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sickle cell disease managment

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https://www.readbyqxmd.com/read/27924667/contraceptive-practices-in-women-with-sickle-cell-disease
#1
Natália S Carvalho, Josefina Pellegrini Braga, Márcia Barbieri, Maria R Torloni, Maria S Figueiredo, Cristina A F Guazzelli
This was a cross-sectional study of sexually active women with sickle-cell disease (SCD) managed at São Paulo Federal University during a one-year period. A total of 54 women were included. Mean age was 32.0 (11.2, standard deviation) years and almost 95% were black or of mixed racial ancestry. Almost 80% reported a history of multiple blood transfusions, 50% had kidney disease and 17% had a history of thrombosis. Over 80% of them had used some form of contraception, mostly combined hormonal contraceptive (52%) or progestin-only contraceptives (46%)...
December 7, 2016: Journal of Obstetrics and Gynaecology: the Journal of the Institute of Obstetrics and Gynaecology
https://www.readbyqxmd.com/read/27913540/sickle-cell-disease-an-inherited-thrombophilia
#2
Ted Wun, Ann Brunson
Activation of the hemostatic system occurs in patients with sickle cell disease. The extent to which this activation contributes to sickle cell pathophysiology is uncertain. Clinical trials of anticoagulants or platelet inhibitors have demonstrated the ability to decrease biomarkers of hemostatic activation, but this has generally not resulted in improvement in clinically relevant outcomes. Venous thromboembolism (VTE: deep venous thrombosis and pulmonary embolism) has been until recently an underappreciated complication of sickle cell disease, with incident event and recurrence rates consistent with a strong thrombophilia...
December 2, 2016: Hematology—the Education Program of the American Society of Hematology
https://www.readbyqxmd.com/read/27913539/prevention-of-central-nervous-system-sequelae-in-sickle-cell-disease-without-evidence-from-randomized-controlled-trials-the-case-for-a-team-based-learning-collaborative
#3
Michael R DeBaun, Allison A King
Since 1998, the National Institutes of Health has funded 5 randomized controlled trials (RCTs) for primary and secondary prevention of strokes in children with sickle cell anemia (SCA). In a systematic fashion, these trials have significantly advanced the care of children with SCA. In the absence of an RCT, clinicians are often compelled to make decisions at the bedside, based on experience, observational studies, and principles of hematology. We will provide an initial example that describes how a team-based, learning collaborative developed a multisite standard care protocol with a low budget (<$10 000 per year) to overcome the intrinsic limitations of advancing the care of neurologic complications in sickle cell disease (SCD)...
December 2, 2016: Hematology—the Education Program of the American Society of Hematology
https://www.readbyqxmd.com/read/27913538/sickle-cell-disease-when-and-how-to-transfuse
#4
Jo Howard
Blood transfusion remains an important therapeutic intervention in patients with sickle cell disease (SCD), aiming to both increase the oxygen carrying capacity of blood and to reduce the complications of vaso-occlusion. Simple, manual exchange and automated exchange can be effective in reducing the acute and chronic complications of SCD, and the advantages and disadvantages of each methodology mean they all have a role in different situations. Evidence for the role of emergency transfusion in the management of the acute complications of SCD, including acute pain and acute chest syndrome, comes from observational data...
December 2, 2016: Hematology—the Education Program of the American Society of Hematology
https://www.readbyqxmd.com/read/27880985/daily-pain-in-adults-with-sickle-cell-disease-a-different-perspective
#5
Charlotte F J van Tuijn, Joep W R Sins, Karin Fijnvandraat, Bart J Biemond
Previous reports demonstrated that patients with sickle cell disease (SCD) experience pain on more than half of the observed days. Yet, these high incidences do not seem to match observations in our population. In this prospective cohort study we aimed to assess the frequency and characteristics of daily, self-reported pain among adult SCD patients in the Netherlands. Consecutive patients were enrolled during routine outpatient visits and followed up to 6 months. A total of 55 patients completed 5 982 diary observation days...
November 23, 2016: American Journal of Hematology
https://www.readbyqxmd.com/read/27869662/the-cell-killing-mechanisms-of-hydroxyurea
#6
REVIEW
Amanpreet Singh, Yong-Jie Xu
Hydroxyurea is a well-established inhibitor of ribonucleotide reductase that has a long history of scientific interest and clinical use for the treatment of neoplastic and non-neoplastic diseases. It is currently the staple drug for the management of sickle cell anemia and chronic myeloproliferative disorders. Due to its reversible inhibitory effect on DNA replication in various organisms, hydroxyurea is also commonly used in laboratories for cell cycle synchronization or generating replication stress. However, incubation with high concentrations or prolonged treatment with low doses of hydroxyurea can result in cell death and the DNA damage generated at arrested replication forks is generally believed to be the direct cause...
November 17, 2016: Genes
https://www.readbyqxmd.com/read/27869039/the-role-of-rs1984112_g-at-cd36-gene-in-increasing-reticulocyte-level-among-sickle-cell-disease-patients
#7
Miniar Kalai, Marwa Dridi, Leila Chaouch, Imen Moumni, Houyem Ouragini, Imen Darragi, Imen Boudrigua, Dorra Chaouachi, Fethi Mellouli, Mohamed Bejaoui, Salem Abbes
AIMS AND BACKGROUND: Mediators of adhesion become a potential new target for pharmacological therapy to struggle the complications of sickle cell disease (SCD). Several mechanisms for increased adherence have been postulated and the well-studied are CD36 and VLA4 which encoded by ITGA4. Herein, we sought to determine whether one polymorphism of CD36 namely: rs1984112 and three exons of ITGA4 (4, 5, and 6) are implicated in hemolytic status and clinical events among SCD Tunisian patients...
November 20, 2016: Hematology (Amsterdam, Netherlands)
https://www.readbyqxmd.com/read/27866856/free-tissue-transfer-in-patients-with-sickle-cell-disease-considerations-for-multi-disciplinary-peri-operative-management
#8
REVIEW
Lilli Cooper, Rohit Seth, Elizabeth Rhodes, Mohammed Alousi, Bran Sivakumar
INTRODUCTION AND AIMS: Sickle cell disease (SCD) is an increasingly common condition in the UK. The safety of free tissue transfer in these patients is controversial, and no specific guidelines exist. The aim of this paper is to create recommendations for the plastic surgical multidisciplinary team for use in the assessment and management of SCD patients undergoing free tissue transfer and reconstruction. METHOD: A literature review was performed in PubMed of 'sickle [TiAb] AND plast* adj3 surg*...
October 26, 2016: Journal of Plastic, Reconstructive & Aesthetic Surgery: JPRAS
https://www.readbyqxmd.com/read/27800652/opioid-prescription-practices-at-discharge-and-30-day-returns-in-children-with-sickle-cell-disease-and-pain
#9
Leslie M Okorji, Devin S Muntz, Robert I Liem
BACKGROUND: Acute pain episodes in children with sickle cell disease (SCD) represent a leading cause of readmissions. We examined prescription practices at the time of discharge in children with SCD presenting with acute pain to determine their impact on 30-day emergency department (ED) revisits and readmissions. METHODS: In this single-institution, 5-year retrospective study, we reviewed 290 encounters of patients with SCD aged 7-21 years hospitalized or discharged from the ED with acute pain...
November 1, 2016: Pediatric Blood & Cancer
https://www.readbyqxmd.com/read/27798391/pain-management-for-sickle-cell-disease-in-the-pediatric-emergency-department-medications-and-hospitalization-trends
#10
Chantel Cacciotti, Sarah Vaiselbuh, Eleny Romanos-Sirakis
The majority of emergency department (ED) visits and hospitalizations for patients with sickle cell disease (SCD) are pain related. Adequate and timely pain management may improve quality of life and prevent worsening morbidities. We conducted a retrospective chart review of pediatric patients with SCD seen in the ED, selected by sickle cell-related ICD-9 codes. A total of 176 encounters were reviewed from 47 patients to record ED pain management and hospitalization trends. Mean time to pain medication administration was 63 minutes...
October 23, 2016: Clinical Pediatrics
https://www.readbyqxmd.com/read/27798028/neurological-complications-of-sickle-cell-disease-in-africa-protocol-for-a-systematic-review
#11
Michel K Mengnjo, Joseph Kamtchum-Tatuene, Nicolas Nicastro, Jean Jacques N Noubiap
INTRODUCTION: Sickle cell disease (SCD) is highly prevalent in Africa. Considered as a public health problem, it is associated with high morbidity and mortality. Neurological complications of SCD can cause significant disability with important socioeconomic and psychological impact on the patients and their families, and can even lead to death if not properly managed. There are important knowledge gaps regarding the burden of neurological complications of SCD in African populations. We propose to conduct the first systematic review to summarise the epidemiological data available on neurological complications of SCD in Africa...
October 19, 2016: BMJ Open
https://www.readbyqxmd.com/read/27797344/objective-basis-for-chronic-pain-in-patients-with-adult-sickle-cell-disease
#12
Samip Master, Abhishek Patel, Glenn Mills, Richard Mansour
Sickle Cell Disease (SCD) affects approximately 100,000 Americans and due to lack of an organized treatment approach, patients with SCD pose a high economic burden on medical services. The patients with SCD have chronic bone damage from bone marrow infraction and vaso-occulsive events. These bone damages lead to chronic pain in patients with SCD. The inadwquate treatment of chronic pain in adult patients with SCD can lead to pseudo-addictive behavior and also affect their psycho-social life. There are certain barriers to adequate pain management in adult patients with SCD, namely, limited knowledge among the clinicians, inadequate assessment, concerns about addiction, and biases against opioid use...
September 2016: Journal of the Louisiana State Medical Society: Official Organ of the Louisiana State Medical Society
https://www.readbyqxmd.com/read/27793727/pilot-of-the-chronic-disease-self-management-program-for-adolescents-and-young-adults-with-sickle-cell-disease
#13
Lori E Crosby, Naomi E Joffe, James Peugh, Russell E Ware, Maria T Britto
PURPOSE: This study evaluated the feasibility of a group self-management intervention, the well-established Stanford Chronic Disease Self-Management Program (CDSMP), for adolescents and young adults (AYA) with sickle cell disease (SCD). METHODS: A total of 22 AYA participants with SCD, ages 16-24 years, completed self-efficacy and quality of life measures before the CDSMP, after, and 3 and 6 months later. RESULTS: This AYA cohort showed significant improvements in self-efficacy (primary outcome) after the intervention...
October 25, 2016: Journal of Adolescent Health: Official Publication of the Society for Adolescent Medicine
https://www.readbyqxmd.com/read/27786409/the-quality-of-information-about-sickle-cell-disease-on-the-internet-for-youth
#14
Vicky R Breakey, Lauren Harris, Omar Davis, Arnav Agarwal, Carley Ouellette, Elizabeth Akinnawo, Jennifer Stinson
BACKGROUND: Adolescence is a vulnerable time for teens with sickle cell disease (SCD). Although there is evidence to support the use of web-based education to promote self-management skills in patients with chronic illnesses, the quality of SCD-related information on the Internet has not been assessed. PROCEDURE: A website review was conducted to appraise the quality, content, accuracy, readability, and desirability of online information for the adolescents with SCD...
October 27, 2016: Pediatric Blood & Cancer
https://www.readbyqxmd.com/read/27785921/multimodality-ultrasound-imaging-in-stroke-current-concepts-and-future-focus
#15
Yumei Liu, Yang Hua, Wuwei Feng, Bruce Ovbiagele
Stroke is a leading cause of disability and mortality worldwide. Ultrasound is a real-time imaging technique that is inexpensive, portable, non-invasive, and safe, with high diagnostic accuracy. Ultrasonic imaging can provide useful direct and indirect information about the characteristics of various vessels in the both intracranial and extracranial segments. Areas covered: In this review, we will discuss multimodal applications of ultrasonic imaging in stroke prevention and management including checking carotid intima-media thickness progression, evaluating the plaque morphology, calibrating the degree of stenosis, detecting the presence of patent foramen ovale, monitoring microembolization, and screening for stroke risk in patients with sickle cell disease...
December 2016: Expert Review of Cardiovascular Therapy
https://www.readbyqxmd.com/read/27778040/modern-american-scurvy-experience-with-vitamin-c-deficiency-at-a-large-children-s-hospital
#16
Farahnaz Golriz, Lane F Donnelly, Sridevi Devaraj, Raj Krishnamurthy
BACKGROUND: Until recently scurvy has been viewed in developed countries as a disease of the past. More recently there have been reports of case series of children with scurvy who have had a delayed diagnosis after an extensive diagnostic workup that included imaging. Most of these children have had underlying neurologic conditions such as autism. OBJECTIVE: To review the medical records of children diagnosed with vitamin C (ascorbic acid) deficiency based on serum ascorbic acid levels at a large pediatric health care system, to determine imaging findings and utility of imaging in management, and to identify at-risk pediatric populations...
October 24, 2016: Pediatric Radiology
https://www.readbyqxmd.com/read/27775898/managing-acute-complications-of-sickle-cell-disease-in-pediatric-patients
#17
Sathyaseelan Subramaniam, Jennifer H Chao
Sickle cell disease is a chronic hematologic disease with a variety of acute, and often recurring, complications. Vaso-occlusive crisis, a unique but common presentation in sickle cell disease, can be challenging to manage. Acute chest syndrome is the leading cause of death in patients with sickle cell disease, occurring in more than half of patients who are hospitalized with a vaso-occlusive crisis. Uncommon diagnoses in children, such as stroke, priapism, and transient red cell aplasia, occur more frequently in patients with sickle cell disease and necessitate a degree of familiarity with the disease process and its management...
November 2016: Pediatric Emergency Medicine Practice
https://www.readbyqxmd.com/read/27773982/review-hemodynamic-characteristics-and-outcomes-of-sickle-cell-disease-associated-pulmonary-hypertension
#18
REVIEW
Alem Mehari, Alvin V Thomas, Alicia N Thomas, Mark S Johnson
Pulmonary hypertension (PH) is a leading cause of morbidity and early mortality in adults with sickle cell disease (SCD). However, the prevalence, hemodynamic profile and prognosis of SCD-PH remain controversial and need frequent updates. Pulmonary hypertension determined by right heart catheterization (RHC) occurs in 6% to 10% of adults with SCD. Hemodynamically, SCD-PH may be pre-capillary or post-capillary in nature. The exact etiology is unknown and often multifactorial; hence a thorough diagnostic evaluation following established PH guidelines is essential to determine disease prevalence, etiology and outcomes...
October 20, 2016: Ethnicity & Disease
https://www.readbyqxmd.com/read/27771375/spleen-histology-in-children-with-sickle-cell-disease-and-hereditary-spherocytosis-hints-on-the-disease-pathophysiology
#19
Marco Pizzi, Fabio Fuligni, Luisa Santoro, Elena Sabattini, Martina Ichino, Rita De Vito, Pietro Zucchetta, Raffaella Colombatti, Laura Sainati, Piergiorgio Gamba, Rita Alaggio
Hereditary spherocytosis (HS) and sickle cell disease (SCD) are associated with splenomegaly and spleen dysfunction in pediatric patients. Scant data exist on possible correlations between spleen morphology and function in HS and SCD. This study aimed to assess the histological and morphometric features of HS and SCD spleens, in order to get possible correlations with disease pathophysiology. In a large series of spleens from SCD, HS and control patients the following parameters were considered: (i) macroscopic features; (ii) lymphoid follicle (LF) density; (iii) presence of peri-follicular marginal zones (MZs); (iv) presence of Gamna-Gandy bodies; (v) density of CD8-positive sinusoids; (vi) density of CD34-positive microvessels; (vii) presence/distribution of fibrosis and SMA-positive myoid cells; (viii) density of CD68-positive macrophages...
October 19, 2016: Human Pathology
https://www.readbyqxmd.com/read/27759170/erythrocytapheresis-for-chronic-transfusion-therapy-in-sickle-cell-disease-survey-of-current-practices-and-review-of-the-literature
#20
Shannon Kelly, Keith Quirolo, Anne Marsh, Lynne Neumayr, Alicia Garcia, Brian Custer
BACKGROUND: Chronic red blood cell (RBC) transfusion therapy (CTT) is an integral component of the management of severe sickle cell disease (SCD) and can prevent complications, such as stroke. RBC units can be administered via simple transfusion or exchange transfusion, and erythrocytapheresis (automated RBC exchange transfusion [aRBX]), is increasingly used for CTT. Comparisons of simple and aRBX transfusions are limited, and the current scope of aRBX use is not known. STUDY DESIGN AND METHODS: We administered a survey to define current transfusion practices for CTT and performed a review of the erythrocytapheresis literature...
October 19, 2016: Transfusion
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