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pediatrics, pediatric hematology

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https://www.readbyqxmd.com/read/28211293/hfe-gene-mutation-and-iron-overload-in-egyptian-pediatric-acute-lymphoblastic-leukemia-survivors-a-single-center-study
#1
Farida H El-Rashedi, Mahmoud A El-Hawy, Sally M El-Hefnawy, Mona M Mohammed
BACKGROUND: Hereditary hemochromatosis gene (HFE) mutations have a role in iron overload in pediatric acute lymphoblastic leukemia (ALL) survivors. We aimed to evaluate the genotype frequency and allelic distribution of the two HFE gene mutations (C282Y and H63D) in a sample of Egyptian pediatric ALL survivors and to detect the impact of these two mutations on their iron profile. PATIENTS AND METHODS: This study was performed on 35 ALL survivors during their follow-up visits to the Hematology and Oncology Unit, Pediatric Department, Menoufia University Hospitals...
February 17, 2017: Hematology (Amsterdam, Netherlands)
https://www.readbyqxmd.com/read/28205066/a-single-center-analysis-of-chronic-graft-versus-host-disease-free-relapse-free-survival-after-alternative-donor-stem-cell-transplantation-in-children-with-hematological-malignancies
#2
Jiro Inagaki, Reiji Fukano, Maiko Noguchi, Jun Okamura
We assessed the clinical outcomes of allogeneic hematopoietic stem cell transplantation (SCT) from alternative donors for pediatric patients with hematological malignancies, defining graft-versus-host disease (GVHD)-free, relapse-free survival (GRFS) as a composite endpoint. We also defined chronic GVHD-free, relapse-free survival (cGRFS) as survival without severe chronic GVHD, relapse, or death. The probabilities of 2-year disease-free survival from a human leukocyte antigen (HLA) matched unrelated donor (n = 57), related donor with HLA-1 antigen mismatch in the graft-versus-host direction (1Ag-GvH-MMRD, n = 28), and unrelated umbilical cord blood (n = 35) were 52...
February 15, 2017: International Journal of Hematology
https://www.readbyqxmd.com/read/28191766/concise-review-mesenchymal-stem-cell-therapy-for-pediatric-disease-perspectives-on-success-and-potential-improvements
#3
Christopher R Nitkin, Tracey L Bonfield
Mesenchymal stem cells (MSCs) represent a potentially revolutionary therapy for a wide variety of pediatric diseases, but the optimal cell-based therapeutics for such diversity have not yet been specified. The published clinical trials for pediatric pulmonary, cardiac, orthopedic, endocrine, neurologic, and hematologic diseases provide evidence that MSCs are indeed efficacious, but the significant heterogeneity in therapeutic approaches between studies raises new questions. The purpose of this review is to stimulate new preclinical and clinical trials to investigate these factors...
February 2017: Stem Cells Translational Medicine
https://www.readbyqxmd.com/read/28188006/changes-in-hemostasis-caused-by-different-replacement-fluids-and-outcome-in-therapeutic-plasma-exchange-in-pediatric-patients-in-a-retrospective-single-center-study
#4
REVIEW
Volker Witt, Herbert Pichler, Elisabeth Beiglboeck, Friedrich Kursten, Lisa Weidner
BACKGROUND: Therapeutic plasma exchange (TPE) is a generally accepted and frequently performed procedure for numerous therapeutic indications in adults. Slowly, TPE is also becoming more and more popular in the treatment of pediatric patients. Although, we know that TPE is safe in pediatric patients, the outcome of children treated with TPE is rarely reported. Furthermore, there are only general recommendations regarding the plasma replacement fluid for children and these are adopted from adults...
January 19, 2017: Transfusion and Apheresis Science
https://www.readbyqxmd.com/read/28185203/hla-haploidentical-hematopoietic-cell-transplantation-using-clofarabine-and-busulfan-for-refractory-pediatric-hematological-malignancy
#5
Masatoshi Takagi, Yasuyoshi Ishiwata, Yuki Aoki, Satoshi Miyamoto, Akihiro Hoshino, Kazuaki Matsumoto, Akira Nishimura, Mari Tanaka, Masakatsu Yanagimachi, Noriko Mitsuiki, Kohsuke Imai, Hirokazu Kanegane, Michiko Kajiwara, Kanako Takikawa, Tsukasa Mae, Osamu Tomita, Junya Fujimura, Masato Yasuhara, Daisuke Tomizawa, Shuki Mizutani, Tomohiro Morio
Haploidentical hematopoietic cell transplantation (HCT) conditioning with clofarabine and target area under the blood concentration-time curve (AUC)-based busulfan adjustment was performed in three patients with refractory pediatric leukemia. The target AUC for two patients who had already received multiple transplantations was 3600 and 4000 μmol min/L, and that for the patient with Down's syndrome was 3000 μmol min/L. Regimen-related toxicity was well tolerated in all cases. All three maintained cytological remission throughout the follow-up period (between 31 and 167 weeks)...
February 9, 2017: International Journal of Hematology
https://www.readbyqxmd.com/read/28184979/association-between-hematologic-and-inflammatory-markers-and-31-thrombotic-and-hemorrhagic-events-in-berlin-heart-excor-patients
#6
Amit Iyengar, Matthew L Hung, Kian Asanad, Oh Jin Kwon, Nicholas J Jackson, Brian L Reemtsen, Myke D Federman, Reshma M Biniwale
Bleeding and thrombotic events remain a significant cause of morbidity in pediatric patients supported with ventricular assist devices (VADs). The objective of this study is to identify the association between markers of anticoagulation and bleeding and thrombosis events during Berlin Heart ExCor support. A retrospective, single-center analysis of 9 patients supported with the Berlin Heart ExCor was performed. Inflammatory and anticoagulation parameters including C-reactive protein, fibrinogen, partial thromboplastin time (PTT), and platelet count were measured at 48 and 24 h before and after bleeding or thrombosis events...
February 10, 2017: Pediatric Cardiology
https://www.readbyqxmd.com/read/28183683/study-of-cd4-cd8-and-natural-killer-cells-cd16-cd56-in-children-with-immune-thrombocytopenic-purpura
#7
Farida Hussein El-Rashedi, Mahmoud Ahmed El-Hawy, Mohamed Ahmed Helwa, Sameh Said Abd-Allah
OBJECTIVE/BACKGROUND: To assess the percentage of CD4(+), CD8(+), and natural killer cells (CD16(+), CD56(+)) in children with immune thrombocytopenic purpura (ITP) at presentation and study their impact on disease chronicity. METHODS: This case-control study was conducted at the Pediatric Hematology and Oncology Unit, Menoufia University Hospital (tertiary care center in Egypt). The study was held on 30 children presenting with ITP; they were followed-up and classified into two groups: 15 children with acute ITP; and 15 children with chronic ITP...
February 1, 2017: Hematology/oncology and Stem Cell Therapy
https://www.readbyqxmd.com/read/28182021/procedural-moderate-sedation-with-ketamine-in-pediatric-critical-care-unit
#8
Tarek R Hazwani, Hala Al-Alem
OBJECTIVE: To evaluate the safety and efficacy of moderate sedation in the Pediatric Intensive Care Unit (PICU) settings according to moderate sedation protocol using ketamine and midazolam and to determine areas for the improvement in our clinical practice. SETTINGS AND DESIGN: A retrospective study was conducted in the PICU. MATERIALS AND METHODS: Retrospective chart review was performed for patients who had received moderate sedation between January and the end of December 2011 and who are eligible to inclusion criteria...
January 2017: Avicenna Journal of Medicine
https://www.readbyqxmd.com/read/28179213/juvenile-myelomonocytic-leukemia-in-turkey-a-retrospective-analysis-of-65-patients
#9
Özlem Tüfekçi, Ülker Koçak, Zühre Kaya, İdil Yenicesu, Canan Albayrak, Davut Albayrak, Şebnem Yılmaz Bengoa, Türkan Patıroğlu, Musa Karakükçü, Ekrem Ünal, Elif Ünal İnce, Talia İleri, Mehmet Ertem, Tiraje Celkan, Gül Nihal Özdemir, Nazan Sarper, Dilek Kaçar, Neşe Yaralı, Namık Yaşar Özbek, Alphan Küpesiz, Tuba Karapınar, Canan Vergin, Ümran Çalışkan, Hüseyin Tokgöz, Melike Sezgin Evim, Birol Baytan, Adalet Meral Güneş, Deniz Yılmaz Karapınar, Serap Karaman, Vedat Uygun, Gülsun Karasu, Mehmet Akif Yeşilipek, Ahmet Koç, Erol Erduran, Berna Atabay, Haldun Öniz, Hale Ören
OBJECTIVE: To define the status of juvenile myelomonocytic leukemia (JMML) patients in Turkey, in terms of time of diagnosis, clinical characteristics, mutational studies, clinical course and treatment strategies. MATERIALS AND METHODS: Data including clinical and labarotory characteristics and treatment strategies of JMML patients were collected retrospectively from pediatric hematology-oncology centers in Turkey. RESULTS: Sixty-five children with JMML diagnosed between 2002 and 2016 in 18 institutions throughout Turkey were enrolled into the study...
February 9, 2017: Turkish Journal of Haematology: Official Journal of Turkish Society of Haematology
https://www.readbyqxmd.com/read/28173661/-meeting-minutes-on-pediatric-hematology-forum-session-of-the-21st-annual-congress-of-chinese-pediatric-society
#10
(no author information available yet)
No abstract text is available yet for this article.
February 2, 2017: Zhonghua Er Ke za Zhi. Chinese Journal of Pediatrics
https://www.readbyqxmd.com/read/28168833/childhood-cancer-predisposition-syndromes-a-concise-review-and-recommendations-by-the-cancer-predisposition-working-group-of-the-society-for-pediatric-oncology-and-hematology
#11
Tim Ripperger, Stefan S Bielack, Arndt Borkhardt, Ines B Brecht, Birgit Burkhardt, Gabriele Calaminus, Klaus-Michael Debatin, Hedwig Deubzer, Uta Dirksen, Cornelia Eckert, Angelika Eggert, Miriam Erlacher, Gudrun Fleischhack, Michael C Frühwald, Astrid Gnekow, Gudrun Goehring, Norbert Graf, Helmut Hanenberg, Julia Hauer, Barbara Hero, Simone Hettmer, Katja von Hoff, Martin Horstmann, Juliane Hoyer, Thomas Illig, Peter Kaatsch, Roland Kappler, Kornelius Kerl, Thomas Klingebiel, Udo Kontny, Uwe Kordes, Dieter Körholz, Ewa Koscielniak, Christof M Kramm, Michaela Kuhlen, Andreas E Kulozik, Britta Lamottke, Ivo Leuschner, Dietmar R Lohmann, Andrea Meinhardt, Markus Metzler, Lüder H Meyer, Olga Moser, Michaela Nathrath, Charlotte M Niemeyer, Rainer Nustede, Kristian W Pajtler, Claudia Paret, Mareike Rasche, Dirk Reinhardt, Olaf Rieß, Alexandra Russo, Stefan Rutkowski, Brigitte Schlegelberger, Dominik Schneider, Reinhard Schneppenheim, Martin Schrappe, Christopher Schroeder, Dietrich von Schweinitz, Thorsten Simon, Monika Sparber-Sauer, Claudia Spix, Martin Stanulla, Doris Steinemann, Brigitte Strahm, Petra Temming, Kathrin Thomay, Andre O von Bueren, Peter Vorwerk, Olaf Witt, Marcin Wlodarski, Willy Wössmann, Martin Zenker, Stefanie Zimmermann, Stefan M Pfister, Christian P Kratz
Heritable predisposition is an important cause of cancer in children and adolescents. Although a large number of cancer predisposition genes and their associated syndromes and malignancies have already been described, it appears likely that there are more pediatric cancer patients in whom heritable cancer predisposition syndromes have yet to be recognized. In a consensus meeting in the beginning of 2016, we convened experts in Human Genetics and Pediatric Hematology/Oncology to review the available data, to categorize the large amount of information, and to develop recommendations regarding when a cancer predisposition syndrome should be suspected in a young oncology patient...
February 7, 2017: American Journal of Medical Genetics. Part A
https://www.readbyqxmd.com/read/28156496/influence-of-early-phase-clinical-trial-enrollment-on-patterns-of-end-of-life-care-for-children-with-advanced-cancer
#12
Chalinee Monsereenusorn, Clement Ma, Hasan Al-Sayegh, Joanne Wolfe, Carlos Rodriguez-Galindo
: 151 Background: Early phase clinical trials are critical to enhancing therapies for children with advanced cancer. However, trial enrollment may intensify end-of-life (EOL) care. We evaluated patterns of EOL care for patients at a large cancer center. METHODS: Single-center, retrospective cohort study of pediatric oncology patients, ages 6 months-21 years, who died in 2010-2014. We queried electronic medical records to assess frequencies of medical procedures (e...
October 9, 2016: Journal of Clinical Oncology: Official Journal of the American Society of Clinical Oncology
https://www.readbyqxmd.com/read/28152897/burden-of-bloodstream-infections-among-ambulatory-pediatric-hematology-oncology-patients-with-a-central-line
#13
David G Bundy, Aditya H Gaur, Jeffrey D Hord, Marlene R Miller, Eric J Werner, Cindi Winkle, Amy Billett
: 262 Background: Pediatric hematology/oncology (PHO) patients are at high risk of bloodstream infections (BSI). The burden of BSI in PHO patients in the ambulatory setting has not been well documented. METHODS: The Children's Hospital Association leads the Childhood Cancer and Blood Disorders Network, a multicenter United States quality improvement collaborative, working to reduce the incidence of inpatient and ambulatory Central Line-Associated BSI (CLABSI) among PHO patients...
March 2016: Journal of Clinical Oncology: Official Journal of the American Society of Clinical Oncology
https://www.readbyqxmd.com/read/28138379/non-invasive-methods-to-diagnose-fungal-infections-in-pediatric-patients-with-hematologic-disorders
#14
Parisa Badiee, Zahra Hashemizadeh, Mani Ramzi, Mohammad Karimi, Rasoul Mohammadi
BACKGROUND: Invasive fungal infection (IFIs) is a major infectious complication in immunocompromised patients. Early diagnosis and initiation of antifungal therapy is important to achieve the best outcome. OBJECTIVES: The current study aimed to investigate the incidence of IFIs and evaluate the diagnostic performance of non-invasive laboratory tests: serologic (β-D-glucan, galactomannan) and molecular (nested polymerase chain reaction) tests to diagnose fungal infections in hematologic pediatric patients...
November 2016: Jundishapur Journal of Microbiology
https://www.readbyqxmd.com/read/28137343/identifying-domains-of-quality-of-life-in-children-with-cancer-undergoing-palliative-care-a-qualitative-study-with-professionals
#15
Josianne Avoine-Blondin, Véronique Parent, Magali Lahaye, Nago Humbert, Michel Duval, Serge Sultan
OBJECTIVE: The goal of pediatric palliative care (PPC) is to maintain the quality of life (QoL) of children whose lives are threatened. However, there are sparse scientific data on the domains of QoL in this particular context, and no measurement strategies are available. The present study aims to describe the domains of QoL in the context of PPC in oncology, according to the perceptions of professional caregivers. METHOD: Semistructured interviews were conducted with a random sample of 20 professional caregivers from the Division of Hematology/Oncology at Le Centre Hospitalier Universitaire Sainte-Justine (Montréal, Canada)...
January 31, 2017: Palliative & Supportive Care
https://www.readbyqxmd.com/read/28123335/posterior-reversible-encephalopathy-syndrome-in-children-a-case-series
#16
Serhat Emeksiz, Nurettin Onur Kutlu, Hüseyin Çaksen, Gülsüm Alkan, Hülya Şeker Yıkmaz, Hüseyin Tokgöz
Posterior reversible encephalopathy syndrome is characterized by hypertension, seizure, headache, clouding of consciousness, and visual disturbance, and is diagnosed in the presence of typical lesions on magnetic resonance imaging. We retrospectively evaluated five patients who were diagnosed as having posterior reversible encephalopathy syndrome and followed up in Meram Medical Faculty, Pediatric Intensive Care and Hematology wards, between January 2010 and January 2014. We reviewed the demographic and clinical data, and neuroimaging findings...
December 2016: Türk Pediatri Arşivi
https://www.readbyqxmd.com/read/28115275/current-knowledge-and-priorities-for-future-research-in-late-effects-after-hematopoietic-cell-transplantation-hct-for-inherited-bone-marrow-failure-syndromes-ibmfs-consensus-statement-from-the-second-pediatric-blood-and-marrow-transplant-consortium-international
#17
REVIEW
Andrew C Dietz, Parinda A Mehta, Adrianna Vlachos, Sharon A Savage, Dorine Bresters, Jakub Tolar, Farid Boulad, Jean Hugues Dalle, Carmem Bonfim, Josu de la Fuente, Christine N Duncan, K Scott Baker, Michael A Pulsipher, Jeffrey M Lipton, John E Wagner, Blanche P Alter
Fanconi anemia (FA), dyskeratosis congenita (DC), and Diamond Blackfan anemia (DBA) are three of the most common inherited bone marrow failure syndromes (IBMFS), in which the hematologic manifestations can be cured with hematopoietic cell transplantation (HCT). Later in life these patients face a variety of medical conditions which may be a manifestation of underlying disease, may be due to their pre-HCT therapy, may be due to their HCT, or may be a combination of all these elements. Very limited long-term follow-up data exist in these populations, with FA the only IBMFS that has specific published data...
January 20, 2017: Biology of Blood and Marrow Transplantation
https://www.readbyqxmd.com/read/28103644/flow-cytometric-osmotic-fragility-test-increased-assay-sensitivity-for-clinical-application-in-pediatric-hematology
#18
Olga Ciepiela, Anna Adamowicz-Salach, Agata Zgodzińska, Magdalena Łazowska, Iwona Kotuła
BACKGROUND: Osmotic Fragility Test (OFT) is widely considered as a sensitive indicator of red blood cells' sensitivity to the hypotonic solution. It is often used as a screening test for the diagnosis of hereditary spherocytosis (HS). Nowadays, the osmotic fragility test based on flow cytometric analysis (FCM OF) is widely used in laboratory practice. The purpose of this study was to optimize the assay sensitivity and to validate its clinical application in the diagnostic screening of childhood anemias...
January 19, 2017: Cytometry. Part B, Clinical Cytometry
https://www.readbyqxmd.com/read/28095119/fecal-carriage-of-extended-spectrum-%C3%AE-lactamase-producing-enterobacteriaceae-strains-is-associated-with-worse-outcome-in-patients-hospitalized-in-the-pediatric-oncology-unit-of-beni-messous-hospital-in-algiers-algeria
#19
Chafiaa Medboua-Benbalagh, Abdelaziz Touati, Rachida Kermas, Alima Gharout-Sait, Lucien Brasme, Halima Mezhoud, Djamila Touati, Thomas Guillard, Christophe de Champs
OBJECTIVES: The current study aimed to investigate extended-spectrum β-lactamase-producing Enterobacteriaceae (ESBL-E) fecal carriage in children with different cancers admitted in the pediatric oncology unit of Beni-Messous Hospital (Algiers, Algeria). MATERIALS AND METHODS: Rectal swabs from children with cancer were sampled from February 2012 to May 2013 within 48 hours following their admission. After species identification and detection of ESBL production by double-disk synergy test (DD test), antibiotic susceptibility was determined by the standard disk diffusion method...
January 17, 2017: Microbial Drug Resistance: MDR: Mechanisms, Epidemiology, and Disease
https://www.readbyqxmd.com/read/28089099/clinical-characteristics-and-outcome-of-invasive-fungal-infections-in-pediatric-acute-myeloid-leukemia-patients-in-a-medical-center-in-taiwan
#20
Gu-Lung Lin, Hsiu-Hao Chang, Chun-Yi Lu, Chung-Ming Chen, Meng-Yao Lu, Ping-Ing Lee, Shiann-Tarng Jou, Yung-Li Yang, Li-Min Huang, Luan-Yin Chang
BACKGROUND: Invasive fungal infection (IFI) causes significant morbidity and mortality in patients with hematological malignancies, especially those with acute myeloid leukemia (AML), recurrent acute leukemia, high-risk acute lymphoblastic leukemia, and after allogeneic hematopoietic stem cell transplantation. The study aimed to investigate the clinical characteristics and outcome of IFIs in pediatric AML patients in a medical center in Taiwan. METHODS: We performed retrospective chart reviews...
December 19, 2016: Journal of Microbiology, Immunology, and Infection, Wei Mian Yu Gan Ran za Zhi
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