journal
Journals Journal of Pediatric Hematolog...

Journal of Pediatric Hematology/oncology

https://read.qxmd.com/read/37027191/assessment-of-factors-associated-with-the-evaluation-of-children-for-leukemia-predisposition-syndromes-a-retrospective-single-center-study
#21
JOURNAL ARTICLE
Kristen R D'Aquila, Elena Kessler, Kristine L Cooper, Andrea Durst, Julia Meade
Five to 10% of children with cancer are thought to have a cancer predisposition syndrome (CPS). Referral guidelines for leukemia predisposition syndromes are limited and vague, requiring the treating provider to determine whether patients should have a genetics evaluation. We evaluated referrals to the pediatric cancer predisposition clinic (CPP), the prevalence of CPS in those who elected to pursue germline genetic testing, and assessed for associations between a patient's medical history and the diagnosis of a CPS...
March 16, 2023: Journal of Pediatric Hematology/oncology
https://read.qxmd.com/read/36898054/precursor-b-cell-lymphoblastic-lymphoma-in-children-hacettepe-experience
#22
JOURNAL ARTICLE
Hilal Susam-Sen, Bilgehan Yalcin, Diclehan Orhan, Burca Aydin, Nilgun Kurucu, Ali Varan, Aysegul Uner, Tezer Kutluk
The purpose of the study was to review the clinical and pathologic characteristics and treatment results of children with precursor B-cell lymphoblastic lymphoma. Of 530 children diagnosed with non-Hodgkin lymphomas between 2000 and 2021, 39 (7.4%) were identified as having precursor B-cell lymphoblastic lymphoma. Clinical characteristics, pathologic, radiologic, laboratory data, treatments, responses, and overall outcomes were recorded from hospital files and analyzed. The median age of 39 patients (males/females, 23/16) was 8...
March 13, 2023: Journal of Pediatric Hematology/oncology
https://read.qxmd.com/read/37083275/subcutaneous-microabscesses-and-myositis-as-part-of-immune-reconstitution-inflammatory-syndrome-due-to-chronic-disseminated-candidiasis-in-a-child-with-acute-lymphoblastic-leukemia
#23
JOURNAL ARTICLE
Nalla A Reddy, Soundarya Addala, Shyam S S, Harsha P Lashkari
BACKGROUND: Immune reconstitution inflammatory syndrome (IRIS) occurs when there is immune recovery after a prolonged period of leucopenia as a response to an underlying latent or chronic infection due to a proinflammatory cascade. It can occur in a child on chemotherapy for acute lymphoblastic leukemia (ALL) with underlying chronic disseminated candidiasis (CDC). OBSERVATION: We present a 7-year-old girl with pre-B ALL on chemotherapy who had prolonged febrile neutropenia and CDC with microabscesses in the liver, spleen, and kidney and a prolonged intensive care unit stay...
May 1, 2023: Journal of Pediatric Hematology/oncology
https://read.qxmd.com/read/37083274/treatment-refractory-soft-tissue-myoepithelial-carcinoma-with-an-arid1a-mutation
#24
JOURNAL ARTICLE
Cody R Ashcroft, Scott W Penney, Susan L Whiteway
Soft tissue myoepithelial carcinoma is a rare tumor first reported in the salivary gland. There is considerable tumor heterogeneity between pathology findings, tumor aggressiveness, and response to treatment. Recent molecular testing has identified recurrent genetic changes with PLAG mutations in salivary gland primary tumors and loss of SMARCB1 and EWSR1/FUS gene changes in myoepithelial carcinoma. SMARCB1 is a component of the switch/sucrose nonfermentable (SWI/SNF) complex, an essential cellular regulator...
May 1, 2023: Journal of Pediatric Hematology/oncology
https://read.qxmd.com/read/37083273/covid-19-infection-and-outcomes-in-newborn-screening-cohorts-of-sickle-cell-trait-and-sickle-cell-disease-in-michigan-and-georgia
#25
JOURNAL ARTICLE
Susan T Paulukonis, Angela Snyder, Matthew P Smeltzer, Ankit N Sutaria, Isabel Hurden, Krista Latta, Swathi Chennuri, Elliott Vichinsky, Sarah L Reeves
The sickle cell mutation increases morbidity in those with sickle cell disease (SCD) and potentially sickle cell trait, impacting pulmonary, coagulation, renal, and other systems that are implicated in COVID-19 severity. There are no population-based registries for hemoglobinopathies, and they are not tracked in COVID-19 testing. We used COVID-19 test data from 2 states linked to newborn screening data to estimate COVID outcomes in people with SCD or trait compared with normal hemoglobin. We linked historical newborn screening data to COVID-19 tests, hospitalization, and mortality data and modeled the odds of hospitalization and mortality...
May 1, 2023: Journal of Pediatric Hematology/oncology
https://read.qxmd.com/read/37027323/skin-toxicity-due-to-mercaptopurine-in-maintenance-therapy-among-children-with-acute-lymphoblastic-leukemia
#26
JOURNAL ARTICLE
Kayeleigh Higgerson, Terrie Flatt
Mercaptopurine is a crucial component in the treatment of acute lymphoblastic leukemia. It is associated with toxicities that can delay treatment. Mercaptopurine is metabolized into 6-thioguanine nucleotides and 6-methylomercaptopurine nucleotides (6MMPN). Accumulation of 6MMPN has previously been associated with hepatotoxicity, pancreatitis, and hypoglycemia. However, skin toxicity has rarely been reported. We report 5 cases of elevated 6MMPN levels associated with cutaneous manifestations.
May 1, 2023: Journal of Pediatric Hematology/oncology
https://read.qxmd.com/read/36730444/success-of-trametinib-in-the-treatment-of-langerhans-cell-histiocytosis-with-novel-mapk-pathway-mutations
#27
JOURNAL ARTICLE
Kaci Orr, Samantha Hustak, Rebecca Beaudoin, Anish Ray
Approximately a third of patients with Langerhans cell histiocytosis (LCH) experience recurrence of disease. Genomic analysis has revealed that this condition is often driven by oncogenic mutations in the MAP kinase (MAPK) pathway, and agents that target components of this pathway have been explored as a second-line treatment for LCH. Here, we examine 2 pediatric patients with LCH and confirmed but rarely reported MAPK pathway mutations treated with trametinib, a MAP2K inhibitor approved to treat several cancers with a BRAFV600E mutation...
May 1, 2023: Journal of Pediatric Hematology/oncology
https://read.qxmd.com/read/36729669/safety-and-efficacy-of-alendronate-to-treat-osteopenia-in-children-during-therapy-for-acute-lymphoblastic-leukemia-a-retrospective-cohort-study-of-sequential-outcomes
#28
JOURNAL ARTICLE
Paula MacDonald, Amy Cranston, Misha Virdee, Troy Farncombe, Uma Athale, Ronald D Barr
BACKGROUND: Low bone mineral density is encountered in children with acute lymphoblastic leukemia (ALL) before, during, and after treatment. Prior experience with alendronate, an oral bisphosphonate, demonstrated high tolerability and evident clinical efficacy. However, concerns have been expressed about the long-term safety and utility of such agents in children. PROCEDURE: Sixty-nine children with ALL received alendronate for a mean of 87 weeks after dual-energy radiograph absorptiometry...
May 1, 2023: Journal of Pediatric Hematology/oncology
https://read.qxmd.com/read/36952466/contributions-of-arid5b-ikzf1-pip4k2a-and-gata3-gene-polymorphisms-to-childhood-acute-lymphoblastic-leukemia-in-a-chinese-population
#29
JOURNAL ARTICLE
Xiaorong Liu, Min Xiao, Zhihao Xing, Hanfang Jiang, Chunqing Zhu, Xingliang Zhang, Weiqiong Li, Zijie Wang, Feng Wu, Yunsheng Chen
Various studies have shown that single nucleotide polymorphisms in the AT-rich interaction domain 5B (ARID5B), IKAROS family zinc finger 1 (IKZF1), phosphatidylinositol-5-phosphate 4-kinase type 2 alpha (PIP4K2A), and GATA binding protein 3 (GATA3) genes may be associated with the susceptibility and prognosis of childhood acute lymphoblastic leukemia (ALL). The present study aimed to investigate the association of ARID5B rs10821936, IKZF1 rs4132601, PIP4K2A rs7088318, and GATA3 rs3824662 gene polymorphisms with the susceptibility and prognosis of childhood ALL in China...
April 1, 2023: Journal of Pediatric Hematology/oncology
https://read.qxmd.com/read/36731007/lncrna-gas5-and-runx1-genes-in-children-with-primary-immune-thrombocytopenia
#30
JOURNAL ARTICLE
Hossam Hodeib, Doaa El Amrousy, Eman Elaskary, Nahed Hablas, Amira Youssef, Dina Abdelhai
We aimed to evaluate the expression levels and the prognostic value of growth arrest specific 5 (GAS5) and runt-related transcription factor 1 (RUNX1) genes in children with ITP. This prospective cohort study included 100 patients with newly diagnosed ITP (patient group) and 100 healthy children of matched age and sex (control group). We evaluated the expression levels of both GAS5 and RUNX1 genes at the time of diagnosis before the introduction of treatment. GAS5 was under-expressed, while RUNX1 was over-expressed among the newly diagnosed ITP children compared with the control group...
April 1, 2023: Journal of Pediatric Hematology/oncology
https://read.qxmd.com/read/36706306/utility-of-whole-exome-sequencing-in-the-early-diagnosis-of-atypical-diamond-blackfan-anemia
#31
JOURNAL ARTICLE
Abdulla Al-Mulla, Frances Austin, Marieka Helou
Diamond-Blackfan anemia (DBA) is a rare congenital bone marrow failure syndrome, with a hallmark of erythroblastopenia and congenital anomalies. DBA demonstrates genetic heterogeneity and variable phenotypic expression. We present two cases of atypical DBA harboring de novo mutations in the RPS-19 gene with c.49 G>C and c.357-1G>T allelic variants. The two cases presented confounding critical illness demonstrated by multiorgan failure, aplastic crisis, with case 2 meeting the criteria for hemophagocytic lymphohistiocytosis...
April 1, 2023: Journal of Pediatric Hematology/oncology
https://read.qxmd.com/read/36898050/mature-cystic-teratoma-of-the-gall-bladder-masquerading-a-hepatic-space-occupying-lesion
#32
JOURNAL ARTICLE
Jagadeesh Menon, Naresh Shanmugam, Shamnad Madathil, Mukul Vij, Ashwin Rammohan, Mohamed Rela
No abstract text is available yet for this article.
March 6, 2023: Journal of Pediatric Hematology/oncology
https://read.qxmd.com/read/36898038/description-of-a-colocated-comprehensive-care-model-for-people-with-sickle-cell-and-comorbid-pulmonary-disease
#33
JOURNAL ARTICLE
Courtney Pugh, Rachel N Zeno, Joseph Stanek, Michelle Gillespie, Benjamin T Kopp, Susan E Creary
Comorbid pulmonary complications in people with sickle cell disease (pwSCD) are associated with high rates of morbidity and mortality, and poor access to care contributes to poor outcomes among this particularly high-risk pwSCD. Our purpose was to describe the population served and the resources required for hematology, pulmonary, nursing, respiratory therapy, social work, genetics, psychology, and school liaison providers to see these patients in an integrated clinic. We abstracted demographic, medication, clinical, and diagnostics data of the pwSCD seen at least once in this clinic from February 1, 2014 to December 10, 2020 from the electronic medical record and identified 145 unique pwSCD...
March 6, 2023: Journal of Pediatric Hematology/oncology
https://read.qxmd.com/read/36898046/clinical-significance-of-plasma-soluble-micb-in-children-with-ebv-associated-hemophagocytic-lymphohistiocytosis
#34
JOURNAL ARTICLE
Ang Wei, Liping Zhang, Honghao Ma, Lei Cui, Qing Zhang, Dong Wang, Sitong Chen, Junye Du, Xiaoxi Zhao, Tianyou Wang, Rui Zhang, Huyong Zheng, Zhigang Li
BACKGROUND: Hemophagocytic lymphohistiocytosis (HLH) is a potentially fatal systemic inflammation disease in children. The most common cause is Epstein-Barr virus (EBV) infection. MHC class I polypeptide-related sequence B (MICB) is a membrane protein inducibly expressed upon cellular stress, viral infection, or malignant transformation, thus marking these cells for clearance through natural killer group 2 member D-positive lymphocytes. MICB can be released into plasma through several mechanisms, reducing NK cell cytotoxicity...
May 1, 2023: Journal of Pediatric Hematology/oncology
https://read.qxmd.com/read/36898042/next-steps-survivor-healthcare-passport-a-novel-form-of-survivorship-care-plan-in-the-pediatric-population
#35
JOURNAL ARTICLE
M Clarise Valencia, Lindsay Hang, Deb Schmidt, Julie Nichols, Jian Zhang, Ke Yan, Alyssa Gonzalez, Cindy L Schwartz, Rachel Phelan
PURPOSE: Survivorship care plans (SCPs) have been instrumental in aiding transition from cancer treatment to survivorship care, which contains the diagnosis, treatment, potential late effects, and recommended follow-up. There has been paucity of research on its efficacy and lack of guidelines on development and delivery of SCPs. The Next Steps Survivorship Clinic at Children's Wisconsin uses a Survivorship Healthcare Passport (SHP), a SCP pocket-sized card. This study aims to improve understanding of patient and parent use of the SHP at a single institution...
March 3, 2023: Journal of Pediatric Hematology/oncology
https://read.qxmd.com/read/36898033/a-rare-report-of-the-coexistence-of-sickle-cell-disease-neurofibromatosis-type-1-and-intracranial-hypertension-in-a-pediatric-patient
#36
JOURNAL ARTICLE
Amie Patel, Timothy Winter, Akshat Jain
A pediatric female with sickle cell disease (SCD) and neurofibromatosis type 1 was noted to have incidental papilledema, with subsequent workup showing an elevated opening pressure. She was diagnosed with intracranial hypertension and began treatment with acetazolamide. Hydroxyurea was also discontinued. Acetazolamide was tapered off, and hydroxyurea was restarted with no worsening in her ophthalmologic exam. We report this case due to the rare occurrence of all 3 conditions, and while intracranial hypertension has been reported in SCD, the diagnostic workup for papilledema in hemoglobinopathies is not well defined...
March 3, 2023: Journal of Pediatric Hematology/oncology
https://read.qxmd.com/read/36898034/a-cross-sectional-survey-to-review-food-safety-practices-within-pediatric-oncology-and-stem-cell-transplant-centers-in-the-united-kingdom
#37
JOURNAL ARTICLE
Alisa Morris, Graeme O' Connor, Gemma Renshaw
FOOD SAFETY AND PEDIATRIC CANCER: Neutropenia is a common complication of chemotherapy, which poses a high risk of infection and mortality. Neutropenic diet has historically been recommended for those undergoing chemotherapy. The rationale is to reduce the risk of foodborne infection by avoiding foods considered to be of high microbial risk. However, evidence for this diet is limited, and there is a lack of national consensus guidelines. AIM: Ascertain food safety advice across specialist centers providing high-dose chemotherapy for malignancy or stem cell transplants in the United Kingdom...
March 1, 2023: Journal of Pediatric Hematology/oncology
https://read.qxmd.com/read/36898032/prognostic-factors-and-long-term-outcomes-in-41-children-with-primary-hemophagocytic-lymphohistiocytosis-report-of-a-single-center-experience-and-review-of-the-literature
#38
JOURNAL ARTICLE
Cengiz Bayram, Tuba Nur Tahtakesen, Esra Arslantaş, Ebru Yilmaz, Gül Nihal Özdemir, Ezgi Pasli Uysalol, Müge Gökçe, Arzu Akçay, Deniz Tuğcu, Ali Ayçiçek
Hemophagocytic lymphohistiocytosis (HLH) is a rare life-threatening hyperinflammatory syndrome with diverse clinical manifestations leading to major diagnostic and therapeutic difficulties. This study aimed to evaluate clinical manifestations, prognostic factors, and long-term outcomes in children with primary HLH. Forty-one patients diagnosed with primary HLH were retrospectively evaluated for patient characteristics, HLH gene mutations, clinical and laboratory manifestations, prognostic factors, and long-term outcomes...
March 1, 2023: Journal of Pediatric Hematology/oncology
https://read.qxmd.com/read/36757045/successful-tcr%C3%AE-%C3%AE-cd19-depleted-hematopoietic-cell-transplantation-for-a-patient-with-artemis-deficiency
#39
JOURNAL ARTICLE
Dan Tomomasa, Takeshi Isoda, Noriko Mitsuiki, Kento Inoue, Akira Nishimura, Kazuhiro Uda, Toru Uchiyama, Motoi Yamashita, Takahiro Kamiya, Akifumi Endo, Masatoshi Takagi, Kohsuke Imai, Michiko Kajiwara, Morton J Cowan, Tomohiro Morio, Hirokazu Kanegane
Artemis deficiency is characterized by DNA double-strand breaks repairing dysfunction and increased sensitivity to ionizing radiation and alkylating reagents. We describe the first successful case of T-cell receptor [TCR]αβ/CD19-depleted hematopoietic cell transplantation [HCT] for Artemis deficiency in Japan. A 6-month-old Korean boy was diagnosed with Artemis-deficient severe combined immunodeficiency. He had no human leukocyte antigen (HLA)-matched sibling or unrelated donor. Therefore, TCRαβ/CD19-depleted HCT from his haploidentical mother was performed...
March 1, 2023: Journal of Pediatric Hematology/oncology
https://read.qxmd.com/read/36716049/evidence-of-chemoresponsiveness-in-unresectable-metastatic-angiomatoid-fibrous-histiocytoma
#40
JOURNAL ARTICLE
Elizabeth A Corley, Erika Pace, Alex M Barnacle, Premal A Patel, Khin Thway, Julia C Chisholm
Angiomatoid fibrous histiocytoma (AFH) is a soft tissue neoplasm of intermediate biological potential. Typically a slow-growing tumor, it can recur locally. Rarely, it manifests as a soft tissue sarcoma capable of metastasis. When metastases are nonamenable to local therapy, it is believed uniformly fatal. We present 3 patients with metastatic AFH who demonstrated a sustained response to chemotherapy; including one who achieved complete remission with cryoablation. These cases reinforce the potential value of chemotherapy in some patients with unresectable metastatic AFH and provide the first case in the literature of cryoablation in AFH...
March 1, 2023: Journal of Pediatric Hematology/oncology
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