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Sickle Cell Crisis

Anirudh Karanam, S Gurushanthappa
No abstract text is available yet for this article.
January 2016: Journal of the Association of Physicians of India
Olufunmilayo E Adejumo, Taiwo R Kotila, Adeyinka G Falusi, Boladale O Silva, Jacinta N Nwogu, Pius S Fasinu, Chinedum P Babalola
Polymorphic expression of metabolic enzymes have been identified as one of the key factors responsible for the interindividual/ethnic/racial variability in drug metabolism and effect. In Nigeria, there is a disproportionately high incidence of sickle-cell disease (SCD), a condition characterized by painful crisis frequently triggered by malaria. Proguanil, a substrate of the polymorphic CYP2C19, is a chemoprophylactic antimalarial drug widely used among SCD patients in Nigeria. This study aimed to conduct a comparative CYP2C19 phenotyping among SCD patients and healthy controls and to compare the results with those previously reported...
October 2016: Pharmacology Research & Perspectives
Udo Ego Anyaehie, Cajetan Uwatoronye Nwadinigwe, Arinze Duke Nwosu, Valentine Ogochukwu Ogbui
INTRODUCTION: A Jehovah's Witness belongs to the religious group that does not accept blood transfusion in any form, while a sickle cell disease patient has abnormal haemoglobins that do not last in circulation predisposing one to anaemia and other systemic complications. Performing a major surgery in a Jehovah's Witness who has sickle cell disease is tasking for a surgeon. CASE PRESENTATION: This case reports a 28-year-old African female with sickle cell disease who outrightly refused any form of blood transfusion as being a Jehovah's Witness and having a complex primary hip that required total hip replacement...
April 2016: Journal of Orthopaedic Case Reports
Chengzhong Cai, Dmitry A Nedosekin, Yulian A Menyaev, Mustafa Sarimollaoglu, Mikhail A Proskurnin, Vladimir P Zharov
Control of sickle cell disease (SCD) stage and treatment efficiency are still time-consuming which makes well-timed prevention of SCD crisis difficult. We show here that in vivo photoacoustic (PA) flow cytometry (PAFC) has a potential for real-time monitoring of circulating sickled cells in mouse model. In vivo data were verified by in vitro PAFC and photothermal (PT) and PA spectral imaging of sickle red blood cells (sRBCs) expressing SCD-associated hemoglobin (HbS) compared to normal red blood cells (nRBCs)...
2016: Analytical Cellular Pathology (Amsterdam)
S Deneuve, L Maire, V Bachelot, M-A Dammacco, P Zrounba, E Delay
Sickle cell anaemia is rare in France but frequent in Africa, leading to rigid, sickle-like shape red blood cells which bind together blocking microcirculation under certain circumstances. The vaso-occlusive crisis is the most frequent clinical manifestation especially in case of homozygous disease. Sickle cells disease is therefore usually considerated as a contraindication to microsurgery, however sometimes, a free flap procedure is mandatory. We here report the case of a 47-year-old man suffering with homozygous sickle cell anaemia and needing an antebrachial free flap procedure for a tongue reconstruction...
September 28, 2016: Annales de Chirurgie Plastique et Esthétique
Salwa Al Najjar, Soheir Adam, Nessar Ahmed, Mohamed Qari
Sickle cell disease (SCD) is an autosomal recessive inherited hemoglobinopathy, characterized by chronic hemolysis and recurrent vaso-occlusive crisis (VOC). This study investigates changes in leucocyte subsets and the relationship between cell adhesion molecule expression and disease manifestations in patients during steady state and acute VOC. We compared soluble E-selectin and P-selectin levels in 84 SCD patients, in steady state and during VOC to 84 healthy controls. Using immunophenotyping, we also compared lymphocyte subsets in these three groups...
September 30, 2016: Annals of Hematology
Jean-François Deux, Vincent Audard, Pierre Brugières, Anoosha Habibi, Elena-Maria Manea, Constance Guillaud-Danis, Bertrand Godeau, Frédéric Galactéros, Thomas Stehlé, Philippe Lang, Philippe Grimbert, Etienne Audureau, Alain Rahmouni, Pablo Bartolucci
BACKGROUND: Our understanding of the pathophysiologic processes underlying sickle cell nephropathy remains incomplete. We performed a pilot study to investigate the potential value of magnetic resonance imaging (MRI) for the assessment of kidney oxygenation and detection of potential changes to tissue perfusion and cellular integrity during a vaso-occlusive crisis. STUDY DESIGN: A case-control study. SETTING & PARTICIPANTS: 10 homozygous patients with sickle cell disease (SCD), without kidney disease (based on estimated glomerular filtration rate and albuminuria), underwent renal MRI during a vaso-occlusive crisis episode...
September 20, 2016: American Journal of Kidney Diseases: the Official Journal of the National Kidney Foundation
Sri L H Yeruva, Yonette Paul, Patricia Oneal, Mehdi Nouraie
Renal dysfunction in sickle cell disease is not only a chronic comorbidity but also a mortality risk factor. Though renal dysfunction starts early in life in sickle cell patients, the predictors that can identify sickle cell disease patients at risk of developing renal dysfunction is not known. We used the Truven Health MarketScan(®) Medicaid Databases from 2007 to 2012. Incidence of new acute renal failure (ARF) and chronic kidney disease (CKD) was calculated in this cohort. There were 9481 patients with a diagnosis of sickle cell disease accounting for 64,201 hospital admissions, during the study period...
September 18, 2016: Hemoglobin
T Pincez, L Calamy, Z Germont, A Lemoine, A-A Lopes, A Massiot, J Tencer, C Thivent, A Hadchouel
Acute and chronic pulmonary complications are frequent in sickle cell disease (SCD), with different spectrum and characteristics in children and adults. Chronic hypoxia is frequent and plays a role in several respiratory complications in SCD. Furthermore, hypoxia has been associated with a higher risk of cerebral ischemia. Despite differing oxygen affinity between hemoglobin A and S, standard pulse oximetry was shown to be accurate in diagnosing hypoxia in SCD patients. Whereas acute hypoxia management is similar to non-SCD patients, chronic hypoxia treatment is mainly based on a transfusion program rather than long-term oxygen therapy...
October 2016: Archives de Pédiatrie: Organe Officiel de la Sociéte Française de Pédiatrie
Deogratias Munube, Elly Katabira, Grace Ndeezi, Moses Joloba, Samden Lhatoo, Martha Sajatovic, James K Tumwine
BACKGROUND: Stroke is a major complication of sickle cell anaemia (SCA). It occurs commonly in childhood with about 10 % of children with sickle cell anaemia getting affected by this complication. In Uganda, there is paucity of data on the prevalence of stroke in children admitted in a tertiary institution. We determined the prevalence of stroke amongst children with SCA admitted to Mulago National Referral Hospital in Uganda and described the ir co-morbidities. METHODS: We conducted a retrospective record review of children with SCA admitted from August 2012 to August 2014 to the Paediatric Haematology Ward of Mulago Hospital in Kampala, Uganda...
2016: BMC Neurology
Taciana Furtado de Mendonça Belmont, Kleyton Palmeira do Ó, Andreia Soares da Silva, Kamila de Melo Vilar, Fernanda Silva Medeiros, Luydson Richardson Silva Vasconcelos, Ana Claudia Mendonça Dos Anjos, Betânia Lucena Domingues Hatzlhofer, Maíra Galdino da Rocha Pitta, Marcos André Cavalcanti Bezerra, Aderson da Silva Araújo, Moacyr Jesus Barreto de Melo Rego, Patrícia Moura, Maria do Socorro Mendonça Cavalcanti
INTRODUCTION: Patients with sickle cell anemia (SCA) may present chronic hemolytic anemia, vaso-occlusion and respiratory tract infection (RTI) episodes. Galectin-3 (GAL-3) is a multifunctional protein involved in inflammation, apoptosis, adhesion and resistance to reactive oxygen species. Studies point to a dual role for GAL-3 as both a circulation damage-associated molecular pattern and a cell membrane associated pattern recognition receptor. OBJECTIVE: To investigate associations between the SNPs of GAL-3 gene (LGALS3) and serum levels with RTI and vaso-occlusive crisis (VOC) in children with SCA...
2016: PloS One
Carly S Gardner, Daniel T Boll, Priya Bhosale, Tracy A Jaffe
Sickle cell disease (SCD) is the most prevalent hemoglobinopathy. Survival in patients with SCD has improved over the past few decades. These patients experience a lifetime of repeated acute pain crises, which are thought to result from sickling and microvascular occlusions; acute abdominal pain is common. Moreover, repeated crises often lead to organ dysfunction, such as asplenia, hepatic failure, and renal failure. The spleen, liver, biliary system, kidneys, and gastrointestinal tract can all be affected...
September 7, 2016: Abdominal Radiology
Azza Abdel Gawad Tantawy, Amira Abdel Moneam Adly, Eman Abdel Rahman Ismail, Mai Abdelazeem
BACKGROUND: Patients with sickle cell disease (SCD) are at high risk of renal dysfunction and cardiovascular morbidity. The association between cystatin C and renal function is well known, however, cystatin C has recently emerged as a strong predictor of cardiovascular events and adverse outcomes in patients with and without kidney disease, mostly related to both inflammation and atherosclerosis. AIM: To determine cystatin C levels in 53 children and adolescents with SCD compared to 40 age- and sex-matched healthy controls and assess its relation to markers of hemolysis, iron overload, sickle vasculopathy, and carotid intima-media thickness (CIMT)...
August 31, 2016: Clinical and Applied Thrombosis/hemostasis
Erica Simon, Brit Long, Alex Koyfman
BACKGROUND: Sickle cell disease (SCD) affects approximately 100,000 individuals in the United States. Due to alterations in the structural conformation of hemoglobin molecules under deoxygenated conditions, patients with SCD are predisposed to numerous sequelae, many of which require acute intervention. OBJECTIVE: Our aim was to provide emergency physicians with an evidence-based update regarding the diagnosis and management of SCD complications. DISCUSSION: SCD patients experience significant morbidity and mortality secondary to cerebrovascular accident, acute chest syndrome, acute vaso-occlusive pain crises, SCD-related multi-organ failure, cholecystitis, acute intrahepatic cholestasis, acute sickle hepatic crisis, acute hepatic sequestration, priapism, and renal disease...
October 2016: Journal of Emergency Medicine
Peter E Keipert
MP4CO, developed by Sangart Inc. (San Diego, CA), is a pegylated human hemoglobin-based carbon monoxide (CO) delivery agent and oxygen therapeutic that has shown potential to prevent and reverse red cell sickling. A double blind, comparator controlled, dose-escalation, Phase 1b study was conducted to assess the safety of MP4CO. Adult sickle cell patients with HbSS or S/β(0) Thal genotype who were not experiencing a painful crisis were randomized to receive either MP4CO or normal saline (NS) in a sequential series of six escalating dose cohorts (A-F)...
2016: Advances in Experimental Medicine and Biology
Amanda M Brandow, Mark Nimmer, Timothy Simmons, T Charles Casper, Lawrence J Cook, Corrie E Chumpitazi, J Paul Scott, Julie A Panepinto, David C Brousseau
The impact of emergency department (ED) treatment on outcomes of sickle cell disease (SCD) acute pain hospitalizations is not well described. We investigated whether length of stay (LOS) and change in health-related quality of life (HRQL) are affected by initial opioid dose and time to administration. We conducted secondary analyses of data from the randomized-controlled Magnesium for children in Crisis (MAGiC) trial. The primary outcome was LOS. Secondary outcome was change in HRQL, assessed using PedsQL SCD Pain and Hurt and Pain Impact Domains measured in ED and at discharge...
August 12, 2016: American Journal of Hematology
Poorya Hosseini, Sabia Z Abidi, E Du, Dimitrios P Papageorgiou, Youngwoon Choi, YongKeun Park, John M Higgins, Gregory J Kato, Subra Suresh, Ming Dao, Zahid Yaqoob, Peter T C So
Hydroxyurea (HU) has been used clinically to reduce the frequency of painful crisis and the need for blood transfusion in sickle cell disease (SCD) patients. However, the mechanisms underlying such beneficial effects of HU treatment are still not fully understood. Studies have indicated a weak correlation between clinical outcome and molecular markers, and the scientific quest to develop companion biophysical markers have mostly targeted studies of blood properties under hypoxia. Using a common-path interferometric technique, we measure biomechanical and morphological properties of individual red blood cells in SCD patients as a function of cell density, and investigate the correlation of these biophysical properties with drug intake as well as other clinically measured parameters...
August 23, 2016: Proceedings of the National Academy of Sciences of the United States of America
Abdulrahman Alsultan, Wasil Jastaniah, Sameera Al Afghani, Muneer H Al Bagshi, Zaki Nasserullah, Ahmed M Al-Suliman, Mohammed K Alabdulaali
Allogeneic HSCT is the only curative treatment for SCD. In this study, we estimated the number of Saudi patients with SCD who are candidates for HSCT. We used the presence of overt stroke, recurrent ACS, and frequent severe pain crisis as indications for HSCT. We calculated the frequencies of these complications among a Saudi SCD cohort of 376 patients with SCD, 250 from SW and 126 from Eastern (E) provinces. We found that 59 (23.6%) of SW patients were transplant candidates compared to 22 (17.4%) from E province...
September 2016: Pediatric Transplantation
Jin Han, Santosh L Saraf, Xu Zhang, Michel Gowhari, Robert E Molokie, Johara Hassan, Chaher Alhandalous, Shivi Jain, Jewel Younge, Taimur Abbasi, Roberto F Machado, Victor R Gordeuk
Pain, the hallmark complication of sickle cell disease (SCD), is largely managed with opioid analgesics in the United States, but comprehensive data regarding the long-term use of opioids in this patient population is lacking. The pain medication prescription records from a cohort of 203 SCD patients were analyzed. Twenty-five percent were not prescribed opioid medications while 47% took only short-acting opioids, 1% took only long-acting opioids, and 27% took a combination of short-acting and long-acting opioids...
July 28, 2016: American Journal of Hematology
M Joshua Daniel, Prakash H Muddegowda, Chezhiansubash, Jyothi B Lingegowda, Niranjan Gopal, Krishna Prasad
INTRODUCTION: Red Cell Exchange (RCE) is removal of a patient's red blood cells while replacing with donor red blood cells either manually or using automated systems. RCE is beneficial in patients with Sickle Cell Disease (SCD) either during sickling crisis or prior to major surgical procedures to bring down the sickling percentage as high sickling percentage during prolonged anaesthesia may lead to vaso-occlusive crisis. It is also employed in patients infested with malaria and babesiosis where parasitic index remain high despite medical management...
May 2016: Journal of Clinical and Diagnostic Research: JCDR
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