1.Transformation of Pleomorphic Xanthoastrocytoma with Germline ATM Mutation into a SMARCB1-Deficient Rhabdoid Tumor: A Case Report
Hyeonseung LEE ; Hyun Jin PARK ; Bo Kyung KIM ; Kyung Taek HONG ; Hyoung Jin KANG ; Sung-Hye PARK ; Ji Hoon PHI ; June-Young KOH ; Jung Yoon CHOI
Clinical Pediatric Hematology-Oncology 2026;33(1):34-38
Secondary rhabdoid tumors (RTs) with atypical teratoid/rhabdoid tumor-like features rarely arise from, or coexist with, pleomorphic xanthoastrocytomas (PXAs), and their clinicopathological and molecular characteristics remain poorly understood. We report a 17-year-old girl with a temporal lobe mass that, upon gross total resection, pathologically contained both RT and PXA components. Immunohistochemistry revealed loss of INI1 expression restricted to the RT component, while the PXA area retained INI1. Next-generation sequencing identified a shared BRAF::TRIM24 fusion and homozygous deletion of CDKN2A/2B in both components, indicating a shared clonal origin. Additionally, a germline ATM frameshift mutation (c.5288_5289insGA) was identified in both tumor components, making the first such report in central nervous system tumors. SMARCB1 loss was confined to the RT component, further supporting the hypotheses of clonal evolution and secondary transformation. Despite gross total resection, craniospinal irradiation, and chemotherapy, the patient developed rapid leptomeningeal dissemination and died 5 months after surgery. This case provides clinicopathological and molecular evidence for clonal evolution and secondary transformation of PXA into an RT. The presence of germline ATM mutation may have therapeutic and biological relevance. Further studies are required to clarify the pathogenesis and optimal management of these rare and aggressive tumors.
2.The KAPARD guidelines for atopic dermatitis in children and adolescents:Part II. Systemic treatment, novel therapeutics, and adjuvant therapy
Hwan Soo KIM ; Eun LEE ; Kyunghoon KIM ; Taek Ki MIN ; Dong In SUH ; Yoon Ha HWANG ; Sungsu JUNG ; Minyoung JUNG ; Young A PARK ; Minji KIM ; In Suk SOL ; You Hoon JEON ; Sung-Il WOO ; Yong Ju LEE ; Jong Deok KIM ; Hyeon-Jong YANG ; Gwang Cheon JANG ;
Allergy, Asthma & Respiratory Disease 2025;13(1):3-11
Atopic dermatitis is the most common chronic inflammatory skin disease in children and adolescents. The Korean Academy of Pediatric Allergy and Respiratory Disease published the Atopic Dermatitis Treatment Guideline in 2008, which has been helpful in atopic dermatitis treatment until now. Various reports on the development and effectiveness of new drugs have suggested that there is a need to develop and revise old treatment guidelines. Part 1 aimed to provide evidence-based recommendations for skin care management and topical treatment for atopic dermatitis. Part 2 focuses on systemic treatment, novel therapeutics, and adjuvant therapy. The goal of this guideline is intended to assist front-line doctors treating pediatric and adolescent atopic dermatitis patients make safer, more effective, and more rational decisions regarding systemic treatment, novel therapeutics, and adjuvant therapy by providing evidence-based recommendations with a clear level of evidence and benefit regarding treatment.
3.The KAPARD guidelines for atopic dermatitis in children and adolescents:Part II. Systemic treatment, novel therapeutics, and adjuvant therapy
Hwan Soo KIM ; Eun LEE ; Kyunghoon KIM ; Taek Ki MIN ; Dong In SUH ; Yoon Ha HWANG ; Sungsu JUNG ; Minyoung JUNG ; Young A PARK ; Minji KIM ; In Suk SOL ; You Hoon JEON ; Sung-Il WOO ; Yong Ju LEE ; Jong Deok KIM ; Hyeon-Jong YANG ; Gwang Cheon JANG ;
Allergy, Asthma & Respiratory Disease 2025;13(1):3-11
Atopic dermatitis is the most common chronic inflammatory skin disease in children and adolescents. The Korean Academy of Pediatric Allergy and Respiratory Disease published the Atopic Dermatitis Treatment Guideline in 2008, which has been helpful in atopic dermatitis treatment until now. Various reports on the development and effectiveness of new drugs have suggested that there is a need to develop and revise old treatment guidelines. Part 1 aimed to provide evidence-based recommendations for skin care management and topical treatment for atopic dermatitis. Part 2 focuses on systemic treatment, novel therapeutics, and adjuvant therapy. The goal of this guideline is intended to assist front-line doctors treating pediatric and adolescent atopic dermatitis patients make safer, more effective, and more rational decisions regarding systemic treatment, novel therapeutics, and adjuvant therapy by providing evidence-based recommendations with a clear level of evidence and benefit regarding treatment.
4.The KAPARD guidelines for atopic dermatitis in children and adolescents:Part II. Systemic treatment, novel therapeutics, and adjuvant therapy
Hwan Soo KIM ; Eun LEE ; Kyunghoon KIM ; Taek Ki MIN ; Dong In SUH ; Yoon Ha HWANG ; Sungsu JUNG ; Minyoung JUNG ; Young A PARK ; Minji KIM ; In Suk SOL ; You Hoon JEON ; Sung-Il WOO ; Yong Ju LEE ; Jong Deok KIM ; Hyeon-Jong YANG ; Gwang Cheon JANG ;
Allergy, Asthma & Respiratory Disease 2025;13(1):3-11
Atopic dermatitis is the most common chronic inflammatory skin disease in children and adolescents. The Korean Academy of Pediatric Allergy and Respiratory Disease published the Atopic Dermatitis Treatment Guideline in 2008, which has been helpful in atopic dermatitis treatment until now. Various reports on the development and effectiveness of new drugs have suggested that there is a need to develop and revise old treatment guidelines. Part 1 aimed to provide evidence-based recommendations for skin care management and topical treatment for atopic dermatitis. Part 2 focuses on systemic treatment, novel therapeutics, and adjuvant therapy. The goal of this guideline is intended to assist front-line doctors treating pediatric and adolescent atopic dermatitis patients make safer, more effective, and more rational decisions regarding systemic treatment, novel therapeutics, and adjuvant therapy by providing evidence-based recommendations with a clear level of evidence and benefit regarding treatment.
5.The KAPARD guidelines for atopic dermatitis in children and adolescents:Part II. Systemic treatment, novel therapeutics, and adjuvant therapy
Hwan Soo KIM ; Eun LEE ; Kyunghoon KIM ; Taek Ki MIN ; Dong In SUH ; Yoon Ha HWANG ; Sungsu JUNG ; Minyoung JUNG ; Young A PARK ; Minji KIM ; In Suk SOL ; You Hoon JEON ; Sung-Il WOO ; Yong Ju LEE ; Jong Deok KIM ; Hyeon-Jong YANG ; Gwang Cheon JANG ;
Allergy, Asthma & Respiratory Disease 2025;13(1):3-11
Atopic dermatitis is the most common chronic inflammatory skin disease in children and adolescents. The Korean Academy of Pediatric Allergy and Respiratory Disease published the Atopic Dermatitis Treatment Guideline in 2008, which has been helpful in atopic dermatitis treatment until now. Various reports on the development and effectiveness of new drugs have suggested that there is a need to develop and revise old treatment guidelines. Part 1 aimed to provide evidence-based recommendations for skin care management and topical treatment for atopic dermatitis. Part 2 focuses on systemic treatment, novel therapeutics, and adjuvant therapy. The goal of this guideline is intended to assist front-line doctors treating pediatric and adolescent atopic dermatitis patients make safer, more effective, and more rational decisions regarding systemic treatment, novel therapeutics, and adjuvant therapy by providing evidence-based recommendations with a clear level of evidence and benefit regarding treatment.
6.The KAPARD guidelines for atopic dermatitis in children and adolescents:Part II. Systemic treatment, novel therapeutics, and adjuvant therapy
Hwan Soo KIM ; Eun LEE ; Kyunghoon KIM ; Taek Ki MIN ; Dong In SUH ; Yoon Ha HWANG ; Sungsu JUNG ; Minyoung JUNG ; Young A PARK ; Minji KIM ; In Suk SOL ; You Hoon JEON ; Sung-Il WOO ; Yong Ju LEE ; Jong Deok KIM ; Hyeon-Jong YANG ; Gwang Cheon JANG ;
Allergy, Asthma & Respiratory Disease 2025;13(1):3-11
Atopic dermatitis is the most common chronic inflammatory skin disease in children and adolescents. The Korean Academy of Pediatric Allergy and Respiratory Disease published the Atopic Dermatitis Treatment Guideline in 2008, which has been helpful in atopic dermatitis treatment until now. Various reports on the development and effectiveness of new drugs have suggested that there is a need to develop and revise old treatment guidelines. Part 1 aimed to provide evidence-based recommendations for skin care management and topical treatment for atopic dermatitis. Part 2 focuses on systemic treatment, novel therapeutics, and adjuvant therapy. The goal of this guideline is intended to assist front-line doctors treating pediatric and adolescent atopic dermatitis patients make safer, more effective, and more rational decisions regarding systemic treatment, novel therapeutics, and adjuvant therapy by providing evidence-based recommendations with a clear level of evidence and benefit regarding treatment.
7.Evidence-based management guidelines for noncystic fibrosis bronchiectasis in children and adolescents
Eun LEE ; Kyunghoon KIM ; You Hoon JEON ; In Suk SOL ; Jong Deok KIM ; Taek Ki MIN ; Yoon Ha HWANG ; Hyun-Ju CHO ; Dong In SUH ; Hwan Soo KIM ; Yoon Hee KIM ; Sung-Il WOO ; Yong Ju LEE ; Sungsu JUNG ; Hyeon-Jong YANG ; Gwang Cheon JANG
Clinical and Experimental Pediatrics 2024;67(9):418-426
Noncystic fibrosis bronchiectasis is a chronic respiratory disease that carries high socioeconomic and medical burdens and is caused by diverse respiratory illnesses. To improve clinical outcomes, early recognition, active treatment of exacerbations, and prevention of further exacerbations are essential. However, evidence for the treatment and prevention of acute exacerbation of noncystic fibrosis bronchiectasis, especially in children, is lacking. Therefore, the evidence- and consensus-based guidelines for medical and nonmedical treatment strategies for noncystic fibrosis bronchiectasis in children and adolescents were developed by the Korean Academy of Pediatric Allergy and Respiratory Disease using the methods recommended by the Grading of Recommendations Assessment, Development, and Evaluation working group with evidence published through July 2, 2020. This guideline encompasses evidence-based treatment recommendations as well as expert opinions, addressing crucial aspects of the treatment and management of non-cystic fibrosis bronchiectasis in children. This includes considerations for antibiotics and airway clearance strategies, particularly in areas where evidence may be limited. Large, well-designed, and controlled studies are required to accumulate further evidence of management strategies for noncystic fibrosis bronchiectasis in children and adolescents.
8.Evidence-based management guidelines for noncystic fibrosis bronchiectasis in children and adolescents
Eun LEE ; Kyunghoon KIM ; You Hoon JEON ; In Suk SOL ; Jong Deok KIM ; Taek Ki MIN ; Yoon Ha HWANG ; Hyun-Ju CHO ; Dong In SUH ; Hwan Soo KIM ; Yoon Hee KIM ; Sung-Il WOO ; Yong Ju LEE ; Sungsu JUNG ; Hyeon-Jong YANG ; Gwang Cheon JANG
Clinical and Experimental Pediatrics 2024;67(9):418-426
Noncystic fibrosis bronchiectasis is a chronic respiratory disease that carries high socioeconomic and medical burdens and is caused by diverse respiratory illnesses. To improve clinical outcomes, early recognition, active treatment of exacerbations, and prevention of further exacerbations are essential. However, evidence for the treatment and prevention of acute exacerbation of noncystic fibrosis bronchiectasis, especially in children, is lacking. Therefore, the evidence- and consensus-based guidelines for medical and nonmedical treatment strategies for noncystic fibrosis bronchiectasis in children and adolescents were developed by the Korean Academy of Pediatric Allergy and Respiratory Disease using the methods recommended by the Grading of Recommendations Assessment, Development, and Evaluation working group with evidence published through July 2, 2020. This guideline encompasses evidence-based treatment recommendations as well as expert opinions, addressing crucial aspects of the treatment and management of non-cystic fibrosis bronchiectasis in children. This includes considerations for antibiotics and airway clearance strategies, particularly in areas where evidence may be limited. Large, well-designed, and controlled studies are required to accumulate further evidence of management strategies for noncystic fibrosis bronchiectasis in children and adolescents.
9.Evidence-based management guidelines for noncystic fibrosis bronchiectasis in children and adolescents
Eun LEE ; Kyunghoon KIM ; You Hoon JEON ; In Suk SOL ; Jong Deok KIM ; Taek Ki MIN ; Yoon Ha HWANG ; Hyun-Ju CHO ; Dong In SUH ; Hwan Soo KIM ; Yoon Hee KIM ; Sung-Il WOO ; Yong Ju LEE ; Sungsu JUNG ; Hyeon-Jong YANG ; Gwang Cheon JANG
Clinical and Experimental Pediatrics 2024;67(9):418-426
Noncystic fibrosis bronchiectasis is a chronic respiratory disease that carries high socioeconomic and medical burdens and is caused by diverse respiratory illnesses. To improve clinical outcomes, early recognition, active treatment of exacerbations, and prevention of further exacerbations are essential. However, evidence for the treatment and prevention of acute exacerbation of noncystic fibrosis bronchiectasis, especially in children, is lacking. Therefore, the evidence- and consensus-based guidelines for medical and nonmedical treatment strategies for noncystic fibrosis bronchiectasis in children and adolescents were developed by the Korean Academy of Pediatric Allergy and Respiratory Disease using the methods recommended by the Grading of Recommendations Assessment, Development, and Evaluation working group with evidence published through July 2, 2020. This guideline encompasses evidence-based treatment recommendations as well as expert opinions, addressing crucial aspects of the treatment and management of non-cystic fibrosis bronchiectasis in children. This includes considerations for antibiotics and airway clearance strategies, particularly in areas where evidence may be limited. Large, well-designed, and controlled studies are required to accumulate further evidence of management strategies for noncystic fibrosis bronchiectasis in children and adolescents.
10.Evidence-based management guidelines for noncystic fibrosis bronchiectasis in children and adolescents
Eun LEE ; Kyunghoon KIM ; You Hoon JEON ; In Suk SOL ; Jong Deok KIM ; Taek Ki MIN ; Yoon Ha HWANG ; Hyun-Ju CHO ; Dong In SUH ; Hwan Soo KIM ; Yoon Hee KIM ; Sung-Il WOO ; Yong Ju LEE ; Sungsu JUNG ; Hyeon-Jong YANG ; Gwang Cheon JANG
Clinical and Experimental Pediatrics 2024;67(9):418-426
Noncystic fibrosis bronchiectasis is a chronic respiratory disease that carries high socioeconomic and medical burdens and is caused by diverse respiratory illnesses. To improve clinical outcomes, early recognition, active treatment of exacerbations, and prevention of further exacerbations are essential. However, evidence for the treatment and prevention of acute exacerbation of noncystic fibrosis bronchiectasis, especially in children, is lacking. Therefore, the evidence- and consensus-based guidelines for medical and nonmedical treatment strategies for noncystic fibrosis bronchiectasis in children and adolescents were developed by the Korean Academy of Pediatric Allergy and Respiratory Disease using the methods recommended by the Grading of Recommendations Assessment, Development, and Evaluation working group with evidence published through July 2, 2020. This guideline encompasses evidence-based treatment recommendations as well as expert opinions, addressing crucial aspects of the treatment and management of non-cystic fibrosis bronchiectasis in children. This includes considerations for antibiotics and airway clearance strategies, particularly in areas where evidence may be limited. Large, well-designed, and controlled studies are required to accumulate further evidence of management strategies for noncystic fibrosis bronchiectasis in children and adolescents.

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