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Acute chest syndrome (ACS) is a common cause of death for sickle cell disease patients. This syndrome is defined as: respiratory symptoms, new X-ray findings developed and/or fever; ACS requires prompt treatment to avoid clinical deterioration and death in adults with sickle cell disease. Sixteen episodes of acute chest syndrome were studied in 16 adults with sickle cell disease. The clinical and radiological findings, treatment, response and outcome of the episode were evaluated respectively. The patient's past history and comorbidities were taken into account in the outcome and days of hospitalization. Fourteen patients recovered with no sequelae; one patient who required mechanical ventilation also recovered; one patient died due to pulmonary emboli. The mean hospitalization days were 7.43. [ABSTRACT FROM AUTHOR]

Background: We tried to understand prevalence and clinical severity of sickle cell anemia (SCA) alone or sickle cell diseases (SCD) with associated alpha- or beta-thalassemias in adults. Methods: All adults with the SCA or SCD were studied. Results: The study included 441 patients (215 females). The prevalence of SCA was significantly lower than the SCD in adults (29.0% versus 70.9%, p<0.001). The mean age and female ratio were similar in the SCA and SCD groups (31.2 versus 30.5 years and 52.3% versus 47.2%, p>0.05 for both, respectively). The mean body mass index was similar in both groups, too (21.5 versus 21.7 kg/m2, p>0.05, respectively). On the other hand, the total bilirubin value of the plasma was higher in the SCA, significantly (5.7 versus 4.4 mg/dL, p= 0.000). Whereas the total number of transfused units of red blood cells in their lives was similar in the SCA and SCD groups (43.6 versus 37.1 units, p>0.05, respectively). Conclusion: The SCA alone and SCD are severe inflammatory processes on vascular endothelium particularly at the capillary level, and terminate with an accelerated atherosclerosis and end-organ failures in early years of life. The relatively suppressed hemoglobin S synthesis in the SCD secondary to the associated thalassemias may decrease sickle cell-induced chronic endothelial damage, inflammation, edema, fibrosis, and end-organ failures. The lower prevalence of the SCA in adults and the higher total bilirubin value of the plasma in them may indicate the relative severity of hemolytic process, vascular endothelial inflammation, and hepatic involvement in the SCA. [ABSTRACT FROM AUTHOR]

Background: We tried to understand whether or not there is a significant relationship between acute chest syndrome (ACS) and atherosclerosis in sickle cell diseases (SCD). Methods: All patients with the SCD were included. Results: The study included 434 patients (222 males) with similar mean ages in male and female genders (30.8 versus 30.3 years, respectively, p>0.05). Smoking (23.8% versus 6.1%, p<0.001) and alcohol (4.9% versus 0.4%, p<0.001) were higher in males, significantly. Transfused units of red blood cells (RBC) in their lives (48.1 versus 28.5, p=0.000) were also higher in males, significantly. Similarly, disseminated teeth losses (<20 teeth present) (5.4% versus 1.4%, p<0.001), chronic obstructive pulmonary disease (COPD) (25.2% versus 7.0%, p<0.001), ileus (7.2% versus 1.4%, p<0.001), cirrhosis (8.1% versus 1.8%, p<0.001), leg ulcers (19.8% versus 7.0%, p<0.001), digital clubbing (14.8% versus 6.6%, p<0.001), coronary heart disease (CHD) (18.0% versus 13.2%, p<0.05), chronic renal disease (CRD) (9.9% versus 6.1%, p<0.05), and stroke (12.1% versus 7.5%, p<0.05) were all higher in males but not ACS (2.7% versus 3.7%, p>0.05) in the SCD. Conclusion: SCD are severe inflammatory processes on vascular endothelium, particularly at the capillary level since the capillary system is the main distributor of hardened RBC into the tissues. Although the higher smoking and alcohol-like strong atherosclerotic risk factors and disseminated teeth losses, COPD, ileus, cirrhosis, leg ulcers, digital clubbing, CHD, CRD, and stroke-like obvious atherosclerotic consequences in male gender, ACS was not higher in them, significantly. In another definition, ACS may not have an atherosclerotic background in the SCD. [ABSTRACT FROM AUTHOR]

Summary: Intravenous fluid therapy (IV‐FT) is routinely used in the treatment of vaso‐occlusive crises (VOCs), as dehydration possibly promotes and sustains erythrocyte sickling. Patients with sickle cell disease (SCD) are at risk of developing diastolic dysfunction and fluid overload due to IV‐FT. However, data on the adverse effects of IV‐FT for VOC is sparse. We aimed to evaluate the incidence and risk factors of fluid overload due to IV‐FT in patients with SCD. Consecutive hospitalisations for VOC treated with IV‐FT between September 2016 and September 2018 were retrospectively analysed. The median (interquartile range) age was 25·0 (18·3–33·8) years and 65% had a severe genotype (HbSS/HbSβ0‐thal). Fluid overload occurred in 21% of 100 patients. Hospital stay was longer in patients with fluid overload (6·0 vs. 4·0 days, P = 0·037). A positive history of fluid overload (P = 0·017), lactate dehydrogenase level (P = 0·011), and top‐up transfusion during admission (P = 0·005) were independently associated with fluid overload occurrence. IV‐FT was not reduced in 86% of patients despite a previous history of fluid overload. Fluid overload is frequently encountered during IV‐FT for VOC. IV‐FT is often not adjusted despite a positive history of fluid overload or when top‐up transfusion is indicated, emphasising the need for more awareness of this complication and a personalised approach. [ABSTRACT FROM AUTHOR]

Background: We tried to understand prevalence of chronic obstructive pulmonary disease (COPD) in both genders in sickle cell diseases (SCD). Methods: All cases with the SCD in the absence of smoking and alcohol were included. Results: The study included 368 patients (168 males). Mean ages were similar in males and females (29.4 versus 30.2 years, respectively, p>0.05). Mean values of body mass index (BMI) were similar in males and females, too (21.7 versus 21.6 kg/m2, respectively, p>0.05). Interestingly, total bilirubin value of the plasma (5.2 versus 4.0 mg/dL, p=0.011), transfused units of red blood cells (RBC) in their lives (46.8 versus 29.2, p=0.002), COPD (20.8% versus 6.0%, p<0.001), and digital clubbing (13.0% versus 5.5%, p<0.001) were all higher in males. Whereas painful crises per year (5.0 versus 5.0), pulmonary hypertension (10.1% versus 12.5%), acute chest syndrome (2.3% versus 3.5%), mortality (8.3% versus 6.5%), and mean age of mortality (29.0 versus 32.5 years) were similar in males and females, respectively (p>0.05 for all). Conclusion: SCD are severe inflammatory processes on vascular endothelium particularly at the capillary level, since capillary system is the main distributor of hardened RBC into tissues. The capillary endothelial damage, inflammation, edema, and fibrosis induced hypoxia may be the underlying cause of COPD in the SCD. Although the similar BMI and absence of smoking and alcohol, the much higher prevalence of COPD may be explained by the dominant role of male sex in life according to the physical power that may accelerate systemic atherosclerotic process in whole body. [ABSTRACT FROM AUTHOR]

Background: We tried to understand roles of systemic disorders in the pathophysiology of rheumatic heart disease (RHD). Methods: All patients who applied to the Internal Medicine Units were included. Results: We detected 29 females and 20 males with RHD. Their mean ages were 42.7 versus 33.0 years, respectively (p= 0.016). There were 26 cases with sickle cell diseases (SCDs), two cases with rheumatoid arthritis (RA), two cases with systemic lupus erythematosus (SLE), one case with juvenile rheumatoid arthritis (JRA), one case with common variable immunodeficiency, one case with celiac sprue, two cases with cirrhosis, and one case with chronic renal disease. In other words, there were associated systemic pathologies in 73.4% of RHD patients. Mitral valve was involved in 41.3%, mitral and aortic valves were involved in 26.0%, mitral and tricuspid valves were involved in 17.3%, aortic valve was involved in 8.6%, and all of the three valves were involved in 6.5% of the cases. In another definition, mitral, aortic, and tricuspid valves were involved in 91.3%, 41.3%, and 23.9% of cases, respectively. Interestingly, tricuspid valve was never involved alone, instead always together with mitral valve. There were 69.3% of patients with mitral regurgitation, 26.5% with aortic regurgitation, 22.4% with tricuspid regurgitation, 20.4% with mitral stenosis, and 18.3% with aortic stenosis. Conclusion: Systemic disorders including SCDs, RA, SLE, and JRA induce chronic inflammatory processes on vascular endothelium in the whole body, and terminate with an immunosuppression by several mechanisms that may be the cause of higher prevalence of RHD together with such disorders. [ABSTRACT FROM AUTHOR]

Background: Pulmonary complications of sickle cell disease (SCD) are diverse and encompass acute and chronic disease. The understanding of the natural history of pulmonary complications of SCD is limited, no specific therapies exist, and these complications are a primary cause of morbidity and mortality.Methods: We gathered a multidisciplinary group of pediatric and adult hematologists, pulmonologists, and emergency medicine physicians with expertise in SCD-related lung disease along with an SCD patient advocate for an American Thoracic Society-sponsored workshop to review the literature and identify key unanswered clinical and research questions. Participants were divided into four subcommittees on the basis of expertise: 1) acute chest syndrome, 2) lower airways disease and pulmonary function, 3) sleep-disordered breathing and hypoxia, and 4) pulmonary vascular complications of SCD. Before the workshop, a comprehensive literature review of each subtopic was conducted. Clinically important questions were developed after literature review and were finalized by group discussion and consensus.Results: Current knowledge is based on small, predominantly observational studies, few multicenter longitudinal studies, and even fewer high-quality interventional trials specifically targeting the pulmonary complications of SCD. Each subcommittee identified the three or four most important unanswered questions in their topic area for researchers to direct the next steps of clinical investigation.Conclusions: Important and clinically relevant questions regarding sickle cell lung disease remain unanswered. High-quality, multicenter, longitudinal studies and randomized clinical trials designed and implemented by teams of multidisciplinary clinician-investigators are needed to improve the care of individuals with SCD. [ABSTRACT FROM AUTHOR]

Background: We tried to understand whether or not there is a lower prevalence of systemic lupus erythematosus (SLE) due to an immunosuppression in the sickle cell diseases (SCDs). Methods: All patients with the SCDs and age and sex-matched controls were studied. Results: The study included 428 patients with the SCDs (220 males) and 433 controls (223 males). Mean ages of the SCDs patients were similar in males and females (30.6 versus 30.1 years, respectively, p>0.05). Both smoking (24.0% versus 6.2%) and alcohol (5.0% versus 0.4%) were higher in males with the SCDs (p<0.001 for both). Although SLE was diagnosed in 6.0% of the control cases (24 females and two males), this ratio was only 0.4% (one female and one male) in the SCDs patients (p<0.001). On the other hand, transfused units of red blood cells in their lives (47.6 versus 28.4, p=0.000), chronic obstructive pulmonary disease (25.4% versus 7.2%, p<0.001), ileus (7.2% versus 1.4%, p<0.001), cirrhosis (7.2% versus 1.9%, p<0.001), leg ulcers (20.0% versus 7.2%, p<0.001), digital clubbing (14.0% versus 6.2%, p<0.001), coronary artery disease (18.1% versus 12.9%, p<0.05), chronic renal disease (10.4% versus 6.2%, p<0.05), and stroke (12.2% versus 7.6%, p<0.05) were all higher in males with SCDs. Conclusion: SCDs are severe and continuous inflammatory processes on vascular endothelium, particularly at the capillary level, and terminate with end-organ failure in early years of life. Beside that, SCDs may cause moderate to severe immunosuppression by several mechanisms that may be the cause of the lower prevalence of SLE in the SCDs. [ABSTRACT FROM AUTHOR]

Summary: ACS (ACS) is a serious complication of sickle cell anaemia (SCA). We set out to describe the burden, presentation and organisms associated with ACS amongst children with SCA attending Mulago Hospital, Kampala, Uganda. In a cross‐sectional study, 256 children with SCA and fever attending Mulago Hospital were recruited. Chest X‐rays, blood cultures, complete blood count and sputum induction were performed. Sputum samples were investigated by Ziehl‐Nielsen staining, culture and DNA polymerase chain reaction (PCR) for Chlamydia pneumoniae. Of the 256 children, 22·7% had ACS. Clinical and laboratory findings were not significantly different between children with ACS and those without, besides cough and abnormal signs on auscultation. Among the 83 sputum cultures Streptococcus pneumoniae (12%) and Moraxella spp (8%), were the commonest. Of the 59 sputa examined with DNA PCR, 59·3% were positive for Chlamydia pneumoniae. Mycobacterium tuberculosis was isolated in 6/83 sputa. These results show that one in 5 SCA febrile children had ACS. There were no clinical and laboratory characteristics of ACS, but cough and abnormalities on auscultation were associated with ACS. The high prevalence of Chlamydia pneumoniae in children with ACS in this setting warrants the addition of macrolides to treatment, and M. tuberculosis should be differential in sub‐Saharan children with ACS. [ABSTRACT FROM AUTHOR]

Sickle cell disease (SCD) is one of the most common single disease disorders world-wide. It is remarkable for its clinical heterogeneity, even among individuals with identical genotypes. Some individuals experience morbidity and mortality in early childhood, while others have a relatively mild course, and normal or near normal life expectancy. Many clinical complications are associated with SCD; most notably frequent pain episodes, stroke, acute chest syndrome, avascular necrosis, nephropathy, retinopathy and pulmonary hypertension. While the effects of higher fetal hemoglobin (HbF) levels, UGTA1A polymorphisms, alpha-thalassemia and G6PD deficiency on SCD has been extensively studied, these variables do not explain all of the clinical heterogeneity of SCD. It is not known why some patients develop certain complications, and it is difficult to predict which complications a particular patient will experience. Much work has been done to identify genetic variants associated with these disease complications; many associations remain unvalidated. As the field continues to move beyond small sample collections and candidate gene approaches into whole genome sequencing and merging of samples from all over the world, we will identify more genetic variants associated with development of specific SCD related complications, and hopefully leverage this knowledge into targeted therapies. [ABSTRACT FROM AUTHOR]

Background: There is a high burden of tuberculosis (TB) in HIV antiretroviral programmes in Africa. However, few studies have looked at predictors of incident TB while on Truvada-based combination antiretroviral therapy (cART) regimens. Methods: We estimated TB incidence among individuals enrolled into an observational cohort evaluating the efficacy and tolerability of Truvada-based cART in Gaborone, Botswana between 2008 and 2011. We used Cox proportional hazards regressions to determine predictors of incident TB. Results: Of 300 participants enrolled, 45 (15%) had a diagnosis of TB at baseline. During 428 person-years (py) of follow-up, the incidence rate of TB was 3.04/100py (95% CI, 1.69–5.06), with 60% of the cases occurring within 3 months of ART initiation. Incident cases had low baseline CD4+ T cell counts (153cells/mm3 [Q1, Q3: 82, 242]; p = 0.69) and hemoglobin levels (9.2g/dl [Q1, Q3: 8.5,10.1]; p<0.01). In univariate analysis, low BMI (HR = 0.73; 95% CI 0.58–0.91; p = 0.01) and hemoglobin levels <8 g/dl (HR = 10.84; 95%CI: 2.99–40.06; p<0.01) were risk factors for TB. Time to incident TB diagnosis was significantly reduced in patients with poor immunological recovery (p = 0.04). There was no association between baseline viral load and risk of TB (HR = 1.75; 95%CI: 0.70–4.37). Conclusion: Low hemoglobin levels prior to initiation of ART are significant predictors of incident tuberculosis. Therefore, there is potential utility of iron biomarkers to identify patients at risk of TB prior to initiation on ART. Furthermore, additional strategies are required for patients with poor immunological recovery to reduce excess risk of TB while on ART. [ABSTRACT FROM AUTHOR]

Background: Hematological changes can drive damage of endothelial cells, which potentially lead to an early endothelial dysfunction in patients with sickle cell anemia (SCA). An association may exist between endothelial dysfunction and several clinical manifestations of SCA. The present study aims to evaluate the links between changes in endothelial function and clinical and laboratory parameters in children and adolescents with SCA. Methods: This study included 40 children and adolescents with stable SCA as well as 25 healthy children; aged 6–18 years. All study subjects were evaluated for endothelial function using Doppler ultrasonography. In addition, a number of laboratory assays were performed, including reticulocyte and leukocyte counts as well as measurement of circulating levels of total bilirubin, C-reactive protein (CRP), glucose, lipoproteins and peripheral oxyhemoglobin saturation. These parameters were also compared between SCA patients who were undertaking hydroxyurea (HU) and those who were not. Results: Flow-mediated vasodilation (FMD) values were found to be reduced in SCA patients compared with those detected in healthy controls. SCA individuals with lower FMD values exhibited higher number of hospital admissions due to vaso-occlusive events. Additional analyses revealed that patients who had decreased FMD values exhibited higher odds of acute chest syndrome (ACS) episodes. A preliminary analysis with limited number of individuals failed to demonstrate significant differences in FMD values between SCA individuals who were treated with HU and those who were not. Conclusions: Children and adolescents with SCA exhibit impaired endothelial function. Reductions in FMD values are associated with ACS. These findings underline the potential use of FMD as screening strategy of SCA patients with severe prognosis at early stages. [ABSTRACT FROM AUTHOR]

Background: Sickle cell diseases (SCDs) are accelerated atherosclerotic processes. We tried to understand whether or not there is an atherosclerotic background of benign prostatic hyperplasia in the SCDs patients. Methods: All patients with the SCDs were included into the study. Results: The study included 428 patients (220 males). The mean ages were similar in males and females (30.6 versus 30.1 years, respectively, P>0.05). Smoking (24.0% versus 6.2%) and alcohol (5.0% versus 0.4%) were significantly higher in males (P<0.001 for both). Transfused units of red blood cells in their lives (47.6 versus 28.4, P=0.000), chronic obstructive pulmonary disease (25.4% versus 7.2%, P<0.001), ileus (7.2% versus 1.4%, P<0.001), cirrhosis (7.7% versus 1.9%, P<0.001), leg ulcers (20.0% versus 7.2%, P<0.001), digital clubbing (14.0% versus 6.2%, P<0.001), coronary artery disease (18.1% versus 12.9%, P<0.05), chronic renal disease (10.4% versus 6.2%, P<0.05), and stroke (12.2% versus 7.6%, P<0.05) were all higher in males, too. There were 11 males (5.0%) with lower urinary tract symptoms (LUTS) including urgency, weak stream, incomplete emptying, and nocturia with a mean age of 41.4 years. All patients could be treated with once daily 4 milligrams of doxazosin, orally. Conclusion: SCDs are chronic inflammatory processes on vascular endothelium particularly at the capillary level, and terminate with accelerated atherosclerosis induced end-organ failures in early years of life. Although the relatively younger mean age of the patients, LUTS are probably due to the disseminated endothelial damage, inflammation, edema, and fibrosis both in the arterial and venous systems of the prostate in the SCDs. [ABSTRACT FROM AUTHOR]

Background: Sickle cell diseases (SCDs) are chronic catastrophic processes on vascular endothelium initiating at birth all over the body. We tried to understand whether or not there is a chronic inflammatory background of acute chest syndrome (ACS) in the SCDs. Methods: All patients with the SCDs were taken into the study. Results: The study included 411 patients (199 females). As one of the significant endpoints of SCDs, patients with chronic obstructive pulmonary disease (COPD) and without were collected into two groups. There were 60 patients (14.5%) with COPD. Mean age (33.0 versus 29.5 years, P=0.005) and male ratio (80.0% versus 46.7%, P<0.001) were higher in the COPD group. Smoking (36.6% versus 9.9%, P<0.001) and alcohol (3.3% versus 0.8%, P<0.05) were also higher among the COPD cases. Transfused red blood cell units in their lives (69.1 versus 32.9, P=0.001), priapism (10.0% versus 1.9%, P<0.001), leg ulcers (26.6% versus 11.6%, P<0.001), digital clubbing (25.0% versus 7.1%, P<0.001), coronary heart disease (26.6% versus 13.1%, P<0.01), chronic renal disease (16.6% versus 7.1%, P<0.01), and stroke (20.0% versus 7.9%, P<0.001) were all higher among the COPD cases, too. Interestingly, against the higher rates of the above problems in the COPD group, incidence of ACS was even lower among them, nonsignificantly (1.6% versus 3.9%, P>0.05). Conclusion: SCDs cause severe chronic endothelial damage particularly at the capillary level, and terminate with accelerated atherosclerosis induced end-organ failures in early years of life. Probably ACS is a sudden onset event without any chronic inflammatory background in the SCDs. [ABSTRACT FROM AUTHOR]

Background: Sickle cell diseases (SCDs) are accelerated atherosclerotic processes. We tried to understand whether or not there is a prolonged survival with the increased number of red blood cells (RBC) transfusion in the SCDs. Methods: As one of the significant endpoints of the SCDs, cases with chronic obstructive pulmonary disease (COPD) and without, were collected into the two groups. Results: The study included 428 patients (221 males). There were 71 patients (16.5%) with COPD. Mean age was significantly higher in the COPD group (32.8 versus 29.8 years, P=0.005). Male ratio was significantly higher in the COPD group, too (78.8% versus 46.2%, P<0.001). Smoking (35.2% versus 11.4%, P<0.001) and alcohol (7.0% versus 1.9%, P<0.01) were also higher among the COPD cases. Beside these, priapism (14.0% versus 3.0%, P<0.001), HCV RNA positivity (2.7% versus 0.5%, P<0.05), cirrhosis (8.4% versus 3.3%, P<0.05), leg ulcers (23.9% versus 12.0%, P<0.01), digital clubbing (25.3% versus 6.7%, P<0.001), coronary heart disease (23.9% versus 13.7%, P<0.05), chronic renal disease (15.4% versus 7.0%, P<0.01), stroke (16.9% versus 8.1%, P<0.01), and mean transfused RBC units in their lives (63.8 versus 33.0, P=0.003) were all higher among the COPD cases. This was probably due to the higher number of transfused RBC units; the mean age of mortality was also higher in the COPD group, significantly (38.3 versus 30.4 years, P=0.04). Conclusion: SCDs are chronic catastrophic processes on vascular endothelium terminating with accelerated atherosclerosis induced endorgan failures in early years of life. RBC supports in severe clinical conditions probably prolong survival of the patients. [ABSTRACT FROM AUTHOR]

Objective : Background: We tried to understand whether or not there are some positive correlations between leg ulcers and severity of sickle cell diseases (SCDs). Methods: All patients with SCDs were taken into the study. Results: The study included 346 patients with the SCDs (175 males). There were 50 cases (14.4%) with leg ulcers. Interestingly, the male ratio was significantly higher in patients with leg ulcers (74.0% ver- sus 46.6%, p<0.001). Additionally, mean ages of the patients with leg ulcers were significantly higher than the patients without (35.0 versus 28.5 years, p<0.000). Prevalence of associated thalassemia minor was similar in both groups (64.0% versus 66.5%, respectively, p>0.05). On the other hand, smoking was significantly higher in patients with leg ulcers (28.0% versus 11.8%, p<0.05). Although the mean white blood cell and platelet counts of the peripheric blood were similar in both groups (p>0.05 for both), the mean hematocrit value was significantly lower in patients with leg ulcers (21.7% versus 24.0%, p= 0.002). On the other hand, although the painful crises per year, priapism, pulmonary hypertension, chronic obstructive pulmonary disease, coronary heart disease, rheumatic heart disease, and avascular necrosis of bones were all higher in patients with leg ulcers, the differences were only significant for digital clubbing, chronic renal disease, and stroke (p<0.05 for all). Conclusion: SCDs are chronic destructive processes on capillaries initiating at birth, and terminate with early organ failures in life. Probably leg ulcers are found among the terminal consequences of the inflammatory processes that may indicate shortened survival. [ABSTRACT FROM AUTHOR]

IMPORTANCE Sickle cell disease (SCD) is a life-threatening genetic disorder affecting nearly 100 000 individuals in the United States and is associated with many acute and chronic complications requiring immediate medical attention. Two disease-modifying therapies, hydroxyurea and long-term blood transfusions, are available but underused. OBJECTIVE To support and expand the number of health professionals able and willing to provide care for persons with SCD. EVIDENCE REVIEW Databases of MEDLINE (including in-process and other nonindexed citations), EMBASE, Cochrane Database of Systematic Reviews, Cochrane Central Register of Controlled Trials, CINAHL, TOXLINE, and Scopus were searched using prespecified search terms and keywords to identify randomized clinical trials, nonrandomized intervention studies, and observational studies. Literature searches of English-language publications from 1980 with updates through April 1,2014, addressed key questions developed by the expert panel members and methodologists. FINDINGS Strong recommendations for preventive services include daily oral prophylactic penicillin up to the age of 5 years, annual transcranial Doppler examinations from the ages of 2 to 16 years in those with sickle cell anemia, and long-term transfusion therapy to prevent stroke in those children with abnormal transcranial Doppler velocity (>200 cm/s). Strong recommendations addressing acute complications include rapid initiation of opioids for treatment of severe pain associated with a vasoocclusive crisis, and use of incentive spirometry in patients hospitalized for a vasoocclusive crisis. Strong recommendations for chronic complications include use of analgesics and physical therapy for treatment of avascular necrosis, and use of angiotensin-converting enzyme inhibitor therapy for microalbuminuria In adults with SCD. Strong recommendations for children and adults with proliferative sickle cell retinopathy include referral to expert specialists for consideration of laser photocoagulation and for echocardiography to evaluate signs of pulmonary hypertension. Hydroxyurea therapy is strongly recommended for adults with 3 or more severe vasoocclusive crises during any 12-month period, with SCD pain or chronic anemia interfering with daily activities, or with severe or recurrent episodes of acute chest syndrome. A recommendation of moderate strength suggests offering treatment with hydroxyurea without regard to the presence of symptoms for Infants, children, and adolescents. In persons with sickle cell anemia, preoperative transfusion therapy to increase hemoglobin levels to 10 g/dL is strongly recommended with a moderate strength recommendation to maintain sickle hemoglobin levels of less than 30% prior to the next transfusion during long-term transfusion therapy. A strong recommendation to assess iron overload is accompanied by a moderate strength recommendation to begin iron chelation therapy when indicated. CONCLUSIONS AND RELEVANCE Hydroxyurea and transfusion therapy are strongly recommended for many individuals with SCD. Many other recommendations are based on quality of evidence that is less than high due to the paucity of clinical trials regarding screening, management, and monitoring for individuals with SCD. [ABSTRACT FROM AUTHOR]

Introduction The lung computed tomography (CT) features of acute chest syndrome (ACS) in sickle cell disease patients is not well described, and the diagnostic performance of bedside chest radiograph (CR) has not been tested. Our objectives were to describe CT features of ACS and evaluate the reproducibility and diagnostic performance of bedside CR. Methods We screened 127 consecutive patients during 166 ACS episodes and 145 CT scans (in 118 consecutive patients) were included in the study. Results Among the 145 CT scans, 139 (96%) exhibited a new pulmonary opacity and 84 (58%) exhibited at least one complete lung segment consolidation. Consolidations were predominant as compared with ground-glass opacities and atelectasis. Lung parenchyma was increasingly consolidated from apex to base; the right and left inferior lobes were almost always involved in patients with a new complete lung segment consolidation on CT scan (98% and 95% of cases, respectively). Patients with a new complete lung segment consolidation on CT scan had a more severe presentation and course as compared with others. The sensitivity of bedside CR for the diagnosis of ACS using CT as a reference was good (>85%), whereas the specificity was weak (<60%). Conclusions ACS more frequently presented on CT as a consolidation pattern, predominating in lung bases. The reproducibility and diagnostic capacity of bedside CR were far from perfect. These findings may help improve the bedside imaging diagnosis of ACS. [ABSTRACT FROM AUTHOR]

The pulmonary complications of sickle cell disease are a major cause of morbidity and mortality in affected patients. The acute chest syndrome (ACS) is a leading cause of death in patients with sickle cell disease and has a multifactorial etiology. Hydroxyurea (HU), stem cell transplantation (SCT) and chronic transfusions are known to prevent the recurrence of ACS. Careful management of patients admitted for pain crises and surgery including use of incentive spirometry is critical in preventing this complication. Pulmonary hypertension is well known to be associated with sickle cell disease and patients with pulmonary hypertension have increased mortality. Asthma is also commonly seen in patients with sickle cell disease and is associated with a more complicated course. Chronic lung disease develops in a significant proportion of patients with sickle cell disease. [ABSTRACT FROM AUTHOR]

Acute chest syndrome (ACS) is a common complication and reason for hospital admission in patients with sickle cell disease (SCD). It is also the most common cause of death in this patient population. Most of the time, the trigger for ACS in an individual patient cannot be identified. However, although infection is the most common identifiable cause for ACS, other important triggers are vaso-occlusive crisis (VOC) and asthma. This comprehensive review will focus on the pathogenesis, clinical characteristics, complications and treatment available to manage ACS. But importantly, this review will highlight new possible etiologies, with the goal of improving oxygenation and, therefore, a reduction in sickling and lung damage in this patient population. [ABSTRACT FROM AUTHOR]

Features Spectra Clinical Research in Rockleigh, New Jersey. Corporate description; Markets served; Services.

Purpose To test the feasibility of a method to quantify regional pulmonary parenchymal motion via nonrigid registration algorithm at small animal scales. Materials and Methods Voxel-wise displacement vector field maps were generated between end-inspiratory and end-expiratory coronal thoracic MR images on normal mice ( N = 5) to analyze the magnitude and direction of parenchymal motion in the segmented regions. The analysis was repeated before and after short-term exposure to hypoxia to demonstrate the effect of hypoxia on the respiratory motion in transgenic (Tg) mice with sickle cell disease (SCD) ( N = 4). Results Normal mice revealed that the right and left lungs moved symmetrically but that there was greater movement in the lower regions than in the upper regions. Calculated strain was uniform in the entire lung. In the Tg mice, the pulmonary motion before hypoxia was similar to that observed in the normal mice. Upon exposure to hypoxia, the displacement magnitude reduced and the direction of motion in some areas became distorted. Conclusion MR quantification of pulmonary motion was feasible in mice and the principle that the method could detect mechanical abnormalities due to pathologic changes was proven. Quantification of pulmonary motion has the potential to lead to earlier disease diagnosis and better monitoring of disease treatments. J. Magn. Reson. Imaging 2007. © 2007 Wiley-Liss, Inc. [ABSTRACT FROM AUTHOR]

BACKGROUND AND OBJECTIVES: Genetic studies suggest that the sickle cell mutation has arisen on at least four separate occasions in Africa and as a fifth independent mutation in the Eastern Province of Saudi Arabia or India. The pathophysiology of sickle cell disease (SCD) is essentially similar in these different areas although the frequency and severity of complications may vary between areas. The aim of this study was to evaluate the prevalence and outcome of acute chest syndrome (ACS) in SCD patients from Eastern province of Saudi Arabia in comparison with patients with African haplotypes. MATERIALS AND METHODS: This was a retrospective study involving 317 SCD patients who were two years or older, admitted to King Fahad Hospital Hofuf between January-May 2003 for different etiologies. Twenty six patients presented with different causes of ACS; 11 patients presented with different pathologies other than ACS, but had past history of ACS; 280 patients presented with different pathologies and never presented with ACS. Clinical features, CBC, Hb-electrophoresis, G6PD activity, cultures, chest X-ray, arterial oxygen saturation, blood transfusion rates and outcome were studied. Univariate and multiple regression analysis were carried out to evaluate influence on ACS. Comparison between SCD patients with ACS from this study and from Eastern province of Saudi Arabia to patients with African haplotypes were carried out, using data reported in the literature. RESULTS: During the period of this study, 37 patients with new or previous episodes of ACS were studied (accounting for 11.67% of admitted SCD patients). Most of the patients with ACS had only one episode, but five patients (13.51%) had had episodes or more. One patient died giving an in-hospital mortality rate of 1/26 (3.85%). Comparison of recurrence of ACS and mortality between SCD patients in Eastern province of Saudi Arabia to that of patients with African haplotype showed that recurrence is significantly lower (P<0.025) in patients from Eastern province compared to patients with African haplotype, mortality also is lower but not statistically significant. CONCLUSION: Acute chest syndrome in SCD patients in Eastern province of Saudi Arabia is relatively uncommon, but causes significant morbidity and mortality. Its prevalence and recurrence is low if compared to that of patients with African haplotypes. [ABSTRACT FROM AUTHOR]

Reviews the case of a 35-year-old woman hospitalized with fever for 2 weeks and 3 days of increasing shortness of breath and pleuritic chest pain. Personal history of the woman; Clinical diagnosis of compound heterozygosity for hemoglobulin S (Hb S) and Hb C disease; Coexistence of the diseases with AIDS.

Acute chest syndrome (ACS) is defined as the development of a new pulmonary infiltrate and respiratory symptoms in a patient with sickle cell disease (SCD). One of the most serious complications of SCD, ACS is the leading cause of mortality in patients with SCD. ACS is age dependent, with children having milder disease that often is infectious. Adults often have more severe disease, with pulmonary fat embolism secondary to preceding long bone infarction frequently as a contributing factor. Rapid diagnosis and a high index of suspicion are crucial since this syndrome may have a high mortality rate. A high white blood cell count and a falling hemoglobin tend to be associated with this illness. Patients are often febrile, but may not have positive blood or sputum cultures. Appropriate therapy includes judicious fluids, close attention to respiratory care, antibiotics, and transfusion therapy. Use of the drug, hydroxyurea, has been shown to decrease the incidence of ACS. Patients with repeated episodes are at risk for the development of chronic lung disease and pulmonary hypertension. New treatment strategies such as inhibitors of cytokines and pulmonary vasodilators such as nitric oxide may reduce the high mortality of ACS. [ABSTRACT FROM AUTHOR]