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Journal of Pediatric Hematology/oncology

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https://www.readbyqxmd.com/read/29683961/acquired-versus-congenital-neonatal-purpura-fulminans-a-case-report-and-literature-review
#1
Tina Findley, Minal Patel, John Chapman, Deborah Brown, Andrea F Duncan
Neonatal purpura fulminans (PF) is a life-threatening disorder caused by congenital or acquired deficiencies of protein C (PC) or S. PF presents as a cutaneous manifestation of disseminated intravascular coagulation. We describe a case of PF in a newborn with left leg ischemia and undetectable PC levels soon after birth. Despite anticoagulation therapy and PC concentrate, left foot amputation was required. Genetic testing of PROC for congenital PC deficiency was normal. This case highlights the course of PF due to acquired PC deficiency in a newborn treated with PC concentrate which is rarely described in the literature...
April 20, 2018: Journal of Pediatric Hematology/oncology
https://www.readbyqxmd.com/read/29683960/excessive-treatment-failures-in-patients-with-parameningeal-rhabdomyosarcoma-with-reduced-dose-cyclophosphamide-and-delayed-radiotherapy
#2
John T Lucas, Alberto S Pappo, Jianrong Wu, Daniel J Indelicato, Matthew J Krasin
Pediatric patients with parameningeal rhabdomyosarcoma and high-risk features are recommended to receive radiotherapy at initiation of protocol therapy due to concerns about the increased risk of local and leptomeningeal failure from treatment delay. We report our early experience on a multi-institutional prospective trial incorporating delayed primary site radiation and reduced dose cyclophosphamide in all patients with parameningeal tumors. We observed an excessive number of locoregional treatment failures following this approach and have subsequently amended our trial to move radiation therapy upfront for those patients with high-risk features...
April 20, 2018: Journal of Pediatric Hematology/oncology
https://www.readbyqxmd.com/read/29683959/malignant-peripheral-nerve-sheath-tumors-in-neurofibromatosis-impact-of-family-history
#3
Fatema Malbari, Menachem Spira, Pamela B Knight, Chong Zhu, Michael Roth, Jonathan Gill, Rick Abbott, Adam S Levy
OBJECTIVE: The main objective of this study was to determine if family history of malignant peripheral nerve sheath tumor (MPNST) increases risk of developing an MPNST in patients with neurofibromatosis-1 (NF-1). MATERIALS AND METHODS: Individuals with NF-1 registered with the Children's Tumor Foundation's Neurofibromatosis Registry were emailed an anonymous 15-minute survey with regard to personal and family history of NF-1, MPNST, ages of onset, and symptomatology...
April 20, 2018: Journal of Pediatric Hematology/oncology
https://www.readbyqxmd.com/read/29683958/kidney-protective-effect-of-magnesium-supplementation-in-cisplatin-containing-chemotherapy-for-pediatric-cancer-a-retrospective-study
#4
Motohiro Matsui, Yuya Saito, Shoko Yamaoka, Yuichi Yokokawa, Yoshihiko Morikawa, Atsushi Makimoto, Yuki Yuza
In total, 158 chemotherapy courses containing cisplatin for 37 pediatric cases of newly diagnosed cancer were divided into 2 groups depending on whether magnesium (Mg) supplementation was administered (Mg+: 92 courses) or not (Mg-: 66 courses). Renal impairment was defined as grade 2 or higher creatinine elevation (CE) after each chemotherapy course. The incidence of CE in the Mg+ was significantly lower than in the Mg- (9.8% vs. 22.7%; P=0.025). Multivariate analysis revealed that Mg supplementation significantly reduced the incidence of CE (odds ratio, 0...
April 20, 2018: Journal of Pediatric Hematology/oncology
https://www.readbyqxmd.com/read/29683957/biliary-rhabdomyosarcoma-in-an-infant-male-with-neurofibromatosis-type-1
#5
Fatema Alhashem, Ebru Yilmaz, Mehmet A Ozdemir, Kemal Deniz, Ali Yikilmaz, Turkan Patiroglu, Musa Karakukcu, Ekrem Unal
Different types of malignancies can be seen in patients with neurofibromatosis type 1 (NF-1). Herein we present a rare combination of NF-1 and biliary rhabdomyosarcoma in a male infant. An 11-month-old boy, who was recently diagnosed with NF-1, presented to the outpatient clinic with a 3-month history of prolonged jaundice, and failure to thrive. Clinical examination showed >20 café au let spots distributed mainly over the abdominal trunk. Hepatomegaly (4 cm below the costal margin) was additionally observed...
April 20, 2018: Journal of Pediatric Hematology/oncology
https://www.readbyqxmd.com/read/29683956/hemolytic-uremic-syndrome-associated-with-non-shigatoxin-producing-infectious-agents-expanding-the-shigatoxin-theory
#6
Werner Keenswijk, Eva Degraeuwe, Evelyn Dhont, Ann Raes, Johan Vande Walle
Diarrhea-associated hemolytic uremic syndrome (HUS) is usually associated with shigatoxin-producing Escherichia coli or shigella infections. We report 2 cases of HUS, respectively, caused by salmonella and Campylobacter jejuni infections. None of these bacteria produce shigatoxins, and the underlying mechanism of HUS development remains unknown. In streptococcus pneumoniae-associated HUS, bacterial neuraminidase cleaves neuraminic acid and causes exposure of Thomsen-Friedenreich cryptantigen on the cell surface of, for example, erythrocytes, which induces an inflammatory response caused by binding of preformed IgM...
April 20, 2018: Journal of Pediatric Hematology/oncology
https://www.readbyqxmd.com/read/29683955/successful-management-of-a-child-with-drug-induced-necrotizing-pancreatitis-during-acute-lymphoblastic-leukemia-therapy-a-case-report
#7
Anna Płotka, Agnieszka Wziątek, Jacek Wachowiak, Katarzyna Derwich
Acute pancreatitis in children acute lymphoblastic leukemia is commonly caused by drugs, for example, L-Asparaginase, pegapargase, steroids. The incidence of this complication is estimated at 6.7% to 18%. Although the majority of drug-induced acute pancreatitis cases are mild, severe cases can rarely occur. This work presents a case of successful management of a child with drug-induced necrotizing pancreatitis during acute lymphoblastic leukemia therapy. This case illustrates that comprehensive care and immediate intensive treatment can rescue patient despite poor prognosis...
April 20, 2018: Journal of Pediatric Hematology/oncology
https://www.readbyqxmd.com/read/29683954/a-retrospective-analysis-of-sociodemographic-and-hematologic-characteristics-associated-with-achieving-optimal-hydroxyurea-therapy-in-children-with-sickle-cell-disease
#8
Paul E George, Juan C Bazo-Alvarez, Vivien A Sheehan
Hydroxyurea (HU) has proven hematologic and clinical benefits, especially when escalated to the maximum tolerated dose (MTD). We reviewed clinical data from patients with sickle cell disease (January 2011 to 2016) to determine baseline sociodemographic and laboratory parameters associated with reaching HU MTD without significant delays. In total, 210 patients (mean HU start age, 6.6 y) were included. Initial Kaplan-Meier event analysis showed 1 year to be an inflection point for reaching MTD. In total, 116 patients (55%) reached MTD in <1 year, with 56 (27%) taking >1 year to reach MTD and 38 (18%) patients not successfully reaching MTD during follow-up...
April 20, 2018: Journal of Pediatric Hematology/oncology
https://www.readbyqxmd.com/read/29683953/outcomes-of-proton-beam-radiation-therapy-for-retinoblastoma-with-vitreous-seeds
#9
Eun Hye Jung, Jeong Hun Kim, Joo Young Kim, Dong Hyun Jo, Young Suk Yu
Vitreous seeds are the most challenging aspect in the management of retinoblastoma. We report the outcomes of treatment with proton beam radiation therapy (PBRT) for retinoblastoma with vitreous seeds in naive or previously treated eyes. In this retrospective case series, we analyzed data of 4 retinoblastoma patients with vitreous seeds who received PBRT at the Proton Therapy Center, National Cancer Center in Korea between June 2007 and August 2017. All 4 eyes treated by PBRT were classified as group D according to the International Classification of Retinoblastoma (ICRB) criteria, and the vitreous seeds, as class 3 (clouds)...
April 20, 2018: Journal of Pediatric Hematology/oncology
https://www.readbyqxmd.com/read/29683952/murine-typhus-and-hemophagocytic-syndrome
#10
Chiara Iaria, Claudia Colomba, Paola Di Carlo, Francesco Scarlata, Antonio Cascio
No abstract text is available yet for this article.
April 20, 2018: Journal of Pediatric Hematology/oncology
https://www.readbyqxmd.com/read/29683951/respiratory-distress-and-severe-anemia-in-a-child-with-idiopathic-pulmonary-hemosiderosis
#11
Münevver T Temel, Levent Temel, Mehmet E Coskun, Bugra T Konduk, Sinan Akbayram, Abdullah T Demiryürek
Idiopathic pulmonary hemosiderosis is an infrequent cause of pulmonary hemorrhage in children. It is classically defined by the triad of recurrent hemoptysis, iron-deficiency anemia, and diffuse parenchymal infiltration without an obvious cause. The pathogenesis remains unexplained, diagnosis may be difficult, and the clinical course exceedingly variable. A 4-year-old girl was admitted to the hospital with complaints of dyspnea, and skin and mucous membrane pallor. The suspicion of idiopathic pulmonary hemosiderosis led to the use of corticosteroid therapy with rapid improvement in clinical condition and discharge from hospital...
April 20, 2018: Journal of Pediatric Hematology/oncology
https://www.readbyqxmd.com/read/29683950/atypical-autoimmune-hematological-disorders-in-a-patient-with-kabuki-syndrome
#12
Ara C Almécija, Vanesa Pérez, María Baro, Pilar Guerra-García, Jose L Vivanco
Kabuki syndrome is a rare genetic disorder characterized by congenital anomalies and developmental delay. It is often associated with impaired immune response and autoimmune abnormalities. We report the clinical case of a girl with Kabuki syndrome who developed autoimmune neutropenia, not previously reported, followed by hemolytic anemia and autoimmune thrombocytopenia.
April 20, 2018: Journal of Pediatric Hematology/oncology
https://www.readbyqxmd.com/read/29683949/a-rare-case-of-hemophagocytic-lymphohistiocytosis-associated-with-myelodysplastic-syndrome-and-trisomy-8-in-a-pediatric-patient
#13
Seh Hyun Kim, Dae Yong Yi, Na Mi Lee, Sin Weon Yun, Soo Ahn Chae, In Seok Lim
Hemophagocytic lymphohistiocytosis (HLH) is a rare disease resulting in clinical and biochemical manifestations of extreme inflammation. Myelodysplastic syndrome (MDS) represents a heterogenous group of clonal hematopoietic disorders. The development of MDS is common in children with trisomy of chromosome 8. Here, we report a fatal case of 8-year-old girl who was admitted to the emergency department with status epilepticus, and later diagnosed with HLH associated with MDS and trisomy of chromosome 8. We believe this is the first reported case of HLH associated with MDS and trisomy 8 in a pediatric patient...
April 20, 2018: Journal of Pediatric Hematology/oncology
https://www.readbyqxmd.com/read/29683948/hematopoietic-stem-cell-transplantation-from-unrelated-donors-in-2-cases-of-interleukin-10-receptor-deficiency-is-surgery-not-a-requirement
#14
Dilara F Kocacik Uygun, Vedat Uygun, Hayriye Daloğlu, Seda Öztürkmen, Gülsün Karasu, İsmail Reisli, Ersin Sayar, Hasan A Yüksekkaya, Erik-Oliver Glocker, Kaan Boztuğ, Akif Yeşilipek
Mutations in interleukin-10 and its receptors cause infantile inflammatory bowel disease (IBD), a hyperinflammatory disorder characterized by severe, treatment-refractory colitis, multiple abscesses, and enterocutaneous fistulas. Patients with infantile IBD often require several surgical interventions, including complete colectomy, and hematopoietic stem cell transplantation is currently the only known medical therapy. Traditionally, operative management has been preferred before stem cell transplantation because of the latter's increased susceptibility to procedural complications; however, surgical intervention could be delayed, and possibly reconsidered, because our 2 patients with infantile IBD demonstrated a rapid response to treatment via engraftment...
April 20, 2018: Journal of Pediatric Hematology/oncology
https://www.readbyqxmd.com/read/29683947/encephalocraniocutaneous-lipomatosis
#15
Abhishek Bavle, Rikin Shah, Naina Gross, Theresa Gavula, Alejandro Ruiz-Elizalde, Klaas Wierenga, Rene McNall-Knapp
A 5-year-old boy presented with worsening headaches for 3 months. On examination, he was found to have a hairless fatty tissue nevus of the scalp (nevus psiloliparus), subcutaneous soft tissue masses on the right side of his face, neck, mandible and right buttock and epibulbar dermoid of the right eye (choristoma) (Figs. 1A, B). Magnetic resonance imaging revealed a large suprasellar mass, which was debulked and found to be a pilocytic astrocytoma. Testing was not performed for the BRAF/KIAA1549 fusion or BRAFV600E mutation...
April 20, 2018: Journal of Pediatric Hematology/oncology
https://www.readbyqxmd.com/read/29683946/romiplostim-for-immune-thrombocytopenia-in-neuroblastoma-patients-receiving-chemotherapy
#16
Hannah Fassel, James B Bussel, Stephen S Roberts, Shakeel Modak
Thrombocytopenia, a serious complication of myelosuppressive chemotherapy in cancer patients, is managed with platelet transfusions until recovery of platelet counts. However, children receiving chemotherapy can rarely develop immune thrombocytopenia (ITP) that is refractory to transfused platelets. This limits the ability to achieve adequate platelet counts and administer further myelosuppressive chemotherapy safely, especially if first-line ITP therapy is ineffective. We report 2 cases of intravenous immunoglobulin refractory ITP in children receiving chemotherapy for high-risk neuroblastoma...
April 20, 2018: Journal of Pediatric Hematology/oncology
https://www.readbyqxmd.com/read/29683945/ebv-related-cold-agglutinin-disease-presenting-with-conjugated-hyperbilirubinemia-a-pediatric-case-report-and-mini-review
#17
Elpis Mantadakis, Eleftherios Chatzimichael, Eftychia Kontekaki, Maria Panopoulou, Georges Martinis, Aggelos Tsalkidis
Hemolytic anemia occurs in only 1% to 3% of hospitalized patients with infectious mononucleosis. The authors describe an 8-year-old girl without cervical lymphadenopathy or splenomegaly, who presented with conjugated hyperbilirubinemia and was diagnosed with cold agglutinin disease caused by an immunoglobulin M autoantibody with anti-i specificity. Acute Epstein-Barr virus infection was confirmed by serological and molecular methods. She recovered uneventfully after a 3-week course of methylprednisolone. Epstein-Barr virus infection should be considered in any case of hemolytic anemia associated with hepatic dysfunction, especially when direct antiglobulin test is positive for C3d...
April 20, 2018: Journal of Pediatric Hematology/oncology
https://www.readbyqxmd.com/read/29683944/slco1b1-polymorphisms-are-associated-with-drug-intolerance-in-childhood-leukemia-maintenance-therapy
#18
İrem Eldem, Duygu Yavuz, Özge Cumaoğullari, Talia İleri, Elif Ünal İnce, Mehmet Ertem, Beyza Doğanay Erdoğan, Recep Bindak, Hilal Özdağ, N Lale Şatiroğlu-Tufan, L Zümrüt Uysal
BACKGROUND: Therapy discontinuations and toxicities occur because of significant interindividual variations in 6-mercaptopurine (6-MP) and methotrexate (MTX) response during maintenance therapy of childhood acute lymphoblastic leukemia (ALL). 6-MP/MTX intolerance in some of the patients cannot be explained by thiopurine S-methyl transferase (TPMT) gene variants. In this study, we aimed to investigate candidate pharmacogenetic determinants of 6-MP and MTX intolerance in Turkish ALL children...
April 20, 2018: Journal of Pediatric Hematology/oncology
https://www.readbyqxmd.com/read/29683943/pentoxifylline-added-to-steroid-window-treatment-phase-modified-apoptotic-gene-expression-in-pediatric-patients-with-acute-lymphoblastic-leukemia
#19
Jesus Meza-Arroyo, Alejandro Bravo-Cuellar, Luis Felipe Jave-Suárez, Georgina Hernández-Flores, Pablo Ortiz-Lazareno, Adriana Aguilar-Lemarroy, Marlin Padilla-Corona, Fernando Sanchez-Zubieta, Oscar Gonzalez-Ramella
Pentoxifylline is a xanthine that possesses antitumor properties and that can induce higher apoptosis in the leukemic cells of pediatric patients with acute lymphoblastic leukemia (ALL) during treatment with prednisone. We conducted a phase 1 pilot, controlled, randomized trial to evaluate the gene expression modified by pentoxifylline during the steroid window of induction to remission phase in patients newly diagnosed with ALL. Experimental and control treatments induced broad changes in the gene expression profile...
April 20, 2018: Journal of Pediatric Hematology/oncology
https://www.readbyqxmd.com/read/29668553/glucose-homeostasis-markers-in-beta-thalassemia
#20
Sunil Gomber, Anjali Bagaria, Sri V Madhu, Pooja Dewan
BACKGROUND: Glycosylated hemoglobin (HbA1c) has been a well-recognized marker of glucose homeostasis among thalassemics. Recently some studies have proposed the role of fructosamine as a better marker as compared with HbA1c. Hence, the study was carried out to find out which marker holds promise among Indian beta-thalassemic children. METHODS: In this case-control study, 60 diagnosed cases of beta-thalassemia major and equal number of normal controls who were ≥8 years of age were enrolled...
April 17, 2018: Journal of Pediatric Hematology/oncology
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