The Significance of a Cerebrovascular Accident Outcome Prediction Model for Patients, Family Members, and Health Care Professionals: Qualitative Evaluation Study.

Background: Patients with cerebrovascular accident (CVA) should be involved in setting their rehabilitation goals. A personalized prediction of CVA outcomes would allow care professionals to better inform patients and informal caregivers. Several accurate prediction models have been created, but acceptance and proper implementation of the models are prerequisites for model adoption.

Histological evidence of MAPK pathway activation across subtypes of adult orbital xanthogranulomatous disease irrespective of the detection of oncogenic mutations.

Adult orbital xanthogranulomatous disease (AOXGD) is a spectrum of histiocytoses with four subtypes. Mitogen-activated protein kinase (MAPK) pathway mutations have been detected in various histiocytic neoplasms, little is known about this in AOXGD. Targeted regions of cancer- and histiocytosis-related genes were analyzed and immunohistochemical staining of phosphorylated ERK (pERK), cyclin D1 and PU.

Demographics and additional haematologic cancers of patients with histiocytic/dendritic cell neoplasms.

Aims: The discovery of somatic genetic alterations established many histiocytic disorders as haematologic neoplasms. We aimed to investigate the demographic characteristics and additional haematologic cancers of patients diagnosed with histiocytic disorders in The Netherlands.

Methods And Results: We retrieved data on histiocytosis patients from the Dutch Nationwide Pathology Databank (Palga).

Predicting stroke outcome: A case for multimodal deep learning methods with tabular and CT Perfusion data.

Motivation: Acute ischemic stroke is one of the leading causes of morbidity and disability worldwide, often followed by a long rehabilitation period. To improve and personalize stroke rehabilitation, it is essential to provide a reliable prognosis to caregivers and patients. Deep learning techniques might improve the predictions by incorporating different data modalities.

Proactive and Integrated Management and Empowerment in Parkinson's Disease protocol for a randomised controlled trial (PRIME-UK) to evaluate a new model of care.

Background: People living with Parkinson's disease experience progressive motor and non-motor symptoms, which negatively impact on health-related quality of life and can lead to an increased risk of hospitalisation. It is increasingly recognised that the current care models are not suitable for the needs of people with parkinsonism whose care needs evolve and change as the disease progresses. This trial aims to evaluate whether a complex and innovative model of integrated care will increase an individual's ability to achieve their personal goals, have a positive impact on health and symptom burden and be more cost-effective when compared with usual care.

Clinicogenomic associations in childhood Langerhans cell histiocytosis: an international cohort study.

Langerhans cell histiocytosis (LCH) is a rare neoplastic disorder caused by somatic genetic alterations in hematopoietic precursor cells differentiating into CD1a+/CD207+ histiocytes. LCH clinical manifestation is highly heterogeneous. BRAF and MAP2K1 mutations account for ∼80% of genetic driver alterations in neoplastic LCH cells.

A unique immune signature in blood separates therapy-refractory from therapy-responsive acute graft-versus-host disease.

Acute graft-versus-host disease (aGVHD) is an immune cell‒driven, potentially lethal complication of allogeneic hematopoietic stem cell transplantation affecting diverse organs, including the skin, liver, and gastrointestinal (GI) tract. We applied mass cytometry (CyTOF) to dissect circulating myeloid and lymphoid cells in children with severe (grade III-IV) aGVHD treated with immune suppressive drugs alone (first-line therapy) or in combination with mesenchymal stromal cells (MSCs; second-line therapy). These results were compared with CyTOF data generated in children who underwent transplantation with no aGVHD or age-matched healthy control participants.

Aggressive unifocal bone Langerhans cell histiocytosis with soft tissue extension both responsive to radiotherapy: a case report.

Background: Langerhans cell histiocytosis (LCH) is a rare haematological neoplasm characterized by the accumulation of CD1a, CD207/Langerin histiocytes within inflammatory lesions. LCH can involve any organ, but osteolytic bone lesions are most often encountered. Unifocal bone lesions may regress spontaneously after a thick needle biopsy has been taken.

Effectiveness of a Pharmacist-Led Web-Based Medication Adherence Tool With Patient-Centered Communication: Results of a Clustered Randomized Controlled Trial.

Background: Growing numbers of people use medication for chronic conditions; nonadherence is common, leading to poor disease control. A web-based tool to identify an increased risk for nonadherence with related potential individual barriers might facilitate tailored interventions and improve adherence.

Objective: This study aims to assess the effectiveness of a newly developed tool aimed at improving medication adherence.

ALK-positive histiocytosis: a new clinicopathologic spectrum highlighting neurologic involvement and responses to ALK inhibition.

ALK-positive histiocytosis is a rare subtype of histiocytic neoplasm first described in 2008 in 3 infants with multisystemic disease involving the liver and hematopoietic system. This entity has subsequently been documented in case reports and series to occupy a wider clinicopathologic spectrum with recurrent KIF5B-ALK fusions. The full clinicopathologic and molecular spectra of ALK-positive histiocytosis remain, however, poorly characterized.

Rationale and design to evaluate the PRIME Parkinson care model: a prospective observational evaluation of proactive, integrated and patient-centred Parkinson care in The Netherlands (PRIME-NL).

Background: Culminating evidence shows that current care does not optimally meet the needs of persons with parkinsonism, their carers and healthcare professionals. Recently, a new model of care was developed to address the limitations of usual care: Proactive and Integrated Management and Empowerment in Parkinson's Disease (PRIME Parkinson). From 2021 onwards, PRIME Parkinson care will replace usual care in a well-defined region in The Netherlands.

IL-7 and IL-15 Levels Reflect the Degree of T Cell Depletion during Lymphopenia and Are Associated with an Expansion of Effector Memory T Cells after Pediatric Hematopoietic Stem Cell Transplantation.

Differentially and functionally distinct T cell subsets are involved in the development of complications after allogeneic hematopoietic stem cell transplantation (HSCT), but little is known about factors regulating their recovery after HSCT. In this study, we investigated associations between immune-regulating cytokines, T cell differentiation, and clinical outcomes. We included 80 children undergoing allogeneic HSCT for acute leukemia using bone marrow or peripheral blood stem cells grafted from a matched sibling or unrelated donor.

Successful mismatched hematopoietic stem cell transplantation for pediatric hemoglobinopathy by using ATG and post-transplant cyclophosphamide.

The use of HLA-mismatched (un)related donors is historically associated with a higher incidence of transplant-related complications and mortality. However, the use of such donors may overcome the limited availability of HLA-matched donors for patients with β-thalassemia major (TM) and sickle cell disease (SCD). We investigated hematopoietic stem cell transplantation (HSCT) outcomes of pediatric TM and SCD patients treated with a mismatched donor using a treosulfan-based conditioning in combination with ATG and post-transplant cyclophosphamide (PT-CY) and compared these results to the clinical outcome of patients treated by matched donor HSCT without PT-CY.

The Impact of Sex and Gender on the Multidisciplinary Management of Care for Persons With Parkinson's Disease.

The impact of sex and gender on disease incidence, progression, and provision of care has gained increasing attention in many areas of medicine. Biological factors-sex-and sociocultural and behavioral factors-gender-greatly impact on health and disease. While sex can modulate disease progression and response to therapy, gender can influence patient-provider communication, non-pharmacological disease management, and need for assistance.

Personalized Care Management for Persons with Parkinson's Disease.

There is a growing awareness that delivery of integrated and personalized care is necessary to meet the needs of persons living with Parkinson's disease. In other chronic diseases than Parkinson's disease, care management models have been deployed to deliver integrated and personalized care, yielding positive effects on patients' health outcomes, quality of life and health care utilization. However, care management models have been highly heterogeneous, as there is currently no clear operationalization of its core elements.

Spectrum of histiocytic neoplasms associated with diverse haematological malignancies bearing the same oncogenic mutation.

Histiocytic disorders are a spectrum of rare diseases characterised by the accumulation of macrophage-, dendritic cell-, or monocyte-differentiated cells in various tissues and organs. The discovery of recurrent genetic alterations in many of these histiocytoses has led to their recognition as clonal neoplastic diseases. Moreover, the identification of the same somatic mutation in histiocytic lesions and peripheral blood and/or bone marrow cells from histiocytosis patients has provided evidence for systemic histiocytic neoplasms to originate from haematopoietic stem/progenitor cells (HSPCs).

Personalized care management for persons with Parkinson's disease: A telenursing solution.

Poor recognition and inadequate treatment of motor and non-motor symptoms negatively impact on the quality of life of persons with Parkinson's Disease (PD). Furthermore, failure to incorporate timely detection and management of symptoms increases the risk of partially avoidable complications. A promising approach to overcome these pitfalls is telenursing, which entails proactive care delivery by a PD Nurse Specialist (PDNS) through telephone contacts.

Understanding the complexities of Bluetooth for representing real-life social networks: A methodology for inferring and validating Bluetooth-based social network graphs.

Bluetooth (BT) data has been extensively used for recognizing social patterns and inferring social networks, as BT is widely present in everyday technological devices. However, even though collecting BT data is subject to random noise and may result in substantial measurement errors, there is an absence of rigorous procedures for validating the quality of the inferred BT social networks. This paper presents a methodology for inferring and validating BT-based social networks based on parameter optimization algorithm and social network analysis (SNA).

Bone marrow-derived myeloid progenitors as driver mutation carriers in high- and low-risk Langerhans cell histiocytosis.

Langerhans cell histiocytosis (LCH) is a myeloid neoplasia, driven by sporadic activating mutations in the MAPK pathway. The misguided myeloid dendritic cell (DC) model proposes that high-risk, multisystem, risk-organ-positive (MS-RO+) LCH results from driver mutation in a bone marrow (BM)-resident multipotent hematopoietic progenitor, while low-risk, MS-RO- and single-system LCH would result from driver mutation in a circulating or tissue-resident, DC-committed precursor. We have examined the CD34+c-Kit+Flt3+ myeloid progenitor population as potential mutation carrier in all LCH disease manifestations.

Proactive and Integrated Management and Empowerment in Parkinson's Disease: Designing a New Model of Care.

Parkinson's disease is the second most common neurodegenerative condition after Alzheimer's disease. The number of patients will rise dramatically due to ageing of the population and possibly also due to environmental issues. It is widely recognised that the current models of care for people with Parkinson's disease or a form of atypical parkinsonism lack continuity, are reactive to problems rather than proactive, and do not adequately support individuals to self-manage.

Proceeding of the European Group for Blood and Marrow Transplantation (EBMT) congress on sickle cell disease, 16-17 may 2019, Regensburg, Germany: What is the impact of antithymocyte globulin pharmacokinetics on haploidentical hematopoietic stem cell transplantation?

Antithymocyte globulin (ATG) is a widely accepted part of the conditioning regimen applied in the setting of hematopoietic stem cell transplantation (HSCT) to prevent graft rejection and graft-versus-host disease. Although weight-based dosing of ATG has been introduced to optimize ATG dosing, substantial variance in clearance of active ATG, the actual lymphocyte binding component, remains a challenge. Therefore, further research regarding ATG pharmacokinetics and pharmacodynamics in different HSCT settings and in patients with different types of underlying diseases is required.