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https://www.readbyqxmd.com/read/29150103/body-composition-of-fanconi-anemia-patients-after-hematopoietic-stem-cell-transplantation
#1
Priscilla Peixoto Policarpo da Silva, Daniella Schmit, Carmem Bonfim, Denise Johnsson Campos, Estela Iraci Rabito, Regina Maria Vilela
INTRODUCTION: Fanconi anemia is a rare genetic disease linked to bone marrow failure; a possible treatment is hematopoietic stem cell transplantation. Changes in the nutritional status of Fanconi anemia patients are not very well known. This study aimed to characterize body composition of adult, children and adolescent patients with Fanconi anemia who were submitted to hematopoietic stem cell transplantation or not. METHODS: This cross-sectional study enrolled 63 patients (29 adults and 34 children and adolescents)...
October 2017: Revista Brasileira de Hematologia e Hemoterapia
https://www.readbyqxmd.com/read/29146883/a-landscape-of-germline-mutations-in-a-cohort-of-inherited-bone-marrow-failure-patients
#2
Olivier Bluteau, Marie Sebert, Thierry Leblanc, Régis Peffault de Latour, Samuel Quentin, Elodie Lainey, Lucie Hernandez, Jean-Hugues Dalle, Flore Sicre de Fontbrune, Etienne Lengline, Raphael Itzykson, Emmanuelle Clappier, Nicolas Boissel, Naddia Vasquez, Mélanie Da Costa, Julien Masliah-Planchon, Wendy Cuccuini, Anna Raimbault, Louis De Jaegere, Lionel Adès, Pierre Fenaux, Sébastien Maury, Claudine Schmitt, Marc Muller, Carine Domenech, Nicolas Blin, Bénédicte Bruno, Isabelle Pellier, Mathilde Hunault, Stéphane Blanche, Arnaud Petit, Guy Leverger, Gérard Michel, Yves Bertrand, André Baruchel, Gérard Socié, Jean Soulier
Bone marrow failure (BMF) in children and young adults is often suspected to be inherited, but in many cases diagnosis remains uncertain. We studied a cohort of 179 patients (from 173 families) with BMF of suspected inherited origin but unresolved diagnosis after medical evaluation and Fanconi anemia exclusion. All patients had cytopenias, and 12.0% presented ≥5% bone marrow blast cells. Median age at genetic evaluation was 11 years; 20.7% of patients were aged ≤2 years and 36.9% were ≥18 years. We analyzed genomic DNA from skin fibroblasts using whole-exome sequencing, and were able to assign a causal or likely causal germline mutation in 86 patients (48...
November 16, 2017: Blood
https://www.readbyqxmd.com/read/29145822/ultrasonographic-parameters-of-the-liver-spleen-and-kidneys-among-a-cohort-of-school-children-in-sri-lanka
#3
Dianne Tania Dayanthi Warnakulasuriya, Pattiya Pathirennahalage Uddika Chamila Peries, Yaddehi Arachchillage Chaminda Rathnasekara, KaluArachchige Thushari Madurika Jayawardena, Angappulige Upasena, Anada Rajitha Wickremasinghe
BACKGROUND: Liver, spleen and kidney dimensions on ultrasonography vary with the age, weight and ethnicity. Reference standards of these parameters for normal Sri Lankan children are not available. Our aim was to establish normative data for longitudinal length of liver, spleen and kidneys in healthy children. METHOD: Three hundred fifty-seven children, 5-13 years of age were selected from two randomly selected schools in the Gampaha district in the western province of Sri Lanka...
November 16, 2017: BMC Pediatrics
https://www.readbyqxmd.com/read/29143641/effects-of-deworming-on-child-and-maternal-health-a-literature-review-and-meta-analysis
#4
Winter Maxwell Thayer, Adrienne Clermont, Neff Walker
BACKGROUND: Soil-transmitted helminth infections are widespread. Many studies have been published on the topic of deworming. The Lives Saved Tool (LiST) is a software package that uses a deterministic mathematical model to estimate the effect of scaling up interventions on maternal and child health outcomes. This review investigates the scope of available evidence for benefits of deworming treatments in order to inform a decision about possible inclusion of deworming as an intervention in LiST...
November 7, 2017: BMC Public Health
https://www.readbyqxmd.com/read/29140934/response-of-symptomatic-persistent-chronic-disseminated-candidiasis-to-corticosteroid-therapy-in-immunosuppressed-pediatric-patients-case-study-and-review-of-the-literature
#5
Vered Shkalim-Zemer, Itzhak Levi, Salvador Fischer, Hannah Tamary, Joanne Yakobovich, Gali Avrahami, Gil Gilad, Sara Elitzur, Isaac Yaniv, Ronit Elhasid, Michal Manistersky, Itamar Shalit
BACKGROUND: Chronic disseminated candidiasis (CDC) is a severe invasive fungal infection principally observed during neutrophil recovery in patients with acute leukemia treated with intensive chemotherapy. Its pathophysiology remains unclear. We describe the management of six children with symptomatic CDC who did not respond to antifungal therapy. METHODS: The databases of the hematology-oncology departments of two tertiary pediatric medical centers were searched for all patients diagnosed with CDC from 2003 to 2015 who responded to corticosteroids after failing antifungal therapy...
November 14, 2017: Pediatric Infectious Disease Journal
https://www.readbyqxmd.com/read/29127678/allogeneic-matched-related-transplantation-for-%C3%AE-thalassemia-and-sickle-cell-anemia
#6
Françoise Bernaudin, Corinne Pondarré, Claire Galambrun, Isabelle Thuret
Allogeneic hematopoietic stem cell transplantation (HSCT) can cure single gene disorders such as thalassemia and sickle cell anemia (SCA). These non-malignant diseases have in common severe hemolytic anemia and high proliferative bone marrow, requiring frequent transfusions. The risk of rejection is high and graft-vs-host disease is not desirable. Important progress has been made in the management of these diseases, including leukocyte depletion of blood products, and chelation therapy, for both diseases, and erythrocytapheresis and hydroxycarbamide for SCA...
2017: Advances in Experimental Medicine and Biology
https://www.readbyqxmd.com/read/29091726/are-there-synergies-from-combining-hygiene-and-sanitation-promotion-campaigns-evidence-from-a-large-scale-cluster-randomized-trial-in-rural-tanzania
#7
Bertha Briceño, Aidan Coville, Paul Gertler, Sebastian Martinez
SUMMARY: The current evidence on handwashing and sanitation programs suggests limited impacts on health when at-scale interventions have been tested in isolation. However, no published experimental evidence currently exists that tests the interaction effects between sanitation and handwashing. We present the results of two large-scale, government-led handwashing and sanitation promotion campaigns in rural Tanzania, with the objective of tracing the causal chain from hygiene and sanitation promotion to changes in child health outcomes and specifically testing for potential interaction effects of combining handwashing and sanitation interventions...
2017: PloS One
https://www.readbyqxmd.com/read/29070567/assessment-of-iron-status-in-settings-of-inflammation-challenges-and-potential-approaches
#8
REVIEW
Parminder S Suchdev, Anne M Williams, Zuguo Mei, Rafael Flores-Ayala, Sant-Rayn Pasricha, Lisa M Rogers, Sorrel Ml Namaste
The determination of iron status is challenging when concomitant infection and inflammation are present because of confounding effects of the acute-phase response on the interpretation of most iron indicators. This review summarizes the effects of inflammation on indicators of iron status and assesses the impact of a regression analysis to adjust for inflammation on estimates of iron deficiency (ID) in low- and high-infection-burden settings. We overviewed cross-sectional data from 16 surveys for preschool children (PSC) (n = 29,765) and from 10 surveys for nonpregnant women of reproductive age (WRA) (n = 25,731) from the Biomarkers Reflecting the Inflammation and Nutritional Determinants of Anemia (BRINDA) project...
October 25, 2017: American Journal of Clinical Nutrition
https://www.readbyqxmd.com/read/29070550/iron-assessment-to-protect-the-developing-brain
#9
REVIEW
Michael K Georgieff
Iron deficiency (ID) before the age of 3 y can lead to long-term neurological deficits despite prompt diagnosis of ID anemia (IDA) by screening of hemoglobin concentrations followed by iron treatment. Furthermore, pre- or nonanemic ID alters neurobehavioral function and is 3 times more common than IDA in toddlers. Given the global prevalence of ID and the enormous societal cost of developmental disabilities across the life span, better methods are needed to detect the risk of inadequate concentrations of iron for brain development (i...
October 25, 2017: American Journal of Clinical Nutrition
https://www.readbyqxmd.com/read/29070549/iron-status-of-young-children-in-europe
#10
REVIEW
Liandré F van der Merwe, Simone R Eussen
Iron deficiency (ID) is common in young children aged 6-36 mo. Although the hazards associated with iron deficiency anemia (IDA) are well known, concerns about risks associated with excess iron intake in young children are emerging. To characterize iron status in Europe, we describe the prevalence of ID, IDA, iron repletion, and excess stores with the use of published data from a systematic review on iron intake and deficiency rates, combined with other selected iron status data in young European children. Various definitions for ID and IDA were applied across studies...
October 25, 2017: American Journal of Clinical Nutrition
https://www.readbyqxmd.com/read/29070548/excess-iron-considerations-related-to-development-and-early-growth
#11
REVIEW
Marianne Wessling-Resnick
What effects might arise from early life exposures to high iron? This review considers the specific effects of high iron on the brain, stem cells, and the process of erythropoiesis and identifies gaps in our knowledge of what molecular damage may be incurred by oxidative stress that is imparted by high iron status in early life. Specific areas to enhance research on this topic include the following: longitudinal behavioral studies of children to test associations between iron exposures and mood, emotion, cognition, and memory; animal studies to determine epigenetic changes that reprogram brain development and metabolic changes in early life that could be followed through the life course; and the establishment of human epigenetic markers of iron exposures and oxidative stress that could be monitored for early origins of adult chronic diseases...
October 25, 2017: American Journal of Clinical Nutrition
https://www.readbyqxmd.com/read/29067568/anemia-at-pediatric-intensive-care-unit-discharge-prevalence-and-risk-markers
#12
Pierre Demaret, Oliver Karam, Marisa Tucci, Jacques Lacroix, Hélène Behal, Alain Duhamel, Frédéric Lebrun, André Mulder, Stéphane Leteurtre
BACKGROUND: Anemia is prevalent at pediatric intensive care unit (PICU) admission and incident during PICU stay, but little is known about anemia at PICU discharge . Anemia after critical illness is an important issue because it could impact post-PICU outcome. We aimed to estimate the prevalence of anemia at PICU discharge and to determine its risk markers. METHODS: This is an ancillary study of a prospective observational study on transfusion practices conducted in the PICU of a tertiary care children's hospital...
October 24, 2017: Annals of Intensive Care
https://www.readbyqxmd.com/read/29051184/novel-use-of-hydroxyurea-in-an-african-region-with-malaria-noharm-a-trial-for-children-with-sickle-cell-anemia
#13
Robert O Opoka, Christopher M Ndugwa, Teresa S Latham, Adam Lane, Heather A Hume, Phillip Kasirye, James S Hodges, Russell E Ware, Chandy C John
Hydroxyurea treatment is recommended for children with sickle cell anemia (SCA) living in high-resource malaria-free regions, but its safety and efficacy in malaria-endemic sub-Saharan Africa, where the greatest sickle cell burden exists, remain unknown. In vitro studies suggest hydroxyurea could increase malaria severity, and hydroxyurea-associated neutropenia could worsen infections. NOHARM was a randomized, double-blinded, placebo-controlled trial conducted in malaria-endemic Uganda, comparing hydroxyurea to placebo at 20 ± 2...
October 19, 2017: Blood
https://www.readbyqxmd.com/read/29045955/-comparison-between-continuous-noninvasive-hemoglobin-monitoring-and-venous-blood-hemoglobin-monitoring-in-children-with-kidney-disease
#14
X Y Liu, X Q Yang, H J Xiao, J Ding
OBJECTIVE: To assess the correlation and consistency between continuous noninvasive hemoglobin detection and venous blood hemoglobin detection in children with kidney disease, and try to analyze the affecting factors. Try to provide a reference for the monitoring of hemoglobin in children with kidney disease by continuous noninvasive hemoglobin detection technique. METHODS: Eighty-five inpatient children with kidney disease, 50 boys (58.8%) and 35 girls (41.2%), aged from 3 years old to 18 years old (9...
October 18, 2017: Beijing da Xue Xue Bao. Yi Xue Ban, Journal of Peking University. Health Sciences
https://www.readbyqxmd.com/read/29043149/pre-b-acute-lymphoblastic-leukemia-with-t-1-19-in-an-adult-initially-presenting-as-hematuria-and-bilateral-renal-enlargement-a%C3%A2-case-report-and-literature-review
#15
Jian Wu, Xiao-Ling Pi, Zhi-Bin Ye
Although pre-B acute lymphoblastic leukemia (ALL) is the most common type of renal leukemic infiltration; the renal infiltration with leukemia cells as the initial manifestation of leukemia is very rare. Translocation (1;19)(q23;p13) is one of the most common chromosomal abnormalities in patients with ALL and is observed in 5 - 6% of children with pre-B ALL. However, the incidence of t(1;19) in adults is lower, not exceeding 3%, and the prognosis of adult patients is usually poor. Herein, we report a 52-year-old female patient with pre-B ALL who initially presented as bilateral renal enlargement...
2017: Clin Nephrol Case Stud
https://www.readbyqxmd.com/read/29042868/characteristics-and-prognosis-of-hepatic-cytomegalovirus-infection-in-children-10-years-of-experience-at-a-university-hospital-in-korea
#16
Chae-Yeon Min, Joo Young Song, Su Jin Jeong
PURPOSE: Studies on cytomegalovirus (CMV) infections in immunocompetent children are lacking, and minimal information is available in the medical literature on hepatic manifestations and complications of CMV. The aims of this study were to evaluate the clinical characteristics, laboratory data, and prognosis of children with CMV hepatitis, and to investigate its prevalence at a single medical center in Korea over a 10-year period. METHODS: One hundred thirty-two children diagnosed with CMV infection based on specific markers (anti-CMV IgM, CMV polymerase chain reaction in blood and urine, or CMV culture of urine) were included in the study...
August 2017: Korean Journal of Pediatrics
https://www.readbyqxmd.com/read/29025399/plasmodium-falciparum-epcr-binding-pfemp1-expression-increases-with-malaria-disease-severity-and-is-elevated-in-retinopathy-negative-cerebral-malaria
#17
Estela Shabani, Benjamin Hanisch, Robert O Opoka, Thomas Lavstsen, Chandy C John
BACKGROUND: Expression of group A and the A-like subset of group B Plasmodium falciparum erythrocyte membrane protein 1 (PfEMP1) is associated with severe malaria (SM). The diversity of var sequences combined with the challenges of distinct classification of patient pathologies has made studying the role of distinct PfEMP1 variants on malaria disease severity challenging. The application of retinopathy in the recent years has provided a further method to clinically evaluate children with cerebral malaria (CM)...
October 13, 2017: BMC Medicine
https://www.readbyqxmd.com/read/29023457/the-dmt1-ivs4-44c-a-polymorphism-and-the-risk-of-iron-deficiency-anemia-in-children-with-celiac-disease
#18
MULTICENTER STUDY
Carlo Tolone, Giulia Bellini, Francesca Punzo, Alfonso Papparella, Erasmo Miele, Alessandra Vitale, Bruno Nobili, Caterina Strisciuglio, Francesca Rossi
BACKGROUND: Iron deficiency anemia in celiac disease is related to impaired duodenal mucosal uptake, due to villous atrophy. Iron enters the enterocytes through an apical divalent metal transporter, DMT1. Different DMT1 transcripts have been identified, depending on the presence of an iron-responsive element that allows DMT1 up-regulation during iron starvation. An intronic DMT1 polymorphism, IVS4+44C>A, has been associated with metal toxicity, and the CC-carriers show high iron levels...
2017: PloS One
https://www.readbyqxmd.com/read/29016324/hemoglobin-levels-and-the-risk-of-malaria-in-papua-new-guinean-infants-a-nested-cohort-study
#19
Patrick Lombardo, Paul Vaucher, Patricia Rarau, Ivo Mueller, Bernard Favrat, Nicolas Senn
Studies are available that assess the risk of malaria in accordance to the body's iron store and the systematic iron supplementation of preschool children. However, only a few studies evaluated the temporal association between hemoglobin and malaria and their results are opposing. A total of 1,650 3-month-old Papua New Guinean infants were enrolled in this study and followed-up for 12 months. The risk of malaria was assessed in all children every 3 months and with each episode of fever. The incidence of clinical malaria between 3 and 15 months of age was 249 cases per 1,000 infants per year...
September 25, 2017: American Journal of Tropical Medicine and Hygiene
https://www.readbyqxmd.com/read/29016322/demographic-socioeconomic-and-geographic-factors-leading-to-severe-malaria-and-delayed-care-seeking-in-ugandan-children-a-case-control-study
#20
Arthur Mpimbaza, Grace Ndeezi, Anne Katahoire, Philip J Rosenthal, Charles Karamagi
We studied associations between delayed care seeking, demographic, socioeconomic, and geographic factors and likelihood of severe malaria in Ugandan children. The study was based at Jinja Hospital, Uganda. We enrolled 325 severe malaria cases and 325 uncomplicated malaria controls matched by age and residence. Patient details, an itinerary of events in response to illness, household information, and location of participants' residences were captured. Conditional logistic regression was used to determine risk factors for severe malaria and delayed care seeking...
September 11, 2017: American Journal of Tropical Medicine and Hygiene
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