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Paediatric Haematology

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https://www.readbyqxmd.com/read/28439893/outcome-after-intensive-reinduction-therapy-and-allogeneic-stem-cell-transplant-in-paediatric-relapsed-acute-myeloid-leukaemia
#1
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
#2
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
#3
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
#4
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...
March 22, 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
#5
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
#6
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
#7
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
#8
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
#9
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
#10
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
#11
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
#12
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
#13
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
#14
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
#15
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
https://www.readbyqxmd.com/read/28028587/safety-of-zidovudine-lamivudine-scored-tablets-in-children-with-hiv-infection-in-europe-and-thailand
#16
(no author information available yet)
BACKGROUND: Zidovudine (ZDV) has been associated with risk of haematological toxicity. Safety data from clinical trials is generally limited to 48 weeks. We assessed the short- and mid-term toxicity of ZDV/lamivudine (3TC) fixed-dose combination scored tablets in HIV-infected children followed in the European Pregnancy and Paediatric HIV Cohort Collaboration (EPPICC) network. METHODS: Fourteen cohorts provided data on patients <18 years of age taking ZDV/3TC scored tablets between 2008 and 2012...
April 2017: European Journal of Clinical Pharmacology
https://www.readbyqxmd.com/read/28004528/minimal-residual-disease-monitoring-in-childhood-b-lymphoblastic-leukemia-with-t-12-21-p13-q22-etv6-runx1-concordant-results-using-quantitation-of-fusion-transcript-and-flow-cytometry
#17
S J Alm, C Engvall, J Asp, L Palmqvist, J Abrahamsson, L Fogelstrand
INTRODUCTION: The translocation t(12;21)(p13;q22) resulting in the fusion gene ETV6-RUNX1, is the most frequent gene fusion in childhood B lymphoblastic leukemia. In the Nordic Society of Paediatric Haematology and Oncology ALL-2008 treatment protocol, treatment stratification in B-lineage ALL is based on results of minimal residual disease (MRD) analysis with fluorescence-activated cell sorting (FACS). In this study, we determined whether RT-qPCR of the ETV6-RUNX1 fusion transcript can be a reliable alternative for MRD analysis...
April 2017: International Journal of Laboratory Hematology
https://www.readbyqxmd.com/read/28003896/laparoscopic-splenectomy-alone-for-sickle-cell-disease-account-of-50-paediatric-cases
#18
Zainab N Al-Balushi, Khalid M Bhatti, Muhammad T Ehsan, Yousuf Al-Shaqsi, Nawal A R Al-Sharji, Hatem A A Mady, Mahmoud H Sherif
OBJECTIVES: In Oman, the most frequent indication for a splenectomy in children is sickle cell disease (SCD), which is one of the most common haematological disorders in the Gulf region. This study aimed to describe paediatric laparoscopic splenectomies alone for SCD at a tertiary hospital in Oman. METHODS: This study was conducted between February 2010 and October 2015 at the Sultan Qaboos University Hospital, Muscat, Oman. The medical records of all children aged ≤15 years old undergoing splenectomies during the study period were reviewed...
November 2016: Sultan Qaboos University Medical Journal
https://www.readbyqxmd.com/read/28001297/impact-of-platelet-pathogen-inactivation-on-blood-component-utilization-and-patient-safety-in-a-large-austrian-regional-medical-centre
#19
M Amato, H Schennach, M Astl, C Y Chen, J-S Lin, R J Benjamin, W Nussbaumer
BACKGROUND: In clinical studies, pathogen inactivation (PI) of platelet concentrates (PC) with amotosalen and UVA light did not impact patient risk for haemorrhage but may affect transfusion frequency and component utilization. We evaluated the influence of platelet PI on PC, red cell concentrate (RCC) and plasma use and safety in routine practice in a large regional hospital. STUDY DESIGN AND METHODS: Comparative effectiveness of conventional vs. PI-treated PC was analysed during two 21-month periods, before and after PI implementation...
January 2017: Vox Sanguinis
https://www.readbyqxmd.com/read/27891440/radiation-therapy-in-paediatric-orbital-granulocytic-sarcomas-experience-from-a-tertiary-cancer-center
#20
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
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