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https://www.readbyqxmd.com/read/28641096/hematopoietic-stem-cell-transplantation-for-sickle-cell-anemia-the-changing-landscape
#1
REVIEW
Adetola A Kassim, Deva Sharma
Allogeneic hematopoietic stem cell transplantation (HSCT) remains the only curative therapy for sickle cell disease (SCD); however, its use is limited by lack of suitable human leukocyte antigen (HLA)-matched donors and decreased application in older patients with significant morbidity. Myeloablative, HLA-identical sibling transplantation in children with SCD offers excellent long-term survival, with overall and event-free survival rates of 95% and 92%, respectively. However, the risk of graft-versus-host-disease, infections, infertility, and other long-term transplant complications, further limits its widespread use...
June 15, 2017: Hematology/oncology and Stem Cell Therapy
https://www.readbyqxmd.com/read/28626543/cerebral-stroke-in-a-teenage-girl-with-paroxysmal-nocturnal-hemoglobinuria
#2
Francesco Gervasi, Lucia D'Amelio, Antonino Trizzino, Fabrizia Ferraro, Delia Russo, Giuseppe Santangelo, Francesca Cardella, Angela Trizzino, Floriana Di Marco, Piero Farruggia
We report a case of paroxysmal nocturnal hemoglobinuria (PNH) in a 14 year-old girl presenting a cerebral arterial thrombosis. The initial diagnosis was carential anemia due to menarche following identification of slight macrocytic anemia, leucopenia and mild thrombocytopenia at routine blood analysis. The child was eventually referred to a children's hospital after the onset of progressive fatigue, anorexia and paleness. Severe anemia (hemoglobin 6 g/dL) with negative Coombs test, mild leucopenia (white blood cells 4...
June 1, 2017: Hematology Reports
https://www.readbyqxmd.com/read/28626540/acute-crises-and-complications-of-sickle-cell-anemia-among-patients-attending-a-pediatric-tertiary-unit-in-kinshasa-democratic-republic-of-congo
#3
Michel Ntetani Aloni, Bertin Tshimanga Kadima, Pépé Mfutu Ekulu, Aléine Nzazi Budiongo, René Makuala Ngiyulu, Jean Lambert Gini-Ehungu
In the Democratic Republic of Congo, the incidence of sickle cell anemia (SCA) is estimated to affect 30,000 to 40,000 neonates per year. However, there is paucity of data on acute clinical manifestations in sickle cell children. In these circumstances, it is difficult to develop a health care policy for an adequate management of sickle cell patients. This was a seven years' retrospective study of children admitted with acute sickle cell crisis in the Department of Pediatrics in University Hospital of Kinshasa, Kinshasa, the Democratic Republic of Congo...
June 1, 2017: Hematology Reports
https://www.readbyqxmd.com/read/28626279/nutritional-status-of-foster-children-in-the-u-s-implications-for-cognitive-and-behavioral-development
#4
Ursula A Tooley, Zeina Makhoul, Philip A Fisher
OBJECTIVE: Children in foster care are at greater risk for poor health, physical, cognitive, behavioral, and developmental outcomes than are children in the general population. Considerable research links early nutrition to later cognitive and behavioral outcomes. The aim of this narrative review is to examine the prevalence of poor nutrition and its relation to subsequent health and development in foster children. METHOD: Relevant studies for inclusion were identified from numerous sources (e...
November 2016: Children and Youth Services Review
https://www.readbyqxmd.com/read/28624811/altered-erythropoiesis-and-decreased-number-of-erythrocytes-in-children-with-neuroblastoma
#5
Fabio Morandi, Sebastiano Barco, Sara Stigliani, Michela Croce, Luca Persico, Corrado Lagazio, Francesca Scuderi, Maria Luisa Belli, Mariapina Montera, Giuliana Cangemi, Sarah Pozzi, Valentina Rigo, Paola Scaruffi, Loredana Amoroso, Giovanni Erminio, Vito Pistoia, Silvano Ferrini, Maria Valeria Corrias
Neuroblastoma (NB) is a pediatric tumor presenting at diagnosis either as localized or metastatic disease, which mainly involves the bone marrow (BM). The physical occupancy of BM space by metastatic NB cells has been held responsible for impairment of BM function. Here, we investigated whether localized or metastatic NB may alter hematopoietic lineages' maturation and release of mature cells in the periphery, through gene expression profiling, analysis of BM smears, cell blood count and flow cytometry analysis...
May 30, 2017: Oncotarget
https://www.readbyqxmd.com/read/28624578/temporary-removal-accuracy-of-tests-for-antibodies-against-tissue-transglutaminase-in-diagnosis-of-celiac-disease-without-biopsy
#6
K J Werkstetter, I R Korponay-Szabó, A Popp, V Villanacci, M Salemme, G Heilig, S T Lillevang, M L Mearin, C Ribes-Koninckx, A Thomas, R Troncone, B Filipiak, M Mäki, J Gyimesi, M Najafi, J Dolinšek, S Dydensborg Sander, R Auricchio, A Papadopoulou, A Vécsei, P Szitanyi, E Donat, R Nenna, Ph Alliet, F Penagini, H Garnier-Lengliné, G Castillejo, K Kurppa, R Shamir, A C Hauer, F Smets, S Corujeira, M van Winckel, S Buderus, S Chong, S Husby, S Koletzko
The publisher regrets that this article has been temporarily removed. A replacement will appear as soon as possible in which the reason for the removal of the article will be specified, or the article will be reinstated. The full Elsevier Policy on Article Withdrawal can be found at https://www.elsevier.com/about/our-business/policies/article-withdrawal.
June 15, 2017: Gastroenterology
https://www.readbyqxmd.com/read/28623553/-kaposi-s-disease-in-an-hiv-positive-child-with-probable-contamination-from-his-grandmother
#7
A Dicko, Y Fofana, A Traoré, S Berthé, S Touré, C Lamissa, B Guindo, A Keita, O Faye
Kaposi's disease in children with HIV is rarely reported in everyday practice. This is a case study of cutaneous Kaposi's disease revealing HIV in a 5-year-old child with polymorphic eruption of papules and nodules on the face, trunk, back, and limbs. Histopathological examination confirmed the diagnosis of Kaposi's disease. The child's HIV serology was positive with a CD4 count of 240/mm3, normochromic and normocytic anemia, and a hemoglobin level at 8.5 g/dl. It was found that the child, after early weaning from his HIV-negative mother, had repeatedly suckled his healthy grandmother, who had no skin lesions but was HIV1 positive...
June 16, 2017: Bulletin de la Société de Pathologie Exotique
https://www.readbyqxmd.com/read/28623394/disease-specific-hematopoietic-stem-cell-transplantation-in-children-with-inherited-bone-marrow-failure-syndromes
#8
Qian Li, Changying Luo, Chengjuan Luo, Jianmin Wang, Benshang Li, Lixia Ding, Jing Chen
Hematopoietic stem cell transplantation (HSCT) using an optimized conditioning regimen is essential for the long-term survival of patients with inherited bone marrow failure syndromes (IBMFS). We report HSCT in 24 children with Fanconi anemia (FA, n = 12), Diamond-Blackfan anemia (DBA, n = 7), and dyskeratosis congenita (DC, n = 5) from a single HSCT center. The graft source was peripheral blood stem cells (n = 19) or cord blood stem cells (n = 5). FA and DC patients received reduced-intensity conditioning, while DBA patients had myeloablative conditioning...
June 16, 2017: Annals of Hematology
https://www.readbyqxmd.com/read/28622783/-biological-diagnosis-of-iron-deficiency-in-children
#9
I Thuret
Measurement of serum ferritin (SF) is currently the laboratory test recommended for diagnosing iron deficiency. In the absence of an associated disease, a low SF value is an early and highly specific indicator of iron deficiency. The WHO criteria proposed to define depleted storage iron are 12μg/L for children under 5 years and 15μg/L for those over 5 years. A higher threshold of 30μg/L is used in the presence of infection or inflammation. Iron deficiency anemia, with typical low mean corpuscular volume and mean corpuscular hemoglobin, is only present at the end stage of iron deficiency...
May 2017: Archives de Pédiatrie: Organe Officiel de la Sociéte Française de Pédiatrie
https://www.readbyqxmd.com/read/28622782/-prevalence-of-iron-deficiency
#10
C Dupont
Studies of prévalence in iron deficiency separate iron depletion (defined as decreased blood ferritin) and iron deficiency anemia (defined as blood decrease in both ferritin and hemoglobin). In Europe, most studies are outdated. Prevalence of iron depletion varies from 7 to 18 % and 24 to 36% in toddlers and adolescents, respectively. Prevalence of iron deficiency anemia varies from 2 to 8.5% and 7 to 10% in toddlers and adolescents. In French speaking African countries, Demography Health Surveys show that 80% of children aged 0 to 2 years are anemic, severely for 5 to 9% of them...
May 2017: Archives de Pédiatrie: Organe Officiel de la Sociéte Française de Pédiatrie
https://www.readbyqxmd.com/read/28622778/-causes-of-iron-deficiency-in-children
#11
J-P Olives
Iron deficiency and iron deficiency anemia are common conditions worldwide affecting especially children. In developing countries, iron deficiency is caused by poor iron intake and parasitic infection. Poor iron intake linked to inadequate diets, low iron intestinal absorption, chronic blood losses and increased requirements are common causes in high-income countries.
May 2017: Archives de Pédiatrie: Organe Officiel de la Sociéte Française de Pédiatrie
https://www.readbyqxmd.com/read/28622742/intravenous-ferumoxytol-in-pediatric-patients-with-iron-deficiency-anemia
#12
Nabil Hassan, Brian Boville, Diann Reischmann, Akunne Ndika, David Sterken, Karen Kovey
BACKGROUND: Iron deficiency anemia (IDA) is common in children. Limited data exist on the efficacy and safety of ferumoxytol in children. OBJECTIVE: To assess the efficacy of 10 mg/kg dose given over 15-60 minutes in correcting IDA and report any adverse drug reactions (ADRs). METHODS: We conducted a retrospective review of all patients who received ferumoxytol infusions for the management of IDA by the Pediatric Blood Management Program between October 2010 and March 2015...
July 2017: Annals of Pharmacotherapy
https://www.readbyqxmd.com/read/28621877/autoimmune-hemolytic-anemia-in-solid-organ-transplantation-the-role-of-immunosuppression
#13
REVIEW
Theoni Kanellopoulou
Hemolysis after solid organ transplantation can be caused by both immune and non-immune mediated mechanisms and the evaluation must take into account issues distinctive to the post-transplant period. Autoimmune hemolytic anemia usually occurs within the first year and has been attributed to immunosuppressive treatment, infections or underlying post-transplant lymphoproliferative disorder. Review of the literature revealed 59 cases with autoimmune hemolytic anemia mostly in children after liver transplantation...
June 16, 2017: Clinical Transplantation
https://www.readbyqxmd.com/read/28616421/cystic-fibrosis-prevalence-among-a-group-of-high-risk-children-in-the-main-referral-children-hospital-in-iran
#14
Mohammad Reza Modaresi, Jamal Faghinia, Mohsen Reisi, Majid Keivanfar, Shiva Navaie, Javad Seyyedi, Faride Baharzade
BACKGROUND: Knowledge about cystic fibrosis (CF) in Iran is very limited. The objective of this study was to determine the prevalence of CF among a group of high-risk children with suggestive clinical features in the main referral hospital in Iran. MATERIALS AND METHODS: This study children consisted of 505 patients who had presented with one or more of the following symptoms: chronic or recurrent respiratory symptoms, gastrointestinal symptoms as rectal prolapse, steatorrhea, hepatobiliary disease as prolonged jaundice, failure to thrive, hyperglycemia and glycosuria, hypochloremic metabolic alkalosis, hypoprothrombinemia, anemia or edema, and positive family history of CF...
2017: Journal of Education and Health Promotion
https://www.readbyqxmd.com/read/28615263/factors-associated-with-inflammation-in-preschool-children-and-women-of-reproductive-age-biomarkers-reflecting-inflammation-and-nutritional-determinants-of-anemia-brinda-project
#15
REVIEW
Rebecca D Merrill, Rachel M Burke, Christine A Northrop-Clewes, Pura Rayco-Solon, Rafael Flores-Ayala, Sorrel Ml Namaste, Mary K Serdula, Parminder S Suchdev
Background: In many settings, populations experience recurrent exposure to inflammatory agents that catalyze fluctuations in the concentrations of acute-phase proteins and certain micronutrient biomarkers such as C-reactive protein (CRP), α-1-acid glycoprotein (AGP), ferritin, and retinol. Few data are available on the prevalence and predictors of inflammation in diverse settings.Objective: We aimed to assess the relation between inflammation (CRP concentration >5 mg/L or AGP concentration >1 g/L) and covariates, such as demographics, reported illness, and anthropometric status, in preschool children (PSC) (age range: 6-59 mo) and women of reproductive age (WRA) (age range: 15-49 y)...
June 14, 2017: American Journal of Clinical Nutrition
https://www.readbyqxmd.com/read/28615260/predictors-of-anemia-in-preschool-children-biomarkers-reflecting-inflammation-and-nutritional-determinants-of-anemia-brinda-project
#16
REVIEW
Reina Engle-Stone, Grant J Aaron, Jin Huang, James P Wirth, Sorrel Ml Namaste, Anne M Williams, Janet M Peerson, Fabian Rohner, Ravi Varadhan, O Yaw Addo, Victor Temple, Pura Rayco-Solon, Barbara Macdonald, Parminder S Suchdev
Background: A lack of information on the etiology of anemia has hampered the design and monitoring of anemia-control efforts.Objective: We aimed to evaluate predictors of anemia in preschool children (PSC) (age range: 6-59 mo) by country and infection-burden category.Design: Cross-sectional data from 16 surveys (n = 29,293) from the Biomarkers Reflecting Inflammation and Nutritional Determinants of Anemia (BRINDA) project were analyzed separately and pooled by category of infection burden. We assessed relations between anemia (hemoglobin concentration <110 g/L) and severe anemia (hemoglobin concentration <70 g/L) and individual-level (age, anthropometric measures, micronutrient deficiencies, malaria, and inflammation) and household-level predictors; we also examined the proportion of anemia with concomitant iron deficiency (defined as an inflammation-adjusted ferritin concentration <12 μg/L)...
June 14, 2017: American Journal of Clinical Nutrition
https://www.readbyqxmd.com/read/28615259/adjusting-ferritin-concentrations-for-inflammation-biomarkers-reflecting-inflammation-and-nutritional-determinants-of-anemia-brinda-project
#17
REVIEW
Sorrel Ml Namaste, Fabian Rohner, Jin Huang, Nivedita L Bhushan, Rafael Flores-Ayala, Roland Kupka, Zuguo Mei, Rahul Rawat, Anne M Williams, Daniel J Raiten, Christine A Northrop-Clewes, Parminder S Suchdev
Background: The accurate estimation of iron deficiency is important in planning and implementing interventions. Ferritin is recommended as the primary measure of iron status, but interpretability is challenging in settings with infection and inflammation.Objective: We assessed the relation between ferritin concentrations and inflammation and malaria in preschool children (PSC) (age range: 6-59 mo) and women of reproductive age (WRA) (age range: 15-49 y) and investigated adjustment algorithms to account for these effects...
June 14, 2017: American Journal of Clinical Nutrition
https://www.readbyqxmd.com/read/28615256/adjusting-soluble-transferrin-receptor-concentrations-for-inflammation-biomarkers-reflecting-inflammation-and-nutritional-determinants-of-anemia-brinda-project
#18
REVIEW
Fabian Rohner, Sorrel Ml Namaste, Leila M Larson, O Yaw Addo, Zuguo Mei, Parminder S Suchdev, Anne M Williams, Fayrouz A Sakr Ashour, Rahul Rawat, Daniel J Raiten, Christine A Northrop-Clewes
Background: Iron deficiency is thought to be one of the most prevalent micronutrient deficiencies globally, but an accurate assessment in populations who are frequently exposed to infections is impeded by the inflammatory response, which causes iron-biomarker alterations.Objectives: We assessed the relation between soluble transferrin receptor (sTfR) concentrations and inflammation and malaria in preschool children (PSC) (age range: 6-59 mo) and women of reproductive age (WRA) (age range: 15-49 y) and investigated adjustment algorithms to account for these effects...
June 14, 2017: American Journal of Clinical Nutrition
https://www.readbyqxmd.com/read/28615255/adjusting-total-body-iron-for-inflammation-biomarkers-reflecting-inflammation-and-nutritional-determinants-of-anemia-brinda-project
#19
REVIEW
Zuguo Mei, Sorrel Ml Namaste, Mary Serdula, Parminder S Suchdev, Fabian Rohner, Rafael Flores-Ayala, O Yaw Addo, Daniel J Raiten
Background: Total body iron (TBI) that is calculated from ferritin and soluble transferrin receptor (sTfR) allows for the evaluation of the full range of iron status from deficiency to excess. However, both ferritin and sTfR are affected by inflammation and malaria, which may require a statistical adjustment. TBI has been used to assess iron status in the United States, but its use worldwide and in settings with inflammation has been limited.Objective: We examine whether inflammation-adjusted ferritin and sTfR concentrations affect TBI values and the prevalence of low TBI (<0 mg/kg) in preschool children (PSC) (age range: 6-59 mo) and women of reproductive age (WRA) (age range: 15-49 y)...
June 14, 2017: American Journal of Clinical Nutrition
https://www.readbyqxmd.com/read/28615254/methodologic-approach-for-the-biomarkers-reflecting-inflammation-and-nutritional-determinants-of-anemia-brinda-project
#20
REVIEW
Sorrel Ml Namaste, Grant J Aaron, Ravi Varadhan, Janet M Peerson, Parminder S Suchdev
Background: The Biomarkers Reflecting Inflammation and Nutritional Determinants of Anemia (BRINDA) project is a multiagency and multicountry collaboration that was formed to improve micronutrient assessment and to better characterize anemia.Objectives: The aims of the project were to 1) identify factors associated with inflammation, 2) assess the relations between inflammation, malaria infection, and biomarkers of iron and vitamin A status and compare adjustment approaches, and 3) assess risk factors for anemia in preschool children (PSC) and women of reproductive age (WRA)...
June 14, 2017: American Journal of Clinical Nutrition
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