Long-Term Outcomes of Childhood Acute Lymphocytic Leukemia Treated with Adapted Berlin-Frankfurt-Münster (BFM) Protocols: A Multicentric Analysis from a Developing Country.

Introduction: The objective of the current study was to determine the survival probabilities of children and adolescents with acute lymphocytic leukemia treated with adapted Berlin-Frankfurt-Münster (BFM) protocols and compare our results with the original BFM reports.

Methods: This retrospective study included 695 patients up to 19 years old treated with adapted BFM protocols between 1997 and 2018 in four hospitals in Rio de Janeiro. The 1997-2007 and 2008-2018 cohorts were analyzed separately.

Accuracy of navigation guided implant surgery for immediate loading complete arch restorations: Prospective clinical trial.

Objectives: To assess navigation accuracy for complete-arch implant placement with immediate loading of digitally prefabricated provisional.

Materials And Methods: Consecutive edentulous and terminal dentition patients requiring at least one complete-arch FDP were treated between December 2020 and January 2022. Accuracy was evaluated by superimposing pre-operative and post-operative cone beam computed tomography (CBCT), recording linear (mm) and angular (degrees) deviations.

Intensive salvage chemotherapy with VDTPACE or mCBAD followed by hematopoietic stem-cell support for refractory/relapsed multiple myeloma.

Introduction: Triple- and quad-refractory multiple myeloma patients usually have an aggressive course and a poor prognosis. Available therapeutic options are scarce.

Methods: The objective of the current study was to evaluate responses and toxicities of VDTPACE or mCBAD with hematopoietic stem-cell support as a bridge to subsequent therapies in patients with refractory/relapsed multiple myeloma.

Infection risk in patients with autoimmune cytopenias and immune dysregulation treated with mycophenolate mofetil and sirolimus.

Introduction: Autoimmune cytopenias (AICs) are a group of disorders characterized by immune-mediated destruction of blood cells. In children, they are often secondary to immune dysregulation that may require long-lasting immunosuppression. Mycophenolate mofetil and sirolimus represent two well-tolerated options to treat these disorders, often as a steroid-sparing option.

Impaired Mitochondrial Function and Marrow Failure in Patients Carrying a Variant of the Gene.

Serine/arginine-rich splicing factors (SRSFs) are a family of proteins involved in RNA metabolism, including pre-mRNA constitutive and alternative splicing. The role of SRSF proteins in regulating mitochondrial activity has already been shown for SRSF6, but SRSF4 altered expression has never been reported as a cause of bone marrow failure. An 8-year-old patient admitted to the hematology unit because of leukopenia, lymphopenia, and neutropenia showed a missense variant of unknown significance of the gene (p.

Functional Neuromyofascial Activity: Interprofessional Assessment to Inform Person-Centered Participative Care-An Osteopathic Perspective.

: Health professionals and bodyworkers may be pivotal in promoting prevention programs, providing tailored advice and guidance to patients' adherence to self-care strategies, such as physical activity. Contemporary evidence encourages manual therapists to involve patients in decision-making and treatment procedures integrating passive and active approaches in treatment planning. This manuscript provides a definition and applications of neuromyofascial movement patterns, discusses the significance of functional assessment, and gives an example of clinical applications in the osteopathic field to highlight how this assessment can promote interdisciplinarity.

Myeloablative or reduced-intensity/non-myeloablative hematopoietic cell transplantation for Philadelphia-positive acute lymphoblastic leukemia in adults older than 40 years old - a secondary analysis of a CIBMTR database.

Few studies have addressed the role of reduced-intensity conditioning (RIC) and non-myeloablative (NMA) regimens in older adults with Philadelphia acute lymphoblastic leukemia (Ph + ALL). The objective of this current study was to compare the outcomes of RIC/NMA versus TBI-based myeloablative (MAC) regimens in Ph + ALL patients older than 40 years old who underwent hematopoietic cell transplantation (HCT) in CR1. We used a freely available database from the CIBMTR.

A Nationwide Study of GATA2 Deficiency in Italy Reveals Novel Symptoms and Genotype-phenotype Association.

GATA2 deficiency is a rare disorder encompassing a broadly variable phenotype and its clinical picture is continuously evolving. Since it was first described in 2011, up to 500 patients have been reported. Here, we describe a cohort of 31 Italian patients (26 families) with molecular diagnosis of GATA2 deficiency.

Accuracy of intraoral optical scan versus stereophotogrammetry for complete-arch digital implant impression: An in vitro study.

Purpose: To assess and compare the accuracies of intraoral scanners (IOS) and stereophotogrammetry (SPG) devices for complete-arch digital implant impressions.

Methods: A 4-analog model was digitized using a desk scanner to obtain a reference file. Thirty test scans were conducted using the investigated IOS device, while an additional 30 scans were performed using the SPG device.

Posttransplant cyclophosphamide beyond haploidentical transplantation.

Posttransplant cyclophosphamide (PTCy) has practically revolutionized haploidentical (Haplo) hematopoietic cell transplantation (HCT). Comparisons between Haplo with PTCy and unrelated donor (URD) with conventional graft-versus-host disease (GVHD) prophylaxis have shown comparable overall survival with lower incidences of GVHD with Haplo/PTCy and led to the following question: is it PTCy so good that can be successfully incorporated into matched related donor (MRD) and URD HCT? In this review, we discuss other ways of doing PTCy, PTCy in peripheral blood haploidentical transplants, PTCy in the context of matched related and matched unrelated donors, PTCy with mismatched unrelated donors, and PTCy following checkpoint inhibitor treatment. PTCy is emerging as a new standard GVHD prophylaxis in haploidentical, HLA-matched, and -mismatched HCT.

Total marrow irradiation in hematopoietic stem cell transplantation for hematologic malignancies.

Total body irradiation (TBI) has been an essential component of the conditioning regimen in hematopoietic cell transplantation for many years. However, higher doses of TBI reduce disease relapse at the expense of more significant toxicities. Therefore, total marrow irradiation and total marrow and lymphoid irradiation have been developed to deliver organ-sparing targeted radiotherapy.

Molecular profile of patients with myelofibrosis: a 10-year experience.

Objective: To analyze the karyotype test and myeloid panel with next-generation sequencing findings in patients with myelofibrosis, and to compare transplant characteristics in patients referred for bone marrow transplantation.

Methods: Retrospective, single-center study with patients diagnosed with myelofibrosis treated at Hospital Israelita Albert Einstein between 2010 and 2020.

Results: A total of 104 patients with myelofibrosis were examined.

Autoimmune Neutropenia and Immune-Dysregulation in a Patient Carrying a Variant.

In recent years, the knowledge about the immune-mediated impairment of bone marrow precursors in immune-dysregulation and autoimmune disorders has increased. In addition, immune-dysregulation, secondary to marrow failure, has been reported as being, in some cases, the most evident and early sign of the disease and making the diagnosis of both groups of disorders challenging. Dyskeratosis congenita is a disorder characterized by premature telomere erosion, typically showing marrow failure, nail dystrophy and leukoplakia, although incomplete genetic penetrance and phenotypes with immune-dysregulation features have been described.

The Austrian Osteopathic Practitioners Estimates and RAtes (OPERA): A cross-sectional survey.

Introduction: Since the previous survey of the osteopathic profession in Austria was almost a decade ago, an update was necessary. The Osteopathic Practitioners Estimates and RAtes (OPERA) project was developed as a Europe-based survey, whereby an updated profile of the profession not only provides new data for Austria, but also allows for a clear comparison with other European countries.

Methods: A voluntary, online-based, closed-ended survey was distributed across Austria in the period between April and August 2020.

The Profile of Belgian Osteopaths: A Cross-Sectional Survey.

Background: This study gives an update on the characteristics of Belgian osteopaths five years after the Benelux Osteosurvey. Additional new data were collected on their professional identity and views on the profession.

Methods: All Belgian osteopaths who could be contacted ( = 1473) were invited to complete a voluntary, online-based, closed-ended survey distributed between May and September 2018.

ATG in HLA-Matched, Peripheral Blood, Hematopoietic Cell Transplantation in Acute Myeloid Leukemia and Myelodysplastic Syndrome: A Secondary Analysis of a CIBMTR Database.

w?>Peripheral blood stem cells (PBSC) are the preferred grafts for hematopoietic cell transplantation (HCT), according to the CIBMTR. Donor recovery is faster with PBSC harvest, but PBSC is associated with higher chronic graft-versus-host disease (GVHD) and poorer quality of life. Anti-T-cell globulin (ATG) is polyclonal IgG from rabbits or horses immunized with human thymocytes or a human T-cell line, which may reduce GVHD in HCT and improve outcomes.