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Journal of Pediatric Hematology/oncology

Mohamed Y Elsaid, Kara G Gill, Ankush Gosain, Peter F Nichol, Charles M Leys, Darya Buehler, Catherine P Leith, Neha J Patel
BACKGROUND: Coincidence of renal cell carcinoma (RCC) and hematologic malignancies has been reported in adults but not in children. OBSERVATION: We report a case of a 16-year-old girl in whom RCC was incidentally discovered on the computed tomography scan that was performed to stage her underlying Hodgkin lymphoma. Analysis of constitutional cytogenetics for common genetic aberrations that predispose to RCC did not reveal any mutations or genetic variations. However, cytogenetics on the RCC tumor demonstrated a rare reciprocal translocation between chromosomes 6 and 11, t(6;11)(p21;q12)...
January 13, 2017: Journal of Pediatric Hematology/oncology
Abigail V W Kumral, William C Petersen, Christopher Heitz, Linda A Waggoner-Fountain, Brian C Belyea
Secondary hemophagocytic lymphohistiocytosis (sHLH) is a rare hyperinflammatory disorder caused by an aberrant immune response to a number of infectious or inflammatory conditions. Successful treatment of this potentially fatal condition requires early recognition and prompt therapy directed at the underlying trigger. In this report, we describe the clinical presentation, diagnostic findings, management, and outcome of a child with Lemierre's syndrome-associated sHLH. This is the first reported association of these 2 rare conditions and expands the number of known triggers for sHLH...
January 12, 2017: Journal of Pediatric Hematology/oncology
Amanda DiNofia, Kate Shafer, Katarina Steacy, Karim Thomas Sadak
Most childhood cancer survivors need life-long care with specialized late-effects surveillance and screening. As these children age into adulthood, it is imperative to continue their survivor-focused care. To do so, health care systems must be prepared to care for this growing and aging population. This includes creating models of care that take into account the needs and desires of all key stakeholders: survivors, parents, pediatric providers, and adult providers. This clinical observation describes that parents desire comprehensive and highly accessible survivorship care that promotes survivor independence; yet, they also want to have a central role in their child's survivorship care...
January 12, 2017: Journal of Pediatric Hematology/oncology
Kevin O McNerney, Juan C Vasquez, Michael W Kent, Joseph M McNamara
We report a case of a child with pre-B cell acute lymphoblastic leukemia undergoing maintenance chemotherapy with 6-mercaptopurine and methotrexate (MTX) who developed sinusoidal obstruction syndrome after being treated with ciprofloxacin for BK viremia. This case represents a rare complication of maintenance therapy with MTX and 6-mercaptopurine, and suggests a drug interaction between ciprofloxacin and MTX.
January 12, 2017: Journal of Pediatric Hematology/oncology
Mohsen S Elalfy, Nayera H K El Sherif, Tarek M Kamal, Nihal H Aly
BACKGROUND: The klf10 gene could indirectly modify γ-globin chain production and hence the level of fetal hemoglobin (HbF) ameliorating the phenotype of β-hemoglobinopathies and the response to hydroxycarbamide (hydroxyurea [HU]) therapy. In this study, we aimed to evaluate the frequency of different genotypes for the klf10 gene in β-thalassemia major (B-TM), β-thalassemia intermedia (B-TI), and sickle cell disease (SCD) patients by polymerase chain reaction and to assess its relation to disease phenotypes and HU response...
January 12, 2017: Journal of Pediatric Hematology/oncology
Amanda Christina C Dujua, Flerida G Hernandez
This retrospective cohort study is primarily aimed to evaluate the outcome of children ages 0 to 18 years old, with acute lymphoblastic leukemia and treated with a modified Berlin-Frankfurt-Muenster/Hong Kong Acute Lymphoblastic Leukemia (BFM95-HKALL97) protocol at University of Santo Tomas Hospital from January 2005 to December 2009. Seventy-eight patients were included. Majority were between 1 and 10 years old (87.2%), male (61.5%), with normal nutritional status, and classified as upper socioeconomic class (65...
January 12, 2017: Journal of Pediatric Hematology/oncology
Prasad R Koduru, Kathleen Wilson, Jiadi Wen, Rolando Garcia, Sangeeta Patel, Sara A Monaghan
Concurrent perturbations in different driver genes have been reported primarily in lymphoma. In acute myeloid leukemia (AML), cases with concurrent alterations in 2 driver genes are infrequently reported. In contrast to pathogenetic pathways in lymphoma with concurrently perturbed genes, the initial gene alteration in AML arrests maturation and the alteration in the second gene promote self-renewal of the blasts. Here, we report a unique case of infantile leukemia in which chromothripsis in chromosome 8 completely altered the G-band structure and resulted in concurrent changes in MOZ/NCOA2, FGFR1, RUNX1T1, and RUNX1...
January 12, 2017: Journal of Pediatric Hematology/oncology
Salaisudhanprabu Solmuthu Azhagan, Arathi Srinivasan, Shobana Rajendran, Dema Ravinder, Rahul Yadav, Priya Ramachandran, Mukul Vij, Julius Xavier Scott
Perinatal neuroblastoma is the most common solid malignant tumor in infancy which comprises one fifth of all neuroblastomas. Most of them are of adrenal origin and extra-adrenal neuroblastoma is uncommon. We present a rare case of perinatal intrarenal neuroblastoma in a neonate who presented with an incidentally detected abdominal mass. These tumors cause diagnostic and therapeutic dilemma because of its uncommon location. Although very rare, neuroblastoma should be considered in the differential diagnosis of perinatally detected renal tumors...
January 12, 2017: Journal of Pediatric Hematology/oncology
Brent D Bauman, Christopher L Moertel, Eugene Zheng, Amanda Louiselle, Elizabeth Berdan, David Berger, Bradley Segura
INTRODUCTION: Neurofibromatosis type 1 (NF1) is the most commonly inherited autosomal dominant disorder in humans. NF1 patients have increased risk for gastrointestinal stromal tumors (GISTs). A Meckel's diverticulum (MD) represents a persistent embryonic omphalomesenteric duct characterized as a true diverticulum located near the ileocecal valve. We report a unique clinical case whereby a patient with NF1 developed a GIST within a MD. CASE: An adolescent male with NF1 presented with persistent lower abdominal pain...
January 12, 2017: Journal of Pediatric Hematology/oncology
Mozhgan Hashemieh, Mitra Radfar, Azita Azarkeivan, Giti Noghabaei, Kourosh Sheibani
The present study was performed to evaluate pancreatic hemosiderosis by means of magnetic resonance imaging (MRI) T2* and its relation to the diabetic state in thalassemic patients. One hundred thirty transfusion-dependent thalassemic patients from Zafar adult thalassemia clinic, Tehran, Iran, were enrolled in the study. Data such as age, type of thalassemia, age at diagnosis, transfusion duration, ferritin level, and fasting blood sugar results were gathered. Pancreatic MRI T2* was performed for all patients...
January 12, 2017: Journal of Pediatric Hematology/oncology
Alper Ozcan, Hamit Acer, Saliha Ciraci, Hakan Gumus, Musa Karakukcu, Turkan Patiroglu, Mehmet A Ozdemir, Ekrem Unal
Neuroblastoma is the most common extracranial solid tumor of childhood originating from sympathetic nervous system cells. Neuroblastoma has also been diagnosed in conjunction with other congenital conditions such as Hirschsprung's disease, congenital hypoventilation disorder, and neurofibromatosis type 1. Wolf-Hirschhorn syndrome is a congenital disorder caused by microdeletion of short arm of chromosome 4 encoding MSX1 gene with characteristic facial features. We describe a child with dysmorphic features, developmental delay, mental retardation who developed neuroblastoma at 2 years of age and cytogenetic analysis of blood lymphocytes revealed an interstitial deletion of 4p(15,2)...
January 12, 2017: Journal of Pediatric Hematology/oncology
Adrienne Viola, Lyn Balsamo, Joseph P Neglia, Pim Brouwers, Xiaomei Ma, Nina S Kadan-Lottick
Neurocognitive problems, including executive dysfunction, are potential late effects of pediatric acute lymphoblastic leukemia treatment. Surveillance for neurocognitive impairment in a timely and efficient manner is imperative to ongoing clinical care. We sought to determine if the Behavior Rating Inventory of Executive Function (BRIEF) Parent Form identified leukemia survivors with cognitive impairment. In this 28-site cross-sectional study, parents of 256 children, a mean of 8.9±2.2 years after treatment for standard-risk precursor-B acute lymphoblastic leukemia and in first remission, completed the BRIEF...
January 12, 2017: Journal of Pediatric Hematology/oncology
Tatsuo Watanabe, Takashi Kurata, Kenji Sano, Shigeru Suzuki, Tomoki Kaneko, Mitsuo Motobayashi, Tomonari Shigemura, Takuro Sumi, Kenichi Koike, Yozo Nakazawa
No abstract text is available yet for this article.
January 6, 2017: Journal of Pediatric Hematology/oncology
Aysel Ünlüsoy Aksu, Angela Caleffi, Antonello Pietrangelo, Sinan Sari, Ödül Eğritaş Gürkan, Zeliha Demirtaş, Güldal Yilmaz, Buket Dalgiç
BACKGROUND: Iron overload disorders are hereditary hemochromatosis and secondary etiologies other than hereditary hemochromatosis. We describe 2 boys presenting with iron overload. Juvenile hemochromatosis and nonalcoholic steatohepatitis (NASH) related iron overload are the genetic and secondary causes, respectively. OBSERVATIONS: Both patients benefited from phlebotomy even if they had different etiologies. CONCLUSIONS: In childhood, the diagnosis of iron overload syndromes is crucial because they do not confront us with obvious symptoms and findings...
January 6, 2017: Journal of Pediatric Hematology/oncology
Szymon Janczar, Beata Zalewska-Szewczyk, Wojciech Mlynarski
Electrolyte abnormalities are hallmark metabolic disturbances during the treatment of acute lymphoblastic leukemia (ALL). Hyponatremia is an ominous laboratory sign in the setting of neoplasia. We analyzed the incidence, risk factors, associations, specific interventions and outcomes of severe hyponatremia in a single-center series of children with ALL. The incidence of severe hyponatremia, defined as serum sodium levels below 130 mmol/L on at least 2 of 3 consecutive days, was 11.9%. History of hyponatremia episode is associated with neurologic complications (P=0...
January 5, 2017: Journal of Pediatric Hematology/oncology
Zeliha Haytoglu, Nalan Yazici, Ayse Erbay
Because of the acute and life-threatening course of the hemophagocytic lymphohistiocytosis (HLH) syndrome, International Histiocyte Society guidelines recommend chemoimmune therapy for the treatment of both primary and secondary HLH (sHLH). To manage children with sHLH, instead of HLH-2004 protocol we considered less immunosuppressive/cytotoxic approach. We assessed 12 children who fulfilled the diagnostic criteria for sHLH between January 2009 and March 2015. Multivariate Cox regression analysis showed that ferritin levels (hazard ratio=1...
January 5, 2017: Journal of Pediatric Hematology/oncology
Nikki Cornell, Michael Eisenhut, Stalin Ramprakash
BACKGROUND: Various different formulae are used to calculate blood transfusion volumes in thalassemia. Using the right formula will avoid iron overload and complications of undertransfusion. OBSERVATIONS: Five years of transfusion data in 11 children with thalassemia showed that no single formula-calculated transfusion volumes accurately. The formula used in the United Kingdom with a hematocrit of the transfused blood of 0.6: (0.4×body weight×desired raise in hemoglobin [g/L]) yielded a volume closest to empirically determined requirements on average...
January 5, 2017: Journal of Pediatric Hematology/oncology
Wafaa M Rashed, Wael Zekri, Madiha Awad, Hala Taha, Badr Abdalla, Ahmad S Alfaar
Childhood adrenocortical carcinoma (ACC) is a rare tumor and its association with acute lymphoblastic leukemia (ALL) is even rarer. One such case is discussed in this case report. A 3-year-old patient was concomitantly diagnosed with ALL and an initially nonmetastatic ACC. Management started by following the Total XV protocol without a window phase. Left adrenalectomy was conducted after the consolidation phase. Recurrence of a mass at the tumor bed was discovered at week 33 of the continuation phase. Reexcision was conducted, followed by the administration of an ACC protocol including cisplatin, etoposide, and doxirubicin...
January 5, 2017: Journal of Pediatric Hematology/oncology
Michael V Ortiz, Heather Magnan, Emily K Slotkin, Srikanth R Ambati, Alexander J Chou, Leonard H Wexler, Paul A Meyers, Michael F Walsh, Todd Heaton, Leonard N Girardi, Suzanne L Wolden, Anita P Price, Jennifer A Kennedy, Ahmet Zehir, Meera Hameed, Michael F Berger, Alex Kentsis, Neerav Shukla
A 16-year-old male was diagnosed with Ewing sarcoma of the ribcage with pulmonary metastases. Six months after completion of scheduled therapy, he was found to have a new intracardiac mass, presumed recurrent Ewing sarcoma. EWSR1 fusion was not detected by droplet digital polymerase chain reaction from blood plasma. After no improvement with salvage chemotherapy, he underwent surgical resection that identified a low-grade spindle cell sarcoma. Despite the near-synchronous presentation of 2 unrelated sarcomas, extensive genomic analyses did not reveal any unifying somatic or germline mutations nor any apparent cancer predisposition...
January 5, 2017: Journal of Pediatric Hematology/oncology
Yasutomo Funakoshi, Masahiko Okada, Soichiro Kawata, Nobuhiro Ito, Kuniko Abe, Hiroyuki Moriuchi
No abstract text is available yet for this article.
January 5, 2017: Journal of Pediatric Hematology/oncology
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