Pediatric discovery最新文献

{"title":"Mechanism and target treatment of primary immunodeficiency diseases with systemic lupus erythematosus-like phenotype.","authors":"Shan Liu, Zhiyong Zhang, Xuemei Tang, Xiaodong Zhao, Yunfei An","doi":"10.1002/pdi3.67","DOIUrl":"10.1002/pdi3.67","url":null,"abstract":"<p><p>Primary immunodeficiency diseases (PIDs) present a heterogeneous group of diseases with aberrant immune response caused by monogenic mutations. Due to the immune dysfunction and dysregulation, PIDs have a wide clinical spectrum such as infections, autoimmunity, autoinflammation, allergy, and malignancies. Systemic lupus erythematosus (SLE) is a systemic autoimmune disease characterized with multiple autoantibodies and multiple organ damage, which could be the predominant phenotype in patients with PIDs. In recent years, the increasing identification of monogenic causes of SLE and PIDs discloses the partially shared genetic background and common pathogenic process. The study of PIDs with SLE-like phenotype paves the way for the exploration of lupus pathogenesis and new perspectives in targeted therapies concurrently.</p>","PeriodicalId":520221,"journal":{"name":"Pediatric discovery","volume":"2 3","pages":"e67"},"PeriodicalIF":0.0,"publicationDate":"2024-05-27","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC12118181/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"144586091","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":"Reproductive adverse events in patients with non-Hodgkin lymphoma treated with chemotherapeutic regimens including cyclophosphamide, doxorubicin, vincristine, prednisone or CHOP with rituximab: A systematic review and meta-analysis.","authors":"Rong Han, Jie Zhao, Chengjun Yu, Ling Wang, Long Chen, Yang Hu, Shengde Wu","doi":"10.1002/pdi3.72","DOIUrl":"10.1002/pdi3.72","url":null,"abstract":"<p><p>The regimen of cyclophosphamide, doxorubicin, vincristine, and prednisone (CHOP) or CHOP with rituximab (R-CHOP) is the first-line treatment for non-Hodgkin lymphoma (NHL). NHL patients treated with CHOP/R-CHOP have a high risk of reproductive adverse events. The aim of this article was to evaluate the reproductive toxicity of regimens and make further suggestions on reproductive protection. We systematically searched with appropriate terms from January 1980 to June 2021 for observational studies in patients treated with CHOP/R-CHOP, without any language restriction. We conducted meta-analyses of one-sample proportions of patients suffering reproductive adverse events after using CHOP/R-CHOP. In addition, subgroup analyses were performed to determine the effect of sex. Nine articles involving 331 patients were included in the meta-analysis, and the pooled proportion of reproductive adverse events was computed to be 22.3% (95% confidence interval [CI] 11.4%-33.2%; heterogeneity test <i>Q</i> = 65.3; <i>τ</i> ² = 0.0231; <i>I</i> ² = 87.70%; <i>p</i> < 0.001) using the random-effects model. And, the pooled proportion of male gonadal toxicity was 29.2% (95% CI 11.0%-47.4%; heterogeneity test <i>Q</i> = 46.65; <i>τ</i> ² = 3.055; <i>I</i> ² = 89.3%; <i>p</i> < 0.0001). The pooled proportion of female gonadal toxicity was 16.5% (95% CI 8.5%-24.5%; heterogeneity test <i>Q</i> = 18.6; <i>τ</i> ² = 0.0112; <i>I</i> ² = 67.8%; <i>p</i> = 0.005). The findings suggest that NHL patients have a relatively high risk of reproductive adverse events after treatment with CHOP/R-CHOP. Men are more likely to have gonadal damage than women. Evaluation of reproductive function is particularly necessary both before and after treatment. Some reproductive protection strategies implemented for patients who want to preserve their fertility.</p>","PeriodicalId":520221,"journal":{"name":"Pediatric discovery","volume":"3 3","pages":"e72"},"PeriodicalIF":0.0,"publicationDate":"2024-05-24","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC12483300/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"145208978","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":"Sleep-related disorders in children: A narrative review.","authors":"Yogev Cohen, Joel Reiter, Alex Gileles-Hillel","doi":"10.1002/pdi3.76","DOIUrl":"10.1002/pdi3.76","url":null,"abstract":"<p><p>Sleep-related disorders in children can significantly impact children's physical, emotional, and cognitive development and constitute a major source of parental concern. This comprehensive review aims to describe sleep-related disorders commonly encountered in pediatric practice, their etiology, diagnosis, and management strategies. The review explores various disorders prevalent in different age groups, including insomnia, sleep apnea, parasomnias, and circadian rhythm disorders, highlighting the importance of early identification and intervention for optimal child health and well-being.</p>","PeriodicalId":520221,"journal":{"name":"Pediatric discovery","volume":"2 2","pages":"e76"},"PeriodicalIF":0.0,"publicationDate":"2024-04-27","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC12118208/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"144586096","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":"Pediatric heart failure classification based on left ventricular ejection fraction.","authors":"Shan Huang, Xue Xiang, Xu Zhu, Jie Tian, Bo Pan, Min Zheng","doi":"10.1002/pdi3.50","DOIUrl":"10.1002/pdi3.50","url":null,"abstract":"<p><p>Left ventricle ejection fraction (LVEF) is still not well acknowledged in classification of pediatric heart failure (PHF). We categorized PHF according to LVEF and aimed to determine the role of LVEF in PHF classification. Patients who were diagnosed with HF were divided into three groups according to their LVEF values: HF with reduced ejection fraction (HFrEF), HF with mildly reduced ejection fraction (HFmrEF), and HF with preserved ejection fraction (HFpEF). The clinical information of PHF patients was compared among those three groups. Factors associated with HF with improved EF (HFimpEF) and risk factors for in-hospital death in PHF patients were analyzed. A total number of 1228 cases were collected. The proportion of HF patients with preserved LVEF (66.3%) was significantly higher than those with mildly reduced LVEF (21.7%) and reduced LVEF (12%). Clinical features such as age, B-type natriuretic peptide (BNP) level, Ross classification, and E/A abnormal proportion in HF children with different LVEF value were statistically different. HF patients with younger age, lower BNP levels, minor cardiac dysfunction and less E/A abnormality could be found with higher LVEF value. The proportion of primary disease in PHF was largely different in HFpEF, HFmrEF and HFrEF groups. Medication treatment was more aggressive in patients with lower LVEF, except for vasoactive drugs. Children with congenital heart disease in HFrEF group were more prone to develop into HFimpEF. Sepsis, renal insufficiency, and an abnormal E/A ratio are risk factors for in-hospital death of HF children. Clinical features of PHF could be well classified by LVEF, which is an essential and helpful indicator for PHF classification and management.</p>","PeriodicalId":520221,"journal":{"name":"Pediatric discovery","volume":"1 3","pages":"e50"},"PeriodicalIF":0.0,"publicationDate":"2023-12-27","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC12118176/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"144586094","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}