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Sickle cell infant

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https://www.readbyqxmd.com/read/28801954/an-analysis-of-inpatient-pediatric-sickle-cell-disease-incidence-costs-and-outcomes
#1
Laura M Bou-Maroun, Fabien Meta, Curtis J Hanba, Andrew D Campbell, Gregory A Yanik
OBJECTIVE: To identify characteristics of pediatric sickle cell disease (SCD) hospitalizations and to examine admission demographics and medical expenditures. METHODS: Admissions with SCD were identified from the 2009 and 2012 releases of the Healthcare and Cost Utilization Project's Kids Inpatient Database. Disease-specific secondary diagnoses including acute chest syndrome (ACS), vaso-occlusive pain crisis (VOC), splenic sequestration, and stroke/transient ischemic attack were analyzed for patient and hospital demographics...
August 12, 2017: Pediatric Blood & Cancer
https://www.readbyqxmd.com/read/28656815/newborn-screening-for-sickle-cell-disorders-using-tandem-mass-spectrometry-three-years-experience-of-using-a-protocol-to-detect-only-the-disease-states
#2
Stuart J Moat, Derek Rees, Roanna S George, Lawrence King, Alan Dodd, Adeboye Ifederu, Tejswurree Ramgoolam, Sharon Hillier
Background Tandem mass spectrometry (MS/MS) has recently become an alternative method for the newborn screening of sickle cell disorders (SCD), as it is able to detect haemoglobin (Hb) peptides following digestion of bloodspots with trypsin. Using the SpOtOn Diagnostics Reagent Kit, we previously developed a screening protocol to detect only the disease states of SCD, using action values based on the ratio between the variant Hb peptide to wild-type peptide abundances for the HbS, C, D(Punjab), O(Arab), E and Lepore peptides...
January 1, 2017: Annals of Clinical Biochemistry
https://www.readbyqxmd.com/read/28493472/turf-wars-exploring-splenomegaly-in-sickle-cell-disease-in-malaria-endemic-regions
#3
REVIEW
Venée N Tubman, Julie Makani
Sickle cell disease (SCD) is a group of recessively inherited disorders of erythrocyte function that presents an ongoing threat to reducing childhood and adult morbidity and mortality around the world. While decades of research have led to improved survival for SCD patients in wealthy countries, survival remains dismal in low- and middle-income countries. Much of the early mortality associated with SCD is attributed to increased risk of infections due to early loss of splenic function. In the West, bacterial infections with encapsulated organisms are a primary concern...
June 2017: British Journal of Haematology
https://www.readbyqxmd.com/read/28477437/national-epidemiological-study-reveals-longer-paediatric-bone-and-joint-infection-stays-for-infants-and-in-general-hospitals
#4
E Laurent, L Petit, Z Maakaroun-Vermesse, L Bernard, T Odent, L Grammatico-Guillon
AIM: Published studies have suggested that two to five days of intravenous treatment could effectively treat paediatric bone and joint infections (PBJI), allowing a faster discharge. This study analysed the factors associated with PBJI hospital stays lasting longer than five days using the French National Hospital Discharge Database. METHODS: We selected children under 15 years hospitalised in 2013 with haematogenous PBJIs using a validated French algorithm based on specific diagnosis and surgical procedure codes...
May 6, 2017: Acta Paediatrica
https://www.readbyqxmd.com/read/28403055/immunogenicity-and-safety-of-10-valent-pneumococcal-nontypeable-haemophilus-influenzae-protein-d-conjugate-vaccine-phid-cv-administered-to-children-with-sickle-cell-disease-between-8-weeks-and-2-years-of-age-a-phase-iii-open-controlled-study
#5
Sodiomon B Sirima, Alfred Tiono, Zakaria Gansané, Mohamadou Siribié, Angèle Zongo, Alphonse Ouédraogo, Nancy François, Ana Strezova, Kurt Dobbelaere, Dorota Borys
BACKGROUND: Immunogenicity, safety and reactogenicity of the 10-valent pneumococcal nontypeable Haemophilus influenzae protein D conjugate vaccine (PHiD-CV) were evaluated in children with sickle cell disease (SCD), who are at increased risk for infections. METHODS: In this phase III, open-label, single-center, controlled study in Burkina Faso (NCT01175083), children with SCD (S) or without SCD (NS) were assigned to 6 groups (N = 300): children 8-11 weeks of age (<6 months; <6S and <6NS groups) received 3 primary doses and a booster dose of PHiD-CV coadministered with routine childhood vaccines; children 7-11 months of age (7-11S and 7-11NS groups) received 2 primary doses and a booster dose of PHiD-CV; children 12-23 months of age (12-23S and 12-23NS groups) received 2 catch-up doses of PHiD-CV...
May 2017: Pediatric Infectious Disease Journal
https://www.readbyqxmd.com/read/28228042/haemoglobin-f-a2-and-s-levels-in-subjects-with-or-without-sickle-cell-trait-in-south-eastern-gabon
#6
Landry-Erik Mombo, Gaël Mabioko-Mbembo, Roland-Fabrice Kassa-Kassa, Emmanuel Ontsitsagui, Statiana Mboui-Ondo, Leatitia Nzé-Kamsi, Dieudonné Nkoghé, Jacques Elion
BACKGROUND: Infant mortality due to sickle cell disease in sub-Saharan Africa is high, necessitating a better understanding of the modulating factors of the disease in this region. METHODS: We assessed the hereditary persistence of foetal haemoglobin and α-thalassemia. We diagnosed 787 subjects, with or without sickle cell trait, by capillary electrophoresis in the Medical Diagnostic Laboratory of the CIRMF (Franceville, Gabon). RESULTS: Heterocellular and pancellular forms of hereditary persistence of foetal haemoglobin occurred at low rates of 10...
February 23, 2017: Hematology (Amsterdam, Netherlands)
https://www.readbyqxmd.com/read/28030521/immunogenicity-and-safety-of-10-valent-pneumococcal-non-typeable-haemophilus-influenzae-protein-d-conjugate-vaccine-phid-cv-administered-to-children-with-sickle-cell-disease-between-8-weeks-and-2-years-of-age-a-phase-iii-open-controlled-study
#7
Sodiomon B Sirima, Alfred Tiono, Zakaria Gansané, Mohamadou Siribié, Angèle Zongo, Alphonse Ouédraogo, Nancy François, Ana Strezova, Kurt Dobbelaere, Dorota Borys
BACKGROUND: Immunogenicity, safety and reactogenicity of the 10-valent pneumococcal non-typeable Haemophilus influenzae protein D conjugate vaccine (PHiD-CV) were evaluated in children with sickle cell disease (SCD), who are at increased risk for infections. METHODS: In this phase III, open-label, single center, controlled study in Burkina Faso (NCT01175083), children with SCD (S) or without SCD (NS) were assigned to 6 groups (N=300): children 8-11 weeks of age (<6 months; <6S and <6NS groups) received 3 primary doses and a booster dose of PHiD-CV co-administered with routine childhood vaccines; children 7-11 months of age (7-11S and 7-11NS groups) received 2 primary doses and a booster dose of PHiD-CV; children 12-23 months of age (12-23S and 12-23NS groups) received 2 catch-up doses of PHiD-CV...
December 27, 2016: Pediatric Infectious Disease Journal
https://www.readbyqxmd.com/read/28005272/prophylactic-versus-selective-blood-transfusion-for-sickle-cell-disease-in-pregnancy
#8
REVIEW
Babasola O Okusanya, Olufemi T Oladapo
BACKGROUND: Pregnant women with sickle cell disease (HbSS, HbSC and HbSβThal) may require blood transfusion to prevent severe anaemia or to manage potential medical complications. Preventive blood transfusion in the absence of complications starting from the early weeks of pregnancy or blood transfusion only for medical or obstetric indications have been used as management policies. There is currently no consensus on the blood transfusion policy that guarantees optimal clinical benefits with minimal risks for such women and their babies...
December 22, 2016: Cochrane Database of Systematic Reviews
https://www.readbyqxmd.com/read/27776167/birth-weights-in-sickle-cell-disease-pregnancies-a-cohort-study
#9
Daveena Meeks, Susan E Robinson, David Macleod, Eugene Oteng-Ntim
Pregnancy in women with Sickle Cell Disease (SCD) has been linked with an increased incidence of adverse foetal outcomes when compared to women without haemoglobinopathies (HbAA). There's a paucity of data into foetal outcomes for infants born to women with SCD. Customised growth charts have been demonstrated to be better than population-based growth charts at identifying unhealthy small babies. We analysed the mean birth weight and customised birth weight centiles of infants born to mothers with SCD versus mothers with HbAA genotype, to quantify the risk of having a smaller baby...
2016: PloS One
https://www.readbyqxmd.com/read/27670685/acquisition-of-natural-humoral-immunity-to-p-falciparum-in-early-life-in-benin-impact-of-clinical-environmental-and-host-factors
#10
Célia Dechavanne, Ibrahim Sadissou, Aziz Bouraima, Claude Ahouangninou, Roukiyath Amoussa, Jacqueline Milet, Kabirou Moutairou, Achille Massougbodji, Michael Theisen, Edmond J Remarque, David Courtin, Gregory Nuel, Florence Migot-Nabias, André Garcia
To our knowledge, effects of age, placental malaria infection, infections during follow-up, nutritional habits, sickle-cell trait and individual exposure to Anopheles bites were never explored together in a study focusing on the acquisition of malaria antibody responses among infants living in endemic areas.Five hundred and sixty-seven Beninese infants were weekly followed-up from birth to 18 months of age. Immunoglobulin G (IgG), IgG1 and IgG3 specific for 5 malaria antigens were measured every 3 months. A linear mixed model was used to analyze the effect of each variable on the acquisition of antimalarial antibodies in 6-to18-month old infants in univariate and multivariate analyses...
September 27, 2016: Scientific Reports
https://www.readbyqxmd.com/read/27625488/factors-affecting-recovery-of-postoperative-bowel-function-after-pediatric-laparoscopic-surgery
#11
Daphnée Michelet, Juliette Andreu-Gallien, Alia Skhiri, Arnaud Bonnard, Yves Nivoche, Souhayl Dahmani
BACKGROUND AND AIMS: Laparoscopic pediatric surgery allows a rapid postoperative rehabilitation and hospital discharge. However, the optimal postoperative pain management preserving advantages of this surgical technique remains to be determined. This study aimed to identify factors affecting the postoperative recovery of bowel function after laparoscopic surgery in children. MATERIAL AND METHODS: A retrospective analysis of factors affecting recovery of bowel function in children and infants undergoing laparoscopic surgery between January 1, 2009 and September 30, 2009, was performed...
July 2016: Journal of Anaesthesiology, Clinical Pharmacology
https://www.readbyqxmd.com/read/27566421/progress-in-pediatrics-in-2015-choices-in-allergy-endocrinology-gastroenterology-genetics-haematology-infectious-diseases-neonatology-nephrology-neurology-nutrition-oncology-and-pulmonology
#12
REVIEW
Carlo Caffarelli, Francesca Santamaria, Dora Di Mauro, Carla Mastrorilli, Virginia Mirra, Sergio Bernasconi
This review focuses key advances in different pediatric fields that were published in Italian Journal of Pediatrics and in international journals in 2015. Weaning studies continue to show promise for preventing food allergy. New diagnostic tools are available for identifying the allergic origin of allergic-like symptoms. Advances have been reported in obesity, short stature and autoimmune endocrine disorders. New molecules are offered to reduce weight gain and insulin-resistance in obese children. Regional investigations may provide suggestions for preventing short stature...
August 27, 2016: Italian Journal of Pediatrics
https://www.readbyqxmd.com/read/27509845/a-pilot-study-of-parent-education-intervention-improves-early-childhood-development-among-toddlers-with-sickle-cell-disease
#13
Melanie E Fields, Catherine Hoyt-Drazen, Regina Abel, Mark J Rodeghier, Janet M Yarboi, Bruce E Compas, Allison A King
BACKGROUND: Young children with sickle cell disease (SCD) are at risk for cognitive delay. In addition to biologic risk factors associated with SCD, environmental factors contribute to cognitive dysfunction within this cohort. METHODS: We completed a single-arm, prospective cohort study. Children with SCD between the ages of 3 and 36 months and their caregivers were followed between October 2010 and December 2013. The aim was to describe the role of a home visitation model, the home environment, and socioeconomic status in the development of young children with SCD...
December 2016: Pediatric Blood & Cancer
https://www.readbyqxmd.com/read/27492747/loss-of-major-dnase-i-hypersensitive-sites-in-duplicated-%C3%AE-globin-gene-cluster-incompletely-silences-hbb-gene-expression
#14
N Scott Reading, Claire Shooter, Jihyun Song, Robin Miller, Archana Agarwal, Lucie Lanikova, Barnaby Clark, Swee Lay Thein, Vladimir Divoky, Josef T Prchal
We report an infant with sickle cell disease phenotype by biochemical analysis whose β-globin gene (HBB) sequencing showed sickle cell mutation (HBB(S) ) heterozygosity. The proband has a unique head-to-tail duplication of the β-globin gene cluster having wild-type (HBB(A) ) and HBB(S) alleles inherited from her father; constituting her HBB(S) /HBB(S) -HBB(A) genotype. Further analyses revealed that proband's duplicated β-globin gene cluster (∼650 kb) encompassing HBB(A) does not include the immediate upstream locus control region (LCR) or 3' DNase I hypersensitivity (HS) element...
November 2016: Human Mutation
https://www.readbyqxmd.com/read/27461265/newborn-screening-and-prophylactic-interventions-for-sickle-cell-disease-in-47-countries-in-sub-saharan-africa-a-cost-effectiveness-analysis
#15
Andreas Kuznik, Abdulrazaq G Habib, Deogratias Munube, Mohammed Lamorde
BACKGROUND: Sickle cell disease (SCD) constitutes a major public health problem in sub-Saharan Africa (SSA). Newborn screening and early subsequent clinical intervention can reduce early mortality and increase life expectancy, but have not been widely implemented in SSA. This analysis assesses the cost-effectiveness of a newborn screening and prophylactic intervention (NSPI) package for SCD in 47 SSA countries. METHODS: A lifetime Markov model with annual cycles was built with infants either being screened using isoelectric focusing (IEF) or not screened...
2016: BMC Health Services Research
https://www.readbyqxmd.com/read/27242200/contemporary-outcomes-of-sickle-cell-disease-in-pregnancy
#16
Kelly Kuo, Aaron B Caughey
BACKGROUND: Data regarding pregnancy outcomes in sickle cell disease are conflicting. Previous studies are limited by small sample size, narrow geographic area, and a wide range of resource availability. OBJECTIVE: The purpose of this study was to examine the association between maternal sickle cell disease and adverse pregnancy outcomes in a contemporary North American cohort. STUDY DESIGN: We performed a retrospective cohort study of 2,027,323 women with singleton pregnancies delivered in California from 2005-2008...
October 2016: American Journal of Obstetrics and Gynecology
https://www.readbyqxmd.com/read/27026724/minireview-prognostic-factors-and-the-response-to-hydroxurea-treatment-in-sickle-cell-disease
#17
REVIEW
Winfred C Wang
This review describes current considerations in the use of hydroxyurea for the management of sickle cell disease in the context of clinical severity. Randomized trials of hydroxyurea have generally enrolled subjects with increased severity based on frequent vaso-occlusive events. An exception was the BABY HUG study in infants which documented substantial benefit even for asymptomatic subjects. Increasing data indicate that hydroxyurea has a substantial effect on reducing mortality in both adults and children-perhaps the most compelling reason for advocating the drug's widespread use...
April 2016: Experimental Biology and Medicine
https://www.readbyqxmd.com/read/27001763/absence-of-association-between-sickle-trait-hemoglobin-and-placental-malaria-outcomes
#18
Jaymin C Patel, Victor Mwapasa, Linda Kalilani, Feiko O Ter Kuile, Carole Khairallah, Kyaw L Thwai, Steven R Meshnick, Steve M Taylor
Heterozygous hemoglobin S (HbAS), or sickle trait, protects children from life-threatening falciparum malaria, potentially by attenuating binding of Plasmodium-infected red blood cells (iRBCs) to extracellular ligands. Such binding is central to the pathogenesis of placental malaria (PM). We hypothesized that HbAS would be associated with reduced risks of PM and low birth weight (LBW). We tested this hypothesis in 850 delivering women in southern Malawi. Parasites were detected by polymerase chain reaction in placental and peripheral blood, and placentae were scored histologically for PM...
May 4, 2016: American Journal of Tropical Medicine and Hygiene
https://www.readbyqxmd.com/read/26908823/examination-of-the-comorbidity-of-mental-illness-and-somatic-conditions-in-hospitalized-children-in-the-united-states-using-the-kids-inpatient-database-2009
#19
Dina M Sztein, Wendy G Lane
OBJECTIVE: To examine the associations between mental and physical illness in hospitalized children. METHODS: The data for this analysis came from the 2009 Kids' Inpatient Database (KID). Any child with an International Classification of Diseases, Ninth Revision code indicative of depressive, anxiety, or bipolar disorders or a diagnosis of sickle cell disease, diabetes mellitus type 1 or 2, asthma, or attention-deficit/hyperactivity disorder (ADHD) were included...
March 2016: Hospital Pediatrics
https://www.readbyqxmd.com/read/26862624/audit-of-blood-transfusion-practices-in-the-paediatric-medical-ward-of-a-tertiary-hospital-in-southeast-nigeria
#20
M D Ughasoro, A N Ikefuna, I J Emodi, S N Ibeziako, S O Nwose
OBJECTIVES: To determine the indications, practices and outcomes of transfusion on children. DESIGN: A descriptive retrospective study. SETTING: Paediatric wards of University of Nigeria Teaching Hospital, Enugu, Nigeria. SUBJECTS: Children one month to 18 years that received blood transfusion. MAIN OUTCOME MEASURES: Indications for the transfusion, haemoglobin rise, vital signs, duration of transfusion and adverse events...
January 2013: East African Medical Journal
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