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Journal of Pediatric Hematology/oncology

Sibel A Tekgündüz, Şule Yeşil, Ayşe C Ören, Hikmet G Tanyildiz, Mehmet O Çandir, Ceyhun Bozkurt, Gürses Şahin
Vitamin D receptor (VDR) polymorphisms are found more commonly in some tumor types than in healthy individuals, suggesting that some polymorphisms (Cdx2, Fok1, Bsm1, Apa1, Taq1) contribute to tumor development. There is no previous report on VDR polymorphism in Hodgkin's lymphoma (HL) patients. VDR polymorphism patterns in 95 pediatric HL cases with 100 healthy controls were compared. No statistically significant difference was found between the patient group and control group in terms of Cdx2, Fok1, Bsm1, Apa1, and Taq1 polymorphisms (P>0...
September 7, 2016: Journal of Pediatric Hematology/oncology
Giang-Kim T Nguyen, Angela Lewis, Carol Goldener, Brenda Reed, Robin Yates Dulman, Elizabeth Yang
Folic acid (FA) is commonly prescribed for patients with sickle cell anemia, but evidence for the efficacy of this practice is lacking. We stopped FA supplementation and measured red blood cell folate levels after discontinuation of FA in 72 patients with clinically severe forms of sickle cell disease. We compared hemoglobin and reticulocyte counts before and after FA discontinuation in 51 of those patients, the majority of whom were on hydroxyurea. No patients had red blood cell folate levels below normal and no significant difference in hemoglobin levels (P=0...
August 26, 2016: Journal of Pediatric Hematology/oncology
Ryo Itoshima, Ryoji Kobayashi, Hirozumi Sano, Daiki Hori, Kenji Kishimoto, Daisuke Suzuki, Masayoshi Miura, Yoshitake Takagi, Hiroshi Yamamoto, Shoji Fujita, Ryota Honjo, Kunihiko Kobayashi
Extrarenal nephroblastoma (ERNB) is a rare disease. We report a case of ERNB in a 4-year-old boy complaining of abdominal pain and vomiting. Imaging showed a retroperitoneal mass and left hydronephrosis. The mass was completely removed by surgery. The pathologic diagnosis was ERNB with favorable histology. Postoperative chemotherapy was administered for 24 weeks with actinomycin D, vincristine, and doxorubicin. No signs of recurrence were found for the next 3 years. We consider 53 reports of ERNB and our own...
August 26, 2016: Journal of Pediatric Hematology/oncology
Fredrick Dapaah-Siakwan, Carleene Bryan, Lashawndra S Walker, Teresa Del Moral
INTRODUCTION: Splenic hemangiomas (SHs) are the most common benign neoplasms of the spleen. However, they are rare in the newborn period. We present an extremely rare case of congenital SH complicated by Kasabach-Merritt syndrome. CASE PRESENTATION: A 2.93 kg male infant was delivered at term with a prenatal diagnosis of a left infrarenal mass diagnosed by ultrasound at 35 weeks of gestation. Magnetic resonance imaging demonstrated a well-defined splenic mass with multiple flow voids and scattered areas of high intensity suggestive of hemorrhage...
August 26, 2016: Journal of Pediatric Hematology/oncology
Trishana Nayiager, Ronald D Barr, Loretta Anderson, Amy Cranston, John Hay
Inadequate physical activity (PA) and elevated overweight/obesity (OW/OB) rates are common in survivors of cancer in childhood, especially acute lymphoblastic leukemia (ALL). Bony morbidity, including fractures, is also prevalent among survivors of ALL. This study examined the interrelationships of PA, measured in hours by the Habitual Activity Estimation Scale; OW/OG, defined by body mass index; and fractures (yes/no) in survivors of ALL (n=75) more than 10 years after diagnosis. All had been treated using protocols of the Dana Farber Cancer Institute Childhood ALL Consortium...
August 26, 2016: Journal of Pediatric Hematology/oncology
Shinya Osone, Toshihiko Imamura, Takuyo Kanayama, Yusuke Tsuma, Sachiko Kawashima-Goto, Takuya Nakatani, Atsuya Sugimoto, Akari Takai, Mitsuru Miyachi, Shinichi Tamura, Hiroyuki Ishida, Hajime Hosoi
Sorafenib is a promising agent for treating pediatric refractory acute myeloid leukemia (AML) exhibiting FMS-like tyrosine kinase 3-internal tandem duplication (FLT3-ITD); however, its optimal use needs to be established. We report 2 cases of refractory pediatric FLT3-ITD-positive AML treated with sorafenib. Case 1 underwent stem cell transplantation (SCT) without entering remission, despite the use of chemotherapy. This patient relapsed despite receiving post-SCT sorafenib. Chemotherapy combined with sorafenib successfully achieved complete remission in case 2...
August 26, 2016: Journal of Pediatric Hematology/oncology
Arzu Yazal Erdem, Suna Emir, Haci A Demir, Derya Özyörük, İlker Çetin, Aynur Turan, Esra Karakuş
Congenital portosystemic shunts are rare vascular malformations that lead to several complications including liver tumors, pulmonary hypertension, and metabolic encephalopathy. We describe a rare case of a 17-year-old girl with an extrahepatic portosystemic shunt presenting recurrent syncope episodes and a liver mass mimicking hepatocellulary carcinoma.
August 26, 2016: Journal of Pediatric Hematology/oncology
Mehran Karimi, Abbas Avazpour, Sezaneh Haghpanah, Foroogh Toosi, Arash Badie
BACKGROUND: β-thalassemia is the most common heredity disease in Iran. Regular blood transfusion is critical to sustain life and normal growth. Deferasirox is an oral chelator. One of the side effects of the deferasirox is proteinuria. OBJECTIVES: This study aimed to investigate the safety of deferasirox on kidney function in diabetic and nondiabetic β-thalassemia major patients. MATERIALS AND METHODS: In this cross-sectional study, 34 diabetic and 36 nondiabetic patients who take deferasirox (Exjade) 20 to 40 mg/kg/d were studied...
August 19, 2016: Journal of Pediatric Hematology/oncology
Oya Balci, Taner Sezer
OBJECTIVE: The association between arterial ischemic stroke (AIS) and celiac disease (CD) has been described in only a few cases in adults and children. We aim to determine the prevalence of CD in children and adolescents with AIS. STUDY DESIGN: We investigated serum levels of tissue transglutaminase antibody immunoglobulin (Ig)A and total IgA from 76 children with AIS and in a healthy control group of 102 children. Study participants who were positive for tissue transglutaminase IgA antibodies underwent a duodenal biopsy...
August 19, 2016: Journal of Pediatric Hematology/oncology
Rumesh Chandar, Venkatesh Chandrasekaran, Barath Jagadisan, Delhi Kumar, Niranjan Biswal
A 7-year-old male child with very severe aplastic anemia developed refractory disease, which was attributed to febrile hemolytic transfusion reactions and was treated with immunosuppressants, which lead to a transient improvement. However, the child worsened subsequently and succumbed to an underlying hemophagocytic lymphohistiocytosis that was recognized late due to an overlap of clinical and biochemical parameters in both the conditions. Hemophagocytic lymphohistiocytosis should be an early suspicion in children with refractory very severe aplastic anemia and the detection of underlying gene mutations can predict disease severity...
August 19, 2016: Journal of Pediatric Hematology/oncology
Adlette Inati, Anthony G Mansour, Toni Sabbouh, Ghid Amhez, Ahmad Hachem, Hussein A Abbas
Hydroxyurea, blood transfusions, and hematopoietic stem cell transplantation represent the 3 disease-modifying therapies in children with sickle cell disease (SCD). Blood transfusions play an increasingly important role in both prevention and management of SCD complications in pediatric population. This review will focus on the indications of blood episodic and chronic transfusion in children with SCD and modalities of its administration. It will also highlight the complications of this life-saving therapy and ways of optimizing transfusion to minimize its associated risks...
August 9, 2016: Journal of Pediatric Hematology/oncology
Archie Ramaswami, Danya J Rosen, Jaime Chu, Birte Wistinghausen, Ronen Arnon
Deferesirox (DFX), an oral chelating agent, is used to treat chronic iron overload in several hematological diseases such as β-thalassemia, sickle cell disease, and myelodysplastic anemia. DFX is generally well tolerated with the exception of gastrointestinal disturbances and rash, although cases of renal toxicity, as well as acute and chronic liver failure, have been reported in adults and children. Here we describe a 3-year-old girl with β-thalassemia undergoing treatment with DFX who presented with acute liver failure and Fanconi's syndrome...
July 29, 2016: Journal of Pediatric Hematology/oncology
Sahin Erdol, Mehmet Ture, Birol Baytan, Tahsin Yakut, Halil Saglam
There are published reports stating that some of the congenital metabolic diseases, such as lysinuric protein intolerance, multiple sulphatase deficiency, galactosemia, Gaucher disease, Pearson syndrome, and galactosialidosis, might lead to secondary hemophagocytic lymphohistiocytosis (HLH). However, to date, to our knowledge, the long-chain 3-hydroxyacyl-CoA dehydrogenase (LCHAD) deficiency has never been investigated among patients with HLH. Here, we report on a patient who was referred to our institution for a differential diagnosis of pancytopenia, liver failure, and rhabdomyolysis...
November 2016: Journal of Pediatric Hematology/oncology
Gem Mohan, Julius X Scott, Rizwana Nasrin, Latha Sneha, Rakesh Manohar, Lalitha Subramanian, Sowmiya Narayani, Aruna Rajendran
BACKGROUND: The first counseling or the exchange between the physician and the parent(s) of children with cancer is of vital importance as it sets the tone for the rest of the treatment. The goal of our study was to find out the preferences among parents of Indian children with cancer regarding communication and breaking of bad news when fully informed about the diagnosis. MATERIALS AND METHODS: A sample of 60 parents who had been counseled within 3 months from diagnosis were interviewed with a prepared questionnaire directed at eliciting their experiences with the physicians who broke the bad news to them and also suggestions to improve the exchange...
November 2016: Journal of Pediatric Hematology/oncology
Mozhgan Hashemieh, Azita Azarkeivan, Kourosh Sheibani
The present study was conducted to compare the coagulation factors between splenectomized and nonsplenectomized thalassemia intermedia (TI) patients as well as a healthy control group. A total of 60 splenectomized and 60 nonsplenectomized TI patients and 60 healthy controls participated in this case-control study. The level of homocysteine, protein C and S, antithrombin III, factors V and VIII, fibrinogen, and D-dimer were measured in all groups. Our results indicated a significant reduction of protein C and S and fibrinogen in TI patients compared with healthy controls (P<0...
November 2016: Journal of Pediatric Hematology/oncology
Mette Levinsen, Arja Harila-Saari, Kathrine Grell, Olafur Gisli Jonsson, Mervi Taskinen, Jonas Abrahamsson, Kim Vettenranta, Ann Åsberg, Juha Risteli, Jesper Heldrup, Kjeld Schmiegelow
We investigated efficacy and toxicity of replacing conventional triple (cytarabine, methotrexate, and hydrocortisone) intrathecal therapy (TIT) with liposomal cytarabine during maintenance therapy among 40 acute lymphoblastic leukemia patients. Twenty-eight of 29 patients in the TIT arm received TIT and 9/11 in the liposomal cytarabine arm received liposomal cytarabine. Arachnoiditis occurred in all initial 5 patients given liposomal cytarabine and intrathecal prednisolone succinate. Subsequently liposomal cytarabine was given with systemic dexamethasone...
November 2016: Journal of Pediatric Hematology/oncology
Alper Ozcan, Turkan Patiroglu, Hamit Acer, Hakan Gumus, Serkan Senol, Musa Karakukcu, Mehmet A Ozdemir, Ekrem Unal
Congenital dyserythropoietic anemia type II belongs to a subtype of bone marrow failure syndrome, which is characterized by monolineage involvement and typical morphologic abnormalities in erythroid precursor cells resulting in different degrees of hyporegenerative anemia. Moreover, reticulocytosis, which is not corresponding to the degree of anemia, with jaundice and splenomegaly are major diagnostic criteria. Causative gene is located at SEC23B. Although stroke among children is rare, it can cause significant morbidity and mortality...
November 2016: Journal of Pediatric Hematology/oncology
Mary E Bauman, Michael Hawkes, Aisha Bruce, Suzanne Siddons, Patti Massicotte
Children with conditions requiring chronic warfarin therapy have increased. The importance of receiving immunizations in this population is magnified due to potential weakness in their immune response. There is concern about immunizing on therapeutic anticoagulation due to risk of hematomas and the influence of vaccine on warfarin metabolism. This study evaluated the influence of vaccines on warfarin effect as measured by the International Normalized Ratio and the clinically relevant hematomas or bruising postimmunization...
November 2016: Journal of Pediatric Hematology/oncology
Mohamed Fawzy, Mohamed Sedky, Hossam ElZomor, Magdy El Sherbiny, Emad Salama, Ahmed Mahdy
BACKGROUND: Nearly half of soft tissue sarcomas are nonrhabdomyosarcomas (NRSTSs). The low-grade (LG) form comprises a heterogenous group of diseases that rarely metastasize but are known for local recurrence. AIM OF THE STUDY: The aim of the study was to retrospectively evaluate pediatric LG-NRSTS with regard to demography, survival, and factors affecting outcome in Egyptian patients. PATIENTS AND METHODS: The study reviewed 66 NRSTS patients who presented to the Pediatric Oncology Department, National Cancer Institute, Cairo University, between January 2008 and December 2013...
November 2016: Journal of Pediatric Hematology/oncology
Line Stensig Lynggaard, Hanne V Marquart, Eigil Kjeldsen, Hans O Madsen, Henrik Hasle
A small group of children with acute lymphoblastic leukemia (ALL) have a preleukemic phase of pancytopenia followed by a period of spontaneous remission before the diagnosis (pre-ALL). A 6-year-old girl presented with pancytopenia, fever, and myelodysplasia. Following transient remission pre-B ALL was diagnosed 14 months later. Clonal B-lineage blasts at the period of pancytopenia were identified retrospectively. The interval between pre-ALL and ALL-diagnosis was longer than previously reported. The infection was clinically severe and might have induced a significant endogenous corticosteroids production resulting in the long-lasting remission...
November 2016: Journal of Pediatric Hematology/oncology
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