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pediatrics, pediatric hematology

Ulkem Kocoglu Barlas, Hasan Serdar Kıhtır, Nilufer Goknar, Melike Ersoy, Nihal Akcay, Esra Sevketoglu
BACKGROUND: Hemolytic uremic syndrome (HUS) is a clinical syndrome characterized by hemolytic anemia, thrombocytopenia, and acute kidney injury. Atypical hemolytic uremic syndrome (aHUS) is a devastating disease with significant mortality and high risk of progression to end-stage kidney disease. It is mostly caused by dysregulation of the alternative complement pathway. Cobalamin C (Cbl C) defect is a genetic disorder of cobalamin metabolism and is a rare cause of HUS. CASE-DIAGNOSIS/TREATMENT: We present a 6-month-old male infant who was admitted to the pediatric intensive care unit (PICU) due to restlessness, severe hypertension, anemia, respiratory distress, and acute kidney injury...
March 20, 2018: Pediatric Nephrology: Journal of the International Pediatric Nephrology Association
Françoise Bernaudin, Cécile Arnaud, Annie Kamdem, Isabelle Hau, Françoise Lelong, Ralph Epaud, Corinne Pondarré, Serge Pissard
Sickle cell anemia (SCA), albeit monogenic, has heterogeneous phenotypic expression, mainly related to the level of hemoglobin F (HbF). No large cohort studies have ever compared biological parameters in patients with major β-globin haplotypes; ie, Senegal (SEN), Benin (BEN), and Bantu/Central African Republic (CAR). The aim of this study was to evaluate the biological impact of α genes, β haplotypes, and glucose-6-phosphate dehydrogenase (G6PD) activity at baseline and with hydroxyurea (HU). Homozygous HbS patients from the Créteil pediatric cohort with available α-gene and β-haplotype data were included (n = 580; 301 females and 279 males) in this retrospective study...
March 27, 2018: Blood Advances
Rosa Ruchlemer, Michal Amit-Kohn, Ariella Tvito, Irena Sindelovsky, Ari Zimran, David Raveh-Brawer
PURPOSE: Bone loss-osteopenia and osteoporosis-is a recognized consequence of solid tumors in adults, of pediatric hematological malignancies, and of the treatment for these diseases, but little research has been published on the adverse effects of hematological malignancies on the bone in adults. The aim of this study is to identify hematological diseases that are associated with the highest prevalence and severity of osteoporosis. METHODS: We evaluated DXA (dual-energy X-ray absorptiometry) in a cross-section of 181 adult patients with hematological neoplasms, excluding multiple myeloma...
March 16, 2018: Supportive Care in Cancer: Official Journal of the Multinational Association of Supportive Care in Cancer
Karin P S Langenberg-Ververgaert, Ronald M Laxer, Angela S Punnett, L Lee Dupuis, Yaron Finkelstein, Oussama Abla
Familial Mediterranean fever (FMF) has been associated with hematological malignancies but has not been reported in association with Hodgkin lymphoma (HL). We hereby describe the first pediatric patient with FMF and stage IIA nodular sclerosis HL. She was treated with prednisone, doxorubicin, vincristine and etoposide (OEPA) being on therapy with colchicine. However, she suffered more than expected treatment-related toxicity attributed either to chemotherapy (severe neutropenia) or colchicine (Abdominal pains and diarrhoea)...
2018: Mediterranean Journal of Hematology and Infectious Diseases
M Colinart-Thomas, V Noël, G Roques, S Gordes-Grosjean, M Abely, C Pluchart
Sickle cell disease, a hemoglobin disorder with autosomal recessive transmission, is one of the most common genetic diseases screened in France. Thanks to early management, 95% of sickle cell patients reach adulthood and require transition from pediatric care to adult care. Through a retrospective study of records from serious sickle cell patients over 17 years old, followed in the hematology-oncology pediatric unit of Reims University Hospital Center in France, we analyzed transition conditions, compared pediatric and adult management, and proposed a plan for transition care...
March 9, 2018: Archives de Pédiatrie: Organe Officiel de la Sociéte Française de Pédiatrie
Diana Bellavia, Rocco Palermo, Maria Pia Felli, Isabella Screpanti, Saula Checquolo
Acute lymphoblastic leukemia (ALL) is the most common pediatric malignancy. Although the therapy of ALL has significantly improved, the heterogeneous genetic landscape of the disease often causes relapse, which is difficult to treat. Achieving a positive outcome for patients with relapsed or refractory ALL remains a challenging issue. The high prevalence of NOTCH-activating mutations in T-cell acute lymphoblastic leukemia (T-ALL) and the central role of NOTCH signaling in regulating cell survival and growth of ALL provide a rationale for the development of Notch signaling-targeted strategies in this disease...
March 12, 2018: Expert Opinion on Therapeutic Targets
Neil Blumberg, Jill M Cholette, Anthony P Pietropaoli, Richard Phipps, Sherry L Spinelli, Michael P Eaton, Suzie A Noronha, Jerard Seghatchian, Joanna M Heal, Majed A Refaai
Crystalloid infusion is widely employed in patient care for volume replacement and resuscitation. In the United States the crystalloid of choice is often normal saline. Surgeons and anesthesiologists have long preferred buffered solutions such as Ringer's Lactate and Plasma-Lyte A. Normal saline is the solution most widely employed in medical and pediatric care, as well as in hematology and transfusion medicine. However, there is growing concern that normal saline is more toxic than balanced, buffered crystalloids such as Plasma-Lyte and Lactated Ringer's...
February 21, 2018: Transfusion and Apheresis Science
Małgorzata Janeczko-Czarnecka, Maryna Krawczuk-Rybak, Irena Karpińska-Derda, Maciej Niedźwiecki, Katarzyna Musioł, Magdalena Ćwiklińska, Katarzyna Drabko, Katarzyna Mycko, Tomasz Ociepa, Katarzyna Pawelec, Danuta Januszkiewicz-Lewandowska, Marek Ussowicz, Blanka Rybka, Renata Ryczan-Krawczyk, Andrzej Kołtan, Grażyna Karolczyk, Agnieszka Zaucha-Prażmo, Wanda Badowska, Krzysztof Kałwak
BACKGROUND: Chronic myeloid leukemia (CML) constitutes only 2-3% of all leukemias in pediatric patients. Philapelphia chromosome and BCR-ABL fusion are genetic hallmarks of CML, and their presence is crucial for targeted molecular therapy with tyrosine kinase inhibitors (TKIs), which replaced hematopoietic stem cell transplantation (HSCT) as a standard first-line therapy. The disease in pediatric population is rare, and despite molecular and clinical similarities to CML in adults, different approach is needed, due to the long lifetime expectancy and distinct developmental characteristics of affected children...
January 2018: Advances in Clinical and Experimental Medicine: Official Organ Wroclaw Medical University
Maria Cecília Freire de Melo, Thuanny Silva de Macedo, Julianelly Alves Biserra, Karina Luz Cavalcanti Rodrigues, Mônica Cruz, Aurora Karla de Lacerda Vidal
AIM: To report a case of a pediatric patient carrier of myelodysplastic syndrome (MDS) with severe oral infectious disease, in which antimicrobial photodynamic therapy (aPDT) was used as a therapeutic choice to support systemic treatment. METHODS AND RESULTS: This case report refers to a 1-year-old male patient with MDS and hospitalized for investigation and treatment of complications at a Pediatric Oncology and Hematology Center in Recife/PE, Brazil. The intraoral examination revealed a violaceous lesion surrounding the lower incisor teeth, which progressed with alteration of color to milky yellowish white, compromising the entire lower gingival border, leading to tooth mobility and consequent loss of teeth 71 and 81...
March 6, 2018: Special Care in Dentistry
Dorit Verhoeven, Arie Jan Stoppelenburg, Friederike Meyer-Wentrup, Marianne Boes
Primary immunodeficiency disorders (PIDs) convey increased susceptibility to infections and sometimes to malignancies, particularly lymphomas. Such cancer development can be contributed by immune impairments resulting in weakened immunological surveillance against (pre)malignant cells and oncogenic viruses. Molecular defects in PID-patients are therefore being clarified, identifying new targets for innovative immunotherapy. Particularly pediatric cancers are being scrutinized, where over one third of cancer-related deaths is accounted for by leukemia and lymphomas...
February 27, 2018: Clinical Immunology: the Official Journal of the Clinical Immunology Society
Min-Hua Tseng, Shih-Hua Lin, Chao-Yi Wu, Hui-Ping Chien, Huang-Yu Yang, Yung-Chang Chen, Yu-Ching Chou, Jing-Long Huang
Although aberrant complement activation is involved in the pathogenesis of systemic lupus erythematosus (SLE), the role of complement regulatory proteins in disease activity of SLE remains limited. We enrolled the pediatric-onset SLE patients from our cohort study over 10 years. The clinical and laboratory data including SLEDAI disease activity score, and serum complement factor H (CFH), CFI, CD46, C5a, and C5b-9 in the active and remission phases were determined. Glomerular C5b-9 deposition as a complement activity marker was also examined...
February 2, 2018: Oncotarget
Parisa Badiee, Maral Choopanizadeh, Abdolkarim Ghadimi Moghadam, Ali Hossaini Nasab, Hadis Jafarian, Ahmad Shamsizadeh, Jafar Soltani
Background and Objectives: Colonization of Candida species is common in pediatric patients admitted to hematology-oncology wards. The aim of this study was to identify colonized Candida species and their susceptibility patterns in hematologic pediatric patients. Materials and Methods: Samples were collected from mouth, nose, urine and stool of the patients admitted to five university hospitals and cultured on sabouraud dextrose agar. The isolates were identified by API 20 C AUX system and their susceptibility patterns were evaluated by CLSI M27-A3 and S4...
December 2017: Iranian Journal of Microbiology
Yeter Düzenli Kar, Zeynep Canan Özdemir, Özcan Bör
Aim: Febrile neutropenia is an important cause of mortality and morbidity in hematology-oncology patients undergoing chemotherapy. The objective of this study was to evaluate febrile neutropenic episodes in children with malignancy. Material and Methods: Sixty-eight children who received chemotherapy for malignancy between 2010 and 2015 were retrospectively reviewed. The demographic characteristics, laboratory data, infection foci, and frequency of microorganisms grown in culture were examined...
December 2017: Türk Pediatri Arşivi
Lars H Breimer, Torbjörn K Nilsson, Michael E Breimer
Declaration of conflicts of interest (COI, understood mainly as financial) in medical publications is long established. Most journals refer only to the guidelines of the International Committee of Medical Journal Editors (ICMJE) but not to those of the WAME (World Association of Medical Editors). We surveyed 17 journals and found only one (BJOG), which explicitly mentioned "religious interest" as an example of a possible COI and one other journal included "personal belief" (Journal of Obstetrics and Gynaecology of India) as a COI...
February 7, 2018: Indian Journal of Medical Ethics
Maria Papadopoulou, Paraskevi Panagopoulou, Anastasia Papadopoulou, Emmanuel Hatzipantelis, Ioannis Efstratiou, Assimina Galli-Tsinopoulou, Efimia Papadopoulou-Alataki
Langerhans cell histiocytosis (LCH) is a rare hematologic disorder that results from the clonal multiplication and accumulation of immature dendritic Langerhans cells. Its reported incidence rate varies, but is considered to be 2.6-8.9 per million children who are <15 years of age each year. It may affect any system or organ. The present study reported 4 pediatric LCH cases in order to highlight the heterogeneity of the initial presentation, and the pitfalls that may mislead clinicians and delay diagnosis...
March 2018: Molecular and Clinical Oncology
Marcin Waligora, Malgorzata M Bala, Magdalena Koperny, Mateusz T Wasylewski, Karolina Strzebonska, Rafał R Jaeschke, Agnieszka Wozniak, Jan Piasecki, Agnieszka Sliwka, Jerzy W Mitus, Maciej Polak, Dominika Nowis, Dean Fergusson, Jonathan Kimmelman
BACKGROUND: Pediatric Phase I cancer trials are critical for establishing the safety and dosing of anti-cancer treatments in children. Their implementation, however, must contend with the rarity of many pediatric cancers and limits on allowable risk in minors. The aim of this study is to describe the risk and benefit for pediatric cancer Phase I trials. METHODS AND FINDINGS: Our protocol was prospectively registered in PROSPERO (CRD42015015961). We systematically searched Embase and PubMed for solid and hematological malignancy Phase I pediatric trials published between 1 January 2004 and 1 March 2015...
February 2018: PLoS Medicine
M Begum, A Islam, A A Rahman, M Akter, S T Alam, R Tasmeen
Acute leukemias are the most common child hood malignancy, of which acute myeloid leukemia (AML) are 15 to 20%. Abandonment is one of the most important causes of treatment failure in AML in developing countries. Lost to follow-up is also a big problem in low income countries. Many patients stop therapy soon after diagnosis due to cost, distance and ignorance. To determine the abandonment, outcome and treatment related mortality (TRM) and morbidity among children with AML. This prospective observational study was conducted in the Department of Pediatric hematology and Oncology, Bangabandhu Sheikh Mujib Medical University (BSMMU), Dhaka from February 2013 to January 2014...
January 2018: Mymensingh Medical Journal: MMJ
Haim Werner, Shilhav Meisel-Sharon, Ilan Bruchim
The insulin-like growth factor-1 receptor (IGF1R) has been identified as a potent anti-apoptotic, pro-survival tyrosine kinase-containing receptor. Overexpression of the IGF1R gene constitutes a typical feature of most human cancers. Consistent with these biological roles, cells expressing high levels of IGF1R are expected not to die, a quintessential feature of cancer cells. Tumor specific chromosomal translocations that disrupt the architecture of transcription factors are a common theme in carcinogenesis...
February 19, 2018: Molecular Cancer
Kimberly D Defoe, Jennifer Jupp, Tara Leslie
Objectives To describe key activities performed by a newly deployed clinical pharmacist in an outpatient pediatric hematology, oncology, transplant clinic. To demonstrate how utilization of the pharmacist evolved, as indicated by changes in frequency of key activities, during the first four months of integration. Design Clinical pharmacists were made consistently available in an outpatient clinic serving hematology, oncology, transplant patients and their families. A list of key activities, based on provincial clinical pharmacist standards, was created to provide a framework for the role...
January 1, 2018: Journal of Oncology Pharmacy Practice
Tal Katz, Jeffrey Schatz, Carla W Roberts
PURPOSE: Sickle cell disease (SCD) imparts an increased risk for obstructive sleep apnea (OSA) in childhood. Studies of pediatric SCD have identified an increased risk for pain and neurologic complications with comorbid OSA. We determined the rate of a broad range of SCD-related medical complications to better characterize the spectrum of SCD complications related to OSA. METHODS: Retrospective chart review at a single hematology clinic identified 641 youth with SCD who received consistent screenings for OSA as part of routine hematological health maintenance visits over an 11-year period...
February 15, 2018: Sleep & Breathing, Schlaf & Atmung
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