Pub Date : 2024-03-01Epub Date: 2024-02-19DOI: 10.3345/cep.2023.01480
Jue Seong Lee, Sang Hyun Park, Yoon Lee, Seunghyun Kim, Wonsuck Yoon, Young Yoo
{"title":"Metabolic syndrome and pulmonary dysfunction in asthmatic children during the COVID-19 pandemic.","authors":"Jue Seong Lee, Sang Hyun Park, Yoon Lee, Seunghyun Kim, Wonsuck Yoon, Young Yoo","doi":"10.3345/cep.2023.01480","DOIUrl":"10.3345/cep.2023.01480","url":null,"abstract":"","PeriodicalId":36018,"journal":{"name":"Clinical and Experimental Pediatrics","volume":null,"pages":null},"PeriodicalIF":4.2,"publicationDate":"2024-03-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC10915456/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"139900589","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
Pub Date : 2024-03-01Epub Date: 2023-06-19DOI: 10.3345/cep.2023.00234
Sukyung Kim, Kangmo Ahn, Jihyun Kim
Oral immunotherapy (OIT) has been recommended to reduce parental burden related to strict allergen avoidance and induce desensitization and immune tolerance for patients with long-lasting allergies to hen's eggs (HE) or cow's milk (CM). OIT should be monitored by pediatric allergists specializing in OIT and oral food challenge tests to manage allergic reactions. Although a previous history of anaphylaxis or multiple food allergies is not a contraindication to OIT, it is contraindicated if the patient has uncontrolled asthma, a malignancy, active systemic autoimmune disorders, or diseases requiring treatment with beta-blockers. A variety of OIT protocols have been de veloped to ensure better outcomes and safe up-dosing, including adjunctive therapies with biologics. This review provides insight into the practical issues of various immunotherapy options for children with HE or CM allergies.
对于对鸡蛋(HE)或牛奶(CM)长期过敏的患者,建议采用口服免疫疗法(OIT)来减轻父母严格回避过敏原的负担,并诱导脱敏和免疫耐受。OIT 应由专门从事 OIT 和口服食物挑战试验的儿科过敏专家进行监控,以控制过敏反应。虽然过敏性休克或多种食物过敏病史并非 OIT 的禁忌症,但如果患者患有未控制的哮喘、恶性肿瘤、活动性全身自身免疫性疾病或需要使用β-受体阻滞剂治疗的疾病,则应禁用 OIT。为了确保更好的疗效和安全的剂量,已经制定了多种 OIT 方案,包括生物制剂的辅助疗法。本综述深入探讨了针对 HE 或 CM 过敏症患儿的各种免疫疗法方案的实际问题。
{"title":"Practical issues of oral immunotherapy for egg or milk allergy.","authors":"Sukyung Kim, Kangmo Ahn, Jihyun Kim","doi":"10.3345/cep.2023.00234","DOIUrl":"10.3345/cep.2023.00234","url":null,"abstract":"<p><p>Oral immunotherapy (OIT) has been recommended to reduce parental burden related to strict allergen avoidance and induce desensitization and immune tolerance for patients with long-lasting allergies to hen's eggs (HE) or cow's milk (CM). OIT should be monitored by pediatric allergists specializing in OIT and oral food challenge tests to manage allergic reactions. Although a previous history of anaphylaxis or multiple food allergies is not a contraindication to OIT, it is contraindicated if the patient has uncontrolled asthma, a malignancy, active systemic autoimmune disorders, or diseases requiring treatment with beta-blockers. A variety of OIT protocols have been de veloped to ensure better outcomes and safe up-dosing, including adjunctive therapies with biologics. This review provides insight into the practical issues of various immunotherapy options for children with HE or CM allergies.</p>","PeriodicalId":36018,"journal":{"name":"Clinical and Experimental Pediatrics","volume":null,"pages":null},"PeriodicalIF":4.2,"publicationDate":"2024-03-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC10915454/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"9676465","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
Pub Date : 2024-02-01Epub Date: 2023-05-24DOI: 10.3345/cep.2022.00731
Suyoung Yoo, Meong Hi Son
Background: COVID-19 pandemic has significantly impacted the field of medical training, necessitating innovative approaches to education and practice. During this period, the use of novel technologies like virtual reality (VR), augmented reality (AR), and mixed reality (MR) has become increasingly vital. These technologies offer the advantage of transcending the limitations of time and space, thus enabling medical professionals to access various personalized programs for both education and service delivery. This shift is particularly relevant in the realm of pediatric medicine, where traditional training and clinical methods face unique challenges.
Purpose: The primary aim of this study is to explore the application of VR, AR, and MR technologies in pediatric medical settings, with a focus on both clinical applications and the training of pediatric medical professionals. We aim to comprehensively search and review studies that have utilized these technologies in the treatment of pediatric patients and the education of healthcare providers in this field.
Methods: Peer-reviewed articles published in PubMed, the Cochrane Library, ScienceDirect, Google Scholar, and Scopus from January 1, 2018, to March 1, 2023, were comprehensively searched. The review was conducted according to the PRISMA (Preferred Reporting Items for Systematic review and Meta-Analyses) guidelines. Among the 89 studies, 63 investigated the clinical applications of VR (n=60) or AR (n=3) in pediatric patients, and 25 investigated the applications of VR (n=19), AR (n=5), or MR (n=1) for training medical professionals.
Results: A total of 36 randomized controlled trials (RCTs) for clinical application (n=31) and medical training (n=5) were retrieved. Among the RCTs, 21 reported significant improvements in clinical applications (n=17) and medical training (n=4).
Conclusion: Despite a few limitations in conducting research on innovative technology, such research has rapidly expanded, indicating that an increasing number of researchers are involved in pediatric research using these technologies.
{"title":"Virtual, augmented, and mixed reality: potential clinical and training applications in pediatrics.","authors":"Suyoung Yoo, Meong Hi Son","doi":"10.3345/cep.2022.00731","DOIUrl":"10.3345/cep.2022.00731","url":null,"abstract":"<p><strong>Background: </strong>COVID-19 pandemic has significantly impacted the field of medical training, necessitating innovative approaches to education and practice. During this period, the use of novel technologies like virtual reality (VR), augmented reality (AR), and mixed reality (MR) has become increasingly vital. These technologies offer the advantage of transcending the limitations of time and space, thus enabling medical professionals to access various personalized programs for both education and service delivery. This shift is particularly relevant in the realm of pediatric medicine, where traditional training and clinical methods face unique challenges.</p><p><strong>Purpose: </strong>The primary aim of this study is to explore the application of VR, AR, and MR technologies in pediatric medical settings, with a focus on both clinical applications and the training of pediatric medical professionals. We aim to comprehensively search and review studies that have utilized these technologies in the treatment of pediatric patients and the education of healthcare providers in this field.</p><p><strong>Methods: </strong>Peer-reviewed articles published in PubMed, the Cochrane Library, ScienceDirect, Google Scholar, and Scopus from January 1, 2018, to March 1, 2023, were comprehensively searched. The review was conducted according to the PRISMA (Preferred Reporting Items for Systematic review and Meta-Analyses) guidelines. Among the 89 studies, 63 investigated the clinical applications of VR (n=60) or AR (n=3) in pediatric patients, and 25 investigated the applications of VR (n=19), AR (n=5), or MR (n=1) for training medical professionals.</p><p><strong>Results: </strong>A total of 36 randomized controlled trials (RCTs) for clinical application (n=31) and medical training (n=5) were retrieved. Among the RCTs, 21 reported significant improvements in clinical applications (n=17) and medical training (n=4).</p><p><strong>Conclusion: </strong>Despite a few limitations in conducting research on innovative technology, such research has rapidly expanded, indicating that an increasing number of researchers are involved in pediatric research using these technologies.</p>","PeriodicalId":36018,"journal":{"name":"Clinical and Experimental Pediatrics","volume":null,"pages":null},"PeriodicalIF":4.2,"publicationDate":"2024-02-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC10839193/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"9521918","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
Pub Date : 2024-02-01Epub Date: 2023-06-14DOI: 10.3345/cep.2022.01452
Ki Wook Yun
Pneumonia is a common pediatric infectious disease that is familiar to pediatricians and a major cause of hospitalization worldwide. Recent well-designed epidemiologic studies in developed countries indicated that respiratory viruses are detected in 30%-70%, atypical bacteria in 7%-17%, and pyogenic bacteria in 2%-8% of children hospitalized with community-acquired pneumonia (CAP). The etiological distribution of CAP varies widely by child age and the epidemiological season of the respiratory pathogen. Moreover, diagnostic tests, particularly for the detection of Streptococcus pneumoniae and Mycoplasma pneumoniae, the 2 major bacterial pathogens involved in pediatric CAP, have several limitations. Therefore, management and empirical antimicrobial therapy for children with CAP should be applied in a stepwise manner based on recent epidemiological, etiological, and microbiological evidence.
肺炎是儿科医生非常熟悉的一种常见儿科传染病,也是全球住院治疗的一个主要原因。最近在发达国家进行的精心设计的流行病学研究表明,在社区获得性肺炎(CAP)住院患儿中,30%-70%的患儿可检测到呼吸道病毒,7%-17%的患儿可检测到非典型细菌,2%-8%的患儿可检测到化脓性细菌。CAP 的病原体分布因儿童年龄和呼吸道病原体流行季节的不同而有很大差异。此外,诊断测试,尤其是检测肺炎链球菌和肺炎支原体(小儿 CAP 的两种主要细菌病原体)的测试,也存在一些局限性。因此,应根据最新的流行病学、病原学和微生物学证据,循序渐进地对 CAP 患儿进行管理和经验性抗菌治疗。
{"title":"Community-acquired pneumonia in children: updated perspectives on its etiology, diagnosis, and treatment.","authors":"Ki Wook Yun","doi":"10.3345/cep.2022.01452","DOIUrl":"10.3345/cep.2022.01452","url":null,"abstract":"<p><p>Pneumonia is a common pediatric infectious disease that is familiar to pediatricians and a major cause of hospitalization worldwide. Recent well-designed epidemiologic studies in developed countries indicated that respiratory viruses are detected in 30%-70%, atypical bacteria in 7%-17%, and pyogenic bacteria in 2%-8% of children hospitalized with community-acquired pneumonia (CAP). The etiological distribution of CAP varies widely by child age and the epidemiological season of the respiratory pathogen. Moreover, diagnostic tests, particularly for the detection of Streptococcus pneumoniae and Mycoplasma pneumoniae, the 2 major bacterial pathogens involved in pediatric CAP, have several limitations. Therefore, management and empirical antimicrobial therapy for children with CAP should be applied in a stepwise manner based on recent epidemiological, etiological, and microbiological evidence.</p>","PeriodicalId":36018,"journal":{"name":"Clinical and Experimental Pediatrics","volume":null,"pages":null},"PeriodicalIF":4.2,"publicationDate":"2024-02-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC10839192/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"9630756","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
Pub Date : 2024-02-01Epub Date: 2024-01-24DOI: 10.3345/cep.2023.00752
Hae Sang Lee
{"title":"Nonalcoholic fatty liver disease in children and adolescents.","authors":"Hae Sang Lee","doi":"10.3345/cep.2023.00752","DOIUrl":"10.3345/cep.2023.00752","url":null,"abstract":"","PeriodicalId":36018,"journal":{"name":"Clinical and Experimental Pediatrics","volume":null,"pages":null},"PeriodicalIF":4.2,"publicationDate":"2024-02-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC10839190/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"139542577","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
Pub Date : 2024-02-01Epub Date: 2023-06-14DOI: 10.3345/cep.2022.01242
Yogesh Waikar
Anorectal manometry is one of the motility tests in children performed by pediatric gastroenterologist. It evaluates the motility function of anorectal tract. It is helpful for diagnosing children with constipation, rectal hypersensitivity, fecal incontinence, Hirschsprung disease, anal achalasia, and anorectal malformations. The most common indication to perform anorectal manometry is to diagnose Hirschsprung disease. It is a safe procedure. This paper discusses recent advances and reviews on anorectal motility disorders in children.
{"title":"High-resolution anorectal manometry in children.","authors":"Yogesh Waikar","doi":"10.3345/cep.2022.01242","DOIUrl":"10.3345/cep.2022.01242","url":null,"abstract":"<p><p>Anorectal manometry is one of the motility tests in children performed by pediatric gastroenterologist. It evaluates the motility function of anorectal tract. It is helpful for diagnosing children with constipation, rectal hypersensitivity, fecal incontinence, Hirschsprung disease, anal achalasia, and anorectal malformations. The most common indication to perform anorectal manometry is to diagnose Hirschsprung disease. It is a safe procedure. This paper discusses recent advances and reviews on anorectal motility disorders in children.</p>","PeriodicalId":36018,"journal":{"name":"Clinical and Experimental Pediatrics","volume":null,"pages":null},"PeriodicalIF":4.2,"publicationDate":"2024-02-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC10839187/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"9630757","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
{"title":"Vitamin B12 deficiency in anemic children before versus after age 2 years: a form of hidden hunger in India.","authors":"Sahil Goel, Ruchika Bhatnagar, Anita Kumari, Brig Prem Lochan Prasad, Lahar Sahai","doi":"10.3345/cep.2023.01347","DOIUrl":"10.3345/cep.2023.01347","url":null,"abstract":"","PeriodicalId":36018,"journal":{"name":"Clinical and Experimental Pediatrics","volume":null,"pages":null},"PeriodicalIF":4.2,"publicationDate":"2024-02-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC10839188/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"139542579","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
Pub Date : 2024-02-01Epub Date: 2023-06-14DOI: 10.3345/cep.2022.00346
Jeong Hee Kim, Mona Salem Samra
Atopic dermatitis (AD) is a lifelong disease that markedly impairs quality of life. AD is considered a starting point of the "atopic march," which begins at a young age and may progress to systemic allergic diseases. Moreover, it is strongly associated with comorbid allergic and inflammatory diseases including arthritis and inflammatory bowel disease. Understanding the pathogenesis of AD is essential for the development of targeted therapies. Epidermal barrier dysfunction, immune deviation toward a T helper 2 proinflammatory profile, and microbiome dysbiosis play important roles via complex interactions. The systemic involvement of type 2 inflammation, wheather acute or chronic, and whether extrinsic or intrinsic, is evident in any type of AD. Studies on AD endotypes with unique biological mechanisms have been conducted according to clinical phenotypes, such as race or age, but the endotype for each phenotype, or endophenotype, has not yet been clearly identified. Therefore, AD is still being treated according to severity rather than endotype. Infancy-onset and severe AD are known risk factors leading to atopic march. In addition, up to 40% of adult AD are cases of infancy-onset AD that persist into adulthood, and these are often accompanied by other allergic diseases. Therefore, early intervention strategies to identify high-risk infants and young children, repair an impaired skin barrier, and control systemic inflamation may improve long-term outcomes in AD patients. However, to the best of our knowledge, no study has evaluated the effectiveness of early intervention on atopic march using systemic therapy in high-risk infants. This narrative review addresses the latest knowledge of systemic treatment, including Th2 cytokine receptor antagonists and Janus kinase inhibitors, for children with moderate to severe AD that is refractory to topical treatment.
特应性皮炎(AD)是一种严重影响生活质量的终身性疾病。特应性皮炎被认为是 "特应性进程 "的起点,它始于幼年,并可能发展为全身性过敏性疾病。此外,它还与合并过敏性和炎症性疾病(包括关节炎和炎症性肠病)密切相关。了解过敏性皮炎的发病机制对于开发靶向疗法至关重要。表皮屏障功能障碍、T 辅助细胞 2 型促炎免疫偏差和微生物群失调通过复杂的相互作用发挥着重要作用。无论是急性还是慢性,无论是外在还是内在,2 型炎症的系统性参与在任何类型的 AD 中都是显而易见的。根据临床表型(如种族或年龄)对具有独特生物机制的 AD 内型进行了研究,但每种表型的内型(或称内表型)尚未明确确定。因此,目前仍在根据严重程度而非内型来治疗注意力缺失症。婴儿期发病和严重的 AD 是导致特应性进展的已知风险因素。此外,多达 40% 的成人 AD 是婴儿期发病并持续到成年的 AD 病例,而且这些病例通常伴有其他过敏性疾病。因此,识别高危婴幼儿、修复受损皮肤屏障和控制全身性炎症的早期干预策略可能会改善 AD 患者的长期预后。然而,据我们所知,还没有研究评估过早期干预对高危婴幼儿特应性进展使用系统疗法的效果。本综述介绍了系统治疗的最新知识,包括 Th2 细胞因子受体拮抗剂和 Janus 激酶抑制剂,用于局部治疗无效的中重度 AD 患儿。
{"title":"Moderate to severe atopic dermatitis in children: focus on systemic Th2 cytokine receptor antagonists and Janus kinase inhibitors.","authors":"Jeong Hee Kim, Mona Salem Samra","doi":"10.3345/cep.2022.00346","DOIUrl":"10.3345/cep.2022.00346","url":null,"abstract":"<p><p>Atopic dermatitis (AD) is a lifelong disease that markedly impairs quality of life. AD is considered a starting point of the \"atopic march,\" which begins at a young age and may progress to systemic allergic diseases. Moreover, it is strongly associated with comorbid allergic and inflammatory diseases including arthritis and inflammatory bowel disease. Understanding the pathogenesis of AD is essential for the development of targeted therapies. Epidermal barrier dysfunction, immune deviation toward a T helper 2 proinflammatory profile, and microbiome dysbiosis play important roles via complex interactions. The systemic involvement of type 2 inflammation, wheather acute or chronic, and whether extrinsic or intrinsic, is evident in any type of AD. Studies on AD endotypes with unique biological mechanisms have been conducted according to clinical phenotypes, such as race or age, but the endotype for each phenotype, or endophenotype, has not yet been clearly identified. Therefore, AD is still being treated according to severity rather than endotype. Infancy-onset and severe AD are known risk factors leading to atopic march. In addition, up to 40% of adult AD are cases of infancy-onset AD that persist into adulthood, and these are often accompanied by other allergic diseases. Therefore, early intervention strategies to identify high-risk infants and young children, repair an impaired skin barrier, and control systemic inflamation may improve long-term outcomes in AD patients. However, to the best of our knowledge, no study has evaluated the effectiveness of early intervention on atopic march using systemic therapy in high-risk infants. This narrative review addresses the latest knowledge of systemic treatment, including Th2 cytokine receptor antagonists and Janus kinase inhibitors, for children with moderate to severe AD that is refractory to topical treatment.</p>","PeriodicalId":36018,"journal":{"name":"Clinical and Experimental Pediatrics","volume":null,"pages":null},"PeriodicalIF":4.2,"publicationDate":"2024-02-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC10839191/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"9639082","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
Pub Date : 2024-01-01Epub Date: 2023-11-17DOI: 10.3345/cep.2023.01074
Jiwon Jung, Joo Hoon Lee
{"title":"Clinical considerations and practical issues of kidney complications in children after COVID-19 infection or vaccination.","authors":"Jiwon Jung, Joo Hoon Lee","doi":"10.3345/cep.2023.01074","DOIUrl":"10.3345/cep.2023.01074","url":null,"abstract":"","PeriodicalId":36018,"journal":{"name":"Clinical and Experimental Pediatrics","volume":null,"pages":null},"PeriodicalIF":4.2,"publicationDate":"2024-01-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC10764664/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"138177442","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
Pub Date : 2024-01-01Epub Date: 2023-06-14DOI: 10.3345/cep.2022.01459
Eujin Park, Hee Gyung Kang
X-linked hypophosphatemia (XLH), the most common cause of hypophosphatemic rickets, affects one in every 20,000 people. Although conventional therapy for XLH was introduced approximately 4 decades ago, the temporary replacement of oral phosphate salts and activated vitamin D cannot completely control chronic hypophosphatemia, leaving patients with incomplete healing and residual skeletal deformity as well as at risk of endocrine abnormalities and adverse drug reactions. However, understanding the pathophysiology has led to the development of a targeted therapy, burosumab, a fibroblast growth factor-23 inhibitor that was recently approved in Korea for the treatment of XLH. This review provides insight into the diagnosis, evaluation, treatment, and recommended follow-up for a typical case of XLH and reviews its pathophysiology.
X连锁性低磷血症(XLH)是低磷血症性佝偻病最常见的病因,每两万人中就有一人患病。虽然 XLH 的传统疗法在大约 40 年前就已问世,但口服磷酸盐和活性维生素 D 的临时替代疗法并不能完全控制慢性低磷酸盐血症,患者的伤口愈合不完全,骨骼残留畸形,还可能出现内分泌异常和药物不良反应。然而,通过对病理生理学的了解,开发出了一种靶向疗法--burosumab,这是一种成纤维细胞生长因子-23抑制剂,最近在韩国获批用于治疗XLH。本综述深入探讨了XLH典型病例的诊断、评估、治疗和建议随访,并回顾了其病理生理学。
{"title":"X-linked hypophosphatemic rickets: from diagnosis to management.","authors":"Eujin Park, Hee Gyung Kang","doi":"10.3345/cep.2022.01459","DOIUrl":"10.3345/cep.2022.01459","url":null,"abstract":"<p><p>X-linked hypophosphatemia (XLH), the most common cause of hypophosphatemic rickets, affects one in every 20,000 people. Although conventional therapy for XLH was introduced approximately 4 decades ago, the temporary replacement of oral phosphate salts and activated vitamin D cannot completely control chronic hypophosphatemia, leaving patients with incomplete healing and residual skeletal deformity as well as at risk of endocrine abnormalities and adverse drug reactions. However, understanding the pathophysiology has led to the development of a targeted therapy, burosumab, a fibroblast growth factor-23 inhibitor that was recently approved in Korea for the treatment of XLH. This review provides insight into the diagnosis, evaluation, treatment, and recommended follow-up for a typical case of XLH and reviews its pathophysiology.</p>","PeriodicalId":36018,"journal":{"name":"Clinical and Experimental Pediatrics","volume":null,"pages":null},"PeriodicalIF":4.2,"publicationDate":"2024-01-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC10764665/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"9639086","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}