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Paediatric Haematology

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https://www.readbyqxmd.com/read/28542715/hyperleucocytosis-in-paediatric-acute-myeloid-leukaemia-the-challenge-of-white-blood-cell-counts-above-200%C3%A2-%C3%A3-%C3%A2-10-9-l-the-nopho-experience-1984-2014
#1
Bernward Zeller, Heidi Glosli, Erik Forestier, Shau-Yin Ha, Kirsi Jahnukainen, Ólafur G Jónsson, Birgitte Lausen, Josefine Palle, Henrik Hasle, Jonas Abrahamsson
Hyperleucocytosis in paediatric acute myeloid leukaemia (AML) is associated with increased morbidity and mortality. We studied hyperleucocytosis in 890 patients with AML aged 0-18 years registered in the Nordic Society of Paediatric Haematology and Oncology (NOPHO) registry, with special focus on very high white blood cell counts (WBC >200 × 10/l). Eighty-six patients (10%) had WBC 100-199 × 10(9) /l and 57 (6%) had WBC ≥200 × 10(9) /l. Patients with WBC ≥200 × 10(9) /l had a high frequency of t(9;11) and a paucity of trisomy 8...
May 25, 2017: British Journal of Haematology
https://www.readbyqxmd.com/read/28538707/self-esteem-and-academic-difficulties-in-preadolescents-and-adolescents-healed-from-paediatric-leukaemia
#2
Marta Tremolada, Livia Taverna, Sabrina Bonichini, Giuseppe Basso, Marta Pillon
Adolescents with cancer may demonstrate problems in their self-esteem and schooling. This study aims to screen the preadolescents and adolescents more at risk in their self-esteem perception and schooling difficulties post-five years from the end of therapy. Twenty-five paediatric ex-patients healed from leukaemia were recruited at the Haematology-Oncologic Clinic (University of Padua). The mean age of the children was 13.64 years (Standard Deviation (SD)) = 3.08, range = 10-19 years), most were treated for acute lymphoblastic leukaemia (ALL) (84%) and relatively equally distributed by gender...
May 24, 2017: Cancers
https://www.readbyqxmd.com/read/28535809/safety-and-tolerability-of-adjunctive-rosiglitazone-treatment-for-children-with-uncomplicated-malaria
#3
Rosauro Varo, Valerie M Crowley, Antonio Sitoe, Lola Madrid, Lena Serghides, Rubao Bila, Helio Mucavele, Alfredo Mayor, Quique Bassat, Kevin C Kain
BACKGROUND: Despite the widespread use and availability of rapidly acting anti-malarials, the fatality rate of severe malaria in sub-Saharan Africa remains high. Adjunctive therapies that target the host response to malaria infection may further decrease mortality over that of anti-malarial agents alone. Peroxisome proliferator-activated receptor-gamma agonists (e.g. rosiglitazone) have been shown to act on several pathways implicated in the pathogenesis of severe malaria and may improve clinical outcome as an adjunctive intervention...
May 23, 2017: Malaria Journal
https://www.readbyqxmd.com/read/28500740/children-with-low-risk-acute-lymphoblastic-leukemia-are-at-highest-risk-of-second-cancers
#4
Stine N Nielsen, Frank Eriksson, Susanne Rosthoej, Mette K Andersen, Erik Forestier, Henrik Hasle, Lisa L Hjalgrim, Ann Aasberg, Jonas Abrahamsson, Mats Heyman, Ólafur G Jónsson, Kaie Pruunsild, Goda E Vaitkeviciené, Kim Vettenranta, Kjeld Schmiegelow
BACKGROUND: The improved survival rates for childhood acute lymphoblastic leukemia (ALL) may be jeopardized by the development of a second cancer, which has been associated with thiopurine therapy. PROCEDURE: We retrospectively analyzed three sequential Nordic Society of Paediatric Haematology and Oncology's protocols characterized by increasing intensity of thiopurine-based maintenance therapy. We explored the risk of second cancer in relation to protocols, risk group, thiopurine methyltransferase (TPMT) activity, ALL high hyperdiploidy (HeH), and t(12;21)[ETV6/RUNX1]...
May 13, 2017: Pediatric Blood & Cancer
https://www.readbyqxmd.com/read/28473426/european-evidence-based-recommendations-for-diagnosis-and-treatment-of-paediatric-antiphospholipid-syndrome-the-share-initiative
#5
Noortje Groot, Nienke de Graeff, Tadej Avcin, Brigitte Bader-Meunier, Pavla Dolezalova, Brian Feldman, Gili Kenet, Isabelle Koné-Paut, Pekka Lahdenne, Stephen D Marks, Liza McCann, Clarissa A Pilkington, Angelo Ravelli, Annet van Royen-Kerkhof, Yosef Uziel, Sebastiaan J Vastert, Nico M Wulffraat, Seza Ozen, Paul Brogan, Sylvia Kamphuis, Michael W Beresford
Antiphospholipid syndrome (APS) is rare in children, and evidence-based guidelines are sparse. Consequently, management is mostly based on observational studies and physician's experience, and treatment regimens differ widely. The Single Hub and Access point for paediatric Rheumatology in Europe (SHARE) initiative was launched to develop diagnostic and management regimens for children and young adults with rheumatic diseases. Here, we developed evidence-based recommendations for diagnosis and treatment of paediatric APS...
May 4, 2017: Annals of the Rheumatic Diseases
https://www.readbyqxmd.com/read/28439893/outcome-after-intensive-reinduction-therapy-and-allogeneic-stem-cell-transplant-in-paediatric-relapsed-acute-myeloid-leukaemia
#6
Lene Karlsson, Erik Forestier, Henrik Hasle, Kirsi Jahnukainen, Ólafur G Jónsson, Birgitte Lausen, Ulrika Norén Nyström, Josefine Palle, Anne Tierens, Bernward Zeller, Jonas Abrahamsson
Given that 30-40% of children with acute myeloid leukaemia (AML) relapse after primary therapy it is important to define prognostic factors and identify optimal therapy. From 1993 to 2012, 543 children from the Nordic countries were treated according to two consecutive protocols: 208 children relapsed. The influence of disease characteristics, first line treatment, relapse therapy and duration of first remission on outcome was analysed. Second complete remission (CR2) was achieved in 146 (70%) patients. Estimated 5-year overall survival (OS5y ) was 39 ± 4% for the whole group and 43 ± 4% for the 190 patients given re-induction therapy, of whom 76% received regimens that included fludarabine, cytarabine (FLA) ± anthracyclines, 18% received Nordic Society for Paediatric Haematology and Oncology (NOPHO) upfront blocks and 5% received other regimens...
April 25, 2017: British Journal of Haematology
https://www.readbyqxmd.com/read/28423235/hepatic-sinusoidal-obstruction-syndrome-during-maintenance-therapy-of-childhood-acute-lymphoblastic-leukemia-is-associated-with-continuous-asparaginase-therapy-and-mercaptopurine-metabolites
#7
Linea Natalie Toksvang, Silvia De Pietri, Stine N Nielsen, Jacob Nersting, Birgitte K Albertsen, Peder S Wehner, Steen Rosthøj, Päivi M Lähteenmäki, Daniel Nilsson, Tove A Nystad, Kathrine Grell, Thomas L Frandsen, Kjeld Schmiegelow
BACKGROUND: Hepatic sinusoidal obstruction syndrome (SOS) during treatment of childhood acute lymphoblastic leukemia (ALL) has mainly been associated with 6-thioguanine. The occurrence of several SOS cases after the introduction of extended pegylated asparaginase (PEG-asparaginase) therapy in the Nordic Society of Paediatric Haematology and Oncology (NOPHO) ALL2008 protocol led us to hypothesize that PEG-asparaginase, combined with other drugs, may trigger SOS during 6-thioguanine-free maintenance therapy...
April 19, 2017: Pediatric Blood & Cancer
https://www.readbyqxmd.com/read/28336313/vancomycin-resistant-enterococci-colonisation-risk-factors-and-risk-for-infection-among-hospitalised-paediatric-patients-a-systematic-review-and-meta-analysis
#8
REVIEW
Myrto Eleni Flokas, Spyridon A Karageorgos, Marios Detsis, Michail Alevizakos, Eleftherios Mylonakis
The objective of this study was to estimate the rate and significance of colonisation with vancomycin-resistant enterococci (VRE) among hospitalised children. The PubMed and EMBASE databases were systematically searched (last accessed on 29 May 2016) to identify studies evaluating VRE colonisation of the gastrointestinal tract of hospitalised children in non-outbreak periods. Of 945 non-duplicate citations, 19 studies enrolling 20 234 children were included. The overall and paediatric intensive care unit (PICU) rate of VRE colonisation were both 5% [95% confidence interval (CI) 3-8% overall and 95% CI 2-9% in the PICU] but was 23% in haematology/oncology units (95% CI 18-29%)...
March 20, 2017: International Journal of Antimicrobial Agents
https://www.readbyqxmd.com/read/28333866/prevention-of-gastrointestinal-side-effects-in-paediatric-oncology-what-are-the-guidelines
#9
Karis K F Cheng
PURPOSE OF REVIEW: Gastrointestinal side-effects, particularly with regard to alimentary tract mucositis and chemotherapy-induced nausea and vomiting (CINV), continue to be frequent and debilitating symptomatic conditions among children and adolescents receiving cytotoxic cancer therapy. Further avenues of progress for mucositis and CINV prevention in paediatric oncology setting are warranted. RECENT FINDINGS: The current article reviews the major guidelines and literature published in 2016 pertaining to the prevention of mucositis and CINV...
June 2017: Current Opinion in Supportive and Palliative Care
https://www.readbyqxmd.com/read/28324923/topotecan-vincristine-doxorubicin-in-stage-4-high-risk-neuroblastoma-patients-failing-to-achieve-a-complete-metastatic-response-to-rapid-cojec-a-siopen-study
#10
Loredana Amoroso, Giovanni Erminio, Guy Makin, Andrew Dj Pearson, Penelope Brock, Dominique Valteau-Couanet, Victoria Castel, Marlène Pasquet, Genevieve Laureys, Caroline Thomas, Roberto Luksch, Ruth Ladenstein, Riccardo Haupt, Alberto Garaventa
Purpose: Metastatic response to induction therapy for high-risk neuroblastoma is a prognostic factor. In the International Society of Paediatric Oncology Europe Neuroblastoma (SIOPEN) HR-NBL-1 protocol, only patients with metastatic complete response (CR) or partial response (PR) with ≤3 abnormal skeletal areas on 123iodine-metaiodobenzylguanidine ([123I]mIBG) scintigraphy and no bone marrow disease proceed to high dose therapy (HDT). In this study, topotecan-vincristine-doxorubicin (TVD) was evaluated in patients failing to achieve these criteria, with the aim of improving the metastatic response rate...
March 21, 2017: Cancer Research and Treatment: Official Journal of Korean Cancer Association
https://www.readbyqxmd.com/read/28292719/donor-age-matters-in-t-cell-depleted-haploidentical-hematopoietic-stem-cell-transplantation-in-pediatric-patients-faster-immune-reconstitution-using-younger-donors
#11
Marta González-Vicent, Blanca Molina, Natalia Deltoro, Julián Sevilla, José Luis Vicario, Ana Castillo, Manuel Ramirez, Miguel Ángel Díaz
T-cell depleted (TCD) haploidentical transplantation is increasingly used in paediatric patients with haematological malignancies and donor selection is a challenge. We conclude that a simple criterion such as donor age should be also considered in depleted haploidentical setting because faster immune reconstitution is achieved using younger donors decreasing non-relapse related mortality.
March 4, 2017: Leukemia Research
https://www.readbyqxmd.com/read/28288233/-dress-syndrome-in-paediatrics-clinical-case
#12
Marcos Silva-Feistner, Elena Ortiz, María Jesús Rojas-Lechuga, Daniel Muñoz
Drug reaction with eosinophilia and systemic symptoms (DRESS) is a rare, potentially life-threatening, drug-induced hypersensitivity reaction that includes skin eruption, haematological abnormalities, lymphadenopathy, and internal organ involvement. OBJECTIVE: Presenting a rare condition in children, to facilitate a rapid diagnostic suspicion and recognition by doctors. CASE REPORT: An 9 months old infant admitted due to a severe viral pneumonia, managed with non-invasive ventilation and ceftriaxone...
February 2017: Revista Chilena de Pediatría
https://www.readbyqxmd.com/read/28281429/single-centre-experience-of-allogeneic-haemopoietic-stem-cell-transplant-in-paediatric-patients-in-cape-town-south-africa
#13
A Van Eyssen, N Novitsky, P De Wit, T Schlaphoff, V Thomas, D Pillay, M Hendricks, A Davidson
BACKGROUND: Allogeneic haemopoietic stem cell transplant (Allo-HSCT) is a specialised and costly intervention, associated with significant morbidity and mortality. It is used to treat a broad range of paediatric conditions. South Africa (SA) is an upper middle-income country with limitations on healthcare spending. The role of paediatric Allo-HSCT in this setting is reviewed. OBJECTIVES: To review paediatric patients who underwent Allo-HSCT at the Groote Schuur Hospital/University of Cape Town Private Academic Hospital transplant unit in Cape Town, South Africa, and received post-transplant care at Red Cross War Memorial Children's Hospital, over the period January 2006 - December 2014 in respect of indications for the transplant, donor sources, conditioning regimens, treatment-related morbidity and overall survival (OS)...
February 27, 2017: South African Medical Journal, Suid-Afrikaanse Tydskrif Vir Geneeskunde
https://www.readbyqxmd.com/read/28279690/-early-clinical-trials-in-paediatric-oncology-in-spain-a-nationwide-perspective
#14
Francisco Bautista, Soledad Gallego, Adela Cañete, Jaume Mora, Cristina Díaz de Heredia, Ofelia Cruz, José María Fernández, Susana Rives, Pablo Berlanga, Raquel Hladun, Antonio Juan Ribelles, Luis Madero, Manuel Ramírez, Rafael Fernández Delgado, Antonio Pérez-Martínez, Cristina Mata, Anna Llort, Javier Martín Broto, María Elena Cela, Gema Ramírez, Constantino Sábado, Tomás Acha, Itziar Astigarraga, Ana Sastre, Ascensión Muñoz, Mercedes Guibelalde, Lucas Moreno
INTRODUCTION: Cancer is the leading cause of death between the first year of life and adolescence, and some types of diseases are still a major challenge in terms of cure. There is, therefore, a major need for new drugs. Recent findings in cancer biology open the door to the development of targeted therapies against individual molecular changes, as well as immunotherapy. Promising results in adult anti-cancer drug development have not yet been translated into paediatric clinical practice...
March 6, 2017: Anales de Pediatría: Publicación Oficial de la Asociación Española de Pediatría (A.E.P.)
https://www.readbyqxmd.com/read/28220744/burden-genotype-and-phenotype-profiles-of-adult-patients-with-sickle-cell-disease-in-cape-town-south-africa
#15
G D Pule, K Mnica, M Joubert, S Mowla, N Novitsky, A Wonkam
BACKGROUND: An exponential increase in the number of sickle cell disease (SCD) patients in paediatric services in Cape Town, South Africa, has been reported. The trend in adult/adolescent services has not been investigated. OBJECTIVES: To evaluate epidemiological trends of SCD and the profile of patients affected by SCD attending the Haematology Clinic at Groote Schuur Hospital (GSH), Cape Town. METHODS: (i) A retrospective review of the number of SCD patients over the past 20 years; (ii) a cross-sectional analysis of clinical and haematological characteristics of SCD patients; and (iii) molecular analysis of the haemoglobin S mutation, the haplotype in the β-globin-like genes cluster, the 3...
January 30, 2017: South African Medical Journal, Suid-Afrikaanse Tydskrif Vir Geneeskunde
https://www.readbyqxmd.com/read/28208985/pattern-of-inpatient-dermatology-consultations-in-a-tertiary-care-centre-from-northern-india
#16
Abhilasha Williams, Anuradha Bhatia, Bimal Kanish, Paulina Rose Chaudhary, Clarence James Samuel
INTRODUCTION: The importance of dermatology inpatient consultation is of significant importance yet is not well documented. AIM: To retrospectively analyse the spectrum of dermatology inpatient consultations from the various departments and to document the most commonly occurring dermatological disorders from each speciality. MATERIALS AND METHODS: This study was done in an 800 bedded premier teaching hospital in Ludhiana, Punjab, North Western India...
December 2016: Journal of Clinical and Diagnostic Research: JCDR
https://www.readbyqxmd.com/read/28164435/childhood-neoplasms-presenting-at-autopsy-a-20-year-experience
#17
Victoria A Bryant, John Booth, Liina Palm, Michael Ashworth, Thomas S Jacques, Neil J Sebire
OBJECTIVE: The aims of the review are to establish the number of undiagnosed neoplasms presenting at autopsy in a single centre and to determine the incidence and most common causes of sudden unexpected death due to neoplasia in infancy and childhood (SUDNIC). DESIGN: Retrospective observational study of paediatric autopsies performed on behalf of Her Majesty's Coroner over a 20-year period (1996-2015; n = 2,432). Neoplasms first diagnosed at autopsy were identified from an established database and cases meeting the criteria for sudden unexpected death were further categorised...
February 6, 2017: Pediatric Blood & Cancer
https://www.readbyqxmd.com/read/28151390/diagnosis-and-management-of-newly-diagnosed-childhood-autoimmune-haemolytic-anaemia-recommendations-from-the-red-cell-study-group-of-the-paediatric-haemato-oncology-italian-association
#18
Saverio Ladogana, Matteo Maruzzi, Piera Samperi, Silverio Perrotta, Giovanni C Del Vecchio, Lucia D Notarangelo, Piero Farruggia, Federico Verzegnassi, Nicoletta Masera, Paola Saracco, Silvia Fasoli, Maurizio Miano, Gabriella Girelli, Wilma Barcellini, Alberto Zanella, Giovanna Russo
Autoimmune haemolytic anaemia is an uncommon disorder to which paediatric haematology centres take a variety of diagnostic and therapeutic approaches. The Red Cell Working Group of the Italian Association of Paediatric Onco-haematology (Associazione Italiana di Ematologia ed Oncologia Pediatrica, AIEOP) developed this document in order to collate expert opinions on the management of newly diagnosed childhood autoimmune haemolytic anaemia.The diagnostic process includes the direct and indirect antiglobulin tests; recommendations are given regarding further diagnostic tests, specifically in the cases that the direct and indirect antiglobulin tests are negative...
December 16, 2016: Blood Transfusion, Trasfusione del Sangue
https://www.readbyqxmd.com/read/28074473/predicting-cd4-t-cell-reconstitution-following-paediatric-haematopoietic-stem-cell-transplantation
#19
Rollo L Hoare, Paul Veys, Nigel Klein, Robin Callard, Joseph F Standing
Haematopoietic stem cell transplantation is an increasingly common treatment for children with a range of haematological disorders. Conditioning with cytotoxic chemotherapy and total body irradiation leaves patients severely immunocompromised. T-cell reconstitution can take several years due to delayed restoration of thymic output. Understanding T-cell reconstitution in children is complicated by normal immune system maturation, heterogeneous diagnoses, and sparse uneven sampling due to the long time spans involved...
January 11, 2017: Clinical Pharmacology and Therapeutics
https://www.readbyqxmd.com/read/28056911/prospective-cohort-study-of-procalcitonin-levels-in-children-with-cancer-presenting-with-febrile-neutropenia
#20
Victoria Hemming, Adam D Jakes, Geoff Shenton, Bob Phillips
BACKGROUND: Febrile neutropenia (FNP) causes significant morbidity and mortality in children undergoing treatment for cancer. The development of clinical decision rules to help stratify risks in paediatric FNP patients and the use of inflammatory biomarkers to identify high risk patients is an area of recent research. This study aimed to assess if procalcitonin (PCT) levels could be used to help diagnose or exclude severe infection in children with cancer who present with febrile neutropenia, both as a single measurement and in addition to previously developed clinical decision rules...
January 5, 2017: BMC Pediatrics
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