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Paediatric Haematology

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https://www.readbyqxmd.com/read/29769501/thyroid-status-in-children-with-transfusion-dependent-hb-e-%C3%AE-thalassaemia
#1
T Sharmin, A H Mollah, A A Morshed, M K Chowdhury
Despite improved haematological care, multi-endocrine dysfunction is a common complication in thalassemia. Iron overload is thought to be the most likely mechanism in thyroid dysfunction in these patients. Moreover, chronic tissue hypoxia might havedirect toxic effect on thyroid gland resulting in hypothyroidism. This study was designed to evaluate the thyroid status of children with Hb-E β-thalassemia. This cross sectional analytic study was conducted among thepatients with Hb-E β-thalassemia attending both in-patient & out-patient department of Paediatrics, Dhaka Medical College & Hospital, Dhaka, Bangladesh from April 2012 to March 2013...
April 2018: Mymensingh Medical Journal: MMJ
https://www.readbyqxmd.com/read/29724741/identifying-children-with-hereditary-coagulation-disorders-ichec-a-protocol-for-a-prospective-cohort-study
#2
Eva Stokhuijzen, Margaret L Rand, Marjon H Cnossen, Tina T Biss, Paula D James, Monique H Suijker, Marjolein Peters, Johanna H van der Lee, Bram Peters, Alexander B Meijer, Victor S Blanchette, Karin Fijnvandraat
INTRODUCTION: It is challenging to obtain a reliable bleeding history in children who are referred for a suspected inherited bleeding disorder. Bleeding symptoms may be subtle as children face fewer haemostatic challenges compared with adults. In order to standardise bleeding histories, questionnaires have been developed, called bleeding assessment tools (BATs). Although it has been shown that high bleeding scores are associated with the presence of a mucocutaneous bleeding disorder, these BATs lack sensitivity, efficiency and flexibility in the paediatric setting...
May 3, 2018: BMJ Open
https://www.readbyqxmd.com/read/29706495/effect-of-red-blood-cell-storage-duration-on-outcome-after-paediatric-cardiac-surgery-a-prospective-observational-study
#3
Arvind Kumar Bishnoi, Pankaj Garg, Kartik Patel, Chandrasekaran Ananthanarayanan, Ritesh Shah, Atul Solanki, Himani Pandya, Sanjay Patel
BACKGROUND: Retrospective reviews have found the use of stored packed red blood cells (PRBCs) in priming to be associated with increased risk of postoperative complications. The purpose of study was to prospectively investigate the influence of duration of storage of PRBCs used in priming the cardiopulmonary bypass (CPB) circuit on the metabolic profile of the patients, and postoperative outcome after paediatric cardiac surgery. METHODS: Between January 2015 and December 2015, 198 consecutive children operated for cardiac surgery using CPB and received blood for priming the circuit were included...
March 31, 2018: Heart, Lung & Circulation
https://www.readbyqxmd.com/read/29681513/antimicrobial-consumption-and-resistance-in-adult-hospital-inpatients-in-53-countries-results-of-an-internet-based-global-point-prevalence-survey
#4
Ann Versporten, Peter Zarb, Isabelle Caniaux, Marie-Françoise Gros, Nico Drapier, Mark Miller, Vincent Jarlier, Dilip Nathwani, Herman Goossens
BACKGROUND: The Global Point Prevalence Survey (Global-PPS) established an international network of hospitals to measure antimicrobial prescribing and resistance worldwide. We aimed to assess antimicrobial prescribing and resistance in hospital inpatients. METHODS: We used a standardised surveillance method to collect detailed data about antimicrobial prescribing and resistance from hospitals worldwide, which were grouped by UN region. The internet-based survey included all inpatients (adults, children, and neonates) receiving an antimicrobial who were on the ward at 0800 h on one specific day between January and September, 2015...
April 19, 2018: Lancet Global Health
https://www.readbyqxmd.com/read/29672778/fear-of-progression-in-parents-of-children-with-cancer-results-of-an-online-expert-survey-in-pediatric-oncology
#5
Katharina Clever, Florian Schepper, Luise Küpper, Holger Christiansen, Julia Martini
BACKGROUND: Fear of Progression (FoP) is a commonly reported psychological strain in parents of children with cancer. This expert survey investigates how professionals in pediatric oncology estimate the burden and consequences of FoP in parents and how they assess and treat parental FoP. METHOD: N=77 professionals in pediatric oncology (members and associates of the Psychosocial Association in Paediatric Oncology and Haematology, PSAPOH) were examined in an online survey with a self-developed questionnaire...
April 19, 2018: Klinische Pädiatrie
https://www.readbyqxmd.com/read/29610659/survival-is-influenced-by-approaches-to-local-treatment-of-ewing-sarcoma-within-an-international-randomised-controlled-trial-analysis-of-eicess-92
#6
Jeremy Whelan, Allan Hackshaw, Anne McTiernan, Robert Grimer, David Spooner, Jessica Bate, Andreas Ranft, Michael Paulussen, Herbert Juergens, Alan Craft, Ian Lewis
Background: Two national clinical trial groups, United Kingdom Children's Cancer and Leukaemia Group (CCLG) and the German Paediatric Oncology and Haematology Group (GPOH) together undertook a randomised trial, EICESS-92, which addressed chemotherapy options for Ewing's sarcoma. We sought the causes of unexpected survival differences between the study groups. Methods: 647 patients were randomised. Cox regression analyses were used to compare event-free survival (EFS) and overall survival (OS) between the two study groups...
2018: Clinical Sarcoma Research
https://www.readbyqxmd.com/read/29582148/assessment-of-dialysis-adequacy-beyond-urea-kinetic-measurements
#7
Lesley Rees
Adequacy of dialysis is a term that has been used for many years based on measurement of small solute clearance using urea and creatinine. This has been shown in some but not all studies in adults to correlate with survival. However, small solute clearance is just one minor part of the effectiveness of dialysis and in fact 'optimum' dialysis, rather than 'adequate' dialysis is what most paediatric nephrologists would want for their patients. Additional ways to assess the success of dialysis in children would include dialysis access complications and longevity, preservation of residual kidney function, body composition, biochemical and haematological control, nutrition and growth, discomfort during the dialysis process and psychosocial adjustment including hospitalisation and school attendance...
March 26, 2018: Pediatric Nephrology: Journal of the International Pediatric Nephrology Association
https://www.readbyqxmd.com/read/29578274/desmopressin-stimulation-testing-response-to-intravenous-and-intranasal-forms
#8
M C McCormick, N Siripong, J D Cooper
INTRODUCTION: Desmopressin is commonly used to reduce bleeding in patients with mucocutaneous bleeding disorders and is available in both intravenous and intranasal forms. Given the variability in response to desmopressin at an individual level, its effectiveness should be assessed with a test dose prior to being advised for use. At this time, no trial has extensively compared the use of intranasal desmopressin to intravenous desmopressin. AIMS: To determine whether both forms of desmopressin are equally effective in yielding a positive response in laboratory assays in paediatric patients with von Willebrand disease or probable von Willebrand disease...
March 26, 2018: Haemophilia: the Official Journal of the World Federation of Hemophilia
https://www.readbyqxmd.com/read/29568847/haematologist-reviewed-peripheral-blood-smear-in-paediatric-practice
#9
Anselm Chi-Wai Lee
Manual examination of the peripheral blood smear (PBS) is currently performed on a fraction of samples sent for automated complete cell count. 39 children (age range 0-16.2 years) referred to a private paediatric practice during a 16-month period were retrospectively reviewed. Clinical scenarios, haematological features, laboratory-initiated PBS review, haematologist's PBS review and final diagnosis were described. Clinical indications included isolated thrombocytopenia (n = 10), unexplained bruises (n = 5), acute febrile illnesses (n = 11), anaemia (n = 8) and others (n = 5)...
February 2018: Singapore Medical Journal
https://www.readbyqxmd.com/read/29550751/how-to-use%C3%A2-a-blood-film
#10
Ben McNaughten, Anthony Thompson, Christine Macartney, Andrew Thompson
The diagnostic relevance of the blood film cannot be underestimated in the assessment of children with suspected primary or secondary haematological conditions. The blood film not only serves as a diagnostic tool but also allows for screening, monitoring of disease progression and therapeutic response in children with a variety of haematological conditions. This article outlines the appearance of normal paediatric and neonatal blood films. The technical aspects involved in preparing a blood film are discussed...
March 17, 2018: Archives of Disease in Childhood. Education and Practice Edition
https://www.readbyqxmd.com/read/29500934/genotype-and-allele-frequencies-of-tyms-rs2790-a-g-polymorphism-in-a-chinese-paediatric-population-with-acute-lymphoblastic-leukaemia
#11
S-M Wang, W-X Zeng, W-S Wu, L-L Sun, D Yan
WHAT IS KNOWN AND OBJECTIVE: Thymidylate synthase (TYMS) is an important target for methotrexate (MTX). Genetic variations in the TYMS gene contribute to the differences in treatment responses to MTX. The aim of this study was to investigate the distribution of a microRNA (miRNA) binding site polymorphism (rs2790 A > G) in the 3'-untranslated region (3'-UTR) of TYMS and its association with MTX concentration and haematological toxicity in Chinese paediatric patients with acute lymphoblastic leukaemia (ALL)...
March 3, 2018: Journal of Clinical Pharmacy and Therapeutics
https://www.readbyqxmd.com/read/29480574/recurrence-of-ewing-sarcoma-is-detection-by-imaging-follow-up-protocol-associated-with-survival-advantage
#12
Melina Heinemann, Andreas Ranft, Thorsten Langer, Herbert Jürgens, Justus Kreyer, Volker Vieth, Michael Schäfers, Matthias Weckesser, Thorsten Simon, Wolf Hassenpflug, Selim Corbacioglu, Stefan Bielack, Regina Mayer-Steinacker, Thomas Kühne, Henk van den Berg, Hans Gelderblom, Sebastian Bauer, Lars Stegger, Uta Dirksen
BACKGROUND: The Cooperative Ewing Sarcoma Study and the Late Effects Surveillance System of the Society for Paediatric Oncology and Haematology recommend a structured follow-up imaging protocol (FUIP) for patients with Ewing sarcoma (EwS) with decreasing frequency of imaging over the first 5 years. The present study aims to assess the effectiveness of the FUIP for EwS patients regarding survival after relapse. PATIENTS AND METHODS: A retrospective multicenter analysis on 160 eligible patients with EwS recurrence was performed...
February 26, 2018: Pediatric Blood & Cancer
https://www.readbyqxmd.com/read/29471850/taliglucerase-alfa-safety-and-efficacy-across-6-clinical-studies-in-adults-and-children-with-gaucher-disease
#13
REVIEW
Ari Zimran, Michael Wajnrajch, Betina Hernandez, Gregory M Pastores
Taliglucerase alfa is an enzyme replacement therapy (ERT) approved for treatment of adult and paediatric patients with Type 1 Gaucher disease (GD) in several countries and the first plant cell-expressed recombinant therapeutic protein approved by the US Food and Drug Administration for humans. Here, we review the findings across six key taliglucerase alfa clinical studies. A total of 33 treatment-naïve adult patients were randomized to taliglucerase alfa 30 U/kg or 60 U/kg in a 9-month, multicentre, randomized, double-blind, parallel-group, dose-comparison pivotal study, after which eligible patients continued into two consecutive extension studies; 17 treatment-naïve adult patients completed 5 total years of treatment with taliglucerase alfa...
February 23, 2018: Orphanet Journal of Rare Diseases
https://www.readbyqxmd.com/read/29432826/usefulness-of-%C3%A3-d-glucan-for-diagnosis-and-follow-up-of-invasive-candidiasis-in-onco-haematological-patients
#14
J Guitard, F Isnard, M-D Tabone, M Antignac, E Brissot, Y Senghor, A Petit, G Leverger, C Hennequin
OBJECTIVES: Definitive diagnosis of invasive candidiasis (IC) may be difficult to achieve in patients with haematological malignancy (PHM). We aimed to evaluate the performance of BDG for the diagnosis and the follow-up of IC in PHM. PATIENTS AND METHODS: We retrospectively reviewed the serological data of BDG assay in adult and paediatric PHM, who developed candidemia or chronic disseminated candidiasis (CDC) through a 4-year period. Sensitivity and kinetics of BDG were determined for both clinical forms...
May 2018: Journal of Infection
https://www.readbyqxmd.com/read/29384118/paediatric-case-series-of-drug-reaction-with-eosinophilia-and-systemic-symptoms-dress-12-year-experience-at-a-single-referral-centre-in-hong-kong-and-the-first-reported-use-of-infliximab
#15
G T Chua, J S Rosa Duque, P C Y Chong, P P W Lee, Y L Lau, M H K Ho
DRESS (drug reaction with eosinophilia and systemic symptoms) is a rare but potentially life-threatening disorder characterized by fever, skin eruption, haematological abnormalities and multi-organ dysfunction after drug exposure. The pathophysiology is thought to be related to interactions between culprit drugs, viral reactivation and T-lymphocytes activation. We report 4 paediatric patients with DRESS who were treated at our centre over the past 12 years. Most cases improved after corticosteroids. Other immunosuppressive medications were attempted in refractory cases with varied outcomes...
November 30, 2017: European Annals of Allergy and Clinical Immunology
https://www.readbyqxmd.com/read/29333497/work-efficiency-improvement-of-90-after-implementation-of-an-annual-inpatient-blood-products-administration-consent-form
#16
Holly Lindsay, Saleh Bhar, Challice Bonifant, Sarah Sartain, Sarah B Whittle, Youngna Lee-Kim, Mona D Shah
Paediatric haematology, oncology and bone marrow transplant (BMT) patients frequently require transfusion of blood products. Our institution required a new transfusion consent be obtained every admission. The objectives of this project were to: revise inpatient blood products consent form to be valid for 1 year, decrease provider time spent consenting from 15 to <5 min per admission, and improve provider frustration with the consent process. Over 6 months, we determined the average number of hospitalisations requiring transfusions in a random sampling of haematology/oncology/BMT inpatients...
2018: BMJ Open Quality
https://www.readbyqxmd.com/read/29313207/survey-on-paediatric-tumour-boards-in-europe-current-situation-and-results-from-the-expo-r-net-project
#17
A Juan Ribelles, P Berlanga, G Schreier, M Nitzlnader, B Brunmair, V Castel, S Essiaf, A Cañete, R Ladenstein
BACKGROUND: Under the ExPO-r-NeT project (European Expert Paediatric Oncology Reference Network for Diagnostics and Treatment), we aimed to identify paediatric oncology tumour boards in Europe to investigate the kind of technologies and logistics that are in place in different countries and to explore current differences between regions. METHODS: A 20-question survey regarding several features of tumor boards was designed. Data collected included infrastructure, organization, and clinical decision-making information from the centres...
January 8, 2018: Clinical & Translational Oncology
https://www.readbyqxmd.com/read/29273020/trends-in-cause-and-place-of-death-for-children-in-portugal-a-european-country-with-no-paediatric-palliative-care-during-1987-2011-a-population-based-study
#18
Ana Forjaz de Lacerda, Barbara Gomes
BACKGROUND: Children and adolescents dying from complex chronic conditions require paediatric palliative care. One aim of palliative care is to enable a home death if desired and well supported. However, there is little data to inform care, particularly from countries without paediatric palliative care, which constitute the majority worldwide. METHODS: This is an epidemiological study analysing death certificate data of decedents aged between 0 and 17 years in Portugal, a developed Western European country without recognised provision of paediatric palliative care, from 1987 to 2011...
December 22, 2017: BMC Pediatrics
https://www.readbyqxmd.com/read/29230958/treatment-related-mortality-in-relapsed-childhood-acute-lymphoblastic-leukemia
#19
Trausti Oskarsson, Stefan Söderhäll, Johan Arvidson, Erik Forestier, Thomas Leth Frandsen, Marit Hellebostad, Päivi Lähteenmäki, Ólafur G Jónsson, Ida Hed Myrberg, Mats Heyman
BACKGROUND: Treatment of relapsed childhood acute lymphoblastic leukemia (ALL) is particularly challenging due to the high treatment intensity needed to induce and sustain a second remission. To improve results, it is important to understand how treatment-related toxicity impacts survival. PROCEDURE: In this retrospective population-based study, we described the causes of death and estimated the risk for treatment-related mortality in patients with first relapse of childhood ALL in the Nordic Society of Paediatric Haematology and Oncology ALL-92 and ALL-2000 trials...
April 2018: Pediatric Blood & Cancer
https://www.readbyqxmd.com/read/29205259/cd34-selected-stem-cell-boosts-can-improve-poor-graft-function-after-paediatric-allogeneic-stem-cell-transplantation
#20
Chiara Mainardi, Martin Ebinger, Sigrid Enkel, Tobias Feuchtinger, Heiko-Manuel Teltschik, Matthias Eyrich, Michael Schumm, Armin Rabsteyn, Patrick Schlegel, Christian Seitz, Carl-Phillip Schwarze, Ingo Müller, Johann Greil, Peter Bader, Paul-Gerhardt Schlegel, David Martin, Ursula Holzer, Michaela Döring, Rupert Handgretinger, Peter Lang
Poor graft function (PGF) is a severe complication of haematopoietic stem cell transplantation (HSCT) and administration of donor stem cell boosts (SCBs) represents a therapeutic option. We report 50 paediatric patients with PGF who received 61 boosts with CD34+ selected peripheral blood stem cells (PBSC) after transplantation from matched unrelated (n = 25) or mismatched related (n = 25) donors. Within 8 weeks, a significant increase in median neutrophil counts (0·6 vs. 1·516 × 109 /l, P < 0·05) and a decrease in red blood cell and platelet transfusion requirement (median frequencies 1 and 7 vs...
January 2018: British Journal of Haematology
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