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Journal of Pediatric Hematology/oncology

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https://www.readbyqxmd.com/read/28437292/use-of-electronic-consultation-system-to-improve-access-to-care-in-pediatric-hematology-oncology
#1
Donna L Johnston, Kimmo Murto, Julia Kurzawa, Clare Liddy, Erin Keely, Lillian Lai
BACKGROUND: Electronic consultations (eConsult) allow for communication between primary care providers and specialists in an asynchronous manner. This study examined provider satisfaction, topics of interest, and efficiency of eConsult in pediatric hematology/oncology in Ottawa, Canada. METHODS: We conducted a cross-sectional assessment of all eConsult cases directed to pediatric hematology/oncology specialists using the Champlain BASE (Building Access to Specialists through eConsultation) eConsult service from June 1, 2014 to May 31, 2016...
April 21, 2017: Journal of Pediatric Hematology/oncology
https://www.readbyqxmd.com/read/28437291/behcet-disease-initially-presenting-as-deep-venous-thrombosis-a-case-report
#2
Arun Gurunathan, David Teachey, Kudakwashe R Chikwava, Char Witmer, Ami V Desai
Behcet disease is a potentially life-threatening multisystemic vasculitis with thrombotic tendency. Mucocutaneous ulcers, arthritis, and uveitis are the most recognizable features, but may be absent at the time of medical evaluation. We report a case in which a 8-year old patient presented with spontaneous bilateral lower extremity deep venous thromboses, and screening for rheumatologic symptoms led to diagnosing Behcet. This case demonstrates that deep venous thromboses can be the initial event bringing a patient with Behcet to medical attention, highlighting the importance of screening for underlying rheumatologic diseases in pediatric patients who present with unprovoked thrombosis...
April 21, 2017: Journal of Pediatric Hematology/oncology
https://www.readbyqxmd.com/read/28406842/hfe-gene-mutations-and-iron-status-in-100-healthy-polish-children
#3
Barbara Kaczorowska-Hac, Marcin Luszczyk, Jedrzej Antosiewicz, Wieslaw Ziolkowski, Elzbieta Adamkiewicz-Drozynska, Malgorzata Mysliwiec, Ewa Milosz, Jan J Kaczor
Iron participates in oxygen transport, energetic, metabolic, and immunologic processes. There are 2 main causes of iron overload: hereditary hemochromatosis which is a primary cause, is a metabolic disorder caused by mutations of genes that control iron metabolism and secondary hemochromatosis caused by multitransfusions, chronic hemolysis, and intake of iron rich food. The most common type of hereditary hemochromatosis is caused by HFE gene mutation. In this study, we analyzed iron metabolism in 100 healthy Polish children in relation to their HFE gene status...
April 12, 2017: Journal of Pediatric Hematology/oncology
https://www.readbyqxmd.com/read/28375943/neck-rhabdoid-tumors-clinical-features-and-consideration-of-autologous-stem-cell-transplant
#4
Adam D Wolfe, Christian M Capitini, Shahriar M Salamat, Kenneth DeSantes, Kristin A Bradley, Tabassum Kennedy, Louis P Dehner, Neha J Patel
Extrarenal malignant rhabdoid tumors (MRT) have a poor prognosis despite aggressive therapy. Adding high-dose chemotherapy with autologous stem cell rescue (HDC-ASCR) as consolidative therapy for MRT is controversial. We describe 2 patients, age 13 years and 19 months, with unresectable neck MRT. After chemotherapy and radiotherapy, both underwent HDC-ASCR and remain in remission over 4 years later. We reviewed all published cases of neck MRT, and found poorer outcomes and more variable age of presentation and time to progression than MRT at other sites...
April 3, 2017: Journal of Pediatric Hematology/oncology
https://www.readbyqxmd.com/read/28375941/two-tumors-in-1-what-should-be-the-therapeutic-target-pediatric-germ-cell-tumor-with-somatic-malignant-transformation
#5
Cecile Faure Conter, Brice Fresneau, Estelle Thebaud, Amandine Bertrand, Frederique Dijoud, Angelique Rome, Cecile Dumesnil, Marie Pierre Castex, Anguella Ghanem, Daniel Orbach
BACKGROUND: Germ cell tumors with somatic malignant transformation (GCT with SMT) are rare in children and poorly described. Data are missing to determine if therapies should target the GCT, the SMT compound, or both simultaneously. PATIENTS AND METHODS: A retrospective national study was conducted in the Société Française des cancers de l'Enfant (SFCE) Centers. Medical records from patients aged 0 to 18 years diagnosed with GCT with SMT between 2000 and 2015 were analyzed...
April 3, 2017: Journal of Pediatric Hematology/oncology
https://www.readbyqxmd.com/read/28353524/massive-mesenteric-lymphadenopathy-causing-protein-losing-enteropathy-in-gaucher-disease-retraction
#6
(no author information available yet)
No abstract text is available yet for this article.
March 28, 2017: Journal of Pediatric Hematology/oncology
https://www.readbyqxmd.com/read/28338570/a-case-of-all-developing-posterior-reversible-encephalopathy-secondary-to-hyponatremia
#7
Nilgun Eroglu, Aysenur Bahadir, Erol Erduran
Posterior reversible encephalopathy syndrome (PRES), may be due to different causes. It may develop secondary to hypertension, renal decompensation, electrolyte imbalance, and chemotherapeutic drugs. We describe a case of acute lymphoblastic leukemia in which PRES developed secondary to hyponatremia despite being normotensive during receipt of chemotherapy. Magnetic resonance imaging findings were suggestive of PRES. Partial diffusion restriction was observed in lesions in the bilateral occipitoparietal regions and the cerebellum...
March 23, 2017: Journal of Pediatric Hematology/oncology
https://www.readbyqxmd.com/read/28338568/pediatric-patient-with-renal-cell-carcinoma-treated-by-successive-antiangiogenics-drugs-a-case-report-and-review-of-the-literature
#8
Irene Jiménez, Hervé J Brisse, Paul Fréneaux, Sabine Sarnacki, Jean Michon, Daniel Orbach, Gaelle Pierron, Nathalie Clément, François Doz, Bernard Escudier, Gudrun Schleiermacher
Antiangiogenic drugs are currently standard of care in adults with renal cell carcinoma (RCC), including translocation RCC. Although antitumor activity and toxicity profile are well known in adults, few data have been reported in children. Here we present the case of a patient diagnosed at 2 years old with a metastatic translocation RCC, consecutively treated with 5 tyrosine kinase inhibitors during 6 years. The antitumor activity and toxic effects are described, and a brief review of the literature is presented...
March 23, 2017: Journal of Pediatric Hematology/oncology
https://www.readbyqxmd.com/read/28328614/a-newborn-with-familial-hemophagocytic-lymphohistiocytosis-complicated-with-transfusion-associated-graft-versus-host-disease
#9
Ahmet Ozdemir, Tamer Gunes, Samuel C C Chiang, Ekrem Unal
Hemophagocytic lymphohistiocytosis (HLH) is characterized by activation of cytotoxic T and natural killer (NK) cells, and macrophages related to a spectrum of hyperinflammatory disorders. The clinical findings mainly include high fever, cytopenia, splenomegaly, phagocytosis, and proliferation of histiocytes in lymphoreticular tissue. To the best of our knowledge, transfusion-associated graft versus host disease (TA-GVHD) in a 13-day old male newborn with HLH is being reported first time in the literature. The aim of this report was to emphasize the importance of blood products irradiation in the prevention of the development of graft versus host disease especially among high-risk subjects such as newborns with HLH...
March 21, 2017: Journal of Pediatric Hematology/oncology
https://www.readbyqxmd.com/read/28306689/pulmonary-relapse-of-osteosarcoma-following-presentation-with-a-pneumomediastinum-and-localized-disease-at-diagnosis
#10
Sarayu Kumar, Adina Alazraki, Bradley George, Melissa Martin, Howard M Katzenstein, Thomas Cash
Pneumothorax is a well-described complication of osteosarcoma. Conversely, the presence of a pneumomediastinum to our knowledge has been reported just once in a patient with osteosarcoma, and never without detectable lung metastasis. We report the case of an 18-year-old male with a localized, distal femur osteosarcoma who was found to have an asymptomatic pneumomediastinum and pneumatocele at diagnosis, and then 16 months later experienced a pulmonary relapse. Our case suggests that these findings may represent the presence of occult metastatic disease and cautions providers to treat appropriately and provide surveillance with a high index of suspicion for pulmonary recurrence...
March 16, 2017: Journal of Pediatric Hematology/oncology
https://www.readbyqxmd.com/read/28267090/a-rare-cause-of-neonatal-hemolytic-anemia-glutathione-synthetase-deficiency
#11
Pembe Soylu Ustkoyuncu, Fatma Türkan Mutlu, Aslihan Kiraz, Zuhal Tag Balkis, Sibel Yel
BACKGROUND: Isolated hemolysis or hemolytic anemia and 5-oxoprolinuria are 2 distinct medical conditions in the clinical spectrum associated with glutathione synthetase deficiency. CLINICAL OBSERVATION: A 1-day-old female baby presented with anemia and respiratory distress. Her hemoglobin level was 9.5 g/dL and the total serum bilirubin level was 5.6 mg/dL. Metabolic acidosis was detected in her blood gas analysis. Metabolic acidosis recurred despite treatment and further investigation was required...
March 6, 2017: Journal of Pediatric Hematology/oncology
https://www.readbyqxmd.com/read/28267088/sirolimus-as-an-effective-agent-in-the-treatment-of-immune-thrombocytopenia-itp-and-evans-syndrome-es-a-single-institution-s-experience
#12
Sylwia Jasinski, Mark E Weinblatt, Chana L Glasser
BACKGROUND: Autoimmune cytopenias are characterized by immune-mediated destruction of hematopoietic cell lines with immune thrombocytopenia (ITP) affecting platelets and Evans syndrome (ES) affecting platelets and red blood cells. For patients with persistent disease, limited options for effective and well-tolerated therapies exist. OBJECTIVES: Our aim is to describe our institution's experience with sirolimus as therapy for pediatric patients with persistent ITP and ES...
March 6, 2017: Journal of Pediatric Hematology/oncology
https://www.readbyqxmd.com/read/28267086/should-we-pay-attention-to-the-delay-before-admission-to-a-pediatric-intensive-care-unit-for-children-with-cancer-impact-on-1-month-mortality-a-report-from-the-french-children-s-oncology-study-group-goce
#13
Jeanne-L Fausser, Aude Tavenard, Fanny Rialland, Philippe Le Moine, Odile Minckes, Anne Jourdain, Olivier Tirel, Isabelle Pellier, Virginie Gandemer
Acute complications requiring admission to pediatric intensive care unit (PICU) are frequent for children with cancer. Our objective was to determine early prognostic factors of mortality in a cohort of children with cancer hospitalized in PICU for acute complications and particularly to assess whether the delay before admission to a PICU is an early predictor of mortality. We conduct a retrospective multicenter analysis. All patients transferred in PICU for acute complications between January 2002 and December 2012 were included...
March 6, 2017: Journal of Pediatric Hematology/oncology
https://www.readbyqxmd.com/read/28267085/hemolytic-anemia-due-to-right-ventricular-to-pulmonary-artery-conduit-stenosis
#14
Sudha Rao, Julie A Creaden, Shunyou Gong, Cynthia Rigsby, John M Costello
Hemolytic anemia is a well-recognized complication in patients with left-sided mechanical heart valves. It is rare to see hemolysis with a bioprosthetic valve in the right ventricular outflow tract. We report a 4-year-old-girl with history of truncus arteriosus status-post repair who developed hemolytic anemia as a result of a calcified and obstructed bioprosthetic right ventricular to pulmonary artery-valved conduit. The hemolysis was alleviated by replacing the obstructed conduit with a larger valved conduit...
March 6, 2017: Journal of Pediatric Hematology/oncology
https://www.readbyqxmd.com/read/28267084/monocyte-chemoattractant-protein-1-mcp-1-as-a-potential-therapeutic-target-and-a-noninvasive-biomarker-of-liver-fibrosis-associated-with-transient-myeloproliferative-disorder-in-down-syndrome
#15
Kenichiro Kobayashi, Takako Yoshioka, Jun Miyauchi, Atsuko Nakazawa, Shigeaki Yamazaki, Hiromi Ono, Michiko Tatsuno, Kenta Iijima, Chiaki Takahashi, Yoko Okada, Kenji Teranishi, Takaaki Matsunaga, Chieko Matsushima, Mayo Inagaki, Minoru Suehiro, Saori Suehiro, Masahiko Nishitani, Hirohito Kubota, Jun Iio, Yoshinobu Nishida, Tetsuo Katayama, Narito Takada, Kentaro Watanabe, Tetsuro Yamamoto, Ryoji Yasumizu, Kentaro Matsuoka, Kentaro Ohki, Nobutaka Kiyokawa, Toshiro Maihara, Ikuya Usami
Liver fibrosis is one of the common complications of transient myeloproliferative disorder (TMD) in Down syndrome (DS), but the exact molecular pathogenesis is largely unknown. We herein report a neonate of DS with liver fibrosis associated with TMD, in which we performed the serial profibrogenic cytokines analyses. We found the active monocyte chemoattractant protein-1 expression in the affected liver tissue and also found that both serum and urinary monocyte chemoattractant protein-1 concentrations are noninvasive biomarkers of liver fibrosis...
March 6, 2017: Journal of Pediatric Hematology/oncology
https://www.readbyqxmd.com/read/28267080/atic-gene-polymorphism-and-histologic-response-to-chemotherapy-in-pediatric-osteosarcoma
#16
Jeong A Park, Hee Young Shin
Accumulating evidence indicates that polymorphisms in folate pathway genes play a role in response to methotrexate (MTX) treatment in various diseases. This study explored the influence of these genetic polymorphisms on treatment outcome in pediatric osteosarcoma. Blood and tissue samples from 48 osteosarcoma patients were obtained, and the following polymorphisms were analyzed; SLC19A1 80G>A, DHFR 829C>T, MTHFR 677C>T, MTHFR 1298A>C, and ATIC 347C>G. We evaluated associations between these candidate gene polymorphisms and treatment outcome, including histologic response and event-free and overall survival, of patients treated with high-dose MTX...
March 6, 2017: Journal of Pediatric Hematology/oncology
https://www.readbyqxmd.com/read/28267078/pantoea-species-bacteremia-in-a-child-with-sickle-cell-disease-looking-for-a-culprit
#17
Marisa I Oliveira, Sara Batalha, Catarina Gouveia, Raquel Maia, Paula Kjöllerstrom
Pantoea agglomerans has been classically associated with cellulitis or synovitis secondary to penetrating trauma by vegetation. It is an infrequent cause of systemic infections. We describe the case of a 5-year-old girl with sickle cell disease with P. agglomerans bacteremia and review its potential causes.
March 6, 2017: Journal of Pediatric Hematology/oncology
https://www.readbyqxmd.com/read/28234741/concomitant-use-of-panobinostat-and-reirradiation-in-progressive-dipg-report-of-2-cases
#18
Zhihong J Wang, Yubin Ge, Deniz Altinok, Janet Poulik, Sandeep Sood, Jeffrey W Taub, Holly Edwards, Mark W Kieran, Miller Steven
Diffuse intrinsic pontine glioma (DIPG) remains a devastating disease. Panobinostat has been shown to have therapeutic efficacy both in vitro and in DIPG orthotopic xenograft models; however, clinical data in patients with DIPG are lacking. We present 2 cases of DIPG, who were treated with panobinostat at 22 to 25 mg/m/dose, 3 times weekly for 2 weeks in 3-week cycles and concomitant reirradiation after disease progression. Two episodes of asymptomatic thrombocytopenia were observed in 1 patient. Hyperacetylation of histone H4 of peripheral blood mononuclear cells was evident following treatment...
February 23, 2017: Journal of Pediatric Hematology/oncology
https://www.readbyqxmd.com/read/28230570/refractory-pure-red-cell-aplasia-manifesting-as-deficiency-of-adenosine-deaminase-2
#19
Hasan Hashem, Rachel Egler, Jignesh Dalal
Primary progress has been made in the last 2 years, particularly in finding novel disease-causing genes for a number of autoinflammatory diseases and primary immunodeficiencies. Whole-exome sequencing has dramatically increased the pace at which causative genes are being discovered. CECR1 (Cat eye syndrome chromosome region, candidate 1) gene encodes adenosine deaminase 2 (ADA2) protein. Patients who carry CECR1 mutation(s) suffer from deficiency of ADA2 (DADA2). Here, we describe a patient with pure red cell aplasia discovered to have DADA2...
February 22, 2017: Journal of Pediatric Hematology/oncology
https://www.readbyqxmd.com/read/28221267/health-related-quality-of-life-and-its-predictors-among-transfusion-dependent-thalassemia-patients
#20
Gulen Tuysuz, Funda Tayfun
PURPOSE: With regular blood transfusions and modern medical management strategies, transfusion-dependent thalassemia (TDT) patients are currently living into adulthood, but they still suffer from the chronicity of the disease and its complications. This study was aimed to assess the health-related quality of life (HRQoL) scores in pediatric TDT patients compared with healthy controls. PATIENTS AND METHODS: The PedsQL 4.0 Generic Core Scales were administered to 80 pediatric TDT patients (aged 5 to 18 y, child self-report) and their parents (parent-proxy report), as well as age-matched and sex-matched healthy controls (80 participants and their parents)...
February 17, 2017: Journal of Pediatric Hematology/oncology
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