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https://www.readbyqxmd.com/read/28431807/rhabdomyosarcoma-of-the-maxillofacial-region-in-children-and-adolescents-report-of-9-cases-and-literature-review
#1
Ioannis Iatrou, Nadia Theologie-Lygidakis, Ourania Schoinohoriti, Fotis Tzermpos, Anna-Maria Vessala
OBJECTIVE: To review clinical presentation, histology, treatment and survival for pediatric maxillofacial rhabdomyosarcoma (RMS) and evaluate the role of surgical treatment. STUDY DESIGN: Retrospective analysis of medical charts. METHODS: Files of patients, treated for primary maxillofacial RMS from 1997 to 2016, were examined for clinical presentation, staging, histology, treatment protocol and complications, recurrence and final outcome...
March 23, 2017: Journal of Cranio-maxillo-facial Surgery
https://www.readbyqxmd.com/read/28430703/evidence-based-recommendations-for-pediatric-orthopaedic-practice
#2
Kishore Mulpuri, Emily K Schaeffer, H Kerr Graham, Mininder S Kocher, James Sanders, Ira Zaltz
BACKGROUND: Evidence-based medicine has become the cornerstone to guide clinical practice decision-making. Evidence-based medicine integrates the strongest available evidence with clinical expertise to make decisions about clinical care. The quality of the evidence depends upon the soundness of the study methodology to allow for meaningful interpretation of the clinical results. The purpose of this review is to analyze the methodological design and clinical findings of published pediatric orthopaedic studies to determine their ability to change or influence clinical practice...
April 20, 2017: Journal of Pediatric Orthopedics
https://www.readbyqxmd.com/read/28428651/utility-of-the-inspiratory-phase-in-high-resolution-computed-tomography-evaluations-of-pediatric-patients-with-bronchiolitis-obliterans-after-allogeneic-bone-marrow-transplant-reducing-patient-radiation-exposure
#3
Paulo Henrique Togni Filho, João Luiz Marin Casagrande, Henrique Manoel Lederman
OBJECTIVE: To evaluate the utility of the inspiratory phase in high-resolution computed tomography (HRCT) of the chest for the diagnosis of post-bone marrow transplantation bronchiolitis obliterans. MATERIALS AND METHODS: This was a retrospective, observational, cross-sectional study. We selected patients of either gender who underwent bone marrow transplantation and chest HRCT between March 1, 2002 and December 12, 2014. Ages ranged from 3 months to 20.7 years...
March 2017: Radiologia Brasileira
https://www.readbyqxmd.com/read/28427923/-acute-leukemia-revealed-by-bone-magnetic-resonance-imaging-two-pediatric-case-reports
#4
C Flatrès, A Pennanéach, G Blondin, E Rivoal, S Haro, P Vic
Bone and joint pain are common causes of pediatric consultation for a variety of etiologies. The causes are mostly traumatic or infectious or have an inflammatory origin. Acute leukemia (AL) can sometimes begin with osteoarticular signs and radiological investigations such as magnetic resonance imaging (MRI) can help guide the diagnosis. We report on two cases of pediatric AL revealed by osteoarticular signs, with MRI helpful in the diagnostic procedure. In these children, the first signs were recurrent joint pain, with no blood count anomalies...
April 17, 2017: Archives de Pédiatrie: Organe Officiel de la Sociéte Française de Pédiatrie
https://www.readbyqxmd.com/read/28427520/lymphoblastic-lymphoma
#5
REVIEW
Sergio Cortelazzo, Andrés Ferreri, Dieter Hoelzer, Maurilio Ponzoni
Lymphoblastic lymphoma (LBL) is a neoplasm of immature B cells committed to the B-(B-LBL) or T-cell lineage (T-LBL) that accounts for approximately 2% of all lymphomas. Although histological features are usually sufficient to distinguish lymphoblastic from mature B- or T-cell neoplasms, of greater importance for diagnosis is the characterization of immunophenotype by flow cytometry. LBL occurs more commonly in children than in adults, mostly in males. A bone marrow involvement <25% (or 20% according to WHO) formally distinguishes LBL from ALL...
May 2017: Critical Reviews in Oncology/hematology
https://www.readbyqxmd.com/read/28427477/plasma-chitotriosidase-activity-versus-ccl18-level-for-assessing-type-i-gaucher-disease-severity-protocol-for-a-systematic-review-with-meta-analysis-of-individual-participant-data
#6
Tatiana Raskovalova, Patrick B Deegan, Ruby Yang, Elena Pavlova, Jérome Stirnemann, José Labarère, Ari Zimran, Pramod K Mistry, Marc Berger
BACKGROUND: Gaucher disease (GD) is an autosomal recessive lysosomal storage disorder caused by deficiency in acid beta-glucosidase. GD exhibits a wide clinical spectrum of disease severity with an unpredictable natural course. Plasma chitotriosidase activity and CC chemokine ligand 18 (CCL18) have been exchangeably used for monitoring GD activity and response to enzyme replacement therapy in conjunction with clinical assessment. Yet, a large-scale head-to-head comparison of these two biomarkers is currently lacking...
April 20, 2017: Systematic Reviews
https://www.readbyqxmd.com/read/28427053/growing-skull-fracture-and-the-orbitocranial-variant-nuances-of-surgical-management
#7
Vikram Singh, Gopalakrishnan M Sasidharan, Dhananjaya Ishwar Bhat, Bhagavatula Indira Devi
AIMS: Growing skull fracture (GSF) is a rare complication resulting from diastatic enlargement of a pediatric skull fracture. Orbitocranial GSF is a distinct variant with more complex management issues due to the displacement of the globe. This study aims to discuss surgical considerations in the management of GSF, with particular reference to orbitocranial variants. METHODS: We conducted a retrospective analysis of 7 children operated for GSFs at our institute from 2008 to 2015...
April 21, 2017: Pediatric Neurosurgery
https://www.readbyqxmd.com/read/28427039/low-dose-growth-hormone-treatment-in-infants-and-toddlers-with-prader-willi-syndrome-is-comparable-to-higher-dosage-regimens
#8
Elly Scheermeyer, Mark Harris, Ian Hughes, Patricia A Crock, Geoffrey Ambler, Charles F Verge, Phil Bergman, George Werther, Maria E Craig, Catherine S Choong, Peter S W Davies
OBJECTIVE: Evaluate benefit and risk of low dose growth hormone treatment (GHT, 4.5mg/m(2)/week) in very young children with Prader-Willi Syndrome (PWS). DESIGN: Prospective longitudinal clinical intervention. METHODS: We evaluated 31 infants (aged 2-12months) and 42 toddlers (13-24months) from the PWS-OZGROW database for height, weight and BMI using the World Health Organization standard deviation scores (SDSWHO) and PWS specific BMI (SDSPWS), bone age, insulin-like growth factor 1 (IGF-I) levels and adverse events over 3years of GHT...
March 24, 2017: Growth Hormone & IGF Research
https://www.readbyqxmd.com/read/28426528/comparative-analysis-of-multicolor-flow-cytometry-and-immunohistochemistry-for-the-detection-of-disseminated-tumor-cells
#9
Eszter Szánthó, Bettina Kárai, Gergely Ivády, Judit Bedekovics, István Szegedi, Miklós Petrás, György Ujj, Anikó Ujfalusi, Csongor Kiss, János Kappelmayer, Zsuzsanna Hevessy
Disseminating cells of a primary solid tumor may represent the origin of metastases and relapses. We aimed at comparing the diagnostic efficacy of multicolor flow cytometry (MFC) and morphology/immunohistochemistry (IHC) in the detection of disseminated tumor cells in the bone marrow (BM) and body fluids of patients with solid tumors, and in pediatric neuroblastoma cases. We investigated 72 samples retrospecively from 50 patients by MFC. Morphology/IHC data were available in 48 cases. In the first cohort, 36 samples derived from 34 patients with various forms of suspected and proven solid tumors and in the second cohort, 36 samples of 16 children with suspected and proven neuroblastoma were analyzed at diagnosis or during follow-up in a 4-color setting by MFC, and the results were compared with those obtained by IHC...
April 19, 2017: Applied Immunohistochemistry & Molecular Morphology: AIMM
https://www.readbyqxmd.com/read/28426102/infection-related-complications-during-treatment-for-childhood-acute-lymphoblastic-leukemia
#10
H Inaba, D Pei, J Wolf, S C Howard, R T Hayden, M Go, O Varechtchouk, T Hahn, J Buaboonnam, M L Metzger, J E Rubnitz, R C Ribeiro, J T Sandlund, S Jeha, C Cheng, W E Evans, M V Relling, C-H Pui
Background: Comprehensive studies on neutropenia and infection-related complications in patients with acute lymphoblastic leukemia (ALL) are lacking. Patients and methods: We evaluated infection-related complications that were grade ≥3 on National Cancer Institute's Common Terminology Criteria for Adverse Events (version 3.0) and their risk factors in 409 children with newly diagnosed ALL throughout the treatment period. Results: Of the 2420 infection episodes, febrile neutropenia and clinically or microbiologically documented infection were seen in 1107 and 1313 episodes, respectively...
February 1, 2017: Annals of Oncology: Official Journal of the European Society for Medical Oncology
https://www.readbyqxmd.com/read/28423674/a-single-center-clinical-analysis-of-children-with-high-risk-neuroblastoma
#11
Xiangdong Tian, Yanna Cao, Jingfu Wang, Jie Yan, Yao Tian, Zhongyuan Li, Huijuan Wang, Xiaofeng Duan, Yan Jin, Qiang Zhao
The current multidisciplinary treatment for patients with high-risk neuroblastoma (NB) is the common census. However, protocols and opinions are different in different regions and institutions. We aimed to assess the protocol formulated by Chinese Children's Cancer Group study in 2009, and the impact of surgery extent was highlightly evaluated. METHODS: This study enrolled patients with high-risk neuroblastoma between 2009 and 2014 in Department of Pediatric Oncology of Tianjin Medical University Cancer Institute and Hospital...
March 7, 2017: Oncotarget
https://www.readbyqxmd.com/read/28423603/adipose-derived-mesenchymal-stem-cells-promote-osteosarcoma-proliferation-and-metastasis-by-activating-the-stat3-pathway
#12
Yan Wang, Yijing Chu, Bin Yue, Xuexiao Ma, Guoqing Zhang, Hongfei Xiang, Yong Liu, Tianrui Wang, Xiaolin Wu, Bohua Chen
Osteosarcoma is the most common primary bone malignancy in children and young adults, but the role of adipose-derived mesenchymal stem cells (ADSCs) in the rapid progression of osteosarcoma is still unclear. Here, we found that ADSCs promoted tumour growth and invasion by increasing matrix metalloproteinase 2/9 (MMP2/9) expression in tumour cells. The persistent activation of signal transducer and activator of transcription 3 (STAT3) has been shown to directly promote tumour growth by mediating a wide spectrum of cellular responses, and STAT3 activation was detected in osteosarcoma cells co-cultured with ADSCs or treated with ADSC-conditioned medium...
April 4, 2017: Oncotarget
https://www.readbyqxmd.com/read/28422876/low-complication-rate-of-elastic-stable-intramedullary-nailing-esin-of-pediatric-forearm-fractures-a-retrospective-study-of-202-cases
#13
Christiane Kruppa, Pamela Bunge, Thomas A Schildhauer, Marcel Dudda
Elastic stable intramedullary nailing (ESIN) has been established as state of the art treatment for forearm fractures in children, if operative stabilization is required. Their use has been expanded to single bone shaft fractures, and also more complex injuries such as Monteggia fractures or Monteggia-like lesions. A wide range of complications has been reported in the literature, up to 70% in certain investigations. The purpose of this study was to assess the complication rate after ESIN treatment of forearm fractures in children and adolescents in a representative cohort of patients from a level 1 trauma center in Germany...
April 2017: Medicine (Baltimore)
https://www.readbyqxmd.com/read/28422603/factors-responsible-for-unfavorable-dental-arch-relationship-in-non-syndromic-unilateral-cleft-lip-and-palate-children
#14
Sanjida Haque, Mohammad Khursheed Alam, Mohd Fadhli Khamis
OBJECTIVES: Multiple factors are whispered to be crucial cause of unfavourable dental arch relationship in cleft lip and palate (CLP).This study aims to evaluate the dental arch relationship of Bangladeshi children with non syndromic unilateral cleft lip and palate (UCLP) following cheiloplasty and palatoplasty. Also to explore the various congenital (UCLP type, UCLP side, family history of cleft, family history of class III) and environmental (cheiloplasty, palatoplasty) factors that affects dental arch relationship of UCLP patients...
2017: Journal of Clinical Pediatric Dentistry
https://www.readbyqxmd.com/read/28420931/paediatrics-primary-myelofibrosis-and-acute-stroke-a-rare-presentation
#15
Wajida Mazher, Ather Hasan Rizvi, Arsalan Majeed Adam, Muhammad Saad Ali Mallick, Ansab Godil
Idiopathic Myelofibrosis is a rare myeloproliferative disorder. In children, it becomes even rarer. Herein, we report a case of idiopathic myelofibrosis of a 6-year old male patient who presented with complaints of pallor, petechiae and bleeding from gums. Bone marrow aspirate showed afragmented haemodiluted smears with erythroid and myeloid precursors scattered throughout. Trephine biopsy showed increased background fibrotic activity along with clusters of histiocytes. A diagnosis of paediatric primary myelofibrosis was made on biopsy...
April 2017: JPMA. the Journal of the Pakistan Medical Association
https://www.readbyqxmd.com/read/28419547/perinatal-dna-methylation-at-cdkn2a-is-associated-with-offspring-bone-mass-findings-from-the-southampton-women-s-survey
#16
Elizabeth M Curtis, Robert Murray, Philip Titcombe, Eloïse Cook, Rebecca Clarke-Harris, Paula Costello, Emma Garratt, Joanna D Holbrook, Sheila Barton, Hazel Inskip, Keith M Godfrey, Christopher G Bell, Cyrus Cooper, Karen A Lillycrop, Nicholas C Harvey
Poor intrauterine and childhood growth has been linked with the risk of osteoporosis in later life, a relationship which may in part be mediated through altered epigenetic regulation of genes. We previously identified a region within the promoter of the long non-coding RNA ANRIL encoded by the CDKN2A locus, at which differential DNA methylation at birth showed correlations with offspring adiposity. Given the common lineage of adipocytes and osteoblasts, we investigated the relationship between perinatal CDKN2A methylation and bone mass at ages 4 and 6 years...
April 17, 2017: Journal of Bone and Mineral Research: the Official Journal of the American Society for Bone and Mineral Research
https://www.readbyqxmd.com/read/28419441/detailed-immunophenotyping-of-b-cell-precursors-in-regenerating-bone-marrow-of-acute-lymphoblastic-leukaemia-patients-implications-for-minimal-residual-disease-detection
#17
Prisca M J Theunissen, Lukasz Sedek, Valerie De Haas, Tomasz Szczepanski, Alita Van Der Sluijs, Ester Mejstrikova, Michaela Nováková, Tomas Kalina, Quentin Lecrevisse, Alberto Orfao, Arjan C Lankester, Jacques J M van Dongen, Vincent H J Van Der Velden
Flow cytometric detection of minimal residual disease (MRD) in children with B-cell precursor acute lymphoblastic leukaemia (BCP-ALL) requires immunophenotypic discrimination between residual leukaemic cells and B-cell precursors (BCPs) which regenerate during therapy intervals. In this study, EuroFlow-based 8-colour flow cytometry and innovative analysis tools were used to first characterize the immunophenotypic maturation of normal BCPs in bone marrow (BM) from healthy children, resulting in a continuous multiparametric pathway including transition stages...
April 17, 2017: British Journal of Haematology
https://www.readbyqxmd.com/read/28417857/nuclear-medicine-imaging-in-pediatric-infection-or-chronic-inflammatory-diseases
#18
REVIEW
Alberto Signore, Andor W J M Glaudemans, Olivier Gheysens, Chiara Lauri, Onofrio A Catalano
In this review article, we focus on the most recent applications of nuclear medicine techniques (mainly (99m)Tc/(111)In white blood cells (WBC) scan, [(18)F]-FDG-PET/CT, [(18)F]-FDG-PET/MRI, and (99m)Tc-IL-2 scintigraphy) in the study of children affected by peripheral bone osteomyelitis, fungal infections, inflammatory bowel diseases, and type 1 diabetes, owing to recent important published evidences of their role in the management of these diseases. For osteomyelitis in children, both bone scintigraphy and [(18)F]-FDG-PET have a major advantage of assessing the whole body in one imaging session to confirm or exclude multifocal involvement, whereas WBC scan has a limited role...
May 2017: Seminars in Nuclear Medicine
https://www.readbyqxmd.com/read/28417852/nuclear-medicine-in-pediatric-nephro-urology-an-overview
#19
REVIEW
Iosif Mendichovszky, Bernardita Troncoso Solar, Naima Smeulders, Marina Easty, Lorenzo Biassoni
In the context of ante-natally diagnosed hydronephrosis, the vast majority of children with a dilated renal pelvis do not need any surgical treatment, as the dilatation resolves spontaneously with time. Slow drainage demonstrated at Tc-99m-mercaptoacetyltriglycine (MAG3) renography does not necessarily mean obstruction. Obstruction is defined as resistance to urinary outflow with urinary stasis at the level of the pelvic-ureteric junction (PUJ) which, if left untreated, will damage the kidney. Unfortunately this definition is retrospective and not clinically helpful...
May 2017: Seminars in Nuclear Medicine
https://www.readbyqxmd.com/read/28417851/nuclear-medicine-techniques-in-pediatric-bone-imaging
#20
REVIEW
Laura A Drubach
An important mandate when imaging pediatric patients is the reduction of radiation exposure to the lowest possible level consistent with good quality diagnostic imaging, so individual selection of radiopharmaceutical dosage and imaging technique is essential. Although the logistics of image acquisition and the approach to interpretation mirror the more common use in adults, the challenges of imaging young children typically require greater attention to patient preparation, positioning, and supervision during imaging, with the use of parental or family engagement in the procedure, gentle restraint, and occasionally the need for sedation...
May 2017: Seminars in Nuclear Medicine
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