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Sickle cell infant

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)
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
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
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
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
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
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
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
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
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
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
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
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
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
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
Grace Ndeezi, Charles Kiyaga, Arielle G Hernandez, Deogratias Munube, Thad A Howard, Isaac Ssewanyana, Jesca Nsungwa, Sarah Kiguli, Christopher M Ndugwa, Russell E Ware, Jane R Aceng
BACKGROUND: Sickle cell disease contributes substantially to mortality in children younger than 5 years in sub-Saharan Africa. In Uganda, 20,000 babies per year are thought to be born with sickle cell disease, but accurate data are not available. We did the cross-sectional Uganda Sickle Surveillance Study to assess the burden of disease. METHODS: The primary objective of the study was to calculate prevalence of sickle cell trait and disease. We obtained punch samples from dried blood spots routinely collected from HIV-exposed infants in ten regions and 112 districts across Uganda for the national Early Infant Diagnosis programme...
March 2016: Lancet Global Health
Tyler Shirel, Collin P Hubler, Rena Shah, Amy B Mager, Kathryn L Koch, Darshita Sheth, Michael R Uhing, Cresta W Jones, Joshua J Field
The objective of this study was to test the hypothesis that higher daily opioid dose is associated with the presence and severity of neonatal abstinence syndrome (NAS) in pregnant women with sickle cell disease (SCD). This was a retrospective study of pregnant women with SCD who required opioids. NAS was evaluated using the Finnegan scoring system and classified as none, mild, and severe. Severe NAS was defined as a Finnegan score ≥ 8 on 3 consecutive tests. Thirty-four pregnancies were examined in 30 women with SCD...
June 2016: American Journal of Hematology
Venée N Tubman, Roseda Marshall, Wilhemina Jallah, Dongjing Guo, Clement Ma, Kwaku Ohene-Frempong, Wendy B London, Matthew M Heeney
BACKGROUND: In malaria-endemic countries in West Africa, sickle cell disease (SCD) contributes to childhood mortality. Historically, Liberia had regions wherein hemoglobin S and beta-thalassemia trait were mutually exclusive. Data on hemoglobinopathies in the Monrovia, the capital, are outdated and do not reflect urban migration. Updating the epidemiology of SCD is necessary to plan a public health and clinical agenda. Neither newborn screening (NBS) nor screening tools were available in country...
April 2016: Pediatric Blood & Cancer
Aline Menezes Carlos, Renata Andréia Volpe Souza, Bruna Maria Bereta de Souza, Gilberto de Araujo Pereira, Sebastião Tostes Júnior, Paulo Roberto Juliano Martins, Helio Moraes-Souza
CONTEXT AND OBJECTIVE: Hemoglobinopathies are among the commonest and most widespread genetic disorders worldwide. Their prevalence varies according to ethnic composition and/or geographical region. The aim of this study was to investigate the presence of hemoglobinopathies and their association with ethnicity among 1,004 newborns, to confirm the guideline of the Brazilian National Neonatal Screening Program. DESIGN AND SETTING: Cross-sectional study conducted in a public referral hospital in the Triângulo Mineiro region, Minas Gerais, Brazil...
September 2015: São Paulo Medical Journal, Revista Paulista de Medicina
Aggrey Dhabangi, Brenda Ainomugisha, Christine Cserti-Gazdewich, Henry Ddungu, Dorothy Kyeyune, Ezra Musisi, Robert Opoka, Christopher P Stowell, Walter H Dzik
IMPORTANCE: Although millions of transfusions are given annually worldwide, the effect of red blood cell (RBC) unit storage duration on oxygen delivery is uncertain. OBJECTIVE: To determine if longer-storage RBC units are not inferior to shorter-storage RBC units for tissue oxygenation as measured by reduction in blood lactate levels and improvement in cerebral tissue oxygen saturation among children with severe anemia. DESIGN, SETTING, AND PARTICIPANTS: Randomized noninferiority trial of 290 children (aged 6-60 months), most with malaria or sickle cell disease, presenting February 2013 through May 2015 to a university-affiliated national referral hospital in Kampala, Uganda, with a hemoglobin level of 5 g/dL or lower and a lactate level of 5 mmol/L or higher...
December 15, 2015: JAMA: the Journal of the American Medical Association
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