Tumor lysis syndrome (TLS) is an oncologic emergency due to the rapid lysis of tumor cells and subsequent release of large amounts of intracellular potassium, phosphate, and uric acid into the bloodstream. Precipitation of uric acid and/or calcium phosphate crystals in the renal tubules can result in acute kidney injury. TLS is frequently observed in children with malignancy, which has high tumor burden, rapid cell turnover or high chemosensitivity (particularly, Burkitt's lymphoma and acute lymphoblastic leukemia), following the initiation of cytotoxic therapy. The current recommendations for prophylaxis and management are based on the TLS risk stratification. It is essential to administer adequate fluid and hypouricemic agents (allopurinol and/or rasburicase) to prevent acute kidney injury. In children susceptible to TLS, prompt diagnosis and aggressive treatment, such as renal replacement therapy, should be performed through close monitoring.
Acute Kidney Injury ; Burkitt Lymphoma ; Calcium ; Child ; Diagnosis ; Emergencies ; Humans ; Hyperkalemia ; Hyperphosphatemia ; Hyperuricemia ; Hypocalcemia ; Monitoring, Physiologic ; Potassium ; Primary Prevention ; Renal Replacement Therapy ; Tumor Burden ; Tumor Lysis Syndrome* ; Uric Acid

Over the past few decades, the survival rates of childhood acute leukemia have improved remarkably through treatment in risk-stratified prospective multicenter studies, and treatment protocols have evolved according to prognostic factors and treatment response. Recently, new molecular prognostic factors have been discovered through leukemic genome studies, and new subtypes with independent gene expression profiles have also been found. Furthermore, candidate mutations for target agents are being discovered, which will broaden therapeutic options for patients with poor prognostic factors. New drugs are currently being evaluated in clinical trials, and immunotherapy is now attracting interest in the treatment of recurrent or refractory disease in pediatric acute leukemia.
Child ; Clinical Protocols ; Genome ; Humans ; Immunotherapy ; Leukemia* ; Leukemia, Myeloid, Acute ; Prognosis ; Prospective Studies ; Survival Rate ; Transcriptome

Health insurance big data provides real-world evidence of unmet needs in clinical practice and breakthroughs in the medical industry that will impact the future of health care. Big data is expected to revolutionize the current medical paradigm and usher in an era of personalized medicine. In Korea, the Health Insurance Review and Assessment Service and the National Health Insurance Service established large-capacity healthcare big data open systems in 2011 and 2013, respectively, and are providing researchers with secured healthcare big data. However, concerns have been raised regarding the quality of big data-based research. Thus, numerous obstacles remain in leveraging big data research to change medical practice. This paper describes the understanding and practical applications of healthcare big data in pediatric cancer research, ranging from clinical research design using health insurance big data to medical writing.

In recent decades, survival rates for childhood acute myeloid leukemia have remarkably improved, owing to chemotherapy intensification, allogeneic hematopoietic stem cell transplantation, and improved supportive care. Furthermore, treatment protocols have evolved and are currently better matched to prognostic factors and treatment responses. Recently, new molecular prognostic factors were discovered via leukemia genomic studies. Moreover, new tumor subtypes with independent gene expression profiles have been characterized. To broaden the therapeutic options for patients with poor prognoses, therapies that target specific candidate mutations are being identified. Additionally, new drugs are undergoing clinical trials, and immunotherapy is attracting significant interest as a treatment option for recurrent or refractory childhood acute myeloid leukemia.

In recent decades, survival rates for childhood acute lymphoblastic leukemia have improved remarkably, as demonstrated by risk-stratified, prospective multicenter studies. Treatment protocols have evolved and become better matched to both prognostic factors and treatment responses. Recently, new molecular prognostic factors have been discovered in leukemia genomic studies. New tumor subtypes with independent gene expression profiles have also been characterized. Furthermore, therapies targeted to specific candidate mutations are being identified to broaden therapeutic options for patients with poor prognoses. Many new drugs are in clinical trials and immunotherapy is attracting significant interest for the treatment of recurrent or refractory disease in childhood acute lymphoblastic leukemia.
Clinical Protocols ; Humans ; Immunotherapy ; Leukemia ; Precursor Cell Lymphoblastic Leukemia-Lymphoma ; Prognosis ; Prospective Studies ; Survival Rate ; Transcriptome

Human taeniasis is presumed to have almost disappeared from Korea. Recently, we incidentally detected a Taenia saginata infection in an 8-year-old boy undergoing lymphoma diagnosis. The patient had been suffering for 4 months from intensifying snoring and obstructive sleep apnea. A neck computed tomography scan revealed a nasopharyngeal mass, and malignant B-cell lymphoma was supported by punch biopsy. On day 6 of the lymphoma workup period, the patient experienced anal itching, and two proglottids were detected in his stool. The patient had experienced four or five similar episodes within the past 2 years. He self-reported a history of raw beef and fish consumption and no history of traveling abroad. Laboratory findings revealed mild eosinophilia (eosinophil count: 791/μL).Two proglottids exhibited movement and possessed more than 15 branched uterine structures. Long segments approximately 84 cm in length were expelled after praziquantel treatment. Sequencing of the cytochrome oxidase 1 gene confirmed T. saginata, ruling out related Taenia species. After treatment, no proglottids or ova were detected in his stool, and the patient finally started chemotherapy for lymphoma. This case highlights the importance of timely diagnosis of hidden taeniasis in low-frequency endemic regions.

The neurocognitive function and quality of life of 58 Korean survivors of childhood medulloblastoma were assessed after surgery, cranial radiation and chemotherapy. All patients were evaluated with a battery of neurocognitive function tests and the Pediatric Functional Assessment of Cancer Therapy-Brain Tumor Survivors, which consists of self-report questionnaires on quality of life. The mean full-scale intelligence quotient (IQ), verbal IQ, and performance IQ scores were 90.2, 97.1, and 84.16, respectively. The mean memory quotient (MQ) score was 86.78, which was within 1 standard deviation of the average score of 100. Processing speed, attention, and executive function showed mild to moderate deficits. Intelligence, memory, executive function, visuospatial function, and simple motor function were significantly lower in the patients diagnosed before 8 years of age compared with those diagnosed after 8. The cognitive deficits in the patients diagnosed at younger ages might be related to earlier exposure to craniospinal irradiation and chemotherapy. The patient and parent proxy evaluations of attention, fine motor function, and quality of life did not differ. We found significant neurocognitive changes in a wide range of neurocognitive functional domains in Korean survivors of childhood medulloblastoma. Long-term follow-up studies of survivors of childhood medulloblastoma beginning at the time of their first diagnosis are required to better understand the deficits exhibited by survivors of childhood medulloblastoma, so that intervention strategies and treatment refinements that reduce the long-term neurocognitive decline can be developed.
Cognition ; Cognition Disorders ; Craniospinal Irradiation ; Diagnosis ; Drug Therapy ; Executive Function ; Follow-Up Studies ; Humans ; Intelligence ; Korea ; Medulloblastoma* ; Memory ; Parents ; Proxy ; Quality of Life* ; Survivors*

Spinal neurenteric cyst results from the persistence of an abnormal communication between endodermal and neuroectodermal layer. It is a rare malformation that lead to spinal cord compression. The patient is a 22-month-old boy with sudden, progressive left side motor weakness. Preoperative magnetic resonance image revealed intradural extramedullary cyst in C4-C5 level that compressed the spinal cord on the left side. After the resection of the cyst, motor weakness of the left side was partially improved, and his motor power was completely recovered after rehabilitation. Histologic examination revealed a ciliated columnar epithelial neurenteric cyst. The pre- and postoperative clinical features of the case of neurenteric cyst is described with the review of literature.
Endoderm ; Hemiplegia* ; Humans ; Infant ; Male ; Neural Plate ; Neural Tube Defects* ; Rehabilitation ; Spinal Cord ; Spinal Cord Compression

Background: Aggressive mature B-cell non-Hodgkin lymphoma (B-NHL) is the most common non-Hodgkin lymphoma in children. The outcome of chemotherapy for B-NHL has improved over decades. Methods: We reviewed 82 children and adolescents with B-NHL diagnosed at Asan Medical Center between 1993 and 2020. The D-COMP/COMP (daunomycin–cyclophosphamide, doxorubicin, vincristine, and prednisolone), Pediatric Oncology Group (POG)-9219/9315/ 9317, R-CHOP/CHOP (rituximab–cyclophosphamide, doxorubicin, vincristine, and prednisolone), and Lymphomes Malins B 89 (LMB89)/LMB96 regimens were administered. In 2018, rituximab was added to the LMB protocol (R-LMB) for advanced-staged Burkitt lymphoma (BL). The patients’ clinical features and treatment outcomes were retrospectively analyzed. Results: The most common subtype was BL (61%), followed by diffuse large B-cell lymphoma (DLBCL) (35%). The median age was 7.8 (range, 1.3‒16.4) years, and the most frequently used regimen was French‒American‒British (FAB)/LMB96 (58 patients, 70.7%). The 5-year overall survival (OS) and event-free survival (EFS) rates were 92.5% and 85.7%, respectively. The EFS rates of patients with BL and DLBCL were 90.0% and 79.3%, respectively. Among the FAB/LMB risk groups, group C (85.7%) had a significantly lower 5-year OS (P =0.037). Eleven events occurred (6 relapses, 3 deaths, and 2 secondary malignancies) during the median follow-up of 7.1 (range, 3.7‒118.5) months. Two patients treated with R-LMB had good outcomes without complications. Conclusion Various treatment regimens have favorable outcomes in pediatric patients with B-NHL.However, further studies are needed to improve survival in high-risk patients. In addition, careful monitoring for acute toxicity or secondary malignancy due to intensive multidrug chemotherapy is required.