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Journal of Pediatric Hematology/oncology

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https://www.readbyqxmd.com/read/29219892/toxicity-of-vincristine-combined-with-posaconazole-in-children-with-acute-lymphoblastic-leukemia
#1
Esra Pekpak, Talia İleri, Elif İnce, Mehmet Ertem, Zümrüt Uysal
BACKGROUND: Vincristine (VCR) is one of the main drugs of acute lymphoblastic leukemia (ALL) treatment. Azole antifungal medications are used for treatment or prophylaxis of invasive fungal infections in acute leukemia. Coadministration of these drugs increases the risk of VCR toxicity. OBSERVATIONS: We presented a girl with ALL using posaconazole prophylaxis. She developed VCR toxicity that included tubulopathy, high blood pressure, neuropathic pain, difficulty walking, diffuse muscular weakness, constipation, abdominal pain...
December 6, 2017: Journal of Pediatric Hematology/oncology
https://www.readbyqxmd.com/read/29219891/thrombotic-thrombocytopenic-purpura-in-a-child-treated-for-acute-lymphoblastic-leukemia-case-report-and-review-of-literature
#2
Francesco De Leonardis, Roberta Koronica, Rosa Maria Daniele, Nicola Santoro
Thrombotic thrombocytopenic purpura (TTP) is a thrombotic microangiopathy caused by deficiency of von Willebrand factor-cleaving protease ADAMTS13. Large von Willebrand multimer formation and intravascular platelet aggregation affecting the arterioles and capillaries can result in death unless early treatment is administered. We report on the case of a child with TTP associated with a human herpes virus type-6 infection occurring during chemotherapy for acute lymphoblastic leukemia who was effectively treated by fresh frozen plasma infusions and antiviral therapy...
December 6, 2017: Journal of Pediatric Hematology/oncology
https://www.readbyqxmd.com/read/29219890/a-cryptic-nup214-abl1-fusion-in-b-cell-precursor-acute-lymphoblastic-leukemia
#3
Shin-Ichi Tsujimoto, Yoshiko Nakano, Tomoo Osumi, Keiko Okada, Meri Ouchi-Uchiyama, Keisuke Kataoka, Yoichi Fujii, Kentaro Ohki, Masafumi Seki, Nobuyoshi Tamagawa, Junko Takita, Seishi Ogawa, Nobutaka Kiyokawa, Junichi Hara, Motohiro Kato
Fluorescent in situ hybridization (FISH) analysis is the standard methods for screening ABL1 fusions, which is recurrently translocated in pediatric acute lymphoblastic leukemia (ALL), and potentially targetable by kinase inhibitors. Here we demonstrated a case of B-cell precursor ALL with NUP214-ABL1 fusion, which break-apart FISH assay for ABL1 failed to detect. The cryptic fusion was generated by small duplication from ABL1 to NUP214, which was detected by copy number analysis using genomic microarray and confirmed by PCR...
December 6, 2017: Journal of Pediatric Hematology/oncology
https://www.readbyqxmd.com/read/29219889/the-value-of-neutrophil-to-lymphocyte-ratio-and-platelet-to-lymphocyte-ratio-for-detecting-early-onset-neonatal-sepsis
#4
Emrah Can, Şahin Hamilcikan, Ceren Can
OBJECTIVE: The purpose of this study was to investigate the relationship between neonate early-onset sepsis (EOS) and the neutrophil to lymphocyte ratio (NLR) and the platelet to lymphocyte ratio (PLR) of term neonates. MATERIALS AND METHODS: This prospective observational study was conducted with term neonates diagnosed with EOS compared with 44 healthy controls. Exclusion criteria were prematurity, postmaturity, small or large for gestational age according to week of pregnancy, preeclampsia, gestational diabetes mellitus, chorioamnionitis, congenital major anomalies, and cyanotic congenital heart disease...
December 6, 2017: Journal of Pediatric Hematology/oncology
https://www.readbyqxmd.com/read/29200172/a-rare-case-of-chronic-active-epstein-barr-virus-ebv-infection-accompanied-by-the-infiltration-of-ebv-infected-cd8-t-cells-into-the-muscle
#5
Nobuhiko Kobayashi, Takeki Mitsui, Yoshiyuki Ogawa, Hirono Iriuchishima, Makiko Takizawa, Akihiko Yokohama, Takayuki Saitoh, Hiromi Koiso, Norifumi Tsukamoto, Hirokazu Murakami, Yoshihisa Nojima, Hiroshi Handa
We describe a rare case of chronic active Epstein-Barr virus (CAEBV) infection, with infiltration of the skeletal muscle. A 19-year-old woman with swollen cervical lymph nodes and a fever was referred to our hospital. Swelling of the trapezium muscle and elevation of creatinine kinase level were observed. Biopsy results of the brachialis muscle revealed infiltration of Epstein-Barr virus (EBV)-encoded RNA-positive CD8 T lymphocytes. The EBV virus load in the peripheral blood was high, and EBV monoclonality was determined by Southern blot analysis...
December 1, 2017: Journal of Pediatric Hematology/oncology
https://www.readbyqxmd.com/read/29200171/reduced-neutrophil-elastase-activity-and-neutrophil-extracellular-traps-in-pediatric-acute-myeloid-leukemia-may-increase-the-rate-of-infections
#6
Sivan Berger-Achituv, Ronit Elhasid
Data on the production of neutrophil extracellular traps (NETs) in leukemia patients are scant. Phagocytosis, hydrogen peroxide, neutrophil elastase and myeloperoxidase enzymatic activity as well as NETs formation were studied in 10 pediatric acute lymphoblastic leukemia and 7 pediatric acute myeloid leukemia (AML) patients after induction chemotherapy. Median neutrophil elastase activity and NETs formation were lower in AML versus acute lymphoblastic leukemia (41% vs. 90%, P=0.005 and 51% vs. 94%, P=0.008, respectively)...
December 1, 2017: Journal of Pediatric Hematology/oncology
https://www.readbyqxmd.com/read/29200170/terson-syndrome-before-induction-chemotherapy-for-acute-lymphoblastic-leukemia
#7
YunZu Michele Wang, Eric Nudleman, Daniel Vinocur, Dennis John Kuo
A previously healthy 2-year-old female infant presented with pancytopenia and was diagnosed with acute lymphoblastic leukemia. Before the initiation of treatment, she developed symptoms concerning for increased intracranial pressure. Head imaging revealed left parietal hemorrhage, in addition to a right vitreous hemorrhage, which was confirmed on ophthalmology examination later. Terson syndrome, in which intraocular hemorrhage is associated with intracranial hemorrhage, is more commonly reported in adults, although ocular manifestations of leukemia have been reported at presentation and are typically asymptomatic...
December 1, 2017: Journal of Pediatric Hematology/oncology
https://www.readbyqxmd.com/read/29200169/sinus-histiocytosis-with-massive-lymphadenopathy-rosai-dorfman-disease-diagnostic-and-treatment-modalities-for-this-rare-entity-revisited
#8
Andrew W Averitt, Kenneth Heym, Lauren Akers, Fernando Castro-Silva, Anish Ray
Rosai-Dorfman disease, also known as sinus histiocytosis with massive lymphadenopathy is a rare non-Langerhans' cell histiocytic disease resulting from the proliferation and accumulation of sinus histiocytes within lymph nodes. Extranodal involvement frequently occurs, which increases the morbidity and mortality of the disease. There is no clear consensus with regard to the most effective diagnostic and treatment modalities. This report will focus on the diagnostic imaging, treatment, and outcomes for 3 cases of Rosai-Dorfman disease...
December 1, 2017: Journal of Pediatric Hematology/oncology
https://www.readbyqxmd.com/read/29200168/spontaneous-regression-in-a-patient-with-infantile-fibrosarcoma
#9
Sameer Farouk Sait, Enrico Danzer, Daniel Ramirez, Michael P LaQuaglia, Meyers Paul
Infantile fibrosarcoma usually presents as a rapidly growing mass on the extremities or trunk. We describe spontaneous regression in a 5-month-old female infant with biopsy proven, molecularly confirmed, right leg infantile fibrosarcoma currently at 26 months of age with no signs of local recurrence. Previously reported cases of spontaneous regression are reviewed, suggesting a benign clinical course in some cases. Although evidence for spontaneous regression is anecdotal in this rare tumor type, physicians should weigh the risks and benefits of surgery and chemotherapy against watchful waiting...
December 1, 2017: Journal of Pediatric Hematology/oncology
https://www.readbyqxmd.com/read/29200167/a-severe-case-of-murine-typhus-presenting-with-anemia-and-severe-thrombocytopenia
#10
Jade Wulff, Judith Margolin, Nana E Coleman, Gail Demmler-Harrison, Fong Lam, Mona D Shah
We discuss a child with severe thrombocytopenia and mild anemia admitted to the Hematology service who quickly deteriorated to a life-threatening state. However, once rickettsial disease was considered in the differential diagnosis and empiric doxycycline begun, she quickly and fully recovered. A diagnostic panel, including Rickettsia typhi serology, confirmed the diagnosis of murine typhus but this occurred weeks after she had recovered. Given the potential severity of rickettsial diseases and the ease of modern travel across geographic borders, hematology-oncology providers everywhere must consider rickettsial diseases in their differential diagnosis of critically ill children and begin empiric therapy with doxycycline promptly...
December 1, 2017: Journal of Pediatric Hematology/oncology
https://www.readbyqxmd.com/read/29200166/ataxia-telangiectasia-and-cancer-predisposition-challenges-in-management
#11
Santhosh A Upadhyaya, Rajen Mody, Kelly Walkovich, Raymond J Hutchinson, John T Sandlund, James A Connelly
Immune dysregulation and predisposition to malignancies are critical comorbidities in children affected with ataxia telangiectasia. In addition, these children exhibit increased toxicity to conventional cancer therapy and dose reductions have been proposed to prevent life threatening adverse effects. These modifications to the treatment regimen may result in suboptimal outcomes for these patients. Our report of 3 children with ataxia telangiectasia and cancer highlight the immense challenges in the management of these children, underlining the need for the development of novel, biological agents with reduced acute and long-term side effects in the treatment of cancers in these children...
December 1, 2017: Journal of Pediatric Hematology/oncology
https://www.readbyqxmd.com/read/29200165/programmed-death-ligand-1-expression-in-classical-hodgkin-lymphoma-in-pediatric-patients
#12
(no author information available yet)
No abstract text is available yet for this article.
December 1, 2017: Journal of Pediatric Hematology/oncology
https://www.readbyqxmd.com/read/29200164/neuropilin-1-cd304-expression-by-flow-cytometry-in-pediatric-precursor-b-acute-lymphoblastic-leukemia-a-minimal-residual-disease-and-potential-prognostic-marker
#13
Hala M Abaza, Mervat A A Alfeky, Deena S Eissa, Mona F Abdel Fattah, Laila M Annaka, Fatma S Ebeid
Flow cytometry (FCM) is used for quantification of minimal residual disease (MRD) in acute lymphoblastic leukemia (ALL) through discriminating leukemic B-lymphoblasts from normal B-cell precursor counterparts "hematogones." Neuropilin-1 (NRP-1)/CD304 is a vascular endothelial growth factor receptor implicated in the progression of hematological malignancies. We evaluated NRP-1/CD304 as MRD and prognostic marker in pediatric precursor B-ALL using FCM. Seventy children with precursor B-ALL and 40 control children were enrolled...
December 1, 2017: Journal of Pediatric Hematology/oncology
https://www.readbyqxmd.com/read/29200163/therapy-related-acute-myeloid-leukemia-after-the-treatment-of-primary-solid-cancer-in-children-a-single-center-experience
#14
Kyung Taek Hong, Jung Yoon Choi, Che Ry Hong, Hyoung Jin Kang, Kyung Duk Park, Hee Young Shin
Therapy-related acute myeloid leukemia (t-AML) has a dismal prognosis and is one of the most frequent second malignant neoplasms which could be encountered by pediatric oncologists. Between October 2000 and September 2016, 16 patients who had primary solid tumors were diagnosed with t-AML at the Seoul National University Children's Hospital. The median patient age at the time of diagnosis of their primary solid tumors was 9.6 years (range, 0.1 to 15.4 y), and that of t-AML was 14.0 years (range, 4.7 to 23...
December 1, 2017: Journal of Pediatric Hematology/oncology
https://www.readbyqxmd.com/read/29200162/study-of-notch1-and-fbxw7-mutations-and-its-prognostic-significance-in-south-indian-t-cell-acute-lymphoblastic-leukemia
#15
Natarajan Valliyammai, Nirmala K Nancy, Tenali G Sagar, Thangarajan Rajkumar
NOTCH1/FBXW7 mutations trigger oncogenic NOTCH1 signaling and its downstream target genes play crucial roles in the molecular pathogenesis of T-cell acute lymphoblastic leukemia (T-ALL). In the present study, NOTCH1 and FBXW7 mutations were studied in 25 primary T-ALL samples. All 34 exons of NOTCH1 and hotspot exons (exon 9 and exon 10) of FBXW7 were polymerase chain reaction amplified and sequenced for mutations. Our results showed that 13/25 (52%) were NOTCH1-mutated, of which 11 patients (44%) showed mutation in the hotspot exons...
December 1, 2017: Journal of Pediatric Hematology/oncology
https://www.readbyqxmd.com/read/29200161/noninvasive-measurement-of-hemoglobin-using-spectrophotometry-is-it-useful-for-the-critically-ill-child
#16
Basak Akyildiz
This study compared the accuracy of noninvasively measuring hemoglobin using spectrophotometry (SpHb) with a pulse CO-oximeter and laboratory hemoglobin (Hb) measurements. A total of 345 critically ill children were included prospectively. Age, sex, and factors influencing the reliabilityof SpHb such as SpO2, heart rate, perfusion index (PI), and vasoactive inotropic score were recorded. SpHb measurements were recorded during the blood draw and compared with the Hb measurement. Thirteen patients (low PI in 9 patients and no available Hb in 4 patients) were excluded and 332 children were eligible for final analysis...
December 1, 2017: Journal of Pediatric Hematology/oncology
https://www.readbyqxmd.com/read/29200160/glucose-homeostasis-and-effect-of-chelation-on-%C3%AE-cell-function-in-children-with-%C3%AE-thalassemia-major
#17
Sunil Gomber, Aashima Dabas, Shilpa Bagmar, Sri Venkata Madhu
OBJECTIVE: To assess the prevalence of impaired glucose tolerance in β-thalassemia major and correlate it with chelation therapy. MATERIALS AND METHODS: Sixty-seven subjects with β-thalassemia major, aged 1 to 20 years, were enrolled in our prospective cohort. Clinical details were recorded. Baseline oral glucose tolerance test, serum insulin, C peptide, and insulin resistance were measured. The biochemical profile was repeated after 6 months. RESULTS: The mean age of subjects was 7...
December 1, 2017: Journal of Pediatric Hematology/oncology
https://www.readbyqxmd.com/read/29200159/posterior-reversible-encephalopathy-syndrome-risk-factors-and-impact-on-the-outcome-in-children-with-acute-lymphoblastic-leukemia-treated-with-nordic-protocols
#18
Joanna S Banerjee, Mats Heyman, Maarit Palomäki, Päivi Lähteenmäki, Mikko Arola, Pekka V Riikonen, Merja I Möttönen, Tuula Lönnqvist, Mervi H Taskinen, Arja H Harila-Saari
Posterior reversible encephalopathy syndrome (PRES) in children with acute lymphoblastic leukemia has been increasingly recognized as a clinicoradiological entity. Our aim was to describe the incidence of PRES in pediatric patients with ALL, identify its risk factors, and examine its prognostic importance. For this research, we conducted a systematic, retrospective review of the patient records in a population-based series of children with acute lymphoblastic leukemia (n=643) treated in Finland from 1992 to 2008...
December 1, 2017: Journal of Pediatric Hematology/oncology
https://www.readbyqxmd.com/read/29200158/cardiac-toxicity-after-craniospinal-irradiation-a-late-effect-that-may-be-eliminated-with-proton-therapy
#19
Gabrielle D Welch, Kimberly Y Lin, Michael J Fisher, Christine E Hill-Kayser
BACKGROUND: Craniospinal irradiation (CSI) is commonly required for pediatric patients with central nervous system malignancies. Traditionally, CSI is given using x-rays to deliver radiation to the brain and spine, exposing normal anterior structures, including heart, to unnecessary radiation. OBSERVATIONS: We present a patient treated with x-ray CSI for medulloblastoma with spinal metastasis (3600 cGy CSI with focal boost to 5000 cGy), who subsequently developed significant cardiac toxicity, likely related to radiation exposure...
December 1, 2017: Journal of Pediatric Hematology/oncology
https://www.readbyqxmd.com/read/29200157/an-infant-with-unusually-high-unconjugated-hyperbilirubinemia-due-to-coexistence-of-hereditary-spherocytosis-and-gilbert-syndrome
#20
Ivona Butorac Ahel, Kristina Baraba Dekanic, Goran Palcevski, Jelena Roganovic
Hereditary spherocytosis is the most frequent congenital hemolytic anemia and is characterized with variable degree of anemia, jaundice, and splenomegaly. In the case of severe hyperbilirubinemia out of proportion with hemolysis, other causes of hyperbilirubinemia must be considered. Gilbert syndrome (GS) is an autosomal dominant disorder characterized with intermittent hyperbilirubinemia without any other sign and symptom of liver disease as a result of reduced activity of uridine diphosphate-glucuronyl transferase 1A1...
December 1, 2017: Journal of Pediatric Hematology/oncology
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