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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/29146708/inflammatory-molecule-reduction-with-hydroxyurea-therapy-in-children-with-sickle-cell-anemia
#3
Rhiannon R Penkert, Julia L Hurwitz, Paul Thomas, Jason Rosch, Jola Dowdy, Yilun Sun, Li Tang, Jane S Hankins
No abstract text is available yet for this article.
November 16, 2017: Haematologica
https://www.readbyqxmd.com/read/29145822/ultrasonographic-parameters-of-the-liver-spleen-and-kidneys-among-a-cohort-of-school-children-in-sri-lanka
#4
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
#5
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/29141752/differences-in-nutritional-and-health-status-in-school-children-from-the-highlands-and-lowlands-of-bolivia
#6
Graciela Terán, Washington Cuna, Froilán Brañez, Kristina E M Persson, Martín E Rottenberg, Susanne Nylén, Celeste Rodriguez
Children in the Bolivian Andes are exposed to endemic infections and meager nourishment, and live under poor hygienic conditions. The prevention of children malnutrition is a priority in many countries including Bolivia. In this study, the health status of schoolchildren in Taraco, a Puna district, at 4,000 meters above sea level (masl) and in Caranavi, at 650 masl in the wealthier subtropical valleys, was compared. The weight, height, and hematological and biochemical parameters in blood, parasites in stool, and clinical information in 120 children from rural Taraco and in 96 from semi-urban Caranavi, both predominantly of Aymara ethnicity, were registered...
November 6, 2017: American Journal of Tropical Medicine and Hygiene
https://www.readbyqxmd.com/read/29141590/health-nutrition-and-development-of-children-born-preterm-and-low-birth-weight-in-rural-rwanda-a-cross-sectional-study
#7
Catherine M Kirk, Jean Claude Uwamungu, Kim Wilson, Bethany L Hedt-Gauthier, Neo Tapela, Peter Niyigena, Christian Rusangwa, Merab Nyishime, Evrard Nahimana, Fulgence Nkikabahizi, Christine Mutaganzwa, Eric Ngabireyimana, Francis Mutabazi, Hema Magge
BACKGROUND: As care for preterm and low birth weight (LBW) infants improves in resource-limited settings, more infants are surviving the neonatal period. Preterm and (LBW) infants are at high-risk of nutritional and medical comorbidities, yet little is known about their developmental outcomes in low-income countries. This study evaluated the health, nutritional, and developmental status of preterm/LBW children at ages 1-3 years in Rwanda. METHODS: Cross-sectional study of preterm/LBW infants discharged between October 2011 and October 2013 from a hospital neonatal unit in rural Rwanda...
November 15, 2017: BMC Pediatrics
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
#8
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/29137512/microfinance-institutions-successful-delivery-of-micronutrient-powders-a-randomized-trial-in-rural-haiti
#9
Aaron Baum, Wesly Elize, Florence Jean-Louis
Globally, two-thirds of child deaths could be prevented by increased provision of health interventions such as vaccines, micronutrient supplements, and water purification tablets. We report the results from a randomized controlled trial in Haiti during 2012 that tested whether microfinance institutions-which reach 200 million households worldwide-can effectively deliver health products. These institutions provide loans to underserved entrepreneurs, primarily poor women in rural areas. In the intervention group, micronutrient powders to improve the nutrition of young children were distributed at regularly occurring microfinance meetings by a trained borrower...
November 2017: Health Affairs
https://www.readbyqxmd.com/read/29132419/success-of-anti-cd20-monoclonal-antibody-treatment-for-severe-autoimmune-hemolytic-anemia-caused-by-warm-reactive-immunoglobulin-a-immunoglobulin-g-and-immunoglobulin-m-autoantibodies-in-a-child-a-case-report
#10
Houda Ajmi, Sameh Mabrouk, Saida Hassayoun, Haifa Regaieg, Minyar Tfifha, Chemli Jalel, Hadef Skouri, Noura Zouari, Saoussan Abroug
BACKGROUND: Autoimmune hemolytic anemia is rare in children. First-line therapies for this disease consist of corticosteroids and intravenously administered immunoglobulin that are effective in most patients. However, a small proportion of cases (5 to 10%) is refractory to these therapies and may represent a medical emergency, especially when hemolysis is due to warm immunoglobulin M. Recently, reports of the use of rituximab in adult autoimmune diseases have shown promising results. In children, there are few studies on the use of rituximab in the treatment for autoimmune hemolytic anemia, especially on its long-term efficacy and adverse effects...
November 14, 2017: Journal of Medical Case Reports
https://www.readbyqxmd.com/read/29127678/allogeneic-matched-related-transplantation-for-%C3%AE-thalassemia-and-sickle-cell-anemia
#11
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/29127616/worm-infestation-diagnosis-treatment-and-prevention
#12
REVIEW
Bhavneet Bharti, Sahul Bharti, Sumeeta Khurana
Worm infections continue to be among the most common diseases affecting children from low and middle income countries. Major worm infections of public health importance include Ascariasis, Trichuriasis, Hookworm, and Enterobiasis, which are transmitted through contaminated soil. In India, combined prevalence rates of worm infestation as per pooled data of 127 surveys is over 20%. Although most helminthic infections are mild and are often asymptomatic, but moderate to heavy worm infestations are generally associated with growth faltering, nutritional compromise, anemia and suboptimal academic performance among children from endemic regions...
November 11, 2017: Indian Journal of Pediatrics
https://www.readbyqxmd.com/read/29125443/neurosurgical-management-in-children-with-bleeding-diathesis-auditing-neurological-outcome
#13
Zaitun Zakaria, Chandrasekaran Kaliaperumal, Darach Crimmins, John Caird
OBJECTIVE The aim of this study was to assess the outcome of neurosurgical treatment in children with bleeding diathesis and also to evaluate the current management plan applied in the authors' service. METHODS The authors retrospectively analyzed all cases in which neurosurgical procedures were performed in pediatric patients presenting with intracranial hematoma due to an underlying bleeding tendency over a 5-year period at their institution. They evaluated the patients' neurological symptoms from the initial referral, hematological abnormalities, surgical treatment, neurological outcome, and scores on the Pediatric Glasgow Outcome Scale-Extended (GOS-E Peds) obtained 1 year after the last operation...
November 10, 2017: Journal of Neurosurgery. Pediatrics
https://www.readbyqxmd.com/read/29123343/multiple-nutritional-deficiencies-in-cerebral-palsy-compounding-physical-and-functional-impairments
#14
P G Hariprasad, K E Elizabeth, Mathew J Valamparampil, D Kalpana, T S Anish
Introduction: Cerebral palsy (CP) refers to a spectrum of disorders causing physical and intellectual morbidity. Macro and micro nutrient deficiencies often contribute to the subnormal physical and mental capabilities of them. Objectives: To assess the growth, nutritional status, physical and functional ability and quality of life in cerebral palsy children and to determine any relation with their gross motor and functional capabilities. Method: The study was conducted at a Tertiary Care Centre, with the participants in the age group 1-16 years...
October 2017: Indian Journal of Palliative Care
https://www.readbyqxmd.com/read/29121448/malaria-exacerbates-inflammation-associated-elevation-in-ferritin-and-soluble-transferrin-receptor-with-only-modest-effects-on-iron-deficiency-and-iron-deficiency-anemia-among-rural-zambian-children
#15
Maxwell A Barffour, Kerry J Schulze, Christian L Coles, Justin Chileshe, Ng'andwe Kalungwana, Ward Siamusantu, Margia Arguello, William J Moss, Keith P West, Amanda C Palmer
OBJECTIVE: In 4-8-year-old Zambian children (n=744), we evaluated the effects of adjusting for inflammation (α1-acid glycoprotein > 1 g/L), with or without additional adjustment for malaria, on prevalence estimates of iron deficiency (ID) and iron deficiency anemia (IDA) during low malaria (LowM) and high malaria (HighM) transmission seasons. METHODS: To estimate adjustment factors, children were classified as: a) reference (malaria negative without inflammation), b) inflammation without malaria (I), c) malaria without inflammation (M), and d) inflammation with malaria (IM)...
November 9, 2017: Tropical Medicine & International Health: TM & IH
https://www.readbyqxmd.com/read/29119033/the-utility-of-mri-in-the-diagnosis-of-takayasu-arteritis
#16
Marian Gaballah, Rachelle Goldfisher, John B Amodio
Takayasu Arteritis (TA) is an inflammatory disorder involving the thoracoabdominal aorta and its branches and the pulmonary arteries, with eventual vascular stenosis, occlusion, or aneurysm formation. Conventional angiography has been the reference imaging standard for diagnosis of TA. The purpose of this case report is to demonstrate the utility of MR imaging and MR angiography in the diagnosis of Takayasu Arteritis in a pediatric patient. The patient is a 15-year-old female patient presenting with anemia, hypertension, and acute kidney injury...
2017: Case Reports in Pediatrics
https://www.readbyqxmd.com/read/29114484/epidemiology-and-clinical-features-of-soil-transmitted-helminths
#17
Subhash Chandra Parija, Meenachi Chidambaram, Jharna Mandal
Soil-transmitted helminths (STH) consist of Ascaris lumbricoides, Trichuris trichiura, and hookworm (Necator americanus and Ancylostoma duodenale). It affects nearly 1.7 billion people globally in which Ascaris contributes nearly 1.2 billion cases. The main mode for transmission of Ascaris and Trichuris is through contaminated food and water, whereas hookworm transmitted by skin penetration. STH were mainly seen in areas with poverty, overcrowding, and poor sanitation. The prevalence is more in rural areas compared to urban areas...
July 2017: Tropical Parasitology
https://www.readbyqxmd.com/read/29104118/clinical-trial-conduct-in-special-populations-and-developing-regions-an-overview-of-the-dove-study-in-pediatric-patients-with-sickle-cell-disease
#18
Stephen G Reams, Linda Messana, Patricia B Brown, Kevin Nanry, Alexandria E Gunnell
Clinical trials conducted in unique patient populations or individuals with rare diseases are typically hampered by limitations in availability of qualified patients, requiring sponsors to broaden their global outreach to achieve enrollment. Engaging clinical study centers in developing regions may offer access to a substantially larger patient pool. However, they provide a unique set of challenges based on local cultures and requirements. The DOVE study (Determining effects Of platelet inhibition on Vaso-occlusive Events) was a clinical trial of prasugrel hydrochloride (prasugrel) in pediatric patients (aged 2 to <18years) with sickle cell anemia...
November 2, 2017: Contemporary Clinical Trials
https://www.readbyqxmd.com/read/29099741/infantile-cytomegalovirus-associated-severe-warm-autoimmune-hemolytic-anemia-a-case-report
#19
Hassan K Khalifeh, Youmna M Mourad, Cynthia T Chamoun
Autoimmune hemolytic anemia is a rare hematologic entity in children. Etiologies are mainly viruses or bacteria. We describe here a case of severe warm autoimmune hemolytic anemia (IgG- and C3d-positive direct antiglobulin test) in an immunocompetent 6-month-old infant with acute Cytomegalovirus infection that responded well to corticotherapy and intraveneous immunoglobulins without using blood component transfusion. This case demonstrates the importance of recognizing CMV in infantile Autoimmune Hemolytic Anemia, especially because hemolysis can be severe and lethal...
November 3, 2017: Children
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
#20
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
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