Sickle cell disease: a comprehensive program of care from birth.

Sickle cell disease: a comprehensive program of care from birth. Hematology Am Soc Hematol Educ Program. 2019 Dec 06;2019(1):490-495 Authors: de Montalembert M, Tshilolo L, Allali S Abstract As more children are appropriately being diagnosed, the burden of sickle cell disease is increasing greatly in Africa and in high-resource countries such as the United States and Europe. Early management is mandatory, but newborn screening is not implemented everywhere. Point-of-care testing devices are increasingly being used in low-resource countries, showing good sensitivity and specificity. Because the diagnosis is often traumatic for the families, the announcement should be made by an experienced person. The development of care networks is urgently required to facilitate daily life by defining the respective functions of nearby and highly specialized health care professionals, who should work in close collaboration. Comprehensive programs targeting the prevention of pneumococcal infections, malaria in infested zones, and stroke may substantially improve patient care. Hydroxyurea is increasingly being used, but whether it should be systematically prescribed in all children is debated, and its access is still limited in many African countries. Yearly checkups should be organized early in life in order to screen and then treat any organ impairment. Enhancing parents' and patients' knowledge and skills is mandatory. PMID: 31808910 [PubMed - in process]
Source: Hematology ASH Education Program - Category: Hematology Tags: Hematology Am Soc Hematol Educ Program Source Type: research

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Publication date: Available online 17 January 2020Source: American Journal of Kidney DiseasesAuthor(s): Erin P. Flanagin, Yashodhan Chivate, Daniel E. Weiner
Source: American Journal of Kidney Diseases - Category: Urology & Nephrology Source Type: research
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Source: SafetyLit - Category: International Medicine & Public Health Tags: Alcohol and Other Drugs Source Type: news
Researchers at Florida Atlantic University have developed a microfluidic chip that can rapidly assess blood samples from sickle cell disease patients to help monitor the disease. The technique is much faster and more convenient than traditional optic...
Source: Medgadget - Category: Medical Devices Authors: Tags: Diagnostics Medicine Source Type: blogs
CONCLUSIONS: Clinical suspicion for transfusion-related adverse events, including hemolytic transfusion reactions and transfusion-transmitted infections, should be high in typically and atypically immunocompromised patient populations (like SCD), especially those on chronic transfusion protocols. Manual blood smear review aids in the evaluation of patients with SCD presenting with severe vaso-occlusive crisis and MSOF and can alert clinicians to the need for initiating aggressive therapy like RBC exchange and artesunate therapy. PMID: 29524230 [PubMed - as supplied by publisher]
Source: Transfusion - Category: Hematology Authors: Tags: Transfusion Source Type: research
Abstract 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, 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. Following comprehensive training of local researc...
Source: American Journal of Hematology - Category: Hematology Authors: Tags: Research Article Source Type: research
This week’s topics include managing preeclampsia, diet soft drinks and stroke, autoimmune conditions and malaria, and fecal immunochemical testing and colorectal cancer. Program notes: 0:36 Malaria, lupus and MS 1:36 B-cell activating factor or BAF 2:34 Sickle cell anemia also 3:16 Diet soft drinks and stroke 4:16 Three fold increased risk of stroke and Alzheimer’s […]
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Authors: Bello-Manga H, DeBaun MR, Kassim AA Abstract INTRODUCTION: Sickle cell disease (SCD) is the most common inherited hemoglobinopathy in sub-Saharan Africa with about 300,000 births annually. Children with SCD have pre-existing anemia that can be exacerbated when they have concomitant nutritional deficiencies, infections or exposure to environmental toxins. The resulting relative anemia in children with SCD is associated with increased risk of strokes; poor cognitive function, poor growth, and may also attenuate optimal response to hydroxyurea therapy, the only effective and practical treatment option for SCD...
Source: Expert Review of Hematology - Category: Hematology Tags: Expert Rev Hematol Source Type: research
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Source: Hematology - Category: Hematology Tags: Hematology Source Type: research
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Source: Science - The Huffington Post - Category: Science Source Type: news
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