The impact of infused red blood cell volume on major and bidirectional ABO-mismatched bone marrow transplantation.

Background Aims: ABO incompatibility does not hinder bone marrow transplantation (BMT), but it has been associated with worse outcomes and additional adverse events. This study aimed to verify the impact of incompatible red blood cells (iRBCs) in allogeneic BMT and to determine a safe number of iRBCs to be infused.

Methods: We compared ABO-incompatible (iABO) allogeneic BMT (n = 42) with ABO-compatible allogeneic BMT (n = 44) and evaluated the impact of the number of infused iRBCs on outcomes and adverse events.

Consecutive Liver and Bone Marrow Transplantation for Erythropoietic Protoporphyria: Case Report and Literature Review.

Background: Erythropoietic protoporphyria (EPP) is a rare inherited disease of heme biosynthesis resulting in the accumulation of protoporphyrin, characterized by liver failure in a minority of cases. Although liver transplant (LT) is the therapeutic strategy for advanced hepatic disease, it does not correct the primary defect, which leads to recurrence in liver graft. Thus, hematopoietic stem cell transplantation (HSCT) is an approach for treating EPP.

Aspects of laboratory characteristics, analysis of alloimmunization, and searching for predictors related to survival in patients with Autoimmune Hemolytic Anemia.

Autoimmune hemolytic anemia (AIHA) is characterized by hemolysis caused by autoantibodies. However, many patients do not respond to therapies and may have an unfavorable outcome. It has been hypothesized that patients with AIHA and alloantibodies have a lower survival compared to patients with this disease and without alloimmunization.

Hereditary hemochromatosis beyond hyperferritinemia: Clinical and laboratory investigation of the patient's profile submitted to phlebotomy in two reference centers in southern Brazil.

Hereditary Hemochromatosis is a disorder characterized by iron deposition in several organs and hyperferritinemia. The most studied variants are linked to the HFE gene. In Brazil, surveys that characterize this population are scarce, with no sampling in the state of Rio Grande do Sul.

High microbiome variability in pediatric tracheostomy cannulas in patients with similar clinical characteristics.

Objectives: To evaluate the bacterial microbiome found in tracheostomy cannulas of a group of children diagnosed with glossoptosis secondary to Robin Sequence (RS), and its clinical implications.

Methods: Pediatric patients were enrolled in the study at the time of the cannula change in the hospital. During this procedure, the removed cannula was collected and stored for amplicon sequencing of 16s rRNA.

Validation of blood components transport through a pneumatic tube system.

Introduction: Urgent blood component transfusions may be life-saving for patients in hemorrhagic shock. Measures to reduce the time taken to provide these transfusions, such as uncrossmatched transfusion or abbreviated testing, are available. However, transport time is still an additional delay and the use of a pneumatic tube system (PTS) may be an alternative to shorten the transport time of blood components.

Instrumental Swallowing Assessments in the Neonatal and Pediatric Populations: A Systematic Review.

The scientific scope of swallowing disorders in the neonatal and pediatric populations is growing exponentially; however, the preponderance of evidence for evaluation protocols has been concentrated in non-instrumental evaluations creating a lack of research about protocols for instrumental swallowing assessment. Thus, the purpose of this study was to systematically review the literature to identify and to report protocols used in instrumental assessments through videofluoroscopic swallow study (VFSS) and fiberoptic endoscopic evaluation of swallowing (FEES) in the neonatal and pediatric populations to support clinical decision making. The search strategy was applied in five online databases, no filters were applied to restrict languages or publication dates and the gray literature was reviewed.

Adoptive immunotherapy with double-bright (CD56 /CD16 ) expanded natural killer cells in patients with relapsed or refractory acute myeloid leukaemia: a proof-of-concept study.

Patients with acute myeloid leukaemia (AML) have a five-year survival rate of 28·7%. Natural killer (NK)-cell have anti-leukaemic activity. Here, we report on a series of 13 patients with high-risk R/R AML, treated with repeated infusions of double-bright (CD56 /CD16 ) expanded NK cells at an academic centre in Brazil.

Convalescent plasma for COVID-19 in hospitalised patients: an open-label, randomised clinical trial.

Background: The effects of convalescent plasma (CP) therapy in hospitalised patients with coronavirus disease 2019 (COVID-19) remain uncertain. This study investigates the effect of CP on clinical improvement in these patients.

Methods: This is an investigator-initiated, randomised, parallel arm, open-label, superiority clinical trial.

Incidence of venous thromboembolism among patients with severe COVID-19 requiring mechanical ventilation compared to other causes of respiratory failure: a prospective cohort study.

Previous studies have suggested that COVID-19 pneumonia is associated with an increased risk of venous thromboembolism (VTE). This study aimed to investigate the incidence of VTE among mechanically ventilated adults with COVID-19 pneumonia, compared to patients with respiratory failure related to other causes. Prospective study that enrolled critically ill adults with suspected COVID-19 pneumonia between June 2, 2020 and August 11, 2020.

Translation and validation of the drooling impact scale questionnaire into Brazilian Portuguese.

Introduction: Inadequate drooling can cause serious clinical, functional and social problems. Validated questionnaires to evaluate drooling impact on quality of life are lacking in Brazilian Portuguese.

Objectives: To translate and validate the drooling impact scale to Brazilian Portuguese.

Diagnosis and treatment of immunological platelet refractoriness by histocompatibility.

Immunological platelet refractoriness occurs when polytransfused patients develop antibodies against donors' HLA class I antigens, HPA (human platelet antigens) and few cases against both systems. Flow cytometry crossmatch with the patient serum against platelets from several donors can determine whether the refractoriness is or is not of immunological origin. Patients with moderate sensitization will be given transfusions from donors with a negative platelets crossmatch; those who are hypersensitized will need to have antibodies assessed against a reactivity panel (RP) for HLA class I and HPA.

Cigarette smoking and antioxidant defences in packed red blood cells prior to storage.

Background: Red blood cells from smoking donors can have more lesions from oxidative stress, decreasing the benefits of blood transfusion. We aimed to explore the effect of cigarette smoking on the oxidative status of packed red blood cells (PRBCs) prior to storage.

Materials And Methods: We compared serum vitamin C, plasmatic malondialdehyde (MDA), and non-protein thiol groups (GSH) levels in PRBCs, as well glutathione peroxidase (GPx) and glutathione s-transferase (GST) activity in PRBCs from smoking (n=36) and non-smoking (n=36) donors.

Toxic elements in packed red blood cells from smoker donors: a risk for paediatric transfusion?

Background And Objectives: Smokers currently have no defined restrictions for blood donation. However, cigarette smoke contains toxic substances such as carbon monoxide (CO) and trace elements that can affect the packed red blood cells (PRBCs) quality and safety of transfusion. This study evaluated the effects of smoking on the concentration of essential and trace elements and on carboxyhemoglobin (COHb) levels in PRBCs from smoker donors.

Upfront treatment for newly diagnosed transplant-ineligible multiple myeloma patients: A systematic review and network meta-analysis of 14,533 patients over 29 randomized clinical trials.

Choice of treatment for newly diagnosed transplant-ineligible multiple myeloma poses a difficult task due to an ever-increasing plethora of different regimens. Attempting to clarify this subject, we performed a systematic review and Bayesian network meta-analysis of 29 randomized clinical trials, enrolling 14,533 patients, and comparing 25 different treatment regimens regarding overall survival(OS), progression-free survival(PFS), complete response(CR), overall response rate(ORR) and toxicity. Head-to-head comparisons for all regimens and ranking of best treatments are reported.

Intraoperative cell salvage with autologous transfusion in liver transplantation.

Liver transplant (LT) is the primary treatment for patients with end-stage liver disease. About 25000 LTs are performed annually in the world. The potential for intraoperative bleeding is quite variable.

Sinonasal computed tomography in pediatric cystic fibrosis: do we know the indications?

Objectives: To investigated the correlation between sinonasal computed tomography (SNCT) findings and sinonasal symptoms, genotype, chronic colonization by Pseudomonas aeruginosa, forced expiratory volume in the first second (FEV1), body mass index (BMI), Shwachman-Kulczycki (SK) score, and Bhalla score in patients with cystic fibrosis.

Methods: We retrospectively reviewed the medical records of 61 patients aged 2-16 years who received care at the Pediatric Cystic Fibrosis Center of our institution over a 10-year period. SNCT findings were graded using the Lund-Mackay scoring system.

Frontline treatment for transplant-eligible multiple myeloma: A 6474 patients network meta-analysis.

Autologous transplantation continues to be the cornerstone of younger and fit multiple myeloma patients. It is known that frontline induction therapy before transplantation can influence post-transplant results. Therefore, best frontline treatment for transplant-eligible patients should be based on best available evidence to guide therapy.

Quantification of peripheral blood CD34 cells prior to stem cell harvesting by leukapheresis: a single center experience.

Background: Due to laboratory logistic issues, our center has traditionally scheduled peripheral blood stem cell harvests based on timing from the start of mobilization. This has proved to be useful in some cases, but also resulted in many fruitless harvests due to poor mobilization. In order to improve the efficiency of collections and compare the effectiveness of peripheral blood CD34 cells as a predictor with data from other reports, this study analyzed the implementation of this routine.

Impact of balloon laryngoplasty on management of acute subglottic stenosis.

Purpose: To assess the impact of balloon laryngoplasty on clinical and surgical outcomes in pediatric patients with acute subglottic stenosis.

Methods: Two case series were included and compared. The first group included patients treated initially either with tracheostomy (if severe symptoms) or with close follow-up (if mild symptoms).

The effect of mandibular distraction osteogenesis on airway obstruction and polysomnographic parameters in children with Robin sequence.

Introduction: The optimal surgical technique for the management of patients with Robin Sequence (RS) has not been established. One of the most commonly used surgical techniques, mandibular distraction osteogenesis (MDO), is still controversial because of its potential risks and the lack of clear evidence of its efficacy.

Objectives: To assess variations in airway patency, clinical symptoms, and polysomnographic parameters in children with RS who underwent MDO.

Association of polysomnographic parameters with clinical symptoms severity grading in Robin sequence patients: a cohort nested cross-sectional study.

Objectives: To evaluate the association of polysomnographic parameters with clinical symptom severity in Robin sequence (RS) patients.

Methods: All patients diagnosed as presenting with RS at Hospital de Clínicas de Porto Alegre from October 2012 to June 2016 were enrolled. They were classified as isolated RS, RS-plus, and syndromic RS.

Diagnostic accuracy of current glossoptosis classification systems: A nested cohort cross-sectional study.

Objectives/hypothesis: To assess the performance of endoscopic grading systems of glossoptosis in identifying severe clinical manifestations in children with Robin sequence (RS).

Study Design: Nested cohort cross-sectional study.

Methods: All RS patients diagnosed at Hospital Clinics of Porto Alegre from October 2012 to June 2016 were enrolled in this cohort.