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Paediatric Haematology

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https://www.readbyqxmd.com/read/27891440/radiation-therapy-in-paediatric-orbital-granulocytic-sarcomas-experience-from-a-tertiary-cancer-center
#1
Sushmita Pathy, Bhanu Prasad Venkatesulu, Supriya Mallick, Subhash Chander
INTRODUCTION: Orbital Granulocytic Sarcoma (OGS) is an uncommon manifestation associated with haematological malignancies. Chemotherapy remains the cornerstone of the treatment. The role of radiation is not well-defined. AIM: To evaluate the effect of radiation in OGS and to define an optimal dose for achieving adequate local control. MATERIALS AND METHODS: This was a retrospective analysis of 11 patients who received radiation therapy to orbit for Granulocytic Sarcoma (GS) between 2007 and 2014 at a tertiary cancer center in India...
October 2016: Journal of Clinical and Diagnostic Research: JCDR
https://www.readbyqxmd.com/read/27888526/the-optimal-single-dose-regimen-of-rasburicase-for-management-of-tumour-lysis-syndrome-in-children-and-adults-a-systematic-review-and-meta-analysis
#2
REVIEW
X Yu, L Liu, X Nie, J Li, J Zhang, L Zhao, X Wang
WHAT IS KNOWN AND OBJECTIVE: To perform a meta-analysis exploring the optimal single-dose regimen for managing tumour lysis syndrome (TLS) in children and adults with haematological malignancies. METHODS: We systematically searched PubMed, MEDLINE, Web of Science, the Cochrane Library and the ClinicalTrials.gov website for studies regarding single-dose rasburicase in paediatric and adult patients with TLS. Data were analysed using Open MetaAnalyst statistical software...
November 25, 2016: Journal of Clinical Pharmacy and Therapeutics
https://www.readbyqxmd.com/read/27884679/risk-stratified-staging-in-paediatric-hepatoblastoma-a-unified-analysis-from-the-children-s-hepatic-tumors-international-collaboration
#3
Rebecka L Meyers, Rudolf Maibach, Eiso Hiyama, Beate Häberle, Mark Krailo, Arun Rangaswami, Daniel C Aronson, Marcio H Malogolowkin, Giorgio Perilongo, Dietrich von Schweinitz, Marc Ansari, Dolores Lopez-Terrada, Yukichi Tanaka, Rita Alaggio, Ivo Leuschner, Tomoro Hishiki, Irene Schmid, Kenichiro Watanabe, Kenichi Yoshimura, Yurong Feng, Eugenia Rinaldi, Davide Saraceno, Marisa Derosa, Piotr Czauderna
BACKGROUND: Comparative assessment of treatment results in paediatric hepatoblastoma trials has been hampered by small patient numbers and the use of multiple disparate staging systems by the four major trial groups. To address this challenge, we formed a global coalition, the Children's Hepatic tumors International Collaboration (CHIC), with the aim of creating a common approach to staging and risk stratification in this rare cancer. METHODS: The CHIC steering committee-consisting of leadership from the four major cooperative trial groups (the International Childhood Liver Tumours Strategy Group, Children's Oncology Group, the German Society for Paediatric Oncology and Haematology, and the Japanese Study Group for Paediatric Liver Tumours)-created a shared international database that includes comprehensive data from 1605 children treated in eight multicentre hepatoblastoma trials over 25 years...
November 21, 2016: Lancet Oncology
https://www.readbyqxmd.com/read/27832748/invasive-mucormycosis-in-children-an-epidemiologic-study-in-european-and-non-european-countries-based-on-two-registries
#4
Zoi Dorothea Pana, Danila Seidel, Anna Skiada, Andreas H Groll, Georgios Petrikkos, Oliver A Cornely, Emmanuel Roilides
BACKGROUND: Mucormycosis has emerged as a rare but frequently fatal invasive fungal disease. Current knowledge on paediatric mucormycosis is based on case reports and small series reported over several decades. Contemporary data on a large cohort of patients is lacking. METHODS: Two large international registries (Zygomyco.net and FungiScope™) were searched for mucormycosis cases in ≤19 year-old patients. Cases enrolled between 2005 and 2014 were extracted, and dual entries in the two databases merged...
November 10, 2016: BMC Infectious Diseases
https://www.readbyqxmd.com/read/27807959/-an-example-of-the-integrated-use-of-administrative-health-databases-for-the-study-of-childhood-cancer-clusters
#5
Patrizia Schifano, Claudia Marino, Federica Asta, Manuela De Sario, Paola Michelozzi
We developed an algorithm to select incident cases of lymphatic and haematopoietic system in children, in order to create a timely and accurate database of incident cases to answer the needs of cluster analysis. This group of cancers is by far the most frequent in the paediatric age. The procedure has been applied to identify incident cases in children aged 0-14 years, resident in Rome (Central Italy), and diagnosed between 2000 and 2011. The algorithm links hospital discharge records (SDO), the hospital registry of the Italian Association of paediatric haematology and oncology (AIEOP), and the Population Registry...
September 2016: Epidemiologia e Prevenzione
https://www.readbyqxmd.com/read/27807958/-the-contribution-of-the-italian-association-of-cancer-registries-airtum
#6
Emanuele Crocetti, Carlotta Buzzoni
The study of cluster requires the ability to identify, with accuracy and completeness, the health events of interest and their geographical location and time of occurrence. For rare and complex diseases, such as childhood cancers, it is possible to observe a significant health migration from the place of residence, which makes the detection even more complex. The best tool to identify these rare diseases is represented by cancer registries (CRs). In fact, CRs collect, through many sources, information related to tumours that arise in the population resident in their areas of activity...
September 2016: Epidemiologia e Prevenzione
https://www.readbyqxmd.com/read/27807957/-the-contribution-of-the-italian-association-of-paediatric-haematology-and-oncology-aieop
#7
Roberto Rondelli, Momcilo Jankovic, Annarosa Soresina, Maria Grazia Valsecchi, Marisa De Rosa, Marina Cuttini, Riccardo Haupt, Maurizio Aricò, Gianni Bisogno, Franco Locatelli, Corrado Magnani, Franco Merletti, Marco Zecca, Andrea Pession
Since 1972, children affected by cancer took advantage from multi-centric diagnostic and therapeutic protocols produced by the Italian Association of paediatric haematology and oncology (AIEOP). At the beginning, these protocols were used in few well-trained centres, later in almost all Italian haemato-oncological centres. The need of a careful monitoring of his own activity induced AIEOP to achieve, in 1989, an Italian hospital- based registry (database Mod.1.01) of malignant tumours diagnosed and treated in the participating centres, with the aim to quantify the number of cases diagnosed and treated in the different centres, the agreement (or not) to official diagnostic- therapeutic protocols, and the extraregional migration...
September 2016: Epidemiologia e Prevenzione
https://www.readbyqxmd.com/read/27781102/cytomegalovirus-and-paediatric-hiv-infection
#8
EDITORIAL
Jennifer A Slyker
Cytomegalovirus (CMV) was among the most common AIDS-defining illnesses prior to the advent of combination antiretroviral therapy (ART). In the ART era, CMV disease remains a significant public health threat among HIV-infected adults and children with delayed HIV diagnosis. CMV co-infection may additionally contribute to accelerated HIV progression, development of inflammation-related comorbidities, immune senescence and developmental deficits. Elimination of CMV would have tremendous public health significance and is an important priority; however, current vaccine strategies are not targeted at HIV-infected individuals...
October 5, 2016: Journal of Virus Eradication
https://www.readbyqxmd.com/read/27773490/-adapting-immunisation-schedules-for-children-undergoing-chemotherapy
#9
María Fernández-Prada, María Rodríguez-Martínez, Rebeca García-García, María Dolores García-Corte, Carmen Martínez-Ortega
INTRODUCTION: Children undergoing chemotherapy for cancer have special vaccination needs after completion of the treatment. The aim of this study was to evaluate the adaptation of post-chemotherapy vaccination schedules. METHOD: An observational study was performed on a retrospective cohort that included all children aged from 0 to 14 years, who completed chemotherapy in a tertiary hospital between 2009 and 2015. Inclusion and exclusion criteria were applied. Immunisation was administered in accordance with the guidelines of the Vaccine Advisory Committee of the Spanish Association of Paediatrics...
October 20, 2016: Enfermedades Infecciosas y Microbiología Clínica
https://www.readbyqxmd.com/read/27771618/juvenile-systemic-lupus-erythematosus-in-nigeria
#10
O O Adelowo, B H Olaosebikan, B A Animashaun, R O Akintayo
Juvenile systemic lupus erythematosus (JSLE) is a complex multisystemic autoimmune disorder of unknown cause. It accounts for about one in five cases of SLE. The tendency for SLE to run a fulminant course when it starts in childhood has made JSLE a potentially more severe disease than adult SLE. Reports of JSLE from sub-Saharan Africa are scanty in spite of the increasing reports of adult SLE. We conducted a 4-year retrospective study of JSLE cases seen at the Lagos State University Teaching Hospital between January 2010 and December 2014...
October 21, 2016: Lupus
https://www.readbyqxmd.com/read/27766616/genomics-of-primary-chemoresistance-and-remission-induction-failure-in-paediatric-and-adult-acute-myeloid-leukaemia
#11
Fiona C Brown, Paolo Cifani, Esther Drill, Jie He, Eric Still, Shan Zhong, Sohail Balasubramanian, Dean Pavlick, Bahar Yilmazel, Kristina M Knapp, Todd A Alonzo, Soheil Meshinchi, Richard M Stone, Steven M Kornblau, Guido Marcucci, Alan S Gamis, John C Byrd, Mithat Gonen, Ross L Levine, Alex Kentsis
Cure rates of children and adults with acute myeloid leukaemia (AML) remain unsatisfactory partly due to chemotherapy resistance. We investigated the genetic basis of AML in 107 primary cases by sequencing 670 genes mutated in haematological malignancies. SETBP1, ASXL1 and RELN mutations were significantly associated with primary chemoresistance. We identified genomic alterations not previously described in AML, together with distinct genes that were significantly overexpressed in therapy-resistant AML. Defined gene mutations were sufficient to explain primary induction failure in only a minority of cases...
October 21, 2016: British Journal of Haematology
https://www.readbyqxmd.com/read/27748030/the-effect-of-central-nervous-system-involvement-and-irradiation-in-childhood-acute-lymphoblastic-leukemia-lessons-from-the-nopho-all-92-and-all-2000-protocols
#12
Mervi Taskinen, Trausti Oskarsson, Mette Levinsen, Matteo Bottai, Marit Hellebostad, Olafur Gisli Jonsson, Päivi Lähteenmäki, Kjeld Schmiegelow, Mats Heyman
BACKGROUND: Central nervous system irradiation (CNS-RT) has played a central role in the cure of acute lymphoblastic leukemia (ALL), but due to the risk of long-term toxicity, it is now considered a less-favorable method of CNS-directed therapy. PROCEDURES: Retrospectively, we estimated the effect of CNS involvement and CNS-RT on events and overall survival (OS) in 835 children treated for high-risk ALL in the Nordic Society of Paediatric Haematology and Oncology (NOPHO) ALL-92 and ALL-2000 trials...
October 17, 2016: Pediatric Blood & Cancer
https://www.readbyqxmd.com/read/27720178/child-onset-and-adolescent-onset-acquired-thrombotic-thrombocytopenic-purpura-with-severe-adamts13-deficiency-a-cohort-study-of-the-french-national-registry-for-thrombotic-microangiopathy
#13
Bérangère S Joly, Alain Stepanian, Thierry Leblanc, David Hajage, Hervé Chambost, Jérôme Harambat, Fanny Fouyssac, Vincent Guigonis, Guy Leverger, Tim Ulinski, Thérésa Kwon, Chantal Loirat, Paul Coppo, Agnès Veyradier
BACKGROUND: Thrombotic thrombocytopenic purpura is a rare thrombotic microangiopathy, related to a severe ADAMTS13 deficiency (a disintegrin and metalloprotease with thromboSpondin type 1 repeats, member 13; activity <10% of normal). Childhood-onset thrombotic thrombocytopenic purpura is very rare and initially often misdiagnosed, especially when ADAMTS13 deficiency is acquired (ie, not linked to inherited mutations of the ADAMTS13 gene). We aimed to investigate initial presentation, management, and outcome of acquired thrombotic thrombocytopenic purpura in children...
October 3, 2016: Lancet Haematology
https://www.readbyqxmd.com/read/27688725/impact-of-mtor-expression-on-clinical-outcome-in-paediatric-patients-with-b-cell-acute-lymphoblastic-leukaemia-preliminary-report
#14
Edyta Ulińska, Katarzyna Mycko, Elżbieta Sałacińska-Łoś, Agata Pastorczak, Alicja Siwicka, Wojciech Młynarski, Michał Matysiak
AIM OF THE STUDY: To characterise expression of mTOR (mammalian target of rapamycin) in childhood B-cell acute lymphoblastic leukaemia (ALL), and to evaluate a possible link between mTOR and clinical characteristics. MATERIAL AND METHODS: The examined group consisted of 21 consecutive patients, aged 1-18 years, diagnosed with B-cell ALL in 2010, and 10 relapsed B-cell ALL patients diagnosed for the first time between 2009 and 2011, who developed relapse before 2014...
2016: Contemporary Oncology Współczesna Onkologia
https://www.readbyqxmd.com/read/27643863/treatment-of-relapse-after-allogeneic-stem-cell-transplantation-in-children-and-adolescents-with-all-the-frankfurt-experience
#15
A M Willasch, E Salzmann-Manrique, T Krenn, M Duerken, J Faber, J Opper, H Kreyenberg, R Bager, S Huenecke, C Cappel, M Bremm, V Pfirrmann, M Merker, E Ullrich, S Bakhtiar, E Rettinger, A Jarisch, J Soerensen, T E Klingebiel, P Bader
Therapy for post-transplant relapse of paediatric ALL is limited. Standardised curative approaches are not available. We hereby describe our local procedure in this life-threatening situation. A total of 101 ALL patients received their first allogeneic stem cell transplantation (SCT) in our institution. After relapse, our primary therapeutic goal was to cure the patient with high-dose chemotherapy or specific immunotherapy (HDCHT/SIT) followed by a second SCT from a haploidentical donor (transplant approach)...
September 19, 2016: Bone Marrow Transplantation
https://www.readbyqxmd.com/read/27639979/prevalence-of-stroke-in-children-admitted-with-sickle-cell-anaemia-to-mulago-hospital
#16
Deogratias Munube, Elly Katabira, Grace Ndeezi, Moses Joloba, Samden Lhatoo, Martha Sajatovic, James K Tumwine
BACKGROUND: Stroke is a major complication of sickle cell anaemia (SCA). It occurs commonly in childhood with about 10 % of children with sickle cell anaemia getting affected by this complication. In Uganda, there is paucity of data on the prevalence of stroke in children admitted in a tertiary institution. We determined the prevalence of stroke amongst children with SCA admitted to Mulago National Referral Hospital in Uganda and described the ir co-morbidities. METHODS: We conducted a retrospective record review of children with SCA admitted from August 2012 to August 2014 to the Paediatric Haematology Ward of Mulago Hospital in Kampala, Uganda...
September 17, 2016: BMC Neurology
https://www.readbyqxmd.com/read/27616321/an-11-year-experience-of-acquired-von-willebrand-syndrome-in-children-diagnosed-with-wilms-tumour-in-a-tertiary-referral-centre
#17
Emma Fosbury, Elwira Szychot, Olga Slater, Mary Mathias, Keith Sibson
Wilms tumour (WT) is the commonest primary malignant renal tumour of childhood. Acquired von Willebrand syndrome (avWS) is a well-described paraneoplastic phenomenon, but it is uncommon and may not be detected until clinically significant bleeding is encountered during interventional procedures. Previous studies on small cohorts of patients have determined an incidence of between 4 and 8%. We have performed a retrospective study on cases of WT presenting over an 11.5-year period to a paediatric haematology/oncology unit in a tertiary referral centre to review the incidence of avWS, bleeding phenotype, management, and response to treatment of the primary pathology...
September 12, 2016: Pediatric Blood & Cancer
https://www.readbyqxmd.com/read/27614870/critical-review-of-current-clinical-practice-guidelines-for-antifungal-therapy-in-paediatric-haematology-and-oncology
#18
Jessica E Morgan, Hadeel Hassan, Julia V Cockle, Christopher Lethaby, Beki James, Robert S Phillips
PURPOSE: The incidence of invasive fungal disease (IFD) is rising, but its treatment in paediatric haematology and oncology patients is not yet standardised. This review aimed to critically appraise and analyse the clinical practice guidelines (CPGs) that are available for paediatric IFD. METHODS: Electronic searches of MEDLINE, MEDLINE in-Process & Other non-Indexed Citations, the Guidelines International Network (GIN), guideline.gov and Google were performed and combined fungal disease (Fung* OR antifung*OR Candida* OR Aspergill*) with prophylaxis or treatment (prophyl* OR therap* OR treatment)...
January 2017: Supportive Care in Cancer: Official Journal of the Multinational Association of Supportive Care in Cancer
https://www.readbyqxmd.com/read/27609328/safety-and-effectiveness-of-total-splenic-vessel-ligations-in-paediatric-patients-with-splenomegaly
#19
Chen Zhen, Ma Lishuang, Zhang Jinshan, Qiao Guoliang, Zhang Zhen, Liu Shuili, Zhang Jun, Guan Kaoping, Li Long
BACKGROUNDS: Splenomegaly may contribute to hypersplenism and can result in thrombocytopenia. Many approaches are used to treat splenomegaly; however, the current management of splenomegaly has intrinsic limitations or disadvantages. Now, we initiate a new approach, that of total splenic vessel (artery and vein) ligations (TSVLs) in paediatric patients with splenomegaly. The purpose of our study is to evaluate the results obtained with TVSLs procedure for paediatric patients. PATIENTS AND METHODS: Seventeen paediatric patients with splenomegaly were screened for enrolment into this retrospective analysis...
October 2016: Journal of Minimal Access Surgery
https://www.readbyqxmd.com/read/27606107/first-implementation-of-transfusion-consent-policy-in-oman-audit-of-compliance-from-a-tertiary-care-university-hospital
#20
Arwa Z Al-Riyami, Naif Al-Ghafri, Fehmida Zia, Mohammed Al-Huneini, Abdul-Hakeem Al-Rawas, Salam Al-Kindi, Sachin Jose, Murtadha Al-Khabori, Hilal Al-Sabti, Shahina Daar
OBJECTIVES: Transfusions are a common medical intervention. Discussion of the benefits, risks and alternatives with the patient is mandated by many legislations prior to planned transfusions. At the Sultan Qaboos University Hospital (SQUH), Muscat, Oman, a written transfusion consent policy was introduced in March 2014. This was the first time such a policy was implemented in Oman. This study therefore aimed to assess adherence to this policy among different specialties within SQUH. METHODS: The medical records of patients who underwent elective transfusions between June and August 2014 were reviewed to assess the presence of transfusion consent forms...
August 2016: Sultan Qaboos University Medical Journal
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