These Patients Had Sickle-Cell Disease. Experimental Therapies May Have Cured Them.

Success against sickle-cell would mark “ the first genetic cure of a common genetic disease ” and could free tens of thousands of Americans from agonizing pain.
Source: NYT Health - Category: Consumer Health News Authors: Tags: Genetics and Heredity Hemoglobin Crispr (DNA) Blood Pain Sickle Cell Anemia National Institutes of Health Harvard University bluebird bio Inc. Africa Source Type: news

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Authors: Eleutério RMN, Nascimento FO, Araújo TG, Castro MF, Filho TPA, Filho PAM, Eleutério J, Elias DBD, Lemes RPG Abstract Background: Sickle cell anaemia (SCA) is the most prevalent monogenic disease in Brazil. In SCA, haemoglobin S (HbS) is formed, which modifies red blood cell morphology. Intravascular haemolysis occurs, in which free Hb and free radicals degrade nitric oxide (NO) and release arginase, which reduces arginine levels. Because arginine is a substrate for NO formation, this decrease leads to reduced NO (vasodilator) synthesis. SCA treatment uses hydroxyurea (HU) to maintain h...
Source: Advances in Hematology - Category: Hematology Tags: Adv Hematol Source Type: research
Authors: Niccoli Asabella A, Altini C, Nappi AG, Lavelli V, Ferrari C, Marzullo A, Loiodice A, Rubini G Abstract Sickle cell disease (SCD) is the best known haemoglobinopathy, caused by a mutation substituting valina for glutamic acid at position 6 of the beta-globin chain of adult hemoglobin A, resulting in hemoglobin S (HbS). The homozygous HbS disease (HbSS), an autosomal recessive disorder, is the most common form and the Mediterranean area, along with sub-Saharian African and India, have the highest prevalence (1%-15%). In particular, Sicily with a prevalence of 2%-5%, is among the most interested regions. How...
Source: Hellenic Journal of Nuclear Medicine - Category: Nuclear Medicine Tags: Hell J Nucl Med Source Type: research
Success against sickle-cell would be “ the first genetic cure of a common genetic disease ” and could free tens of thousands of Americans from agonizing pain.
Source: NYT Health - Category: Consumer Health News Authors: Tags: Genetics and Heredity Hemoglobin Crispr (DNA) Blood Pain Sickle Cell Anemia National Institutes of Health Harvard University bluebird bio Inc. Africa Source Type: news
Conclusion: Zinc deficiency occurs in children with SCA and the deficiency is worsened by acute painful events Therefore, it is recommended that zinc level should be assessed and any deficiency treated. Supplementation of zinc should also be enhanced as this may reduce painful crisis in SCA.
Source: Nigerian Journal of Clinical Practice - Category: Rural Health Authors: Source Type: research
Conclusion: Camp offers unique opportunities for care coordination. Camps for children with SCD provide a setting for dissemination of best practices for the disease. Community-based organizations should partner in recruiting young adults with SCD as counselors. Educational material now available could be modified for use in the camp setting. All stakeholders in the care of children with SCD should work in unison to ensure these children enjoy the benefits of summer camp. PMID: 30559621 [PubMed]
Source: Ochsner Journal - Category: General Medicine Tags: Ochsner J Source Type: research
Conclusion: This study showed a higher prevalence of infection and bone pain crisis among SCA patients with autosplenectomy than in SCA patients without autosplenectomy. It also showed comparable level of IFN-γ in the 2 groups of patients. Patients with autosplenectomy may benefit from early institution of drugs such as hydroxyurea to improve quality of life.
Source: Nigerian Journal of Clinical Practice - Category: Rural Health Authors: Source Type: research
[VOA] Africans who have the blood disorder sickle cell anemia met this week in the northern Cameroon town of Garoua to step up an awareness campaign.
Source: AllAfrica News: Health and Medicine - Category: African Health Source Type: news
Authors: Liem RI Abstract Development of exercise guidelines for individuals with sickle cell trait (SCT) and sickle cell anemia (SCA) is hampered by the need to weigh the benefits against risks of exercise in these populations. In SCT, concern for exercise collapse associated with sickle cell trait has resulted in controversial screening of student athletes for SCT. In SCA, there exists unsubstantiated concerns that high-intensity exercise may result in pain and other complications. In both, finding the "right dose" of exercise remains a challenge for patients and their providers. Despite assumptions tha...
Source: Hematology ASH Education Program - Category: Hematology Tags: Hematology Am Soc Hematol Educ Program Source Type: research
“Where there is no struggle, there is no compassion.” -Frederick Douglass Growing up with sickle cell anemia exposed me to the field of medicine. As I go through the pain and complications, it humbles me to the point that motivates me to work harder to be the doctor I want for myself. I want to […]Find jobs at  Careers by KevinMD.com.  Search thousands of physician, PA, NP, and CRNA jobs now.  Learn more.
Source: Kevin, M.D. - Medical Weblog - Category: General Medicine Authors: Tags: Education Medical school Oncology/Hematology Source Type: blogs
Hydroxyurea is a potent and safe disease-modifying therapy for sickle cell anemia (SCA), with available data proving laboratory and clinical efficacy for both children and adults. Although the global burden of SCA is greatest within sub-Saharan Africa, almost all studies with hydroxyurea to date have been conducted in the US and Europe. Since additional comorbidities may affect children with SCA in low-resource settings, including malnutrition, malaria, and other infections, prospective research is needed to develop locally appropriate guidelines for hydroxyurea use.To assess the feasibility, safety, and benefits of hydrox...
Source: Blood - Category: Hematology Authors: Tags: Plenary Scientific Session Source Type: research
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