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Paediatric Haematology

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https://www.readbyqxmd.com/read/28220744/burden-genotype-and-phenotype-profiles-of-adult-patients-with-sickle-cell-disease-in-cape-town-south-africa
#1
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
#2
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
#3
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
#4
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
#5
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
#6
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
#7
(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...
December 27, 2016: 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
#8
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...
December 22, 2016: International Journal of Laboratory Hematology
https://www.readbyqxmd.com/read/28003896/laparoscopic-splenectomy-alone-for-sickle-cell-disease-account-of-50-paediatric-cases
#9
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
#10
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
#11
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
#12
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...
February 2017: 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
#13
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...
January 2017: 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
#14
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
#15
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
#16
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
#17
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
#18
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
#19
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
#20
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
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