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https://www.readbyqxmd.com/read/29773497/indications-for-red-cell-transfusions-in-pediatric-patients
#1
REVIEW
Jill M Cholette, Suzie A Noronha, Jerard Seghatchian, Neil Blumberg
Red cell transfusions are amongst the most common therapeutic procedures in seriously ill children, particularly in the inpatient setting. This is despite the fact that there is no evidence base for most clinical settings, with the exception of patients with hemoglobinopathies, particularly thalassemia and sickle cell anemia. Obviously exsanguinating hemorrhage and life threatening anemia are urgent indications for which no other therapeutic approach is currently available. Most transfusions are, however, given prophylactically to prevent the complications of hypoxia or hemodynamic stability, based upon expert opinion and a faith in the oxygen carrying capacity and beneficial hemodynamic properties of transfused red cells...
May 10, 2018: Transfusion and Apheresis Science
https://www.readbyqxmd.com/read/29772478/bacillus-thuringiensis-cry5b-protein-as-a-new-pan-hookworm-cure
#2
Yan Hu, Thanh-Thanh Nguyen, Alice C Y Lee, Joseph F Urban, Melanie M Miller, Bin Zhan, David J Koch, Jason B Noon, Ambily Abraham, Ricardo Toshio Fujiwara, Dwight D Bowman, Gary R Ostroff, Raffi V Aroian
Hookworms are intestinal nematode parasites that infect nearly half a billion people and are globally one of the most important contributors to iron-deficiency anemia. These parasites have significant impacts in developing children, pregnant women and working adults. Of all the soil-transmitted helminths or nematodes (STNs), hookworms are by far the most important, with disease burdens conservatively estimated at four million DALYs (Disability-Adjusted Life Years) and with productivity losses of up to US$139 billion annually...
May 4, 2018: International Journal for Parasitology, Drugs and Drug Resistance
https://www.readbyqxmd.com/read/29769814/combined-deficiencies-of-25-hydroxyvitamin-d-and-anemia-in-preschool-children-with-severe-early-childhood-caries-a-case-control-study
#3
Shannon Deane, Robert J Schroth, Atul Sharma, Celia Rodd
Background: Severe early childhood caries (S-ECC) is common and has adverse affects on children's health. Children with S-ECC have been shown to have anemia or vitamin D deficiency. No studies have assessed the presence of combined deficiencies with S-ECC. The purpose of our study was to examine whether those with S-ECC had a higher prevalence of combined anemia and low 25-hydroxyvitamin D (25(OH)D) compared to controls. Covariates associated with elevated parathyroid hormone (PTH), previously noted in S-ECC, were examined...
May 2018: Paediatrics & Child Health
https://www.readbyqxmd.com/read/29767699/impact-of-double-fortified-salt-with-iron-and-iodine-on-hemoglobin-anemia-and-iron-deficiency-anemia-a-systematic-review-and-meta-analysis
#4
María J Ramírez-Luzuriaga, Leila M Larson, Venkatesh Mannar, Reynaldo Martorell
Double-fortified salt (DFS) containing iron and iodine has been proposed as a feasible and cost-effective alternative for iron fortification in low- and middle-income countries (LMICs). We conducted a systematic review and meta-analysis from randomized and quasi-randomized controlled trials to 1) assess the effect of DFS on biomarkers of iron status and the risk of anemia and iron deficiency anemia (IDA) and 2) evaluate differential effects of DFS by study type (efficacy or effectiveness), population subgroups, iron formulation (ferrous sulfate, ferrous fumarate, and ferric pyrophosphate), iron concentration, duration of intervention, and study quality...
May 1, 2018: Advances in Nutrition
https://www.readbyqxmd.com/read/29765934/autoimmune-gastritis-in-the-pediatric-age-an-underestimated-condition-report-of-two-cases-and-review
#5
Chiara Saglietti, Amedeo Sciarra, Karim Abdelrahman, Vanessa Schneider, Arti Karpate, Andreas Nydegger, Christine Sempoux
Background: Diagnosis of pediatric autoimmune gastritis (AIG) in children is important due to poor outcome and risk of malignancy. This condition is often underestimated in the clinico-pathologic diagnostic work-up, leading to delayed time-to-diagnosis. To increase the awareness of this condition in the pediatric population, we present two cases encountered at our institution, discuss their clinical, biological, and histological presentations in relation with evidence from the literature, and propose an algorithm for diagnosis and follow-up of AIG in children...
2018: Frontiers in Pediatrics
https://www.readbyqxmd.com/read/29755496/banana21-from-gene-discovery-to-deregulated-golden-bananas
#6
REVIEW
Jean-Yves Paul, Robert Harding, Wilberforce Tushemereirwe, James Dale
Uganda is a tropical country with a population in excess of 30 million, >80% of whom live in rural areas. Bananas ( Musa spp.) are the staple food of Uganda with the East African Highland banana, a cooking banana, the primary starch source. Unfortunately, these bananas are low in pro-vitamin A (PVA) and iron and, as a result, banana-based diets are low in these micronutrients which results in very high levels of inadequate nutrition. This inadequate nutrition manifests as high levels of vitamin A deficiency, iron deficiency anemia, and stunting in children...
2018: Frontiers in Plant Science
https://www.readbyqxmd.com/read/29747664/adverse-effects-in-children-exposed-to-maternal-hiv-and-antiretroviral-therapy-during-pregnancy-in-brazil-a-cohort-study
#7
Adriane M Delicio, Giuliane J Lajos, Eliana Amaral, Fernanda Cavichiolli, Marina Polydoro, Helaine Milanez
BACKGROUND: Antiretroviral therapy (ART) in pregnancy was associated with a drastic reduction in HIV mother-to-child transmission (MTCT), although it was associated with neonatal adverse effects. The aim of this study was to evaluate the neonatal effects to maternal ART. METHODS: This study was a cohort of newborns from HIV pregnant women followed at the CAISM/UNICAMP Obstetric Clinic from 2000 to 2015. The following adverse effects were evaluated: anemia, thrombocytopenia, liver function tests abnormalities, preterm birth, low birth weight and congenital malformation...
May 10, 2018: Reproductive Health
https://www.readbyqxmd.com/read/29747646/effect-of-dietary-intervention-treatment-on-children-with-iron-deficiency-anemia-in-china-a-meta-analysis
#8
Jian Sun, Lei Zhang, Jing Cui, Shanshan Li, Hongting Lu, Yong Zhang, Haiming Li, Jianping Sun, Zulqarnain Baloch
BACKGROUND: Previous studies have shown beneficial effects of dietary approaches for iron deficiency anemia (IDA) control. This study was design to investigate the effect of dietary intervention treatment on children with iron deficiency anemia. METHODS: We performed a systematic review of published dietary interventions effect on IDA treatment through meta-analysis. CBM, CNKI, Wanfang database, EMBASE, VIP, PubMed and Web of science database were searched to identify studies published between January, 1980 and December, 2016...
May 10, 2018: Lipids in Health and Disease
https://www.readbyqxmd.com/read/29746746/prevalence-of-anemia-and-consumption-of-iron-rich-food-groups-in-mexican-children-and-adolescents-ensanut-mc-2016
#9
Vanessa De la Cruz-Góngora, Salvador Villalpando, Teresa Shamah-Levy
OBJECTIVE: To describe the prevalence of anemia and con-sumption of iron rich groups among Mexican children and adolescents who participated in the Halfway National Health and Nutrition Survey, 2016. MATERIALS AND METHODS: Our study sample included children and adolescents who provided full capillary hemoglobin data. Anemia was defined accord-ing to WHO criteria. Logistic regression models were used to explore the association among consumption of iron-rich food groups, sociodemographic characteristics and anemia...
May 2018: Salud Pública de México
https://www.readbyqxmd.com/read/29744996/hematopoietic-stem-cell-transplantation-without-in-vivo-t-cell-depletion-for-pediatric-aplastic-anemia-a-single-center-experience
#10
Sidan Li, Bin Wang, Lingling Fu, Yilin Pang, Guanghua Zhu, Xuan Zhou, Jie Ma, Yan Su, Maoquan Qin, Runhui Wu
For young patients, HLA-MRD HSCT is the first-line treatment of SAA. However, due to China's birth control policy, few patients could find suitable sibling donors and HLA-MUD. More and more transplantation centers have used Haplo-D as the donor source for young adult and pediatric patients. However, studies with larger amount of pediatric patients are rare. We retrospectively analyzed the data of children with AA who were treated with allogeneic HSCT and compared the therapeutic efficacy of Haplo-HSCT and MRD/MUD group...
May 10, 2018: Pediatric Transplantation
https://www.readbyqxmd.com/read/29735420/-post-streptococcal-glomerulonephritis-in-the-south-of-tunisia-a-12-year-retrospective-review
#11
Bayen Maalej, Mona Ben Amor, Manel Jallouli, Manel Weli, Mohamed Charfi, Yassine Abdelkefi, Olfa Chakroun, Khaoula Kamoun, Jamil Hachicha, Noureddine Rekik, Taher Gargah, Lamia Gargouri, Abdelmajid Mahfoudh
AIM: Post-streptococcal glomerulonephritis (PSGN) is a frequent cause of acute nephritis in children. This study aimed to describe the epidemiology, clinical characteristics and outcomes of PSGN and look for predictor's factors of severity. METHODS: A 12-year retrospective review of case notes and laboratory data was conducted at a department of pediatrics, pediatric emergency and intensive care, Hedi Chaker Hospital. RESULTS: One hundred seventy eight children were treated for PSGN with a mean age of 7...
May 4, 2018: Néphrologie & Thérapeutique
https://www.readbyqxmd.com/read/29729108/high-prevalence-of-anemia-in-10-month-old-japanese-infants-with-breastfeeding
#12
Masahiko Kimura, Youichi Kurozawa, Yumi Saito, Hiroshi Watanabe, Ayame Kobayashi, Takeshi Taketani
BACKGROUND: Anemia in infancy is still prevalent in developing countries. Commercial iron-fortified complementary foods or iron drops are not available in Japan and breastfed infants have a higher risk of anemia. We studied anemia screening in infants in 10-month old infants and evaluated whether breastfeeding is a risk factor for anemia. METHODS: Anemia screening was performed during a regular health check of 10-month children at four local pediatric clinics in Shimane prefecture, Japan...
May 5, 2018: Pediatrics International: Official Journal of the Japan Pediatric Society
https://www.readbyqxmd.com/read/29724291/comparison-of-two-dosages-of-rabbit-antithymocyte-globulin-r-atg-in-treating-children-with-severe-aplastic-anemia
#13
Shao-Fen Lin, Su Liu, Hong-Man Xue, Jun-Bin Huang, Jian Wang, Qi-Hui Chen, Bi-Hong Zhang, Chun Chen
In this study, efficacy and safety of two different dosages of rabbit antithymocyte globulin (r-ATG) combined with cyclosporine (CsA) in treating children with severe aplastic anemia (SAA) were compared. The clinical data of 122 SAA children treated by r-ATG/CsA between Jan 2005 and Jan 2017 at Sun Yat-sen Memorial Hospital of Sun Yat-sen University were retrospectively analyzed. The r-ATG dose of 55 cases was 2.5mg/(kg·d, group 1), and in the other 67 cases it was 3.5 mg/(kg·d, group 2). r-ATG was continuously administered for 5 days...
May 1, 2018: Die Pharmazie
https://www.readbyqxmd.com/read/29724144/arrested-pneumatization-of-the-sphenoid-sinus-mimicking-skull-base-tumors-mri-prevalence-in-children-with-haematologic-diseases
#14
Taner Arpaci
OBJECTIVE: Arrested pneumatization of the sphenoid sinus (APSS) is a developmental anatomic variation but may be confused with serious diseases of the skull base. The purpose of this study was to investigate the prevalence of APSS in pediatric patients with haemotologic diseases like sickle cell anemia (SCA), thalassemia and leukemia. MATERIALS AND METHODS: One hundred and eight pediatric patients (43 girl, 65 boy; age range, 4-18 years; median age, 13 years) with haemotologic diseases who underwent at least one magnetic resonance imaging (MRI) of the head and neck between 2010-2017 in a single instution were included...
May 3, 2018: International Journal of Neuroscience
https://www.readbyqxmd.com/read/29722478/successful-reduced-intensity-conditioning-hematopoietic-stem-cell-transplantation-for-paroxysmal-nocturnal-hemoglobinuria-with-aplastic-anemia-in-two-children
#15
Jeffrey R Andolina, Ariel L Reinish, Razia Akhtar, Suzie Noronha, Jessica C Shand, Angela Girvin, David N Korones, Lauren B Bruckner, Craig A Mullen, Kevin J Curran, Farid Boulad
Paroxysmal nocturnal hemoglobinuria (PNH) is an extremely rare cause of bone marrow failure in children. We report two children who presented with pancytopenia, and were diagnosed with PNH with severe aplastic anemia. Both children underwent upfront, successful hematopoietic stem cell transplantation with reduced-intensity conditioning. One patient had a syngeneic donor, and one patient had a 10/10 matched unrelated donor. Neither patient developed graft versus host disease, infections, or recurrent PNH. Reduced-intensity conditioning hematopoietic stem cell transplantation is a reasonable therapy for PNH with marrow failure in children...
May 3, 2018: Pediatric Blood & Cancer
https://www.readbyqxmd.com/read/29712865/mechanism-for-survival-of-homozygous-nonsense-mutations-in-the-tumor-suppressor-gene-brca1
#16
Aaron Seo, Orna Steinberg-Shemer, Sule Unal, Silvia Casadei, Tom Walsh, Fatma Gumruk, Stavit Shalev, Akiko Shimamura, Nurten Ayse Akarsu, Hannah Tamary, Mary-Claire King
BRCA1 is essential for repair of DNA double-strand breaks by homologous recombination, and hence for survival. Complete loss of its function is lethal during early embryonic development. Patients who are compound heterozygous for BRCA1 truncating mutations and missense alleles that retain some DNA repair capacity may survive, albeit with very high risk of early onset breast or ovarian cancer and features of Fanconi anemia. However, a mechanism enabling survival of patients homozygous for BRCA1 truncating mutations has not been described...
April 30, 2018: Proceedings of the National Academy of Sciences of the United States of America
https://www.readbyqxmd.com/read/29702541/novel-variations-of-fanca-gene-provokes-fanconi-anemia-molecular-diagnosis-in-a-special-chinese-family
#17
Niu Li, Aiyun Song, Lixia Ding, Hua Zhu, Guoqiang Li, Yan Miao, Jian Wang, Benshang Li, Jing Chen
Fanconi anemia (FA) is a rare autosomal recessive or X-linked disorder with highly variable clinical manifestations and an incidence of ∼1 to 5 in 1 million births. To date, 15 bona fide FA genes have been reported to be responsible for the known FA complementation groups and the FANCA gene accounts for almost 60%. In the present study, we report a special Chinese family, which has 2 children with classic FA characteristics. Via 2-step analysis of the whole-exome sequencing data and verification using multiplex ligation-dependent probe amplification test, one child was found to have a novel compound heterozygous mutation of a splicing variant (c...
April 26, 2018: Journal of Pediatric Hematology/oncology
https://www.readbyqxmd.com/read/29700810/refining-the-phenotype-associated-with-biallelic-dnajc21-mutations
#18
G D'Amours, F Lopes, J Gauthier, V Saillour, C Nassif, R Wynn, N Alos, T Leblanc, Y Capri, S Nizard, E Lemyre, J L Michaud, V-A Pelletier, Y D Pastore, J-F Soucy
Inherited bone marrow failure syndromes (IBMFS) are caused by mutations in genes involved in genomic stability. Although they may be recognized by the association of typical clinical features, variable penetrance and expressivity are common, and clinical diagnosis is often challenging. DNAJC21, which is involved in ribosome biogenesis, was recently linked to bone marrow failure. However, the specific phenotype and natural history remain to be defined. We correlate molecular data, phenotype, and clinical history of five unreported affected children and all individuals reported in the literature...
April 26, 2018: Clinical Genetics
https://www.readbyqxmd.com/read/29695942/genotype-phenotype-correlation-among-beta-thalassemia-and-beta-thalassemia-hbe-disease-in-thai-children-predictable-clinical-spectrum-using-genotypic-analysis
#19
Chanchai Traivaree, Chalinee Monsereenusorn, Piya Rujkijyanont, Warakorn Prasertsin, Boonchai Boonyawat
Introduction: Beta-thalassemia is a group of inherited hemolytic anemias and one of the most common genetic disorders in Thailand. The clinical spectrum of beta-thalassemia disease ranges from mild to severe clinical symptoms including mild beta-thalassemia intermedia (TI) and severe beta-thalassemia major (TM). Objective: This study aimed to determine the correlation between beta-globin gene ( HBB ) mutations and their phenotypic manifestations by evaluating patients' clinical characteristics, transfusion requirements, growth and hematologic parameters, and hemoglobin typing among pediatric patients treated at Phramongkutklao Hospital...
2018: Journal of Blood Medicine
https://www.readbyqxmd.com/read/29695584/copper-deficiency-anemia-and-neutropenia-due-to-ketogenic-diet
#20
Alan Chin
Copper deficiency is an uncommon cause of hematologic abnormalities in children that is often overlooked or misdiagnosed. Although cases have been reported because of malabsorption syndromes or after gastrointestinal surgeries, we report a case of copper deficiency-associated anemia and neutropenia in a child because of dietary restrictions, specifically, transitioning from a formula-based ketogenic diet to a pureed food-based ketogenic diet. On copper supplementation, the patient's anemia and neutropenia resolved...
April 25, 2018: Pediatrics
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