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Journal of Pediatric Hematology/oncology

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https://www.readbyqxmd.com/read/29462001/infantile-osteopetrosis
#1
Liron Barnea Slonim, Chana Glasser
No abstract text is available yet for this article.
February 16, 2018: Journal of Pediatric Hematology/oncology
https://www.readbyqxmd.com/read/29462000/clinical-burdens-of-%C3%AE-thalassemia-major-in-affected-children
#2
Huda Gharaibeh, Moussa A Barqawi, Khetam Al-Awamreh, Mohammed Al Bashtawy
BACKGROUND: β-thalassemia major (BTM) is an inherited blood disorder leading to severe anemia. A better understanding of BTM complications can be considered an important factor in developing effective health care provision. METHOD: A descriptive exploratory design was used to identify the clinical burden of BTM from affected children's perspective. A convenience sample of 45 patients with BTM, accompanied by a family member, was recruited from a governmental hospital during April-May 2015...
February 16, 2018: Journal of Pediatric Hematology/oncology
https://www.readbyqxmd.com/read/29432315/dexrazoxane-significantly-reduces-anthracycline-induced-cardiotoxicity-in-pediatric-solid-tumor-patients-a-systematic-review
#3
Kelly Liesse, Jamie Harris, Megan Chan, Mary L Schmidt, Bill Chiu
Cardiotoxicity is a dose-limiting and potentially lethal complication of anthracycline administration. Previous studies failed to determine definitive toxic doses or cardioprotective factors. Current dosing strategies may utilize unnecessarily high anthracycline doses, such that survival benefit may not outweigh increased toxicity rates. A systematic review of randomized controlled trials and prospective/retrospective studies investigating anthracycline treatment in pediatric solid tumors was performed from PubMed/MEDLINE and Cochrane databases...
February 9, 2018: Journal of Pediatric Hematology/oncology
https://www.readbyqxmd.com/read/29432314/retroperitoneal-angiomatoid-fibrous-histiocytoma-presenting-as-a-recurrent-spontaneous-retroperitoneal-hemorrhage-in-a-9-year-old-boy
#4
Jonathan C Slack, Carlos Sanchez-Glanville, MacGregor Steele, Andrew L Wong, Marie-Anne Bründler
Angiomatoid fibrous histiocytoma (AFH) is a rare soft tissue tumor that typically presents in children and young adults. Occurrence outside of the extremities and the head and neck region is exceedingly rare. We report the case of a 9-year-old boy who presented with recurrent retroperitoneal hemorrhage initially thought to be a manifestation of an underlying bleeding disorder. After comprehensive diagnostic work-up, including multiple negative biopsies, the patient underwent surgical resection of an extensively hemorrhagic intramuscular mass and to date remains well...
February 9, 2018: Journal of Pediatric Hematology/oncology
https://www.readbyqxmd.com/read/29432313/cup-like-blasts-in-2-pediatric-patients-with-npm-1-positive-acute-myeloid-leukemia
#5
Özlem Tüfekçi, Melek Erdem, Hale Ören, Şebnem Yilmaz
Cup-like phenotype is defined in some subtypes of acute myeloid leukemia (AML) and have been associated with NPM-1 and/or FLT3-ITD positivity in the presence of normal karyotype in >60% of patients. Herein we present two pediatric AML-M1 patients with cuplike nuclear morphology and NPM-1 positivity. Both patients were negative for FLT3-ITD mutation. NPM-1 mutation and FLT3-ITD mutation should be kept in mind in AML patients with cup-like blast morphology as these two mutations are important molecular markers for prognosis, risk group classification and also for response to treatment...
February 9, 2018: Journal of Pediatric Hematology/oncology
https://www.readbyqxmd.com/read/29432312/survival-following-tumor-recurrence-in-children-with-medulloblastoma
#6
Donna L Johnston, Daniel Keene, Douglas Strother, Maria Taneva, Lucie Lafay-Cousin, Chris Fryer, Katrin Scheinemann, Anne-Sophie Carret, Adam Fleming, Samina Afzal, Beverly Wilson, Lynette Bowes, Shayna Zelcer, Chris Mpofu, Mariana Silva, Valerie Larouche, Josee Brossard, Eric Bouffet
Medulloblastoma is the most common malignant brain tumor in children. Published survival rates for this tumor are ∼70%; however, there is limited published information on outcome after disease recurrence. This was an observational study which included all persons under the age of 18 years diagnosed with medulloblastoma from 1990 to 2009 inclusive in Canada. Data collected included date of diagnosis, age at diagnosis, sex, stage, pathology, treatment, recurrence, and current status. Survival rates were determined...
February 9, 2018: Journal of Pediatric Hematology/oncology
https://www.readbyqxmd.com/read/29432311/a-rare-cause-of-secondary-immunodeficiency-generalized-lymphatic-anomaly
#7
Saliha Esenboga, Deniz Çagdas, Berna Oguz, Inci Bajin, Burca Aydin, Canan Akyuz, Ilhan Tezcan
No abstract text is available yet for this article.
February 9, 2018: Journal of Pediatric Hematology/oncology
https://www.readbyqxmd.com/read/29432310/a-congenital-case-of-ependymoblastoma-a-rare-and-aggressive-brain-tumor
#8
Courtney D Grassham, Sara Rady, Kimberly Foster, Karen S SantaCruz, John F Kuttesch, Jessie R Maxwell
Congenital brain tumors are rare, representing <2% of all childhood brain tumors. Of these, ependymoblastoma is a profoundly aggressive embryonal brain tumor that is included in the diagnostic entity known as an embryonal tumor with multilayered rosettes. This report of a congenital ependymoblastoma diagnosed at birth aims to highlight how much remains unknown about embryonal tumor with multilayered rosettes and the devastating prognosis of this condition. Despite recent advancements made in identifying molecular targets for therapy, this tumor continues to have a high rate of recurrence with few successful treatment options, especially when diagnosed in the newborn period...
February 9, 2018: Journal of Pediatric Hematology/oncology
https://www.readbyqxmd.com/read/29432309/maternal-exposure-to-pesticides-paternal-occupation-in-the-army-police-force-and-cyp2d6-4-polymorphism-in-the-etiology-of-childhood-acute-leukemia
#9
Giovanni M Ferri, Chiara M Guastadisegno, Graziana Intranuovo, Domenica Cavone, Francesco Birtolo, Valerio Cecinati, Brigida Pappalardi, Patrizia Corsi, Luigi Vimercati, Nicola Santoro
Epidemiologic studies have suggested that parental occupations, pesticide use, environmental factors, and genetic polymorphism are involved in the etiology of childhood acute leukemia (CAL). In total, 116 cases of CAL and 162 controls were recruited and submitted to blood drawing to assess the presence of genetic polymorphisms. Parental occupations, pesticides exposure, and other potential determinants were investigated. Increased risk for CAL was associated with prenatal maternal use of insecticides/rodenticides (odds ratio [OR]=1...
February 9, 2018: Journal of Pediatric Hematology/oncology
https://www.readbyqxmd.com/read/29432308/abvd-without-radiation-for-newly-diagnosed-pediatric-and-young-adult-patients-with-hodgkin-lymphoma-a-single-center-retrospective-analysis-of-28-consecutive-patients
#10
Elliot Stieglitz, Tu Dinh, Andrew S Phelps, Miguel H Pampaloni, Adam B Olshen, Elizabeth Robbins
Hodgkin lymphoma (HL) is the most common malignancy affecting adolescents and young adults. Treatment with a combination of chemotherapy and radiation results in cure rates of >90%. However, radiation therapy causes significant late effects and avoiding radiation entirely for patients who respond to chemotherapy is an accepted strategy. Since 2011, 28 consecutive patients diagnosed with classic HL have been treated with doxorubicin, bleomycin, vinblastine, and dacarbazine (ABVD) for 4 to 6 cycles. Patients who achieved a complete metabolic response (CMR) as assessed by [F] fluorodeoxyglucose positron emission tomography by the end of chemotherapy did not receive radiation...
February 9, 2018: Journal of Pediatric Hematology/oncology
https://www.readbyqxmd.com/read/29432307/extranodal-hodgkin-lymphoma-of-the-bone-marrow-without-lymphadenopathy-and-unusual-cytogenetic-translocation
#11
Karen S Fernández, Eric Bugaieski, Diane K Jordan
No abstract text is available yet for this article.
February 9, 2018: Journal of Pediatric Hematology/oncology
https://www.readbyqxmd.com/read/29432306/parvovirus-b19-associated-hemophagocytic-lymphohistiocytosis-in-a-patient-with-glucose-6-phosphate-dehydrogenase-deficiency
#12
Aida Zečkanović, Matej Perovnik, Janez Jazbec, Miroslav Petrovec, Marko Pokorn, Marko Kavčič
We report a case of a 12-year-old male with glucose-6-phosphate dehydrogenase deficiency presenting with clinical signs of sepsis and pancytopenia. Investigations revealed parvovirus B19 (PVB19)-associated hemophagocytic lymphohistiocytosis (HLH). The patient recovered fully and quickly with symptomatic treatment. Current evidence suggests that PVB19-associated HLH has a favorable prognosis. Mild undiagnosed cases of HLH may be the cause of pancytopenia in PVB19 infections.
February 9, 2018: Journal of Pediatric Hematology/oncology
https://www.readbyqxmd.com/read/29432305/pediatric-bronchial-carcinoid-tumors-a-case-series-and-review-of-the-literature
#13
Samara L Potter, Josephine HaDuong, Fatih Okcu, Hao Wu, Murali Chintagumpala, Rajkumar Venkatramani
Bronchial carcinoid tumor, while rare, remains the most common primary malignant lung tumor in children. We present a retrospective analysis of 7 patients with typical bronchial carcinoid tumors diagnosed at 2 pediatric tertiary care referral centers between 1990 and 2014. The most common presenting symptom was pneumonia, followed by respiratory distress. Somatostatin scans were performed in selected patients. All patients had negative resection margin following surgery and were alive without disease at last follow-up...
February 9, 2018: Journal of Pediatric Hematology/oncology
https://www.readbyqxmd.com/read/29432304/anemia-in-adolescents-and-young-adult-patients-with-congenital-heart-disease
#14
Juan L Rodríguez-Hernández, Fayna Rodríguez-González, Efrén Martínez-Quintana
INTRODUCTION: Despite anemia in acquired heart disease being a common problem, little is known in patients with congenital heart disease (CHD). METHODS: In total, 544 consecutive stable noncyanotic CHD patients were studied to determine demographic, clinical, and analytic parameters. Anemia was defined as a condition in which hemoglobin concentration was <13 g/dL in male individuals and <12 g/dL in female individuals. RESULTS: In total, 49 (9%) CHD patients had anemia...
February 9, 2018: Journal of Pediatric Hematology/oncology
https://www.readbyqxmd.com/read/29432303/utility-of-echocardiography-as-screening-for-late-onset-anthracycline-induced-cardiotoxicity-in-pediatric-cancer-survivors-observations-from-the-first-decade-after-end-of-therapy
#15
Diana J Moke, Lynn E Schubert, Heather Y Sun, Beth F Printz, Andrew C Dietz
Current screening guidelines are available for anthracycline-induced cardiotoxicity. However, the utility of echocardiogram screening for late-onset anthracycline cardiotoxicity especially in the decade immediately after end of therapy is debatable. A retrospective chart review of patients seen in the Thriving after Cancer Clinic at Rady Children's Hospital January 2006 to December 2013 was performed. Treatment data, echocardiogram results, cardiology referral notes and cardiac medication data were abstracted from anthracycline-exposed survivors...
February 9, 2018: Journal of Pediatric Hematology/oncology
https://www.readbyqxmd.com/read/29432302/allogeneic-matched-related-donor-bone-marrow-transplantation-for-pediatric-patients-with-severe-aplastic-anemia-using-low-dose-cyclophosphamide-atg-plus-fludarabine
#16
Chayamon Takpradit, Susan E Prockop, Nancy A Kernan, Andromachi Scaradavou, Kevin Curran, Julianne Ruggiero, Nicole Zakak, Richard J O'Reilly, Farid Boulad
BACKGROUND: The combination of cyclophosphamide (CY) and antithymocyte globulin (ATG) has been used as a standard conditioning regimen for matched related donor transplantation in patients with severe aplastic anemia. PROCEDURE: To decrease the regimen-related toxicity while maintaining appropriate engraftment and survival rates, fludarabine (FLU) was added to the regimen. Four pediatric patients received matched related donor bone marrow transplantation with CY (50 mg/kg×2) (instead of the 50 mg/kg×4 standard dosing), equine ATG (30 mg/kg×3), with the addition of FLU (30 mg/m×4)...
February 9, 2018: Journal of Pediatric Hematology/oncology
https://www.readbyqxmd.com/read/29420373/the-zinc-and-copper-levels-in-thalassemia-major-patients-receiving-iron-chelation-therapy
#17
Omid R Zekavat, Ayda Bahmanjahromi, Sezaneh Haghpanah, Sara Ebrahimi, Nader Cohan
Essential trace elements deficiency including zinc and copper are frequently reported in the literature, but the results are controversial. The aim of this study was to evaluate zinc and copper levels in thalassemia major (TM) patients who were on regular transfusion and iron chelation therapy. In a case-control cross-sectional study 43 TM patients and 43 age-matched and sex-matched healthy controls were examined. Patients were selected by convenience sampling method from TM patients who were registered in Thalassemia Clinic during 6 months...
February 7, 2018: Journal of Pediatric Hematology/oncology
https://www.readbyqxmd.com/read/29420372/mutation-screening-of-the-kr%C3%A3-ppel-like-factor-1-gene-in-individuals-with-increased-fetal-hemoglobin-referred-for-hemoglobinopathy-investigation-in-south-of-iran
#18
Mohammad Hamid, Sanaz Ershadi Oskouei, Gholamreza Shariati, Esmaeil Babaei, Hamid Galehdari, Alihossein Saberi, Alireza Sedaghat
BACKGROUND: Any mutation in the Krüppel-like factor 1 (KLF1) gene may interfere with its proper related function in the erythropoiesis process and lead to alterations in proper activation of its downstream protein through globin switching, which results in an increase in fetal hemoglobin (HbF). This study aimed to investigate whether KLF1 mutation can associate with high level of HbF in individuals with increased fetal hemoglobin referred for screening of hemoglobinopathies in south of Iran...
February 7, 2018: Journal of Pediatric Hematology/oncology
https://www.readbyqxmd.com/read/29420371/high-dose-chemotherapy-is-efficacious-and-well-tolerated-in-a-toddler-with-aicardi-syndrome-and-malignant-sacrococcygeal-teratoma
#19
Jessica D Wharton, Shaina Johnson, James A Connelly, Tracy Hills, Lauren Gingles, Margaret Wood, Gabriella L Crane, Howard M Katzenstein
Aicardi syndrome (AS) is a rare neurodevelopmental disorder, predominantly seen in female individuals, which appears to have an increased risk of both benign and malignant neoplasia. We report the case of a child with AS who presented with metastatic malignant sacrococcygeal tumor (with yolk sac elements) which recurred and then was treated with 3 cycles of high-dose chemotherapy with autologous stem cell rescue. The patient tolerated therapy with acceptable toxicity and remains in clinical remission 3 months after the completion of therapy...
February 7, 2018: Journal of Pediatric Hematology/oncology
https://www.readbyqxmd.com/read/29420370/hepatoblastoma-in-a-child-with-early-onset-cirrhosis
#20
Julie Bennett, Melanie Kirby-Allen, Vicky Ng, John S Waye, Catherine T Chung, Furqan Shaikh
Hepatoblastoma is the most common hepatic malignancy of childhood with known genetic predispositions and perinatal risk factors, with rare case reports occurring in the setting of cirrhosis. This case describes a young patient with cirrhosis attributed to early-onset hereditary hemochromatosis who was diagnosed with hepatoblastoma with uncommon histologic findings, evidence of chemotherapy resistance who ultimately succumbed to her disease. It is important to consider diagnoses beyond hepatocellular carcinoma in this scenario and consider early biopsy...
February 7, 2018: Journal of Pediatric Hematology/oncology
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