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SCD Patients (scd + patient)
Selected AbstractsAdult Emergency Department Patients with Sickle Cell Pain Crisis: A Learning Collaborative Model to Improve Analgesic ManagementACADEMIC EMERGENCY MEDICINE, Issue 4 2010Paula Tanabe PhD Abstract Objectives:, The objectives were to report the baseline (prior to quality improvement interventions) patient and visit characteristics and analgesic management practices for each site participating in an emergency department (ED) sickle cell learning collaborative. Methods:, A prospective, multisite longitudinal cohort study in the context of a learning-collaborative model was performed in three midwestern EDs. Each site formed a multidisciplinary team charged with improving analgesic management for patients with sickle cell disease (SCD). Each team developed a nurse-initiated analgesic protocol for SCD patients (implemented after a baseline data collection period of 3.5 months at one site and 10 months at the other two sites). All sites prospectively enrolled adults with an acute pain crisis and SCD. All medical records for patients meeting study criteria were reviewed. Demographic, health services, and analgesic management data were abstracted, including ED visit frequency data, ED disposition, arrival and discharge pain score, and name and route of initial analgesic administered. Ten interviews per quarter per site were conducted with patients within 14 days of their ED discharge, and subjects were queried about the highest level of pain acceptable at discharge. The primary outcome variable was the time to initial analgesic administration. Variable data were described as means and standard deviations (SDs) or medians and interquartile ranges (IQR) for nonnormal data. Results:, A total of 155 patients met study criteria (median age = 32 years, IQR = 24,40 years) with a total of 701 ED visits. Eighty-six interviews were conducted. Most patients (71.6%) had between one and three visits to the ED during the study period. However, after removing Site 3 from the analysis because of the short data enrollment period (3.5 months), which influenced the mean number of visits for the entire cohort, 52% of patients had between one and three ED visits over 10 months, 21% had four to nine visits, and 27% had between 10 and 67 visits. Fifty-nine percent of patients were discharged home. The median time to initial analgesic for the cohort was 74 minutes (IQR = 48,135 minutes). Differences between choice of analgesic agent and route selected were evident between sites. For the cohort, 680 initial analgesic doses were given (morphine sulfate, 42%; hydromorphone, 46%; meperidine, 4%; morphine sulfate and ibuprofen or ketorolac, 7%) using the following routes: oral (2%), intravenous (67%), subcutaneous (3%), and intramuscular (28%). Patients reported a significantly lower targeted discharge pain score (mean ± SD = 4.19 ± 1.18) compared to the actual documented discharge pain score within 45 minutes of discharge (mean ± SD = 5.77 ± 2.45; mean difference = 1.58, 95% confidence interval = .723 to 2.44, n = 43). Conclusions:, While half of the patients had one to three ED visits during the study period, many patients had more frequent visits. Delays to receiving an initial analgesic were common, and post-ED interviews reveal that sickle cell pain patients are discharged from the ED with higher pain scores than what they perceive as desirable. ACADEMIC EMERGENCY MEDICINE 2010; 17:399,407 © 2010 by the Society for Academic Emergency Medicine [source] Management of acute painful crises in sickle cell diseaseINTERNATIONAL JOURNAL OF LABORATORY HEMATOLOGY, Issue 4 2005T. R. KOTILA Summary Pain is a common mode of manifestation of sickle cell disease (SCD) but there is limited information on pain management in this disorder. This study examines the use of opioids and non-opioid analgesia in the management of painful crisis in adult SCD patients; the routine use of antimalarials and antibiotics as adjunct therapy was also examined. A total of 87% of the patients had had a form of analgesics before presentation, 20% of which had parenteral analgesia. Ten per cent had not used any form of medication while another 10% used non-steroidal anti-inflammatory drugs. When asked, 59% of the patients desired oral non-opioid analgesics while 31% were not concerned about the type of analgesic given. Only 8% requested opioids. Hospital admission was not necessary in 65% of the patients; they were observed in the day-care unit and allowed home within 24 h. Sixty per cent did not have a test for malaria; 66% of those who had the test performed were negative, 35% of those whose thick film for malaria was negative had antimalarials prescribed. Only five patients (7%) were febrile at presentation. Thirty-four per cent had antibiotics prescribed, a third of these parenterally. Thirty-nine per cent had no fever but received antibiotics. [source] Activated monocytes and platelet-monocyte aggregates in patients with sickle cell disease*INTERNATIONAL JOURNAL OF LABORATORY HEMATOLOGY, Issue 2 2002TED WUN Tumour necrosis factor-, (TNF-,) and interleukin-1, (IL-1,) increase endothelial surface receptors that mediate the adherence of sickle erythrocytes to the endothelium. Increased circulating levels of these cytokines have been found in patients with sickle cell disease (SCD). Monocytes are a source of both of these inflammatory mediators; we therefore determined whether circulating monocytes were activated in SCD, as defined by intracellular expression of these cytokines. Blood was also assayed for the presence of platelet,monocyte aggregates (PMAs), as platelet adherence is one possible mechanism for monocyte activation. The median percentages of monocytes expressing intracellular TNF-, and IL-1, in SCD patients were 6.8 (2.8,17.3) [median (range)] and 14.1 (1.3,44.8), respectively. In African-American controls the corresponding percentages were 0.3 (0.1,0.5) and 0.4 (0.1,3.0), and in Caucasians 0.2 (0.1,0.5) and 0.8 (0.8,1.9) (P < 0.001, Kruskal,Wallis). The mean percentage (± SD) of PMA was 14.0 ± 8.3 for Caucasian controls, 25.7 ± 7.3 for African-American controls, and 45.7 ± 21.6 for patients with SCD (P < 0.001, RM ANOVA; P < 0.05, Newman,Keuls posthoc test). We conclude that there are increased circulating PMAs and monocyte activation in patients with SCD. [source] Cytochrome P450 CYP2C19 genotypes in Nigerian sickle-cell disease patients and normal controlsJOURNAL OF CLINICAL PHARMACY & THERAPEUTICS, Issue 4 2010C. P. Babalola PhD Summary Background and objective:, Subjects with different CYP2C19 genotypes may metabolize proguanil, a pro-drug used for malaria prophylaxis differently and the frequency of the different alleles may be different in patients with sickle-cell disease (SCD) and normal controls. The objective of this study was to evaluate CYP2C19 *1, *2 and *3 allele and genotype frequencies in Nigerian normal controls and SCD patients, and to further compare variant CYP2C19 frequencies in Nigerians with other African populations. Methods:, Genotyping was carried out with PCR and restriction fragment length polymorphism analysis. Results and discussion:, CYP2C19 *1 (84·3 vs. 84·9%) or *2 allele frequency (15·7 vs. 15·1%) was not significantly different between patients with SCD and normal subjects. No *3 allele was detected in the cohort. The SCD group exhibited a statistically significantly lower frequency of *1/*1 genotype (69·6%) compared with normal controls (74·4%). Frequency of *2/*2 was significantly lower in SCD (0·9%) compared with normal controls (4·7%). Frequencies of *1/*2 (29·6 vs. 20·9%) were no different in SCD and normal controls. Conclusion:, Prevalence of CYP2C19 polymorphisms was defined for the first time in Nigerian normal and SCD populations. Nigerian SCD patients exhibited significantly lower CYP2C19 *1/*1 and *2/*2 frequencies than normal controls. No differences were detected in CYP2C19 allele or genotype frequencies in normal subjects between this study and previous reports in other African populations. [source] Life threatening parvovirus B19 and herpes simplex virus associated acute myocardial dysfunction in a child with homozygous sickle cell diseasePEDIATRIC BLOOD & CANCER, Issue 7 2007Lakshmanan Krishnamurti MD Abstract Human parvovirus (HPV) B19, a common infection, frequently causes transient red cell aplasia in children with hemolytic anemia, such as sickle cell disease (SCD). It was considered to be a self-limited condition, easily treated with blood transfusion. However, acute splenic sequestration, acute chest syndrome, nephrotic syndrome, and stroke have been reported in SCD patients following HPV B19 infection. We report a 3-year-old child with SCD who developed fulminant myocarditis following HPV B19-related aplastic crisis. The diagnosis of myocarditis should be considered in a patient with hemolytic anemia with an infection with HPV B19 who develops signs of cardiopulmonary failure despite correction of anemia. Pediatr Blood Cancer 2007;49:1019,1021. © 2006 Wiley-Liss, Inc. [source] Serum ferritin underestimates liver iron concentration in transfusion independent thalassemia patients as compared to regularly transfused thalassemia and sickle cell patientsPEDIATRIC BLOOD & CANCER, Issue 3 2007Zahra Pakbaz MD Abstract Serum ferritin (SF) and liver iron concentration (LIC), as measured by SQUID biosusceptometry, were assessed in a convenience sample of transfusion independent thalassemia patients (nTx-Thal, n,=,26), regularly transfused thalassemia (Tx-Thal, n,=,89), or sickle cell patients (SCD, n,=,45) to investigate the severity of iron overload and the relationship between SF and LIC in nTx-Thal compared to SCD and Tx-Thal. SF correlated with LIC (RS,=,0.53, P,<,0.001), but was found to be a poor predictor for LIC. SF was significantly lower (P,<,0.001) in nTx-Thal patients than in other groups, despite similar LIC values. The SF-to-LIC ratio was significantly lower in nTx-Thal compared to Tx-Thal and SCD patients (median of 0.32, 0.87, and 1.2, respectively: P,<,0.001). Due to underestimation of LIC by ferritin levels, chelation treatment may be delayed or misdirected in patients with thalassemia intermedia. Pediatr Blood Cancer 2007;49:329,332. © 2007 Wiley-Liss, Inc. [source] Nutritional deficiencies in iron overloaded patients with hemoglobinopathies,AMERICAN JOURNAL OF HEMATOLOGY, Issue 6 2009Susan Claster One of the hallmarks of both sickle cell disease (SCD) and thalassemia major (TM) is accelerated oxidative damage. Decreased antioxidant levels and increased oxidant stress biomarkers are found in both diseases. Although isolated vitamin deficiencies have been reported in TM and nontransfused SCD patients, a comprehensive evaluation of vitamin and trace mineral levels has never been performed in chronically transfused SCD or TM patients. As vitamins and trace minerals may be consumed as a result of chronic oxidative stress; we hypothesized that levels of these compounds would correlate with surrogates of iron overload, hemolysis, and inflammation in chronically transfused patients. Using a convenience sample of our group of chronically transfused patients we studied 43 patients with SCD (17 male, 26 female) and 24 patients with TM (13 male and 11 female). The age range for our patients varied from 1.5 to 31.4 years. Levels of vitamins A, thiamin, B6, B12, C, D, E as well as selenium, zinc, copper, and ceruloplasmin were measured. We found that 40,75% of the patients were deficient in A, C, D and selenium and 28,38% of the patients had low levels of B vitamins and folate. There was little association with iron overload, hemolysis, or inflammation. Although the precise mechanism of these deficiencies is unclear, they may contribute to the morbidity of chronically transfused hemoglobinopathy patients. Am. J. Hematol., 2009. © 2009 Wiley-Liss, Inc. [source] Cerebral oximetry improves detection of sickle cell patients at risk for nocturnal cerebral hypoxia,PEDIATRIC PULMONOLOGY, Issue 11 2006Ashok B. Raj MD Abstract We previously used cerebral oximetry to identify low cerebral venous oxygen saturation in waking children with sickle cell disease (SCD). Because arterial oxyhemoglobin desaturation is common during sleep in SCD patients, this study compared both waking and sleeping systemic arterial and cerebral venous oxygenation dynamics in children with and without SCD. Seventeen African-American (AA) children with homozygous SCD [8 (4,15) years; 29% male; normal transcranial Doppler velocities] were compared with a control cohort (CON) comprised of six healthy AA children [9 (4,16) years, 33% male]. Standard all-night polysomnographic recordings were performed, including measurement of arterial oxygen saturation by pulse oximetry (SpO2). Regional cerebral oxygen saturation (rSO2) was measured non-invasively with cerebral oximetry. Intra-cohort comparisons examined the influence of sleep on SpO2 and rSO2 in the subjects. Inter-cohort comparisons of SpO2, rSO2, and the rSO2/SpO2 ratio assessed the impact of SCD on systemic and cerebral oxygenation during wakefulness and sleep. Cohort differences in SpO2 were not statistically significant in either wakefulness or sleep. However, only in the SCD cohort was the magnitude of SpO2 change statistically significant (P,=,0.002). In contrast, both waking and sleep rSO2 cohort median values did differ significantly [awake: CON 76 (67,86) vs. SCD 62 (58,71), P,=,0.01; sleep: CON 65 (60,77) vs. SCD 55 (48,61), P,=,0.01)]. The waking rSO2/SpO2 ratio was also significantly lower in the SCD group [CON 0.78 (0.68,0.88) vs. SCD of 0.66 (0.61,0.72); P,=,0.015]. During sleep, the ratio was also significantly lower in the SCD group [CON 0.71 (0.66,0.81) vs. SCD 0.59 (0.52,0.65); P,=,0.011]. Our findings suggest that SCD patients may be at increased risk of cerebral hypoxia during both wakefulness and sleep. Pediatr Pulmonol. 2006, 41:1088,1094. © 2006 Wiley-Liss, Inc. [source] Cytokine profile of sickle cell disease in OmanAMERICAN JOURNAL OF HEMATOLOGY, Issue 4 2004Anil Pathare Abstract The aim of our study was to assess the cytokine profile of sickle cell disease (SCD) patients in steady state and in vaso-occlusive crisis (VOC). VOC has a complex nature, involving interactions between sickle red blood cells (RBC), the endothelium, and leucocytes. Endothelial damage due to recurrent adhesion of sickle RBCs may disrupt endothelial function, leading to altered cytokine release. It is therefore pertinent to study the cytokine profile of SCD patients in steady state and in crisis prior to exploring its contribution to vaso-occlusive manifestations, since it is believed that an altered balance of proinflammatory and anti-inflammatory cytokines plays an important role in painful crisis. Cytokines including IL-1,, IL-2, IL-4, IL-6, IL-8, TNF-,, and IFN-, were measured by commercially available ELISA kits in SCD patients (n = 60); in steady state (n = 26) and in painful crisis (n = 34) and compared with nonanemic age- and sex-matched normal Omani controls (n = 20). SCD patients in crisis showed elevated levels of TNF-, (P < 0.092) and IL-6 (P < 0.024) when compared with steady state. It was also observed that SCD patients in steady state showed a significant elevation in IL-1, (P < 0.04), IL-6 (P < 0.0001), and IFN-, (P < 0.02) as compared to normal subjects. It is thus evident that both type I and type II cytokines are significantly altered in SCD patients. In steady state, type II proinflammatory cytokines are elevated, whereas in crisis, an additional augmentation of type I cytokines occurs, with persistent elevation of type II cytokines, emphasizing the role of perturbed endothelium and activated monocytes in the pathophysiology of vaso-occlusion in sickle cell crisis. Am. J. Hematol. 77:323,328, 2004 © 2004 Wiley-Liss, Inc. [source] Emergency Department Sickle Cell Assessment of Needs and Strengths (ED-SCANS), a Focus Group and Decision Support Tool Development ProjectACADEMIC EMERGENCY MEDICINE, Issue 8 2010Paula Tanabe PhD ACADEMIC EMERGENCY MEDICINE 2010; 17:848,858 © 2010 by the Society for Academic Emergency Medicine Abstract Objectives:, A decision support tool may guide emergency clinicians in recognizing assessment, analgesic and overall management, and health service delivery needs for patients with sickle cell disease (SCD) in the emergency department (ED). We aimed to identify data and process elements important in making decisions regarding evaluation and management of adult patients in the ED with painful episodes of SCD. Methods:, Qualitative methods using a series of focus groups and grounded theory were used. Eligible participants included adult clients with SCD and emergency physicians and nurses with a minimum of 1 year of experience providing care to patients with SCD in the ED. Patients were recruited in conjunction with annual SCD meetings, and providers included clinicians who were and were not affiliated with sickle cell centers. Groups were conducted until saturation was reached and included a total of two patient groups, three physician groups, and two nurse groups. Focus groups were held in New York, Durham, Chicago, New Orleans, and Denver. Clinician participants were asked the following three questions to guide the discussion: 1) what information would be important to know about patients with SCD in the ED setting to effectively care for them and help you identify patient analgesic, treatment, and referral needs? 2) What treatment decisions would you make with this information? and 3) What characteristics would a decision support tool need to have to make it meaningful and useful? Client participants were asked the same questions with rewording to reflect what they believed providers should know to provide the best care and what they should do with the information. All focus groups were audiotaped and transcribed. The constant comparative method was used to analyze the data. Two coders independently coded participant responses and identified focal themes based on the key questions. An investigator and assistant independently reviewed the transcripts and met until the final coding structure was determined. Results:, Forty-seven individuals participated (14 persons with SCD, 16 physicians, and 17 nurses) in a total of seven different groups. Two major themes emerged: acute management and health care utilization. Major subthemes included the following: physiologic findings, diagnostics, assessment and treatment of acute painful episodes, and disposition. The most common minor subthemes that emerged included past medical history, presence of a medical home (physician or clinic), individualized analgesic treatment plan for treatment of painful episodes, history of present illness, medical home follow-up available, patient-reported analgesic treatment that works, and availability of analgesic prescription at discharge. Additional important elements in treatment of acute pain episodes included the use of a standard analgesic protocol, need for fluids and nonpharmacologic interventions, and the assessment of typicality of pain presentation. The patients' interpretation of the need for hospital admission also ranked high. Conclusions:, Participants identified several areas that are important in the assessment, management, and disposition decisions that may help guide best practices for SCD patients in the ED setting. [source] Arginine therapy: a novel strategy to induce nitric oxide production in sickle cell diseaseBRITISH JOURNAL OF HAEMATOLOGY, Issue 2 2000SHORT REPORT To determine the effects of l -arginine (l -Arg) supplementation on nitric oxide metabolite (NOx) production, oral l -Arg was given to normal controls, sickle cell disease (SCD) patients at steady state and SCD patients hospitalized with a vaso-occlusive crisis (VOC). l -Arg (0·1 g/kg) increased NOx formation by 18·8 ± 68% in normal controls, whereas steady-state SCD patients demonstrated a paradoxical decrease in NOx of ,16·7 ± 4% (P = 0·004). In contrast, patients with VOC demonstrated a dramatic increase in NOx production by +77·7 ± 103%, a response that was dose dependent. l -Arg appears to be the rate-limiting step in NOx production during VOC. Oral arginine may therefore benefit SCD patients by inducing an increase in NO production during VOC. [source] Childhood stroke in Eastern Province, KSA: pattern, risk factors, diagnosis and outcomeACTA PAEDIATRICA, Issue 10 2009Abdelhady Taha Emam Abstract Background and purpose:, Stroke has been increasingly recognized in children in recent years, but diagnosis and management can be difficult because of the diversity of underlying risk factors, atypical presentation and the absence of a uniform treatment approach. The aim of this study was to examine risk factors, clinical presentation, imaging findings and outcomes of paediatric stroke in Eastern Province, Kingdom of Saudi Arabia (KSA). Subjects and methods:, We evaluated 25 patients (11 boys and 14 girls) using computerized tomography scan of the brain, magnetic resonance (MR) imaging and MR angiography. Cardiac assessment, haematological tests, immunological tests, infection and metabolic screening were also performed in the patients. After discharge, the patients were monitored regularly in the neurology clinic to detect their outcomes. Results:, A total of 76% of the patients presented with ischaemic stroke, while the remaining 24% had haemorrhagic stroke. Sickle cell disease (SCD) was the commonest risk factor for stroke (36%) followed by non determinate causes (20%). Seizure was the commonest clinical presentation (54%) followed by haemiplegia (31%) and decreased level of consciousness (30%). Recurrence occurred in SCD patients (80%) and patients with moyamoya disease (20%). Regarding the outcome, long-term deficit was the commonest (44%), while short-term deficit and death were equal (28% each). Conclusion:, Our study in Eastern Province, KSA, showed agreement with other studies regarding risk factors, clinical presentation, imaging features and outcomes of stroke in children, yet with some points of differences, which are as follows: (1) SCD is the commonest risk factor in our study population, while in Chinese study it was not, (2) The percentage of cardiac disorders as a risk factor in this study was less than that in the European and American studies, and (3) there was relative discrepancy regarding predictors of outcome. [source] SICKLE CELL DISEASE: ROLE OF REACTIVE OXYGEN AND NITROGEN METABOLITESCLINICAL AND EXPERIMENTAL PHARMACOLOGY AND PHYSIOLOGY, Issue 9 2007Katherine C Wood SUMMARY 1Sickle cell disease (SCD) is an inherited disorder of haemoglobin synthesis that is associated with significant morbidity and mortality due to sequelae of episodic vaso-occlusive events: pain crises and multiorgan damage. The microvascular responses to the initiation, progression and resolution of vaso-occlusive events are consistent with an inflammatory phenotype as suggested by activation of multiple cell types, an oxidatively stressed environment and endothelial cell dysfunction. 2Decreased anti-oxidant defences in SCD patients and mice are accompanied by activation of enzymatic (NADPH oxidase, xanthine oxidase) and non-enzymatic (sickle haemoglobin auto-oxidation) sources of reactive oxygen species. The resultant oxidative stress leads to dysfunction/activation of arteriolar and venular endothelial cells, resulting in impaired vasomotor function and blood cell,endothelial cell adhesion. 3Changes in substrate and cofactor availability for endothelial cell nitric oxide synthase may underlie reactive oxygen- and nitrogen-induced events that contribute to SCD-induced vasculopathy. 4The emerging role of reactive oxygen and nitrogen species in the pathogenesis of SCD provides a platform for the development of novel agents to treat this painful and lethal disease. [source] | ||||||||||||||||||||||