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Journal articles on the topic 'SS sickle cell disease'

Author: Grafiati

Published: 5 June 2025

Last updated: 2 August 2025

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1

Parrish, James M., Paul A. Page, David Cohen, et al. "Prebypass Pheresis and Red Blood Cell Exchange in a Patient with Homozygous SS Sickle Cell Disease Undergoing Cardiopulmonary Bypass: A Case Report." Journal of ExtraCorporeal Technology 26, no. 3 (1994): 143–51. http://dx.doi.org/10.1051/ject/1994263143.

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Sickle cell disease was first described by Herrick in 1910. This disease involves an abnormality of the hemoglobin molecule which, under certain conditions, causes the red cell to take on a sickle shape. This abnormal shape and hemoglobin prevent the red cell from performing the normal respiratory functions and interfere with the normal flow through the circulatory system. Individuals demonstrate either a homozygous (dominant) SS or a heterozygous Ss state. Clinical symptoms for patients with SS disease are most often characterized by recurrent painful crises, sequestration of sickled cells, c

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2

Adhikari, Ram Chandra, T. B. Shrestha, R. B. Shrestha, R. C. Subedi, K. P. Parajuli, and S. Dali. "SICKLE CELL DISEASE - CASE REPORTS." Journal of Nepal Medical Association 42, no. 145 (2003): 36–38. http://dx.doi.org/10.31729/jnma.715.

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ABSTRACTSickle cell diseases are inherited hematological diseases, prevalent in certain parts of the world. We reporttwo cases of sickle cell diseases, first being sickle cell b-thalassaemia and second homozygous sickle celldisease (SS). Our first case was 5 year old boy presenting with hemolytic anaemia & hepatosplenomegalyhaving sickle cell b-thalassaemia disease . Second case was 17 years female presenting with hemolyticanaemia & joint pain having homozygous sickle cell disease.Key Words: Homozygous sickle cell disease, sickle cell b - thalassaemia, hemoglobin electrophoresis.

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3

Koshy, M., SJ Weiner, ST Miller, et al. "Surgery and anesthesia in sickle cell disease. Cooperative Study of Sickle Cell Diseases." Blood 86, no. 10 (1995): 3676–84. http://dx.doi.org/10.1182/blood.v86.10.3676.bloodjournal86103676.

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From 1978 to 1988, The Cooperative Study of Sickle Cell Disease observed 3,765 patients with a mean follow-up of 5.3 +/- 2.0 years. One thousand seventy-nine surgical procedures were conducted on 717 patients (77% sickle cell anemia [SS], 14% sickle hemoglobin C disease [SC], 5.7% S beta zero thalassemia, 3% S beta zero + thalassemia). Sixty-nine percent had a single procedure, 21% had two procedures, and the remaining 11% had more than two procedures during the study follow- up. The most frequent procedure was abdominal surgery for cholecystectomy or splenectomy (24% of all surgical procedure

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4

Gill, FM, LA Sleeper, SJ Weiner, et al. "Clinical events in the first decade in a cohort of infants with sickle cell disease. Cooperative Study of Sickle Cell Disease [see comments]." Blood 86, no. 2 (1995): 776–83. http://dx.doi.org/10.1182/blood.v86.2.776.bloodjournal862776.

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Within the Cooperative Study of Sickle Cell Disease, 694 infants with confirmed sickle cell disease were enrolled at less than 6 months of age. Information about the nature and frequency of complications was collected prospectively over a 10-year period. Painful crises and acute chest syndrome were the most common sickle cell-related events in homozygous sickle cell anemia (SS), hemoglobin SC disease (SC), and S beta thalassemia patients (overall incidence in SS patients of 32.4 and 24.5 cases per 100 person-years, respectively). Bacteremia occurred most frequently in SS children under 4 years

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5

Hlouedjè, Houénoukpo Wilfried, Jacques Ezéchiel Lokonon, Maximin Sènou, et al. "Some markers of inflammation in patients with sickle cell disease at Zou-Collines departmental hospital in Benin." International Journal of Research in Medical Sciences 10, no. 6 (2022): 1219. http://dx.doi.org/10.18203/2320-6012.ijrms20221475.

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Background: Sickle cell disease was a genetic pathology of the red blood cell, which caused systemic functional and tissue damage. The present work aimed to study some biomarkers of inflammation and renal function in sickle cell patients.Methods: The biochemical and inflammatory markers were assayed with a spectrophotometer and the hemogram was performed on a hematology analyzer in SS homozygous sickle cell subjects and controls with AA phenotypes.Results: The male sex (63.49%) and the age group 5-18 years (58.73%) were predominant among the SS subjects of the study population. Blood urea and

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6

Boisson, Camille, Minke A. E. Rab, Elie Nader, et al. "Effects of Genotypes and Treatment on Oxygenscan Parameters in Sickle Cell Disease." Cells 10, no. 4 (2021): 811. http://dx.doi.org/10.3390/cells10040811.

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(1) Background: The aim of the present study was to compare oxygen gradient ektacytometry parameters between sickle cell patients of different genotypes (SS, SC, and S/β+) or under different treatments (hydroxyurea or chronic red blood cell exchange). (2) Methods: Oxygen gradient ektacytometry was performed in 167 adults and children at steady state. In addition, five SS patients had oxygenscan measurements at steady state and during an acute complication requiring hospitalization. (3) Results: Red blood cell (RBC) deformability upon deoxygenation (EImin) and in normoxia (EImax) was increased,

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7

Raghuwanshi, Pranav Kumar, and Somnath Singh Raghuvanshi. "Vascular endothelial dysfunction in sickle cell disease by brachial artery flow mediated dilatation." Asian Journal of Medical Sciences 5, no. 3 (2014): 105–7. http://dx.doi.org/10.3126/ajms.v5i3.9445.

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Objective: The present study was conducted aiming to assess endothelial function in sickle cell disease (SS), sickle cell trait(SA) and compare to endothelial dysfunction between sickle cell anemia (SS), sickle cell trait (SA) cases and control (AA) patients to evaluate correlation of endothelial dysfunction. Methods: The study population comprised of, total 25 cases having sickle cell disease and sickle cell trait and 25 age and sex matched normal control. Endothelial dysfunction as assessed by brachial artery flow mediated dilatation by colour Doppler (non-invasive method)by using Siemens So

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8

Pranav Kumar Raghuwanshi and Somnath Singh Raghuvanshi. "Vascular endothelial dysfunction in sickle cell disease by brachial artery flow mediated dilatation." Asian Journal of Medical Sciences 5, no. 3 (2014): 105–7. https://doi.org/10.71152/ajms.v5i3.3338.

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Objective: The present study was conducted aiming to assess endothelial function in sickle cell disease (SS), sickle cell trait(SA) and compare to endothelial dysfunction between sickle cell anemia (SS), sickle cell trait (SA) cases and control (AA) patients to evaluate correlation of endothelial dysfunction. Methods: The study population comprised of, total 25 cases having sickle cell disease and sickle cell trait and 25 age and sex matched normal control. Endothelial dysfunction as assessed by brachial artery flow mediated dilatation by colour Doppler (non-invasive method)by using Siemens So

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9

Quinn, Charles T., Zora R. Rogers, and George R. Buchanan. "Survival of children with sickle cell disease." Blood 103, no. 11 (2004): 4023–27. http://dx.doi.org/10.1182/blood-2003-11-3758.

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Abstract Contemporary survival data are not available for children with sickle cell disease (SCD). The few previous childhood SCD cohort studies do not reflect the benefits of modern therapy. We defined an inception cohort of newborns with sickle cell anemia (SS), sickle-β°-thalassemia (S β°), sickle-hemoglobin C disease (SC), or sickle-β+-thalassemia (Sβ+) who were identified by newborn screening and followed for up to 18 years. The incidence of death and stroke were calculated. Overall survival, SCD-related survival (considering only SCD-related deaths), and strokefree survival were determin

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10

Nabors, Nina A., and Angela K. Freymuth. "Attention Deficits in Children with Sickle Cell Disease." Perceptual and Motor Skills 95, no. 1 (2002): 57–67. http://dx.doi.org/10.2466/pms.2002.95.1.57.

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Previous research has suggested that children with sickle cell disease may exhibit cognitive deficits even in the absence of direct cerebrovascular involvement (stroke). This study was designed to assess specific attentional deficits in children with sickle cell disease. 12 children with sickle cell disease (Hb SS) with a prior history of stroke, 14 children with sickle cell disease (Hb SS) without evidence of stroke, and 13 similar aged siblings (Hb AA or Hb AS) were compared on measures of attention, intellectual functioning, achievement, and adaptive Functioning, Significant differences wer

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11

Castro, O., DJ Brambilla, B. Thorington, et al. "The acute chest syndrome in sickle cell disease: incidence and risk factors. The Cooperative Study of Sickle Cell Disease." Blood 84, no. 2 (1994): 643–49. http://dx.doi.org/10.1182/blood.v84.2.643.643.

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Abstract The acute chest syndrome (ACS), a pneumonia-like illness in sickle cell patients, is one of the most frequent causes of their morbidity and hospitalizations. Repeated ACS events may predict the development of chronic lung disease. ACS is reported as a frequent cause of death in these patients. We examine here the incidence and risk factors of ACS in 3,751 patients with sickle cell disease who were observed prospectively for at least 2 years (19,867 patient-years [pt-yrs]) as part of a multicenter national study group. The ACS, defined by a new pulmonary infiltrate on x-ray, occurred a

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12

Castro, O., DJ Brambilla, B. Thorington, et al. "The acute chest syndrome in sickle cell disease: incidence and risk factors. The Cooperative Study of Sickle Cell Disease." Blood 84, no. 2 (1994): 643–49. http://dx.doi.org/10.1182/blood.v84.2.643.bloodjournal842643.

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The acute chest syndrome (ACS), a pneumonia-like illness in sickle cell patients, is one of the most frequent causes of their morbidity and hospitalizations. Repeated ACS events may predict the development of chronic lung disease. ACS is reported as a frequent cause of death in these patients. We examine here the incidence and risk factors of ACS in 3,751 patients with sickle cell disease who were observed prospectively for at least 2 years (19,867 patient-years [pt-yrs]) as part of a multicenter national study group. The ACS, defined by a new pulmonary infiltrate on x-ray, occurred at least o

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13

Saganuwan, Saganuwan Alhaji. "The Pattern of Sickle Cell Disease in Sickle Cell Patients from Northwestern Nigeria." Clinical Medicine Insights: Therapeutics 8 (January 2016): CMT.S38164. http://dx.doi.org/10.4137/cmt.s38164.

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Sickle cell disease is caused due to a genetic disorder, which accounts for people dying at an early age in Nigeria. A retrospective study of sickle cell disease patients was carried out with a view to determining the disease pattern in sickle cell patients from the Northwestern Nigeria. Case notes of 319 sickle cell patients were collected and reviewed retrospectively. The prevalence of sickle cell trait, comorbidity of sickle cell disease and malaria, and the effects of sickle cell disease and age on the weight and hematological parameters of sickle cell patients were determined and analyzed

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14

Swensen, Jeffrey J., Archana M. Agarwal, Jose M. Esquilin, et al. "Sickle cell disease resulting from uniparental disomy in a child who inherited sickle cell trait." Blood 116, no. 15 (2010): 2822–25. http://dx.doi.org/10.1182/blood-2010-05-284331.

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Abstract Sickle cell disease (SCD) is a classic example of a disorder with recessive Mendelian inheritance, in which each parent contributes one mutant allele to an affected offspring. However, there are exceptions to that rule. We describe here the first reported case of conversion of inherited sickle cell trait to SCD by uniparental disomy (UPD) resulting in mosaicism for SS and AS erythrocytes. A 14-year-old boy presented with splenomegaly and hemolysis. Although his father has sickle cell trait, his mother has no abnormal hemoglobin (Hb). DNA sequencing, performed to rule out Hb S/β-thalas

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15

Yee, Donald L., Rachel M. Edwards, Brigitta U. Mueller, and Jun Teruya. "Thromboelastographic and Hemostatic Characteristics in Pediatric Patients With Sickle Cell Disease." Archives of Pathology & Laboratory Medicine 129, no. 6 (2005): 760–65. http://dx.doi.org/10.5858/2005-129-760-tahcip.

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Abstract Context.—Patients with sickle cell disease suffer from a variety of vaso-occlusive events that may be related to activation of the hemostatic system. Thromboelastography assesses the functionality of this system from a global standpoint and has demonstrated some utility in detecting hypercoagulable states in varied clinical settings, but it has not been systematically evaluated in patients with sickle cell disease. Objective.—To characterize the findings of thromboelastography in patients with sickle cell disease during periods of steady state and illness, to compare these results wit

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16

Bhagat, Sanjana, Amar Singh Thakur, and Pradeep Kumar Patra. "Association between XmnI Polymorphism and HbF Level in Sickle Cell Disease Patients from Chhattisgarh." International Journal of Biomedical Science 8, no. 1 (2012): 36–39. http://dx.doi.org/10.59566/ijbs.2012.8036.

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The γG-158 (C-T) polymorphism plays important function in the disease severity of sickle cell anemia.The XmnI restriction site at -158 position of the γG-gene is associated with increased expression of the γG-globin gene and higher production of HbF. This study aims to determine the frequency of the different genotypes of the γG Xmn I polymorphism in sickle cell anemia and sickle cell trait patients in Chhattisgarh and its association with high HbFlevel. The Xmn1 polymorphic site was determined by PCR-RFLP procedure. XmnI polymorphism were studied in 100 sickle cell patients (SS), 50 sickle ce

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17

Dawkins, Fitzroy, Hilary Ufearo, Jeremy Pantin, et al. "Cancer Incidence in Sickle Cell Disease. Howard University, 1996–2004." Blood 106, no. 11 (2005): 3788. http://dx.doi.org/10.1182/blood.v106.11.3788.3788.

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Abstract There are no prospectively collected data on cancer incidence and mortality in patients with sickle cell disease. This information would be a useful baseline for cancer surveillance, particularly in sickle patients exposed to potentially carcinogenic interventions such as hydroxurea administration and stem cell transplantation. A review of cancer cases among 696 adult sickle cell patients (mean age 28.8 y) at Howard University for the years 1986–1995 (2,684 patient-years, pt. yrs.) estimated the cancer incidence and mortality at 1.74 and 1.04 per 1000 pt. yrs, respectively (Am J Hemat

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18

Jain, Dipty, Vinit Warthe, Roshan Colah, and Graham Roger Serjeant. "Sickle Cell Disease in Central India: High Prevalence of Sickle/Beta Thalassemia and Severe Dsiease Phenotype." Blood 126, no. 23 (2015): 4588. http://dx.doi.org/10.1182/blood.v126.23.4588.4588.

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Abstract Objectives: To assess the clinical, haematological and molecular features of sickle cell disease in central India where the disease has been reported to be more severe than the mild clinical course usually observed in the Asian haplotype of homozygous sickle cell (SS) disease. Methods: A cross-sectional assessment of 91 consecutive patients with sickle cell disease attending clinics at the Akola Government Medical College, Akola, Maharastra State, India. Results: Of the 91 patients, who were predominantly of the scheduled caste community, 49 had SS disease, 6 had sickle cell-HbD Punja

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19

Memon, Mohd Jafar, Renuka Gahine, Nighat Hussain, and Yashita Gupta. "Influence of glucose-6 phosphate dehydrogenase (G6-PD) deficiency upon clinico-haematological and biochemical expression of patients with sickle cell disease." International Journal of Advances in Medicine 4, no. 2 (2017): 573. http://dx.doi.org/10.18203/2349-3933.ijam20171063.

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Background: According to world health organization, G6PD deficiency constitute 7.5% of world population as carriers whereas 2.9% as deficient. It has been reported from India that the prevalence varies from 0-27% in different caste, ethnic and linguistic groups. Various studies have revealed strong interaction between G6PD deficiency and sickle cell genes on one hand and environment on the other, one may therefore expect changes in their frequencies over a period of time in the population. We studied the influence of G6PD deficiency upon the clinico-haematological and biochemical expression of

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20

Verma, Ashish, Basant Maheshwari, and Debapriya Rath. "Hematological profile of sickle cell disease in Chhattisgarh." International Journal of Basic & Clinical Pharmacology 9, no. 4 (2020): 552. http://dx.doi.org/10.18203/2319-2003.ijbcp20201175.

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Background: Sickle cell disease hemoglobinopathy gets inherited in autosomal recessive pattern. In sickle cell disease substitution of amino acid valine for glutamic acid at the sixth position on beta globin chain takes place resulting in sickled hemoglobin which is a hemoglobin tetramer.Methods: A prospective observational study was conducted in the Sickle Cell Institute, Raipur, India, and Department of Pharmacology in collaboration with Department of Biochemistry, Pt. J.N.M. Medical College, Raipur, Chhattisgarh, India, from February 2018 to June 2018. Patients included were in the steady s

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21

Tshibumbu, Kabeya Edouard, Ilunga Eric Kasamba, Anzwal Philomene Lungu, et al. "Sickle cell disease and assessment of energy metabolism and serum cortisol in Lubumbashi." World Journal of Advanced Research and Reviews 22, no. 3 (2024): 2082–88. https://doi.org/10.5281/zenodo.14769932.

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<strong>Introduction</strong>: Sickle cell disease is an inherited red blood cells disorder which leads to oxidative stress and resulting in the disturbance of energy metabolism. <strong>Objective</strong>: The aim of this study was to evaluate changes in serum glucose, triglycerides and cortisol concentrations in subjects with sickle cell disease in the city of Lubumbashi. <strong>Methods</strong>: This is a 1-1 matched case-control study involving 64 subjects with sickle cell disease attending the sickle cell care center (C-fare) and 64 subjects without sickle cell disease taken as controls.

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22

Kamble, Milind B., and Vidhya S. Totewad. "Renal manifestations of sickle cell disease in children." International Journal of Contemporary Pediatrics 7, no. 7 (2020): 1524. http://dx.doi.org/10.18203/2349-3291.ijcp20202609.

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Background: Sickle cell disease prevalence is more in central part of India. This study tries to find extent of renal involvement, risk factors and screening tests in sickle cell disease.Methods: Study was Cross sectional observational study. Demographic and clinical findings were recorded. Renal function tests like serum creatinine, blood urea nitrogen, eGFR were studied. Presence of microalbuminuria was checkeed. Ultrasonography abdomen was done to see the texture and corticomedullary differentiation of kidney.Results: Total 143 patients were studied. Out of which, 117 homozygous (SS type),

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23

Gilli, Simone, Fernando V. Pericole, Luis Gustavo Fernandes, et al. "Th17/Treg Imbalance in Sickle Cell Disease and Hydroxyurea Therapy." Blood 118, no. 21 (2011): 1050. http://dx.doi.org/10.1182/blood.v118.21.1050.1050.

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Abstract Abstract 1050 Background: Tregs and proinflammatory Th17 cells have been described as two distinct subsets from Th1 and Th2 cells and have opposite effects in autoimmunity and inflammation. Th17/Treg balance controls inflammation and is important in the pathogenesis of autoimmune, infectious and chronic diseases. In view of growing evidence linking sickle cell disease (SS) and chronic inflammation and the potential anti-inflammatory role of Tregs cells, we hypothesized that SS is associated with decreased Treg subpopulation and consequent T cell dysfunction. Furthermore, the effects o

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Okwan-Duodu, Derick, David R. Archer, and W. Robert Taylor. "Aged Neutrophils in Sickle Cell Disease Impede Ischemic Repair." Blood 132, Supplement 1 (2018): 3688. http://dx.doi.org/10.1182/blood-2018-99-119384.

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Abstract INTRODUCTION: Repeated bout of tissue ischemia is a hallmark of sickle cell disease (SCD). Neutrophils are critical first responder cells that initiate host response to sterile injury, including ischemia. Of the numerous neutrophils in circulation, a small proportion undergo physiologic changes (termed aging) which directs their homing to the bone marrow for clearance. How neutrophil homeostasis is regulated in sickle cell disease (SCD) to influence ischemic repair is unknown. HYPOTHESIS: We hypothesized that SCD may alter the homeostasis and function of aged neutrophil. METHODS: Aged

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Finnegan, Eileen M., Aslihan Turhan, Jennifer Gaines, David E. Golan, and Gilda Barabino. "Effect of Hydroxyurea Treatment on In Vitro Adhesion of Sickle Erythrocytes to Sickle Leukocyte Subpopulations." Blood 104, no. 11 (2004): 362. http://dx.doi.org/10.1182/blood.v104.11.362.362.

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Abstract Microvascular vaso-occlusion in sickle cell disease is thought to involve adhesive interactions among erythrocytes (RBCs), leukocytes and vascular endothelial cells. Recent studies have demonstrated the presence of a significant inflammatory response in sickle cell disease, including changes in the cell surface adhesion molecules that mediate cell-cell interactions in the microvasculature. In this study, we used a parallel-plate flow chamber assay to determine the subpopulations of leukocytes that are involved in sickle leukocyte-RBC interactions. We also studied the effect of treatme

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Saraf, Santosh L., Justin R. Sysol, Alexandru Susma, et al. "Progressive Glomerular Damage in Sickle Cell Trait and Sickle Cell Anemia Mouse Models." Blood 128, no. 22 (2016): 3637. http://dx.doi.org/10.1182/blood.v128.22.3637.3637.

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Abstract Homozygous inheritance of the hemoglobin S mutation (Hb SS; sickle cell anemia) affects 1 in 500 African Americans and is consistently associated with an increased risk for kidney disease, although the mechanisms are poorly understood. Heterozygous inheritance (Hb AS; sickle cell trait) affects 1 in 8 African Americans and has also been associated with an increased risk for kidney disease in some, but not all cohorts. We first investigated whether inheritance of the Hb S mutation resulted in incremental kidney damage in Hb AS and Hb SS mice compared to Hb AA mice using transgenic sick

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Haverfield, Eden V., Colin A. McKenzie, Terrence Forrester, et al. "UGT1A1 variation and gallstone formation in sickle cell disease." Blood 105, no. 3 (2005): 968–72. http://dx.doi.org/10.1182/blood-2004-02-0521.

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AbstractPigment gallstones are a common clinical complication of sickle cell (SS) disease. Genetic variation in the promoter of uridine diphosphate (UDP)–glucuronosyltransferase 1A1 (UGT1A1) underlies Gilbert syndrome, a chronic form of unconjugated hyperbilirubinemia, and appears to be a risk factor for gallstone formation. We investigated the association between UGT1A1 (TA)n genotype, hyperbilirubinemia, and gallstones in a sample of Jamaicans with SS disease. Subjects were from the Jamaican Sickle Cell Cohort Study (cohort sample, n = 209) and the Sickle Cell Clinic at the University of the

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28

Dei-Adomakoh, Yvonne A., Jane S. Afriyie-Mensah, Audrey Forson, Martin Adadey, Thomas A. Ndanu, and Joseph K. Acquaye. "Lung Function Abnormalities in Sickle Cell Anaemia." Advances in Hematology 2019 (April 1, 2019): 1–8. http://dx.doi.org/10.1155/2019/1783240.

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Background. Abnormalities in lung function tests have been shown to commonly occur in a majority of patients with sickle cell disease (SCD) even at steady state. The prevalence and pattern of these lung function abnormalities have been described in other populations but this is unknown among our sickle cell cohort. There is generally little information available on risk factors associated with the lung function abnormalities and its relevance in patient care. Method. This was an analytical cross-sectional study involving 76 clinically stable, hydroxyurea-naive adult Hb-SS participants and 76 n

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Patel, Saahil, Fiona Busser, Caitlin Lu, Jennifer Light, and Marwah Farooqui. "Heavy Menstrual Bleeding in Adult Women with Sickle Cell Disease." Blood 144, Supplement 1 (2024): 3897. https://doi.org/10.1182/blood-2024-211904.

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Sickle cell disease (SCD) is an inherited hemoglobin disorder. It is characterized by the presence of abnormal hemoglobin S. SCD leads to chronic hemolysis, vaso-occlusive episodes, various organ complications, and varying degrees of anemia. The prevalence of heavy menstrual bleeding (HMB) is 10-35% in the United States, with a higher prevalence in the Black population. Women with SCD often face challenges with their menstrual and reproductive health. Earlier studies have reported heavy menstrual bleeding as well as dysmenorrhea in adolescent women with sickle cell disease; however, there is l

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Wandersee, Nancy J., Sandra L. Holzhauer, Dawn M. Retherford, Thomas D. Foster, and Cheryl A. Hillery. "Evidence for Transient Acute Liver Injury in Mouse Models of Sickle Cell Disease during Steady State Health." Blood 124, no. 21 (2014): 1373. http://dx.doi.org/10.1182/blood.v124.21.1373.1373.

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Abstract Individuals with sickle cell disease experience episodes of acute vaso-occlusive complications interspersed by periods of feeling well and apparent health during “steady state”. It is likely that episodic and subclinical sickling and vaso-occlusion occur on an ongoing basis even during periods of steady state. In order to determine whether mice with sickle disease have acute vaso-occlusion sufficient for organ injury at apparent steady state health, we chose to study 2 mouse models of severe sickle cell disease with serial blood draws over time. Since we have shown in previous studies

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Master, Samip, Shajadi Patan, Shashank Cingam, and Richard Preston Mansour. "Prevalence of Disease Related Complications from in Adult Patients with Sickle Cell Disease." Blood 128, no. 22 (2016): 4862. http://dx.doi.org/10.1182/blood.v128.22.4862.4862.

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Abstract Introduction: Sickle cell disease can lead to variety of complications like strokes, acute chest syndrome (ACS), sickle cell hepatopathy, priapism, renal dysfunction, avascular necrosis (AVN) ofjoints , pulmonary hypertension and leg ulcers. Average life expectancy of patient with SCD has improved with better treatments available. Most of literature on SCD in form pediatric literature and data on adults is considerably lacking. We did a retrospective analysis to investigate the prevalence of complications in adult patients with SCD. Methods: We take care of around 300 adult active pat

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Madany, Emaan, Najwa El Kadi, Sumaarg Pandya, Jeanne E. Hendrickson, and David R. Gibb. "Increased Expression of Type 1 Interferon Stimulated Genes in Sickle Cell Disease and a Potential Association with RBC Alloimmunization." Blood 134, Supplement_1 (2019): 716. http://dx.doi.org/10.1182/blood-2019-124899.

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Background RBC transfusion can lead to the production of alloantibodies against RBC antigens and is a clinically significant issue in transfusion medicine. Patients with sickle cell disease have an increased risk for alloimmunization; 30-50% of SS patients have alloantibodies compared to 3-10% of other hospitalized patients. These alloantibodies can cause dangerous hemolytic transfusion reactions and limit the availability of compatible antigen-negative RBC products. This is of particular importance in SS patients, who commonly make alloantibodies against multiple RBC antigens and need regular

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Presley, Tennille, Lauren Bain, Samir Ballas, et al. "The Mechanism of Hemolysis in Sickle Cell Anemia." Blood 112, no. 11 (2008): 1439. http://dx.doi.org/10.1182/blood.v112.11.1439.1439.

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Abstract Accumulating transgenic animal, large animal and human epidemiological evidence supports a role for hemolysis in the pathobiology of sickle cell disease. However, the mechanism of hemolysis or more specifically the relative contribution of sickling and oxidative damage has yet to be determined. Early studies have shown that repetitive sickling/unsickling via cycles of deoxygenation/reoxygenation lead to a decrease in sickle red cell deformability (even under oxygenated conditions), suggesting an important role for sickle hemoglobin polymerization probably associated with membrane loss

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34

Rosse, Wendell F., Mohandas Narla, Lawrence D. Petz, and Martin H. Steinberg. "New Views of Sickle Cell Disease Pathophysiology and Treatment." Hematology 2000, no. 1 (2000): 2–17. http://dx.doi.org/10.1182/asheducation.v2000.1.2.2.

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Abstract This review addresses several areas of concern in the care of patients with sickle cell disease. In Sections I and II, the fundamental pathogenetic mechanisms of sickle cell disease and their clinical consequences are discussed. Dr. Narla presents the evidence for abnormal cell adhesiveness by SS cells and Dr. Rosse examines the role of the increased whole blood viscosity. In Section III, Dr. Petz reviews common and uncommon alloimmune consequences of transfusion in sickle cell disease and discusses the diagnosis and management of sickle cell patients with hyperhemolysis after transfu

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Rosse, Wendell F., Mohandas Narla, Lawrence D. Petz, and Martin H. Steinberg. "New Views of Sickle Cell Disease Pathophysiology and Treatment." Hematology 2000, no. 1 (2000): 2–17. http://dx.doi.org/10.1182/asheducation.v2000.1.2.20000002.

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This review addresses several areas of concern in the care of patients with sickle cell disease. In Sections I and II, the fundamental pathogenetic mechanisms of sickle cell disease and their clinical consequences are discussed. Dr. Narla presents the evidence for abnormal cell adhesiveness by SS cells and Dr. Rosse examines the role of the increased whole blood viscosity. In Section III, Dr. Petz reviews common and uncommon alloimmune consequences of transfusion in sickle cell disease and discusses the diagnosis and management of sickle cell patients with hyperhemolysis after transfusion. In

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Bakhtar, Vijay, Niyati Bakhtar, Nikhil Bakhtar, and Kirit Pandey. "Study of pulmonary hypertension as a complication of sickle cell disease." International Journal of Advances in Medicine 7, no. 2 (2020): 297. http://dx.doi.org/10.18203/2349-3933.ijam20200084.

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Background: Sickle cell anaemia is the most common inherited hematological abnormality across human race, particularly prevalent in pockets of central India and pulmonary hypertension (PH) supposedly worsens prognosis. Data from central India is lacking though. Present study aims to study incidence of pulmonary hypertension as a complication in patients of sickle cell disease.Methods: Patients aged more than 12 years diagnosed to have sickle cell anaemia on hemoglobin electrophoresis during the study period (total- 94; 54 SS and 40 AS) were enrolled as cases. Sixty four age/sex matched healthy

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Archer, Natasha M., and Manoj Duraisingh. "Fetal Hemoglobin Promotes P. Falciparum Growth in Sickle Cell Disease Erythrocytes." Blood 134, Supplement_1 (2019): 2252. http://dx.doi.org/10.1182/blood-2019-130763.

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Introduction: We have demonstrated that sickle hemoglobin (HbS) polymerization in low oxygen (O2) is the main driver of sickle cell trait (AS) resistance to P.falciparum malaria. This suggests that homozygous sickle cell disease (SS) individuals should have even greater resistance to malaria. Instead SS individuals infected by P. falciparum often have increased malaria morbidity and mortality compared to individuals with normal hemoglobin (AA) or AS. The reasons for this paradox are poorly understood. We propose that fetal hemoglobin (HbF) inhibits polymerization thereby allowing parasite prol

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Cohen, Alice J., and Chaim Tuckman-Vernon. "Association of Pulmonary Hypertension with Sickle Cell Disease Subtypes." Blood 112, no. 11 (2008): 4824. http://dx.doi.org/10.1182/blood.v112.11.4824.4824.

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Abstract Pulmonary hypertension (PH) is a common complication of sickle cell disease (SD) and a significant cause of morbidity and mortality. PH, measured by Doppler echocardiography and defined as a tricuspid regurgitant jet velocity (TRV) &gt; 2.5 m per second (m/s), is hypothesized to be related to the chronic hemolytic anemia of SD, but causality is unproven. If so, the presence of hemoglobin C, which reduces hemolysis, would be expected to have a reduced likelihood of PH. This study reviewed the prevalence of PH in 3 categories of patients with SD: homozygous S (SS), sickle-beta thala

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Hsu, Lewi s. L., Hazim El-Haddad, Marcelo Amar, Gregory J. Kato, Alan T. Remaley, and Hunter C. Champion. "Sickle Cell Pulmonary Hypertension and Dysregulated NO Axis in a Mouse Model Are Modulated by Apolipoprotein a-1 Availability." Blood 112, no. 11 (2008): 2499. http://dx.doi.org/10.1182/blood.v112.11.2499.2499.

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Abstract We have recently shown that the pulmonary hypertension and pulmonary vascular response impairment in models of sickle cell disease are associated with a dysregulation of nitric oxide synthase (NOS) coupling and cGMP signaling. However, the pathophysiologic mechanisms and exacerbating factors leading to pulmonary vasculopathy remain unclear. In the present study, we investigated the effect of sickle cell disease in the context of ApoE deficient mice (ApoEKO) that express advanced atherosclerosis and mice that express the protective ApoA-1 protein (TgApoA1) that received bone marrow tra

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Elenga, Narcisse, Aurélie Adeline, John Balcaen, et al. "Pregnancy in Sickle Cell Disease Is a Very High-Risk Situation: An Observational Study." Obstetrics and Gynecology International 2016 (2016): 1–5. http://dx.doi.org/10.1155/2016/9069054.

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Sickle cell disease is a serious genetic disorder affecting 1/235 births in French Guiana. This study aimed to describe the follow-up of pregnancies among sickle cell disease patients in Cayenne Hospital, in order to highlight the most reported complications. 62 records of pregnancies were analyzed among 44 females with sickle cell disease, between 2007 and 2013. Our results were compared to those of studies conducted in Brazil and Guadeloupe. There were 61 monofetal pregnancies and 2 twin pregnancies, 27 pregnancies among women with SS phenotype, 30 SC pregnancies, and five S-beta pregnancies

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Campbell, Andrew, Osamu Tanabe, Rebekah Urbonya, et al. "Analysis of Fetal Hemoglobin Expression within Humanized Sickle Cell Disease Mice Overexpressing the TR2/4 Transgene." Blood 116, no. 21 (2010): 1619. http://dx.doi.org/10.1182/blood.v116.21.1619.1619.

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Abstract Abstract 1619 Background: Sickle Cell Disease (SCD) is a chronic debilitating hematologic condition caused by a missense mutation within the adult beta globin gene leading to significant morbidity and mortality. Increased Fetal Hemoglobin production has been shown to significantly ameliorate SCD symptoms and improve survival. A novel specific DNA-binding factor DRED (direct repeat erythroid definitive) was recently identified that regulated epsilon and gamma globin gene expression (Tanimoto et al Genes Dev 2000). Purification of DRED revealed that it harbored the nuclear orphan hormon

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MIKOBI, Tite Minga, and Prosper Tshilobo LUKUSA. "HOMOZYGOUS DELETION ALFA-THALASSEMIA AND HEREDITARY PERSISTENCE OF FETAL HEMOGLOBIN, TWO GENETIC FACTORS PREDICTIVE THE REDUCTION OF MORBIDITY AND MORTALITY DURING PREGNANCY IN SICKLE CELL PATIENTS . A REPORT FROM DEMOCRATIC REPUBLIC OF CONGO." Mediterranean Journal of Hematology and Infectious Diseases 11, no. 1 (2019): e2019039. http://dx.doi.org/10.4084/mjhid.2019.039.

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FHb and alpha-thal are two genetic factors that modulate the clinical expression of sickle cell disease. Objective: to determine the beneficial role of FHb and alpha-thal on fetal and maternal morbidity during pregnancy in sickle cell patients. This is a documentary and analytical study that included 960 deliveries of homozygous sickle cell patients. The deliveries were divided into three genotype subgroups: Hb-SS / alpha-thal, HbSS / HPFH and HbSS. The diagnosis of SCD and the quantification of FHb were performed by the capillary electrophoresis technique. The diagnosis of SCD has

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Mukarram, Osama, Gian Lima, Samuel Crooks, Min Jung Kim, and Agnes S. Kim. "Assessment of Cardiac Abnormalities in Sickle Cell Disease Patients Using Echocardiography." Blood 134, Supplement_1 (2019): 4837. http://dx.doi.org/10.1182/blood-2019-128390.

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Background: Sickle Cell Disease (SCD) is characterized by chronic anemia and recurrent ischemia-reperfusion episodes that contribute to high output heart failure. The effects of SCD on the heart are significantly underrecognized. Methods: SCD patients who underwent echocardiography between March 2016 and March 2018 were retrospectively analyzed. Patients with reduced Left Ventricular Ejection Fraction (LVEF) and valvular heart disease were excluded. Cardiac chamber size, systolic and diastolic function parameters, and LV and RV strain were compared between hemoglobin SS (most severe form of SC

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Claudino, Mário Angelo, Luiz Osório Leiria, Carla Fernanda Franco-Penteado, et al. "Young and Old Sickle Cell Disease Transgenic Mice Present Underactive Bladder." Blood 122, no. 21 (2013): 2248. http://dx.doi.org/10.1182/blood.v122.21.2248.2248.

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Abstract Urinary bladder dysfunction is rarely spontaneously reported by sickle cell disease (SCD) patients to their caregivers. With increasing survival of these patients, physicians may expect that urinary complaints increase in association with classical urological disorders associated with advanced age. Nocturia has long been attributed to constant increased urinary volumes in SCD. As part of the renal complications of sickling, renal medullary infarcts lead to decreased ability to concentrate urine, yielding higher daily urinary volumes, compensatory polydipsia and, eventually, the need f

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Quinn, Charles T., Kimberly Thomas, Zora R. Rogers, and George R. Buchanan. "Improved Survival of Children and Adolescents with Sickle Cell Disease." Blood 112, no. 11 (2008): 1425. http://dx.doi.org/10.1182/blood.v112.11.1425.1425.

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Abstract The survival of children with sickle cell disease (SCD) has improved over the past several decades, especially for very young children. However, we know less about mortality during adolescence, and we do not accurately know the current proportion of children born with SCD who survive to adulthood. The first report from the Dallas Newborn Cohort (DNC), which included follow-up through June 2002, estimated overall survival at 18 years of age to be 85.6% (95% C.I.: 73.4 – 97.8) for individuals with sickle cell anemia (SS) or sickle-β0-thalassemia (Sβ0) (Blood2004;103:4023–7). The confide

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Sparkenbaugh, Erica, Kathryn Wilson, Malgorzata Kasztan, David M. Pollock, Keith R. McCrae, and Rafal Pawlinski. "High Molecular Weight Kininogen Contributes to End-Organ Damage and Mortality in a Mouse Model of Sickle Cell Disease." Blood 132, Supplement 1 (2018): 268. http://dx.doi.org/10.1182/blood-2018-99-116526.

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Abstract Introduction: Recent advances in preventive care, such as hydoxyurea and prophylactic antibiotics, have reduced the mortality of children with sickle cell disease (SCD) s in developed countries. Yet, the chronic hemolytic anemia and recurrent vaso-occlusive crises result in systemic inflammation and coagulopathy. Markers of coagulation activation correlate with painful crises, acute chest syndrome, stroke, venous thromboembolism, pulmonary hypertension, left ventricular diastolic heart disease, and sickle nephropathy. These complications result in end-organ failure that causes increas

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King, L., R. Fraser, M. Forbes, M. Grindley, S. Ali, and M. Reid. "Newborn sickle cell disease screening: the Jamaican experience (1995–2006)." Journal of Medical Screening 14, no. 3 (2007): 117–22. http://dx.doi.org/10.1258/096914107782066185.

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Objectives: The aim of this study was to evaluate the existing newborn sickle haemoglobinopathy screening programme in Jamaica. Methods: A retrospective analysis of infants screened during the period 8 November 1995 to 22 July 2006 was performed. Patient data for analyses was restricted to patients with homozygous (Hb SS) sickle cell disease. Published data from the Jamaican Sickle Cell Cohort Study was used to make comparisons with the study sample. Results: The study sample consisted of 435 patients with Hb SS disease. Acute chest syndrome was the most common clinical (non-death) event accou

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Wu, Catherine J., Lakshamanan Krishnamurti, Jeffery L. Kutok, et al. "Evidence for ineffective erythropoiesis in severe sickle cell disease." Blood 106, no. 10 (2005): 3639–45. http://dx.doi.org/10.1182/blood-2005-04-1376.

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AbstractPeripheral destruction of sickled erythrocytes is a cardinal feature of sickle cell disease (SCD). Less well established is the potential contribution of ineffective erythropoiesis to the pathophysiology of this hemoglobinopathy. Since patients with SCD frequently develop mixed hematopoietic chimerism after allogeneic nonmyeloablative stem cell transplantation, we used this opportunity to directly compare the differentiation and survival of SCD and donor-derived erythropoiesis in vivo. Donor and recipient erythropoiesis was compared in 4 patients with SCD and 4 without SCD who develope

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Inoue, Susumu, and Christina Anagonye. "Frequency of Thrombotic Events in Hospitalized Patients with Sickle Cell Disease." Blood 136, Supplement 1 (2020): 13. http://dx.doi.org/10.1182/blood-2020-143444.

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Background Thrombosis is one of the most common complications described in patients with sickle cell disease. However, little is known about the prevalence, variety and severity of thrombus particularly in sickle cell patients hospitalized with vaso-occlusive crisis. Methods We made a retrospective chart review of patients (age 21 years or older) admitted with sickle cell disease at Hurley Medical Center (Flint, Michigan) and was diagnosed with a thrombotic event between April 2012 and April 2020. To obtain a logistic model, we identified all patients with the the final discharge diagnosis of

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Stevens, MC, GH Maude, M. Beckford, et al. "Alpha thalassemia and the hematology of homozygous sickle cell disease in childhood." Blood 67, no. 2 (1986): 411–14. http://dx.doi.org/10.1182/blood.v67.2.411.411.

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Abstract alpha Thalassemia modifies the hematologic expression of homozygous sickle cell (SS) disease, resulting in increased total hemoglobin and HbA2 and decreased HbF, mean cell volume, reticulocytes, irreversibly sickled cells, and bilirubin levels. The age at which these changes develop in children with SS disease is unknown. Ascertainment of globin gene status in a large representative sample of children with SS disease has afforded an opportunity to study the hematologic indices in nine children homozygous for alpha thalassemia 2 (two-gene group), 90 children heterozygous for alpha thal

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