Unraveling the Genetic Landscape of Langerhans Cell Histiocytosis in Korean Patients: Comprehensive Insights from Mutational Profiles and Clinical Correlations.

Purpose: This study aimed to conduct a comprehensive genetic analysis of patients with Langerhans cell histiocytosis (LCH), focusing on the frequency of MAPK pathway mutations, detailed mutation profiles of MAPK pathway genes, and their correlation with clinical features and prognosis in Korean LCH patients.

Materials And Methods: We performed targeted next-generation sequencing, capable of capturing exons from 382 cancer-related genes, on genomic DNA extracted from formaldehyde-fixed and paraffin-embedded samples of 45 pathologically confirmed LCH patients.

Results: The majority of patients (91.

Machine Learning-based Prediction of Blood Stream Infection in Pediatric Febrile Neutropenia.

Objectives: This study aimed to develop machine learning (ML) prediction models for identifying bloodstream infection (BSI) and septic shock (SS) in pediatric patients with cancer who presenting febrile neutropenia (FN) at emergency department (ED) visit.

Materials And Methods: A retrospective study was conducted on patients, younger than 18 years of age, who visited a tertiary university-affiliated hospital ED due to FN between January 2004 and August 2022. ML models, based on XGBoost, were developed for BSI and SS prediction.

Local control and toxicity outcomes following consolidative radiation therapy in patients with high-risk neuroblastoma: a 20-year experience at a single center.

Background: Intensive multimodal treatment can improve survival in patients with high-risk neuroblastoma, and consolidative radiation therapy has contributed to local control. We examined the clinical outcomes of patients who underwent consolidative radiation therapy at our institution.

Methods: We retrospectively reviewed the records of patients with high-risk neuroblastoma who underwent consolidative radiation therapy from March 2001 to March 2021 at Asan Medical Center.

Comorbidity Differences by Trajectory Groups as a Reference for Identifying Patients at Risk for Late Mortality in Childhood Cancer Survivors: Longitudinal National Cohort Study.

Background: Childhood cancer has a high long-term morbidity and mortality rate. Five years after the initial cancer diagnosis, approximately two-thirds of childhood cancer survivors experience at least one late complication, with one-quarter experiencing severe, life-threatening complications. Chronic health conditions can impact survivors' life planning and daily activities, reducing their health-related quality of life.

Epidemiologic and Clinical Outcomes of Pediatric Renal Tumors in Korea: A Retrospective Analysis of The Korean Pediatric Hematology and Oncology Group (KPHOG) Data.

Purpose: Renal tumors account for approximately 7% of all childhood cancers. These include Wilms tumor (WT), clear cell sarcoma of the kidney (CCSK), malignant rhabdoid tumor of the kidney (MRTK), renal cell carcinoma (RCC), congenital mesoblastic nephroma (CMN) and other rare tumors. We investigated the epidemiology of pediatric renal tumors in Korea.

Favorable outcomes with durable chimerism after hematopoietic cell transplantation using busulfan and fludarabine-based reduced-intensity conditioning for pediatric patients with hemophagocytic lymphohistiocytosis.

Background: The incorporation of a reduced-intensity conditioning (RIC) regimen in hematopoietic cell transplantation (HCT) for patients with hemophagocytic lymphohistiocytosis (HLH) has decreased early mortality but is associated with a high rate of mixed chimerism and graft failure. Here, we present a successful single-center experience using busulfan and a fludarabine-based RIC regimen for the treatment of HLH.

Methods: The medical records of pediatric patients with HLH who underwent HCT using a busulfan/ fludarabine-based RIC regimen between January 2008 and December 2017 were reviewed retrospectively.

Recent improvement in survival outcomes and reappraisal of prognostic factors in hepatoblastoma.

Background: Prognostic factors in hepatoblastoma need to be reevaluated considering the advances in treatment modalities. The study aimed to evaluate current outcomes of hepatoblastoma and reappraise the association of prognostic factors, including pre-treatment extent of tumor (PRETEXT) stage with annotation factors and Children's Hepatic tumors International Collaboration-Hepatoblastoma Stratification (CHIC-HS) system, with survival outcomes.

Methods: We evaluated 103 consecutive patients with hepatoblastoma retrospectively according to the treatment period based on the introduction of a liver transplantation program.

Erratum: Correction of Affiliations in the Article "Clinical Characteristics and Treatment Outcomes in Children, Adolescents, and Young-adults with Hodgkin's Lymphoma: a KPHOG Lymphoma Working-party, Multicenter, Retrospective Study".

This corrects the article on p. e393 in vol. 35, PMID: 33258329.

Genetic Alterations Detected by Targeted Next-generation Sequencing and Their Clinical Implications in Neuroblastoma.

Background/aim: This study was conducted to evaluate the clinical usefulness of panel next-generation sequencing (NGS) and to investigate the spectrum of genetic alterations and their clinical implications in neuroblastoma.

Patients And Methods: Formalin-fixed, paraffin-embedded archival samples from 41 cases of neuroblastoma were used for targeted sequencing.

Results: A total of 145 somatic mutations were identified, including 51 synonymous, 86 missense, 3 nonsense, 2 frameshift deletion, 2 splice-site, and 1 in-frame deletion mutations.

Clinical Characteristics and Treatment Outcomes in Children, Adolescents, and Young-adults with Hodgkin's Lymphoma: a KPHOG Lymphoma Working-party, Multicenter, Retrospective Study.

Background: Hodgkin's lymphoma (HL) constitutes 10%-20% of all malignant lymphomas and has a high cure rate (5-year survival, around 90%). Recently, interest has increased concerning preventing secondary complications (secondary cancer, endocrine disorders) in long-term survivors. We aimed to study the epidemiologic features and therapeutic outcomes of HL in children, adolescents, and young adults in Korea.

Development and Application of Advance Care Planning Workbooks to Facilitate Communication with Children and Adolescent Patients: A Pilot Test.

Purpose: This study presents the process of designing workbooks for advance care planning appropriate for the Korean cultural setting and describes actual case studies.

Methods: This study focused on single inductive case studies of the utilization of an advance care planning workbook and recruited individual participants.

Results: The workbook for adolescents contained six sessions and the workbook for children contained seven sessions.

Differences in perspectives of pediatricians on advance care planning: a cross-sectional survey.

Background: The increase in the number of pediatric patients with complex health conditions necessitates the application of advance care planning for children. Earlier, withdrawal of life-sustaining treatment was taboo in the medical society in South Korea due to the history of such practice being punishable by law, and physicians tended to pursue aggressive treatment. With changes in public opinion on end-of-life care, the Korean government enacted a new law that protect human dignity by respecting patients' self-determination and facilitating advance care planning.

Improved outcomes of allogeneic hematopoietic stem cell transplantation including haploidentical transplantation for childhood myelodysplastic syndrome.

This retrospective study aimed to investigate the outcomes of allogeneic hematopoietic stem cell transplantation (HSCT) for childhood myelodysplastic syndrome (MDS). Thirty-six patients (low-grade MDS, 24; advanced MDS, 12) received HSCT at the Asan Medical Center over two decades (early period, 1997-2007; recent period, 2008-2017). The transplantation outcomes were analyzed according to disease status, conditioning regimen, various donor types, and period of HSCT.

Expression of Immune Checkpoint Receptors on T-Cells and Their Ligands on Leukemia Blasts in Childhood Acute Leukemia.

Background: Possible correlations between the expression of immune checkpoint molecules and prognosis in childhood acute leukemia were investigated.

Materials And Methods: The expression of programmed-death 1 (PD1), cytotoxic T-lymphocyte-associated protein 4 (CTLA-4), and B- and T-lymphocyte attenuator (BTLA) was determined by flow cytometry on peripheral αβ and γδ T-cells from patients with newly diagnosed acute lymphoblastic leukemia (ALL) (n=9) or acute myeloid leukemia (AML) (n=12), and from healthy volunteers (n=7). The expression of programmed-death ligand 1 (PD-L1), B7-1, B7-2, human leukocyte antigen-ABC (HLA-ABC), and herpesvirus-entry mediator (HVEM) ligands was determined on leukemia blasts.

Comparable Outcome with a Faster Engraftment of Optimized Haploidentical Hematopoietic Stem Cell Transplantation Compared with Transplantations from Other Donor Types in Pediatric Acquired Aplastic Anemia.

Haploidentical family donors have been used as an alternative source in hematopoietic cell transplantation for patients with severe aplastic anemia. We evaluated and compared the outcomes of transplantation in pediatric acquired severe aplastic anemia based on donor type. Sixty-seven patients who underwent transplantation between 1998 and 2017 were included.

Recent change in congenital syphilis in Korea: Retrospective 10 year study.

Background: This study was conducted to evaluate recent clinical and anthropologic features of neonates with reactive serology for syphilis and their mothers from three institutions in Korea over an 11-year-period.

Method: The medical records of 20 neonates with reactive serology for syphilis and their mothers at three centers (Kyung Hee University Hospital, Kyung Hee University Hospital at Gangdong, and Korea Electric Power Corporation Hospital) seen between January 2000 and December 2010 were reviewed retrospectively.

Results: Among 20 mothers, 16 (80%) were native Korean and four (20%) were foreign-born immigrants.

Cognitive function in childhood epilepsy: importance of attention deficit hyperactivity disorder.

Background And Purpose: To determine how cognitive function is related to epilepsy classification and comorbid attention deficit hyperactivity disorder (ADHD) in children with newly diagnosed epilepsy of genetic or unknown etiology.

Methods: The medical records of children aged 6-16 years with newly diagnosed epilepsy of genetic or unknown etiology were reviewed retrospectively. The Korean Education Development Institute-Wechsler Intelligence Scale for Children and the Comprehensive Attention Test were used to evaluate intelligence and attention/executive function, respectively.

Effect of paracetamol use on the modification of the development of asthma by reactive oxygen species genes.

Background: Recent studies have identified an increase in the prevalence of asthma associated with paracetamol use.

Objective: To identify the relationship among asthma, biomarkers, genes, and paracetamol use in preschool children.

Methods: We undertook a population-based, cross-sectional survey of 933 preschool children.

Bronchial Hyperresponsiveness to Methacholine and AMP in Children With Atopic Asthma.

Purpose: Bronchial hyperresponsiveness (BHR) is typically measured by bronchial challenge tests that employ direct stimulation by methacholine or indirect stimulation by adenosine 5'-monophosphate (AMP). Some studies have shown that the AMP challenge test provides a better reflection of airway inflammation, but few studies have examined the relationship between the AMP and methacholine challenge tests in children with asthma. We investigated the relationship between AMP and methacholine testing in children and adolescents with atopic asthma.