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Sickle cell infant

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https://www.readbyqxmd.com/read/29666710/acute-chest-syndrome-progressing-to-ards-in-a-patient-of-25-week-gestation
#1
Jefferson Chambers, Nichole Smith, Matthew Sehring, Subramanyam Chittivelu
Acute chest syndrome is a complication of sickle cell disease and represents the highest cause of mortality in those afflicted with the disorder. Pregnancy represents an increased risk for complications of sickle cell disease in both the mother and fetus. We present a case of a 20-year-old patient with known sickle cell disease who was at 25-week gestation and developed acute chest syndrome refractory to conventional therapies and requiring emergency cesarean section. Following delivery, the patient developed acute respiratory distress syndrome (ARDS) requiring extracorporeal membrane oxygenation (ECMO)...
2018: Case Reports in Critical Care
https://www.readbyqxmd.com/read/29580450/opioid-utilization-by-pregnant-women-with-sickle-cell-disease-and-the-risk-of-neonatal-abstinence-syndrome
#2
Aisha Nnoli, Neil S Seligman, Kevin Dysart, Jason K Baxter, Samir K Ballas
BACKGROUND: Pregnant women with sickle cell disease (SCD) are at increased risk of maternal and fetal complications. There are limited data on the outcome of the treatment of VOCs with opioids in relation to neonatal complications during pregnancy. METHODS: This is a retrospective cohort study of women with SCD from January 1999 to December 2008. Women with SCD were identified by ICD-9 codes and matched 2:1 to a control group of women on methadone for opioid dependence...
April 2018: Journal of the National Medical Association
https://www.readbyqxmd.com/read/29419921/the-evolving-contribution-of-non-communicable-diseases-ncds-to-maternal-mortality-in-jamaica-1998-2015-a-population-based-study
#3
Affette M McCaw-Binns, Leroy V Campbell, Simone S Spence
OBJECTIVE: Describe trends in indirect cause-specific pregnancy-related mortality from 1998-2015. DESIGN: Secondary analysis of annual, national cross-sectional database of maternal and late maternal deaths, identified through active surveillance of deaths among women 10-50 years. SETTING: Jamaica, a middle-income Caribbean country. POPULATION: Maternal and late maternal deaths. METHODS: Descriptive trend analyses of demographic and cause-specific maternal and pregnancy-related mortality ratios undertaken comparing the periods 1998-2003, 2004-9 and 2010-15...
February 8, 2018: BJOG: An International Journal of Obstetrics and Gynaecology
https://www.readbyqxmd.com/read/29195085/clinical-and-hematological-profile-in-a-newborn-cohort-with-hemoglobin-sc
#4
Paulo V Rezende, Millane V Santos, Gustavo F Campos, Laura L M Vieira, Maristela B Souza, André R Belisário, Celia M Silva, Marcos B Viana
OBJECTIVES: Hemoglobin SC is the second most common variant of sickle-cell disease worldwide, after hemoglobin SS. The objectives of the study were to describe the clinical and laboratory characteristics of hemoglobin SC disease in children from a newborn screening program and treated at a blood center. METHODOLOGY: This study assessed a cohort of 461 infants born between 01/01/1999 and 12/31/2012 and followed-up until 12/31/2014. Clinical events were expressed as rates for 100 patient-years, with 95% confidence intervals...
November 29, 2017: Jornal de Pediatria
https://www.readbyqxmd.com/read/29182088/perinatal-maternal-mortality-in-sickle-cell-anemia-two-case-reports-and-review-of-the-literature
#5
Sanaa Rizk, Elizabeth D Pulte, David Axelrod, Samir K Ballas
As outcomes of patients with sickle cell anemia improve and survival into adulthood with good quality of life and expectation of long-term survival becomes more common, challenges have developed, including issues related to reproduction. Pregnancy is frequently complicated in patients with sickle cell anemia with mortality up to 4.0%. Here we report maternal perinatal mortality in two women with sickle cell anemia who died post-partum due to acute chest syndrome (ACS), caused by bone marrow fat embolism and review the literature pertinent to this subject...
July 2017: Hemoglobin
https://www.readbyqxmd.com/read/29178654/sickle-cell-trait-knowledge-and-health-literacy-in-caregivers-who-receive-in-person-sickle-cell-trait-education
#6
Susan Creary, Ismahan Adan, Joseph Stanek, Sarah H O'Brien, Deena J Chisolm, Tanica Jeffries, Kristin Zajo, Elizabeth Varga
BACKGROUND: Despite universal screening that detects sickle cell trait (SCT) in infancy, only 16% of Americans with SCT know their status. To increase SCT status awareness, effective education for patients and their families is needed. The objective of this study was to assess caregivers' SCT knowledge before and after an in-person SCT education session. METHODS: A trained educator provides in-person SCT education to caregivers of referred infants with SCT at Nationwide Children's Hospital...
November 2017: Molecular Genetics & Genomic Medicine
https://www.readbyqxmd.com/read/29147675/newborn-screening-for-sickle-cell-disease-in-st-vincent-and-the-grenadines-results-of-a-pilot-newborn-screening-program
#7
Shelly-Ann Williams, Beneka Browne-Ferdinand, Ynolde Smart, Kristen Morella, Susan G Reed, Julie Kanter
Objective: To pilot a newborn screening program for sickle cell disease (SCD) in St. Vincent and the Grenadines using a novel partnership method to determine the feasibility of a universal newborn screening program in this country. Methods : A prospective study of mothers and their newborns was conducted between January 1, 2015, and November 1, 2015, at the country's main hospital. Mothers of infants born at this hospital were offered screening for SCD for their infants. If accepted, the newborn's heel-stick blood specimen was obtained and mailed to the South Carolina Department of Health and Environmental Control Newborn Screening Laboratory for testing...
2017: Global Pediatric Health
https://www.readbyqxmd.com/read/29104181/evaluation-of-newborn-sickle-cell-screening-programme-in-england-2010-2016
#8
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
#9
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
#10
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...
April 2018: 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
#11
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...
September 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
#12
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
#13
COMPARATIVE STUDY
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...
January 2018: 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
#14
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
#15
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
#16
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
#17
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
#18
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
#19
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
#20
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
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