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Paediatric malignancy

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https://www.readbyqxmd.com/read/29680797/xp11-translocation-renal-cell-carcinoma-paraneoplastic-syndrome-presenting-as-cutaneous-vasculitis-first-reported-case-of-yet-another-mask
#1
Claire Pascoe, Daniel Christidis, David Clouston, Nathan Lawrentschuk
Renal cell carcinoma is historically known as the 'great masquerader' with 40% of patients experiencing a paraneoplastic syndrome. Translocation carcinoma represents one-third of renal cancer in paediatric patients but less than 3% of renal cancers in patients aged 18-45 years where the clinical course is often rapidly terminal. There are less than 10 reported cases of leucoclastic vasculitis associated with clear cell carcinoma reported in the literature and 10 case reports of translocation carcinoma in adults...
April 21, 2018: BMJ Case Reports
https://www.readbyqxmd.com/read/29672904/growth-hormone-gh-insulin-like-growth-factor-1-igf-1-axis-hyperactivity-on-bone-fibrous-dysplasia-in-mccune-albright-syndrome
#2
Daniele Tessaris, Alison M Boyce, Margaret Zacharin, Patrizia Matarazzo, Roberto Lala, Luisa de Sanctis, Michael T Collins
CONTEXT: In fibrous dysplasia (BFD) normal bone and bone marrow are replaced by fibro-osseous tissue, leading to fracture, deformity and pain. BFD may be isolated, or in association with cutaneous hyperpigmentation and/or hyperfunctioning endocrinopathies, termed McCune-Albright syndrome (MAS). GH hypersecretion has been described in 10-20% of MAS-BFD patients. Aim of the study is to determine the impact of GH-insulin like growth factor 1 (IGF1) axis hyperactivity on MAS-BFD morbidities and the efficacy of GH excess therapy...
April 19, 2018: Clinical Endocrinology
https://www.readbyqxmd.com/read/29666178/causes-of-death-in-a-contemporary-adult-congenital-heart-disease-cohort
#3
Christopher Yu, Benjamin M Moore, Irina Kotchetkova, Rachael L Cordina, David S Celermajer
OBJECTIVE: The life expectancy of patients with congenital heart disease (CHD) has significantly improved with advances in their paediatric medical care. Mortality patterns are changing as a result. Our study aims to describe survival and causes of death in a contemporary cohort of adult patients with CHD. METHODS: We reviewed 3068 patients in our adult CHD database (age ≥16 years, seen at least once in our centre between 2000 and 2015), and documented the number and causes of death, via Australia's National Death Index...
April 17, 2018: Heart: Official Journal of the British Cardiac Society
https://www.readbyqxmd.com/read/29623582/childhood-and-adolescent-lymphoma-in-spain-incidence-and-survival-trends-over-20-years
#4
R Marcos-Gragera, M Solans, J Galceran, R Fernández-Delgado, A Fernández-Teijeiro, A Mateos, J R Quirós-Garcia, N Fuster-Camarena, V De Castro, M J Sánchez, P Franch, M D Chirlaque, E Ardanaz, C Martos, D Salmerón, R Peris-Bonet
BACKGROUND: Lymphoma is the third most common malignancy in children (0-14 years) and the first in adolescents (15-19 years). This population-based study-the largest ever done in Spain-analyses incidence and survival of lymphomas among Spanish children and adolescents. PATIENTS AND METHODS: 1664 lymphoma cases (1983-2007) for incidence and 1030 for survival (1991-2005) followed until 31/12/2010, were provided by 11 cancer registries. Age-adjusted incidence rates (ASRw) to the world standard population were obtained; incidence trends were modelled using the Joinpoint programme, observed survival (OS) was estimated with Kaplan-Meier and trends tested with a log-rank test...
April 5, 2018: Clinical & Translational Oncology
https://www.readbyqxmd.com/read/29606586/larotrectinib-for-paediatric-solid-tumours-harbouring-ntrk-gene-fusions-phase-1-results-from-a-multicentre-open-label-phase-1-2-study
#5
Theodore W Laetsch, Steven G DuBois, Leo Mascarenhas, Brian Turpin, Noah Federman, Catherine M Albert, Ramamoorthy Nagasubramanian, Jessica L Davis, Erin Rudzinski, Angela M Feraco, Brian B Tuch, Kevin T Ebata, Mark Reynolds, Steven Smith, Scott Cruickshank, Michael C Cox, Alberto S Pappo, Douglas S Hawkins
BACKGROUND: Gene fusions involving NTRK1, NTRK2, or NTRK3 (TRK fusions) are found in a broad range of paediatric and adult malignancies. Larotrectinib, a highly selective small-molecule inhibitor of the TRK kinases, had shown activity in preclinical models and in adults with tumours harbouring TRK fusions. This study aimed to assess the safety of larotrectinib in paediatric patients. METHODS: This multicentre, open-label, phase 1/2 study was done at eight sites in the USA and enrolled infants, children, and adolescents aged 1 month to 21 years with locally advanced or metastatic solid tumours or CNS tumours that had relapsed, progressed, or were non-responsive to available therapies regardless of TRK fusion status; had a Karnofsky (≥16 years of age) or Lansky (<16 years of age) performance status score of 50 or more, adequate organ function, and full recovery from the acute toxic effects of all previous anticancer therapy...
March 29, 2018: Lancet Oncology
https://www.readbyqxmd.com/read/29601482/oxamate-but-not-selective-targeting-of-ldh-a-inhibits-medulloblastoma-cell-glycolysis-growth-and-motility
#6
Cara J Valvona, Helen L Fillmore
Medulloblastoma is the most common malignant paediatric brain tumour and current therapies often leave patients with severe neurological disabilities. Four major molecular groups of medulloblastoma have been identified (Wnt, Shh, Group 3 and Group 4), which include additional, recently defined subgroups with different prognosis and genetic characteristics. Lactate dehydrogenase A (LDHA) is a key enzyme in the aerobic glycolysis pathway, an abnormal metabolic pathway commonly observed in cancers, associated with tumour progression and metastasis...
March 30, 2018: Brain Sciences
https://www.readbyqxmd.com/read/29599574/adult-rhabdomyoma-of-the-tongue-in-a-child-report-of-a-case-and-a-literature-appraisal
#7
REVIEW
Neelam N Andrade, Trupti Gandhewar, Neha Aggarwal, Paul Mathai
Rhabdomyoma, by definition is a benign muscle tumour.. Rhabdomyomas constitute 2% of all myogenous neoplasms. This tumour is in incongruence with other benign soft tissue tumours, in that it is rarer than its malignant counterpart. They are broadly categorised as cardiac and extra-cardiac. Three different subtypes exists as 1) the adult type, 2) the fetal type and 3) the genital type, the adult type being the most common.[1] AR (Adult Rhabdomyoma) generally occurs in the 4th and 5th decade with a male predilection...
January 2018: Contemporary Clinical Dentistry
https://www.readbyqxmd.com/read/29557349/oral-and-maxillofacial-tumours-in-children-and-adolescents-clinicopathologic-audit-of-75-cases-in-an-academic-medical-centre-sokoto-northwest-nigeria
#8
Abdurrazaq Olanrewaju Taiwo, Ramat Oyebunmi Braimah, Adebayo Aremu Ibikunle, Mutiat Feyisetan Obileye, Nma Muhammed Jiya, Saddiku Malami Sahabi, Idris Kabiru Jaja
Background: Maxillofacial tumours in children and adolescents have been documented worldwide; however, few studies were reported from Africa, especially sub-Saharan Africa. In Nigeria, most of the studies emanated from the Southwest region. Aim: To present an audit of clinicopathologic features and treatment of orofacial tumours in children and adolescents in Sokoto, Northwest Nigeria. Patients and Methods: Clinicopathologic records of the Departments of Dental and Maxillofacial Surgery, Paediatrics and Histopathology, Usmanu Danfodiyo University Teaching Hospital, Sokoto, Nigeria, were reviewed for all the oral and maxillofacial tumours managed in children <19 years from January 2011 to December 2015...
July 2017: African Journal of Paediatric Surgery: AJPS
https://www.readbyqxmd.com/read/29525139/transoral-robotic-excision-of-laryngeal-papillomas-with-flex%C3%A2-robotic-system-a-novel-surgical-approach
#9
Bernar Tan Wen Sheng, Patrick Wong, Constance Teo Ee Hoon
INTRODUCTION: Recurrent respiratory papillomatosis results in hoarseness, stridor and airway obstruction. Management is surgical, with most surgeons using microdebrider or laser. Transoral robotic surgery (TORS) has been successfully utilised for the excision of oropharyngeal malignancies and paediatric airway surgery. This is the first case report of TORS being used for the excision of laryngeal papillomas. CASE REPORT: A 36 year old Chinese female was diagnosed with juvenile onset recurrent respiratory papillomatosis...
March 3, 2018: American Journal of Otolaryngology
https://www.readbyqxmd.com/read/29514143/long-term-follow-up-after-paediatric-kidney-transplantation-and-influence-factors-on-graft-survival-a-single-centre-experience-of-16-years
#10
Frank Friedersdorff, Therese-Marie Koch, Beatriz Banuelos-Marco, Ricardo Gonzalez, Tom Florian Fuller, Stefanie von Mechow, Dominik Müller, Anja Lingnau
INTRODUCTION: To evaluate graft- and patient survival after paediatric kidney transplantation and detecting influence factors, which affect the post-transplant time. MATERIALS AND METHODS: We analysed long-term survival rates and complications after paediatric kidney transplantation and searched for predictive parameters for graft function. RESULTS: In 132 patients, 143 kidney transplantations were performed. Graft failure occurred in 25%...
March 7, 2018: Urologia Internationalis
https://www.readbyqxmd.com/read/29508129/large-paediatric-central-osteoma-with-osteoblastoma-like-features-in-the-mandible
#11
Anik Saha, Omar Breik, Ian Simpson, Ricky Kumar
The diagnosis of osteomas in the paediatric population can pose a challenge to pathologists in excluding malignant bony tumours. We present the case of a 10-year old male presenting with a large left mandibular radiopaque lesion. This paper discusses the case of a central osteoma with osteoblastoma-like features, literature review, differential diagnosis of radiopaque lesions of the maxilla and mandible as well as a detailed discussion of the pathology of the lesion. Although similar lesions have been described in the sino-orbital region, this is believed to be the first report of this pathological entity in the mandible...
March 5, 2018: Head and Neck Pathology
https://www.readbyqxmd.com/read/29479631/clinical-aspects-of-tacrolimus-use-in-paediatric-renal-transplant-recipients
#12
Agnieszka Prytuła, Teun van Gelder
The calcineurin inhibitor tacrolimus, cornerstone of most immunosuppressive regimens, is a drug with a narrow therapeutic window: underexposure can lead to allograft rejection and overexposure can result in an increased incidence of infections, toxicity and malignancies. Tacrolimus is metabolised in the liver and intestine by the cytochrome P450 3A (CYP3A) isoforms CYP3A4 and CYP3A5. This review focusses on the clinical aspects of tacrolimus pharmacodynamics, such as efficacy and toxicity. Factors affecting tacrolimus pharmacokinetics, including pharmacogenetics and the rationale for routine CYP3A5*1/*3 genotyping in prospective paediatric renal transplant recipients, are also reviewed...
February 26, 2018: Pediatric Nephrology: Journal of the International Pediatric Nephrology Association
https://www.readbyqxmd.com/read/29474644/dicer1-mutations-are-frequent-in-adolescent-onset-papillary-thyroid-carcinoma
#13
Jonathan D Wasserman, Nelly Sabbaghian, Somayyeh Fahiminiya, Rose Chami, Ozgur Mete, Meryl Acker, Mona K Wu, Adam Shlien, Leanne de Kock, William D Foulkes
Context: Papillary thyroid carcinoma (PTC) is a common malignancy in adolescence and is molecularly and clinically distinct from adult PTC. Mutations in the DICER1 gene are associated with thyroid abnormalities including multinodular goitre and differentiated thyroid carcinoma. Objective: In this study, we sought to characterize the prevalence of DICER1 variants in paediatric PTC, specifically in tumours without conventional PTC oncogenic alterations. Patients: Patients (n = 40) who underwent partial or total thyroidectomy and who were <18 years of age at the time of surgery were selected...
February 21, 2018: Journal of Clinical Endocrinology and Metabolism
https://www.readbyqxmd.com/read/29469189/incorporation-of-gsta1-genetic-variations-into-a-population-pharmacokinetic-model-for-iv-busulfan-in-paediatric-hematopoietic-stem-cell-transplantation
#14
T Nava, N Kassir, M A Rezgui, C R S Uppugunduri, P Huezo-Diaz Curtis, M Duval, Y Théoret Y, L E Daudt, C Litalien, M Ansari, M Krajinovic, H Bittencourt
AIMS: To develop a population pharmacokinetic (PopPK) model for intravenous busulfan in children that incorporates variants of GSTA1, gene coding for the main enzyme in busulfan imetabolism. METHODS: Busulfan concentration-time data was collected from 112 children and adolescents (median 5.4 years old, range: 0.1 to 20) who received intravenous busulfan during the conditioning regimen prior to stem cell transplantation. Weight, sex, baseline disease (malignant vs...
February 22, 2018: British Journal of Clinical Pharmacology
https://www.readbyqxmd.com/read/29451709/shortcomings-in-the-management-of-undescended-testis-guideline-intention-vs-reality-and-the-underlying-causes-insights-from-the-biggest-german-cohort
#15
Philip Boehme, Berit Geis, Johannes Doerner, Stefan Wirth, Kai O Hensel
OBJECTIVES: To assess the implementation of the current guideline and identify potential underlying causes for late surgery in children with Undescended testis (UDT) in Germany. UDT is the most common surgical issue in paediatric urology. To avoid malignant degeneration and subfertility current guidelines recommend orchidopexy during the first year of life. However, this seems not to be implemented in practice. PATIENTS AND METHODS: 5547 patients with cryptorchidism at 16 hospitals nationwide were studied regarding age at orchidopexy between 2003 and 2016...
February 16, 2018: BJU International
https://www.readbyqxmd.com/read/29440001/risk-of-malignancy-associated-with-paediatric-use-of-tumour-necrosis-factor-inhibitors
#16
Timothy Beukelman, Fenglong Xie, Lang Chen, Daniel B Horton, James D Lewis, Ronac Mamtani, Melissa M Mannion, Kenneth G Saag, Jeffrey R Curtis
OBJECTIVE: To determine whether tumour necrosis factor inhibitor (TNFi) use is associated with an increased rate of incident malignancy compared with no TNFi use in the treatment of juvenile idiopathic arthritis (JIA), paediatric inflammatory bowel disease (pIBD) and paediatric plaque psoriasis (pPsO). METHODS: We performed a retrospective cohort study of administrative claims data from the USA from 2000 to 2014. Exposure to TNFi was considered permanent from the first observed exposure onward...
February 9, 2018: Annals of the Rheumatic 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
#17
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...
February 9, 2018: Journal of Infection
https://www.readbyqxmd.com/read/29431252/factors-associated-with-abandonment-of-therapy-by-children-diagnosed-with-solid-tumors-in-peru
#18
Liliana Vasquez, Rosdali Diaz, Sharon Chavez, Fanny Tarrillo, Ivan Maza, Eddy Hernandez, Monica Oscanoa, Juan García, Jenny Geronimo, Nuria Rossell
BACKGROUND: Abandonment of treatment is a major cause of treatment failure and poor survival in children with cancer in low- and middle-income countries. The incidence of treatment abandonment in Peru has not been reported. The aim of this study was to examine the prevalence of and factors associated with treatment abandonment by pediatric patients with solid tumors in Peru. METHODS: We retrospectively reviewed the sociodemographic and clinical data of children referred between January 2012 and December 2014 to the two main tertiary centers for childhood cancer in Peru...
February 12, 2018: Pediatric Blood & Cancer
https://www.readbyqxmd.com/read/29428360/adolescent-testicular-microlithiasis-a-case-based-multinational-survey-of-clinical-management-practices
#19
Katie E Brodie, Amanda F Saltzman, Nicholas G Cost
INTRODUCTION: Testicular microlithiasis (TM) is a condition characterized by calcium deposits within the testis, usually detected incidentally during ultrasonography of the scrotum. TM has been associated with the presence of, and possibly the development of, testicular malignancy. Our aim was to document international clinical management practices for TM and to analyze what factors and perception of risk influence conservative versus active management and follow-up. METHODS: European Society for Paediatric Urology (ESPU) and Society for Pediatric Urology (SPU) members were invited to complete an online case-based survey of clinical management practices of TM...
January 31, 2018: Journal of Pediatric Urology
https://www.readbyqxmd.com/read/29427028/2017-pretext-radiologic-staging-system-for-primary-hepatic-malignancies-of-childhood-revised-for-the-paediatric-hepatic-international-tumour-trial-phitt
#20
REVIEW
Alexander J Towbin, Rebecka L Meyers, Helen Woodley, Osamu Miyazaki, Christopher B Weldon, Bruce Morland, Eiso Hiyama, Piotr Czauderna, Derek J Roebuck, Greg M Tiao
Imaging is crucial in the assessment of children with a primary hepatic malignancy. Since its inception in 1992, the PRETEXT (PRE-Treatment EXTent of tumor) system has become the primary method of risk stratification for hepatoblastoma and pediatric hepatocellular carcinoma in numerous cooperative group trials across the world. The PRETEXT system is made of two components: the PRETEXT group and the annotation factors. The PRETEXT group describes the extent of tumor within the liver while the annotation factors help to describe associated features such as vascular involvement (either portal vein or hepatic vein/inferior vena cava), extrahepatic disease, multifocality, tumor rupture and metastatic disease (to both the lungs and lymph nodes)...
February 9, 2018: Pediatric Radiology
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