Copper Deficiency in Children With Intestinal Failure: Risk Factors and Influence on Hematological Cytopenias.

Background: We investigated to what extent serum copper influences hemoglobin (Hb), neutrophil, and platelet counts and the factors associated with serum copper in children with intestinal failure (IF) who have their micronutrient status monitored according to a standard protocol.

Methods: Children with IF admitted to a pediatric intestinal rehabilitation program and receiving home parenteral nutrition (PN) were followed up prospectively. Patients received vitamins and multi-trace elements (TEs) as part of the PN.

Correction: Worldwide Network for Blood and Marrow Transplantation (WBMT) perspective: the role of biosimilars in hematopoietic cell transplant: current opportunities and challenges in low- and lower-middle income countries.

An amendment to this paper has been published and can be accessed via a link at the top of the paper.

Worldwide Network for Blood and Marrow Transplantation (WBMT) perspective: the role of biosimilars in hematopoietic cell transplant: current opportunities and challenges in low- and lower-middle income countries.

Health care costs attributed to biologics have increased exponentially in the recent years, thus biosimilars offer a possible solution to limit costs while maintaining safety and efficacy. Reducing expenditure is vital to health care especially in developing countries where affordability and access to health care is a major challenge. We discuss the opportunities and the challenges of biosimilars in the field of hematopoietic cell transplantation (HCT) in low- and lower-middle income countries.

Worldwide Network for Blood and Marrow Transplantation (WBMT) recommendations for establishing a hematopoietic cell transplantation program (Part I): Minimum requirements and beyond.

Hematopoietic cell transplantation (HCT) is a highly complex procedure that requires a dedicated multidisciplinary team to optimize its safety. In addition, institutions may have different needs regarding indications based on regional disease prevalence or may have an interest in developing specialized services. Yet, structured recommendations are not commonly available.

Brazilian consensus on non-cystic fibrosis bronchiectasis.

Bronchiectasis is a condition that has been increasingly diagnosed by chest HRCT. In the literature, bronchiectasis is divided into bronchiectasis secondary to cystic fibrosis and bronchiectasis not associated with cystic fibrosis, which is termed non-cystic fibrosis bronchiectasis. Many causes can lead to the development of bronchiectasis, and patients usually have chronic airway symptoms, recurrent infections, and CT abnormalities consistent with the condition.

Is Preoperative Preparation Time a Barrier to Small Children Being Ready for Kidney Transplantation?

Background: Small children are less frequently transplanted when compared with older. The objective of the present study was to compare the preparation time for transplantation in children of different weights and to identify factors associated with a delay in the workup of small children.

Methods: We report on a retrospective cohort comprising all children referred for renal transplantation (RTx) workup between 2009 and 2017.

Collaborative Brazilian pediatric renal transplant registry (CoBrazPed-RTx): A report from 2004 to 2018.

The Brazilian collaborative registry for pediatric renal transplantation began in 2004 as a multicenter initiative aimed at analyzing, reporting, and disseminating the results of pediatric renal transplantation in Brazil. Data from all pediatric renal transplants performed from January 2004 to May 2018 at the 13 participating centers were analyzed. A total of 2744 pediatric renal transplants were performed in the thirteen participating centers.

Allogeneic Bone Marrow Transplants for Pediatric Severe Aplastic Anemia: Real-world Data comparing Matched Related and Unrelated Donors in a Developing Country. Retrospective study on behalf of the Pediatric Hematopoietic Stem Cell Transplant Working Group of the Brazilian Bone Marrow Transplantation Society (SBTMO) and the Brazil-Seattle Consortium (Gedeco).

In this study, we report on major MRD or URD BMT outcomes in pediatric patients with SAA in Brazil. This was a retrospective study, which included 106 patients ≤18 years old who received a first BMT for SAA. All patients received bone marrow as graft source from an MRD (n = 69) or a URD (n = 37).

The Value of T1 Mapping Techniques in the Assessment of Myocardial Interstitial Fibrosis.

Cardiac fibrosis, characterized by net accumulation of extracellular matrix in the myocardium, is a common final pathway of heart failure. This myocardial fibrosis (MF) is not necessarily the primary cause of dysfunction; it often results from a reparative process activated in response to cardiomyocyte injury. In light of currently available treatments, late-identified MF could be definitive or irreversible, associated with worsening ventricular systolic function, abnormal cardiac remodeling, and increased ventricular stiffness and arrhythmia.

Treatment of Human Immunodeficiency Virus Infection With Tenofovir Disoproxil Fumarate-Containing Antiretrovirals Maintains Low Bone Formation Rate, But Increases Osteoid Volume on Bone Histomorphometry.

Bone mineral density (BMD) loss is a known complication of human immunodeficiency virus (HIV) infection and its treatment, particularly with tenofovir disoproxil fumarate (TDF)-containing antiretroviral regimens. Although renal proximal tubular dysfunction and phosphaturia is common with TDF, it is unknown whether BMD loss results from inadequate mineralization. We evaluated change in BMD by dual-energy X-ray absorptiometry (DXA) and bone histomorphometry by tetracycline double-labeled transiliac crest biopsies in young men living with HIV before (n = 20) and 12 months after (n = 16) initiating TDF/lamivudine/efavirenz.

Pharmacokinetics analysis results are similar for oral compared to intravenous busulfan in patients undergoing hematopoietic stem cell transplantation, except for the earlier onset of mucositis. A controlled clinical study.

Busulfan is used in myeloablative schemes for hematopoietic stem cell transplantation (HSCT), with monitoring of dosage through the area under the curve (AUC) of the drug plasma concentration (µMol min). In this study, we compared the complete pharmacokinetics of busulfan administered orally (Bu-Oral) and intravenously (Bu-IV). We evaluated 40 patients who underwent HSCT with different types of conditioning regimens.

Worldwide Network for Blood and Marrow Transplantation Recommendations for Establishing a Hematopoietic Cell Transplantation Program, Part I: Minimum Requirements and Beyond.

Hematopoietic cell transplantation (HCT) is a highly complex procedure that requires a dedicated multidisciplinary team to optimize safety. In addition, institutions may have different needs regarding indications based on regional disease prevalence or may have an interest in developing specialized services. Structured recommendations are not commonly available, however.

Development of a risk score for earlier diagnosis of chronic kidney disease in children.

Objective: To develop a clinical score for the early identification of chronic kidney disease (CKD) in children and adolescents. The early diagnosis of CKD in childhood allows the adoption of measures to slow the progression of the disease, thereby reducing morbidity and mortality. Nevertheless, the diagnosis is often made too late for proper patient management.

"Worldwide Network for Blood & Marrow Transplantation (WBMT) special article, challenges facing emerging alternate donor registries".

Hematopoietic cell transplantation (HCT) activity is increasing at an unprecedented pace with > 50,000 allogeneic transplants occurring annually worldwide. Establishing a functional HCT donor registry can be very challenging with respect to ethnicities, financial, technical, and geopolitical issues. Extensive planning steps are essential to overcome the expected challenges while establishing the registry, and to maintain its functionality.

Effects of parathyroidectomy on the biology of bone tissue in patients with chronic kidney disease and secondary hyperparathyroidism.

Secondary hyperparathyroidism is a complication of chronic kidney disease that compromises skeletal integrity. In patients with secondary hyperparathyroidism undergoing parathyroidectomy, parathyroid hormone levels dramatically decrease. The effects of parathyroidectomy on bone tissue are poorly understood, especially regarding the proteins expressed by osteocytes, such as fibroblast growth factor 23, dentin matrix protein 1, matrix extracellular phosphoglycoprotein, sclerostin, receptor activator of nuclear factor kappa B ligand (RANKL) and osteoprotegerin, which regulate bone turnover.

Chronic kidney disease is associated with low BMD at the hip but not at the spine.

Unlabelled: Although chronic kidney disease is associated with other bone disorders, osteoporosis can be found in this context, and it is defined based on bone mineral density (BMD), measured by dual-energy X-ray absorptiometry. As CKD progresses, the percentage of normal BMD decreases, whereas that of osteopenia/osteoporosis increases, mostly due to hip involvement, particularly in patients with reduced renal function.

Introduction: Osteoporosis is a highly prevalent disease in patients with chronic kidney disease (CKD).

Optimal testing policies for diagnosing patients with intermediary probability of disease.

This paper proposes a stochastic shortest path approach to find an optimal sequence of tests to confirm or discard a disease, for any prescribed optimality criterion. The idea is to select the best sequence in which to apply a series of available tests, with a view at reaching a diagnosis with minimum expenditure of resources. The proposed approach derives an optimal policy whereby the decision maker is provided with a test strategy for each a priori probability of disease, aiming to reach posterior probabilities that warrant either immediate treatment or a not-ill diagnosis.

Thromboembolic Events Related to Treatment with Checkpoint Inhibitors: Report of Two Cases.

Despite the significant clinical benefits, checkpoint inhibition is associated with a unique spectrum of immune-related adverse events. It is sometimes difficult to distinguish some rare adverse effects from a cancer progression; thus, such effects should be reported in clinical trials to be diagnosed by physicians. Only a few cases of arterial embolic events have been described in studies related to patients treated by immunotherapy.