Publications by authors named "BOS C"

Background: The distal convoluted tubule (DCT) plays an indispensable role in magnesium (Mg) reabsorption in the kidney. Yet, the extrusion mechanism of Mg has not been identified. The solute carrier 41A3 (SLC41A3) has been suggested to be involved in Mg extrusion, but this has never been conclusively demonstrated.

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Autologous hematopoietic stem cell transplantation is used to restore bone marrow function after high-dose chemotherapy. For apheresis, granulocyte colony-stimulating factor (G-CSF) is standard of care, but obtaining sufficient stem cells can be challenging. Other mobilization agents include plerixafor and PEGylated G-CSF (PEG-G-CSF).

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Immune checkpoint inhibition (ICI) has significantly advanced the field of immuno-oncology, yet not all patients benefit from this therapy. Combining ICI with other therapeutic modalities, including tumor ablation, is currently being explored as a method to enhance ICI efficacy. Mechanical High-Intensity Focused Ultrasound (M-HIFU) represents a promising tumor ablative therapy, inducing cavitation within the tumor, resulting in tumor cell destruction and the release of danger signals and tumor antigens, two key factors contributing to anti-tumor immune responses.

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  • Juvenile xanthogranuloma (JXG) is a type of skin tumor mostly found in children, but some cases occur in other parts of the body, and their genetic causes are not fully understood.* -
  • A study analyzed 16 children and 5 adults with extracutaneous JXG using advanced sequencing, finding that numerous genetic alterations, especially related to kinase fusions, were present in most children.* -
  • The findings indicate that while surgery is common for treatment, some cases can regress on their own, and specific genetic alterations could help inform better treatment options in the future.*
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  • * Researchers used intestine-specific TRPM6 knockout mice to examine magnesium absorption after a 4-day omeprazole treatment, finding that these knockout mice had lower Mg absorption and serum levels compared to control mice.
  • * Ultimately, the results suggest that TRPM6 isn't impacted by short-term PPI use for magnesium absorption in the intestines, as no significant changes in magnesium homeostasis were observed.
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Infections lead to substantial morbidity during the treatment of acute lymphoblastic leukemia (ALL) in which the adaptive immune system is severely affected, leading to declining serum immunoglobulin levels. We performed a trial to investigate whether intravenous immunoglobulin (IVIG) prophylaxis in pediatric patients with ALL could prevent admissions for fever. This randomized controlled trial was a subtrial of the national Dutch multicenter ALL study.

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Background: Some clients who access healthcare services experience problems due to the wider determinants of health which cannot be addressed (solely) by the medical sector. Social Prescribing (SP) addresses clients ' wider health needs and is based on linkworkers who support primary care clients in accessing social, community and voluntary care services that support their needs. Previous literature has provided valuable insights about what works (or not) in an early stage of implementing SP.

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  • Mutations in the PKD1 and PKD2 genes lead to autosomal dominant polycystic kidney disease (ADPKD), which causes fluid-filled cysts in the kidneys and can result in low blood calcium and magnesium in some patients.
  • The study used a specific mouse model (iKsp-Pkd1) to investigate whether inhibiting ATP release, through the pannexin-1 blocker brilliant blue-FCF (BB-FCF), could help normalize these electrolyte levels in ADPKD.
  • Despite treatment with BB-FCF reducing the expression of certain ion channels, it did not successfully normalize serum calcium and magnesium levels in the mice, nor did it show any protective effects on kidney function.
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Objective: T2-relaxometry could differentiate between physiological and haemorrhagic joint effusion (≥ 5% blood) in vitro. Are quantitative T2-relaxation time measurements of synovial fluid feasible and reproducible in vivo in clinically bleed-free joints of men with haemophilia?

Materials And Methods: In this cross-sectional study, we measured T2-relaxation times of synovial fluid in clinically bleed-free ankles, knees or elbows of men with severe haemophilia A using a T2-mapping sequence (duration ≤ 7 min) at 3 Tesla MRI. Manual and circular regions of interest (ROI) were drawn in the synovial fluid of each joint by two independent observers to measure T2-relaxation times.

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Calciprotein particles (CPPs) provide an efficient mineral buffering system to prevent the complexation of phosphate and calcium in the circulation. However, in chronic kidney disease (CKD), the phosphate load exceeds the mineral buffering capacity, resulting in the formation of crystalline CPP2 particles. CPP2 have been associated with cardiovascular events and mortality.

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Aim: Suboptimal self-management with controller inhalation therapy in asthma and COPD is frequently observed with poor treatment outcomes. The developed 'Respiratory Adherence Care Enhancer' (RACE) instrument identifies and addresses individual barriers to self-management with a theoretical underpinning. This study investigates the feasibility of pharmaceutical support with this instrument.

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Purpose: The primary objective of this randomized study was to determine whether a continuous dosing schedule (without the asparaginase-free interval) would result in less hypersensitivity reactions to PEGasparaginase (PEGasp) compared with the standard noncontinuous dosing schedule.

Methods: Eight hundred eighteen patients (age 1-18 years) with ALL were enrolled in the Dutch Childhood Oncology Group-ALL11 protocol and received PEGasp. Three hundred twelve patients stratified in the medium-risk arm were randomly assigned to receive 14 individualized PEGasp doses once every two weeks in either a noncontinuous or continuous schedule after the first three doses in induction (EudraCT: 2012-000067-25).

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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).

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A combined avulsion of both the latissimus dorsi muscle and teres major muscle is a rare occurrence and data focused on the treatment of this type of injury is limited to case studies and series. This case report presents the outcomes of early surgical repair for avulsions of the latissimus dorsi and teres major tendons in a high-demanding athlete. The patient underwent surgical repair using a single incision technique and endosteal button fixation of the avulsed tendons.

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  • The study investigates the health-related quality of life (HRQoL) in children with acute lymphoblastic leukaemia (ALL) during different treatment protocols (ALL-10 and ALL-11), focusing on long-term impacts and factors influencing HRQoL.* -
  • Conducted in Dutch paediatric oncology hospitals from 2006 to 2017, the research involves two prospective cohort studies, collecting HRQoL data through parent-proxy questionnaires at various time intervals after diagnosis.* -
  • Out of 268 participating families, the study aims to compare the specific and generic HRQoL assessments against healthy norms and evaluate changes over time using advanced statistical models.*
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Introduction: Vincristine is an integral component of treatment for children with cancer. Its main dose-limiting side effect is vincristine-induced peripheral neuropathy (VIPN). The VINCA trial was a randomized controlled trial that explored the effect of 1-hour infusion compared with push injection of vincristine on the development of VIPN in children with cancer.

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The Indonesian island of Sulawesi has a unique geology and geography, which have produced an astoundingly diverse and endemic flora and fauna and a fascinating biogeographic history. Much biodiversity research has focused on the regional endemism in the island's Central Core and on its four peninsulas, but the biodiversity of the island's many upland regions is still poorly understood for most taxa, including amphibians and reptiles. Here, we report the first of several planned full-mountain checklists from a series of herpetological surveys of Sulawesi's mountains conducted by our team.

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  • - The study analyzed 156 adults with Langerhans cell histiocytosis to see if BRAF mutation status affected disease severity or survival rates, finding no significant correlation.
  • - It was noted that BRAFV600E mutations were linked to a higher occurrence of second cancers, particularly hematological types, in patients.
  • - These second malignancies may share a common origin with the original condition, suggesting potential connections between them.
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Erdheim-Chester disease (ECD) is a rare histiocytic disorder that can present as a localized infiltration of foamy histiocytes or a multisystem disease that may be life-threatening. It is extremely rare in children. Pegoraro and colleagues present the clinical and molecular features of 21 patients with pediatric ECD through a large international collaboration, documenting that it resembles its adult counterpart, with similar molecular features and responses to agents targeting BRAF and MEK.

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Stem cell-based cell therapeutics and especially those based on human mesenchymal stem cells (hMSCs) and induced pluripotent stem cells (hiPSCs) are said to have enormous developmental potential in the coming years. Their applications range from the treatment of orthopedic disorders and cardiovascular diseases to autoimmune diseases and even cancer. However, while more than 27 hMSC-derived therapeutics are currently commercially available, hiPSC-based therapeutics have yet to complete the regulatory approval process.

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  • Thyroid dysfunction (both hypo- and hyperthyroidism) is a potential late effect of hematopoietic stem cell transplantation (HSCT) in children, but its short-term effects are not clearly understood.
  • A study conducted on 72 children before and 3 months after HSCT showed no cases of thyroid imbalance, although some changes in thyroid function were observed.
  • The findings suggest that thyroid issues post-HSCT are rare shortly after the procedure, indicating that monitoring for thyroid problems can be delayed, and changes in thyroid parameters may point to a condition known as euthyroid sick syndrome.
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