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Sickle cell infant

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https://www.readbyqxmd.com/read/29104181/evaluation-of-newborn-sickle-cell-screening-programme-in-england-2010-2016
#1
Allison Streetly, Rupa Sisodia, Moira Dick, Radoslav Latinovic, Kirsty Hounsell, Elizabeth Dormandy
OBJECTIVE: To evaluate England's NHS newborn sickle cell screening programme performance in children up to the age of 5 years. DESIGN: Cohort of resident infants with sickle cell disease (SCD) born between 1 September 2010 and 31 August 2015 and followed until August 2016. PARTICIPANTS: 1317 infants with SCD were notified to the study from all centres in England and 1313 (99%) were followed up. INTERVENTIONS: Early enrolment in clinical follow-up, parental education and routine penicillin prophylaxis...
November 5, 2017: Archives of Disease in Childhood
https://www.readbyqxmd.com/read/28994899/prophylactic-antibiotics-for-preventing-pneumococcal-infection-in-children-with-sickle-cell-disease
#2
REVIEW
Angela E Rankine-Mullings, Shirley Owusu-Ofori
BACKGROUND: Persons with sickle cell disease (SCD) are particularly susceptible to infection. Infants and very young children are especially vulnerable. The 'Co-operative Study of Sickle Cell Disease' observed an incidence rate for pneumococcal septicaemia of 10 per 100 person years in children under the age of three years. Vaccines, including customary pneumococcal vaccines, may be of limited use in this age group. Therefore, prophylactic penicillin regimens may be advisable for this population...
October 10, 2017: Cochrane Database of Systematic Reviews
https://www.readbyqxmd.com/read/28988213/management-of-pneumatosis-intestinalis-in-children-over-the-age-of-6-months-a-conservative-approach
#3
Leel Nellihela, Mohamed Mutalib, David Thompson, Kammermeier Jochen, Manasvi Upadhyaya
BACKGROUND: Pneumatosis intestinalis (PI) is an uncommon and poorly understood condition. Although it can be an incidental finding in asymptomatic individuals, it can also be secondary to life-threatening bowel ischaemia and sepsis. In premature infants, it is a pathognomonic sign of necrotising enterocolitis. There is no consensus regarding management and long-term outcome of children with PI. AIM: Review of our experience of PI in children beyond the early infantile period...
October 7, 2017: Archives of Disease in Childhood
https://www.readbyqxmd.com/read/28844459/the-pressing-need-for-point-of-care-diagnostics-for-sickle-cell-disease-a-review-of-current-and-future-technologies
#4
Patrick T McGann, Carolyn Hoppe
Sickle cell disease (SCD) is a common and life threatening inherited blood disorder, affecting over 300,000 newborns per year. Over 75% of SCD births occur in sub-Saharan Africa, where the lack of timely and accurate diagnosis results in premature death within the first few years of life for a majority of affected infants. Current methods to diagnosis SCD require expensive laboratory equipment and reagents, and adequately trained laboratory personnel. In addition, test results are often delayed due to transport and batching of samples in a central laboratory...
August 8, 2017: Blood Cells, Molecules & Diseases
https://www.readbyqxmd.com/read/28816916/developmental-screening-in-pediatric-sickle-cell-disease-disease-related-risk-and-screening-outcomes-in-4-year-olds
#5
Jeffrey Schatz, Alyssa Schlenz, Laura Reinman, Kelsey Smith, Carla W Roberts
OBJECTIVE: Studies of early child development in sickle cell disease (SCD) have found modest associations between disease-related risks and developmental status in infants and toddlers, but such associations are evident by early elementary school. We screened 4-year-old children with SCD using 2 screening strategies to assess if biomedical risk factors for neurologic disease are related to developmental screening outcomes at this intermediate age. METHODS: Seventy-seven 4-year-old children with SCD (M = 4...
October 2017: Journal of Developmental and Behavioral Pediatrics: JDBP
https://www.readbyqxmd.com/read/28801954/an-analysis-of-inpatient-pediatric-sickle-cell-disease-incidence-costs-and-outcomes
#6
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
#7
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...
September 2017: Annals of Clinical Biochemistry
https://www.readbyqxmd.com/read/28493472/turf-wars-exploring-splenomegaly-in-sickle-cell-disease-in-malaria-endemic-regions
#8
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
#9
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
#10
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
#11
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...
September 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
#12
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
#13
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
#14
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
#15
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
#16
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
#17
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
#18
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
#19
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
#20
MULTICENTER STUDY
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...
July 26, 2016: BMC Health Services Research
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