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Objectives: The cryptic fusion oncogene NUP98::NSD1 is known to be associated with FLT3-ITD mutation in acute myeloid leukemia (AML), and an independent poor prognostic factor in pediatric AML. However, there are little data regarding the clinical significance of NUP98::NSD1 in adult cohort., Methods: We conducted a multicenter retrospective study to investigate the prevalence, clinical characteristics, and prognostic impact of NUP98::NSD1 in adult FLT3-ITD-positive AML patients., Results: In a total of 97 FLT3-ITD-positive AML patients, six cases (6.2%) were found to harbor the NUP98::NSD1 fusion transcript. NUP98::NSD1 positive cases had significantly higher platelet counts and a higher frequency of FAB-M4 morphology than NUP98::NSD1 negative cases. NUP98::NSD1 was found to be mutually exclusive with NPM1 mutation, and was accompanied by the WT1 mutation in three of the six cases. The presence of NUP98::NSD1 fusion at the time of diagnosis predicted poor response to cytarabine-anthracycline-based intensive induction chemotherapy (induction failure rate: 83% vs. 36%, p = .038). Five of the six cases with NUP98::NSD1 underwent allogeneic hematopoietic stem cell transplantation (HSCT). Two of the five cases have successfully maintained remission, with one of them being rescued through a second HSCT., Conclusions: Detecting NUP98::NSD1 in adult FLT3-ITD-positive AML is crucial to recognizing chemotherapy-resistant group., (© 2023 John Wiley & Sons A/S. Published by John Wiley & Sons Ltd.)

Background: The aim of this study was to examine risk factors of respiratory complications at the diagnosis and establish an algorithm of clinical management in children and adolescents with mediastinal tumors., Methods: We retrospectively collected clinical information of all children and adolescents who presented with mediastinal tumors at Saitama Children's Medical Center from 1999 to 2019, including age, sex, pathological diagnosis, eight major clinical symptoms (cough, dyspnea, hypoxia, orthopnea, chest pain, wheeze, superior vena cava syndrome, and stridor), chest computed tomography (CT) findings (tumor location, mediastinal mass ratio, pleural fluid, pericardial effusion, and compression of trachea and bronchi), types of diagnostic procedure and anesthesia, respiratory complications (severe hypoxia, difficult ventilation, respiratory failure, and cardiopulmonary arrest), and clinical outcome. Subsequently, we calculated the risk score for predicting respiratory complications by combining clinical and radiological findings., Results: Of the 57 patients, 7 (12%) developed respiratory complications. Cough, dyspnea, hypoxia, and orthopnea were significantly more common in patients with complications (p = 0.02, p = 0.02, p < 0.01, p = 0.03, respectively). The reduction of percentage of tracheal cross-sectional area (%TCA) and compression of the carina in chest CT were also significantly more common in patients with complications (p < 0.01 and <0.01, respectively). We calculated the risk score of respiratory complications by combining cough, wheeze, stridor, orthopnea, dyspnea, hypoxia, %TCA < 0.5, and compression of the carina. A risk score ≥ 7 showed high predictive accuracy for complications (sensitivity: 100%, specificity: 97.7%, positive likelihood ratio: 43.0)., Conclusion: The risk score combining clinical symptoms with radiological findings is a promising predictive tool for respiratory complications in children with mediastinal tumors., (© 2022 The Authors. Cancer Medicine published by John Wiley & Sons Ltd.)

Acute focal bacterial nephritis (AFBN) refers to the bacterial infection of the renal parenchyma without abscess formation. Although AFBN has mainly been reported in pediatric patients, it may be underdiagnosed in adults as it resembles acute pyelonephritis in its clinical presentation. However, the symptoms suggesting acute abdomen is an important clue to diagnose AFBN, which requires additional imaging studies such as contrast-enhanced computed tomography (CECT). Here, we present the case of a 49-year-old female presenting to our emergency room with acute abdomen as well as acute kidney injury (AKI). CECT was performed to rule out critical etiologies of severe abdominal pain and the results revealed multifocal wedge-shaped shadows in the right kidney and diffuse enlargement of bilateral kidneys. We diagnosed the patient with AFBN and treated her through temporal hemodialysis (two sessions) and antibiotics for 23 days. Although AKI associated with AFBN has rarely been reported, her renal dysfunction and other symptoms were completely improved. In conclusion, clinicians should be aware of AFBN and be cautious to avoid the unnecessary invasive interventions., (© 2022. The Author(s) under exclusive licence to The Japan Society of Nephrology.)