Pub Date : 2024-04-01Epub Date: 2024-01-08DOI: 10.1080/08880018.2023.2296949
Archana Sasi, Sindhura Chitikela, Shuvadeep Ganguly, Bivas Biswas, Deepam Pushpam, Akash Kumar, Shah Alam Khan, Venkatesan Sampath Kumar, Shashank Sharad Kale, Ahitagni Biswas, Adarsh Barwad, Asit Ranjan Mridha, Sanjay Thulkar, Sameer Bakhshi
Ewing sarcoma (ES) of the spine is a rare childhood cancer with sparse literature on treatment outcomes. We aimed to describe survival outcomes and prognostic factors in patients with spinal ES treated at a single institute in a resource-challenged setting. We conducted a retrospective analysis of patients with spinal ES registered at a tertiary care oncology center between 2003-2019. Clinical patient data was retrieved from hospital records. Cox regression analysis was used to identify the association of baseline clinical parameters with event free survival (EFS) and overall survival (OS). A cohort of 85 patients was analyzed including 38 (45%) patients with metastatic disease. The median age was 15 years with 73% being male. Local therapy was administered in 62 (72.9%) patients with surgery alone in 8 (9.4%), radiotherapy alone in 36 (42.4%) and both in 18 (21.2%) patients. A higher proportion of males received local therapy than females (80.3% versus 59.1%; p = 0.049). The median EFS and OS were 20.1 and 28.6 months, respectively. On univariable analysis, age ≤ 15 years, female sex, serum albumin ≤3.5 g/dL and hemoglobin ≤11 g/dL were associated with inferior EFS while younger age, female sex, hypoalbuminemia and metastatic disease were associated with inferior OS. On multivariable analysis, only hypoalbuminemia was predictive for inferior EFS (HR:2.41; p = 0.005) while hypoalbuminemia (HR:2.06;p = 0.033) and female sex (HR:1.83; p = 0.046) were associated with inferior OS. We concluded that hypoalbuminemia confers poor prognosis in ES spine. Survival outcomes are poorer in females treated in our setting, possibly due to prevailing sex-based biases.
脊柱尤文肉瘤(ES)是一种罕见的儿童癌症,有关治疗效果的文献很少。我们的目的是描述在资源有限的情况下在一家机构接受治疗的脊柱ES患者的生存结果和预后因素。我们对 2003-2019 年间在一家三级肿瘤中心登记的脊柱 ES 患者进行了回顾性分析。临床患者数据来自医院病历。Cox回归分析用于确定基线临床参数与无事件生存期(EFS)和总生存期(OS)的关系。共分析了 85 例患者,其中包括 38 例(45%)转移性疾病患者。中位年龄为15岁,73%为男性。62名(72.9%)患者接受了局部治疗,其中8名(9.4%)患者仅接受了手术治疗,36名(42.4%)患者仅接受了放射治疗,18名(21.2%)患者同时接受了两种治疗。接受局部治疗的男性比例高于女性(80.3% 对 59.1%;P = 0.049)。中位生存期和生存期分别为20.1个月和28.6个月。在单变量分析中,年龄≤15岁、女性、血清白蛋白≤3.5 g/dL和血红蛋白≤11 g/dL与较差的EFS相关,而年龄较小、女性、低白蛋白血症和转移性疾病与较差的OS相关。在多变量分析中,只有低白蛋白血症可预测较差的 EFS(HR:2.41; p = 0.005),而低白蛋白血症(HR:2.06; p = 0.033)和女性性别(HR:1.83; p = 0.046)与较差的 OS 相关。我们的结论是,低白蛋白血症会导致 ES 脊椎预后不良。在我们的病例中,女性患者的生存率较低,这可能是由于普遍存在的性别偏见。
{"title":"Treatment outcomes in patients with Ewing sarcoma of the spine in a resource-challenged setting: 17-year experience from a single center in India.","authors":"Archana Sasi, Sindhura Chitikela, Shuvadeep Ganguly, Bivas Biswas, Deepam Pushpam, Akash Kumar, Shah Alam Khan, Venkatesan Sampath Kumar, Shashank Sharad Kale, Ahitagni Biswas, Adarsh Barwad, Asit Ranjan Mridha, Sanjay Thulkar, Sameer Bakhshi","doi":"10.1080/08880018.2023.2296949","DOIUrl":"10.1080/08880018.2023.2296949","url":null,"abstract":"<p><p>Ewing sarcoma (ES) of the spine is a rare childhood cancer with sparse literature on treatment outcomes. We aimed to describe survival outcomes and prognostic factors in patients with spinal ES treated at a single institute in a resource-challenged setting. We conducted a retrospective analysis of patients with spinal ES registered at a tertiary care oncology center between 2003-2019. Clinical patient data was retrieved from hospital records. Cox regression analysis was used to identify the association of baseline clinical parameters with event free survival (EFS) and overall survival (OS). A cohort of 85 patients was analyzed including 38 (45%) patients with metastatic disease. The median age was 15 years with 73% being male. Local therapy was administered in 62 (72.9%) patients with surgery alone in 8 (9.4%), radiotherapy alone in 36 (42.4%) and both in 18 (21.2%) patients. A higher proportion of males received local therapy than females (80.3% versus 59.1%; <i>p</i> = 0.049). The median EFS and OS were 20.1 and 28.6 months, respectively. On univariable analysis, age ≤ 15 years, female sex, serum albumin ≤3.5 g/dL and hemoglobin ≤11 g/dL were associated with inferior EFS while younger age, female sex, hypoalbuminemia and metastatic disease were associated with inferior OS. On multivariable analysis, only hypoalbuminemia was predictive for inferior EFS (HR:2.41; <i>p</i> = 0.005) while hypoalbuminemia (HR:2.06;<i>p</i> = 0.033) and female sex (HR:1.83; <i>p</i> = 0.046) were associated with inferior OS. We concluded that hypoalbuminemia confers poor prognosis in ES spine. Survival outcomes are poorer in females treated in our setting, possibly due to prevailing sex-based biases.</p>","PeriodicalId":19746,"journal":{"name":"Pediatric Hematology and Oncology","volume":null,"pages":null},"PeriodicalIF":1.7,"publicationDate":"2024-04-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"139378107","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":4,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
Pub Date : 2024-04-01Epub Date: 2024-01-25DOI: 10.1080/08880018.2023.2295454
Angela Zanfardino, Patrizia Bruzzi, Michela Trada, Novella Rapini, Emanuela Laudani, Gianluca Tornese, Federica Ortolani, Gianluca Piccolo, Lorena Matonti, Maria Alessandra Saltarelli, Tiziana Timpanaro, Giuseppe D'Annunzio, Barbara Predieri, Francesca Rossi, Rossella Mura, Veronica Barat, Arcangelo Prete, Riccardo Schiaffini, Stefano Zucchini
Glycemic abnormalities are a frequent finding in pediatric oncological patients, both during treatment and after its discontinuation. Moreover, impaired glucose tolerance (IGT), impaired fasting glycemia (IFG) and diabetes mellitus (DM) are not rarely diagnosed in non-oncological hematological diseases. To explore the current pediatric Italian approach to the diagnosis and the management of the glycemic alterations in this clinical setting and, thus, to identify and enforce current clinical needs, we submitted an online 23-items survey to all the Italian Associazione Italiana Ematologia Oncologia Pediatrica (AIEOP) centers, and surveys were descriptively analyzed. Thirty-nine AIEOP centers were involved in the study. In 2021, among 75278 children and adolescents affected by an oncological or a hematological disease, 1.2 and 0.65% developed DM, while IGT or IFG were widespread in 2.3 and 2.8%, respectively. The main causes of DM were the use of corticosteroids in patients with cancer and the iron overload in patients with thalassemia. Venous fasting plasma glycemia was the most used tool to detect glycemic abnormalities. The performance of oral glucose tolerance test (OGTT) was extremely limited, except when IFG occurred. Despite the diagnosis of DM, ∼45% of patients with cancer and 30% of patients with one hematological disease did not receive an appropriate treatment. In the other cases, insulin was the drug of first choice. Emerging technologies for diabetes care (glucose sensors and insulin pumps) are not largely used yet. The results of our study support the standardization of the care of the glycemic abnormalities during or after onco-hematologic diseases in the pediatric age. Despite the scarce data in pediatric literature, proper guidelines are needed.
{"title":"The real-life management of glucose homeostasis abnormalities in pediatric onco-hematological diseases: data from a national survey.","authors":"Angela Zanfardino, Patrizia Bruzzi, Michela Trada, Novella Rapini, Emanuela Laudani, Gianluca Tornese, Federica Ortolani, Gianluca Piccolo, Lorena Matonti, Maria Alessandra Saltarelli, Tiziana Timpanaro, Giuseppe D'Annunzio, Barbara Predieri, Francesca Rossi, Rossella Mura, Veronica Barat, Arcangelo Prete, Riccardo Schiaffini, Stefano Zucchini","doi":"10.1080/08880018.2023.2295454","DOIUrl":"10.1080/08880018.2023.2295454","url":null,"abstract":"<p><p>Glycemic abnormalities are a frequent finding in pediatric oncological patients, both during treatment and after its discontinuation. Moreover, impaired glucose tolerance (IGT), impaired fasting glycemia (IFG) and diabetes mellitus (DM) are not rarely diagnosed in non-oncological hematological diseases. To explore the current pediatric Italian approach to the diagnosis and the management of the glycemic alterations in this clinical setting and, thus, to identify and enforce current clinical needs, we submitted an online 23-items survey to all the Italian Associazione Italiana Ematologia Oncologia Pediatrica (AIEOP) centers, and surveys were descriptively analyzed. Thirty-nine AIEOP centers were involved in the study. In 2021, among 75278 children and adolescents affected by an oncological or a hematological disease, 1.2 and 0.65% developed DM, while IGT or IFG were widespread in 2.3 and 2.8%, respectively. The main causes of DM were the use of corticosteroids in patients with cancer and the iron overload in patients with thalassemia. Venous fasting plasma glycemia was the most used tool to detect glycemic abnormalities. The performance of oral glucose tolerance test (OGTT) was extremely limited, except when IFG occurred. Despite the diagnosis of DM, ∼45% of patients with cancer and 30% of patients with one hematological disease did not receive an appropriate treatment. In the other cases, insulin was the drug of first choice. Emerging technologies for diabetes care (glucose sensors and insulin pumps) are not largely used yet. The results of our study support the standardization of the care of the glycemic abnormalities during or after onco-hematologic diseases in the pediatric age. Despite the scarce data in pediatric literature, proper guidelines are needed.</p>","PeriodicalId":19746,"journal":{"name":"Pediatric Hematology and Oncology","volume":null,"pages":null},"PeriodicalIF":1.7,"publicationDate":"2024-04-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"139546932","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":4,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
Pub Date : 2024-04-01Epub Date: 2024-02-29DOI: 10.1080/08880018.2023.2286962
Dea Aprilianti Permana, Susi Susanah, Riyadi Adrizain, Sri Endah Rahayuningsih, Meita Dhamayanti, Lulu Eva Rakhmilla
Objective: Increased survival rate of patients with Transfusion-dependent Thalassemia (TDT) should be in line with their good quality of life (QoL). The study aimed to analyze the relationship between sociodemographic factors and clinical characteristics with the QoL of children with TDT. Methods: A cross-sectional study was conducted at Hasan Sadikin General Hospital from December 2022 to February 2023. A total of 158 eligible subjects aged 5-18 years with TDT were included in the analysis. QoL assessment was performed using child self-report and parent-proxy report questionnaires, along with physical examination findings. Bivariate and multivariate analyses were conducted to analyze the data. Results: A total of 158 subjects who met the research criteria were included in the analysis. Of 58.9% of children with TDT had a low adherence rate to iron chelating therapy (ICT). School function had the lowest score in QoL based on child-self report and parent proxy. Gender (p<0,05) and adherence to ICT (p<0,05) were significantly associated with lower quality of life. Conclusion: Female and adherence to ICT were predictors of children with TDT's QoL.
{"title":"Factors Related to the Quality of Life in Children with Transfusion-Dependent Thalassemia.","authors":"Dea Aprilianti Permana, Susi Susanah, Riyadi Adrizain, Sri Endah Rahayuningsih, Meita Dhamayanti, Lulu Eva Rakhmilla","doi":"10.1080/08880018.2023.2286962","DOIUrl":"10.1080/08880018.2023.2286962","url":null,"abstract":"<p><p><b>Objective:</b> Increased survival rate of patients with Transfusion-dependent Thalassemia (TDT) should be in line with their good quality of life (QoL). The study aimed to analyze the relationship between sociodemographic factors and clinical characteristics with the QoL of children with TDT. <b>Methods:</b> A cross-sectional study was conducted at Hasan Sadikin General Hospital from December 2022 to February 2023. A total of 158 eligible subjects aged 5-18 years with TDT were included in the analysis. QoL assessment was performed using child self-report and parent-proxy report questionnaires, along with physical examination findings. Bivariate and multivariate analyses were conducted to analyze the data. <b>Results:</b> A total of 158 subjects who met the research criteria were included in the analysis. Of 58.9% of children with TDT had a low adherence rate to iron chelating therapy (ICT). School function had the lowest score in QoL based on child-self report and parent proxy. Gender (p<0,05) and adherence to ICT (p<0,05) were significantly associated with lower quality of life. <b>Conclusion:</b> Female and adherence to ICT were predictors of children with TDT's QoL.</p>","PeriodicalId":19746,"journal":{"name":"Pediatric Hematology and Oncology","volume":null,"pages":null},"PeriodicalIF":1.7,"publicationDate":"2024-04-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"139996988","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":4,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
Pub Date : 2024-04-01Epub Date: 2023-10-29DOI: 10.1080/08880018.2023.2271974
Yogi Chopra, Carly Hong, Tal Schechter, Muhammad Ali, Kuang-Yueh Chiang, Donna Wall, Joerg Krueger
After primary infection, Varicella Zoster (VZV) persists in sensory dorsal root ganglia and may be reactivated in periods of diminished T-cell immunity. Varicella Zoster reactivation post allogenic stem cell transplantation (HSCT) can be challenging to diagnose as it does not always present with characteristic skin lesions. We describe a pediatric patient who presented with isolated severe abdominal pain with no other symptoms. Cutaneous lesions appeared only 10 days later resulting in delayed diagnosis and treatment. He was successfully treated with intravenous acyclovir and recovered after a prolonged hospital stay with post-herpetic neuralgia. Abdominal pain in children post HSCT has a broad differential and VZV reactivation should be considered even in absence of cutaneous lesions. Early diagnosis and treatment are essential to reduce VZV-related morbidity and mortality. In this article we present a case report and review clinical presentation and outcome of similar cases in the literature.
{"title":"Intractable abdominal pain as the sole symptom of Varicella Zoster reactivation after allogeneic stem cell transplantation: brief case report and review of the literature.","authors":"Yogi Chopra, Carly Hong, Tal Schechter, Muhammad Ali, Kuang-Yueh Chiang, Donna Wall, Joerg Krueger","doi":"10.1080/08880018.2023.2271974","DOIUrl":"10.1080/08880018.2023.2271974","url":null,"abstract":"<p><p>After primary infection, Varicella Zoster (VZV) persists in sensory dorsal root ganglia and may be reactivated in periods of diminished T-cell immunity. Varicella Zoster reactivation post allogenic stem cell transplantation (HSCT) can be challenging to diagnose as it does not always present with characteristic skin lesions. We describe a pediatric patient who presented with isolated severe abdominal pain with no other symptoms. Cutaneous lesions appeared only 10 days later resulting in delayed diagnosis and treatment. He was successfully treated with intravenous acyclovir and recovered after a prolonged hospital stay with post-herpetic neuralgia. Abdominal pain in children post HSCT has a broad differential and VZV reactivation should be considered even in absence of cutaneous lesions. Early diagnosis and treatment are essential to reduce VZV-related morbidity and mortality. In this article we present a case report and review clinical presentation and outcome of similar cases in the literature.</p>","PeriodicalId":19746,"journal":{"name":"Pediatric Hematology and Oncology","volume":null,"pages":null},"PeriodicalIF":1.7,"publicationDate":"2024-04-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"71413404","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":4,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
Pub Date : 2024-04-01Epub Date: 2023-10-29DOI: 10.1080/08880018.2023.2273876
Ishaq Alkooheji, Elizabeth Secord, Colleen Buggs-Saxton, Tony Lulgjuraj, Süreyya Savaşan
{"title":"Sustained improvement in IPEX-like syndrome course following failed umbilical cord blood transplantation.","authors":"Ishaq Alkooheji, Elizabeth Secord, Colleen Buggs-Saxton, Tony Lulgjuraj, Süreyya Savaşan","doi":"10.1080/08880018.2023.2273876","DOIUrl":"10.1080/08880018.2023.2273876","url":null,"abstract":"","PeriodicalId":19746,"journal":{"name":"Pediatric Hematology and Oncology","volume":null,"pages":null},"PeriodicalIF":1.7,"publicationDate":"2024-04-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"71413405","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":4,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
The conventional treatment of Kasabach-Merritt Phenomenon (KMP) consists of corticosteroids with vincristine/vinblastine or others. The aim of the study is to compare the first-year direct costs and effectiveness between sirolimus and conventional treatment. A retrospective case-control study of KMP patients was conducted at a mean age of 9 months (1 day to 12 years) between 2000 and 2022 from four tertiary centers in Thailand. The direct costs, hematologic and clinical complete response (HCR, CCR), hospitalization, length of stay, and complications were compared. Of 29 patients, 13 underwent sirolimus (four upfront and nine were refractory to the conventional). The first-year total cost had no statistically significant difference between sirolimus VS conventional treatment (8,852.63 VS 9,083.56 USD: p value: 0.94). The therapeutics achievement was the same in both HCR (244.75 VS 168.94 days; p value: 0.60) and CCR (419.77 VS 399.87 days; p value: 0.90). The subgroup analysis of the first-line sirolimus (n = 4) compared with the conventional (n = 25) showed a more reduced total cost (4,907.84 VS 9,664.05 USD; p value: 0.26) rendered net total cost of -4,756.21 USD per patient (cost saving). A more significant contrast of therapeutic achievement by reduction of both HCR (11.67 VS 224.20 days; p value: 0.36) and CCR (38.50 VS 470.88 days; p value: 0.04) was shown. The sirolimus had no difference in hospitalization, length of stay, and complications. Even though, it was unable to identify significant differences in cost-effectiveness. Sirolimus is suitable for all patients who have diagnosis of KMP either for rescue therapy or first-line treatment.
卡萨斯巴赫-梅里特现象(KMP)的传统治疗方法包括皮质类固醇与长春新碱/长春花碱或其他药物。本研究的目的是比较西罗莫司和传统治疗的第一年直接费用和疗效。2000 年至 2022 年期间,泰国四家三级医疗中心对平均年龄为 9 个月(1 天至 12 岁)的 KMP 患者进行了一项回顾性病例对照研究。研究比较了直接费用、血液学和临床完全反应(HCR、CCR)、住院时间、住院时间和并发症。在29名患者中,13名接受了西罗莫司治疗(4名前期患者和9名常规治疗无效患者)。西罗莫司与常规治疗的第一年总费用差异无统计学意义(8852.63 美元对 9083.56 美元:P 值:0.94)。HCR(244.75 天 VS 168.94 天;P 值:0.60)和 CCR(419.77 天 VS 399.87 天;P 值:0.90)的治疗效果相同。对一线西罗莫司(n = 4)与常规西罗莫司(n = 25)进行的亚组分析显示,每位患者的总费用(4,907.84 美元 VS 9,664.05美元;P 值:0.26)减少了-4,756.21美元(节省费用)。通过减少 HCR(11.67 天 VS 224.20 天;P 值:0.36)和 CCR(38.50 天 VS 470.88 天;P 值:0.04),治疗效果对比更为明显。西罗莫司在住院时间、住院时间和并发症方面没有差异。尽管如此,西罗莫司在成本效益方面仍无法找出显著差异。西罗莫司适用于所有确诊为 KMP 的患者,无论是作为抢救治疗还是一线治疗。
{"title":"Cost and effectiveness comparison of sirolimus versus standard treatment in Kasabach-Merritt phenomenon: a real-world evidence study in Thailand.","authors":"Chanikarn Tongruang, Siriwan Wananukul, Susheera Chatproedprai, Nattee Narkbunnam, Rattanavalai Nitiyarom, Nongnuch Sirachainan, Rungrote Natesirinilkul, Phumin Chaweephisal, Darintr Sosothikul","doi":"10.1080/08880018.2023.2300485","DOIUrl":"10.1080/08880018.2023.2300485","url":null,"abstract":"<p><p>The conventional treatment of Kasabach-Merritt Phenomenon (KMP) consists of corticosteroids with vincristine/vinblastine or others. The aim of the study is to compare the first-year direct costs and effectiveness between sirolimus and conventional treatment. A retrospective case-control study of KMP patients was conducted at a mean age of 9 months <i>(</i>1 day to 12 years) between 2000 and 2022 from four tertiary centers in Thailand. The direct costs, hematologic and clinical complete response (HCR, CCR), hospitalization, length of stay, and complications were compared. Of 29 patients, 13 underwent sirolimus (four upfront and nine were refractory to the conventional). The first-year total cost had no statistically significant difference between sirolimus VS conventional treatment (8,852.63 VS 9,083.56 USD: <i>p</i> value: 0.94). The therapeutics achievement was the same in both HCR (244.75 VS 168.94 days; <i>p</i> value: 0.60) and CCR (419.77 VS 399.87 days; <i>p</i> value: 0.90). The subgroup analysis of the first-line sirolimus (<i>n</i> = 4) compared with the conventional (<i>n</i> = 25) showed a more reduced total cost (4,907.84 VS 9,664.05 USD; <i>p</i> value: 0.26) rendered net total cost of -4,756.21 USD per patient (cost saving). A more significant contrast of therapeutic achievement by reduction of both HCR (11.67 VS 224.20 days; <i>p</i> value: 0.36) and CCR (38.50 VS 470.88 days; <i>p</i> value: 0.04) was shown. The sirolimus had no difference in hospitalization, length of stay, and complications. Even though, it was unable to identify significant differences in cost-effectiveness. Sirolimus is suitable for all patients who have diagnosis of KMP either for rescue therapy or first-line treatment.</p>","PeriodicalId":19746,"journal":{"name":"Pediatric Hematology and Oncology","volume":null,"pages":null},"PeriodicalIF":1.7,"publicationDate":"2024-04-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"139486235","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":4,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
Pub Date : 2024-01-01Epub Date: 2024-02-01DOI: 10.1080/08880018.2023.2256780
A Farrag, K Mohammed, M H Ghazaly, F Berthold
Noncompliance with therapy is a big obstacle to successful therapy. We aimed to evaluate the prevalence and risk factors affecting the compliance of pediatric cancer patients with therapy in a tertiary care center far away from the capital in a lower-middle income country (LMIC). A retrospective cohort study of reports of all pediatric cancer patients who were diagnosed and started treatment between 2006 and 2010 at South Egypt Cancer Institute (SECI) was done. The following data were collected: Age, sex, diagnosis, compliance with therapy, and data on potential risk factors that might affect compliance, including time duration of travel from the patient's home to SECI, time lag between the first symptom until the first visit to SECI and until the start of treatment, results of reevaluation after the initial course of therapy, and therapy-related severe adverse events. Noncompliance with therapy was defined as when patients missed their determined therapy appointment for one week or more or abandoned therapy. This study included 510 patients. Eighty-three (16.3%) were non-compliant, as forty patients missed their therapy appointment (7.8%), and 43 abandoned further therapy (8.4%). Noncompliance was found to be more prevalent among patients with solid tumors. Non-compliant patients suffered a significantly higher relapse rate (47.7% vs. 11.2% in compliant patients, p < .001). Unfortunately, 75% of the abandoned patients who returned for further therapy suffered a relapse. Noncompliance with treatment is still a big problem facing cancer management in LMICs.
{"title":"Noncompliance of pediatric cancer patients with chemotherapy: A single-center experience in a lower-middle income country.","authors":"A Farrag, K Mohammed, M H Ghazaly, F Berthold","doi":"10.1080/08880018.2023.2256780","DOIUrl":"10.1080/08880018.2023.2256780","url":null,"abstract":"<p><p>Noncompliance with therapy is a big obstacle to successful therapy. We aimed to evaluate the prevalence and risk factors affecting the compliance of pediatric cancer patients with therapy in a tertiary care center far away from the capital in a lower-middle income country (LMIC). A retrospective cohort study of reports of all pediatric cancer patients who were diagnosed and started treatment between 2006 and 2010 at South Egypt Cancer Institute (SECI) was done. The following data were collected: Age, sex, diagnosis, compliance with therapy, and data on potential risk factors that might affect compliance, including time duration of travel from the patient's home to SECI, time lag between the first symptom until the first visit to SECI and until the start of treatment, results of reevaluation after the initial course of therapy, and therapy-related severe adverse events. Noncompliance with therapy was defined as when patients missed their determined therapy appointment for one week or more or abandoned therapy. This study included 510 patients. Eighty-three (16.3%) were non-compliant, as forty patients missed their therapy appointment (7.8%), and 43 abandoned further therapy (8.4%). Noncompliance was found to be more prevalent among patients with solid tumors. Non-compliant patients suffered a significantly higher relapse rate (47.7% vs. 11.2% in compliant patients, <i>p</i> < .001). Unfortunately, 75% of the abandoned patients who returned for further therapy suffered a relapse. Noncompliance with treatment is still a big problem facing cancer management in LMICs.</p>","PeriodicalId":19746,"journal":{"name":"Pediatric Hematology and Oncology","volume":null,"pages":null},"PeriodicalIF":1.7,"publicationDate":"2024-01-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"41159046","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":4,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
Pub Date : 2024-01-01Epub Date: 2024-02-10DOI: 10.1080/08880018.2023.2286970
Natsaruth Songthawee, Pornpun Sripornsawan, Shevachut Chavananon, Sirinthip Kittivisuit, Edward B McNeil, Thirachit Chotsampancharoen
Few studies have reported the survival outcomes of myeloid leukemia associated with Down syndrome (DS) in resource-limited countries. This study aimed to compare characteristics and survival outcomes of children with acute myeloid leukemia (AML) between those with and without DS in Thailand. The medical records of AML patients aged 0-15 years treated in a major tertiary center in Southern Thailand between October 1978 and December 2019 were reviewed retrospectively. The overall (OS) and event-free survivals (EFS) rates were calculated using the Kaplan-Meier method. A total of 362 AML patients were included, of which 41 (11.3%) had DS. The mean age at diagnosis of the DS patients was 2.5 ± 1.9 years and most of them (90.2%) were under the age of five. The DS patients had lower initial white blood cell counts and peripheral blasts compared to the non-DS patients. The AML-M7 subtype was more common in the DS than in the non-DS patients (80.5% vs. 9.1%, p < 0.01, respectively). The 5-year OS and EFS rates of the DS patients were lower compared to the non-DS patients (12.9% vs. 20.5%, p = 0.05 and 13.7% vs. 18.4%, p = 0.03, respectively). DS patients had a significantly higher rate of early and treatment-related deaths compared to non-DS patients (30.3% vs. 13.5%, p < 0.01 and 39.4% vs. 19.5%, p = 0.02, respectively). Over the study period, there were a decrease in early death rate and an increase in survival rates of DS patients, which suggests that chemotherapy regimens and supportive care have improved over time.
在资源有限的国家,很少有研究报道与唐氏综合征(DS)相关的髓系白血病的生存结局。本研究旨在比较泰国患有和不患有DS的急性髓性白血病(AML)儿童的特征和生存结局。回顾性回顾了1978年10月至2019年12月在泰国南部一家主要三级医疗中心治疗的0-15岁AML患者的医疗记录。采用Kaplan-Meier法计算总生存率(OS)和无事件生存率(EFS)。共纳入362例AML患者,其中41例(11.3%)患有DS。DS患者的平均诊断年龄为2.5±1.9岁,以5岁以下患者居多(90.2%)。与非DS患者相比,DS患者有较低的初始白细胞计数和外周细胞。AML-M7亚型在DS患者中比在非DS患者中更常见(分别为80.5%比9.1%,p = 0.05和13.7%比18.4%,p = 0.03)。与非退行性痴呆患者相比,退行性痴呆患者的早期死亡率和治疗相关死亡率明显更高(30.3%比13.5%,p p = 0.02)。在研究期间,退行性痴呆患者的早期死亡率有所下降,生存率有所提高,这表明化疗方案和支持性护理随着时间的推移有所改善。
{"title":"Survival outcomes of myeloid leukemia associated with Down syndrome and de novo acute myeloid leukemia in children: Experience from a single tertiary center in Thailand.","authors":"Natsaruth Songthawee, Pornpun Sripornsawan, Shevachut Chavananon, Sirinthip Kittivisuit, Edward B McNeil, Thirachit Chotsampancharoen","doi":"10.1080/08880018.2023.2286970","DOIUrl":"10.1080/08880018.2023.2286970","url":null,"abstract":"<p><p>Few studies have reported the survival outcomes of myeloid leukemia associated with Down syndrome (DS) in resource-limited countries. This study aimed to compare characteristics and survival outcomes of children with acute myeloid leukemia (AML) between those with and without DS in Thailand. The medical records of AML patients aged 0-15 years treated in a major tertiary center in Southern Thailand between October 1978 and December 2019 were reviewed retrospectively. The overall (OS) and event-free survivals (EFS) rates were calculated using the Kaplan-Meier method. A total of 362 AML patients were included, of which 41 (11.3%) had DS. The mean age at diagnosis of the DS patients was 2.5 ± 1.9 years and most of them (90.2%) were under the age of five. The DS patients had lower initial white blood cell counts and peripheral blasts compared to the non-DS patients. The AML-M7 subtype was more common in the DS than in the non-DS patients (80.5% vs. 9.1%, <i>p</i> < 0.01, respectively). The 5-year OS and EFS rates of the DS patients were lower compared to the non-DS patients (12.9% vs. 20.5%, <i>p</i> = 0.05 and 13.7% vs. 18.4%, <i>p</i> = 0.03, respectively). DS patients had a significantly higher rate of early and treatment-related deaths compared to non-DS patients (30.3% vs. 13.5%, <i>p</i> < 0.01 and 39.4% vs. 19.5%, <i>p</i> = 0.02, respectively). Over the study period, there were a decrease in early death rate and an increase in survival rates of DS patients, which suggests that chemotherapy regimens and supportive care have improved over time.</p>","PeriodicalId":19746,"journal":{"name":"Pediatric Hematology and Oncology","volume":null,"pages":null},"PeriodicalIF":1.7,"publicationDate":"2024-01-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"138445653","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":4,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
Severe sepsis and septic shock are life-threatening for pediatric hematology and oncology patient receiving chemotherapy. Th1/Th2 cytokines, C-reactive protein (CRP), and procalcitonin (PCT) are all thought to be associated with disease severity. The aim of this study was to prospectively verify the utility of Th1/Th2 cytokines and compare them with PCT and CRP in the prediction of adverse outcomes. Data on patients were collected from January 1, 2011, to December 31, 2020. Blood samples were taken for Th1/Th2 cytokine, CRP, and PCT measurements at the initial onset of infection. Severe infection (SI) was defined as severe sepsis or septic shock. Th1/Th2 cytokine levels were determined by using flow cytometric bead array technology. In total, 7,735 febrile episodes were included in this study. For SI prediction, the AUCs of IL-6, IL-10 and TNF-α were 0.814, 0.805 and 0.624, respectively, while IL-6 and IL-10 had high sensitivity and specificity. IL-6 > 220.85 pg/ml and IL-10 > 29.95 pg/ml had high odds ratio (OR) values of approximately 3.5 in the logistic regression. Within the subgroup analysis, for bloodstream infection (BSI) prediction, the AUCs of IL-10 and TNF-α were 0.757 and 0.694, respectively. For multiorgan dysfunction syndrome (MODS) prediction, the AUC of CRP was 0.606. The AUC of PCT for mortality prediction was 0.620. In conclusion, IL-6 and IL-10 provide good predictive value for the diagnosis of SI. For children with SI, IL-10 and TNF-α are associated with BSI, while CRP and PCT are associated with MODS and death, respectively.
{"title":"The predictive utility of cytokines, procalcitonin and C-reactive protein among febrile pediatric hematology and oncology patients with severe sepsis or septic shock.","authors":"Shu-Peng Lin, Xiao-Jun Xu, Chan Liao, Ning Zhao, Yuan-Yuan Chen, Hua Song, Wei-Qun Xu, Juan Liang, Di-Ying Shen, Jing-Ying Zhang, He-Ping Shen, Fen-Ying Zhao, Yong-Min Tang","doi":"10.1080/08880018.2023.2233567","DOIUrl":"10.1080/08880018.2023.2233567","url":null,"abstract":"<p><p>Severe sepsis and septic shock are life-threatening for pediatric hematology and oncology patient receiving chemotherapy. Th1/Th2 cytokines, C-reactive protein (CRP), and procalcitonin (PCT) are all thought to be associated with disease severity. The aim of this study was to prospectively verify the utility of Th1/Th2 cytokines and compare them with PCT and CRP in the prediction of adverse outcomes. Data on patients were collected from January 1, 2011, to December 31, 2020. Blood samples were taken for Th1/Th2 cytokine, CRP, and PCT measurements at the initial onset of infection. Severe infection (SI) was defined as severe sepsis or septic shock. Th1/Th2 cytokine levels were determined by using flow cytometric bead array technology. In total, 7,735 febrile episodes were included in this study. For SI prediction, the AUCs of IL-6, IL-10 and TNF-α were 0.814, 0.805 and 0.624, respectively, while IL-6 and IL-10 had high sensitivity and specificity. IL-6 > 220.85 pg/ml and IL-10 > 29.95 pg/ml had high odds ratio (OR) values of approximately 3.5 in the logistic regression. Within the subgroup analysis, for bloodstream infection (BSI) prediction, the AUCs of IL-10 and TNF-α were 0.757 and 0.694, respectively. For multiorgan dysfunction syndrome (MODS) prediction, the AUC of CRP was 0.606. The AUC of PCT for mortality prediction was 0.620. In conclusion, IL-6 and IL-10 provide good predictive value for the diagnosis of SI. For children with SI, IL-10 and TNF-α are associated with BSI, while CRP and PCT are associated with MODS and death, respectively.</p>","PeriodicalId":19746,"journal":{"name":"Pediatric Hematology and Oncology","volume":null,"pages":null},"PeriodicalIF":1.7,"publicationDate":"2024-01-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"9779608","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":4,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
Pub Date : 2024-01-01Epub Date: 2024-02-10DOI: 10.1080/08880018.2023.2261986
Hyun Jin Kim, Yeon Jung Lim
Iron deficiency anemia (IDA) can be caused by occult gastrointestinal (GI) blood loss; however, the endoscopic findings in children with anemia are unclear. The study aimed to determine the frequency and factors related to lesions in children with IDA undergoing endoscopy. We retrospectively analyzed the clinical and endoscopic findings of children with a laboratory-based diagnosis of IDA. Of 58 patients, 36 (62.1%) had upper GI tract lesions, with erosive gastritis being the most common lesion. Further, 26 patients underwent concomitant colonoscopy, and 12 (46.2%) had lower GI tract lesions. Overall, 44 (75.9%) patients had lesions in either the upper or lower GI tract. Helicobacter pylori infection was detected in 13 patients (22.4%). Patients with lesions found by endoscopy had significantly lower hemoglobin level (8.9 vs. 10.0 g/dL, p = 0.047) and mean corpuscular volume (75.5 vs. 80.9 fL, p = 0.038). The proportion of patients with previous treatment for IDA was also higher in those with lesions on endoscopy. In multivariate analysis, age of ≥10 years (odds ratio [OR], 6.00; 95% confidence Interval [CI], 0.56-10.75) and positive fecal occult blood test (FOBT) findings (OR, 2.25; 95% CI, 0.14-4.52) were factors related to GI lesions. The presence of GI symptoms was not associated with GI lesions. A high proportion of GI lesions were found by endoscopy in children with IDA in this study. Endoscopy should be considered in children with IDA even without GI symptoms, especially in older children, and those with positive FOBT results.
{"title":"Endoscopic findings and predictors of gastrointestinal lesions in children with iron deficiency anemia.","authors":"Hyun Jin Kim, Yeon Jung Lim","doi":"10.1080/08880018.2023.2261986","DOIUrl":"10.1080/08880018.2023.2261986","url":null,"abstract":"<p><p>Iron deficiency anemia (IDA) can be caused by occult gastrointestinal (GI) blood loss; however, the endoscopic findings in children with anemia are unclear. The study aimed to determine the frequency and factors related to lesions in children with IDA undergoing endoscopy. We retrospectively analyzed the clinical and endoscopic findings of children with a laboratory-based diagnosis of IDA. Of 58 patients, 36 (62.1%) had upper GI tract lesions, with erosive gastritis being the most common lesion. Further, 26 patients underwent concomitant colonoscopy, and 12 (46.2%) had lower GI tract lesions. Overall, 44 (75.9%) patients had lesions in either the upper or lower GI tract. <i>Helicobacter pylori</i> infection was detected in 13 patients (22.4%). Patients with lesions found by endoscopy had significantly lower hemoglobin level (8.9 <i>vs.</i> 10.0 g/dL, <i>p</i> = 0.047) and mean corpuscular volume (75.5 <i>vs.</i> 80.9 fL, <i>p</i> = 0.038). The proportion of patients with previous treatment for IDA was also higher in those with lesions on endoscopy. In multivariate analysis, age of ≥10 years (odds ratio [OR], 6.00; 95% confidence Interval [CI], 0.56-10.75) and positive fecal occult blood test (FOBT) findings (OR, 2.25; 95% CI, 0.14-4.52) were factors related to GI lesions. The presence of GI symptoms was not associated with GI lesions. A high proportion of GI lesions were found by endoscopy in children with IDA in this study. Endoscopy should be considered in children with IDA even without GI symptoms, especially in older children, and those with positive FOBT results.</p>","PeriodicalId":19746,"journal":{"name":"Pediatric Hematology and Oncology","volume":null,"pages":null},"PeriodicalIF":1.7,"publicationDate":"2024-01-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"41145722","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":4,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}