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https://www.readbyqxmd.com/read/29431623/prevalence-and-risk-factors-for-venous-thromboembolism-in-children-with-sickle-cell-disease-an-administrative-database-study
#1
Riten Kumar, Joseph Stanek, Susan Creary, Amy Dunn, Sarah H O'Brien
A hypercoagulable state resulting in increased venous thromboembolism (VTE) has been described in adults with sickle cell disease (SCD), but similar data for children are lacking. The objective of this retrospective cohort study was to describe the rate of VTE and risk factors associated with VTE in children with SCD across tertiary-care children's hospitals in the United States between the years 2009 and 2015. We used the Pediatric Health Information System database to investigate all pediatric patients with SCD admitted to 1 of 48 participating institutions between 1 January 2009 and 30 September 2015...
February 13, 2018: Blood Advances
https://www.readbyqxmd.com/read/29415213/risk-of-exertional-heat-illnesses-associated-with-sickle-cell-trait-in-u-s-military
#2
Darrell E Singer, Celia Byrne, Ligong Chen, Stephanie Shao, Jonathan Goldsmith, David W Niebuhr
Introduction: A number of studies have found an association between sickle cell trait (SCT) and exertional heat illnesses (EHIs) including heat stroke, a potentially fatal condition. The strength of this association varied across studies, limiting the ability to quantify potential benefits of SCT-screening policies for competitive athletics and military service members. We determined the relative rate and attributable risk of developing EHI associated with being SCT positive and the EHI health care utilization...
February 5, 2018: Military Medicine
https://www.readbyqxmd.com/read/29411418/feasibility-trial-for-primary-stroke-prevention-in-children-with-sickle-cell-anemia-in-nigeria-spin-trial
#3
Najibah A Galadanci, Shehu Umar Abdullahi, Leah D Vance, Abdulkadir Musa Tabari, Shehi Ali, Raymond Belonwu, Auwal Salihu, Aisha Amal Galadanci, Binta Wudil Jibir, Halima Bello-Manga, Kathleen Neville, Fenella J Kirkham, Yu Shyr, Sharon Phillips, Brittany V Covert, Adetola A Kassim, Lori C Jordan, Muktar H Aliyu, Michael R DeBaun
No abstract text is available yet for this article.
March 2018: American Journal of Hematology
https://www.readbyqxmd.com/read/29405840/changes-in-transcranial-ultrasound-velocities-in-children-with-sickle-cell-disease-undergoing-adenotonsillectomy
#4
Griffin Santarelli, Sarah C DeShields, Stacey L Ishman, Michael Randall, Tina D Cunningham, Cristina M Baldassari
Objectives (1) To assess for changes in cerebral blood flow velocity in children with sickle cell disease and obstructive sleep apnea (OSA) following adenotonsillectomy. (2) To determine if clinical factors such as OSA severity affect cerebral blood flow velocity values. Study Design Case series with chart review over 10 years. Settings Two tertiary children's hospitals. Subjects and Methods Children aged 2 to 18 years with a history of sickle cell disease and OSA, as defined by an apnea hypopnea index (AHI) >1 on polysomnography, were eligible for inclusion...
January 1, 2018: Otolaryngology—Head and Neck Surgery
https://www.readbyqxmd.com/read/29401500/mobile-direct-observation-of-therapy-mdot-a-rapid-systematic-review-and-pilot-study-in-children-with-asthma
#5
Michael D Shields, Fahad ALQahtani, Michael P Rivey, James C McElnay
We describe, for the first time, the use of a mobile device platform for remote direct observation of inhaler use and technique. The research programme commenced with a rapid systematic review of mobile device (or videophone) use for direct observation of therapy (MDOT). Ten studies (mainly pilots) were identified involving patients with tuberculosis, sickle cell disease and Alzheimer's disease. New studies are ongoing (ClinicalTrials.gov website) in TB, stroke, sickle cell disease, HIV and opioid dependence...
2018: PloS One
https://www.readbyqxmd.com/read/29398806/lower-transcranial-doppler-flow-velocities-in-sickle-cell-anemia-patients-on-hydroxyurea-myth-or-fact
#6
Sawsan M Moeen, Ahmad F Thabet, Hosam A Hasan, Medhat A Saleh
Transcranial Doppler (TCD) detects stroke risk in patients with sickle cell anemia (SCA). Hydroxyurea therapy has the ability to induce increased levels of fetal hemoglobin in sickle cells thus decreasing tendency for red cell sickling. This study aimed to evaluate TCD findings in SCA patients on hydroxyurea and correlate the time-averaged mean velocity (TAMV) with their hematological parameters. Forty SCA patients of both sexes, aged 16-22 years with no history of stroke were screened with TCD for an elevated TAMV, divided into: Group T (20 patients on blood transfusion); and Group H (20 patients on daily hydroxyurea)...
January 2018: Indian Journal of Hematology & Blood Transfusion
https://www.readbyqxmd.com/read/29377071/how-i-manage-red-cell-transfusions-in-patients-with-sickle-cell-disease
#7
David C Rees, Susan Robinson, Jo Howard
Sickle cell disease is one of the commonest serious inherited diseases in the world, and red cell transfusion is still one of the few effective treatments for acute and chronic complications. Transfusion corrects anaemia and dilutes out the number of red cells able to cause vaso-occlusion and vascular damage. Urgent red cell transfusions are used to correct acute anaemia, treat acute chest syndrome and patients with acute neurological symptoms. We use elective transfusions preoperatively for moderate risk surgery, and in some pregnant women...
January 29, 2018: British Journal of Haematology
https://www.readbyqxmd.com/read/29373058/a-functional-return-to-play-progression-after-exertional-heat-stroke-in-a-high-school-football-player
#8
Rebecca M Lopez, Patrick Tanner, Sarah Irani, P Patrick Mularoni
OBJECTIVE:   To present a functional return-to-play (RTP) progression after exertional heat stroke (EHS) in a 17-year-old high school football defensive end (height = 185 cm, mass = 145.5 kg). BACKGROUND:   The patient had no pertinent medical history but moved to a warm climate several days before the EHS occurred. After completing an off-season conditioning test (14- × 110-yd [12.6- × 99.0-m] sprints) on a warm afternoon (temperature = approximately 34°C [93°F], relative humidity = 53%), the patient collapsed...
January 26, 2018: Journal of Athletic Training
https://www.readbyqxmd.com/read/29372059/factor-v-leiden-g1691a-and-prothrombin-g20210a-mutations-among-palestinian-patients-with-sickle-cell-disease
#9
Fekri Samarah, Mahmoud A Srour
Background: Vascular thrombosis is an important pathophysiological aspect of sickle cell disease (SCD). This study aimed to investigate the prevalence and clinical impact of factor V Leiden G1691A (FVL) and prothrombin G20210A mutations among Palestinian sickle cell disease (SCD) patients. Methods: A total of 117 SCD patients, including 59 patients with sickle cell anemia (SS), 33 patients with sickle β-thalassemia and 25 individuals with sickle cell trait (AS) were studied...
2018: BMC Hematology
https://www.readbyqxmd.com/read/29318647/realizing-effectiveness-across-continents-with-hydroxyurea-enrollment-and-baseline-characteristics-of-the-multicenter-reach-study-in-sub-saharan-africa
#10
Patrick T McGann, Thomas N Williams, Peter Olupot-Olupot, George A Tomlinson, Adam Lane, José Luís Reis da Fonseca, Robert Kitenge, George Mochamah, Ham Wabwire, Susan Stuber, Thad A Howard, Kathryn McElhinney, Banu Aygun, Teresa Latham, Brígida Santos, Léon Tshilolo, Russell E Ware
Despite its well-described safety and efficacy in the treatment of sickle cell anemia (SCA) in high-income settings, hydroxyurea remains largely unavailable in sub-Saharan Africa, where more than 75% of annual SCA births occur and many comorbidities exist. Realizing Effectiveness Across Continents with Hydroxyurea (REACH, ClinicalTrials.gov NCT01966731) is a prospective, Phase I/II open-label trial of hydroxyurea designed to evaluate the feasibility, safety, and benefits of hydroxyurea treatment for children with SCA in four sub-Saharan African countries...
January 10, 2018: American Journal of Hematology
https://www.readbyqxmd.com/read/29313505/effectiveness-safety-and-cost-of-partial-exchange-transfusions-in-patients-with-sickle-cell-anemia-at-a-sickle-cell-disease-center-in-sub-saharan-africa
#11
P Boma Muteb, J F J Kaluila Mamba, P Muhau Pfutila, V Bilo, J D Panda Mulefu, D A Diallo
The partial exchange transfusions necessary for management of some sickle-cell complications raise the issue of effectiveness in the context of limited resources and inadequate blood safety. This study evaluated the effectiveness, safety, and cost of partial exchange transfusions in 39 patients with sickle-cell anemia in Lubumbashi, looking at the patients' age and gender and the tolerability and direct cost of the transfusions. Excel and SPSS 18 were used for data entry and analysis. Chi2 and Fisher exact tests were used for comparisons...
November 1, 2017: Médecine et Santé Tropicales
https://www.readbyqxmd.com/read/29301288/use-of-pleuroperitoneal-shunt-in-chylothorax-related-to-central-line-associated-thrombosis-in-sickle-cell-disease
#12
Elizabeth Spiwak, Chad Wiesenauer, Arun Panigrahi, Ashok Raj
Central vein thrombosis as a cause of chylothorax is uncommon, and in a few cases in the literature was related to thrombotic complications of central venous access devices (CVAD). Superior vena cava (SVC) occlusion-induced chylothorax has been described in adult sickle cell disease (SCD) in a setting of chronic indwelling CVAD. There are limited reports on chylothorax induced by central venous thrombosis secondary to chronic CVAD in children with SCD. We describe an 8-year-old male patient, with a history of SCD, maintained on long term erythrocytapheresis for primary prevention of stroke, and whose clinical course was complicated by chylothorax which was successfully treated with a pleuroperitoneal shunt...
January 2, 2018: Children
https://www.readbyqxmd.com/read/29300776/prevalence-of-relative-systemic-hypertension-in-adults-with-sickle-cell-disease-in-ghana
#13
Amma Benneh-Akwasi Kuma, Amma Twumwa Owusu-Ansah, Mary Akua Ampomah, Fredericka Sey, Edeghonghon Olayemi, Mehdi Nouraie, Solomon Fiifi Ofori-Acquah
Individuals with sickle cell disease particularly with the homozygous (SS) genotype historically have relatively low blood pressure. Nonetheless, they develop vasculopathy-associated organ dysfunction and the risk of organ dysfunction increases at blood pressures that are normal in the general population. This phenomenon is termed relative systemic hypertension (RSH) with a systolic blood pressure range of 120-139 mmHg, and diastolic blood pressure range of 70-89 mmHg. The significance of RSH lies in its association with renal insufficiency, pulmonary hypertension, stroke and propensity to progress to systemic hypertension...
2018: PloS One
https://www.readbyqxmd.com/read/29296825/disease-severity-and-slower-psychomotor-speed-in-adults-with-sickle-cell-disease
#14
Dana R Jorgensen, Andrea Metti, Meryl A Butters, Joseph M Mettenburg, Caterina Rosano, Enrico M Novelli
Psychomotor slowing is common in children with sickle cell disease (SCD), but little is known about its severity in adults. We conducted a cross-sectional study to quantify psychomotor speed, measured with the digit symbol substitution test (DSST), in relationship with disease severity in adults with SCD attending an outpatient clinic (n = 88, age 36.3 years). Genotype was used to group patients in "severe" (homozygous for hemoglobin S or compound heterozygous with β0 thalassemia) or "moderate" groups (compound heterozygous for HbS, with either HbC or β+ thalassemia)...
September 26, 2017: Blood Advances
https://www.readbyqxmd.com/read/29260615/preliminary-evidence-for-cerebral-capillary-shunting-in-adults-with-sickle-cell-anemia
#15
Meher R Juttukonda, Manus J Donahue, Larry T Davis, Melissa C Gindville, Chelsea A Lee, Niral J Patel, Adetola A Kassim, Sumit Pruthi, Jeroen Hendrikse, Lori C Jordan
Elevated flow velocities in adults with sickle cell anemia (SCA) may cause rapid erythrocyte transit through capillaries. This phenomenon could present as dural venous sinus hyperintensity on arterial spin labeling (ASL)-MRI and could be indicative of capillary shunting. Here, the prevalence of ASL venous hyperintensities and association with relevant physiology in adults with SCA was investigated. SCA ( n = 46) and age-matched control ( n = 16) volunteers were recruited for 3.0 T MRI. Pseudo-continuous ASL-MRI was acquired for cerebral blood flow (CBF) calculation and venous hyperintensity determination; venous signal intensity and a categorical venous score (three raters; 0 = no hyperintensity, 1 = focal hyperintensity, and 2 = diffuse hyperintensity) were recorded...
January 1, 2017: Journal of Cerebral Blood Flow and Metabolism
https://www.readbyqxmd.com/read/29222290/chronic-organ-failure-in-adult-sickle-cell-disease
#16
REVIEW
Elliott Vichinsky
Sickle cell disease is now a chronic adult illness characterized by progressive multiorgan failure, particularly involving the brain and kidney. The etiology is multifactorial; it includes hemolysis and nitric oxide deficiency. As patients age, most experience neurologic insult. Twenty-five percent of older adults have had a clinical stroke and at least half of the population have had a silent infarct, cortical atrophy, and neurocognitive impairment. Periodic screening with neuroimaging and neurocognitive testing is recommended...
December 8, 2017: Hematology—the Education Program of the American Society of Hematology
https://www.readbyqxmd.com/read/29222289/what-is-the-role-of-screening-for-pulmonary-hypertension-in-adults-and-children-with-sickle-cell-disease
#17
REVIEW
Shaina M Willen, Mark T Gladwin
Patient case: An 18-year-old male patient with homozygous hemoglobin SS disease was evaluated for progressive dyspnea and elevated tricuspid regurgitant jet velocity (TRV) on echocardiography. The patient's case is described in detail in Lancet1 He had been treated with regular transfusions since childhood for stroke, had rare episodes of vaso-occlusive pain episodes, and did not take narcotic pain medications. He presented with progressive severe dyspnea on exertion and lower extremity edema. His laboratory tests were notable for a total hemoglobin level of 11...
December 8, 2017: Hematology—the Education Program of the American Society of Hematology
https://www.readbyqxmd.com/read/29201539/assessment-of-cerebral-blood-flow-with-magnetic-resonance-imaging-in-children-with-sickle-cell-disease-a-quantitative-comparison-with-transcranial-doppler-ultrasonography
#18
Paula L Croal, Jackie Leung, Przemyslaw Kosinski, Manohar Shroff, Isaac Odame, Andrea Kassner
Introduction: Transcranial Doppler ultrasonography (TCD) is a clinical tool for stratifying ischemic stroke risk by identifying abnormal elevations in blood flow velocity (BFV) in the middle cerebral artery (MCA). However, TCD is not effective at screening for subtle neurologic injury such as silent cerebral infarcts. To better understand this disparity, we compared TCD measures of BFV with tissue-level cerebral blood flow (CBF) using arterial spin-labeling MRI in children with and without sickle cell disease, and correlated these measurements against clinical hematologic measures of disease severity...
November 2017: Brain and Behavior
https://www.readbyqxmd.com/read/29195085/clinical-and-hematological-profile-in-a-newborn-cohort-with-hemoglobin-sc
#19
Paulo V Rezende, Millane V Santos, Gustavo F Campos, Laura L M Vieira, Maristela B Souza, André R Belisário, Celia M Silva, Marcos B Viana
OBJECTIVES: Hemoglobin SC is the second most common variant of sickle-cell disease worldwide, after hemoglobin SS. The objectives of the study were to describe the clinical and laboratory characteristics of hemoglobin SC disease in children from a newborn screening program and treated at a blood donor center. METHODOLOGY: This study assessed a cohort of 461 infants born between 01/01/1999 and 12/31/2012 and followed-up until 12/31/2014. Clinical events were expressed as rates for 100 patient-years, with 95% confidence intervals...
November 28, 2017: Jornal de Pediatria
https://www.readbyqxmd.com/read/29181140/foetal-haemoglobin-and-disease-severity-in-nigerian-children-with-sickle-cell-anaemia
#20
Oluwagbemiga O Adeodu, Morenike A Akinlosotu, Samuel A Adegoke, Saheed B A Oseni
Background: Foetal haemoglobin (HbF) is a major modifying factor influencing sickle cell disease (SCD) severity. Despite this, HbF estimation is not routinely done in Nigeria. The relationship between HbF and SCD severity among affected children is also poorly studied. Methods: In this descriptive cross-sectional study, we determined the relationship between steady state HbF levels and disease severity of Nigerian children aged 1 - 15 years with homozygous SCD. For each child, the socio-demographic characteristics and SCD clinical severity were determined...
2017: Mediterranean Journal of Hematology and Infectious Diseases
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