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Pediatric Hematology and Oncology

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https://www.readbyqxmd.com/read/29337597/hydroxyurea-for-lifelong-transfusion-dependent-%C3%AE-thalassemia-a-meta-analysis
#1
Ali H Algiraigri, Nicola A M Wright, Elizabeth Oddone Paolucci, Aliya Kassam
OBJECTIVE: Chronic blood transfusion remains the most feasible therapeutic option for lifelong transfusion-dependent β-thalassemia (lifelong TDβT). However, it is associated with serious risks and complications. Hydroxyurea (HU), an oral chemotherapeutic drug, is expected to increase hemoglobin levels, thereby minimizing the burden of blood transfusion and its complications. Growing literature over the last twenty years suggests promising results of the use HU in lifelong TDβT; however, its role and safety remain unanswered questions...
January 16, 2018: Pediatric Hematology and Oncology
https://www.readbyqxmd.com/read/29337595/outcome-of-extraocular-retinoblastoma-in-a-resource-limited-center-from-low-middle-income-country
#2
Gitika Pant, Nishant Verma, Archana Kumar, Vishal Pooniya, Sanjiv Kumar Gupta
Retinoblastoma (RB) is the most common ocular malignancy in children, and is managed by multimodal treatment. There is a paucity of data regarding the clinical profile and outcome of children with extraocular retinoblastoma from Low Middle Income Countries (LMIC) including India. Case records of children with newly diagnosed extraocular RB from January 2013 to August 2016 treated at our unit were analysed for clinical profile, treatment, and outcome. Over the 44 month study period, 91 children were diagnosed with RB, out of which 41 had extraocular disease...
January 16, 2018: Pediatric Hematology and Oncology
https://www.readbyqxmd.com/read/29303660/acute-megakaryoblastic-leukemia-excluding-down-syndrome-remains-an-acute-myeloid-subgroup-with-inferior-outcome-in-the-french-elam02-trial
#3
Anne-Charlotte Teyssier, Hélène Lapillonne, Marlene Pasquet, Paola Ballerini, André Baruchel, Stephane Ducassou, Odile Fenneteau, Arnaud Petit, Wendy Cuccuini, Christine Ragu, Claude Preudhomme, Thomas Mercher, Nicolas Sirvent, Guy Leverger
We report the outcome of 27 children with de novo acute megakaryoblastic leukemia (AMKL) (excluding Down syndrome) enrolled in the French multicenter prospective study ELAM02 (2005-2011). There was no difference in gender, initial leukocyte count, CNS involvement, and complete remission rate (88.9%), as compared to other acute myeloid leukemia (AML) subtypes. AMKL patients had a significantly poorer outcome (5-year overall survival 54% [CI 95% 33%-71%] than children with other AML subtypes (5-year overall survival 73% [CI 95% 68%-77%] p = 0...
January 5, 2018: Pediatric Hematology and Oncology
https://www.readbyqxmd.com/read/29303623/x-linked-hyper-igm-syndrome-a-phenotype-of-crohn-s-disease-with-hemophagocyticlymphohistiocytosis
#4
Kun-Yin Qiu, Xiong-Yu Liao, Ruo-Hao Wu, Ke Huang, Jian-Pei Fang, Dun-Hua Zhou
X-linked hyper-immunoglobulin M (IgM) syndrome is characterized by recurrent infections, low or undetectable levels of IgG and IgA, and normal to increased serum IgM, and is also rare. It is associated with mutation in the gene encoding CD40 ligand. This study aimed to describe the first international report of hemizygous CD40LG c.542G>A mutation in a 5-year-old boy with a phenotype of Crohn's disease and hemophagocyticlymphohistiocytosis. Also, the clinical implications of this mutation and associated atypical phenotype are discussed...
January 5, 2018: Pediatric Hematology and Oncology
https://www.readbyqxmd.com/read/29286874/malignant-peripheral-nerve-sheath-tumor-in-children-a-single-institute-retrospective-analysis
#5
Hong Yul An, Kyung Taek Hong, Hyoung Jin Kang, Jung Yoon Choi, CheRy Hong, Hyun-Young Kim, Tae Hyun Choi, Chang Hyun Kang, Han-Soo Kim, Jung-Eun Cheon, Sung-Hye Park, June Dong Park, Kyung Duk Park, Hee Young Shin
Malignant peripheral nerve sheath tumors are rare tumors that originate from Schwann cells. Patients with neurofibromatosis type 1 are prone to develop these tumors. Due to their rarity and lack of established treatment, the prognosis of malignant peripheral nerve sheath tumors is poor. A retrospective study was conducted on children treated for malignant peripheral nerve sheath tumors at the Seoul National University Children's Hospital between 2007 and 2016. Eleven patients were diagnosed with malignant nerve sheath tumors at a median age of 12 years, eight of whom had neurofibromatosis type 1...
December 29, 2017: Pediatric Hematology and Oncology
https://www.readbyqxmd.com/read/29232169/dietary-nonheme-iron-is-equally-bioavailable-from-ferritin-or-ferrous-sulfate-in-thalassemia-intermedia
#6
Monica Khurana, Ellen B Fung, Elliott P Vichinsky, Elizabeth C Theil
Transfusion-independent patients with thalassemia intermedia (TI) develop fatal iron overload from excessive iron absorption triggered by ineffective erythropoiesis. More information about iron pharmacokinetics and nonheme, dietary iron absorption in such patients is needed to optimize management. To obtain more information, different forms of supplemental nonheme iron sources (ferritin and ferrous sulfate) were compared in 4 TI (hemoglobin <9 g/dL) and 6 control (hemoglobin 12-16 g/dL) patients. Serial serum iron concentrations were measured during the 24 hours following consumption of 1 mg/kg of elemental iron as ferritin or ferrous sulfate...
December 12, 2017: Pediatric Hematology and Oncology
https://www.readbyqxmd.com/read/29227722/glucose-phosphate-isomerase-gpi-tadikonda-characterization-of-a-novel-pro340ser-mutation
#7
Ahmar U Zaidi, Prabhakar Kedar, Prasad Rao Koduri, Gerard W Goyette, Steven Buck, Donald E Paglia, Yaddanapudi Ravindranath
After a thirty-year lag, we serendipitously reestablished contact with a patient with glucose phosphate isomerase deficiency and hydrops fetalis first reported in 1987. We now provide a clinical update and provide results of mutation analysis in this patient, from Southern India. The patient now an adult female of 36 years of age has moderate anemia but requires no transfusions except with some intercurrent illnesses. Exome sequencing studies showed a homozygous c.1018C>T (Pro340Ser) mutation in exon 12 of the glucose phosphate isomerase gene and later confirmed by direct sequencing...
December 11, 2017: Pediatric Hematology and Oncology
https://www.readbyqxmd.com/read/29219666/coincidence-of-celiac-disease-with-nongastrointestinal-tumors-in-children
#8
Majid Naderi, Iraj Shahramian, Mojtaba Delaramnasab, Ali Bazi
The association of celiac disease (CD) with cancers of gastrointestinal origin has been noted. However, coincidence of CD with nongastrointestinal neoplasms is an unusual event. Here we present five children with concurrent CD and nongastrointestinal neoplasms. All of the patients had positive serologic results for anti-tTG antibodies. Histological investigation of intestinal mucosa showed inflammation (Marsh score = 2) in all the patients. Two of these patients represented with germ cell malignancies. One patient had Wilms' tumor...
December 8, 2017: Pediatric Hematology and Oncology
https://www.readbyqxmd.com/read/29211600/understanding-the-bone-marrow-microenvironment-in-hematologic-malignancies-a-focus-on-chemokine-integrin-and-extracellular-vesicle-signaling
#9
Edward Allan Racela Sison, Peter Kurre, Yong-Mi Kim
Signaling between leukemia cells and nonhematopoietic cells in the bone marrow microenvironment contributes to leukemia cell growth and survival. This complicated extrinsic mechanism of chemotherapy resistance relies on a number of pathways and factors, some of which have yet to be determined. Research on cell-cell crosstalk the bone marrow microenvironment in acute leukemia was presented at the 2016 annual Therapeutic Advances in Childhood Leukemia (TACL) investigator meeting. This review summarizes the mini-symposium proceedings and focuses on chemokine signaling via the cell surface receptor CXCR4, adhesion molecule signaling via integrin α4, and crosstalk between leukemia cells and the bone marrow microenvironment that is mediated through extracellular vesicles...
December 6, 2017: Pediatric Hematology and Oncology
https://www.readbyqxmd.com/read/29200325/incidence-and-risk-factors-of-bacterial-and-fungal-infection-during-induction-chemotherapy-for-high-risk-neuroblastoma
#10
Sarah B Whittle, Kaitlin C Williamson, Heidi V Russell
High-risk neuroblastoma is an aggressive childhood cancer with poor outcomes. Treatment begins with an induction phase comprised of intense multi-agent chemotherapy with the goal of maximally reducing tumor bulk. Given the high intensity of induction chemotherapy, neutropenic fever and infectious complications are common; however, the actual incidence is difficult to determine from clinical trial reports. We performed a retrospective review of infection-related complications in 76 children treated for high-risk neuroblastoma at Texas Children's Hospital...
December 4, 2017: Pediatric Hematology and Oncology
https://www.readbyqxmd.com/read/29200320/wiskott-aldrich-syndrome-two-case-reports-with-a-novel-mutation
#11
Karaman Kamuran, Mecnun Çetin, Hadi Geylan, Serap Karaman, Nihat Demir, Eyyup Yurekturk, İbrahim Yavuz, Göknur Yavuz, Oğuz Tuncer
BACKGROUND: The Wiskott-Aldrich syndrome (WAS) is X-linked recessive disorder associated with microplatelet thrombocytopenia, eczema, infections, and an increased risk of autoimmunity and lymphoid neoplasia. The originally described features of WAS include susceptibility to infections, microthrombocytopenia, and eczema. AIM: In this case report, we present our experience about two cases diagnosed with a new mutation. METHODS: We report phenotypical and laboratory description of two cases with WAS...
December 4, 2017: Pediatric Hematology and Oncology
https://www.readbyqxmd.com/read/29200317/pediatric-gist-presenting-as-anemia
#12
Paola Cianci, Chiara Luini, Maddalena Marinoni, Luigi Nespoli, Alessandro Salvatoni, Silvia Salvatore
Gastrointestinal stromal tumors (GIST) are tumors of the gastrointestinal (GI) tract originating from the myenteric ganglion cells (interstitial cells of Cajal), that are very rare in children and adolescents. The most common clinical manifestation is acute or chronic, overt or occult GI bleeding although these tumors are asymptomatic in 10-30% of patients. We report a case of gastric GIST in a 11-year-old girl presenting with an iron deficiency refractory anemia without gastrointestinal symptoms and stool evidence of GI bleeding that caused a slight diagnostic delay...
December 4, 2017: Pediatric Hematology and Oncology
https://www.readbyqxmd.com/read/29190182/checkpoint-inhibition-in-pediatric-hematologic-malignancies
#13
Kara L Davis, Archana M Agarwal, Anupam R Verma
Immune surveillance comprising of adaptive and innate immune systems is naturally designed to eliminate cancer development; overexpression of inhibitory receptors and their ligands prevent this check and lead to evasion and hence cancer progression and metastasis. The use of tumor-specific monoclonal antibodies (MAbs) targeting these checkpoint regulators is promising and has led to this novel field of cancer immunotherapy. The first antibody directed against cytotoxic T-lymphocyte associated protein 4 (CTLA-4), ipilimumab, showed promising results in clinical trials and was approved by the US Food and Drug Administration (FDA) for the treatment of metastatic melanoma in 2011...
November 30, 2017: Pediatric Hematology and Oncology
https://www.readbyqxmd.com/read/29190181/identifying-opportunities-to-bridge-disparity-gaps-in-curing-childhood-cancer-in-malawi-malignancies-with-excellent-curative-potential-account-for-the-majority-of-diagnoses
#14
Nader Kim El-Mallawany, Peter Wasswa, Idah Mtete, Mercy Mutai, Christopher C Stanley, Mary Mtunda, Mary Chasela, Atupele Mpasa, Stella Wachepa, William Kamiyango, Jimmy Villiera, Peter N Kazembe, Parth S Mehta
The majority of African children with cancer die without access to resources. We describe efforts to build a public treatment program with curative intent for childhood cancer in Lilongwe, Malawi despite severe limitations in diagnostic and therapeutic resources. We retrospectively analyzed a cohort of childhood cancer patients at Kamuzu Central Hospital from 12/2011-6/2013. Consistently available chemotherapeutic agents were limited to cyclophosphamide, vincristine, doxorubicin, bleomycin, methotrexate, and prednisone...
November 30, 2017: Pediatric Hematology and Oncology
https://www.readbyqxmd.com/read/29190176/correlation-of-serum-ferritin-levels-with-hepatic-mri-t2-and-liver-iron-concentration-in-nontransfusion-beta-thalassemia-intermediate-patients-a-contemporary-issue
#15
Mehran Karimi, Fatemeh Amirmoezi, Sezaneh Haghpanah, Seyedpouria Ostad, Mehrzad Lotfi, Sepideh Sefidbakht, Shahed Rezaian
BACKGROUND: Beta-thalassemia intermediate is a genetic disease that is milder than beta-thalassemia major. The T2* magnetic resonance imaging (MRI) technique is currently the gold standard for iron load detection. However, it is expensive and needs an expert radiologist to report findings. Therefore, we conducted this study to determine an optimal cut-off value of ferritin in proportion to T2 MRI of liver and measurement of liver iron concentration for early detection of hepatic iron overload in Beta-thalassemia intermediate patients...
November 30, 2017: Pediatric Hematology and Oncology
https://www.readbyqxmd.com/read/29190169/tacl-ing-supportive-care-needs-in-pediatric-early-phase-clinical-trials-for-acute-leukemia-a-report-from-the-therapeutic-advances-in-childhood-leukemia-lymphoma-tacl-consortium-supportive-care-committee
#16
E Orgel, J J Auletta
A Supportive Care Committee was recently developed within the Therapeutic Advances in Childhood Leukemia & Lymphoma (TACL) Consortium. This was substantiated by the significantly high rate of serious adverse events (SAE) (CTCAE Grade ≥3 toxicity) experienced by patients with relapse/refractory acute leukemia enrolled on our phase I trials. Such treatment-related toxicity has resulted in patients being removed from study and thus potentially not receiving clinical benefit from the novel therapy. In addition, increased treatment-related toxicity may compromise new agents from moving forward in their clinical development...
November 30, 2017: Pediatric Hematology and Oncology
https://www.readbyqxmd.com/read/29190168/the-effect-of-anti-thyroid-antibodies-positivity-on-children-with-primary-immune-thrombocytopenia
#17
Suzan O Mousa, Gamal T Soliman, Ahmed A-F Saedii, Emad N Hameed
Primary immune thrombocytopenia (ITP) is the most common cause of acquired thrombocytopenia in children. Anti-thyroid antibodies (aTA) have previously been found to be present in various autoimmune diseases. Our aim was to study the effect of aTA positivity (which are anti-thyroid peroxidase (aTPo) and/or anti-thyroglobulin (aTg)) on children with primary immune thrombocytopenia and their relation to treatment response. Sixty-one children with primary ITP were enrolled in the present study. They were further subdivided into: ND&P group (newly diagnosed and persistent) and chronic ITP group...
November 30, 2017: Pediatric Hematology and Oncology
https://www.readbyqxmd.com/read/29190164/investigating-the-biology-of-relapsed-acute-leukemia-proceedings-of-the-therapeutic-advances-for-childhood-leukemia-lymphoma-tacl-consortium-biology-working-group
#18
Deepa Bhojwani, Michael J Burke, Terzah Horton, David S Ziegler, Maria Luisa Sulis, Kirk R Schultz, Alan Wayne, Shai Izraeli, Bill H Chang
During the 2016 Therapeutic Advances for Childhood Leukemia & Lymphoma (TACL) Consortium investigators' meeting (Los Angeles, CA), a Biology Working Group was established to support the consortium's mission of developing innovative therapies for currently incurable childhood leukemias and lymphomas. The charge of the Biology Working Group was to address how TACL could advance biological investigations of pediatric relapsed/refractory hematologic malignancies while undertaking forward-looking therapeutic trials...
November 30, 2017: Pediatric Hematology and Oncology
https://www.readbyqxmd.com/read/29190162/redefining-treatment-failure-for-pediatric-acute-leukemia-in-the-era-of-minimal-residual-disease-testing
#19
Van Huynh, Theodore W Laetsch, Reuven J Schore, Paul Gaynon, Maureen M O'Brien
Technologies for the detection of minimal residual disease (MRD) in leukemia and our understanding of the prognostic implications of MRD at different phases of treatment have significantly improved over the past decade. As a result, definitions of treatment failure based on bone marrow morphology by light microscopy are becoming increasingly inadequate for clinical care and trial design. In addition, novel therapies that may have increased efficacy and decreased toxicity in the setting of MRD compared to overt disease are changing clinical practice and challenging investigators to redefine treatment failure, the role of disease surveillance in remission, and clinical trial eligibility in the era of MRD...
November 30, 2017: Pediatric Hematology and Oncology
https://www.readbyqxmd.com/read/29148865/metronomic-therapy-has-low-toxicity-and-is-as-effective-as-current-standard-treatment-for-recurrent-high-risk-neuroblastoma
#20
Frank Berthold, Marc Hömberg, Inna Proleskovskaya, Pavel Mazanek, Margarita Belogurova, Angela Ernst, Jaroslav Sterba
The metronomic therapy concept uses low doses of continuously applied chemotherapeutic, anti-angiogenetic, and immunomodulating drugs. Twenty patients with recurrent and 3 with refractory high-risk neuroblastoma were treated by the metronomic concept using celecoxib, cyclophosphamide, vinblastine, and etoposide for up to 24 months. The outcome was compared to 274 matched patients with a first recurrence from stage 4 neuroblastoma using the variables time from diagnosis to first recurrence, number of organs involved, and MYCN amplification...
November 17, 2017: Pediatric Hematology and Oncology
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