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Journal of Pediatric Hematology/oncology

Jose L Lepe-Zuniga, Virginia Ramirez-Nova
No abstract text is available yet for this article.
December 4, 2018: Journal of Pediatric Hematology/oncology
Heather K Schopper, Christopher F D'Esposito, John S Muus, Julie Kanter, Ted A Meyer
This study sought to examine if modern medical evaluations including newborn screening and early diagnosis along with better methods of disease control have improved rates of hearing loss in children with sickle cell disease (SCD). Audiometric and medical data for patients with SCD was obtained from the AudGen Database and analyzed for the presence of hearing loss, type of hearing loss, severity of hearing loss, and correlation with comorbid conditions. Children with sickle cell trait (SCT) were used as a comparison group...
December 4, 2018: Journal of Pediatric Hematology/oncology
Samuel A Adegoke, Olufemi S Smith, Adewuyi T Adeniyi, Adekunle D Adekile
BACKGROUND: Thrombospondin-1 (TSP-1) and 25-hydroxyvitamin D (25-OHD) play significant roles in the pathogenesis of sickle cell anemia (SCA). TSP-1 enhances cellular adhesion/inflammation, hence contributing to vaso-occlusive crisis (VOC); vitamin D, in contrast, retards inflammation and may lower rate of pain episodes. We determined serum levels of TSP-1 and 25-OHD in Nigerian children with SCA and their matched hemoglobin AA controls; and assess the relationship between the 2 biomarkers...
December 4, 2018: Journal of Pediatric Hematology/oncology
Kamleshun Ramphul, Stephanie G Mejias, Jyotsnav Joynauth
No abstract text is available yet for this article.
November 28, 2018: Journal of Pediatric Hematology/oncology
Emine E Yalinbaş, Melike Sezgin Evim, Özcan Bör, Zafer Gülbaş
OBJECTIVE: Immune thrombocytopenia (ITP) is an autoimmune disease, and it has become evident that T lymphocytes play an important role in the pathogenesis of ITP. We investigated the role of T helper (Th) intracellular IL-2, IL-4, IL-6, IFN-γ, and T lymphocyte apoptosis in the pathogenesis of acute ITP and the effect of glucocorticoid treatment on cytokine profile. We investigated also P-glycoprotein (P-gp) and glucocorticoid receptor (GCR) expression as a possible mechanism for glucocorticoid resistance...
November 28, 2018: Journal of Pediatric Hematology/oncology
Masaharu Akiyama, Masayoshi Yamaoka, Wataru Ohyama, Kentaro Yokoi, Shuichi Ashizuka, Daisuke Aizawa, Masahiro Ikegami, Hideaki Suzuki, Koji Ozaki, Hiroyuki Ida, Yuki Yuza
We report on a 16-year-old Japanese boy in whom an esophageal squamous cell carcinoma (ESCC) developed 12 years after allogeneic hematopoietic stem cell transplantation was performed for aplastic anemia. A high frequency of microsatellite instability was detected in samples of ESCC. Moreover, the detection of pathogenic variants, including single nucleotide substitution of TP53 (c.346C>T) and BRCA2 (c.6952C>T) and splicing of KDM6A (c.1194+2T>G), suggest that the development of ESCC in the patient was triggered by impairment of checkpoint and repair for DNA damage and epigenetic modification through accumulation of gene mutations induced by chronic graft-versus-host disease and prolonged administration of tacrolimus...
November 28, 2018: Journal of Pediatric Hematology/oncology
Maria A Pereda, Jaya Isaac, Yaoping Zhang, Rajeswari Jayakumar, Raavi Gupta, Scott T Miller
Massive splenic infarction (MSI) is a rare complication of sickle cell disease, as the spleen generally atrophies within the first few years of life. We report a case of MSI in a 12-year-old boy with homozygous sickle cell anemia (Hb SS) whose chronic transfusion therapy resulted in hypersplenism. The occurrence of a complicated MSI in our patient should perhaps further encourage elective splenectomy in such patients, despite known potential perioperative complications and postsplenectomy risks of infection and thrombosis...
November 28, 2018: Journal of Pediatric Hematology/oncology
Alana Goldstein-Leever, James L Peugh, Charles T Quinn, Lori E Crosby
Adolescents and young adults (AYA) with sickle cell disease (SCD) are at risk for poor health-related quality of life (HRQOL). Research suggests that vulnerability factors (eg, disease severity) and self-management resources (eg, disease self-efficacy) jointly impact health outcomes, including HRQOL; however, this has not been studied among AYA with SCD. This study examined the relationship between disease self-efficacy, HRQOL, and disease severity in AYA with SCD. HRQOL was positively correlated with disease self-efficacy and negatively correlated with disease severity...
November 28, 2018: Journal of Pediatric Hematology/oncology
Bilal Moaed, Oz Mordechai, Myriam Weyl Ben-Arush, Sharon Tamir, Ruth Ofir, Sergey Postovsky
BACKGROUND: It is currently expected that about 20% of children with cancer will ultimately die. Writing advanced life directives sufficiently long before the actual death of a child ensues allows both parents and medical staff to develop optimal treatment plans in the best interests of the child. AIM OF THE STUDY: The aim of the study was to evaluate factors that may influence the process of decision-making regarding Do-Not-Resuscitate (DNR) status. METHODS: Retrospective single institution study...
November 28, 2018: Journal of Pediatric Hematology/oncology
Maria Elena Laino, Serena Monti, David S Klimstra, Laura H Tang, Lorenzo Mannelli
A 7-year-old patient with a stage 4 neuroblastoma underwent chemotherapy, surgery, myeloablative therapy, external beam radiotherapy, and Isoretinoin treatment. A posttreatment magnetic resonance examination performed administering gadoteric acid as contrast agent showed 2 new hypervascular hepatic lesions, suspicious for metastases. A second magnetic resonance imaging performed using a liver-specific contrast medium (gadolinium ethoxybenzyl diethylenetriamine pentaacetic acid) demonstrated that these lesions were consistent with treatment-related focal nodular hyperplasia...
November 28, 2018: Journal of Pediatric Hematology/oncology
Beryl Lin, Chimene Kesserwan, Emily A Quinn, Stephanie L Einhaus, Karen D Wright, Elizabeth M Azzato, Brent A Orr, Santhosh A Upadhyaya
Coffin-Siris syndrome (CSS) is a rare congenital disorder with variable clinical phenotype consisting of developmental delay and characteristic facial features. It is caused by mutations in the chromatin remodeling switch/sucrose nonfermenting complex. Although SWI/SNF genes are widely implicated in tumorigenesis, only 8 cases of neoplasm have been reported in patients with CSS. We report a case of anaplastic astrocytoma (WHO grade III) in an 18-month-old child with CSS due to a de novo germline missense SMARCE1 mutation...
November 28, 2018: Journal of Pediatric Hematology/oncology
Işik Odaman Al, Cengiz Bayram, Başak Koç, Gizem Ersoy, Ezgi Pasli Uysalol, Hilal Aki, Gül Nihal Özdemir
BACKGROUND: Burkitt leukemia (BL) with the precursor B-cell immunophenotype is a rarely reported condition. The prognosis of such patients is similar to that of classic BL. However, the combination of chromosomal translocations associated with bcl-2 and c-myc rearrangement has a poor prognosis. OBSERVATIONS: An 11-year-old child presented with fever and weakness. Bone marrow aspiration showed morphologically L1 type blasts and flow cytometry analysis was compatible with precursor B-cell immunophenotype...
November 28, 2018: Journal of Pediatric Hematology/oncology
Deniz Yilmaz Karapinar, Hamiyet Hekimci Özdemir, Burcu Akinci, Akkiz Şahin Yaşar, Zuhal Önder Siviş, Hüseyin Onay, Ferda Özkinay
Severe Congenital Neutropenia (SCN) is a rare inherited disease characterized by an absolute neutrophil count (ANC) lower than 500/μL. Genetic heterogeneity and biallelic CSF3R mutation has rarely been identified as an underlying genetic defect in SCN. The majority of SCN patients respond to granulocyte colony stimulating factor treatment; however, in patients with inherited CSF3R mutation, ANC cannot generally be increased with granulocyte colony stimulating factor treatment. In such cases, granulocyte macrophage colony stimulating factor presents as an effective treatment option...
November 28, 2018: Journal of Pediatric Hematology/oncology
Chantel Cacciotti, Brian Leber, Stephanie Cox, Jorge Arredondo, Ereny Bassilious, Uma Athale
Hand mirror cell (HMC) leukemia is a variant of acute lymphoblastic leukemia previously described in the adult population where lymphoblasts manifest distinctive hand mirror morphologic features. HMC has been previously identified in 23% of childhood acute lymphoblastic leukemia patients, but its prognostic significance in children is not well understood. Hypercalcemia is also uncommon in childhood leukemias. Hypercalcemia associated with HMC leukemia has not been previously reported. We report a 5-year-old boy with HMC B-lymphoblastic leukemia who presented with hypercalcemia...
November 20, 2018: Journal of Pediatric Hematology/oncology
Rachel Visage, Nicole Kaiser, Megan Williams, Abby Kim
A nationwide shortage of intravenous (IV) sodium bicarbonate required institutions to explore alternative options for urinary alkalinization for high-dose methotrexate (HDMTX). Children's Hospital Colorado implemented a protocol utilizing oral alkalinizing agents as alternatives to intravenous sodium bicarbonate during the shortage. The purpose of this study was to determine the safety and efficacy of oral alkalinization strategies for HDMTX administration. This retrospective study was conducted at a pediatric institution and evaluated cycles of HDMTX administered with at least one dose of oral sodium bicarbonate tablets or sodium citrate-citric acid oral solution...
November 20, 2018: Journal of Pediatric Hematology/oncology
Patrycja Zalas-Więcek, Krzysztof Czyżewski, Tomasz Bogiel, Eugenia Gospodarek-Komkowska, Mariusz Wysocki
Enterobacterales represent a serious threat to transplant patients due to their increase frequency of carbapenem resistance and wide spreading. We present a case of an infant with acute lymphoblastic leukemia undergoing hematopoietic stem cell transplantation. Before transplantation an unusual double colonization of the gastrointestinal tract with extremely resistant Escherichia coli and Klebsiella pneumoniae strains producing metallo-beta-lactamase was diagnosed. Respective epidemiological management was implemented, based on the strict reverse isolation in patient-protective environment, and intensified antimicrobial surveillance...
November 20, 2018: Journal of Pediatric Hematology/oncology
Mustafa A Yoruk, Cigdem U Durakbasa, Cetin Timur, Seyma S Sahin, Esra C Taskin
In total, 74 pediatric oncology patients with hematologic malignancies (n=56) or solid tumors (n=18) and a median age of 78.5 months were included in this prospective study. The aims were to assess malnutrition risks and nutritional status over a 6-month treatment period measured at regular intervals. The rate of patients with high risk for malnutrition at diagnosis was 28.4% by Screening Tool for Risk of Impaired Nutritional Status and Growth tool and 36.5% by Pediatric Yorkhill Malnutrition Score. Body mass index (BMI) z-scores at diagnosis showed 12...
November 20, 2018: Journal of Pediatric Hematology/oncology
Tomasz Ociepa, Magdalena Bartnik, Karolina Zielezińska, Tomasz Urasiński
BACKGROUND: Childhood acute lymphoblastic leukemia (ALL) survivors are at an increased risk of cardiovascular disease including arterial hypertension (AH). The objectives of this study were to assess the prevalence of AH using 24-hour ambulatory blood pressure monitoring, explore characteristics of AH, and define risk factors for the development of AH in childhood ALL survivors. PATIENTS AND METHODS: The study comprised 81 childhood ALL survivors (aged, 5 to 25 y) after a median follow-up time of 5 years...
November 20, 2018: Journal of Pediatric Hematology/oncology
Anselm Chi-Wai Lee
Macropolycytes are tetraploid neutrophils produced during accelerated myelopoiesis. They have been reported in adults with pernicious anemia, sepsis, and after cytotoxic chemotherapy. Two pediatric cases are reported, one after granulocyte colony-stimulating factor treatment and the other following Kawasaki disease, respectively.
November 20, 2018: Journal of Pediatric Hematology/oncology
Amilie Boonstra, Marieke Gielissen, Eline van Dulmen-den Broeder, Nicole Blijlevens, Hans Knoop, Jacqueline Loonen
OBJECTIVES: Fatigue is a common and disabling late effect in childhood cancer survivors (CCS). In this pilot study, the effectiveness of cognitive behavior therapy (CBT) in CCS with persistent and severe fatigue was retrospectively evaluated. MATERIALS AND METHODS: In total, 33 consecutively referred CCS with persistent severe fatigue were offered CBT. The primary outcome was fatigue severity (Checklist Individual Strength, Fatigue Severity Subscale). Secondary outcomes were functional impairment, psychological distress, and quality of life (QoL)...
November 20, 2018: Journal of Pediatric Hematology/oncology
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