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Pediatric Hematology and Oncology

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https://read.qxmd.com/read/30702381/segmental-uniparental-disomy-as-a-rare-cause-of-congenital-severe-factor-xiii-deficiency-in-a-girl-with-only-one-heterozygous-carrier-parent
#1
Ming-Ching Shen, Ming Chen, Shung-Ping Chang, Po-Te Lin, Han-Ni Hsieh, Kai-Hsin Lin
Uniparental disomy (UPD) refers to a situation when a person inherits both homologs of a region or complete part of a chromosome from only one parent. Here, we present an unusual case of UPD in congenital severe factor (F) XIII deficiency. A 6-year-old girl experienced cephalhematoma and umbilical bleeding after birth and easy bruising, and postextraction bleeding since early infancy. FXIII activity was 0% [mother 53.7% and father 132.5% (normal 70-140%)] and the FXIII antigen level was 2.5% [mother 38.9% and father 151% (normal 75-155%)]...
January 31, 2019: Pediatric Hematology and Oncology
https://read.qxmd.com/read/30693828/outcome-of-children-with-langerhans-cell-histiocytosis-and-single-system-involvement-a-retrospective-study-at-a-single-center-in-shanghai-china
#2
Meng Su, Yi-Jin Gao, Ci Pan, Jing Chen, Jing-Yan Tang
BACKGROUND: This is a descriptive review of the clinical patterns and outcomes of children with Langerhans cell histiocytosis and single-system involvement (SS-LCH) treated at Shanghai Children's Medical Center. PROCEDURE: 60 evaluable newly diagnosed patients (37 boys, 23 girls) with a median age of 3.9 years (range: 0.3-15.3 years) and histiopathology-confirmed SS-LCH were enrolled from 2010 to 2014. All patients received systemic chemotherapy using either the DAL HX-83 or LCH-II protocol as determined by the physician...
January 29, 2019: Pediatric Hematology and Oncology
https://read.qxmd.com/read/30681039/high-dose-chemotherapy-and-autologous-hematopoietic-stem-cell-rescue-for-treatment-of-relapsed-and-refractory-wilms-tumor-re-evaluating-outcomes
#3
Jenna Rossoff, William T Tse, Reggie E Duerst, Jennifer Schneiderman, Elaine Morgan, Morris Kletzel, Sonali Chaudhury
Wilms tumor (WT) treatment regimens are curative for more than 80% of patients, but those with relapsed or refractory disease continue to have poor outcomes. High-dose chemotherapy followed by autologous stem cell rescue is often utilized although outcomes remain variable. We report on HD-ASCR outcomes in 24 patients with relapsed or refractory Wilms tumor. Three-year disease free and overall survival are 46% and 60%, respectively, which is similar to those reported for conventional salvage therapies. These outcomes suggest that conventional salvage therapies should be employed for relapsed and refractory WT rather than HD-ASCR...
January 25, 2019: Pediatric Hematology and Oncology
https://read.qxmd.com/read/30681025/factors-affecting-same-day-cancelation-of-outpatient-pediatric-oncologic-procedural-sedation
#4
Jocelyn R Grunwell, Lillian D Long, Leah N Bryan, Tammy Kelley, Himalee S Sabnis, Harold K Simon, Pradip P Kamat
BACKGROUND: Children with cancer undergo serial invasive, painful procedures as a part of their diagnosis, treatment, and surveillance regimens that require procedural sedation (PS). Some may have a delay in their treatment plan due to same-day cancelation (SDC) of the procedure due to issues related to sedation or other factors. The objective of this report was to evaluate the factors resulting in the SDC of hematology and oncology patients in an outpatient pediatric sedation service...
January 25, 2019: Pediatric Hematology and Oncology
https://read.qxmd.com/read/30672361/treatment-of-two-cases-on-the-same-day-of-intrathecal-methotrexate-overdose-using-cerebrospinal-fluid-exchange-and-intrathecal-instillation-of-carboxypeptidase-g2
#5
Ole Mikal Wormdal, Trond Flægstad, Tore Stokland
BACKGROUND: Two 14-year old boys with acute lymphocytic leukemia were treated according to the NOPHO-ALL-08 protocol with intrathecal methotrexate (MTX) on the same day. Due to a preparation error in the hospital pharmacy, they were both given 240 mg of MTX instead of the prescribed 12 mg. Treatment (or methods): Both patients developed acute neurotoxicity with confusion, pain and seizures. Intravenous dexamethasone and folinic acid (leucovorin) was given. Exchange of cerebrospinal fluid was performed...
January 23, 2019: Pediatric Hematology and Oncology
https://read.qxmd.com/read/30661422/challenges-in-the-management-of-localized-intracranial-ependymoma-in-children-experience-from-a-referral-oncology-center-in-eastern-india
#6
Anirban Das, Suman Paul Chowdhury, Rimpa Basu Achari, Lateef Zameer, Saugata Sen, Reghu K Sukumaran, Arpita Bhattacharyya
No abstract text is available yet for this article.
January 20, 2019: Pediatric Hematology and Oncology
https://read.qxmd.com/read/30657003/utility-of-18-fluorodeoxyglucose-positron-emission-tomography-in-children-with-relapsed-refractory-leukemia
#7
Zühre Kaya, Ozgür Umit Akdemir, Ozlem Lütfiye Atay, Nalan Akyürek, Faruk Güçlü Pınarlı, İdil Yenicesu, Ülker Koçak
OBJECTIVE: Few data are available on the clinical significance of 18-fluorodeoxyglucose positron emission tomography (FDG-PET/CT) results in patients with leukemia. We investigated the utility of FDG-PET/CT at the time of relapsed/refractory disease in pediatric patients with leukemia. METHODS: Medical records of 28 children with suspected leukemia progression or recurrence during/after chemotherapy or allogeneic stem cell transplantation (allo-SCT) were retrospectively reviewed to determine the utility of FDG-PET/CT...
January 18, 2019: Pediatric Hematology and Oncology
https://read.qxmd.com/read/30636474/healthcare-utilization-and-hydroxyurea-adherence-in-youth-with-sickle-cell-disease
#8
Sherif M Badawy, Alexis A Thompson, Jane L Holl, Frank J Penedo, Robert I Liem
BACKGROUND: Sickle cell disease (SCD) complications lead to poor health-related quality of life (HRQOL) and increased healthcare utilization in this population, which could be mitigated with hydroxyurea therapy; however, adherence is suboptimal. We assessed the relationship of healthcare utilization to hydroxyurea adherence and HRQOL amongst youth with SCD. METHODS: Thirty-four patients with SCD (median age 14 years, IQR 12-18) on hydroxyurea participated in this cross-sectional study and completed Morisky Adherence Scale 8-items and Patient Reported Outcomes Measurement Information System (PROMIS®) HRQOL measures...
January 12, 2019: Pediatric Hematology and Oncology
https://read.qxmd.com/read/30633619/treatment-outcome-of-pediatric-acute-lymphoblastic-leukemia-in-yeungnam-region-multicenter-retrospective-study-of-study-alliance-of-yeungnam-pediatric-hematology-oncology-sayph
#9
Eu Jeen Yang, Kyung Mi Park, Jae Min Lee, Jeong Ok Hah, Sang Kyu Park, Jin Kyung Suh, Ji Yoon Kim, Kun Soo Lee, Jikyoung Park, Eun Sil Park, Jaeyoung Lim, Ye Jee Shim, Heung Sik Kim, Seom Gim Kong, Heewon Chueh, Eun Jin Choi, Jeong A Park, Young Tak Lim
OBJECTIVES: We aimed to evaluate treatment outcomes of pediatric acute lymphoblastic leukemia (ALL) subgroups by risk-stratification, in the Yeungnam region of Korea. METHODS: We reviewed the courses of 409 newly diagnosed ALL patients from January 2004 to December 2013 in the Yeungnam region. RESULTS: All patients were classified into three risk groups: standard risk (SR, n=212), high risk (HR, n=153) and very high risk (VHR, n=44). The mean follow-up time was 73...
January 11, 2019: Pediatric Hematology and Oncology
https://read.qxmd.com/read/30633606/exon-skipping-in-cybb-mrna-and-skewed-inactivation-of-x-chromosome-cause-late-onset-chronic-granulomatous-disease
#10
Mariko Eguchi, Chihiro Yagi, Hisamichi Tauchi, Masao Kobayashi, Eiichi Ishii, Minenori Eguchi-Ishimae
Chronic granulomatous disease (CGD) is a hereditary immunodeficiency syndrome caused by a defect in the NADPH oxidase complex, which is essential for bactericidal function of phagocytes. Approximately 70% of patients with CGD have a mutation in the CYBB gene on the X chromosome, resulting in defective expression of gp91phox , one of the membrane-bound subunits of NADPH oxidase. Although most patients with X-linked CGD are males, owing to transmission of this disease as an X-linked recessive trait, there are female patients with X-linked CGD...
January 11, 2019: Pediatric Hematology and Oncology
https://read.qxmd.com/read/30596314/rituximab-therapy-for-patients-with-langerhans-cell-histiocytosis-associated-neurologic-dysfunction
#11
Olive Eckstein, Casey L McAtee, Jay Greenberg, Ashish Kumar, Carolyn Fein-Levy, Thomas Smith, Brandon Tran, Kenneth L McClain
OBJECTIVE: Since patients with langerhans cell histiocytosis and neurologic dysfunction (LCH-ND) often have incomplete treatment responses we sought a new treatment regimen. Because of clinical benefit from rituximab in multiple sclerosis patients with neurodegeneration, we evaluated its use in patients with LCH-ND. PARTICIPANTS: Eight LCH-ND patients who had failed prior therapies. METHODS: Charts of the 8 patients treated with rituximab were reviewed...
December 31, 2018: Pediatric Hematology and Oncology
https://read.qxmd.com/read/30595064/foundation-of-pediatric-cancer-treatment-in-lao-people-s-democratic-republic-at-the-lao-korea-national-children-s-hospital
#12
Kyung Duk Park, Che Ry Hong, Jung Yoon Choi, Min Sun Kim, Eun Sang Yi, Sonephet Saysouliyo, Khounthavy Phongsavath, Hee Young Shin
AIM: The Lao-Korea National Children's Hospital initiated and developed a pediatric cancer treatment program for the first time in September 2012, through education by the Lee Jong-Wook project, establishment of infrastructure by the Korea International Cooperation Agency, and cooperation of medical staff. MATERIAL AND METHODS: we describe the experience of initiating and building this program by retrospectively reviewing the data from pediatric patients with cancer diagnosed at the Lao-Korea National Children's Hospital between September 2012 and December 2016...
December 31, 2018: Pediatric Hematology and Oncology
https://read.qxmd.com/read/30592246/relationship-between-cyclosporine-area-under-the-curve-and-acute-graft-versus-host-disease-in-pediatric-patients-undergoing-hematopoietic-stem-cell-transplant-a-prospective-multicenter-study
#13
Tal Schechter, Victor A Lewis, Kirk R Schultz, David Mitchell, Shiyi Chen, Winnie Seto, Oliver Teuffel, Paul Gibson, John J Doyle, Adam Gassas, Lillian Sung, L Lee Dupuis
Traditionally in hematopoietic stem cell transplant (HSCT), cyclosporine doses are individualized using cyclosporine trough concentrations (C0 ) while area under the concentration vs time curve (AUC) is used in solid organ transplant. AUC potentially has an important relationship with the development of acute graft-versus-host-disease (aGVHD). We conducted a prospective study to describe the relationship between severe (grade III-IV) aGVHD and cyclosporine AUC in pediatric HSCT recipients. Pediatric patients who underwent allogeneic myeloablative HSCT and scheduled to receive cyclosporine for aGVHD prophylaxis participated in this multicenter study...
December 28, 2018: Pediatric Hematology and Oncology
https://read.qxmd.com/read/30588876/prevalence-of-serum-markers-of-infection-and-naturally-acquired-immunity-to-hepatitis-b-virus-in-transfused-children-treated-at-a-children-s-hospital-in-salvador-bahia-a-cross-sectional-study
#14
Victor Otero Martinez, Stella Oliveira D'Arede, Eronildes Santos de Almeida, Fernanda Washington de Mendonça Lima
The artificially acquired humoral immunity against hepatitis B virus (HBV) apparently may decline over the years. This study aimed to investigate the prevalence of naturally occurring serological markers of infection and active immunity to HBV in severely ill children and adolescents treated at the Martagão Gesteira Pediatric Hospital who received blood transfusions. The serum samples of 353 children who received poly-transfusions between August 2016 and March 2017 were tested for HBV surface antigen (HBsAg) markers, anti-HBc antibodies (IgG and IgM isotypes), and IgG anti-HBs...
December 27, 2018: Pediatric Hematology and Oncology
https://read.qxmd.com/read/30588872/expression-of-antiapoptotic-proteins-livin-and-survivin-in-pediatric-aml-patients-as-prognostic-markers
#15
Soheila Zareifar, Soudeh Ghorbani, Ahmad Monabbati, Mohammad Reza Bordbar, Omid Reza Zekavat, Babak Abdolkarimi, Sezaneh Haghpanah
OBJECTIVE: Survivin and livin are highly expressed in various malignancies and their expression levels may be related to unfavorable prognosis. The aim was to investigate the relationships of these two markers with some prognostic factors and with survival of the children with acute myeloid leukemia (AML). METHODS: Livin and survivin expression was investigated quantitatively by immunohistochemistry staining technique in 43 primary formalin-fixed, paraffin-embedded bone marrow blocks in pediatric age group (<18 years)...
December 27, 2018: Pediatric Hematology and Oncology
https://read.qxmd.com/read/30570427/first-report-that-prior-ecmo-therapy-does-not-preclude-hematopoietic-cell-transplantation
#16
Larisa Broglie, Rachel Phelan, Julie-An Talano
Hematopoietic stem cell transplantation (HCT) offers a potential cure for patients with high-risk malignancies but carries a risk of death from transplant-related complications. Extracorporeal membrane oxygenation (ECMO) is often considered a contraindication to transplant with the assumption that lung injury puts the patient at risk for pulmonary complications post-HCT. Although patients who have required prolonged intubation show gradual improvement in pulmonary function over time, there is little data on pulmonary functional recovery after ECMO which makes assessment pre-HCT difficult...
December 20, 2018: Pediatric Hematology and Oncology
https://read.qxmd.com/read/30537887/hypothyroidism-after-craniospinal-irradiation-with-proton-or-photon-therapy-in-patients-with-medulloblastoma
#17
Kevin Bielamowicz, M Fatih Okcu, Rona Sonabend, Arnold C Paulino, Susan G Hilsenbeck, Zoann Dreyer, Hilary Suzawa, Rosalind Bryant, Adekunle Adesina, Robert Dauser, Anita Mahajan, Murali Chintagumpala
BACKGROUND: Craniospinal irradiation (CSI) often results in endocrine deficiencies in children with medulloblastoma due to irradiation of the hypothalamic-pituitary axis (HPA) or the thyroid gland. CSI with Proton radiation therapy (PRT) has the potential to decrease the risk of hypothyroidism by reduction in radiation dose to these organs. This study compares the risk for hypothyroidism in patients with medulloblastoma treated with Photon radiation therapy (XRT) or PRT. METHODS: The records of patients with medulloblastoma diagnosed at a single institution between 1997 and 2014 who received CSI were, retrospectively, reviewed...
December 11, 2018: Pediatric Hematology and Oncology
https://read.qxmd.com/read/30526220/alectinib-is-effective-safe-and-tolerable-in-an-adolescent-with-stage-ivb-alk-rearranged-adenocarcinoma-of-the-lung
#18
Ben W R Balzer, Christine Loo, Eva A Wegner, Christa E Nath, Samiuela Lee, Chantelle Smith, Craig R Lewis, Toby N Trahair, Antoinette C Anazodo
Anaplastic lymphoma kinase (ALK) inhibitors such as crizotinib and alectinib have been shown to have significant activity in ALK-rearranged non-small cell lung cancers (NSCLC). There are no data for alectinib's safety or efficacy in younger patients, though it is superior to crizotinib in adult trials. We present a 14-year old girl diagnosed with stage IV-B ALK-positive adenocarcinoma of the lung after presenting with cough and fever. She was commenced on alectinib at adult dose and has had sustained complete metabolic remission for 9 months...
December 9, 2018: Pediatric Hematology and Oncology
https://read.qxmd.com/read/30488741/experience-with-generic-pegylated-l-asparaginase-in-children-with-acute-lymphoblastic-leukemia-and-monitoring-of-serum-asparaginase-activity
#19
Chintan Vyas, Sandeep Jain, Gauri Kapoor, Anurag Mehta, Parul Takkar Chugh
BACKGROUND: Pegylated asparaginase (P-Asp) though integral to acute lymphoblastic leukemia (ALL) therapy is often not accessible to patients in developing countries. We share our clinical experience with generic P-Asp along with monitoring of asparaginase activity. METHODS: In this prospective observational study, patients ≤18 years of age with ALL were assigned to receive either generic P-Asp or native asparaginase (N-Asp) in a non-randomized manner. Treatment protocol was based on ALL BFM-95 backbone...
November 29, 2018: Pediatric Hematology and Oncology
https://read.qxmd.com/read/30468406/anti-tnf-%C3%AE-therapy-in-patients-with-relapsed-and-refractory-langerhans-cell-histiocytosis-a-phase-ii-study
#20
Alejandra Flores Legarreta, Olive Eckstein, Thomas M Burke, Kenneth L McClain
Tumor necrosis factor alpha (TNF-α) is produced in Langerhans cell histiocytosis (LCH) lesions and is elevated in plasma of patients with active LCH. It has been postulated that TNF-α may play a role in the pathophysiology of LCH. Etanercept, an anti-TNF-α antibody, has been used in TNF-modulated diseases such as rheumatoid arthritis (RA). We conducted a phase II study to determine the efficacy of etanercept for patients with refractory or relapsed LCH. Five LCH patients who had failed at least 2 prior treatments (range 2-9) received etanercept at a dose of 0...
November 23, 2018: Pediatric Hematology and Oncology
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