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Pediatric hematology

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https://www.readbyqxmd.com/read/28748759/targeting-kinase-activating-genetic-lesions-to-improve-therapy-of-pediatric-acute-lymphoblastic-leukemia
#1
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
#2
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
#3
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
#4
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
#5
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
https://www.readbyqxmd.com/read/28737569/elevated-hemoglobin-level-is-associated-with-advanced-fibrosis-in-pediatric-nonalcoholic-fatty-liver-disease
#6
Valentina Giorgio, Antonella Mosca, Arianna Alterio, Anna Alisi, Antonio Grieco, Valerio Nobili, Luca Miele
OBJECTIVES: Hemoglobin (Hb) and red blood cell distribution width (RDW) have been reported to be a risk marker of metabolic syndrome and nonalcoholic fatty liver disease (NAFLD). No study exists on pediatric populations. We aimed to determine the association between hematological parameters, and the severity of disease in children with biopsy-proven NAFLD. METHODS: A total of 117 children (85 boys, mean age 12 years) with ultrasound evidence of NAFLD undergoing liver biopsy for diagnosis of nonalcoholic steatohepatitis (NASH), were prospectively enrolled between January 2011 and May 2013 in the setting of a tertiary care center...
August 2017: Journal of Pediatric Gastroenterology and Nutrition
https://www.readbyqxmd.com/read/28734656/patterns-of-emergency-department-care-for-newly-diagnosed-immune-thrombocytopenia-in-united-states-children-s-hospitals
#7
LaQuita Jones, Terah Koch, Joseph Stanek, Sarah H O'Brien
We used the Pediatric Health Information Systems database to ascertain treatment patterns of immune thrombocytopenia across the US. Despite the recently published guidelines by the American Society of Hematology, most patients are still being hospitalized for immune thrombocytopenia, even in the absence of documented bleeding symptoms.
July 19, 2017: Journal of Pediatrics
https://www.readbyqxmd.com/read/28732737/microrna-181a-and-its-target-smad-7-as-potential-biomarkers-for-tracking-child-acute-lymphoblastic-leukemia
#8
Marwa Nabhan, Manal L Louka, Eman Khairy, Fathy Tash, Randa Ali-Labib, Safinaz El-Habashy
Acute lymphoblastic leukemia (ALL) is the most common pediatric hematologic tumor. MiR-181a was expected to have a role in the development of hematological malignancies; it might act as tumor suppressor or oncogene. Smad7 was selected as miR-181a target pair. It is a negative regulator for the TGF-β1 signaling pathway. In this study, relative expression levels of miR-181a by quantitative reverse transcriptase polymerase chain reaction (qRT-PCR), both Smad 7 and TGF β1 proteins levels by enzyme linked immunosorbent assay (ELISA) were all measured in serum of 60 child, 30 with ALL and 30 age and sex matched healthy child as control group...
July 18, 2017: Gene
https://www.readbyqxmd.com/read/28728262/-evaluation-of-efficacy-of-immunosuppressive-therapy-plus-recombinant-human-thrombopoietin-for-children-with-severe-aplastic-anemia
#9
K Zhou, C X Liu, Y Li, J P Li, H H Fan, L Zhang, L P Jing, G X Peng, L Ye, Y Li, L Song, X Zhao, W R Yang, Z J Wu, F Chen, F K Zhang
Objective: To evaluate the therapeutic efficacy and safety of immunosuppressive therapy (IST) combined with recombinant human thrombopoietin (rhTPO) for severe aplastic anemia (SAA) in pediatric patients. Method: A retrospective case-control study was conducted and the clinical data of 45 pediatric patients with de novo SAA admitted to the Anemia Diagnosis and Treatment Center of Chinese Academy of Medical Sciences & Blood Disease Hospital during the period from December 2009 to December 2014 were analyzed...
July 2, 2017: Zhonghua Er Ke za Zhi. Chinese Journal of Pediatrics
https://www.readbyqxmd.com/read/28724202/population-pharmacokinetics-of-lenalidomide-in-healthy-volunteers-and-patients-with-hematologic-malignancies
#10
Jamie N Connarn, Renfang Hwang, Yue Gao, Maria Palmisano, Nianhang Chen
A population pharmacokinetic (PopPK) model of lenalidomide was developed using data pooled from 13 clinical studies (dose range, 5-400 mg) in participants who were considered to have adequate capability for renal excretion of lenalidomide (creatinine clearance [CrCl] > 50 mL/min). The analysis population included 305 healthy volunteers and 83 patients with multiple myeloma or myelodysplastic syndromes. A 1-compartment model with linear absorption and elimination described well the observed data for both healthy volunteers and patients...
July 19, 2017: Clinical Pharmacology in Drug Development
https://www.readbyqxmd.com/read/28719110/benefits-of-attending-a-weekend-childhood-cancer-survivor-family-retreat
#11
Lisa Bashore, Joyce Bender
PURPOSE: To explore the long-term benefits to families of childhood cancer survivors who attended a weekend childhood cancer survivor family retreat. DESIGN: Descriptive-qualitative study including families who had attended the weekend retreat at least once but not in the past 12 months, and who attend a large pediatric hematology and oncology cancer survivorship program in Texas. METHODS: A semistructured interview guide was used during three audio-taped focus groups to explore the benefits of having attended a weekend retreat...
July 18, 2017: Journal of Nursing Scholarship
https://www.readbyqxmd.com/read/28705019/a-rare-case-of-pediatric-primary-cardiac-tumor-in-a-patient-with-down-syndrome
#12
Kimiaki Okada, Ayumu Masuoka, Kentaro Hotoda, Yoshimasa Uno, Takaaki Suzuki
Although hematological malignancies are a known complication of Down syndrome, few reports have described cases involving solid tumors. We describe the case of a 3-year-old Down syndrome girl with a primary solid cardiac tumor. Outpatient echocardiography after intracardiac repair of a ventricular septal defect at 6 months of age revealed a highly mobile pedunculated mass (8 × 9 mm) on the free wall of the right atrium. Due to potential incarceration of the mass in the tricuspid orifice, it was excised under extracorporeal circulation and cardiac arrest...
January 1, 2017: Asian Cardiovascular & Thoracic Annals
https://www.readbyqxmd.com/read/28704210/factor-xiii-deficiency-in-south-of-tunisia
#13
Ines Maaloul, Moez Medhaffer, Nacim Louhichi, Imen Krichen, Sofien Alibi, Sana Kmiha, Hajer Aloulou, Faiza Fakhfakh, Moez Elloumi, Choumous Kallel, Mongia Hachicha
: Factor XIII deficiency is a rare autosomal recessive disorder of hemostasis characterized by a plasmatic factor XIII level less than 1% in homozygote and bleeding as of the youth. The aim of the study is to describe the clinical features and the outcome of the patients and to determine molecular characteristics. A retrospective study, was conducted on seven patients with factor XIII deficiency in the department of hematology and pediatrics, Hedi Chaker Hospital, Sfax, Tunisia during the period of 14 years (2001-2014)...
July 12, 2017: Blood Coagulation & Fibrinolysis: An International Journal in Haemostasis and Thrombosis
https://www.readbyqxmd.com/read/28697168/pretreatment-neutrophil-to-lymphocyte-ratio-and-lymphocyte-recovery-independent-prognostic-factors-for-survival-in-pediatric-sarcomas
#14
Liliana Vasquez, Esmeralda León, Brady Beltran, Ivan Maza, Monica Oscanoa, Jenny Geronimo
BACKGROUND: Pretreatment neutrophil-to-lymphocyte ratio (NLR) and absolute lymphocyte count (ALC) recovery have been shown to be associated with prognosis in several types of cancer in adults. However, evidence in pediatric cancer is scarce. The aim of our study was to evaluate whether pretreatment NLR and lymphocyte recovery are prognostic factors in pediatric sarcomas. MATERIALS AND METHODS: Study participants were identified from a retrospective cohort of 100 children with osteosarcoma (n=55), rhabdomyosarcoma (n=22), and Ewing sarcoma (n=23)...
July 10, 2017: Journal of Pediatric Hematology/oncology
https://www.readbyqxmd.com/read/28696419/associations-between-the-c677t-and-a1298c-polymorphisms-of-mthfr-and-the-toxicity-of-methotrexate-in-childhood-malignancies-a-meta-analysis
#15
C Zhu, Y W Liu, S Z Wang, X L Li, X L Nie, X T Yu, L B Zhao, X L Wang
As a common chemotherapy drug, methotrexate (MTX) has achieved remarkable clinical success. However, high inter-individual variability and unpredictable toxicity continue to challenge its use in clinical practices. Some studies suggest this variation is associated with a methylenetetrahydrofolate reductase (MTHFR) gene polymorphism, but results remain unclear. In this meta-analysis, we include 14 studies that focus on MTHFR C677T and A1298C polymorphisms in pediatric patients with malignancy. We found significant associations of the MTHFR C677T polymorphism with hepatotoxicity (grade ⩾2; CC vs CT/TT: risk ratio (RR): 0...
July 11, 2017: Pharmacogenomics Journal
https://www.readbyqxmd.com/read/28690028/meropenem-versus-piperacillin-tazobactam-with-or-without-immunoglobulin-as-second-line-therapy-for-febrile-neutropenia%C3%A2-in-pediatric-patients
#16
Ryoji Kobayashi, Daiki Hori, Hirozumi Sano, Daisuke Suzuki, Kenji Kishimoto, Kunihiko Kobayashi
BACKGROUND: Although survival of children with hematological diseases and cancer has increased dramatically, life-threatening complications due to bacterial infections occur in 5-10% of febrile episodes in pediatric cancer patients. A prospective randomized study was performed to clarify the usefulness of meropenem (MEPM) and piperacillin/tazobactam (PIPC/TAZ) with or without intravenous immunoglobulin (IVIG) as second-line therapy for pediatric patients with febrile neutropenia (FN)...
June 29, 2017: Journal of Microbiology, Immunology, and Infection, Wei Mian Yu Gan Ran za Zhi
https://www.readbyqxmd.com/read/28689878/systematic-review-of-antimicrobial-lock-therapy-for-prevention-of-central-line-associated-bloodstream-infections-in-adult-and-pediatric-cancer-patients
#17
REVIEW
LeAnn B Norris, Farah Kablaoui, Maggie K Brilhart, P Brandon Bookstaver
BACKGROUND: Central venous catheter (CVC) use is commonplace in cancer patients. Antimicrobial lock therapy (ALT), the instillation of a concentrated antimicrobial solution into the catheter lumen, is one method for preventing infection among CVCs. This systematic review discusses the effectiveness and safety of prophylactic ALT in cancer patients with CVCs. METHODS: A literature search was performed using the Medline database and Google Scholar from inception until April 2016...
July 6, 2017: International Journal of Antimicrobial Agents
https://www.readbyqxmd.com/read/28688551/pediatric-invasive-fungal-rhinosinusitis-an-investigation-of-17-patients
#18
Daniel Vinh, Michael Yim, Ankhi Dutta, John K Jones, Wei Zhang, Matthew Sitton
PURPOSE: To investigate outcomes of pediatric patients at a single institution with invasive fungal rhinosinusitis (IFRS) and to determine variables that impact overall survival. METHODS: All pediatric patients at a large tertiary children's hospital diagnosed with IFRS confirmed by surgical pathology from 2009 to 2015 were retrospectively reviewed. Demographics, underlying diseases, symptoms, antifungal therapy, absolute neutrophil count (ANC), surgical management,and outcomes were analyzed...
August 2017: International Journal of Pediatric Otorhinolaryngology
https://www.readbyqxmd.com/read/28679999/bone-marrow-failure-in-childhood-central-pathology-review-of-a-nationwide-registry
#19
Masafumi Ito
Refractory cytopenia of childhood (RCC) was proposed as a provisional entity in the 2008 WHO classification of myelodysplastic syndromes (MDS). It is defined as a childhood MDS featuring persistent cytopenia without increase blasts in bone marrow (BM) or peripheral blood (PB). Because the majority of RCC cases feature hypocellularity and pancytopenia, it is quite challenging to differentiate RCC from acquired aplastic anemia (AA) and many kinds of inherited bone marrow failure syndromes (IBMFS). Diagnosis of RCC requires BM histology of characteristic features such as isolated erythroid islet with left shift, abnormal localization and micromegakaryocytes...
2017: [Rinshō Ketsueki] the Japanese Journal of Clinical Hematology
https://www.readbyqxmd.com/read/28677113/plasma-chitotriosidase-and-carotid-intima-media-thickness-in-children-with-sickle-cell-disease
#20
Normeen A Kaddah, Dalia A Saied, Hanan A Alwakeel, Rania H Hashem, Sara M Rowizak, Mohamed A Elmonem
The relationship between chronic hemolysis with subsequent iron overload, inflammation, and premature atherosclerosis has been documented in hemolytic anemias, particularly β-thalassemia. However, no such relationship has been established in sickle cell disease (SCD). We sought to evaluate SCD as a risk factor for early vascular insult by measuring carotid intima-media thickness (CIMT) and plasma chitotriosidase and to assess the role of the latter as a potential quantitative indicator of vascular inflammation and atherogenesis...
July 4, 2017: International Journal of Hematology
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