Preventing and treating acute chest syndrome in children with sickle cell disease

(Mary Ann Liebert, Inc./Genetic Engineering News) Acute chest syndrome (ACS), a potentially severe lung complication of sickle cell disease, increases a child's risk of respiratory failure, chronic lung disease, and prolonged hospitalization if not recognized early and treated effectively.
Source: EurekAlert! - Medicine and Health - Category: International Medicine & Public Health Source Type: news

Related Links:

Publication date: Available online 17 August 2018Source: Journal of American Association for Pediatric Ophthalmology and StrabismusAuthor(s): Ricardo Luz Leitão Guerra, Cristina Salles
Source: Journal of American Association for Pediatric Ophthalmology and Strabismus - Category: Opthalmology Source Type: research
The β-hemoglobinopathies, consisting of sickle cell disease (SCD) and the β-thalassemias, are the most common genetic diseases worldwide. These syndromes cause widespread suffering and significant reduction in lifespan in patients. The β-thalassemia syndromes comprise a spectrum of disorders characte rized by deficiencies in the production of the adult β-globin chain [1]. Increased expression of the fetal γ-globin chains and Fetal Hemoglobin [(HbF(α2γ2)] can augment reduced β-globin chain deficiencies and is therefore considered a desirable therapeutic target that can ameliorate t...
Source: Experimental Hematology - Category: Hematology Authors: Tags: Brief Communication Source Type: research
Conclusions: In severe sickle cell crisis thrombocytopenia is more common than thrombocytosis. In the ICU, day 1 platelet counts correlate inversely with prognostic scores and are significantly reduced in multi-organ failure and nonsurvivors. A platelet count above 175 × 109/L predicts patient survival with high specificity and positive predictive value but lacks sensitivity.
Source: Indian Journal of Critical Care Medicine - Category: Intensive Care Authors: Source Type: research
American Journal of Hematology,Volume 0, Issue ja, -Not available-.
Source: American Journal of Hematology - Category: Hematology Authors: Source Type: research
Disease severity of sickle cell anemia is highly variable, and it is commonly accepted that fetal hemoglobin (HbF) levels play a major role as an ameliorating factor. Investigation of genetic variants have identified several genes to be the principal influencers of HbF regulation. Here, we further elucidated the association of rs4527238 and rs35685045 ofANTXR1 genes in the context of HbF level variance in sickle cell anemia patients of the Arab-Indian haplotype. Samples from 630 sickle cell anemia patients were analyzed for the mutations at 2 specific locations of theANTXR1 gene by TaqMan ®-based real-time PCR. The CC ...
Source: Acta Haematologica - Category: Hematology Source Type: research
In 2017, the Food and Drug Administration approved 2 medications for sickle cell anemia (SCA): hydroxyurea for children and l-glutamine for children and adults. The approval of hydroxyurea was long overdue, but the approval of l-glutamine was a surprise to many. Any effective new treatment for SCA is a welcome advance, but there are few published studies of l-glutamine as a specific treatment for SCA. Accordingly, there are many unanswered questions about its efficacy, safety, and role in current therapy.
Source: Blood - Category: Hematology Authors: Tags: Sickle Cell Disease, Red Cells, Iron, and Erythropoiesis, Blood Spotlight, Clinical Trials and Observations Source Type: research
Abstract Disease severity of sickle cell anemia is highly variable, and it is commonly accepted that fetal hemoglobin (HbF) levels play a major role as an ameliorating factor. Investigation of genetic variants have identified several genes to be the principal influencers of HbF regulation. Here, we further elucidated the association of rs4527238 and rs35685045 of ANTXR1 genes in the context of HbF level variance in sickle cell anemia patients of the Arab-Indian haplotype. Samples from 630 sickle cell anemia patients were analyzed for the mutations at 2 specific locations of the ANTXR1 gene by TaqMan®-based rea...
Source: Acta Haematologica - Category: Hematology Authors: Tags: Acta Haematol Source Type: research
Great strides have been made in the management of sickle cell disease. Here, Maria Cappellini, of the University of Milan-Foundation IRCCS Policlinico Hospital, Milan, Italy, discusses the basics of s... Author: VJHemOnc Added: 08/15/2018
Source: Oncology Tube - Category: Cancer & Oncology Source Type: podcasts
Speaking from the 23rd congress of the European Hematology Association (EHA) 2018 press briefing, held in Stockholm, Sweden, Dr Francesca Vinchi, of the Iron Research Program, New York Blood Center, N... Author: VJHemOnc Added: 08/15/2018
Source: Oncology Tube - Category: Cancer & Oncology Source Type: podcasts
Publication date: Available online 14 August 2018Source: Nitric OxideAuthor(s): Li Wang, Luis E.F. Almeida, Sayuri Kamimura, Jack H. van der Meulen, Kanneboyina Nagaraju, Martha Quezado, Paul Wakim, Zenaide M.N. QuezadoAbstractSickle cell disease (SCD) patients can have limited exercise capacity and muscle dysfunction characterized by decreased force, atrophy, microvascular abnormalities, fiber distribution changes, and skeletal muscle energetics abnormalities. Growing evidence suggests that in SCD, there is alteration in nitric oxide (NO) availability/signaling and that nitrate/nitrite can serve as a NO reservoir and enha...
Source: Nitric Oxide - Category: Chemistry Source Type: research
More News: Children | Genetics | International Medicine & Public Health | Respiratory Medicine | Sickle Cell Anemia