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bone marrow transplant pediatric

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https://www.readbyqxmd.com/read/28447322/successful-hepatectomy-for-hepatic-abscess-with-chronic-granulomatous-disease-a-case-report
#1
Ryo Muranushi, Makoto Suzuki, Kenichiro Araki, Norio Kubo, Sayaka Otake, Yutaka Nishida, Takashi Ishige, Hirokazu Arakawa, Hiroyuki Kuwano, Ken Shirabe
BACKGROUND: Chronic granulomatous disease (CGD), a rare inherited disorder, is characterized by impaired ability of phagocytic cells to kill certain bacteria and fungi. Although liver abscess is a common manifestation of CGD, its optimal management in these patients is unknown. Here, we present a case of successful hepatectomy for hepatic abscess in a patient with CGD. CASE PRESENTATION: An adolescent patient with previously diagnosed CGD presented to the pediatrics department of our institution with fever...
December 2017: Surgical Case Reports
https://www.readbyqxmd.com/read/28445240/evaluation-of-a-pediatric-early-warning-score-across-different-subspecialty-patients
#2
Nathan P Dean, J B Fenix, Michael Spaeder, Amanda Levin
OBJECTIVE: To evaluate the ability of a Pediatric Early Warning Score to predict deterioration in different subspecialty patient populations. DESIGN: Single center, retrospective cohort study. Patients were classified into five groups: 1) cardiac; 2) hematology/oncology/bone marrow transplant; 3) surgical; 4) neurologic; and 5) general medical. The relationship between the Pediatric Early Warning Score and unplanned ICU transfer requiring initiation of specific ICU therapies (intubation, high-flow nasal cannula, noninvasive ventilation, inotropes, or aggressive fluid hydration within 12 hr of transfer) was evaluated...
April 25, 2017: Pediatric Critical Care Medicine
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/28415959/the-practices-and-meanings-of-care-for-nurses-working-on-a-pediatric-bone-marrow-transplant-unit
#4
Caroline F Morrison, Edith J Morris
Bone marrow transplant (BMT) units are stressful work environments with high staff burnout, anxiety, and turnover. A qualitative study was undertaken to better understand care for nurses within the context of their clinical practice on a pediatric BMT unit. Understanding care practices for BMT nurses will allow us to design interventions and provide appropriate support for this subspecialty of nurses. Focus groups were held with 24 nurses as key participants, and 2 focus groups with general participants: unit management (N = 2) and caregivers of inpatients (N = 7)...
May 2017: Journal of Pediatric Oncology Nursing: Official Journal of the Association of Pediatric Oncology Nurses
https://www.readbyqxmd.com/read/28398275/natural-killer-t-cell-lymphomas-in-pediatric-and-adolescent-patients
#5
REVIEW
Amanda M Termuhlen
Natural killer/T-cell (NK/T-cell) lymphomas are rare in children and adolescents and consist predominantly of nasal-type extranodal NK/T-cell lymphomas. More than half of pediatric/adolescent patients with NK/T-cell lymphomas present with localized nasal/sinus involvement, but the disease may involve many organs. NK/T-cell lymphoma cells are cytotoxic and associated with necrosis and angioinvasion; they express CD56, CD2, cytoplasmic CD3 epsilon, and to a variable degree CD30. The cells contain Epstein-Barr virus (EBV)-encoded RNA...
March 2017: Clinical Advances in Hematology & Oncology: H&O
https://www.readbyqxmd.com/read/28396164/dose-escalation-of-total-marrow-irradiation-in-high-risk-patients-undergoing-allogeneic-hematopoietic-stem-cell-transplantation
#6
Susanta Hui, Claudio Brunstein, Yutaka Takahashi, Todd DeFor, Shernan G Holtan, Veronika Bachanova, Christopher Wilke, Darren Zuro, Celalettin Ustun, Daniel Weisdorf, Kathryn Dusenbery, Michael R Verneris
Patients with refractory leukemia or minimal residual disease (MRD) at transplant have increased risk of relapse. Augmentation of irradiation, especially to sites of disease (i.e., bone marrow) is one potential strategy to overcome this risk. We studied the feasibility of radiation dose escalation in high risk patients using total marrow irradiation (TMI) in a phase I dose-escalation trial. Four pediatric and 8 adult patients received conditioning with cyclophosphamide and fludarabine in conjunction with image-guided radiation to the bone marrow at 15 Gy and 18 Gy (in 3 Gy/fractions), while maintaining the total body irradiation (TBI) dose to the vital organs (lungs, hearts, eyes, liver, kidneys) at <13...
April 7, 2017: Biology of Blood and Marrow Transplantation
https://www.readbyqxmd.com/read/28395444/-prognostic-value-of-dynamic-monitoring-of-runx1-runx1t1-transcript-in-pediatric-acute-myeloid-leukemia
#7
H T Gao, Y Zhang, K Sun, J M Guo, Y Q Chen, X L Chen, J Shi, X N Niu, F Wang, L Huo
Objective: To investigate the prognostic value of dynamic monitoring of RUNX1-RUNX1T1 transcript in pediatric patients with t (8;21) acute myeloid leukemia (AML) . Methods: The clinical features and RUNX1-RUNX1T1 transcript levels of 55 pediatric t (8;21) AML patients, newly diagnosed from Jan. 2010 to Apr. 2016, were analyzed retrospectively. The relationship between the minimal residual disease (MRD) and prognosis was analysed by dynamic monitoring of RUNX1-RUNX1T1 transcript levels using real-time quantitative PCR (RQ-PCR) technology...
March 14, 2017: Zhonghua Xue Ye Xue za Zhi, Zhonghua Xueyexue Zazhi
https://www.readbyqxmd.com/read/28380398/a-retrospective-study-of-paroxysmal-nocturnal-hemoglobinuria-in-pediatric-and-adolescent-patients
#8
Angela Mercuri, Piero Farruggia, Fabio Timeus, Laura Lombardi, Daniela Onofrillo, Maria Caterina Putti, Marta Pillon, Maria Elena Cantarini, Paola Corti, Gloria Tridello, Massimiliano De Bortoli, Anna Pegoraro, Simone Cesaro
Paroxysmal nocturnal hemoglobinuria (PNH) is a rare disease, especially in children, characterized by intravascular hemolysis, thrombotic events, serious infections and bone marrow failure. We describe 16 patients who were diagnosed with PNH in childhood or adolescence. The time interval between the onset of symptoms and the PNH diagnosis and its treatment were compared in patients with classic PNH versus PNH associated with bone marrow disorder (PNH/BMD). A greater delay in diagnosis was observed in classic PNH compared to PNH/BMD patients...
March 18, 2017: Blood Cells, Molecules & Diseases
https://www.readbyqxmd.com/read/28368380/risk-of-melanocytic-nevi-and-nonmelanoma-skin-cancer-in-children-after-allogeneic-hematopoietic-stem-cell-transplantation
#9
J S Song, W B London, E B Hawryluk, D Guo, M Sridharan, D E Fisher, L E Lehmann, C N Duncan, J T Huang
There is a known increased risk of skin cancer in the adult population after hematopoietic stem cell transplantation (HSCT). However, late dermatologic effects that children may experience after HSCT have not been well described. The primary objective of this study was to characterize nevi and skin cancers affecting children after allogeneic HSCT. A cross-sectional cohort study of 85 pediatric HSCT recipients and 85 controls matched for age, sex and skin phototype was performed at a single institution. All participants underwent a full skin examination...
April 3, 2017: Bone Marrow Transplantation
https://www.readbyqxmd.com/read/28367616/management-of-neuroblastoma-icmr-consensus-document
#10
REVIEW
Deepak Bansal, Sidharth Totadri, Girish Chinnaswamy, Sandeep Agarwala, Tushar Vora, Brijesh Arora, Maya Prasad, Gauri Kapoor, Venkatraman Radhakrishnan, Siddharth Laskar, Tanvir Kaur, G K Rath, Sameer Bakhshi
Neuroblastoma (NBL) is the most common extra-cranial solid tumor in childhood. High-risk NBL is considered challenging and has one of the least favourable outcomes amongst pediatric cancers. Primary tumor can arise anywhere along the sympathetic chain. Advanced disease at presentation is common. Diagnosis is established by tumor biopsy and elevated urinary catecholamines. Staging is performed using bone marrow and mIBG scan (FDG-PET/bone scan if mIBG unavailable or non-avid). Age, stage, histopathological grading, MYCN amplification and 11q aberration are important prognostic factors utilized in risk stratification...
April 3, 2017: Indian Journal of Pediatrics
https://www.readbyqxmd.com/read/28326208/a-child-as-a-donor-for-hematopoietic-stem-cell-transplantation-bioethical-justification-a-case-study-on-sickle-cell-disease
#11
Andrea Z Pereira, Ricardo Hellman, Nelson Hamerschlak, Andrea Kondo, Polianna Mara Rodrigues de Souza, Wilson Leite Pedreira, Luiz Fernando Alves Lima Mantovani, Eduardo Juan Troster, Henrique Grunspun, Marco Aurélio Scarpinella Bueno
Hematopoietic stem cell transplantation (HSCT) is an important treatment option for children with severe and refractory sickle cell disease (SCD) with debilitating clinical complications. HSCT with cells from the bone marrow of a HLA-identical sibling used in SCD has a low mortality risk, high cure rate, and high event-free survival rate after a median follow-up of 5-6 years. However, matched donors are found in only about 20% of the patients. A boy aged 8 years with SCD had a sister, <2 years old, a fully compatible donor...
2017: Case Reports in Hematology
https://www.readbyqxmd.com/read/28319075/autologous-hematopoietic-stem-cell-transplantation-for-pediatric-multiple-sclerosis-a-registry-based-study-of-the-autoimmune-diseases-working-party-adwp-and-pediatric-diseases-working-party-pdwp-of-the-european-society-for-blood-and-marrow-transplantation-ebmt
#12
J Burman, K Kirgizov, K Carlson, M Badoglio, G L Mancardi, G De Luca, B Casanova, J Ouyang, R Bembeeva, J Haas, P Bader, J Snowden, D Farge
Autologous hematopoietic stem cell transplantation (aHSCT) is a promising therapy for multiple sclerosis (MS), which has mainly been used in adults. The purpose of this study was to investigate efficacy and adverse events of aHSCT in the treatment of children with MS using data from the European Society for Blood and Marrow Transplantation registry. Twenty-one patients with a median follow-up time of 2.8 years could be identified. PFS at 3 years was 100%, 16 patients improved in expanded disability status scale score and only 2 patients experienced a clinical relapse...
March 20, 2017: Bone Marrow Transplantation
https://www.readbyqxmd.com/read/28289528/clinical-cardiac-regenerative-studies-in-children
#13
REVIEW
Imre J Pavo, Ina Michel-Behnke
Although the incidence of pediatric heart failure is low, the mortality is relatively high, with severe clinical symptoms requiring repeated hospitalization or intensive care treatment in the surviving patients. Cardiac biopsy specimens have revealed a higher number of resident human cardiac progenitor cells, with greater proliferation and differentiation capacity, in the neonatal period as compared with adults, demonstrating the regeneration potential of the young heart, with rising interest in cardiac regeneration therapy in critically ill pediatric patients...
February 26, 2017: World Journal of Cardiology
https://www.readbyqxmd.com/read/28267083/toxic-epidermal-necrolysis-like-reaction-after-hematopoietic-stem-cell-transplantation-in-children
#14
Maura Faraci, Stefano Giardino, Edoardo Lanino, Giuseppe Morreale, Emilia Ghibaudo, Monica Francesia Berta, Marco Risso, Elio Castagnola, Mimmo Ripaldi, Andrea Moscatelli, Giovanni Ghigliotti
This study report clinical course, etiology, management, and long-term outcome of children who developed toxic epidermal necrolysis-like reaction (TEN-LR) after allogeneic hematopoietic stem cell transplantation (allo-HSCT). We retrospectively collected children with TEN-LR occurring after allo-HSCT performed in 2 pediatric bone marrow units between 2005 and 2014. We identified 6 cases of TEN-LR of 322 patients (1.8%). Possible triggers of TEN included antibiotics, antiepileptics, antimycotics, and Mycoplasma infection...
May 2017: Journal of Pediatric Hematology/oncology
https://www.readbyqxmd.com/read/28218757/allogeneic-stem-cell-transplantation-for-refractory-acute-myeloid-leukemia-in-pediatric-patients-the-uk-experience
#15
P O'Hare, G Lucchini, M Cummins, P Veys, M Potter, S Lawson, A Vora, R Wynn, A Peniket, K Kirkland, R Pearce, J Perry, P J Amrolia
We report outcomes for 44 children who underwent stem cell transplantation (SCT) for refractory AML in the UK between 2000 and 2012. Median age at SCT was 11.5 years. Twenty-three patients had primary refractory and 21 relapsed refractory AML. Refractory disease was confirmed by cytogenetics/molecular genetics in 24 cases. Median follow-up of the whole cohort is 6.8 years (2.1-14.9 years). Thirty patients (68%) achieved a CR following SCT. Transplant-related mortality at 1 year was 18%. Acute GVHD incidence was 52% (grade ⩾III 19%), chronic 7%...
February 20, 2017: Bone Marrow Transplantation
https://www.readbyqxmd.com/read/28174417/amkl-chimeric-transcription-factors-are-potent-inducers-of-leukemia
#16
J Dang, S Nance, J Ma, J Cheng, M P Walsh, P Vogel, J Easton, G Song, M Rusch, A L Gedman, C Koss, J R Downing, T A Gruber
Acute megakaryoblastic leukemia in patients without Down syndrome is a rare malignancy with a poor prognosis. RNA sequencing of fourteen pediatric cases previously identified novel fusion transcripts that are predicted to be pathologic including CBFA2T3-GLIS2, GATA2-HOXA9, MN1-FLI, and NIPBL-HOXB9. In contrast to CBFA2T3-GLIS2 which is insufficient to induce leukemia, we demonstrate that the introduction of GATA2-HOXA9, MN1-FLI1 or NIPBL-HOXB9 into murine bone marrow induces overt disease in syngeneic transplant models...
February 8, 2017: Leukemia: Official Journal of the Leukemia Society of America, Leukemia Research Fund, U.K
https://www.readbyqxmd.com/read/28162930/a-randomized-study-of-autologous-bone-marrow-derived-stem-cells-in-pediatric-cardiomyopathy
#17
E Sian Pincott, Deborah Ridout, Margaret Brocklesby, Angus McEwan, Vivek Muthurangu, Michael Burch
BACKGROUND: Bone marrow mononuclear cell fraction has been used as therapy for dilated cardiomyopathy in adults. Although case series are reported, there are no randomized controlled studies in children. METHODS: We designed a randomized, crossover, controlled pilot study to determine safety and feasibility of intracoronary stem cell therapy in children. The primary safety end-point was freedom from death and transplantation or any complication that could be considered related to bone marrow injection or anesthesia (e...
January 5, 2017: Journal of Heart and Lung Transplantation
https://www.readbyqxmd.com/read/28115275/current-knowledge-and-priorities-for-future-research-in-late-effects-after-hematopoietic-cell-transplantation-for-inherited-bone-marrow-failure-syndromes-consensus-statement-from-the-second-pediatric-blood-and-marrow-transplant-consortium-international-conference
#18
REVIEW
Andrew C Dietz, Parinda A Mehta, Adrianna Vlachos, Sharon A Savage, Dorine Bresters, Jakub Tolar, Farid Boulad, Jean Hugues Dalle, Carmem Bonfim, Josu de la Fuente, Christine N Duncan, K Scott Baker, Michael A Pulsipher, Jeffrey M Lipton, John E Wagner, Blanche P Alter
Fanconi anemia (FA), dyskeratosis congenita (DC), and Diamond Blackfan anemia (DBA) are 3 of the most common inherited bone marrow failure syndromes (IBMFS), in which the hematologic manifestations can be cured with hematopoietic cell transplantation (HCT). Later in life, these patients face a variety of medical conditions, which may be a manifestation of underlying disease or due to pre-HCT therapy, the HCT, or a combination of all these elements. Very limited long-term follow-up data exist in these populations, with FA the only IBMFS that has specific published data...
January 20, 2017: Biology of Blood and Marrow Transplantation
https://www.readbyqxmd.com/read/28112743/fertility-preservation-practices-in-pediatric-and-adolescent-cancer-patients-undergoing-hsct-in-europe-a-population-based-survey
#19
T Diesch, A Rovo, N von der Weid, M Faraci, M Pillon, A Dalissier, J-H Dalle, P Bader
Chemotherapy and irradiation can affect the gonads, leading to impairment of pubertal development and/or infertility. Fertility preservation (FP) is therefore a crucial endeavor in hematopoietic stem cell transplantation (HSCT) because of the severe impact of infertility on the quality of life of long-term survivors. Despite the existence of different international guidelines, FP counseling and procedures are not routinely implemented as part of patient care. We present herein a survey conducted by the Pediatric Working Party of the European Society for Blood and Marrow Transplantation (EBMT), which aims to analyze and compare different FP practices for children and adolescents across EBMT centers in 2013...
January 23, 2017: Bone Marrow Transplantation
https://www.readbyqxmd.com/read/28100970/sickle-cell-disease-with-cyanotic-congenital-heart-disease-long-term-outcomes-in-5-children
#20
Glen J Iannucci, Olufolake A Adisa, Matthew E Oster, Michael McConnell, William T Mahle
Sickle cell disease is a risk factor for cerebrovascular accidents in the pediatric population. This risk is compounded by hypoxemia. Cyanotic congenital heart disease can expose patients to prolonged hypoxemia. To our knowledge, the long-term outcome of patients who have combined sickle cell and cyanotic congenital heart disease has not been reported. We retrospectively reviewed patient records at our institution and identified 5 patients (3 girls and 2 boys) who had both conditions. Their outcomes were uniformly poor: 4 died (age range, 12 mo-17 yr); 3 had documented cerebrovascular accidents; and 3 developed ventricular dysfunction...
December 2016: Texas Heart Institute Journal
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