusion after washing (intraoperative or postoperative recovery). We will focus on the two methods used intraoperatively that are of fundamental importance in the management and conservation of the patient's own blood. techniques predeposit, predeposit pre deposit , (pre-deposit) acute hemodilution intraoperative recovery. recovery . recovery) patients s (pre-deposit

Abstract Understanding the physiological concepts of oxygen delivery is essential to discern the mechanisms that influence its increase, reduction or maintenance in the body. This text explores the different mechanisms that help maintain oxygen delivery even in the face of reduced hemoglobin levels. Adequate oxygen delivery ensures tissue and metabolic balance, which is crucial to avoid harmful consequences such as metabolic acidosis and cellular dysoxia. The complex interaction between variables such as cardiac output, hemoglobin and heart rate (HR) plays a fundamental role in maintaining oxygen delivery, allowing the body to temporarily adjust to situations of anemia or high metabolic demand. It is important to emphasize that blood transfusions should not be based on fixed values, but rather on individual metabolic needs. Strategies to reduce myocardial consumption and monitor macro and micro hemodynamics help in making rational decisions. Individualizing treatment and considering factors such as blood viscosity in relation to the benefits of transfusion are increasingly relevant to optimize therapy and minimize risks, especially in complex clinical scenarios, such as neurocritical patients and trauma victims. increase levels balance dysoxia output HR (HR demand values needs decisions risks scenarios victims

Abstract The management of major bleeding is a critical aspect of modern healthcare and it is imperative to emphasize the importance of applying Patient Blood Management (PBM) principles. Although transfusion support remains a vital component of bleeding control, treating severe bleeding goes beyond simply replacing lost blood. A more comprehensive, multidisciplinary approach is essential to optimize patient outcomes and minimize the risks associated with excessive transfusions. PBM (PBM principles control blood comprehensive transfusions

Abstract Hemostasis plays a critical role in surgical procedures and is essential for a successful outcome. Advances in hemostatic agents offer new approaches to controlling bleeding thereby making surgeries safer. The appropriate choice of these agents is crucial. Volume replacement, another integral part of Patient Blood Management (PBM), maintains adequate tissue perfusion, preventing cellular damage. Individualization in fluid administration is vital with the choice between crystalloids and colloids depending on each case. Colloids, unlike crystalloids, increase oncotic pressure, contributing to fluid retention in the intravascular space. Understanding these aspects is essential to ensure safe and effective surgery, minimizing complications related to blood loss and maintaining the patient's hemodynamic status. outcome safer crucial replacement PBM, PBM , (PBM) perfusion damage case Colloids pressure space surgery patients patient s status (PBM

Abstract Postoperative anemia is a complex clinical issue that requires attention due to its ramifications on the patient's recovery and prognosis. Originating from multiple determinants, such as intraoperative blood loss, hemolysis, nutritional deficiencies, systemic inflammation and impact on the bone marrow, postoperative anemia has varied and often challenging presentations. Patients undergoing major surgical procedures, in particular, are susceptible to developing anemia due to the considerable associated blood loss. Accurate diagnosis plays a crucial role in the approach, requiring meticulous hematological analysis, including hemoglobin, hematocrit and reticulocyte count, as well as an in-depth investigation of the underlying causes. An additional challenge arises in the form of the excessive practice of phlebotomy during hospitalization for clinical monitoring. Although it is essential to assess the progression of anemia, frequent removal of blood may contribute to iatrogenic anemia, further delaying recovery and possibly increasing susceptibility to infection. patients patient s prognosis determinants loss hemolysis deficiencies marrow presentations procedures particular approach analysis hemoglobin count indepth depth causes monitoring infection

Abstract Hemostasis plays a critical role in surgical procedures and is essential for a successful outcome. Advances in hemostatic agents offer new approaches to controlling bleeding thereby making surgeries safer. The appropriate choice of these agents is crucial. Volume replacement, another integral part of Patient Blood Management (PBM), maintains adequate tissue perfusion, preventing cellular damage. Individualization in fluid administration is vital with the choice between crystalloids and colloids depending on each case. Colloids, unlike crystalloids, increase oncotic pressure, contributing to fluid retention in the intravascular space. Understanding these aspects is essential to ensure safe and effective surgery, minimizing complications related to blood loss and maintaining the patient's hemodynamic status. outcome safer crucial replacement PBM, PBM , (PBM) perfusion damage case Colloids pressure space surgery patients patient s status (PBM

Abstract The use of strategies to reduce blood loss and transfusions is essential in the treatment of surgical patients, including in complex cardiac surgeries and those that use cardiopulmonary bypass. Antifibrinolytics, such as epsilon-aminocaproic acid (EACA) and tranexamic acid (TXA), are widely used in these procedures, as well as in other types of surgeries. These medicines are included in the World Health Organization (WHO) list of ‘essential medicines’. Scientific evidence demonstrates the effectiveness of EACA in reducing bleeding and the need for transfusions in heart surgery. EACA is highly recommended for use in heart surgery by the American Society of Anesthesiology Task Force on Perioperative Blood Management. Regarding the safety of EACA, there is no robust evidence of any significant thrombotic potential. TXA has also been shown to be effective in reducing the use of blood transfusions in cardiac and non-cardiac surgeries and is considered safer than other antifibrinolytic agents. There is no evidence of any increased risk of thromboembolic events with TXA, but doses greater than 2 g per day have been associated with an increased risk of seizures. It is also important to adjust the dose in patients with renal impairment. In conclusion, antifibrinolytics, such as EACA and TXA, are effective in reducing blood loss and transfusion use in cardiac and non-cardiac surgeries, without causing serious adverse effects. bypass Antifibrinolytics epsilonaminocaproic epsilon aminocaproic (EACA , (TXA) procedures WHO (WHO medicines. . medicines’ Management potential noncardiac non agents seizures impairment conclusion antifibrinolytics effects (TXA

Abstract Patient Blood Management (PBM) is a multidimensional approach that seeks to optimize the use of blood and its components in patients. This matter emerged as a response to the need to reduce unnecessary exposure to blood transfusions and their potential risks. In the past, blood transfusion was often overused resulting in complications and high costs. The advent of Patient Blood Management has caused a paradigm shift, highlighting anemia prevention, bleeding control and maximizing the production of blood cells by the organism itself. Patient Blood Management guidelines include the early identification of anemia, strategies to minimize blood loss during surgery, intraoperative blood conservation techniques, preoperative hemoglobin optimization and evidence-based approaches to the rational use of blood transfusions. Aiming to improve clinical outcomes, decrease transfusion-related complications and reduce associated costs, this multidisciplinary approach counts on doctors, nurses, pharmacists and other healthcare professionals. Based on research and clinical evidence, Patient Blood Management continues to evolve thereby promoting safer, more effective patient-centered practices. Its implementation has proven beneficial in various medical contexts thereby contributing to improvements in the quality of care provided to patients. Our goal with this Consensus is to present readers with a broad and diverse view of Patient Blood Management so that they have the building blocks to implement this new technique. PBM (PBM patients risks past costs shift prevention itself surgery techniques evidencebased evidence based outcomes transfusionrelated related doctors nurses professionals safer patientcentered patient centered practices technique

Abstract Anemia is a pathological condition in which the hemoglobin and red blood cell mass decrease; it is mainly defined by the concentration of hemoglobin in the blood. The World Health Organization guidelines establish specific values to define anemia in different population groups. Early detection of anemia can also be a valuable indicator of underlying medical conditions. Clinical studies have explored the relationship between perioperative anemia and morbidity, highlighting the need for more judicious therapeutic strategies, such as the use of Patient Blood Management, which aims to prevent and treat anemia in a personalized and effective way. Patient Blood Management emerges as a promising approach to dealing with anemia, recognizing that its correction through transfusion always carries risks and that personalized prevention and treatment can offer better outcomes for patients. decrease groups conditions morbidity strategies way patients

Abstract Managing anemia before surgery is extremely important as it is a clinical condition that can significantly increase surgical risk and affect patient outcomes. Anemia is characterized by a reduction in the number of red blood cells or hemoglobin levels leading to a lower oxygen-carrying capacity of the blood. Proper treatment requires a multifaceted approach to ensure patients are in the best possible condition for surgery and to minimize potential complications. The challenge is recognizing anemia early and implementing a timely intervention to correct it. Anemic patients are more susceptible to surgical complications such as increased infection rates, slower wound healing and increased risk of cardiovascular events during and after surgery. Additionally, anemia can exacerbate existing medical conditions, causing greater strain on organs and organ systems. To correct anemia and optimize patient outcomes, several essential measures must be taken with the most common being identifying and correcting iron deficiency. outcomes oxygencarrying oxygen carrying rates Additionally conditions systems deficiency

Abstract Managing coagulation disorders and potential bleeding risks, especially in the context of anticoagulant medications, is of immense value both clinically and prior to surgery. Coagulation disorders can lead to bleeding complications, affecting patient safety and surgical outcomes. The use of Patient Blood Management protocols offers a comprehensive, evidence-based approach that effectively addresses these challenges. The problem is to find a delicate balance between preventing thromboembolic events (blood clots) and reducing the risk of bleeding. Anticoagulant medications, although crucial to preventing clot formation, can increase the potential for bleeding during surgical procedures. Patient blood management protocols aim to optimize patient outcomes by minimizing blood loss and unnecessary transfusions. risks medications surgery complications comprehensive evidencebased evidence based challenges clots formation procedures transfusions

Abstract The preoperative clinical and laboratory evaluations of the patient is an essential step to ensure the safety and success of any surgical procedure. This assessment aims to identify any underlying medical conditions and risk factors and determine suitability for surgery. With this step, the medical team can adapt the care plan to meet each patient's specific needs, increasing the chances of a successful procedure. Good clinical assessment and comprehensive laboratory testing, when integrated into a Patient Blood Management approach, are invaluable in promoting safety of care, reducing transfusion risks, improving surgical outcomes, and optimizing resource utilization. This approach not only elevates the quality of care, but is also aligned with evidence-based practice and patient-centered principles, making it an essential component of the perioperative process. procedure surgery patients s needs testing risks outcomes utilization evidencebased evidence based patientcentered centered principles process