23,194 results match your criteria Sickle Cell Anemia


Hematopoietic stem cell transplantation in children with sickle cell anemia: The parents' experience.

Pediatr Transplant 2019 Feb 20:e13376. Epub 2019 Feb 20.

Department of Psychopathology, Avicenne Hospital, Paris XIII University & Assistance Publique-Hôpitaux de Paris, Bobigny, France.

Genoidentical HSCT is currently the only curative treatment for SCA, preventing further vascular complications in high-risk children. Studies on the psychological implications of HSCT for recipient, sibling donor, and the rest of the family have been limited in SCA. This study enrolled ten families and used semi-structured interviews to explore the parents' experience at three time points: first before transplantation, then 3 months later, and 1 year later. Read More

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http://dx.doi.org/10.1111/petr.13376DOI Listing
February 2019

Rapid and reproducible characterization of sickling during automated deoxygenation in sickle cell disease patients.

Am J Hematol 2019 Feb 19. Epub 2019 Feb 19.

Laboratory of Clinical Chemistry & Hematology, University Medical Center Utrecht, Utrecht University, Utrecht, The Netherlands.

In sickle cell disease (SCD), sickle hemoglobin (HbS) polymerizes upon deoxygenation, resulting in sickling of red blood cells (RBCs). These sickled RBCs have strongly reduced deformability, leading to vaso-occlusive crises and chronic hemolytic anemia. To date, there are no reliable laboratory parameters or assays capable of predicting disease severity or monitoring treatment effects. Read More

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http://dx.doi.org/10.1002/ajh.25443DOI Listing
February 2019

Hydroxyurea Safe and Effective for Sickle Cell Anemia.

Authors:
M J Friedrich

JAMA 2019 Feb;321(7):637

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http://dx.doi.org/10.1001/jama.2019.0592DOI Listing
February 2019

Magnitude of elevated iron stores and risk associated in steady state sickle cell anemia Congolese children: a cross sectional study.

BMC Hematol 2019 8;19. Epub 2019 Feb 8.

1Division of Nephrology, Nephrology Unit, Department of Internal Medicine, University Hospital of Kinshasa, Faculty of Medicine, University of Kinshasa, Kinshasa XI, PO.BOX 123, Democratic Republic of Congo.

Background: The serum ferritin assay is recommended in Sickle Cell Anemia (SCA) patients receiving regular transfusions. According to several authors, elevated iron stores indicating iron chelation corresponds to hyperferritinemia ≥500 ng/ml, and becomes detectable after twenty blood transfusions. The objectives of the study were to determine the prevalence of elevated iron stores and identify associated risk factors in a case series of Steady state SCA Congolese children. Read More

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http://dx.doi.org/10.1186/s12878-019-0134-7DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6368684PMC
February 2019

HemoTypeSC, a low-cost point-of-care testing device for sickle cell disease: Promises and challenges.

Blood Cells Mol Dis 2019 Feb 8. Epub 2019 Feb 8.

Department of Paediatrics, Kuwait University, Kuwait.

Background: Sickle cell disease (SCD) is a neglected burden of growing importance. >312,000 births are affected annually by sickle cell anaemia (SCA). Early interventions such as newborn screening, penicillin prophylaxis and hydroxyurea can substantially reduce the mortality and morbidity associated with SCD. Read More

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http://dx.doi.org/10.1016/j.bcmd.2019.01.007DOI Listing
February 2019

Overcoming challenges of venous thromboembolism in sickle cell disease treatment.

Expert Rev Hematol 2019 Feb 16. Epub 2019 Feb 16.

a Johns Hopkins School of Medicine , Baltimore , MD , USA.

Introduction: Venous thromboembolism (VTE) is a common comorbid condition found in sickle cell disease (SCD) and is associated with increased mortality for adults with SCD. The pathophysiology that leads to the thrombophilic state in SCD has been previously reviewed; however, evidence-based guidelines to aid in diagnosis, prevention and management of VTE are lacking. Areas covered: This review article will cover the pathophysiology underlying the hypercoagulable state, the epidemiology of VTE, and management strategies of VTE in SCD. Read More

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http://dx.doi.org/10.1080/17474086.2019.1583554DOI Listing
February 2019

Single tube allele specific PCR: a low cost technique for molecular screening of sickle cell anaemia in Nigeria.

Afr Health Sci 2018 Dec;18(4):995-1002

University of Lagos, Department of Cell Biology and Genetics.

Background: Sickle cell anaemia (SCA) is a hereditary blood disorder caused by a single mutation in the haemoglobin gene. The disease burden of SCA is highest in Nigeria. The allele specific polymerase chain reaction (ASPCR) method is applicable for the direct detection of known single nucleotide polymorphisms (SNPs). Read More

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http://dx.doi.org/10.4314/ahs.v18i4.20DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6354853PMC
December 2018

[Quality of Life of Adolescents with Homozygous Sickle-Cell Disease Managed at the Brazzaville Teaching Hospital (Congo)].

Bull Soc Pathol Exot 2018 ;111(1):46-50

Service de pédiatrie nourrissons, CHU de Brazzaville, faculté des sciences de la santé, université Marien-Ngouabi, Brazzaville, BP 32, CG-Brazzaville, Congo.

Adolescence is a particular age group in terms of psychological and somatic changes. Thus, the experience of a chronic disease such as sickle-cell disease is different compared to the younger child who depends entirely on his parents. The purpose of this study is to assess the quality of life and to identify the determinants of good quality of life of such teenagers. Read More

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http://dx.doi.org/10.3166/bspe-2018-0006DOI Listing
January 2018

Automatic depletion with Spectra Optia allows a safe 16% reduction of red blood cell pack consumption in exchanged sickle cell anemia patients.

Transfusion 2019 Feb 12. Epub 2019 Feb 12.

Laboratoire Interuniversitaire de Biologie de la Motricité (LIBM) EA7424, Equipe "Biologie vasculaire et du globule rouge", Université Claude Bernard Lyon 1, COMUE Lyon, Villeurbanne, France.

Background: Chronic red blood cell exchanges (RBCXs) are frequently used to prevent complications in patients with sickle cell anemia, but the scarcity of matched red blood cell packs (RBCPs) is a serious concern. The main goal of this study was to compare the number of RBCPs used during RBCXs between the Spectra Optia (SO) device (with the automatic depletion step) and the former Cobe Spectra (CSP) device.

Study Design And Methods: The performances and safety of 300 SO sessions using the automatic depletion step (SO/DE) in 50 patients with sickle cell anemia under a chronic transfusion program over a 1-year period were prospectively analyzed. Read More

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http://dx.doi.org/10.1111/trf.15188DOI Listing
February 2019
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Circulating Microparticles in Children With Sickle Cell Anemia in a Tertiary Center in Upper Egypt.

Clin Appl Thromb Hemost 2019 Jan-Dec;25:1076029619828839

6 Department of Community Health Nursing, Faculty of Nursing, Assiut University, Assiut, Egypt.

Sickle cell disease (SCD) is a genetically inherited hemolytic anemia increasingly appreciated as a chronic inflammatory condition and hypercoagulable state with high thrombotic risk. It is associated with disturbed immune phenotype and function and circulating microparticles (MPs) derived from multiple cell sources. This study was carried out to determine MPs profiles in patients with sickle cell anemia (either on hydroxyurea (HU) therapy or those with no disease-modifying therapy) and to compare these profiles with healthy children. Read More

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http://dx.doi.org/10.1177/1076029619828839DOI Listing
February 2019
1 Read
1.575 Impact Factor

BMI percentile is an independent predictor of increase in lung function in children with sickle cell anemia.

Am J Hematol 2019 Feb 7. Epub 2019 Feb 7.

Department of Pediatrics, Vanderbilt-Meharry Center of Excellence in Sickle Cell Disease, Vanderbilt University Medical Center, Nashville, Tennessee.

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http://dx.doi.org/10.1002/ajh.25433DOI Listing
February 2019
1 Read

The EBMT activity survey report 2017: a focus on allogeneic HCT for nonmalignant indications and on the use of non-HCT cell therapies.

Bone Marrow Transplant 2019 Feb 6. Epub 2019 Feb 6.

Department of Stem Cell Transplantation, University Hospital Eppendorf, Hamburg, Germany.

Hematopoietic cell transplantation (HCT) is widely used for acquired and congenital disorders of the hematopoietic system. Number of transplants done in Europe and associated countries continues to rise with 45,418 HCT in 41,100 patients [(17,155 allogeneic (42%) and 23,945 autologous (58%)] reported by 683 centers in 50 countries in 2017. Main indications were myeloid malignancies 10,147 (25%; 96% allogeneic), lymphoid malignancies 26,488 (64%; 19% allogeneic), solid tumors 1,607 (3. Read More

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http://dx.doi.org/10.1038/s41409-019-0465-9DOI Listing
February 2019
1 Read

[Delayed hemolytic reaction to transfusion in sickle cell anemia. Report of one case].

Rev Med Chil 2018 Nov;146(11):1347-1350

Servicio de Medicina, Santiago, Chile.

Sickle cell anemia was a rare disease in Chile, especially in adults, however the recent immigration wave from Haiti is changing this scenario. We report a 29 year old black female from Haiti with a non-disclosed history of sickle cell anemia. She was transfused with two units of red blood cells, found unconscious and with jaundice five days later and admitted to the hospital. Read More

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http://dx.doi.org/10.4067/S0034-98872018001101347DOI Listing
November 2018
1 Read

Reprogramming of bone marrow derived mesenchymal stromal cells to human induced pluripotent stem cells from pediatric patients with hematological diseases using a commercial mRNA kit.

Blood Cells Mol Dis 2019 Jan 24. Epub 2019 Jan 24.

Stem Cell Transplant Unit, Aghia Sophia Children's Hospital, Athens 11527, Greece.

The potential use of patient-specific induced pluripotent stem cells (hiPSCs) in the study and treatment of hematological diseases requires the setup of efficient and safe protocols for hiPSC generation. We aimed to adopt a reprogramming method for large-scale production of integration-free patient-specific hiPSC-lines in our stem cell processing laboratory, which supports a pediatric hematopoietic stem cell transplant unit located at a tertiary care children's hospital. We describe our 5-year experience in generation of hiPSC-lines from human bone marrow-derived mesenchymal stromal cells (BM-MSCs) using synthetic mRNAs encoding reprogramming factors. Read More

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http://dx.doi.org/10.1016/j.bcmd.2019.01.003DOI Listing
January 2019
1 Read

Evaluation of Mandible Fractures in Patients With Sickle Cell Anemia-A Nationwide Study.

J Oral Maxillofac Surg 2019 Jan 3. Epub 2019 Jan 3.

Associate Professor, University of Pennsylvania, Philadelphia, PA.

Purpose: Sickle cell anemia is the most commonly inherited blood disorder in the United States. Despite its prevalence, clinicians know little about the extent of its impact on orofacial manifestations.

Materials And Methods: All patients with diagnoses of mandible fracture and sickle cell anemia admitted from 2004 through 2014 were identified using the National Inpatient Sample. Read More

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https://linkinghub.elsevier.com/retrieve/pii/S02782391183140
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http://dx.doi.org/10.1016/j.joms.2018.12.031DOI Listing
January 2019
8 Reads

Differential gene expression analysis of sickle cell anemia in steady and crisis state.

Ann Hum Genet 2019 Jan 30. Epub 2019 Jan 30.

Fundação Oswaldo Cruz, Centro de Pesquisas Gonçalo Moniz - Fiocruz/BA, 40296710, Salvador, Bahia, Brazil.

Sickle cell anemia is one of the most prevalent genetic diseases worldwide, showing great clinical heterogeneity. This study compared the gene expression patterns between sickle cell anemia pediatric patients in steady state and in crisis state, as compared to age-paired, healthy individuals. RNA sequencing was performed from these groups of patients/controls using Illumina HiSeq 2500 equipment. Read More

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http://dx.doi.org/10.1111/ahg.12290DOI Listing
January 2019
1 Read

White Matter Has Impaired Resting Oxygen Delivery in Sickle Cell Patients.

Am J Hematol 2019 Jan 29. Epub 2019 Jan 29.

Department of Biomedical Engineering, University of Southern California Engineering School, Los Angeles, California.

Although modern medical management has lowered overt stroke occurrence in patients with sickle cell disease (SCD), progressive white matter (WM) damage remains common. It is known that cerebral blood flow (CBF) increases to compensate for anemia, but sufficiency of cerebral oxygen delivery, especially in the WM, has not been systematically investigated. Cerebral perfusion was measured by arterial spin labeling in 32 SCD patients (age range: 10-42 years old, 14 males, 7 with HbSC, 25 HbSS) and 25 age and race-matched healthy controls (age range: 15-45 years old, 10 males, 12 with HbAS, 13 HbAA); 8/24 SCD patients were receiving regular blood transfusions and 14/24 non-transfused SCD patients were taking hydroxyurea. Read More

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http://dx.doi.org/10.1002/ajh.25423DOI Listing
January 2019
3 Reads

Recombinant erythropoietin as alternative to red cell transfusion in sickle cell disease.

Vox Sang 2019 Feb 29;114(2):178-181. Epub 2019 Jan 29.

Hematology and Transfusion Medicine Center, University of Campinas, Campinas, Brazil.

Disturbances in the physiological regulation of erythropoietin (EPO) in patients with sickle cell disease (SCD) may contribute to worsening anaemia and increased transfusion requirements, but the use of recombinant EPO in this group of patients is controversial. The objective of this study was to evaluate the use of this drug in adult patients with SCD and its effects on haemoglobin levels and transfusion requirements. We conducted a retrospective analysis at the University of Campinas, with nineteen adults with a diagnosis of SCD (HbSS and HbS/β thalassaemia), who had received at least 1 year of EPO therapy between 2007 and 2014. Read More

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http://dx.doi.org/10.1111/vox.12750DOI Listing
February 2019
3 Reads

Association between MTHFR 677C>T polymorphism and vascular complications in sickle cell disease: A meta-analysis.

Transfus Clin Biol 2019 Jan 10. Epub 2019 Jan 10.

Sickle Cell Institute Chhattisgarh, Forest Block-2, Jail Road, Raipur 492001, Chhattisgarh India. Electronic address:

Sickle cell disease (SCD) is considered as a vascular disease due to its chronic vascular manifestations such as leg ulcers, priapism, acute chest syndrome (ACS), stroke, retinopathy, renal insufficiency, pulmonary hypertension, avascular necrosis of the femoral head (AVNF) and splenic infarction. Emerging evidence has shown that the MTHFR 677C>T variant allele is associated with vascular complications (VC) in patients with SCD; however, results from individual studies are inconclusive. The aim of this meta-analysis is to evaluate the association between the MTHFR 677C>T polymorphism and the susceptibility for VC in SCD patients. Read More

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http://dx.doi.org/10.1016/j.tracli.2019.01.003DOI Listing
January 2019
1 Read

Surfactant protein D as a marker for pulmonary complications in pediatric patients with sickle cell disease: Relation to lung function tests.

Pediatr Pulmonol 2019 Jan 22. Epub 2019 Jan 22.

Faculty of Medicine, Pediatrics Department, Ain Shams University, Cairo, Egypt.

Background: Surfactant protein D (SP-D) is considered a candidate biomarker for lung integrity and for disease progression.

Aim: We determined the level of SP-D in children and adolescents with SCD and assessed its possible relation to pulmonary complications and lung function.

Methods: Serum SP-D levels were assessed in 50 SCD patients compared with 30 healthy controls. Read More

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http://dx.doi.org/10.1002/ppul.24257DOI Listing
January 2019
4 Reads

Chronic Kidney Disease amongst Sickle Cell Anaemia Patients at the University of Maiduguri Teaching Hospital, Northeastern Nigeria: A Study of Prevalence and Risk Factors.

Mediterr J Hematol Infect Dis 2019 1;11(1):e2019010. Epub 2019 Jan 1.

Department of Haematology university of Maiduguri. P.M.B 1069, Maiduguri Nigeria.

Background: Involvement of the kidneys in patients with sickle cell anaemia is a well recognised chronic complication. This study seeks to determine the prevalence of chronic kidney disease in patients with homozygous sickle cell disease (HbSS) and to identify risk factors associated with its development.

Methodology: The subjects consisted of adolescents and adults with HbSS recruited sequentially from the adult haematology outpatient clinic and Daycare ward of the unit. Read More

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http://dx.doi.org/10.4084/MJHID.2019.010DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6328039PMC
January 2019
1 Read

The Use of HPLC as a Tool for Neonatal Cord Blood Screening of haemoglobinopathy: A Validation Study.

Mediterr J Hematol Infect Dis 2019 1;11(1):e2019005. Epub 2019 Jan 1.

Department of Haematology, Sultan Qaboos University Hospital, Oman.

Background: Newborn cord blood screening identifies infants with underlying haemoglobinopathies before they develop the characteristic symptoms or sequelae.

Aims: This study was performed to validate the interpretation high-performance chromatography (HPLC) along with complete blood count (CBC) results as a tool for universal neonatal screening of hemoglobin disorders in Oman.

Methods: HPLC and CBC data on subjects who participated in the National Neonatal screening program at birth were obtained from archival records. Read More

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http://dx.doi.org/10.4084/MJHID.2019.005DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6328035PMC
January 2019
3 Reads

Association of Matched Sibling Donor Hematopoietic Stem Cell Transplantation With Transcranial Doppler Velocities in Children With Sickle Cell Anemia.

JAMA 2019 01;321(3):266-276

Department of Statistics, Saint-Louis Hospital, ECSTRA Team, UMR1153, INSERM, University Paris-Diderot, Paris, France.

Importance: In children with sickle cell anemia (SCA), high transcranial Doppler (TCD) velocities are associated with stroke risk, which is reduced by chronic transfusion. Whether matched sibling donor hematopoietic stem cell transplantation (MSD-HSCT) can reduce velocities in patients with SCA is unknown.

Objective: To determine the association of MSD-HSCT with TCD velocities as a surrogate for the occurrence of ischemic stroke in children with SCA. Read More

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http://dx.doi.org/10.1001/jama.2018.20059DOI Listing
January 2019
13 Reads

Matched Sibling Donor Hematopoietic Stem Cell Transplantation to Prevent Stroke in Children With Sickle Cell Anemia.

JAMA 2019 01;321(3):251-252

Division of Hematology, Children's Hospital of Philadelphia, Philadelphia, Pennsylvania.

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http://dx.doi.org/10.1001/jama.2018.20390DOI Listing
January 2019
1 Read

Serum zinc level during and after acute painful episodes in children with sickle cell anemia at the aminu kano teaching hospital, Kano, Northern Nigeria.

Niger J Clin Pract 2019 Jan;22(1):16-23

Department of Pediatrics, Bayero University/Aminu Kano Teaching Hospital, Kano, Nigeria.

Background: Acute painful crisis due to vaso-occlusive event is the leading cause of hospitalization in patients with sickle cell anemia (SCA). Zinc deficiency in children with SCA is associated with increased frequency and severity of acute painful events. We determined serum zinc level in children with SCA during acute painful crisis and compared the same with children with SCA who are in steady state and healthy non-sickle cell disease children. Read More

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http://dx.doi.org/10.4103/njcp.njcp_169_18DOI Listing
January 2019
2 Reads

Bead-based assays to simultaneously detect multiple human inherited blood disorders associated with malaria.

Malar J 2019 Jan 21;18(1):14. Epub 2019 Jan 21.

Department of Immunology and Infection, London School of Hygiene & Tropical Medicine, London, UK.

Background: Glucose-6-phosphate dehydrogenase deficiency (G6PDd), haemoglobin C (HbC) and S (HbS) are inherited blood disorders (IBD) common in populations in malaria endemic areas. All are associated to some degree with protection against clinical malaria whilst additionally G6PDd is associated with haemolysis following treatment with 8-aminoquinolines. Measuring the prevalence of these inherited blood disorders in affected populations can improve understanding of disease epidemiology. Read More

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https://malariajournal.biomedcentral.com/articles/10.1186/s1
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http://dx.doi.org/10.1186/s12936-019-2648-7DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6341711PMC
January 2019
3 Reads

Insight into the complex pathophysiology of sickle cell anaemia and possible treatment.

Eur J Haematol 2019 Jan 21. Epub 2019 Jan 21.

Department of Paediatric Haematology, Our Lady's Children's Hospital, Dublin, Ireland.

Sickle cell anaemia (SCA) is the consequence of abnormal haemoglobin production due to an inherited point mutation in the β-globin gene. The resulting haemoglobin tetramer is poorly soluble when deoxygenated, and when this is prolonged, intracellular gelation of sickle haemoglobin occurs, followed by haemoglobin polymerisation. If many cycles of sickling and unsickling occur, the red cell membrane will be disrupted leading to haemolysis and vaso-occlusive events. Read More

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http://dx.doi.org/10.1111/ejh.13212DOI Listing
January 2019
2 Reads
2.066 Impact Factor

"Maximum tolerated dose" vs "fixed low-dose" hydroxyurea for treatment of adults with sickle cell anemia.

Am J Hematol 2019 Jan 21. Epub 2019 Jan 21.

Division of Hematology & Oncology, Department of Medicine, University of Illinois at Chicago, Chicago, Illinois.

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http://dx.doi.org/10.1002/ajh.25412DOI Listing
January 2019
4 Reads

Semisynthetic supra plasma expanders: a new class of therapeutics to improve microcircualtion in sickle cell anaemia.

Artif Cells Nanomed Biotechnol 2019 Dec;47(1):73-82

a Department of Medicine, Albert Einstein College of Medicine , Bronx , NY , USA.

Compromised microcirculation and endothelial dysfunction are hallmarks of sickle cell disease (SCD). EAF PEG Haemoglobin (Hb) and EAF PEG Albumin (Alb) represent a novel class of semisynthetic colloidal supra plasma expanders that improve microcirculation. The therapeutic activity of supra plasma expanders to attenuate vaso-occlusion and restore the haemodynamic functions in patients with SCD has been investigated using NY1DD, a transgenic mouse model of mild SCD without anaemia. Read More

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http://dx.doi.org/10.1080/21691401.2018.1543194DOI Listing
December 2019
1 Read

Advances in Understanding Ischemic Stroke Physiology and the Impact of Vasculopathy in Children With Sickle Cell Disease.

Stroke 2019 Feb;50(2):266-273

Department of Pediatrics and Department of Neurology and Neurotherapeutics, University of Texas Southwestern Medical Center, Dallas (M.M.D.).

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http://dx.doi.org/10.1161/STROKEAHA.118.020482DOI Listing
February 2019
2 Reads

Risk factors for recurrent severe anemia among previously transfused children in Uganda: an age-matched case-control study.

BMC Pediatr 2019 Jan 18;19(1):27. Epub 2019 Jan 18.

Department of Global Child Health, Emma Children's Hospital, Academic Medical Centre, University of Amsterdam, Amsterdam, The Netherlands.

Background: In resource-poor settings, transfused children often experience recurrence of severe anemia (SA) following discharge from hospital. This study determined the factors associated with recurrent severe anemia (RSA) among previously transfused Ugandan children aged less than 5 years.

Methods: A case-control study was conducted in five hospitals in Uganda from March 2017 to September 2018. Read More

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https://bmcpediatr.biomedcentral.com/articles/10.1186/s12887
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http://dx.doi.org/10.1186/s12887-019-1398-6DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6337863PMC
January 2019
8 Reads

Invasive Nontyphoidal Disease in Africa.

EcoSal Plus 2019 01;8(2)

Jenner Institute, Nuffield Department of Medicine, University of Oxford, Oxford, UK.

Nontyphoidal salmonellae (NTS) are a major cause of invasive (iNTS) disease in sub-Saharan Africa, manifesting as bacteremia and meningitis. Available epidemiological data indicate that iNTS disease is endemic in much of the region. Antimicrobial resistance is common and case fatality rates are high. Read More

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http://www.asmscience.org/content/journal/ecosalplus/10.1128
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http://dx.doi.org/10.1128/ecosalplus.ESP-0007-2018DOI Listing
January 2019
6 Reads

A phase 1/2 ascending dose study and open-label extension study of voxelotor in patients with sickle cell disease.

Blood 2019 Jan 17. Epub 2019 Jan 17.

Global Blood Therapeutics, Inc., South San Francisco, CA, United States.

New treatments directly targeting polymerization of sickle hemoglobin (HbS), the proximate event in the pathophysiology of sickle cell disease (SCD), are needed to address the severe morbidity and early mortality associated with the disease. Voxelotor (GBT440) is a first-in-class, oral therapy specifically developed to treat SCD by modulating the affinity of hemoglobin for oxygen, thus inhibiting HbS polymerization and the downstream adverse effects of hemolytic anemia and vaso-occlusion. GBT440-001 was a phase 1/2 randomized, double-blind, placebo-controlled, single and multiple ascending dose study of voxelotor in adult healthy volunteers and patients with SCD which was followed by a single-arm, open-label extension study. Read More

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http://www.bloodjournal.org/lookup/doi/10.1182/blood-2018-08
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http://dx.doi.org/10.1182/blood-2018-08-868893DOI Listing
January 2019
12 Reads

Acquisition of Streptococcus mutans and dental caries experience in pediatric sickle cell anaemia patients under various prophylactic therapies.

Eur Arch Paediatr Dent 2019 Jan 14. Epub 2019 Jan 14.

Department of Oral Medicine and Radiology, Maitri Dental College, Durg, Chhattisgarh, India.

Objectives: To determine the acquisition of Streptococcus mutans and dental caries experience in children with sickle cell anaemia (SCA) under various prophylactic therapies.

Methods: This study involved a total of 200 children who were divided into five groups comprising of 40 children each. Group SP included children who received prophylactic penicillin, Group SV who received pneumococcal vaccination, Group SPV who received both prophylactic penicillin and pneumococcal vaccination, Group SW who did not receive any prophylactic therapy and Group CC who were controls. Read More

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http://dx.doi.org/10.1007/s40368-019-00415-xDOI Listing
January 2019
1 Read

[Lead intoxication revealing sickle cell disease].

Pan Afr Med J 2018 31;30:305. Epub 2018 Aug 31.

Service de Rhumatologie, CHU Mohammed VI, BP 2360 principal, Avenue Ibn Sina, Marrakech, Maroc.

We here report the case of a 18 year old patient with two brothers who died from sickle cell anemia. He had a 5-day history of diffuse bone pain. He had drunk a preparation containing lead to obtain an analgesic effect without improvement. Read More

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http://dx.doi.org/10.11604/pamj.2018.30.305.16222DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6320453PMC
January 2019
1 Read

Falsely low activated clotting time measured in a patient with sickle cell trait.

Perfusion 2019 Jan 10:267659118822945. Epub 2019 Jan 10.

Department of Perfusion, Great Ormond Street Hospital for Children, London, UK.

Sickle cell anaemia results from homozygosity of an A-T point mutation leading to a substitution of glutamic acid for valine at point 6 of the β-globin gene. A person with sickle cell trait inherits one normal and one mutated allele. Patients with sickle cell anaemia, and to a lesser extent sickle cell trait, have a wide spectrum of haemostatic abnormalities. Read More

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http://dx.doi.org/10.1177/0267659118822945DOI Listing
January 2019
4 Reads

Hydroxyurea - An Essential Medicine for Sickle Cell Disease in Africa.

N Engl J Med 2019 01;380(2):187-189

From the Department of Hematology and Sickle Cell Program, Muhimbili University of Health and Allied Sciences, Dar es Salaam, Tanzania.

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http://dx.doi.org/10.1056/NEJMe1814706DOI Listing
January 2019
2 Reads

Atypical β-S haplotypes: classification and genetic modulation in patients with sickle cell anemia.

J Hum Genet 2019 Mar 9;64(3):239-248. Epub 2019 Jan 9.

Department of Biology, Hemoglobin and Hematologic Genetic Diseases Laboratory, Institute of Bioscience, Humanities and Exact Sciences (Ibilce), São Paulo State University (UNESP), Campus Sao Jose do Rio Preto, Sao Paulo, Brazil.

β-S globin haplotype (β haplotype) characterization in sickle cell anemia (SCA) patients is important because it assists individualized treatment. However, the patient with atypical haplotypes do not present detailed studies such as clinical and laboratory data. To understand the phenotypic expression of atypical haplotype patients in relation to typical haplotype ones, it may be necessary to assess the main clinical and laboratorial parameters and investigate transcription factors, as possible genetic modulators that can contribute to the improvement of the SCA patients' clinical condition. Read More

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http://dx.doi.org/10.1038/s10038-018-0554-4DOI Listing
March 2019
1 Read

Novel Sickle Cell Disease Therapies: Targeting Pathways Downstream of Sickling.

Semin Hematol 2018 04 20;55(2):68-75. Epub 2018 Apr 20.

Department of Pediatrics, Division of Pediatric Hematology/Oncology, Albert Einstein College of Medicine, Children's Hospital at Montefiore, Bronx, NY. Electronic address:

Sickle cell disease is an inherited hemoglobinopathy characterized by hemolytic anemia, frequent painful episodes, poor quality of life, end organ damage and a shortened lifespan. Although the seminal event is the polymerization of the abnormal hemoglobin, the downstream pathophysiology of vaso-occlusion results from heterotypic interactions between the altered, adhesive sickle cell RBCs, neutrophils, endothelium, and platelets. Ischemia reperfusion injury, hemolysis and oxidant damage all contribute to heightened inflammation and activation of the hemostatic system. Read More

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https://linkinghub.elsevier.com/retrieve/pii/S00371963173020
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http://dx.doi.org/10.1053/j.seminhematol.2018.04.007DOI Listing
April 2018
15 Reads

Human Molecular Genetics Has Not Yet Contributed to Measurable Public Health Advances.

Perspect Biol Med 2018 ;61(4):537-549

For more than half a century, we have lived in a world dominated by the idea that the gene is the central and primary agent in biology, an era some have called the "Genetic Age." Each decade since the discovery of the double-helical structure of DNA in 1953 has seen scientific advances in genetics, discoveries that have led to at least 17 Nobel Prizes. Although the time span occupied by the Genetic Age has also been a time of great public health advances, no advance in human molecular genetics can be shown to have had any measurable effect on any public health parameter of importance. Read More

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http://dx.doi.org/10.1353/pbm.2018.0063DOI Listing
January 2019
2 Reads

Hydroxyurea can be used in children with sickle cell disease and cerebral vasculopathy for the prevention of chronic complications? A meta-analysis.

J Child Health Care 2019 Jan 3:1367493518814922. Epub 2019 Jan 3.

2 UROGIV Research Group, Universidad del Valle, Cali, Colombia.

We conducted a systematic review for evaluating the impact of hydroxyurea and chronic blood transfusion in children with sickle cell disease (SCD). A search was done in four databases from inception to 2017. Trials enrolling pediatric patients with SCD and cerebral vasculopathy with or without previous episode of stroke and that reported outcomes of occurrence of stroke and other events were included. Read More

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http://dx.doi.org/10.1177/1367493518814922DOI Listing
January 2019
5 Reads

Community perceptions of paediatric severe anaemia in Uganda.

PLoS One 2019 3;14(1):e0209476. Epub 2019 Jan 3.

Department of Global Child Health, Emma Children's Hospital, Academic Medical Centre, University of Amsterdam, Amsterdam, the Netherlands.

Background: Severe anaemia remains a major cause of morbidity and mortality among children in sub-Saharan Africa. There is limited research on the beliefs and knowledge for paediatric severe anaemia in the region. The effect of these local beliefs and knowledge on the healthcare seeking of paediatric severe anaemia remains unknown. Read More

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http://journals.plos.org/plosone/article?id=10.1371/journal.pone.0209476PLOS
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6317806PMC
January 2019
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Risk factors of metabolic syndrome among adult Sudanese sickle cell anemia patients.

BMC Hematol 2018 27;18:38. Epub 2018 Dec 27.

Department of Physiology, Faculty of Medicine, Alneelain University, P.O. Box: 11121, 12702 Khartoum, Sudan.

Background: Sickle cell disease is a hereditary disorder characterized by haematological anaemia. Several studies assumed that adult sickle patients might develop metabolic syndrome features as hyperglycaemia, hypertension and dyslipidaemia. The aim of this study was to evaluate the metabolic syndrome risk factors among adult Sudanese with sickle cell anemia in the steady state. Read More

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http://dx.doi.org/10.1186/s12878-018-0110-7DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6307197PMC
December 2018
3 Reads

Plasmodium falciparum.

Trends Parasitol 2018 Dec 27. Epub 2018 Dec 27.

Center for Advanced Microscopy, The Australian National University, Canberra ACT, Australia.

Plasmodium falciparum is the etiological agent of malaria tropica, the leading cause of death due to a vector-borne infectious disease, claiming 0.5 million lives every year. The single-cell eukaryote undergoes a complex life cycle and is an obligate intracellular parasite of hepatocytes (clinically silent) and erythrocytes (disease causing). Read More

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http://dx.doi.org/10.1016/j.pt.2018.11.010DOI Listing
December 2018
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Hyperfiltration during early childhood precedes albuminuria in pediatric sickle cell nephropathy.

Am J Hematol 2018 Dec 27. Epub 2018 Dec 27.

Division of Pediatric Nephrology, University of Alabama at Birmingham, Birmingham, Alabama.

Background: In patients with diabetes mellitus, hyperfiltration precedes the development of albuminuria. Pediatric sickle cell anemia (SCA) patients have a high prevalence of hyperfiltration and albuminuria during early childhood and adolescence. We tested the hypothesis that hyperfiltration precedes the development of albuminuria in a longitudinal pediatric SCA cohort. Read More

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http://dx.doi.org/10.1002/ajh.25390DOI Listing
December 2018
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Voxelotor treatment of a patient with sickle cell disease and very severe anemia.

Am J Hematol 2018 Dec 27. Epub 2018 Dec 27.

Sickle Cell Branch, National Heart Lung and Blood Institute, National Institutes of Health, Bethesda, Maryland.

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http://dx.doi.org/10.1002/ajh.25389DOI Listing
December 2018
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Temperament in preschool children with sickle cell anaemia.

Arch Dis Child 2018 Dec 21. Epub 2018 Dec 21.

Developmental Neurosciences, UCL Great Ormond Street Institute of Child Health, London, UK.

Aims: Few studies have investigated the potential impact of sickle cell anaemia (SCA) on temperament. The aim of the current study was to investigate temperament in preschool children with SCA and to establish the reliability of the Children's Behaviour Questionnaire (CBQ) in this population.

Methods: The CBQ, a parent-report measure of temperament, was completed by parents of 21 preschool children with SCA and a control group of parents of typically developing children, matched for age, ethnicity and socioeconomic status. Read More

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http://adc.bmj.com/lookup/doi/10.1136/archdischild-2018-3150
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http://dx.doi.org/10.1136/archdischild-2018-315054DOI Listing
December 2018
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The Importance of Demodex Mites (Acari: Demodicidae) in Patients With Sickle Cell Anemia.

J Med Entomol 2018 Dec 21. Epub 2018 Dec 21.

Department of Microbiology and Molecular Genetics, The Kuvin Center for the Study of Infectious and Tropical Diseases, Hadassah Medical School, Hebrew University Jerusalem, Israel.

Although demodicosis caused by Demodex folliculorum and Demodex brevis is widely seen throughout the world, the pathogenic mechanisms are not fully known. To the best of our knowledge, the effect of Demodex mites in patients with sickle cell anemia (SCA) is not known. SCA is a genetic disease characterized by abnormal hemoglobin production and suppression of the immune system. Read More

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https://academic.oup.com/jme/advance-article/doi/10.1093/jme
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http://dx.doi.org/10.1093/jme/tjy225DOI Listing
December 2018
6 Reads