2,660 results match your criteria Pediatrics Sickle Cell Disease


Arteriopathy Influences Pediatric Ischemic Stroke Presentation, but Sickle Cell Disease Influences Stroke Management.

Stroke 2019 May;50(5):1089-1094

Departments of Pediatrics, Neurology and Neurotherapeutics, University of Texas Southwestern Medical Center at Dallas and Children's Health Dallas (M.M.D.).

Background and Purpose- Sickle cell disease (SCD) and arteriopathy are pediatric stroke risk factors that are not mutually exclusive. The relative contributions of sickled red blood cells and arteriopathy to stroke risk are unknown, resulting in unclear guidelines for primary and secondary stroke prevention when both risk factors are present. We hypothesized that despite similarities in clinical presentation and radiographic appearance of arteriopathies, stroke evaluation and management differ in children with SCD compared with those without SCD. Read More

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https://www.ahajournals.org/doi/10.1161/STROKEAHA.118.022800
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http://dx.doi.org/10.1161/STROKEAHA.118.022800DOI Listing
May 2019
1 Read

Health-related quality of life in sickle cell disease.

Nat Rev Dis Primers 2019 Apr 18;5(1):27. Epub 2019 Apr 18.

Division of General Internal Medicine, Department of Medicine, Virginia Commonwealth University, Richmond, VA, USA.

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http://www.nature.com/articles/s41572-019-0080-5
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http://dx.doi.org/10.1038/s41572-019-0080-5DOI Listing
April 2019
2 Reads

The impact of sickle cell anemia on the quality of life of sicklers at school age.

J Family Med Prim Care 2019 Feb;8(2):468-471

Department of Pediatrics, College of Medicine, University of Bahri, Khatoum, Sudan.

Background: Sickle cell anemia (SCA) is prevalent in Sudan in general, and in particular in the western part of the country among Misseria tribe. School absence, depressive symptoms, embarrassment, social disturbances, and emotional disturbance present negative impact for sicklers. Therefore, an urgent call for improving the quality of life of sicklers is needed. Read More

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http://www.jfmpc.com/text.asp?2019/8/2/468/253056
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http://dx.doi.org/10.4103/jfmpc.jfmpc_444_18DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6436282PMC
February 2019
6 Reads

Challenges in preventing and treating hemolytic complications associated with red blood cell transfusion.

Transfus Clin Biol 2019 May 25;26(2):130-134. Epub 2019 Mar 25.

Center for Transfusion Medicine and Cellular Therapies, Department of Laboratory Medicine and Pathology, Emory University School of Medicine, 101, Woodruff Circle, 30322 Atlanta, GA, USA. Electronic address:

Red blood cell (RBC) transfusion support represents a critical component of sickle cell disease (SCD) management. However, as with any therapeutic intervention, RBC transfusion is not without risk. Repeat exposure to allogeneic RBCs can result in the development of RBC alloantibodies that can make it difficult to find compatible RBCs for future transfusions and can directly increase the risk of developing acute or delayed hemolytic transfusion reactions, which can be further complicated by hyperhemolysis. Read More

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http://dx.doi.org/10.1016/j.tracli.2019.03.002DOI Listing
May 2019
1 Read

Psychosocial and affective comorbidities in sickle cell disease.

Neurosci Lett 2019 Apr 6;705:1-6. Epub 2019 Apr 6.

Division of Hematology, Center for Cancer and Blood Disorders, Children's National Medical Center, Department of Pediatrics, George Washington University School of Medicine and Health Sciences, Washington D.C., United States. Electronic address:

Psychosocial and affective comorbidities are common in sickle cell disease (SCD) and can strongly influence disease outcomes, especially those related to pain such as frequency and intensity of pain, use of emergency- and hospital-based care and opioid use. Depression, anxiety, sleep disorders, and substance use challenges are among the common comorbidities that inform the patient experience of SCD. Underlying neurocognitive changes may also contribute to the expression of affective disorders in people with SCD. Read More

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http://dx.doi.org/10.1016/j.neulet.2019.04.011DOI Listing
April 2019
2 Reads

Microcytosis in children and adolescents with the sickle cell trait in Basra, Iraq.

Blood Res 2019 Mar 21;54(1):38-44. Epub 2019 Mar 21.

Center for Hereditary Blood Diseases, Basrah Heath Directorate, Basrah, Iraq.

Background: Microcytic anemia, the most common form of anemia in children and adolescents, is a heterogeneous group of diseases that is acquired or inherited. We assessed the frequency and causes of microcytosis in children and adolescents with the sickle cell trait (SCT).

Methods: This descriptive study included 95 subjects (49 males and 46 females) with SCT who attended Basra Center for Hereditary Blood Diseases for evaluation. Read More

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http://dx.doi.org/10.5045/br.2019.54.1.38DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6439302PMC
March 2019
1 Read

Biochemical effects and safety of Gum arabic () supplementation in patients with sickle cell anemia.

Blood Res 2019 Mar 21;54(1):31-37. Epub 2019 Mar 21.

Department of Physiology, Faculty of Medicine, University of Khartoum, Khartoum, Sudan.

Background: Sickle cell anemia (SCA) is a hereditary chronic hemolytic anemia with several clinical consequences. Intravascular sickling of red blood cells leads to multi-organ dysfunction. Moreover, several biochemical abnormalities have been associated with SCA. Read More

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http://dx.doi.org/10.5045/br.2019.54.1.31DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6439290PMC
March 2019
4 Reads

Stroke Recurrence in Nigerian Children With Sickle Cell Disease: Evidence for a Secondary Stroke Prevention Trial.

Pediatr Neurol 2019 Jan 17. Epub 2019 Jan 17.

Department of Hematology and Blood Transfusion, Bayero University/Aminu Kano Teaching Hospital, Kano, Nigeria. Electronic address:

Background: To improve the quality of care for children with sickle cell anemia in Kano, Nigeria, we initiated a standard care protocol in 2014 to manage children with strokes at Aminu Kano Teaching Hospital.

Methods: The standard care protocol requires that children with acute strokes be treated with hydroxyurea at a fixed dose of 20 mg/kg/day within two months of the stroke.

Results: Twenty-nine children with sickle cell anemia and initial stroke were identified based on clinical World Health Organization criteria from 2014 to 2017. Read More

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https://linkinghub.elsevier.com/retrieve/pii/S08878994183089
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http://dx.doi.org/10.1016/j.pediatrneurol.2019.01.008DOI Listing
January 2019
4 Reads

Effect of Sickle Cell Anemia Therapies on the Natural History of Growth and Puberty Patterns.

J Pediatr Hematol Oncol 2019 Apr 3. Epub 2019 Apr 3.

Department of Pediatrics, University of Alabama at Birmingham, Alabama.

As pediatric patients with sickle cell anemia (SCA) have impaired growth and puberty patterns, we studied the effect of disease-modifying therapies on growth and puberty patterns for patients with SCA receiving hydroxyurea (HU), transfusions, or no therapy. We performed a retrospective study of children with SCA in whom anthropometric measurements and therapy type were recorded. Penalized smoothing splines were fitted to estimate growth curves and growth velocity, and linear mixed models were used to examine differences across treatment groups. Read More

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http://dx.doi.org/10.1097/MPH.0000000000001477DOI Listing
April 2019
3 Reads

Kidney biopsy findings in children with sickle cell disease: a Midwest Pediatric Nephrology Consortium study.

Pediatr Nephrol 2019 Apr 3. Epub 2019 Apr 3.

Department of Pediatrics, Division of Nephrology, Emory University School of Medicine and Children's Healthcare of Atlanta, Atlanta, GA, USA.

Background: Renal damage is a progressive complication of sickle cell disease (SCD). Microalbuminuria is common in children with SCD, while a smaller number of children have more severe renal manifestations necessitating kidney biopsy. There is limited information on renal biopsy findings in children with SCD and subsequent management and outcome. Read More

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http://dx.doi.org/10.1007/s00467-019-04237-3DOI Listing
April 2019
3 Reads

Improving Care for Sickle Cell Pain Crisis Using a Multidisciplinary Approach.

Pediatrics 2019 Apr 3. Epub 2019 Apr 3.

Department of Pediatrics, Yale University School of Medicine, New Haven, Connecticut; and

Objectives: Frequent hospitalizations for sickle cell disease (SCD) vaso-occlusive crises (VOCs) are associated with school absenteeism, emotional distress, and financial hardships. Our goal was to decrease hospital days for VOC admissions by 40% over a 5-year period.

Methods: From October 2011 to September 2016, a multidisciplinary quality-improvement project was conducted with a plan-do-study-act methodology. Read More

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http://dx.doi.org/10.1542/peds.2018-2218DOI Listing
April 2019
2 Reads

Greater number of perceived barriers to hydroxyurea associated with poorer health-related quality of life in youth with sickle cell disease.

Pediatr Blood Cancer 2019 Apr 2:e27740. Epub 2019 Apr 2.

Department of Pediatrics, Columbia University Medical Center, New York, New York.

Background: Despite medical benefits, hydroxyurea adherence in adolescents is often poor. As part of a baseline assessment of 28 youth (10-18 years) parent dyads who participated in a 6-month feasibility trial to improve hydroxyurea adherence, we measured the relationship between greater barriers to adherence and health-related quality of life (HRQL) from youth and parent perspectives.

Procedure: Barriers were measured using the Adolescent and Parent Medication Barriers Scales with nine hydroxyurea items added. Read More

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http://dx.doi.org/10.1002/pbc.27740DOI Listing
April 2019
2 Reads

Health literacy and knowledge of chronic transfusion therapy in adolescents with sickle cell disease and caregivers.

Pediatr Blood Cancer 2019 Apr 2:e27733. Epub 2019 Apr 2.

Colorado School of Public Health, Anschutz Medical Campus, University of Colorado, Aurora, Colorado.

Background: Patients with sickle cell disease (SCD) may require chronic transfusion therapy (CTT) for prevention of stroke or other complications. Limited health literacy (HL) is common and is associated with poor health-related knowledge and outcomes in chronic disease. We sought to assess HL and transfusion knowledge in patients with SCD on CTT and their caregivers. Read More

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http://dx.doi.org/10.1002/pbc.27733DOI Listing
April 2019
4 Reads

Mycobacterium Avium Complex Presenting as Pulmonary Nodules in a Child With Sickle Cell Disease.

J Pediatr Hematol Oncol 2019 Mar 29. Epub 2019 Mar 29.

Departments of Pediatrics, Texas A&M Health Science Center, Scott & White Medical Center-Temple, Temple, TX.

The case of a 10-year-old child with sickle cell disease with pulmonary nodules and prolonged fevers is reported here. The child was first diagnosed with sarcoidosis based on lung biopsy, but unresponsiveness to therapy led to a second lung biopsy, which revealed the true diagnosis of mycobacterium avium complex disease. Multiple possible explanations for why the patient became infected exist. Read More

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http://dx.doi.org/10.1097/MPH.0000000000001458DOI Listing
March 2019
1 Read

Quantitative sensory testing in children with sickle cell disease: additional insights and future possibilities.

Br J Haematol 2019 Mar 28. Epub 2019 Mar 28.

Nemours Center for Cancer and Blood Disorders, Nemours/Alfred I duPont Hospital for Children, Wilmington, DE, USA.

Quantitative sensory testing (QST) is used in a variety of pain disorders to characterize pain and predict prognosis and response to specific therapies. In this study, we aimed to confirm results in the literature documenting altered QST thresholds in sickle cell disease (SCD) and assess the test-retest reliability of results over time. Fifty-seven SCD and 60 control subjects aged 8-20 years underwent heat and cold detection and pain threshold testing using a Medoc TSAII. Read More

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http://dx.doi.org/10.1111/bjh.15876DOI Listing
March 2019
1 Read

Plasma histamine elevation in a large cohort of sickle cell disease patients.

Br J Haematol 2019 Mar 28. Epub 2019 Mar 28.

Department of Internal Medicine, Tenon Hospital, AP-HP, Paris VI University, Paris, France.

The role of mast cells has been questioned in sickle cell disease (SCD). We performed a prospective study evaluating plasma histamine and tryptase levels in a cohort of paediatric and adult patients, in steady state (n = 132) and during vaso-occlusive crisis (VOC) (n = 121). Histamine level was elevated in 18% of patients in steady state and in 61% during VOC. Read More

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http://dx.doi.org/10.1111/bjh.15900DOI Listing
March 2019
2 Reads

Altered parasite life-cycle processes characterize Babesia divergens infection in human sickle cell anemia.

Haematologica 2019 Mar 28. Epub 2019 Mar 28.

Department of Blood-Borne Parasites, New York Blood Center, New York, USA;

Babesia divergens is an intra-erythrocytic parasite that causes malaria-like symptoms in infected people. As the erythrocyte provides the parasite with the infra-structure to grow and multiply, any perturbation to the cell should impact parasite viability. Support for this comes from the multitude of studies that have shown that the sickle trait has in fact been selected for because of the protection in provides against a related Apicomplexan parasite, Plasmodium that causes malaria. Read More

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http://dx.doi.org/10.3324/haematol.2018.214304DOI Listing
March 2019
1 Read

50 Years Ago in The Journal of Pediatrics: The Hemolytic Crisis of Sickle Cell Disease: The Role of Glucose-6-Phosphate Dehydrogenase Deficiency.

J Pediatr 2019 Apr;207:204

Department of Pediatrics Tuba City Regional Health Care Corporation Tuba City, Arizona.

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http://dx.doi.org/10.1016/j.jpeds.2018.10.032DOI Listing
April 2019
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Clinical Practice Guideline: Tonsillectomy in Children (Update)-Executive Summary.

Otolaryngol Head Neck Surg 2019 Feb;160(2):187-205

15 Department of Research and Quality, American Academy of Otolaryngology-Head and Neck Surgery Foundation, Alexandria, Virginia, USA.

Objective: This update of a 2011 guideline developed by the American Academy of Otolaryngology-Head and Neck Surgery Foundation provides evidence-based recommendations on the pre-, intra-, and postoperative care and management of children 1 to 18 years of age under consideration for tonsillectomy. Tonsillectomy is defined as a surgical procedure performed with or without adenoidectomy that completely removes the tonsil, including its capsule, by dissecting the peritonsillar space between the tonsil capsule and the muscular wall. Tonsillectomy is one of the most common surgical procedures in the United States, with 289,000 ambulatory procedures performed annually in children <15 years of age, based on the most recent published data. Read More

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http://dx.doi.org/10.1177/0194599818807917DOI Listing
February 2019
2 Reads

Normal Saline Bolus Use in Pediatric Emergency Departments is Associated with Worse Pain Control in Children with Sickle Cell Anemia and Vaso-occlusive Pain.

Am J Hematol 2019 Mar 27. Epub 2019 Mar 27.

Department of Pediatrics, Emory University School of Medicine, Atlanta, GA.

Vaso-occlusive pain events (VOE) are the leading cause of emergency department (ED) visits in sickle cell anemia (SCA). This study assessed the variability in use of intravenous fluids (IVFs) and the association of normal saline bolus (NSB) on pain and other clinical outcomes in children with SCA presenting to pediatric emergency departments (PED) with VOE. Four-hundred charts of children age 3-21 years with SCA/VOE receiving parenteral opioids at 20 high-volume PEDs were evaluated in a retrospective study. Read More

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http://dx.doi.org/10.1002/ajh.25471DOI Listing
March 2019
3.798 Impact Factor

Highly efficient therapeutic gene editing of human hematopoietic stem cells.

Nat Med 2019 Mar 25. Epub 2019 Mar 25.

Division of Hematology/Oncology, Boston Children's Hospital , Boston, MA, USA.

Re-expression of the paralogous γ-globin genes (HBG1/2) could be a universal strategy to ameliorate the severe β-globin disorders sickle cell disease (SCD) and β-thalassemia by induction of fetal hemoglobin (HbF, αγ). Previously, we and others have shown that core sequences at the BCL11A erythroid enhancer are required for repression of HbF in adult-stage erythroid cells but are dispensable in non-erythroid cells. CRISPR-Cas9-mediated gene modification has demonstrated variable efficiency, specificity, and persistence in hematopoietic stem cells (HSCs). Read More

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http://dx.doi.org/10.1038/s41591-019-0401-yDOI Listing
March 2019
3 Reads
27.363 Impact Factor

Delayed hemolytic transfusion reaction in the French hemovigilance system.

Transfus Clin Biol 2019 May 9;26(2):109-111. Epub 2019 Mar 9.

Agence nationale de sécurité du médicament et des produits de santé (ANSM), 93285 Saint-Denis cedex, France.

In France, reporting of adverse events related, or likely to be related, to transfusion is mandatory. Since its creation in 1993, the French hemovigilance system has contributed to a better recognition of unappreciated risks like delayed hemolytic transfusion reactions (DHTR) in sickle-cell disease (SCD) patients. Long under-reported or misclassified, reports of this serious complication of transfusion have improved, particularly through the dissemination of information within the hemovigilance network. Read More

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http://dx.doi.org/10.1016/j.tracli.2019.03.001DOI Listing
May 2019
2 Reads

A Toll-like receptor 2 genetic variant modulates occurrence of bacterial infections in patients with sickle cell disease.

Br J Haematol 2019 Mar 25. Epub 2019 Mar 25.

INSERM U955, Centre Hospitalier Universitaire Henri Mondor, Créteil, France.

Despite adequate immunization and penicillin prophylaxis, bacterial infections remain a leading cause of morbidity and mortality in patients with sickle cell disease (SCD). Besides hyposplenism, inflammatory and genetic factors might modulate their susceptibility to bacterial infections. We performed a candidate gene association of single nucleotide polymorphisms (SNPs) located in Toll-like receptor (TLR) genes, encoding prominent molecules for innate immune responses, with the occurrence of bacterial infections in patients with SCD. Read More

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http://dx.doi.org/10.1111/bjh.15875DOI Listing
March 2019
1 Read

Implementation of an educational intervention to optimize self-management and transition readiness in young adults with sickle cell disease.

Pediatr Blood Cancer 2019 Mar 25:e27722. Epub 2019 Mar 25.

Division of Hematology Oncology, Department of Pediatrics, Washington University School of Medicine, St. Louis, Missouri.

Background: The transition from the pediatric setting to adult care is a well-described period of morbidity and mortality for persons with sickle cell disease (SCD). We sought to measure the feasibility and effectiveness of providing skill-based educational handouts on improving self-management and transition readiness in adolescents with SCD.

Methods: This was a single-center study in which participants completed a self-assessment, the Adolescent Autonomy Checklist (AAC), to assess transition readiness and self-management skills at baseline. Read More

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http://dx.doi.org/10.1002/pbc.27722DOI Listing
March 2019
2 Reads

Improving Pain Management in Patients with Sickle Cell Disease from Physiological Measures Using Machine Learning Techniques.

Smart Health (Amst) 2018 Jun 2;7-8:48-59. Epub 2018 Feb 2.

Division of Hematology, Department of Medicine, Duke University, NC 27710, USA.

Pain management is a crucial part in Sickle Cell Disease treatment. Accurate pain assessment is the first stage in pain management. However, pain is a subjective response and hard to assess via objective approaches. Read More

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http://dx.doi.org/10.1016/j.smhl.2018.01.002DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6428053PMC
June 2018
1 Read

Hydroxyurea use among children with sickle cell anemia.

Pediatr Blood Cancer 2019 Jun 22;66(6):e27721. Epub 2019 Mar 22.

Susan B Meister Child Health Evaluation and Research Center, Department of Pediatrics, University of Michigan, Ann Arbor, Michigan.

This study describes hydroxyurea use among children ages 1 to 17 with sickle cell anemia (SCA) enrolled in at least one year of Medicaid in six states from 2005 to 2012. Administrative claims were used to summarize the number of days' supply of hydroxyurea dispensed by state and year. A total of 7963 children with SCA contributed 22 424 person-years. Read More

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http://dx.doi.org/10.1002/pbc.27721DOI Listing

Candida albicans induced acute chest syndrome in sickle cell disease.

Pediatr Blood Cancer 2019 Jun 22;66(6):e27727. Epub 2019 Mar 22.

Department of Pediatrics, University of New Mexico Health Sciences Center, Albuquerque, New Mexico, USA.

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http://dx.doi.org/10.1002/pbc.27727DOI Listing
June 2019
3 Reads

Vascular TSP1-CD47 Signaling Promotes Sickle Cell-Associated Arterial Vasculopathy and Pulmonary Hypertension in Mice.

Am J Physiol Lung Cell Mol Physiol 2019 Mar 20. Epub 2019 Mar 20.

Medicine, Vascular Medicine Institute, University of Pittsburgh, United States.

Pulmonary hypertension (PH) is a leading cause of death in sickle cell disease (SCD). Hemolysis and oxidative stress are contributing factors to SCD-associated PH. We have reported that the protein thrombospondin-1 (TSP1) is elevated in the plasma of patients with SCD and by interacting with its receptor CD47 limits vasodilation of distal pulmonary arteries ex vivo. Read More

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http://dx.doi.org/10.1152/ajplung.00302.2018DOI Listing
March 2019
2 Reads

MIR29B mediates epigenetic mechanisms of HBG gene activation.

Br J Haematol 2019 Mar 19. Epub 2019 Mar 19.

Department of Pediatrics, Division of Hematology/Oncology, Augusta University, Augusta, GA, USA.

Sickle cell disease (SCD) affects over 2 million people worldwide with high morbidity and mortality in underdeveloped countries. Therapeutic interventions aimed at reactivating fetal haemoglobin (HbF) is an effective approach for improving survival and ameliorating the clinical severity of SCD. A class of agents that inhibit DNA methyltransferase (DNMT) activity show promise as HbF inducers because off-target effects are not observed at low concentrations. Read More

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http://dx.doi.org/10.1111/bjh.15870DOI Listing
March 2019
5 Reads

Asthma in children with sickle cell disease.

Curr Opin Pediatr 2019 Mar 14. Epub 2019 Mar 14.

Division of Hematology/Oncology, Department of Pediatrics, Vanderbilt-Meharry Center for Excellence in Sickle Cell Disease, Vanderbilt University Medical Center, Nashville, Tennessee, USA.

Purpose Of Review: Asthma is common in children with sickle cell disease (SCD) and appears to be associated with increased morbidity. Providers caring for children with SCD have struggled with the question of whether asthma exists as a true comorbidity or whether certain aspects of the chronic inflammatory disease gives children with SCD an asthma-like phenotype.

Recent Findings: Clinical signs and symptoms seen in children with asthma in the general population, such as wheezing, airway hyperresponsiveness, atopy, elevated leukotrienes, and abnormal lung function are seen in children with SCD both with and without a diagnosis of asthma. Read More

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http://dx.doi.org/10.1097/MOP.0000000000000756DOI Listing
March 2019
4 Reads

Unprepared and Misinformed Parents of Children with Sickle Cell Disease: Time to Rethink Awareness Campaigns.

Cureus 2018 Dec 31;10(12):e3806. Epub 2018 Dec 31.

Cardiothoracic Surgery, Lagos State University Teaching Hospital, Lagos, NGA.

Worldwide, sickle cell disease (SCD) poses a significant public health concern. It causes recurrent morbidity, and premature death is a distinct possibility, especially in Nigeria, which bears half the world's burden of SCD patients. Nigeria has yet to establish a newborn screening program; consequently, most affected children are diagnosed between one and three years of age when a health problem arises. Read More

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http://dx.doi.org/10.7759/cureus.3806DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6402737PMC
December 2018
3 Reads

Challenges in the treatment and prevention of delayed hemolytic transfusion reactions with hyperhemolysis in sickle cell disease patients.

Transfusion 2019 Mar 8. Epub 2019 Mar 8.

Center for Transfusion and Cellular Therapy, Department of Pathology and Laboratory Medicine, Emory University School of Medicine, Atlanta, Georgia.

Background: Delayed hemolytic transfusion reactions (DHTRs) are serious complications of RBC transfusion that can occur in previously alloimmunized patients. Patients who require episodic transfusions during heightened inflammatory states, such as patients with sickle cell disease (SCD), are particularly prone to alloimmunization and developing DHTRs with hyperhemolysis. While efforts to mitigate these hemolytic episodes via immunosuppressive drugs can be employed, the relative efficacy of various treatment options remains incompletely understood. Read More

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http://dx.doi.org/10.1111/trf.15227DOI Listing
March 2019
3 Reads

Abnormal submaximal cardiopulmonary exercise parameters predict impaired peak exercise performance in sickle cell anemia patients.

Pediatr Blood Cancer 2019 Jun 7;66(6):e27703. Epub 2019 Mar 7.

Division of Cardiology, Cincinnati Children's Hospital Medical Center, Cincinnati, Ohio.

Purpose: Sickle cell anemia (SCA) patients frequently have many comorbidities, including diastolic dysfunction (DD) and exercise intolerance. SCA patients often cannot reach maximal effort on exercise testing; little is known regarding whether submaximal exercise parameters can predict abnormal maximal exercise results in SCA patients and if there are any possible associations with DD.

Methods: A prospective longitudinal study was performed in SCA patients. Read More

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http://dx.doi.org/10.1002/pbc.27703DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6472963PMC
June 2019
1 Read
2.562 Impact Factor

Spontaneous healing of avascular necrosis of the femoral head in sickle cell disease.

Am J Hematol 2019 Mar 5. Epub 2019 Mar 5.

Division of Hematology/Oncology, Department of Pediatrics, Baylor College of Medicine, Houston, Texas.

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http://dx.doi.org/10.1002/ajh.25453DOI Listing
March 2019
3 Reads

Clinical presentation of delayed hemolytic transfusion reactions and hyperhemolysis in sickle cell disease.

Transfus Clin Biol 2019 May 22;26(2):94-98. Epub 2019 Feb 22.

Center for Transfusion and Cellular Therapies, Department of Pathology and Laboratory Medicine, Emory University School of Medicine, Atlanta, USA.

Red blood cell (RBC) transfusion therapy is a key component in the comprehensive management of patients with sickle cell disease (SCD). Consequently, most adult SCD patients will receive at least one, and many will receive more than a hundred RBC transfusions in their lifetime. SCD patients develop RBC alloantibodies much more frequently than non-SCD transfused patients, which often make the selection of compatible RBCs extremely difficult, in addition to placing patients at significantly higher risk of suffering from delayed hemolytic transfusion reactions (DHTRs). Read More

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http://dx.doi.org/10.1016/j.tracli.2019.02.002DOI Listing
May 2019
2 Reads

Prevalence and factors associated with renal dysfunction in children admitted to two hospitals in northwestern Tanzania.

BMC Nephrol 2019 Mar 5;20(1):79. Epub 2019 Mar 5.

Department of Pediatrics and Child Health, Bugando Medical Centre, P. O. Box 1370, Mwanza, Tanzania.

Background: It is evident that renal dysfunction (RD) is associated with unique infectious and non-infectious causes in African children. However, little data exists about the prevalence and factors associated with RD in children admitted to African hospitals.

Methods: In this cross-sectional study, we enrolled all children admitted to pediatric wards of Bugando Medical Centre (BMC) and Sekou-Toure Regional Referral hospital (SRRH) during a 6 month time period. Read More

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http://dx.doi.org/10.1186/s12882-019-1254-9DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6402082PMC
March 2019
10 Reads
1.520 Impact Factor

Readiness for transition and health-care satisfaction in adolescents with complex medical conditions.

Child Care Health Dev 2019 May;45(3):463-471

Department of Pediatrics, Emory University School of Medicine, Atlanta, Georgia.

Background: The purpose of this study is to examine contributions to patient perceptions of transition readiness and satisfaction with care amongst adolescents and young adults (AYAs) with complex health conditions engaging in paediatric care.

Methods: Participants included 94 patients aged 14-20 years (M = 16.41, SD = 1. Read More

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http://doi.wiley.com/10.1111/cch.12656
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http://dx.doi.org/10.1111/cch.12656DOI Listing
May 2019
9 Reads

Lessons Learned from Building a Pediatric-to-Adult Sickle Cell Transition Program.

South Med J 2019 Mar;112(3):190-197

From the Department of Internal Medicine, Division of General Internal Medicine, Department of Pediatrics, Division of Pediatric Hematology/Oncology and Department of Psychology, Virginia Commonwealth University, Richmond, and Department of Hematology/Oncology, University of California, San Francisco Benioff Children's Hospital, San Francisco.

Objective: More effective transitions and transfers of young people with sickle cell disease (SCD) into the adult healthcare setting is a focus of both primary care and specialty care medical organizations. Effective transition and transfer requires six core elements: establishing a policy, tracking progress, administering transition readiness assessments, planning for adult care, transferring to adult care, and integrating into an adult practice. We developed a program using these six core elements. Read More

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http://dx.doi.org/10.14423/SMJ.0000000000000950DOI Listing
March 2019
4 Reads

Transition to adulthood and adult health care for patients with sickle cell disease or cystic fibrosis: Current practices and research priorities.

J Clin Transl Sci 2018 Oct;2(5):334-342

Department of Pediatrics, Northwestern University Feinberg School of Medicine, Chicago, IL, USA.

Introduction: A growing population of adults living with severe, chronic childhood-onset health conditions has created a need for specialized health care delivered by providers who have expertise both in adult medicine and in those conditions. Optimal care of these patients requires systematic approaches to healthcare transition (HCT). Guidelines for HCT exist, but gaps in care occur, and there are limited data on outcomes of HCT processes. Read More

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http://dx.doi.org/10.1017/cts.2018.338DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6390387PMC
October 2018
3 Reads

Developing a problem-solving intervention to improve self-management and transition readiness in adolescents with sickle cell disease.

J Pediatr Nurs 2019 Feb 28;46:26-32. Epub 2019 Feb 28.

Department of Pediatrics, Boston University Medical Center, Boston, MA, United States of America. Electronic address:

Purpose: Adolescents and young adults (AYA) with sickle cell disease (SCD) are at risk for serious complications including increased morbidity and early mortality during their transition from pediatric to adult care. Self-management support may help improve transition outcomes in this vulnerable population. Interventions based on teaching problem solving skills have been shown to improve adherence to therapy for AYA with other chronic disease and is a promising intervention in SCD. Read More

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http://dx.doi.org/10.1016/j.pedn.2019.02.006DOI Listing
February 2019
1 Read

Premarital Screening and Genetic Counseling Program: Studies from an Endogamous Population.

Int J Appl Basic Med Res 2019 Jan-Mar;9(1):20-26

Division of Cancer and Genetics, Institute of Medical Genetics Building, School of Medicine, Cardiff University, Heath Park Cardiff CF14 4XN Cardiff, UK.

Background: Studies in Arab countries have shown a significant lack of knowledge of Premarital Screening and Genetic Counseling (PMSGC) Program. PMSGC can identify and modify, through prevention and management, some behavioral, medical, and other health risk factors known to impact pregnancy outcomes.

Objective: The aim of this study was to explore the knowledge, attitudes, and practice of Qatari's toward the premarital screening program and shedding more light on a complex matter. Read More

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http://dx.doi.org/10.4103/ijabmr.IJABMR_42_18DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6385533PMC
March 2019
6 Reads

Early Predictors of Renal Dysfunction in Pediatric Patients with Sickle Cell Disease.

Indian J Nephrol 2019 Jan-Feb;29(1):28-33

Department of Medical Microbiology and Immunology, Faculty of Medicine, Tanta University, Egypt.

Sickle cell disease (SCD) is a hereditary hemoglobinopathy characterized by abnormal hemoglobin production which leads to hemolytic anemia and intermittent occlusion of small blood vessels, which further leads to tissue ischemia, chronic organ damage, and organ dysfunction including urinary system. To measure the serum levels of cystatin-C and beta 2 microglobulin in pediatric patients with SCDand to investigate their significance as early biomarkers of glomerular and/or renal tubular dysfunction. This study was conducted among 70 children with SCD and 40 age and sex-matched children as a control group. Read More

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http://dx.doi.org/10.4103/ijn.IJN_40_18DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6375016PMC
March 2019
1 Read

Clinical Practice Guideline: Tonsillectomy in Children (Update).

Otolaryngol Head Neck Surg 2019 Feb;160(1_suppl):S1-S42

15 Department of Research and Quality, American Academy of Otolaryngology-Head and Neck Surgery Foundation, Alexandria, Virginia, USA.

Objective: This update of a 2011 guideline developed by the American Academy of Otolaryngology-Head and Neck Surgery Foundation provides evidence-based recommendations on the pre-, intra-, and postoperative care and management of children 1 to 18 years of age under consideration for tonsillectomy. Tonsillectomy is defined as a surgical procedure performed with or without adenoidectomy that completely removes the tonsil, including its capsule, by dissecting the peritonsillar space between the tonsil capsule and the muscular wall. Tonsillectomy is one of the most common surgical procedures in the United States, with 289,000 ambulatory procedures performed annually in children <15 years of age based on the most recent published data. Read More

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http://dx.doi.org/10.1177/0194599818801757DOI Listing
February 2019
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Oral morphine protocol evaluation for the treatment of vaso-occlusive crisis in paediatric sickle cell patients.

Paediatr Child Health 2019 Feb 31;24(1):e45-e50. Epub 2018 May 31.

Division of Pediatric Emergency Medicine, CHU Ste-Justine, Montréal, Quebec.

Background: Vaso-occlusive crisis (VOC) is one of the most frequent causes of emergency visit and admission in children with sickle cell disease (SCD).

Objectives: This study aimed to evaluate whether the implementation of a protocol promoting the use of oral morphine as a primary intervention has led to improved care of SCD.

Methods: We performed a retrospective chart review of patients with SCD who presented to the emergency department (ED) and hematology outpatient clinic (HOC) with VOC, in the year pre and postimplementation of the protocol. Read More

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http://dx.doi.org/10.1093/pch/pxy074DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6376293PMC
February 2019
3 Reads

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
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"A complex interface: Exploring sickle cell disease from a parent's perspective, after moving from Sub-Saharan Africa to North America".

Pediatr Hematol Oncol 2018 Oct - Nov;35(7-8):373-384. Epub 2019 Feb 20.

b Stollery Children's Hospital , Alberta Health Services , Edmonton , AB , Canada.

Introduction: Sickle cell disease (SCD) is an inherited, multi-system, chronic disease with the highest prevalence affecting people of Sub-Saharan African descent. While major advances in SCD care have occurred over the last few decades in many African countries these advances are not readily available. Prior literature from Ghana and Kenya describe stigma, despair, and economic burden as well as hope when a child has SCD. Read More

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http://dx.doi.org/10.1080/08880018.2018.1541949DOI Listing
April 2019
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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
2 Reads
2.646 Impact Factor

Nonmyeloablative Matched Sibling Donor Hematopoietic Cell Transplantation in Children and Adolescents with Sickle Cell Disease.

Biol Blood Marrow Transplant 2019 Feb 14. Epub 2019 Feb 14.

Section of Oncology/BMT, Departments of Oncology and Pediatrics, Alberta Children's Hospital, University of Calgary, Calgary, Canada.

Sickle cell disease is a potentially debilitating hemoglobinopathy associated with early mortality. The only established curative therapy is hematopoietic cell transplantation (HCT) with a matched sibling donor. The National Institutes of Health nonmyeloablative regimen of alemtuzumab/300 cGy total body irradiation and prolonged sirolimus exposure for graft-versus-host disease (GVHD) prophylaxis was administered to 16 children and adolescents. Read More

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http://dx.doi.org/10.1016/j.bbmt.2019.02.011DOI Listing
February 2019
4 Reads