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pediatrics, pediatric hematology

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https://www.readbyqxmd.com/read/28816916/developmental-screening-in-pediatric-sickle-cell-disease-disease-related-risk-and-screening-outcomes-in-4-year-olds
#1
Jeffrey Schatz, Alyssa Schlenz, Laura Reinman, Kelsey Smith, Carla W Roberts
OBJECTIVE: Studies of early child development in sickle cell disease (SCD) have found modest associations between disease-related risks and developmental status in infants and toddlers, but such associations are evident by early elementary school. We screened 4-year-old children with SCD using 2 screening strategies to assess if biomedical risk factors for neurologic disease are related to developmental screening outcomes at this intermediate age. METHODS: Seventy-seven 4-year-old children with SCD (M = 4...
August 4, 2017: Journal of Developmental and Behavioral Pediatrics: JDBP
https://www.readbyqxmd.com/read/28816795/voriconazole-antifungal-prophylaxis-in-children-with-malignancies-a-nationwide-study
#2
Zoi Dorothea Pana, Maria Kourti, Katerina Vikelouda, Antonia Vlahou, Nikolaos Katzilakis, Maria Papageorgiou, Dimitrios Doganis, Loizos Petrikkos, Anna Paisiou, Dimitrios Koliouskas, Antonios Kattamis, Eftichia Stiakaki, Maria Chatzistilianou, Helen Vasilatou-Kosmidis, Sophia Polychronopoulou, Stelios Grafakos, Emmanuel Roilides
BACKGROUND: Antifungal prophylaxis (AFP) is recommended in at-risk hematology-oncology patients. We evaluated the safety of AFP with voriconazole (VRC) in pediatric hematology/oncology patients. MATERIALS AND METHODS: A retrospective study of VRC AFP in children with malignancies hospitalized in all 7 Greek pediatric hematology/oncology centers during 2008 to 2012 was conducted. Patients' demographics, outcome, and adverse event (AE) data were recorded. RESULTS: Four hundred twenty-nine VRC AFP courses in 249 patients (median age 6 y, 55% boys) were studied...
August 14, 2017: Journal of Pediatric Hematology/oncology
https://www.readbyqxmd.com/read/28810906/atypical-presentation-of-anti-n-methyl-d-aspartate-receptor-encephalitis-two-case-reports
#3
Maria Cristina Maggio, Greta Mastrangelo, Aldo Skabar, Alessandro Ventura, Marco Carrozzi, Giuseppe Santangelo, Francesca Vanadia, Giovanni Corsello, Rolando Cimaz
BACKGROUND: Anti-N-methyl-D-aspartate receptor encephalitis is a rare autoimmune disease characterized by severe neurological and psychiatric symptoms and a difficult diagnosis. The disease is often secondary to a neoplastic lesion, seldom diagnosed years later. Psychiatric symptoms are prevalent in adults; neurologic symptoms are more evident in children, who typically present primarily with neurological symptoms. To the best of our knowledge, the association with juvenile idiopathic arthritis has not been described...
August 16, 2017: Journal of Medical Case Reports
https://www.readbyqxmd.com/read/28810325/-heterogeneity-and-clonal-evolution-in-pediatric-etv6-runx1-acute-lymphoblastic-leukemia-by-quantitative-multigene-fluorescence-in-situ-hybridization
#4
L Zhang, L P Hu, X M Liu, Y Guo, W Y Yang, J Y Zhang, F Liu, T F Liu, S C Wang, X J Chen, M Ruan, B Q Qi, L X Chang, Y M Chen, Y Zou, X F Zhu
Objective: To evaluate heterogeneity and clonal evolution in pediatric ETV6-RUNX1(+) acute lymphoblastic leukemia (ALL) in China. Methods: Totally 48 children (<14 years) with newly diagnosed ETV6-RUNX1(+) ALL in Institute of Hematology and Blood Disease Hospital, CAMS and PUMC, from February 2006 to June 2011 were included. The copy number variations were analyzed by quantitative multigene fluorescence in situ hybridization (QM-FISH) in 48 patients. Non-normal distribution of measurement data were shown with Median (range) , count data were shown with percent (%) ...
July 14, 2017: Zhonghua Xue Ye Xue za Zhi, Zhonghua Xueyexue Zazhi
https://www.readbyqxmd.com/read/28809761/the-t-12-21-p13-q22-in-pediatric-b-acute-lymphoblastic-leukemia-an-update
#5
Maximilian Becker, Kristie Liu, Carlos A Tirado
Pediatric B-cell acute lymphoblastic leukemia (B-ALL) is the most common hematological malignancy in children, and the t(12;21)(p13;q22) occurs in approximately 25% of these cases, making it is the most prevalent chromosomal abnormality. The t(12;21) which disrupts hematopoietic differentiation and proliferation, and can be present as a sole abnormality or within the context of a complex karyotype characterized by three or more chromosomal abnormalities. The prognosis of t(12;21) within a complex karyotype is extensively debated...
2017: Journal of the Association of Genetic Technologists
https://www.readbyqxmd.com/read/28807197/perioperative-blood-transfusion-and-complications-in-children-undergoing-surgery-for-solid-tumors
#6
Dani O Gonzalez, Jennifer N Cooper, Erica Mantell, Peter C Minneci, Katherine J Deans, Jennifer H Aldrink
BACKGROUND: The objective was to assess whether perioperative blood transfusion (PBT) is associated with postoperative complications in children undergoing surgery for a solid tumor. METHODS: Using 2012-2014 National Surgical Quality Improvement Program Pediatric data, we identified patients aged 0-18 years who underwent surgery (biopsy or resection) for solid tumors. We compared demographic, clinical, and 30-day outcome characteristics between children who did and did not receive a PBT within 72 hours after surgery...
August 2017: Journal of Surgical Research
https://www.readbyqxmd.com/read/28803261/advantages-in-prognosis-of-adult-patients-with-ewing-sarcoma-11-years-experiences-and-current-treatment-management
#7
Dagmar Adamkova Krakorova, Katerina Kubackova, Ladislav Dusek, Tomas Tomas, Pavel Janicek, Stepan Tucek, Jana Prausova, Igor Kiss, Iva Zambo
Ewing sarcoma (ES) is an exceptionally rare tumor in adults. Data regarding outcomes of adult patients with ES and experiences with age-adapted therapeutic strategies are very limited. The aim of this study was to evaluate prognostic factors and clinical outcome in a cohort of adult patients treated according to pediatric protocols in the Czech Republic. The records of 58 adult ES patients diagnosed between 2002 and 2013 were reviewed and factors relevant to prognosis and survival were analyzed. The median age of study cohort was 29 years (range, 18-59)...
August 12, 2017: Pathology Oncology Research: POR
https://www.readbyqxmd.com/read/28795915/where-teachers-are-few-documenting-available-faculty-in-five-tanzanian-medical-schools
#8
Charles A Mkony, Ephata E Kaaya, Alex J Goodell, Sarah B Macfarlane
Background Faced with one of the lowest physician-to-population ratios in the world, the Government of Tanzania is urging its medical schools to train more physicians. The annual number of medical students admitted across the country rose from 55 in the 1990s to 1,680 approved places for the 2015/16 academic year. These escalating numbers strain existing faculty. Objective To describe the availability of faculty in medical schools in Tanzania. Design We identified faculty lists published on the Internet by five Tanzanian medical schools for the 2011/12 academic year and analyzed the appointment status, rank, discipline, and qualifications of faculty members...
December 2016: Global Health Action
https://www.readbyqxmd.com/read/28783617/an-age-dependent-response-to-hydroxyurea-in-pediatric-sickle-cell-anemia-patients-with-alpha-thalassemia-trait
#9
Lisa Figueiredo, Kerry Morrone, Catherine Wei, Karen Ireland, Hillel W Cohen, Catherine Driscoll, Deepa Manwani
Hydroxyurea (HU) is a key drug therapy for individuals with sickle cell anemia (SCA), yet its clinical and hematologic responses can be variable. Various studies have reported the role of α-thalassemia as one of the most prevalent heritable traits that may modify HU response. We provide data from 62 pediatric and adolescent patients with SCA, 26 with co-inherited α-thalassemia trait. Our data suggest that altered hematologic and clinical responses to HU therapy are noted in adolescent SCA individuals with co-inherited α-thalassemia trait...
July 31, 2017: Blood Cells, Molecules & Diseases
https://www.readbyqxmd.com/read/28781151/2016-guideline-strategies-for-the-use-of%C3%A2-antifungal-agents-in-patients-with-hematological-malignancies-or-hematopoietic-stem-cell-transplantation-recipients-in-taiwan
#10
REVIEW
Bor-Sheng Ko, Wei-Ting Chen, Hsiang-Chi Kung, Un-In Wu, Jih-Luh Tang, Ming Yao, Yee-Chun Chen, Hwei-Fang Tien, Shan-Chwen Chang, Yin-Ching Chuang, Dong-Tsamn Lin
The Infectious Diseases Society of Taiwan (IDST), the Hematology Society of Taiwan, the Taiwan Society of Blood and Marrow Transplantation, Medical Foundation in Memory of Dr. Deh-Lin Cheng, Foundation of Professor Wei-Chuan Hsieh for Infectious Diseases Research and Education, and CY Lee's Research Foundation for Pediatric Infectious Diseases and Vaccines cooperatively published this guideline for the use of antifungal agents in hematological patients with invasive fungal diseases (IFDs) in Taiwan. The guideline is the first one endorsed by IDST focusing on selection of antifungal strategies, including prophylaxis, empirical (or symptom-driven) and pre-emptive (or diagnostic-driven) strategy...
July 25, 2017: Journal of Microbiology, Immunology, and Infection, Wei Mian Yu Gan Ran za Zhi
https://www.readbyqxmd.com/read/28779880/investigation-of-a-nosocomial-outbreak-of%C3%A2-fungemia-caused-by-candida-pelliculosa-pichia-anomala-in-a-korean-tertiary-care-center
#11
Jiwon Jung, Young Sook Moon, Jung A Yoo, Ji-Hun Lim, Joseph Jeong, Jae-Bum Jun
BACKGROUND: Candida pelliculosa is a rare pathogen of fungemia. There have been a few nosocomial outbreaks of C. pelliculosa fungemia in nurseries and pediatric intensive care units (ICU), hematologic units, and surgical ICU. We describe an epidemiologic outbreak investigation, including case findings of C. pelliculosa fungemia in South Korea. METHODS: This outbreak investigation conducted in a 940-bed, tertiary referral center, Ulsan, South Korea and included active microbial surveillance and a case-control study...
June 28, 2017: Journal of Microbiology, Immunology, and Infection, Wei Mian Yu Gan Ran za Zhi
https://www.readbyqxmd.com/read/28771903/fludarabine-cytarabine-g-csf-and-idarubicin-for-children-with-relapsed-aml
#12
Hideki Nakayama, Daisuke Tomizawa, Shiro Tanaka, Shotaro Iwamoto, Akira Shimada, Akiko M Saito, Yuka Yamashita, Hiroshi Moritake, Kiminori Terui, Takashi Taga, Hidemasa Matsuo, Yoshiyuki Kosaka, Katsuyoshi Koh, Hajime Hosoi, Hidemitsu Kurosawa, Keiichi Isoyama, Keizo Horibe, Shuki Mizutani, Souichi Adachi
BACKGROUND: The combination of fludarabine (Flu), high dose cytarabine (Ara-C) and granulocyte-colony stimulating factor (G-CSF) called "FLAG" with anthracyclines has become a standard chemotherapy for refractory acute myeloid leukemia (AML) in European children and adults. To clarify the efficacy and the safety of FLAG-idarubicin (IDA) for children prospectively, we planned a multicenter phase II study (AML-R11) by Japanese Pediatric Leukemia/Lymphoma Study Group. METHODS: Patients with AML at the age between 2 and 20 years old, who had the first bone marrow (BM) relapse or induction failure, were enrolled...
August 3, 2017: Pediatrics International: Official Journal of the Japan Pediatric Society
https://www.readbyqxmd.com/read/28770610/amino-acid-profiles-as-potential-biomarkers-for-pediatric-cancers-a-preliminary-communication
#13
Anna Synakiewicz, Malgorzata Sawicka-Zukowska, Natalia Adrianowska, Grazyna Galezowska, Joanna Ratajczyk, Anna Owczarzak, Lucyna Konieczna, Teresa Stachowicz-Stencel
AIM: Childhood cancer remains one of the main cause of death in the pediatric population. Amino acids (AAs) level alterations in plasma are considered to play a role in carcinogenesis and further course of the disease. METHODS: Seventy-seven children with cancer, including 47 with hematological and 30 with solid tumors were enrolled in this study and compared with healthy children. Twenty-two plasma-free AAs were determined by HPLC with fluorometric detection. RESULTS: The results revealed significant decrease in glutamine levels for oncological patients and significant increase in aspartic acid, glutamic acid, asparagine, serine, citrulline, alanine, GABA, tryptophan, methionine, valine, phenylalanine and isoleucine levels in cancer children versus control...
August 3, 2017: Biomarkers in Medicine
https://www.readbyqxmd.com/read/28765255/malignancy-in-pediatric-onset-systemic-lupus-erythematosus
#14
Sasha Bernatsky, Ann E Clarke, Omid Zahedi Niaki, Jeremy Labrecque, Laura E Schanberg, Earl D Silverman, Kristen Hayward, Lisa Imundo, Hermine I Brunner, Kathleen A Haines, Randy Q Cron, Kiem Oen, Linda Wagner-Weiner, Alan M Rosenberg, Kathleen M O'Neil, Ciarán M Duffy, Emily von Scheven, Lawrence Joseph, Jennifer L Lee, Rosalind Ramsey-Goldman
OBJECTIVE: To determine cancer incidence in a large pediatric-onset systemic lupus erythematosus (SLE) population. METHODS: Data were examined from 12 pediatric SLE registries in North America. Patients were linked to their regional cancer registries to detect cancers observed after cohort entry, defined as date first seen in the clinic. The expected number of malignancies was obtained by multiplying the person-years in the cohort (defined from cohort entry to end of followup) by the geographically matched age-, sex-, and calendar year-specific cancer rates...
August 1, 2017: Journal of Rheumatology
https://www.readbyqxmd.com/read/28762079/elevated-serum-ferritin-is-not-specific-for-hemophagocytic-lymphohistiocytosis
#15
Zaher K Otrock, Karl G Hock, Sarah B Riley, Theo de Witte, Charles S Eby, Mitchell G Scott
Hemophagocytic lymphohistiocytosis (HLH) is a rare, potentially fatal, syndrome of excessive and ineffective activation of the immune system. The majority of the reported data on HLH is from pediatric patients and lacks specificity. This makes HLH diagnosis challenging especially in adults where HLH is triggered by many conditions and can resemble many disease entities. Elevated ferritin is one of the diagnostic criteria for HLH. We determined the conditions associated with elevated ferritin at our medical center to assess how specific ferritin is for predicting HLH...
July 31, 2017: Annals of Hematology
https://www.readbyqxmd.com/read/28748759/targeting-kinase-activating-genetic-lesions-to-improve-therapy-of-pediatric-acute-lymphoblastic-leukemia
#16
Franca Raffaella, Natasa Karas Kuzelicki, Claudio Sorio, Eleonora Toffoletti, Oksana Montecchini, Alice Poropat, Marco Rabusin, Debora Curci, Dino Paladin, Gabriele Stocco, Giuliana Decorti
Acute lymphoblastic leukemia (ALL) is the most common hematologic malignancy in children, characterized by an abnormal proliferation of immature lymphoid cells. Thanks to risk-adapted combination chemotherapy treatments currently used, survival at 5 years has reached 90%. ALL is a heterogeneous disease from a genetic point of view: patients' lymphoblasts may harbor in fact several chromosomal alterations, some of which have prognostic and therapeutic value. Of particular importance is the translocation t(9;22)(q34;q11...
July 27, 2017: Current Medicinal Chemistry
https://www.readbyqxmd.com/read/28748605/management-of-chemotherapy-induced-febrile-neutropenia-in-pediatric-oncology-patients-a-north-american-survey-of-pediatric-hematology-oncology-and-pediatric-infectious-disease-physicians
#17
Rochelle R Maxwell, Dana Egan-Sherry, Jonathan B Gill, Michael E Roth
BACKGROUND: Chemotherapy-induced febrile neutropenia (FN) is traditionally managed with hospital admission for parenteral antibiotics until neutropenia resolves. Recent studies have explored risk stratification and the safety of managing "low-risk" patients as outpatients. Few studies have directly assessed pediatric provider preferences for managing FN. PROCEDURE: We conducted a survey of practicing US and Canadian pediatric hematology/oncology (PHO) and pediatric infectious disease (PID) physicians to assess their FN management preferences using case scenarios with varying risk profiles...
July 27, 2017: Pediatric Blood & Cancer
https://www.readbyqxmd.com/read/28748541/reliable-assessment-of-the-incidence-of-childhood-autoimmune-hemolytic-anemia
#18
Nathalie Aladjidi, Marthe-Aline Jutand, Cyrielle Beaubois, Helder Fernandes, Julien Jeanpetit, Gaelle Coureau, Véronique Gilleron, Aude Kostrzewa, Pierre Lauroua, Michel Jeanne, Rodolphe Thiébaut, Thierry Leblanc, Guy Leverger, Yves Perel
BACKGROUND: Childhood autoimmune hemolytic anemia (AIHA) is a rare and severe disease characterized by hemolysis and positive direct antiglobulin test (DAT). Few epidemiologic indicators are available for the pediatric population. The objective of our study was to reliably estimate the number of AIHA cases in the French Aquitaine region and the incidence of AIHA in patients under 18 years old. PROCEDURE: In this retrospective study, the capture-recapture method and log-linear model were used for the period 2000-2008 in the Aquitaine region from the following three data sources for the diagnosis of AIHA: the OBS'CEREVANCE database cohort, positive DAT collected from the regional blood bank database, and the French medico-economic information system...
July 27, 2017: Pediatric Blood & Cancer
https://www.readbyqxmd.com/read/28748347/molecular-basis-of-pediatric-brain-tumors
#19
REVIEW
Alexia Klonou, Christina Piperi, Antonios N Gargalionis, Athanasios G Papavassiliou
Brain tumors emerge as the second commonest type of pediatric solid tumors following hematologic malignancies. Genomic profiling of low- and high-grade gliomas, ependymomas and medulloblastomas has revealed chromosomal abnormalities and specific gene mutations which have been associated with aberrant activation of crucial signal transduction pathways, including mitogen-activated protein kinase, mammalian target of rapamycin and retinoblastoma tumor suppressor signaling. Furthermore, pediatric high-grade gliomas are associated with chromatin remodeling defects and somatic histone gene mutations that affect prognosis...
July 26, 2017: Neuromolecular Medicine
https://www.readbyqxmd.com/read/28745855/managing-acute-complications-of-sickle-cell-disease-in-pediatric-patients-digest
#20
Sathyaseelan Subramaniam, Jennifer H Chao, Pradip Chaudhari
Sickle cell disease is a chronic hematologic disease with a variety of acute, and often recurring, complications. Vaso-occlusive crisis, a unique but common presentation in sickle cell disease, can be challenging to manage. Acute chest syndrome is the leading cause of death in patients with sickle cell disease, occurring in more than half of patients who are hospitalized with a vaso-occlusive crisis. Uncommon diagnoses in children, such as stroke, priapism, and transient red cell aplasia, occur more frequently in patients with sickle cell disease and necessitate a degree of familiarity with the disease process and its management...
November 22, 2016: Pediatric Emergency Medicine Practice
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