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Non-Down syndrome pediatric acute megakaryoblastic leukemia (AMKL) may be classified according to the presence of recurrent genetic abnormalities with prognostic relevance. In this case study, we report on a girl with AMKL, 32 months old at the time of diagnosis, in whom we confirmed the presence of the cryptic, poor prognosis NUP98::KDM5A fusion. The patient achieved complete remission (CR) with the first course of chemotherapy, underwent haploidentical family donor hematopoietic stem cell transplantation (HSCT) without event, but relapsed 5 months after HSCT. Through this case report, we emphasize the good initial response to chemotherapy, and the early relapse despite undergoing HSCT in first CR. We review the limited literature on NUP98::KDM5A (+) pediatric AML, and underscore the need for further study to improve the outcome of patients with this rare AML subtype.

Aims: The presence of facet tropism has been correlated with an elevated susceptibility to lumbar disc pathology. Our objective was to evaluate the impact of facet tropism on chronic lumbosacral discogenic pain through the analysis of clinical data and finite element modelling (FEM). Methods: Retrospective analysis was conducted on clinical data, with a specific focus on the spinal units displaying facet tropism, utilizing FEM analysis for motion simulation. We studied 318 intervertebral levels in 156 patients who had undergone provocation discography. Significant predictors of clinical findings were identified by univariate and multivariate analyses. Loading conditions were applied in FEM simulations to mimic biomechanical effects on intervertebral discs, focusing on maximal displacement and intradiscal pressures, gauged through alterations in disc morphology and physical stress. Results: A total of 144 discs were categorized as ‘positive’ and 174 discs as ‘negative’ by the results of provocation discography. The presence of defined facet tropism (OR 3.451, 95% CI 1.944 to 6.126) and higher Adams classification (OR 2.172, 95% CI 1.523 to 3.097) were important predictive parameters for discography-‘positive’ discs. FEM simulations showcased uneven stress distribution and significant disc displacement in tropism-affected discs, where loading exacerbated stress on facets with greater angles. During varied positions, notably increased stress and displacement were observed in discs with tropism compared to those with normal facet structure. Conclusion: Our findings indicate that facet tropism can contribute to disc herniation and changes in intradiscal pressure, potentially exacerbating disc degeneration due to altered force distribution and increased mechanical stress. Cite this article: Bone Joint Res 2024;13(9):452–461.

Purpose Glycated hemoglobin (HbA1c) as a glycemic index may have limited value in pediatric patients with acute leukemia as they often present with anemia and/or pancytopenia. To address this issue, we evaluated the usefulness of glycated albumin (GA) as a glycemic monitoring index in pediatric patients with acute leukemia. Methods Medical records of 25 patients with type 2 diabetes mellitus (T2DM), 63 patients with acute leukemia, and 115 healthy children from Seoul St. Mary's Hospital, The Catholic University of Korea, were retrospectively investigated for serum GA, HbA1c, and fasting blood glucose (FBG) levels, along with demographic data. Results GA, HbA1c, and FBG levels did not differ between the control and acute leukemia groups. In the T2DM group, positive correlations were observed among GA, HbA1c, and FBG (P

Tyrosine kinase (TK) inhibitors are most common used to treat chronic myeloid leukemia (CML). Several studies describe a range of adverse effects of TK inhibitors on the kidney in adult patients, including nephrotic syndrome. However, there are only a few pediatric case reports on TK inhibitor-associated nephrotic syndrome. We report a case of nephrotic syndrome associated with dasatinib, a type of TK inhibitor, in a child who suffered from both the underlying idiopathic nephrotic syndrome and CML. A 14-year-old Korean boy was diagnosed with steroid dependent nephrotic syndrome at 4 years old and CML at 11 years old. He developed a relapse of nephrotic syndrome after treatment with TK inhibitors, especially dasatinib, which he used for four months. Despite receiving steroid pulse therapy due to his underlying steroid dependent nephrotic syndrome, his proteinuria did not respond. He discontinued dasatinib medication due to the possibility to dasatinib induced nephrotic syndrome. After one week, his nephrotic syndrome improved. The case is important because if a CML patient that has been on a TK inhibitor develops a newly onset nephrotic range proteinuria, TK inhibitor-induced nephrotic syndrome should be included in the differential diagnosis.

Background: Varicella can lead to severe complications in immunocompromised children, including those undergoing hematopoietic stem cell transplantation (HSCT) or chemotherapy. Preventing primary varicella zoster virus (VZV) infection is crucial in these populations to mitigate morbidity and mortality. This study aimed to evaluate the immunogenicity and safety of the live attenuated MAV/06 varicella vaccine in pediatric patients post-HSCT and post-chemotherapy. Additionally, it sought to compare fluorescent-antibody-to-membrane-antigen (FAMA) and enzyme-linked immunosorbent assay (ELISA) titers to establish effective cut-off levels for protection against varicella. Methods: The FAMA assay was conducted at the Vaccine Bio Research Institute, and a VARICELLA-ZOSTER ELISA (Vircell, Granada, Spain) kit, which relies on lysate from whole cells infected with VZV, was used to determine VZV IgG. A prospective cohort study was conducted with 76 pediatric patients under 18 years old who tested negative for VZV IgG via ELISA. Patients post-HSCT and post-chemotherapy were included. Participants received the MAV/06 varicella vaccine, and serologic responses were evaluated using ELISA and FAMA. Results: The median age of participants was 9.8 years, with acute lymphoid leukemia and acute myeloid leukemia being the most common underlying disease. Post-dose 1, the seropositive rate was 56.1% by ELISA and 97.2% by FAMA. Based on the FAMA seropositive cut-off ≥1:4, post-dose 1 geometric mean titers (GMTs) of seropositive patients in the post-HSCT group were 14.7 (95% CI, 11.3–19.1) versus 20.2 (95% CI, 13.0–31.3) in the post-chemotherapy group (p = 0.690). Based on a FAMA seropositive cut-off ≥1:16, the post-dose 1 GMT of patients considered seropositive in the post-HSCT group was 19.3 (95% CI, 15.6–24.0) versus 34.1 (95% CI, 21.0–55.4) in the post-chemotherapy group (p = 0.116), and post-dose 2 FAMA titers of 76.1 (95% CI, 14.6–398.1) in the post-HSCT group and 64.0 (95% CI, 11.4–358.1) in the post-HSCT group (p = 0.853) were observed. In patients with lower baseline FAMA titers (1:4 to 1:8), 66.7% in the post-HSCT group and 71.5% in the post-chemotherapy group achieved a greater than four-fold increase in FAMA titers post-dose 1, while those with higher baseline titers (≥1:16) did not. There were no serious adverse events or vaccine-related rashes occurring in any of the patients. Conclusion: The MAV/06 varicella vaccine is immunogenic in pediatric patients post-HSCT and post-chemotherapy, particularly when administered in a two-dose schedule using a cut-off FAMA titer of

In an increasingly complex interconnected world, STEAM practices have gained attention in Higher Education (HE). The integration of Sciences, Technology, Engineering and Mathematics pedagogies with those from the Arts takes many forms with myriad intentions, processes and outcomes. Common aspirations pertain to equipping students with interdisciplinary skills required for the jobs market, increasing intellectual curiosity and collaboratively developing creative, socially equitable responses to complex global challenges. The Erasmus+ project "STEAM Innovation and Curriculum" involves seven European partners who have been pioneering STEAM approaches and methods. Using workshops, discussions, hacking processes and reflective practice, this collaborative work is the first comprehensive attempt to systematically analyse and showcase European approaches to STEAM in HE. Project outputs relate to the three project phases and include defining STEAM and a Handbook on STEAM approaches (Phase 1), the development of new STEAM methods (Phase 2) and developing evaluation frameworks (Phase 3). The Handbook collects and codifies HE STEAM approaches and provides a working definition highlighting key elements of STEAM projects and processes as well as guidance and inspiration for those looking to develop and introduce STEAM approaches in their institution. This paper focuses on Phase 1 outcomes and insights to help foster STEAM thinking and to tackle issues around joint STEM/Arts standards, and concerns in the HE arena such as modes of intellectual investigation, curriculum planning and the case for inter- and transdisciplinarity.

IntroductionIn the modern competitive landscape of football, clubs are increasingly leveraging data-driven decision-making to strengthen their commercial positions, particularly against rival clubs. The strategic allocation of resources to attract and retain profitable fans who exhibit long-term loyalty is crucial for advancing a club's marketing efforts. While the Recency, Frequency, and Monetary (RFM) customer segmentation technique has seen widespread application in various industries for predicting customer behavior, its adoption within the football industry remains underexplored. This study aims to address this gap by introducing an adjusted RFM approach, enhanced with the Analytic Hierarchy Process (AHP) and unsupervised machine learning, to effectively segment football fans based on Customer Lifetime Value (CLV).MethodsThis research employs a novel weighted RFM method where the significance of each RFM component is quantified using the AHP method. The study utilizes a dataset comprising 500,591 anonymized merchandising transactions from Amsterdamsche Football Club Ajax (AFC Ajax). The derived weights for the RFM variables are 0.409 for Monetary, 0.343 for Frequency, and 0.248 for Recency. These weights are then integrated into a clustering framework using unsupervised machine learning algorithms to segment fans based on their weighted RFM values. The simple weighted sum approach is subsequently applied to estimate the CLV ranking for each fan, enabling the identification of distinct fan segments.ResultsThe analysis reveals eight distinct fan clusters, each characterized by unique behaviors and value contributions: The Golden Fans (clusters 1 and 2) exhibit the most favourable scores across the recency, frequency, and monetary metrics, making them relatively the most valuable. They are critical to the club's profitability and should be rewarded through loyalty programs and exclusive services. The Promising segment (cluster 3) shows potential to ascend to Golden Fan status with increased spending. Targeted marketing campaigns and incentives can stimulate this transition. The Needs Attention segment (cluster 4) are formerly loyal fans whose engagement has diminished. Re-engagement strategies are vital to prevent further churn. The New Fans segment (clusters 5 and 6) are fans who have recently transacted and show potential for growth with proper engagement and personalized offerings. Lastly, the Churned/Low Value segment (clusters 7 and 8) are fans who relatively contribute the least and may require price incentives to potentially re-engage, though they hold relatively lower priority compared to other segments.DiscussionThe findings validate the proposed method's utility through its application to AFC Ajax's Customer Relationship Management (CRM) data and provides a robust framework for fan segmentation in the football industry. The approach offers actionable insights that can significantly enhance marketing strategies by identifying and prioritizing high-value segments based on the club's preferences and requirements. By maintaining the loyalty of Golden Fans and nurturing the Promising segment, football clubs can achieve substantial gains in profitability and fan engagement. Additionally, the study underscores the necessity of re-engaging formerly loyal fans and fostering new fans' growth to enable long-term commercial success. This methodology not only aims to bridge a research gap, but also equips marketing practitioners with data-driven tools for effective and efficient customer segmentation in the football industry.

Pediatric patients who have undergone hematopoietic stem cell transplantation (HSCT) or chemotherapy are at increased risk for severe influenza complications, necessitating annual vaccination. This study evaluated the immunogenicity and antibody persistence of the 2021–2022 seasonal quadrivalent influenza vaccine in pediatric patients post-HSCT or chemotherapy, compared to healthy controls. A prospective cohort study included 80 pediatric participants divided into three groups: chemotherapy (n = 33), HSCT (n = 27), and healthy controls (n = 20). All participants were vaccinated with the 2021–2022 GC FLU Quadrivalent vaccine. Hemagglutination inhibition (HI) assays measured seroprotection rates (SPR), geometric mean titers (GMT), and seroconversion rates (SCR) for the four vaccine antigens (A/H1N1, A/H3N2, B/Victoria, B/Yamagata) at one, three, and six months post-vaccination. At one month post-vaccination, all groups met the 70% SPR threshold for A/H1N1 and A/H3N2, but not for B/Victoria. For B/Yamagata, the SPR was low in the chemotherapy and HSCT groups (18.18% and 33.33%, respectively), compared to 80.00% in controls (p < 0.0001 and p = 0.0015). While A/H1N1 and A/H3N2 GMTs were protective in all groups, only controls achieved protective levels for B/Yamagata. Over time, the control group maintained >70% SPR for A/H1N1 up to six months, but the chemotherapy and HSCT groups declined by three and six months, respectively. For A/H3N2, the SPR in controls dropped below 70% at three months, while it remained above 70% in the chemotherapy and HSCT groups until three months. None of the groups achieved protective GMTs for B strains at three or six months. Pediatric patients post-HSCT or chemotherapy demonstrated a comparable immune response to healthy controls for A/H1N1 and A/H3N2, but the rapid decline in A/H1N1 antibody levels suggests the need for ongoing monitoring and adjusted vaccination schedules. The poor response to B antigens, particularly B/Yamagata, underscores the need for improved vaccination strategies in these vulnerable populations.

The high breakdown electric field, n-type doping capability, availability of high-quality substrates, and high Baliga’s figure of merit of Ga2O3 demonstrate its potential as a next-generation power semiconductor material. However, the thermal conductivity of Ga2O3 is lower than that of other wide-bandgap materials, resulting in the degradation of the electrical performance and reduced reliability of devices. The heterostructure formation on substrates with high thermal conductivity has been noted to facilitate heat dissipation in devices. In this work, Ga2O3 thin films with an Al2O3 interlayer were deposited on SiC substrates by radio frequency sputtering. Post-deposition annealing was performed at 900 °C for 1 h to crystallize the Ga2O3 thin films. The Auger electron spectroscopy depth profiles revealed the interdiffusion of the Ga and Al atoms at the Ga2O3/Al2O3 interface after annealing. The X-ray diffraction (XRD) results displayed improved crystallinity after annealing and adding the Al2O3 interlayer. The crystallite size increased from 5.72 to 8.09 nm as calculated by the Scherrer equation using the full width at half maximum (FWHM). The carrier mobility was enhanced from 5.31 to 28.39 cm2 V−1 s−1 in the annealed Ga2O3 thin films on Al2O3/SiC. The transfer and output characteristics of the Ga2O3/SiC and Ga2O3/Al2O3/SiC back-gate transistors reflect the trend of the XRD and Hall measurement results. Therefore, this work demonstrated that the physical and electrical properties of the Ga2O3/SiC back-gate transistors can be improved by post-deposition annealing and the introduction of an Al2O3 interlayer.

In the vast abyssal plains northwest of Iceland, white glass sponges of the genus Caulophacus Schulze, 1886 were inhabited by reddish Bythocaris G.O. Sars, 1870 shrimps and pinkish amphipods. After in situ observations at 3700 m depth, in -1°C waters by a remotely operated vehicle, members of this assemblage were collected and preserved for molecular studies. Based on integrative taxonomic analyses, the amphipods were identified as a new species of the genus Halirages Boeck, 1871 – Halirages spongiae sp. nov. Lörz, Nack & Tandberg –, as described in detail below. Part of our integrative approach was to establish reference DNA barcodes for known species of Halirages. However, our investigation of material of Calliopiidae G.O. Sars, 1895 collected around Iceland and Norway revealed slight morphological discrepancies in all the described species of Halirages. Except for Halirages fulvocinctus (M. Sars, 1858), none of the encountered specimens of Calliopiidae fully matched a current species description. We illuminate the morphological characteristics of nine operational taxonomic units, which also represented clades in COI and 28S. We set the Icelandic samples in the context of Halirages from Canada and Norway. A key to the world species of Halirages is provided.

Untreated tibialis anterior tendon ruptures can lead to gait abnormalities, flatfoot deformities, drop foot and chronic pain. Both traumatic and atraumatic ruptures of the tibialis anterior tendon can occur, though, atraumatic ruptures are often spontaneous and less common. For many patients, their symptoms are vague, which can result in a delay in diagnosis. Drop foot is typically the most noticeable symptom. Surgical treatment has been recommended for both traumatic and atraumatic ruptures to restore normal gait and function of tendon. Several surgical techniques have been published, however, there is no consensus on “gold standard” for optimal treatment. Here, a surgical technique is discussed which involves tenodesis of the extensor hallucis longus tendon to the ruptured tibialis anterior tendon with subsequent transfer into the medial cuneiform along with extensor hallux brevis to extensor hallux longus tenodesis distally over the first metatarsophalangeal joint. The authors find that this technique is reproducible in terms of allowing patients to regain dorsiflexory strength at the ankle joint and avoid postoperative ankle-foot orthoses. To date, all patients that have been treated with this technique have been able to return to normal ambulation without the use of a brace or subsequent gait disturbances.

Conclusion: This study showed that HIV+ patients were carriers of Plasmodium falciparum and Plasmodium vivax with an appreciable overall prevalence. The only factor influencing the prevalence of malaria was using traditional medicine.

Abstract The use of species distribution models has proliferated, providing insights for sustainable management of migratory species in a globally changing environment. However, many of these models are based on statistical relationships developed from historical conditions that may not perform well under changing or even analogous conditions caused by climate change. In this paper, we used a mechanistic species distribution model called GR3D (Global Repositioning Dynamics for Diadromous Fish Distribution) to examine the integrated dynamics of American shad (Alosa sapidissima) populations across their native range along the Eastern U.S. coast, where the species demonstrates latitudinal variations in life histories and reproductive strategies. The initial design of the model was adapted to incorporate region‐specific parameterization to fit the species ecology. Then, a sensitivity analysis was performed to test the influences of uncertain processes regarding American shad distribution at sea, straying and reproduction on key characteristics of the species distribution. The sensitivity analysis showed the influence of the Allee effect (i.e., “depensatory” process) and the homing rate (i.e., fidelity to the breeding sites) on the probability of presence and abundances among catchments and metapopulations estimated by the model. Contrary to the homing rate, the distance of straying did not change the estimated number of metapopulations or abundances. Homing strength, however, was quite influential. The integration of complex migration patterns during the marine phase (i.e., wintering and summering offshore areas) provided more likely estimates of the species' overall distribution. Overall, our study illustrated the utility of incorporating factors governing the large‐scale distribution of migratory species to improve local management.

We aimed to determine the recommendation level for the treatment of acute and chronic low back pain (LBP). A systematic review (SR) of the literature was performed and all English-language articles that discuss acute and chronic LBP, including MEDLINE and the Cochrane Database of Systematic Reviews, were searched. Of the 873 searched literature reports, 259 articles, including 131 clinical trials, 115 SRs, nine meta-analyses, and four clinical guidelines were analyzed. In these articles, high-quality randomized controlled trials, SRs, and used well-written clinical guidelines were reviewed. The results indicated multiple acute and chronic LBP treatment methods in the literature, and these reports when reviewed included general behavior, pharmacological therapy, psychological therapy, specific exercise, active rehabilitation and educational interventions, manual therapy, physical modalities, and invasive procedures. The Trial conclusions and SRs were classified into four categories of A, B, C, and D. If there were not enough high-quality articles, it was designated as “I” (insufficient). This review and summary of guidelines may be beneficial for physicians to better understand and make recommendations in primary care.

Hemophagocytic lymphohistiocytosis-like toxicity following chimeric antigen receptor (CAR)-T cell therapy (carHLH) is a rare and fulminant complication. Currently, there are neither well-established diagnostic criteria nor optimal treatment option for carHLH. Given the similarities of hyperinflammatory process and cytokine profiles between cytokine release syndrome (CRS) and carHLH, tocilizumab and corticosteroids are the suggested front-line treatment options for both conditions. However, when carHLH is refractory to front-line treatment, alternative or adjunctive agents should be introduced to ameliorate ongoing hyperinflammation. Anakinra, a recombinant interleukin (IL)-1 receptor antagonist, has shown promising results in the management of carHLH. Although prospective trials are limited, the use of anakinra for severe CRS and carHLH has been increasing. In this case report, we present two cases of carHLH to discuss its characteristic clinical features, diagnostic criteria, and treatment option. In addition, we also highlight the clinical efficacy of anakinra for managing carHLH which was refractory to tocilizumab and dexamethasone.

Purpose This study aimed to investigate the clinical factors associated with bone mineral density (BMD) among children and adolescents with osteoporosis secondary to treatment for underlying clinical conditions. Methods We retrospectively reviewed the medical records of patients aged 10–18 years and evaluated them for lumbar spine BMD (LSBMD) after treatment for underlying diseases, including hemato-oncologic, rheumatologic system, and inflammatory bowel diseases. LSBMD measured by dual-energy x-ray absorptiometry (DXA) performed from March 2019 to March 2021 was evaluated. We analyzed 117 patients who underwent initial DXA after treatment for underlying diseases. Results Subjects in this study had multiple underlying diseases: hemato-oncologic (78.6%), rheumatologic (11.1%), and inflammatory bowel diseases (10.3%). There was no significant association between the z-score and bone metabolic markers (P>0.05). However, higher cumulative glucocorticoid (GC) dose significantly reduced LSBMD z-score (P=0.029). Moreover, the association between cumulative dose of GC and initial z-score of LSBMD was significant in logarithmic regression analysis (P=0.003, R2=0.149). GC accumulation was a significant risk factor for vertebral fracture when the initial BMD was evaluated after treatment (P=0.043). Bone metabolic markers did not significantly influence the risk of vertebral fracture. Conclusions Initial bone mass density of the lumbar spine evaluated after long-term GC use for underlying diseases is a predictor of further vertebral fractures.

Purpose Survivors of childhood leukemia are at risk of growth impairment due to intensive chemotherapy and radiation treatments. This study investigated the auxological and biochemical characteristics of childhood leukemia survivors diagnosed with growth hormone deficiency (GHD) and the changes in these parameters after 1 year of growth hormone (GH) treatment. Methods A total of 24 children diagnosed with GHD after leukemia treatment was analyzed. Clinical and biochemical data were collected retrospectively at leukemia diagnosis, GHD diagnosis, and 1 year after GH treatment. Standard deviation score (SDS) was calculated based on the age- and gender-adjusted population. Results Of the 24 children included in this study, 19 received GH treatment. The median age at GHD diagnosis was 12.3 years, and the median delay in bone age was 1.46 years. Height SDS decreased from -0.69 at leukemia diagnosis to -2.58 at GHD diagnosis (P