Laura Martina De Castro

Journal Article Am J Hematol · May 2014 In this study, the relationship of clinical differences among patients with sickle cell disease (SCD) was examined to understand the major contributors to early mortality in a contemporary cohort. Survival data were obtained for 542 adult subjects who were ... Full text Link to item Cite

An official American Thoracic Society clinical practice guideline: diagnosis, risk stratification, and management of pulmonary hypertension of sickle cell disease.

Journal Article Am J Respir Crit Care Med · March 15, 2014 BACKGROUND: In adults with sickle cell disease (SCD), an increased tricuspid regurgitant velocity (TRV) measured by Doppler echocardiography, an increased serum N-terminal pro-brain natriuretic peptide (NT-pro-BNP) level, and pulmonary hypertension (PH) di ... Full text Link to item Cite

Differences in pain management between hematologists and hospitalists caring for patients with sickle cell disease hospitalized for vasoocclusive crisis.

Journal Article Clin J Pain · March 2014 OBJECTIVES: Sickle cell disease (SCD) is a chronic disease characterized by multiple vaso-occlusive complications and is increasingly cared for by hospitalists. The purpose of this study is to examine differences in pain management between hematologists an ... Full text Link to item Cite

Emotional reactions to pain predict psychological distress in adult patients with Sickle Cell Disease (SCD).

Journal Article Int J Psychiatry Med · 2014 Differentiating somatic from emotional influences on the experience of chronic pain has been of interest to clinicians and researchers for many years. Although prior research has not well specified these pathways at the anatomical level, some evidence, bot ... Full text Link to item Cite

Patients welcome the Sickle Cell Disease Mobile Application to Record Symptoms via Technology (SMART).

Journal Article Hemoglobin · 2014 The widespread use of mobile phones among patients provides a unique opportunity for the development of mobile health intervention designed specifically for sickle cell disease, which will improve self-management as well as health care delivery. Our object ... Full text Link to item Cite

Evaluation of a train-the-trainer workshop on sickle cell disease for ED providers.

Journal Article J Emerg Nurs · November 2013 OBJECTIVE: (1) Determine the difference in pre-test and post-test knowledge scores for attendees of a train-the-trainer workshop and (2) determine the number of attendees who disseminated the content within 6 months of attending the workshop. METHODS: A 1- ... Full text Link to item Cite

Effect of propranolol as antiadhesive therapy in sickle cell disease.

Journal Article Clin Transl Sci · December 2012 Sickle red blood cells (SSRBCs) adhere to both endothelial cells (ECs) and the extracellular matrix. Epinephrine elevates cyclic adenosine monophosphate in SSRBCs and increases adhesion of SSRBCs to ECs in a β-adrenergic receptor and protein kinase A-depen ... Full text Link to item Cite

Complications of implantable venous access devices in patients with sickle cell disease.

Journal Article Am J Hematol · February 2012 Implantable venous access devices (VADs) are used in sickle cell disease (SCD) for patients with poor venous access to facilitate chronic blood transfusions and manage acute complications. We attempted to define the frequency of bloodstream infections (BSI ... Full text Open Access Link to item Cite

MYH9 and APOL1 are both associated with sickle cell disease nephropathy.

Journal Article Br J Haematol · November 2011 Renal failure occurs in 5-18% of sickle cell disease (SCD) patients and is associated with early mortality. At-risk SCD patients cannot be identified prior to the appearance of proteinuria and the pathobiology is not well understood. The myosin, heavy chai ... Full text Link to item Cite

Living with sickle cell disease: traversing 'race' and identity.

Journal Article Ethn Health · 2011 OBJECTIVES: Sickle cell disease (SCD) has a distinctive social history that continues to influence research and clinical practice related to the disease. Despite the historical link between SCD and concepts of 'race', there is limited empirical information ... Full text Link to item Cite

The association of optimism and perceived discrimination with health care utilization in adults with sickle cell disease.

Journal Article J Natl Med Assoc · November 2010 OBJECTIVE: We evaluated the effect of perceived discrimination, optimism, and their interaction on health care utilization among African American adults with sickle cell disease (SCD). METHODS: Measures of optimism and perceived discrimination were obtaine ... Full text Link to item Cite

Clinical and sociodemographic factors predict coping styles among adults with sickle cell disease.

Journal Article J Natl Med Assoc · November 2010 UNLABELLED: Sickle cell disease (SCD) presents a significant physical and psychological burden for persons with this chronic disease; however, little is known about how individuals with SCD--adult patients in particular--cope with disease-related stressors ... Full text Link to item Cite

The relationship of opioid analgesia to quality of life in an adult sickle cell population

Journal Article Health Outcomes Research in Medicine · July 1, 2010 Background: Pain is a limiting factor in the daily life activities of sickle cell disease (SCD) patients. Although opioid analgesics are widely used, to date there have been no studies on the relationship of daily opioid use to quality of life (QoL) measur ... Full text Cite

The risks and benefits of long-term use of hydroxyurea in sickle cell anemia: A 17.5 year follow-up.

Journal Article Am J Hematol · June 2010 A randomized, controlled clinical trial established the efficacy and safety of short-term use of hydroxyurea in adult sickle cell anemia. To examine the risks and benefits of long-term hydroxyurea usage, patients in this trial were followed for 17.5 years ... Full text Link to item Cite

Neuropsychological dysfunction and neuroimaging abnormalities in neurologically intact adults with sickle cell anemia.

Journal Article JAMA · May 12, 2010 CONTEXT: Sickle cell anemia (SCA) is a chronic illness causing progressive deterioration in quality of life. Brain dysfunction may be the most important and least studied problem affecting individuals with this disease. OBJECTIVE: To measure neurocognitive ... Full text Link to item Cite

Placenta growth factor in sickle cell disease: association with hemolysis and inflammation.

Journal Article Blood · March 11, 2010 Placenta growth factor (PlGF) is released by immature erythrocytes and is elevated in sickle cell disease (SCD). Previous data generated in vitro suggest that PlGF may play a role in the pathophysiology of SCD-associated pulmonary hypertension (PHT) by ind ... Full text Link to item Cite

Cardiopulmonary complications leading to premature deaths in adult patients with sickle cell disease.

Journal Article Am J Hematol · January 2010 Sickle cell disease (SCD) is associated with early mortality. We sought to determine the incidence, cause, and risk factors for death in an adult population of patients with SCD. All patients aged >/=18 years seen at the Adult Sickle Cell Center at Duke Un ... Full text Link to item Cite

Depression, suicidal ideation, and attempts in black patients with sickle cell disease.

Journal Article J Natl Med Assoc · November 2009 There is a strong relationship between suicidal ideation, suicide attempts, and depression. Rates of successful suicides are relatively high among the chronically ill compared to other populations but are reduced with treatment. Depression and suicide rate ... Full text Link to item Cite

Exposure to hydroxyurea and pregnancy outcomes in patients with sickle cell anemia

Journal Article Journal of the National Medical Association · 2009 The Multicenter Study of Hydroxyurea in Sickle Cell Anemia (MSH) was a randomized double-blind placebo-controlled trial to test whether hydroxyurea could reduce the rate of painful crises in adults who had at least 3 painful crises per year. Because hydrox ... Cite

Surgical and obstetric outcomes in adults with sickle cell disease.

Journal Article Am J Med · October 2008 BACKGROUND: Sickle cell disease patients are more likely than the general population to undergo surgery and usually do so at a younger age. Female sickle cell disease patients also have special gynecological and obstetric issues related to their disease. M ... Full text Link to item Cite

Disparity in the management of iron overload between patients with sickle cell disease and thalassemia who received transfusions.

Journal Article Transfusion · September 2008 BACKGROUND: Transfusion therapy is frequently used to prevent morbidity in sickle cell disease (SCD), and subsequent iron overload is common. The objective of this study was to evaluate the current standard of care in monitoring iron overload and related c ... Full text Link to item Cite

Morbidity associated with sickle cell disease in pregnancy.

Journal Article Am J Obstet Gynecol · August 2008 OBJECTIVE: The purpose of this study was to identify morbidity that is associated with sickle cell disease (SCD) in pregnancy. STUDY DESIGN: The Nationwide Inpatient Sample from the Healthcare Cost and Utilization Project of the Agency for Healthcare Resea ... Full text Link to item Cite

Identification of genetic polymorphisms associated with risk for pulmonary hypertension in sickle cell disease.

Journal Article Blood · June 15, 2008 Up to 30% of adult patients with sickle cell disease (SCD) will develop pulmonary hypertension (pHTN), a complication associated with significant morbidity and mortality. To identify genetic factors that contribute to risk for pHTN in SCD, we performed ass ... Full text Link to item Cite

Efficacy and safety of the Gardos channel blocker, senicapoc (ICA-17043), in patients with sickle cell anemia.

Journal Article Blood · April 15, 2008 Senicapoc, a novel Gardos channel inhibitor, limits solute and water loss, thereby preserving sickle red blood cell (RBC) hydration. Because hemoglobin S polymerization is profoundly influenced by intracellular hemoglobin concentration, senicapoc could imp ... Full text Link to item Cite

beta(2)-Adrenergic receptor and adenylate cyclase gene polymorphisms affect sickle red cell adhesion.

Journal Article Br J Haematol · April 2008 Sickle red cell (SS RBC) adhesion is thought to contribute to sickle cell disease (SCD) pathophysiology. SS RBC adhesion to laminin increases in response to adrenaline stimulation of beta(2)-adrenergic receptors (beta(2)ARs) and adenylate cyclase (ADCY6), ... Full text Link to item Cite

Pulmonary hypertension associated with sickle cell disease: clinical and laboratory endpoints and disease outcomes.

Journal Article Am J Hematol · January 2008 Screening for pulmonary hypertension (pHTN) has not yet become routine in sickle cell disease (SCD), despite clinical evidence of its high prevalence and associated mortality. Our objectives are to identify clinical conditions and laboratory findings predi ... Full text Link to item Cite

Role and regulation of sickle red cell interactions with other cells: ICAM-4 and other adhesion receptors.

Journal Article Transfus Clin Biol · 2008 Erythrocytes containing primarily hemoglobin S (SS RBCs) are abnormally adherent. We now know that SS RBCs express numerous adhesion molecules, and that many of these can undergo activation. SS RBCs exposed briefly to epinephrine show markedly increased ad ... Full text Link to item Cite

Neuropsychological assessment, neuroimaging, and neuropsychiatric evaluation in pediatric and adult patients with sickle cell disease (SCD).

Journal Article Neuropsychiatr Dis Treat · December 2007 Traditionally, neuropsychological deficits due to Sickle Cell Disease (SCD) have been understudied in adults. We have begun to suspect, however, that symptomatic and asymptomatic Cerebrovascular Events (CVE) may account for an alarming number of deficits i ... Full text Link to item Cite

Genetic polymorphisms associated with priapism in sickle cell disease.

Journal Article Br J Haematol · May 2007 Priapism occurs in 30-45% of male patients with sickle cell disease (SCD), but the possible influence of genetic risk factors on the incidence of priapism is not well understood. We examined genetic polymorphisms in 199 unrelated, adult (>18 years), male p ... Full text Link to item Cite

Morbidity and mortality in chronically transfused subjects with thalassemia and sickle cell disease: A report from the multi-center study of iron overload.

Journal Article Am J Hematol · April 2007 A natural history study was conducted in 142 Thalassemic (Thal), 199 transfused Sickle Cell Disease (Tx-SCD, n = 199), and 64 non-Tx-SCD subjects to describe the frequency of iron-related morbidity and mortality. Subjects recruited from 31 centers in the U ... Full text Link to item Cite

Prolonged dark adaptation changes connexin expression in the mouse retina.

Journal Article J Neurosci Res · May 15, 2006 In the retina, ambient light levels influence the cell coupling provided by gap junction (GJ) channels, to compensate the visual function for various lighting conditions. However, the effects of ambient light levels on expression of connexins (Cx), the pro ... Full text Link to item Cite

Parental substance abuse, reports of chronic pain and coping in adult patients with sickle cell disease.

Journal Article J Natl Med Assoc · March 2006 There is increasing interest from a social learning perspective in understanding the role of parental factors on adult health behaviors and health outcomes. Our review revealed no studies, to date, that have evaluated the effects of parental substance abus ... Link to item Cite

Moderate chronic pain, weight and dietary intake in African-American adult patients with sickle cell disease.

Journal Article J Natl Med Assoc · December 2005 In this exploratory study, we evaluated weight status and dietary intake patterns during painful episodes in adult patients with SCD. Specifically, we explored the relation between pain severity and body mass index (BMI), and we tested the hypothesis that ... Link to item Cite

Religiosity/spirituality and pain in patients with sickle cell disease.

Journal Article J Nerv Ment Dis · April 2005 Religion/spirituality has been identified by individuals with sickle cell disease (SCD) as an important factor in coping with stress and in determining quality of life. Research has demonstrated positive associations between religiosity/spirituality and be ... Full text Link to item Cite

A brief review of the pathophysiology, associated pain, and psychosocial issues in sickle cell disease.

Journal Article Int J Behav Med · 2005 Featured Publication Sickle cell disease (SCD) is the most common genetic disorder of the blood. The disease produces significantly abnormal hemoglobin (Hgb) molecules in red blood cells (RBCs). The sickling of RBCs occurs when partially or totally deoxygenated Hgb molecules d ... Full text Link to item Cite

Epinephrine acts through erythroid signaling pathways to activate sickle cell adhesion to endothelium via LW-alphavbeta3 interactions.

Journal Article Blood · December 1, 2004 The possible role of physiologic stress hormones in enhancing adhesion of sickle erythrocytes (SS RBCs) to endothelial cells (ECs) in sickle cell disease (SCD) has not been previously explored. We have now found that up-regulation of intracellular cyclic a ... Full text Link to item Cite

B-CAM/LU expression and the role of B-CAM/LU activation in binding of low- and high-density red cells to laminin in sickle cell disease.

Journal Article Am J Hematol · February 2004 Featured Publication Red blood cells from patients with sickle cell disease (SS RBC) adhere to laminin and over-express the high-affinity laminin receptor basal cell adhesion molecule/Lutheran protein (B-CAM/LU). This receptor has recently been shown to undergo activation in v ... Full text Link to item Cite

Effect of Hydroxyurea on Mortality and Morbidity in Adult Sickle Cell Anemia: Risks and Benefits Up to 9 Years of Treatment

Journal Article Journal of the American Medical Association · 2003 Context: Hydroxyurea increases levels of fetal hemoglobin (HbF) and decreases morbidity from vaso-occlusive complications in patients with sickle cell anemia (SCA). High HbF levels reduce morbidity and mortality. Objective: To determine whether hydroxyurea ... Full text Cite

Prevalence of hepatitis c in adults with sickle cell disease

Journal Article Blood · January 1, 2000 Hepatitis C (HCV) is a common viral infection that can lead to chronic hepatitis, liver cirrhosis and hepatocellular carcinoma. This chronic disease is transmitted by various routes, with blood product transfusions being the most frequent (Risk is: 1/100,0 ... Cite

Differences in platelet α-granule release between normals and immune thrombocytopenic patients and between young and old platelets

Journal Article Thrombosis and Haemostasis · 1998 The risk of serious bleeding in patients with immune thrombocytopenic purpura (ITP) appears to be less than in comparably thrombocytopenic patients with megakaryocytic hypoplasia. It has been proposed that this difference is due to enhanced hemostatic acti ... Cite

Phosphonylmethoxyalkyl purine and pyrimidine derivatives for treatment of opportunistic cytomegalovirus and herpes simplex virus infections in murine AIDS.

Journal Article Antiviral Res · July 1991 Murine acquired immunodeficiency syndrome (MAIDS) was induced in C57BL/6 mice following infection with the LP-BM5 retrovirus complex. Infected mice developed splenomegaly, lymphadenopathy and loss of B- and T-cell functions 100 days after virus inoculation ... Full text Link to item Cite