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Sickle Cell Anemia (Hemoglobin S Disease)

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A disease characterized by chronic hemolytic anemia, episodic painful crises, and pathologic involvement of many organs. It is the clinical expression of homozygosity for hemoglobin S.

Also Known As:

Hemoglobin S Disease; Anemia, Sickle Cell; Sickle Cell Disease; Sickle Cell Disorders; Sickling Disorder Due to Hemoglobin S; Anemias, Sickle Cell; Cell Disease, Sickle; Cell Diseases, Sickle; Cell Disorder, Sickle; Cell Disorders, Sickle; Disease, Hemoglobin S; Hemoglobin S Diseases; Sickle Cell Anemias; Sickle Cell Diseases; Sickle Cell Disorder

Networked: 5338 relevant articles (281 outcomes, 608 trials/studies)

Relationship Network

Disease Context: Research Results

Related Diseases

1.	Thalassemia
2.	beta-Thalassemia (Cooley's Anemia)
3.	Pain (Aches)
4.	Infection
5.	Sickle Cell Anemia (Hemoglobin S Disease)

Experts

1.	Gladwin, Mark T: 69 articles (10/2015 - 02/2002)
2.	Ware, Russell E: 58 articles (01/2016 - 01/2002)
3.	Kato, Gregory J: 56 articles (03/2015 - 01/2005)
4.	Steinberg, Martin H: 53 articles (08/2015 - 05/2002)
5.	Ballas, Samir K: 46 articles (10/2015 - 02/2002)
6.	DeBaun, Michael R: 36 articles (09/2015 - 10/2004)
7.	Casella, James F: 33 articles (10/2015 - 03/2002)
8.	Wang, Winfred C: 31 articles (12/2014 - 01/2002)
9.	Kutlar, Abdullah: 28 articles (11/2015 - 05/2002)
10.	Ataga, Kenneth I: 26 articles (11/2015 - 12/2003)

Drugs and Biologics

Drugs and Important Biological Agents (IBA) related to Sickle Cell Anemia:

1.	Hydroxyurea (Hydrea)FDA LinkGeneric 04/01/2013 - "Hydroxyurea (HU) is highly effective treatment for sickle cell disease (SCD). " 07/01/1997 - "Children with sickle cell anemia provide the best opportunity to assess the efficacy of hydroxyurea (HU) in preventing complications and progressive organ damage. " 12/05/2015 - "Hydroxyurea has proven efficacy in numerous clinical trials as a disease-modifying treatment for patients with sickle cell anemia (SCA) but is currently under-used in clinical practice. " 10/01/2003 - "For patients with sickle cell disease, hydroxyurea is no longer an experimental tool; it has given rise to several trials, where it has proven to be effective in terms of both clinical and haematological improvement." 01/01/2007 - "Although supportive therapies such as regular transfusion and chelation for beta-thal and hydroxyurea (HU) for sickle cell disease have significantly improved clinical manifestations and the quality of life, they cannot eliminate disease and therapy-related complications. "
2.	Fetal Hemoglobin (Hemoglobin F)IBA 01/01/2014 - "Decades of research have established that the most effective treatment for sickle cell disease (SCD) is increased fetal hemoglobin (HbF). " 02/01/2008 - "Increasing hemoglobin F (HbF) appears to be beneficial for patients with sickle cell anemia. " 10/01/2004 - "Increased levels of fetal hemoglobin (HbF) are clinically beneficial in patients with sickle cell disease. " 03/15/1999 - "High levels of fetal hemoglobin (Hb F) protect from many of the complications of sickle cell disease and lead to improved survival. " 07/01/2009 - "Inducers of fetal hemoglobin (HbF) have shown considerable promise in the treatment of sickle cell disease (SCD). "
3.	Hemoglobins (Hemoglobin)IBA 04/01/2015 - "A total of 56 hemoglobin SC and 39 sickle cell anemia patients were included in the study, all in steady state, and 27 healthy controls. " 04/01/2015 - "We describe the findings of a cross-sectional observational study evaluating coagulation activation markers in adult patients with hemoglobin SC, comparing them with those in sickle cell anemia patients and healthy controls. " 09/01/2014 - "Prior analyses of the Cooperative Study of Sickle Cell Disease (CSSCD) newborn cohort identified elevated white blood cell (WBC) count, low baseline hemoglobin and dactylitis between the ages of 1 and 2 years as markers of severe disease. " 01/01/2012 - "The purpose of this study was to evaluate the baseline values of steady state hemoglobin and packed cell volume as a guide to managing the early recognition of hemolytic crises in sickle cell anemia. " 01/01/2012 - "Forty-eight male and 48 female children were enrolled in this study, 79 with sickle cell anemia and 17 with hemoglobin SC. "
4.	Penicillins (Penicillin)FDA Link 02/01/1999 - "Since the introduction of penicillin prophylaxis policies, newborn screening, new immunizations, and comprehensive medical care centers, the survival of young children with sickle cell disease has improved. " 06/01/2013 - "Since the publication of the Prophlyactic Penicillin Study II in 1995, the management of penicillin prophylaxis for children with sickle cell disease (SCD) after 5 years of age has been controversial. " 06/01/2013 - "Prophylactic penicillin after 5 years of age in patients with sickle cell disease: a survey of sickle cell disease experts." 01/01/2012 - "Nasopharyngeal carriage of penicillin resistant pneumococci in Ugandan children with homozygous sickle cell disease is high. " 03/01/2011 - "We sought to determine the compliance rate with intramuscular penicillin (IM) prophylaxis in children with sickle cell disease. "
5.	ZincIBA 07/01/1995 - "Efficacy of zinc therapy in prevention of crisis in sickle cell anemia: a double blind, randomized controlled clinical trial." 01/01/1989 - "Plasma zinc concentrations were determined in patients with sickle cell anemia and in disease free controls in the Eastern province of Saudi Arabia. " 10/01/1988 - "We examined the zinc status of 80 children with sickle cell disease (SCD) and 44 disease-free sibling controls aged 3 to 18 years. " 01/01/1987 - "RBC survival, zinc deficiency, and efficacy of zinc therapy in sickle cell disease." 01/01/2013 - "The authors recommend that more trials on zinc supplementation in thalassaemia and sickle cell disease be conducted given that the literature has shown the benefits of zinc in these types of diseases."
6.	GlobinsIBA 01/01/2012 - "Activation of γ-globin gene expression in adults is known to be therapeutic for sickle cell disease. " 07/01/2014 - "This placebo-controlled phase II study evaluated the pharmacodynamics, efficacy and safety of 2,2-dimethylbutyrate (HQK-1001), a fetal globin gene-inducing short-chain fatty acid derivative, administered orally at 15 mg/kg twice daily for 48 weeks in 76 subjects with sickle cell disease (SCD). " 07/01/2014 - "A double-blind, placebo-controlled phase II study of the efficacy and safety of 2,2-dimethylbutyrate (HQK-1001), an oral fetal globin inducer, in sickle cell disease." 11/01/2013 - "A dose-escalation phase IIa study of 2,2-dimethylbutyrate (HQK-1001), an oral fetal globin inducer, in sickle cell disease." 11/01/2012 - "A phase 1/2 trial of HQK-1001, an oral fetal globin inducer, in sickle cell disease."
7.	IronIBA 04/01/2008 - "Following iron therapy, an improvement in the Hb levels and ZPP/H ratios was observed in both sickle cell disorders and normal individual cases. " 09/01/2015 - "Liver iron concentration measurements by MRI in chronically transfused children with sickle cell anemia: baseline results from the TWiTCH trial." 08/01/2013 - "We evaluated all children with sickle cell disease (SCD) receiving chronic transfusion to identify chelation-naive subjects who had quantitative liver iron concentration (LIC) studies. " 01/01/2013 - "Our study showed that the quantification of hepatic iron on 3 T MRI in sickle cell disease patients correlates well with clinical blood test results and biopsy results. " 01/01/2012 - "The aim of this study was to estimate the frequency of the GSTM1 and GSTT1 genotypes in sickle cell disease patients and their effect on iron status. "
8.	Sickle HemoglobinIBA 01/01/1983 - "There is no specific therapy for sickle cell disease, and there is no evidence that sickle hemoglobin screening by conventional methods will lead to a significant reduction in the number of children with sickle cell disease. " 01/01/1992 - "Since cell shrinkage adversely influences red cell rheology, it is unlikely that this class of compounds, despite its ability to inhibit hemoglobin S polymerization, will prove useful in the treatment of sickle cell disease." 08/01/2008 - "Using this improved approach, we also generated hES-like cells from homozygous fibroblasts containing the sickle cell anemia mutation Hemoglobin Sickle. " 03/01/2015 - "Studies of hemoglobin S haplotypes in African subpopulations have potential implications for patient care and our understanding of genetic factors that have shaped the prevalence of sickle cell disease (SCD). " 01/01/2013 - "The goal of this study was to evaluate the quality of life of sickle cell disease patients (hemoglobin SS and SC) and their sociodemographic and clinical characteristics. "
9.	OxygenIBA 12/02/2010 - "We showed previously that pulmonary function and arterial oxygen saturation in NY1DD mice with sickle cell disease (SCD) are improved by depletion of invariant natural killer T (iNKT) cells or blockade of their activation. " 10/07/2010 - "We have developed a microfluidic approach to study the sickling of red blood cells associated with sickle cell anemia by rapidly varying the oxygen partial pressure within flowing microdroplets. " 02/01/2009 - "Studies using near-infrared spectroscopy (NIRS) have reported that patients with sickle cell disease (SCD) have lower cerebral oxygen saturation values (rSO2) than normal individuals. " 06/15/1993 - "This study provides evidence that Hb oxygen desaturation is not a universal finding among children with sickle cell disease and identifies factors associated with Hb oxygen desaturation. " 10/12/2015 - "Oxygen uptake efficiency slope (OUES) and excess post-exercise oxygen consumption (EPOC) are markers of physical fitness in the general population but have never been characterized in sickle cell anemia (SCA) where hematological and hemorheological properties are severely altered. "
10.	deferasirox FDA Link 01/01/2013 - "Safety and efficacy of 4 years of deferasirox treatment for sickle cell disease patients." 10/01/2009 - "Adherence to deferasirox in children and adolescents with sickle cell disease during 1-year of therapy." 05/01/2009 - "Eighty-one percent of patients treated with deferasirox had a diagnosis of sickle cell disease. " 01/01/2012 - "The efficacy and safety of a 2-year treatment with deferasirox was evaluated in 31 patients with sickle cell anemia and transfusional iron overload. " 01/01/2012 - "Two-year analysis of efficacy and safety of deferasirox treatment for transfusional iron overload in sickle cell anemia patients."

Therapies and Procedures

1.	Blood Transfusion (Blood Transfusions) 03/16/2013 - "No consensus exists on whether preoperative blood transfusions are beneficial in patients with sickle-cell disease. " 05/26/2001 - "Screening for, and appropriate management of, nocturnal hypoxaemia might be a safe and effective alternative to prophylactic blood transfusion for primary prevention of CNS events in sickle-cell disease." 12/01/2013 - "Blood transfusion safety in sickle cell anemia patients in Mali should be improved by the introduction of at least the technique for detecting the viral genome in the panel of screening tests and a policy of transfusions of blood units only from regular blood donors. " 02/11/1983 - "From the NIH: Improved methods of blood transfusion for patients with sickle cell anemia." 12/01/2015 - "The life-threatening complication raises the question about the indications of blood transfusion therapy for acute sickle cell disease complications, no randomized controlled trials being available to assess the role of blood transfusion in the acute setting. "
2.	Transplants (Transplant) 05/01/2015 - "Sickle cell disease (SCD) can be cured by allogeneic hematopoietic stem cell transplant. " 08/07/2014 - "Hematopoietic stem cell transplant (HSCT) is the only cure for sickle cell disease (SCD). " 12/01/2013 - "The only available cure for sickle cell disease (SCD) is hematopoietic stem cell transplant (HSCT). " 11/01/2012 - "We describe adolescents' and parents' interest in hematopoietic stem cell transplant (HSCT) as a cure for sickle cell disease (SCD) and factors associated with increased interest. " 09/30/2004 - "[Hematopoietic stem cell graft: cure for sickle cell anemia?]."
3.	Splenectomy 01/01/2006 - "Open splenectomy remains the gold standard for patients with sickle cell disease requiring splenectomy." 04/01/2015 - "In this study we examined the patients with sickle cell disease and partial splenectomy and compared the clinical and laboratory results with non-splenectomized patients. " 01/01/2013 - "However, there is a lack of evidence from trials showing that splenectomy improves survival and decreases morbidity in people with sickle cell disease. " 06/01/2008 - "The purpose of the study was to measure the effect of splenectomy on packed-cell transfusion requirement in children with sickle cell disease. " 01/01/2002 - "There is a need for a well-designed, adequately powered randomised controlled trial to assess the benefits and risks of splenectomy compared to transfusion programmes as a means of improving survival and decreasing mortality from ASS in people with sickle cell disease."
4.	Bone Marrow Transplantation (Transplantation, Bone Marrow) 02/01/1994 - "Features of our previous study and results of the present trial may be helpful in defining indications for bone marrow transplantation in children with sickle cell disease." 03/01/2002 - "A cure for murine sickle cell disease through stable mixed chimerism and tolerance induction after nonmyeloablative conditioning and major histocompatibility complex-mismatched bone marrow transplantation." 04/01/1993 - "There is as yet no cure for thalassaemia and sickle cell disease, except for bone marrow transplantation in a few selected cases. " 11/01/2010 - "This study compared traumatic stress symptoms of youth being considered for solid organ and bone marrow transplantation, youth diagnosed with human immunodeficiency virus, youth diagnosed with sickle cell disease and their parents. " 07/01/2007 - "The aim of this study is to describe the growth, pubertal development, and gonadal function of a cohort of 30 sickle cell disease children who underwent bone marrow transplantation. "
5.	Transplantation (Transplant Recipients) 10/01/2013 - "In utero hematopoietic cellular transplantation (IUHCT) holds great promise for the treatment of congenital diseases of cellular dysfunction such as sickle cell disease, immunodeficiency disorders and inherited metabolic disorders. " 11/01/2014 - "Blood and marrow transplantation is a curative therapy for patients with sickle cell disease yet this option is seldom used. " 11/01/2014 - "Blood and marrow transplantation for sickle cell disease: is less more?" 10/01/2013 - "It has been recognized that transplantation for sickle cell disease does not need to adhere strictly to the principles of transplantation for malignant disorders, such as achievement of full donor cell chimerism. " 05/01/2013 - "The goal is to make unrelated donor cord blood transplantation for sickle cell disease merit early consideration in patients who stand to benefit from this approach."