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Paediatric Haematology

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
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
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
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
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
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
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
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
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...
2016: BMC Neurology
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
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), and Google were performed and combined fungal disease (Fung* OR antifung*OR Candida* OR Aspergill*) with prophylaxis or treatment (prophyl* OR therap* OR treatment)...
September 11, 2016: Supportive Care in Cancer: Official Journal of the Multinational Association of Supportive Care in Cancer
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
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
Neyssa M Marina, Sigbjørn Smeland, Stefan S Bielack, Mark Bernstein, Gordana Jovic, Mark D Krailo, Jane M Hook, Carola Arndt, Henk van den Berg, Bernadette Brennan, Bénédicte Brichard, Ken L B Brown, Trude Butterfass-Bahloul, Gabriele Calaminus, Heike E Daldrup-Link, Mikael Eriksson, Mark C Gebhardt, Hans Gelderblom, Joachim Gerss, Robert Goldsby, Allen Goorin, Richard Gorlick, Holcombe E Grier, Juliet P Hale, Kirsten Sundby Hall, Jendrik Hardes, Douglas S Hawkins, Knut Helmke, Pancras C W Hogendoorn, Michael S Isakoff, Katherine A Janeway, Heribert Jürgens, Leo Kager, Thomas Kühne, Ching C Lau, Patrick J Leavey, Stephen L Lessnick, Leo Mascarenhas, Paul A Meyers, Hubert Mottl, Michaela Nathrath, Zsuzsanna Papai, R Lor Randall, Peter Reichardt, Marleen Renard, Akmal Ahmed Safwat, Cindy L Schwartz, Michael C G Stevens, Sandra J Strauss, Lisa Teot, Mathias Werner, Matthew R Sydes, Jeremy S Whelan
BACKGROUND: We designed the EURAMOS-1 trial to investigate whether intensified postoperative chemotherapy for patients whose tumour showed a poor response to preoperative chemotherapy (≥10% viable tumour) improved event-free survival in patients with high-grade osteosarcoma. METHODS: EURAMOS-1 was an open-label, international, phase 3 randomised, controlled trial. Consenting patients with newly diagnosed, resectable, high-grade osteosarcoma aged 40 years or younger were eligible for randomisation...
October 2016: Lancet Oncology
Carlo Caffarelli, Francesca Santamaria, Dora Di Mauro, Carla Mastrorilli, Virginia Mirra, Sergio Bernasconi
This review focuses key advances in different pediatric fields that were published in Italian Journal of Pediatrics and in international journals in 2015. Weaning studies continue to show promise for preventing food allergy. New diagnostic tools are available for identifying the allergic origin of allergic-like symptoms. Advances have been reported in obesity, short stature and autoimmune endocrine disorders. New molecules are offered to reduce weight gain and insulin-resistance in obese children. Regional investigations may provide suggestions for preventing short stature...
August 27, 2016: Italian Journal of Pediatrics
Shivam Agarwal, Vijay Jaiswal, Dharamveer Singh, Prateek Jaiswal, Amit Garg, Amit Upadhyay
AIM: Placental redistribution has been shown to improve haematological outcomes in the immediate neonatal period and early infancy. This study compared the effects of delayed cord clamping (DCC) and umbilical cord milking (UCM) on haematological and growth parameters at 12 months of age. METHODS: This was a follow-up study of a randomised control trial, conducted in a tertiary care paediatric centre from August 2013 to August 2014. We studied 200 apparently healthy Indian infants randomised at birth to receive DCC for 60-90 seconds or UCM...
November 2016: Acta Paediatrica
Umit Uksal, Pinar Ozturk, Emine Colgecen, Nazan Taslidere, Turkan Patiroglu, Mehmet Akif Ozdemir, Yasemin Altuner Torun, Murat Borlu
OBJECTIVE: Diagnoses of skin, mucosae, hair and nail manifestations in malignant diseases are often challenging because of life-threatening drug reactions, opportunistic infections or skin involvement of primary processes. Description of morphology, configuration and distribution of lesions is important in order to differentiate the self-healing eruptions from serious side effects of chemotherapy. There are case reports from Turkey including dermatological manifestations of malignancies and case series in adult patients but there are no published large group studies assessing all manifestations in children...
June 2016: Eurasian Journal of Medicine
Diane Murray, Cathy Sedgeworth, Moira Kinnear, Lesley Diack
AIM: To gather opinions from doctors and pharmacists to improve the design of the PICU MR form generated by the electronic prescribing and clinical notes system to support transfer of care from PICU to downstream wards that use paper systems. METHOD: A purposive sample of 10 forms covering a comprehensive range of medication information common to PICU patients was selected from practice between March 2014 and May 2014. Pharmacists (n=7) and doctors (n=9) who received these forms on downstream wards were invited to participate in semi-structured one-to-one interviews (n=20) with the PICU pharmacist within 48 hrs of receipt to explore their views about the form...
September 2016: Archives of Disease in Childhood
Rachel Greer, Sally Harvey
AIM: Clarity and accuracy of the pharmacy aspects of cancer clinical trial protocols is essential. Inconsistencies and ambiguities in protocols have the potential to delay research and jeopardise both patient safety and the collection of credible data. The Chemotherapy and Pharmacy Advisory Service (CPAS) was established in 2007 by the UK National Cancer Research Network, currently known as National Institute for Health Research Clinical Research Network, to improve the quality of pharmacy-related content in cancer clinical trial protocols...
September 2016: Archives of Disease in Childhood
Hirminder Ubhi, Mitul Patel, Lisa Ludwig
AIM: Aims of the study included evaluation of outpatient antibiotic prescribing practices relating to good Antimicrobial Stewardship1 focusing on documentation of indication and duration. METHOD: Prescriptions dispensed at the Outpatient Pharmacy over a 6 week were evaluated. Data collected include patient's hospital identification in full, the antibiotic prescribed, duration of treatment, indication (in a specifically allocated area on the prescription) and route of administration, prescriber speciality and grade...
September 2016: Archives of Disease in Childhood
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