Pediatric Hematology Oncology Journal最新文献

{"title":"Management of hypersplenism in hemolytic anemias","authors":"Amita Mahajan","doi":"10.1016/j.phoj.2024.07.004","DOIUrl":"10.1016/j.phoj.2024.07.004","url":null,"abstract":"<div><p>The clinical course of patients with chronic hemolytic anemia can be complicated by the development of splenomegaly and consequent hypersplenism. This may warrant management by medical or surgical methods. Furthermore, in some patients, splenic manipulation may be warranted in the absence of hypersplenism, spleen being the primary site of red cell destruction. Wherever possible, splenectomy is avoided or deferred in view of the life-long risks of infection and thrombosis associated with this procedure. Optimal management in hemolytic anemia, therefore, incorporates prevention of hypersplenism as one of the key treatment goals.</p></div>","PeriodicalId":101004,"journal":{"name":"Pediatric Hematology Oncology Journal","volume":"9 4","pages":"Pages 223-227"},"PeriodicalIF":0.0,"publicationDate":"2024-07-17","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.sciencedirect.com/science/article/pii/S2468124524000597/pdfft?md5=8bb3f5b374070096de5653f7a9b28066&pid=1-s2.0-S2468124524000597-main.pdf","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"141853475","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":"Response to comment on ‘causal factors influencing the quality of treatment and survival in wilms tumor: A retrospective investigation’","authors":"Syed Ibrahim Bukhari,&nbsp;Zahra Saeed Ahmed,&nbsp;Javeria Saeed,&nbsp;Kiran Hilal,&nbsp;Zehra Fadoo,&nbsp;Naureen Mushtaq,&nbsp;Bilal Mazhar Qureshi,&nbsp;Sadaf Altaf","doi":"10.1016/j.phoj.2024.07.002","DOIUrl":"10.1016/j.phoj.2024.07.002","url":null,"abstract":"","PeriodicalId":101004,"journal":{"name":"Pediatric Hematology Oncology Journal","volume":"9 4","pages":"Pages 228-230"},"PeriodicalIF":0.0,"publicationDate":"2024-07-08","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.sciencedirect.com/science/article/pii/S2468124524000573/pdfft?md5=d8b4bde1ae0d4083d72be399b1319f4f&pid=1-s2.0-S2468124524000573-main.pdf","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"141694453","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":"Comment on ‘Causal factors influencing the quality of treatment and survival in Wilms tumor: A retrospective investigation’","authors":"Yogesh Kumar Sarin","doi":"10.1016/j.phoj.2024.07.001","DOIUrl":"10.1016/j.phoj.2024.07.001","url":null,"abstract":"","PeriodicalId":101004,"journal":{"name":"Pediatric Hematology Oncology Journal","volume":"9 4","pages":"Pages 265-266"},"PeriodicalIF":0.0,"publicationDate":"2024-07-04","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.sciencedirect.com/science/article/pii/S2468124524000561/pdfft?md5=641404e5336c06d9ba8a525e05d7b06a&pid=1-s2.0-S2468124524000561-main.pdf","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"141710179","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 cancer research trends and performance in Africa: A bibliometric analysis from 1991 to 2022","authors":"Moawia Mohammed Ali Elhassan ,&nbsp;Ibrahim Mahmoud ,&nbsp;Ibrahim Qaddoumi ,&nbsp;Verna Vanderpuye ,&nbsp;Jeannette Parkes ,&nbsp;Yuh-Shan Ho","doi":"10.1016/j.phoj.2024.06.006","DOIUrl":"10.1016/j.phoj.2024.06.006","url":null,"abstract":"<div><h3>Background</h3><p>Childhood cancer rates in Africa are lower than in high-income countries but increasing, making pediatric cancers a significant public health concern. The purpose of this study was to provide an overview of the publication of pediatric cancer research in Africa, including publication types and citation trends over time.</p></div><div><h3>Methods</h3><p>The Science Citation Index Expanded database within the Web of Science Core Collection was searched for articles published from 1991 to 2022 in the topic domain, using title, abstract, author keywords, and <em>KeyWords Plus</em>. Indicators used to assess publications performance of the countries included: total number of publications, single-country publications, collaborative publications with African countries, collaborative publications with non-African countries, first-author publications, corresponding-author publications, and single-author publications.</p></div><div><h3>Results</h3><p>A total of 4461 relevant documents were retrieved, with 2770 original research articles. Annual number of articles rose from 13 articles in 1991 to 287 in 2022. Most articles were produced by Egypt (28 %) in North Africa, followed by South Africa (20 %) in sub-Saharan Africa. Collaboration between African countries remains low; however, international collaborations have enhanced the average number of citations per article. Most of the first authors (65 %) and corresponding authors (67 %) of these international collaborative articles were affiliated with non-African institutions.</p></div><div><h3>Conclusions</h3><p>The number of publications sharply increased over the study period and diversely represents African countries. Collaborations with international partners increased citations; however, few of these publications had African first authors. Conversely, inter-institutional partnerships between African countries were relatively low, highlighting the need for better collaboration within Africa.</p></div>","PeriodicalId":101004,"journal":{"name":"Pediatric Hematology Oncology Journal","volume":"9 4","pages":"Pages 211-218"},"PeriodicalIF":0.0,"publicationDate":"2024-07-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.sciencedirect.com/science/article/pii/S246812452400055X/pdfft?md5=355fa52d5a781c837ffebf07e41f497a&pid=1-s2.0-S246812452400055X-main.pdf","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"141707937","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":"Duodenal obstruction: A rare complication of langerhans cell histiocytosis","authors":"Vaishnavi Sreenivasan ,&nbsp;Anmol Aatli ,&nbsp;Saahiti Andhavaram ,&nbsp;Apoorva Sharma ,&nbsp;Rashmi Dandriyal ,&nbsp;Shyam S. Meena ,&nbsp;Nidhi Sugandhi ,&nbsp;Sumit Mehndiratta ,&nbsp;Nidhi Chopra ,&nbsp;Amitabh Singh","doi":"10.1016/j.phoj.2024.06.005","DOIUrl":"10.1016/j.phoj.2024.06.005","url":null,"abstract":"<div><h3>Background</h3><p>Langerhans cell histiocytosis (LCH) has a variable presentation. Gastrointestinal involvement is rare and portends a poor prognosis in LCH.</p></div><div><h3>Case report</h3><p>Herein, we describe the presentation, progression, management, and outcome of an 11-month-old female infant with LCH. The patient presented with severe acute malnutrition, hepatosplenomegaly, and gastrointestinal involvement in the form of persistent vomiting. She was managed with chemotherapy initially and subsequently developed complete duodenal narrowing. She underwent Kimura's duodenojejunostomy and improved. Hence, chemotherapy was restarted. Two months later, she developed features of intestinal obstruction with intraoperative findings of ileal gangrene requiring adhesiolysis, ileal resection, and ileostomy. Subsequently, she succumbed to sepsis.</p></div><div><h3>Conclusion</h3><p>This case report aims to highlight the atypical gastrointestinal manifestations of LCH and the challenges faced in managing such a case.</p></div>","PeriodicalId":101004,"journal":{"name":"Pediatric Hematology Oncology Journal","volume":"9 3","pages":"Pages 207-210"},"PeriodicalIF":0.0,"publicationDate":"2024-06-24","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.sciencedirect.com/science/article/pii/S2468124524000548/pdfft?md5=dc370ba1f4347d204547664c24f697a7&pid=1-s2.0-S2468124524000548-main.pdf","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"141954461","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":"Differential metabolomic pathway analysis in Malaysian childhood acute lymphoblastic leukemia patients treated with high-dose methotrexate","authors":"Rizal Husaini Razali ,&nbsp;Lay Kek Teh ,&nbsp;Mohd Zaki Salleh ,&nbsp;Kok Hoi Teh ,&nbsp;Hishamshah Mohd Ibrahim","doi":"10.1016/j.phoj.2024.06.004","DOIUrl":"10.1016/j.phoj.2024.06.004","url":null,"abstract":"<div><h3>Background</h3><p>Methotrexate (MTX) is the mainstay of the consolidation and maintenance phase of chemotherapy protocol for childhood acute lymphoblastic leukemia (ALL)<strong>.</strong> This study aimed to investigate the altered metabolism associated with high dose-MTX and determine the potential of the metabolic markers and differential pathways involved in MTX therapy.</p></div><div><h3>Methods</h3><p>Serum samples were collected from 38 children with ALL at 2 time points: <em>p</em>-MTX; before induction chemotherapy was initiated, and post-MTX; after completion of the first high-dose MTX. The samples were analyzed using HPLC/MS-QTOF. Data acquisition was performed using Agilent MassHunterTMB.05.00 software for subsequent metabolomics analysis. Differential expressions of metabolites were analyzed using univariate Welch's <em>t</em>-test unequal variance. Compounds were identified using the METLIN Database. Pathways and network analyses were performed using Metaboanalyst 4.0. Potential biomarkers were analyzed using the Receiving Operator Characteristic curve.</p></div><div><h3>Results</h3><p>Metabolites with AUC between 0.7 and 0.9 include xanthine (0.889), oxoglutaric acid (0.770), and alpha-linolenic acid (ALA) (0.741). Alpha-linolenic acid abundance was detected in ALL patients with remission status, corresponding to a test sensitivity and specificity of 0.77 and 0.87, respectively. ALA has an antineoplastic effect that potentially inhibits the proliferation of leukemic cells by inhibiting caspase activation in the phosphatidylinositol 3-kinase (PI3K) pathway and Bcl-2 inhibition.</p></div><div><h3>Conclusion</h3><p>In this study, ALA was found to be significantly higher in patients treated with high-dose MTX and associated with remission status than in pre-MTX treatment.</p></div>","PeriodicalId":101004,"journal":{"name":"Pediatric Hematology Oncology Journal","volume":"9 3","pages":"Pages 193-199"},"PeriodicalIF":0.0,"publicationDate":"2024-06-20","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.sciencedirect.com/science/article/pii/S2468124524000536/pdfft?md5=d9f973c17d3c9ba9d751b087d721fde8&pid=1-s2.0-S2468124524000536-main.pdf","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"141952705","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":"A case report of a preterm infant with KBG syndrome and hepatoblastoma","authors":"Kyoung Sung Yun ,&nbsp;Seung Han Shin ,&nbsp;Jaemoon Koh ,&nbsp;Jung Min Ko ,&nbsp;Jung Yoon Choi ,&nbsp;Nam-Joon Yi","doi":"10.1016/j.phoj.2024.06.003","DOIUrl":"10.1016/j.phoj.2024.06.003","url":null,"abstract":"<div><h3>Background</h3><p>KBG syndrome is a rare autosomal dominant genetic disease characterized by facial dysmorphism, developmental disorders, and short stature. The syndrome is caused by the haploinsufficiency of the ankyrin repeat domain-containing protein 11 (ANKRD11). Recently, there have been concerns that <em>ANKRD11</em> serves as a tumor suppressor gene. Herein, we report a patient with KBG syndrome, diagnosed with hepatoblastoma at 21 months of age, which required chemotherapy and liver transplantation.</p></div><div><h3>Case report</h3><p>A male infant born by cesarean section at 29⁺¹ weeks of gestation, weighing 1220 g, was diagnosed with pulmonary atresia with ventricular septal defect and large patent ductus arteriosus. Bilateral low-set ears with a preauricular skin tag, left microtia, swelling of left neck, single umbilical artery, bilateral undescended testis, large anterior fontanelle, and bilateral club foot were noted. At a corrected age (CA) of 7 months, exome sequencing was performed, and a heterozygous nonsense pathogenic variant of ANKRD11 was found (c.7195C &gt; T, p.Gln2399∗). Therefore, KBG syndrome was confirmed. At CA of 18 months, the patient presented with lethargy, poor oral intake, and jaundice symptoms and was diagnosed with hepatoblastoma. He received 12 cycles of chemotherapy for 9 months, but multiple hepatic masses remained; therefore, liver transplantation was performed at 28 months of age.</p></div><div><h3>Conclusions</h3><p>KBG syndrome is a rare genetic disorder that has been identified relatively recently, and not all characteristics have yet been identified. The study of our case may provide evidence of cancer risk in patients with KBG syndrome.</p></div>","PeriodicalId":101004,"journal":{"name":"Pediatric Hematology Oncology Journal","volume":"9 3","pages":"Pages 184-188"},"PeriodicalIF":0.0,"publicationDate":"2024-06-14","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.sciencedirect.com/science/article/pii/S2468124524000524/pdfft?md5=e7baeaffe48ca9a2a55ecc58321b4d92&pid=1-s2.0-S2468124524000524-main.pdf","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"141404663","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":"Alloimmunization and autoimmunization among multitransfused thalassemia and sickle cell disease patients","authors":"","doi":"10.1016/j.phoj.2024.06.002","DOIUrl":"10.1016/j.phoj.2024.06.002","url":null,"abstract":"<div><p>Blood transfusion is the mainstay for management of hemoglobinopathies, including thalassemia and sickle cell disease (SCD). Apart from other transfusion related adverse effects, immunization is a significant complication, particularly in multitransfused recipients. Alloimmunization rates vary widely, both in thalassemia and SCD patients. Alloimmunization complicates the management in form of hemolytic transfusion reactions, difficulty in finding compatible units, delays in transfusion and increasing costs. Exact pathogenesis and prevention modality have not been elucidated. Apart from antigenic differences, immunomodulatory effects and inflammation also play a role in pathogenesis. Ethnic heterogeneity between donors and recipients predisposes to higher alloimmunization.</p><p>Transfusion management of hemoglobinopathies across the globe is fragmented, with different levels of care. Guidelines propose red cell antigen profiling, transfusion of leucoreduced red cells, preferably matched for Rh and Kell antigens, over and above ABO-D matching. For alloimmunized patients, corresponding antibody negative red cells need to be transfused. However, patients from different backgrounds are variably transfused with whole blood/red cells, which may or may not be leukoreduced. Prophylactic antigen matching reduces the risk of alloimmunization, however, has limitations. RH variants may not be picked up by phenotyping alone. Moreover, the approach of prophylactic antigen matching is expensive, labour intensive, time consuming and may cause delays in transfusions. There is limited, low quality evidence on best practices for transfusion management of SCD and thalassemia. The review analyses the magnitude of problem, factors contributing and modalities for prevention and management of immunization.</p></div>","PeriodicalId":101004,"journal":{"name":"Pediatric Hematology Oncology Journal","volume":"9 3","pages":"Pages 200-206"},"PeriodicalIF":0.0,"publicationDate":"2024-06-13","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.sciencedirect.com/science/article/pii/S2468124524000433/pdfft?md5=56ff7c4af608997ffdbd584d0764c928&pid=1-s2.0-S2468124524000433-main.pdf","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"141394362","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":"Fat embolism syndrome in a child with sickle cell disease","authors":"Sohini Chakraborty ,&nbsp;Arun Danewa ,&nbsp;Sunisha Arora ,&nbsp;Saurabh Bansal ,&nbsp;Parminder Pal Singh ,&nbsp;Rahul Bhargava ,&nbsp;Vikas Dua","doi":"10.1016/j.phoj.2024.06.001","DOIUrl":"10.1016/j.phoj.2024.06.001","url":null,"abstract":"<div><h3>Background</h3><p>Fat embolism syndrome (FES) is a rare and underdiagnosed complication associated with sickle cell disease.</p></div><div><h3>Case report</h3><p>We report the case of a 12-year-old girl with sickle cell disease who initially presented with features of acute vaso-occlusive crisis. However, she later developed neurological and respiratory complications along with a decrease in hemoglobin and platelet counts. She was evaluated and diagnosed with FES. The patient was successfully managed with red cell exchange and therapeutic plasma exchange, leading to complete recovery.</p></div><div><h3>Conclusion</h3><p>Prompt diagnosis and early management can significantly improve the outcome of this rare, though highly morbid complication.</p></div>","PeriodicalId":101004,"journal":{"name":"Pediatric Hematology Oncology Journal","volume":"9 3","pages":"Pages 189-192"},"PeriodicalIF":0.0,"publicationDate":"2024-06-13","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.sciencedirect.com/science/article/pii/S2468124524000421/pdfft?md5=4fcc8aa31ba5e28d0f62b08edc752886&pid=1-s2.0-S2468124524000421-main.pdf","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"141395167","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":"Editorial Board Information","authors":"","doi":"10.1016/S2468-1245(24)00047-0","DOIUrl":"https://doi.org/10.1016/S2468-1245(24)00047-0","url":null,"abstract":"","PeriodicalId":101004,"journal":{"name":"Pediatric Hematology Oncology Journal","volume":"9 2","pages":"Page ii"},"PeriodicalIF":0.0,"publicationDate":"2024-06-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.sciencedirect.com/science/article/pii/S2468124524000470/pdfft?md5=b4ee42e6927f006215ea0db256d306ec&pid=1-s2.0-S2468124524000470-main.pdf","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"141482341","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}