Original Article
Causes and Outcomes of the Acute Chest Syndrome in Sickle Cell Disease
N Engl J Med 2000; 342:1855-1865June 22, 2000
- Abstract
Background
The acute chest syndrome is the leading cause of death among patients with sickle cell disease. Since its cause is largely unknown, therapy is supportive. Pilot studies with improved diagnostic techniques suggest that infection and fat embolism are underdiagnosed in patients with the syndrome.
Methods
In a 30-center study, we analyzed 671 episodes of the acute chest syndrome in 538 patients with sickle cell disease to determine the cause, outcome, and response to therapy. We evaluated a treatment protocol that included matched transfusions, bronchodilators, and bronchoscopy. Samples of blood and respiratory tract secretions were sent to central laboratories for antibody testing, culture, DNA testing, and histopathological analyses.
Results
Nearly half the patients were initially admitted for another reason, mainly pain. When the acute chest syndrome was diagnosed, patients had hypoxia, decreasing hemoglobin values, and progressive multilobar pneumonia. The mean length of hospitalization was 10.5 days. Thirteen percent of patients required mechanical ventilation, and 3 percent died. Patients who were 20 or more years of age had a more severe course than those who were younger. Neurologic events occurred in 11 percent of patients, among whom 46 percent had respiratory failure. Treatment with phenotypically matched transfusions improved oxygenation, with a 1 percent rate of alloimmunization. One fifth of the patients who were treated with bronchodilators had clinical improvement. Eighty-one percent of patients who required mechanical ventilation recovered. A specific cause of the acute chest syndrome was identified in 38 percent of all episodes and 70 percent of episodes with complete data. Among the specific causes were pulmonary fat embolism and 27 different infectious pathogens. Eighteen patients died, and the most common causes of death were pulmonary emboli and infectious bronchopneumonia. Infection was a contributing factor in 56 percent of the deaths.
Conclusions
Among patients with sickle cell disease, the acute chest syndrome is commonly precipitated by fat embolism and infection, especially community-acquired pneumonia. Among older patients and those with neurologic symptoms, the syndrome often progresses to respiratory failure. Treatment with transfusions and bronchodilators improves oxygenation, and with aggressive treatment, most patients who have respiratory failure recover.
Media in This Article
Figure 1
Protocol for Treatment and Monitoring.Table 1
Characteristics of the Patients.Article Activity
- Article
The acute chest syndrome is the leading cause of death and hospitalization among patients with sickle cell disease.1-3 The optimal treatment has not been established because the cause remains largely unknown. Both infectious and noninfectious causes have been described, but their frequency and clinical course are unknown.4-12 Pulmonary fat embolism has been identified during autopsies and in studies examining fat-laden pulmonary macrophages from bronchoalveolar fluid.4,11-13 However, the number of patients included in these reports is too small for general recommendations to be made.11
Appropriate therapy remains controversial. The antibiotics used are based on organisms identified decades ago, and data regarding the use of new antibiotics for emerging pathogens are limited. Antiviral treatments have not been carefully studied in patients with sickle cell disease. Furthermore, the benefit of transfusion and bronchodilator therapy has not been clearly established.
In 1993, we initiated a prospective, multicenter study of the acute chest syndrome in order to determine the causes, incidence, and clinical outcome of the syndrome and factors that predict prognosis.
Methods
Patients
Patients were enrolled at 30 centers. Each institution had a principal investigator, data coordinator, pulmonologist, and nurse assigned to the study. Patients were enrolled after they or their parents or guardians provided written informed consent. The institutional review board at each participating center approved the study. To be eligible, patients had to have a phenotype of hemoglobin SS, hemoglobin SC, or hemoglobin SS–β-thalassemia on electrophoretic analysis of the hemoglobin chains and to have had an episode of the acute chest syndrome. The acute chest syndrome was defined on the basis of the finding of a new pulmonary infiltrate involving at least one complete lung segment that was consistent with the presence of alveolar consolidation, but excluding atelectasis. In addition, the patients had to have chest pain, a temperature of more than 38.5°C, tachypnea, wheezing, or cough.
From March 1993 through March 1997, 721 episodes of the acute chest syndrome were reported. Fifty episodes were excluded: 30 because of missing forms, and 20 because of diagnostic error and withdrawal from the study. Data were available on 671 episodes in 538 patients, including 443 patients with a single episode and 95 with recurrent episodes (69 with two episodes, 16 with three episodes, 8 with four episodes, and 2 with five episodes).
Treatment Protocol and Data Collection
A standardized treatment and monitoring protocol was used (Figure 1Figure 1
Protocol for Treatment and Monitoring.). Transfusion guidelines included the use of units that were depleted of leukocytes, negative for sickle cells, and matched with respect to Rh C and E antigens and Kell antigens. Patients were monitored for alloantibodies by standard tests at enrollment, before and after each transfusion, and at follow-up. Patients received transfusions to improve respiratory status at the discretion of their physicians. Standardized forms were used to document medical history, findings on daily physical examinations, findings on radiography, oxygenation status, the need for transfusions, complications of bronchoscopy, and findings on follow-up.Laboratory Studies and Diagnostic Criteria
Blood for culture was obtained before therapy whenever possible. Bronchoscopy was performed to obtain samples for aerobic and anaerobic cultures and for the fat embolism assay. In patients who did not undergo bronchoscopy, sputum was collected either after the inhalation of aerosolized saline or by tracheal aspiration.11 A bacterial cause of the acute chest syndrome was identified on the basis of a positive blood culture or heavy growth of an organism in a bronchial culture with confirmatory results on Gram's staining.
Bronchial and nasopharyngeal samples were used for standard viral cultures,14 and cultures for mycoplasma were performed with use of SP4 and A8 mediums.15 All viral and mycoplasma isolates were identified by immunofluorescence staining with specific reagents. Legionella pneumophila serogroups 1 through 6 were detected by indirect immunofluorescence staining.
Serum samples were collected during the acute phase of illness and during convalescence; the median interval between sample collection was 48 days. An increase in antibody titers by a factor of at least 4 during this interval was used to diagnose Mycoplasma pneumoniae, 16 parvovirus B19,17 and Epstein–Barr virus.18 Increases were confirmed by specific IgM antibody tests.
In a subgroup of patients, pairs of serologic samples and bronchoscopic or sputum samples were sent to the laboratory of Dr. Julias Schachter (University of California, San Francisco) for culture for chlamydia and for measurement of chlamydia antibody titers according to the microimmunofluorescence technique.19 Nasopharyngeal samples were further analyzed by the polymerase chain reaction.20 The diagnosis of Chlamydia pneumoniae infection was based on an increase in IgG antibody titers by a factor of 4 in the absence of cross-reactivity with C. trachomatis on microimmunofluorescence analysis, an initial IgM antibody titer of more than 1:16, or a positive polymerase-chain-reaction assay.
Intracellular lipid from alveolar macrophages obtained from bronchial samples was evaluated for evidence of pulmonary fat embolism according to a modification of the Corwin index, a method for quantification of the amount of lipid found in pulmonary macrophages.11 Samples obtained at autopsy and histopathological slides were analyzed by the central pathology unit for evidence of sickle cell disease.
Statistical Analysis
We performed inferential statistical analyses after eliminating all but the first episode of the acute chest syndrome for each patient. For comparisons of clinical data among age groups, we compared proportions using chi-square analysis with Yates' correction or Fisher's exact test. All confidence intervals were two-sided, and a P value of 0.05 or less was considered to indicate statistical significance. For comparison of means, we used Student's t-test or analysis of variance; results are reported as means ±SD. When assumptions for parametric analyses were not met, the Wilcoxon rank-sum test was used. When summarizing data concerning causation, we included all episodes.
We developed a Cox proportional-hazards regression model for the duration of hospitalization in which we reduced age and 40 other variables from the patients' medical history and hospital course to 19 covariates that were significant on univariate analyses. These variables were included in a multivariable proportional-hazards regression model designed to identify factors that were independently associated with the time of discharge from the hospital. Data on patients were censored at the time of death, and one variable for which more than 20 percent of values were missing was not entered into the model.
Multivariable logistic-regression models were developed for the presence or absence of respiratory failure and neurologic complications in order to identify predictors of these events at the time of diagnosis. We performed univariate logistic-regression analyses of these outcomes and 35 clinical variables of interest. Only clinical variables that were significantly related in the univariate analyses to the outcome variable were included in the multivariable logistic-regression model.
Results
Diagnosis and Symptoms of the Acute Chest Syndrome at Admission
The characteristics of the patients are summarized in Table 1Table 1
Characteristics of the Patients.. Data on one patient (one episode) were excluded because the patient's birth date was not known. Nearly half the patients were admitted with a diagnosis other than the acute chest syndrome; 72 percent of these patients were admitted because of a vaso-occlusive crisis. Among patients who were not admitted for the acute chest syndrome, radiographic and clinical findings of the syndrome appeared a mean of 2.5 days after admission. The symptoms at presentation varied with age (Table 1).Hospital Course and Treatment
The findings on physical and radiographic examination are summarized in Table 2Table 2
Characteristics and Treatment of the Patients during Hospitalization.. During hospitalization, multilobar involvement, especially of the lower lobes, was common in all age groups, and effusion was present in 55 percent of all patients. All patients were treated with antibiotics and, on average, became afebrile after a mean of two days of hospitalization.At diagnosis, the mean oxygen saturation was 92 percent while the patients were breathing room air. There was no significant difference in the extent of hypoxia among the age groups, but younger patients were less likely to be treated with oxygen and mechanical ventilation. Thirteen percent of all patients required mechanical ventilation, and the mean duration of mechanical ventilation was 4.6 days. Eighty-one percent of the patients who required mechanical ventilation recovered.
The mean forced expiratory volume in one second during the acute phase of the syndrome was 53 percent of the predicted value. Sixty-one percent of patients were treated with bronchodilators, and 20 percent of these patients had clinical improvement (defined as an increase of 15 percent in forced expiratory volume in one second, expressed as a percentage of the predicted value). Among those with improvement, the mean forced expiratory volume in one second was initially 41 percent of the predicted value and increased to 52 percent, a 27 percent improvement.
Seventy-two percent of patients received transfusions (Table 2). The mean number of transfusions per patient was 1.6, and the mean number of units per patient was 3.2. Sixty-eight percent of patients received simple transfusions, and 64 percent received phenotypically matched red cells. Oxygenation significantly improved with transfusion. On average, the partial pressure of arterial oxygen while the patients were breathing room air was 63 mm Hg before transfusion and 71 mm Hg after transfusion (P<0.001). Similarly, oxygen saturation increased from 91 percent to 94 percent with transfusion (P<0.001). When we analyzed only patients who had hypoxia before transfusion (defined as an oxygen saturation of less than 91 percent), the values increased from 86 percent to 93 percent (P<0.001). Both simple and exchange transfusion resulted in similar improvements in oxygenation. Overall, 2.4 percent of patients had a new red-cell antibody, including 1 percent of the patients who received phenotypically matched units and 5 percent of those who received standard transfusions (P=0.02).
The mean hospital stay was 10.5 days. Cox proportional-hazards regression analysis of 19 covariates that were significant on univariate analysis showed that after adjustment for the remaining factors, an age of 20 years or more, a history of vaso-occlusive events, a platelet count of 0 to 199,000 per cubic millimeter at diagnosis, pain in the arms and legs at presentation, extensive radiographic abnormalities, evidence of effusion on radiographic analysis, fever, treatment by transfusion, and respiratory failure were independently associated with prolonged hospitalization (Table 3Table 3
Overall Predictors of Prolonged Hospitalization, Respiratory Failure, and Neurologic Complications among Patients with the Acute Chest Syndrome.).Complications
Older patients (particularly those who were 20 years of age or older) were more likely to have complications and to die during hospitalization (Table 2). Respiratory failure was documented in 13 percent of patients. To identify predictors of respiratory failure that were present at the time of diagnosis, we developed a multivariable logistic-regression model that included age and 10 variables that were significant on univariate analyses. Radiographic evidence of extensive lobar involvement, a platelet count of 0 to 199,000 per cubic millimeter at diagnosis, and a history of cardiac disease remained independent predictors of respiratory failure (Table 3).
Neurologic events occurred in 11 percent of patients. The most common were altered mental status (in 56 percent), seizures (11 percent), and neuromuscular abnormalities (8 percent). Anoxic brain injury occurred in three patients, central nervous system hemorrhage in three, and infarction in three. Respiratory failure developed in 46 percent of patients with neurologic complications; 92 percent of these patients underwent transfusion, and 23 percent died. The mean hospital stay for these patients was 19.5 days, as compared with 9.4 days for the remainder of the patients (P=0.008). Multivariable logistic-regression analysis of 11 variables that were significant on univariate analysis showed that only the platelet count remained an independent predictor of neurologic complications during hospitalization (Table 3). Those with relative thrombocytopenia (defined as a platelet count of less than 200,000 per cubic millimeter) were at the greatest risk for neurologic complications.
Complications occurred in 13 percent of all bronchoscopic procedures (28 of 219). Half the complications consisted of a transient decline in oxygen saturation. Laryngospasm occurred in 10 patients, and pneumothorax developed in 2 patients, both of whom had had the acute respiratory distress syndrome before the bronchoscopy. Eight patients underwent intubation because of bronchoscopic complications. As compared with the patients who underwent bronchoscopy without incident, the patients who had complications were more likely to present with dyspnea (61 percent vs. 36 percent, P=0.04) and higher peak respiratory rates (mean, 47 vs. 41 per minute; P=0.03).
Eighteen patients died. The primary causes of death were respiratory failure from pulmonary emboli (bone marrow, fat, or thrombotic) in six patients and bronchopneumonia in six. The causes of death in the remaining patients were pulmonary hemorrhage, cor pulmonale, hypovolemic shock from splenic sequestration, overwhelming sepsis, intracranial hemorrhage, and seizure. Overall, infection was a contributing factor in 10 deaths. The organisms identified included Streptococcus pneumoniae, Escherichia coli, Haemophilus influenzae, legionella, cytomegalovirus, Staphylococcus aureus, and chlamydia.
Causes of the Acute Chest Syndrome
The causes of the acute chest syndrome are given in Table 4Table 4
Causes of the Acute Chest Syndrome.. A specific cause (either pulmonary fat embolism or an infectious agent) was identified in 256 (38 percent) episodes. After the exclusion of episodes with incomplete data, a specific cause was identified in 70 percent of episodes. Pulmonary infarction was presumed to be the cause in 16 percent of episodes in which there were complete data but no cause could be identified.Bronchoscopic or sputum samples were obtained for analysis of fat embolisms in 72 percent of episodes. The results for 15 percent of the bronchoscopic specimens could not be interpreted. The majority of these specimens contained large quantities of inflammatory cells that made it impossible to identify pulmonary macrophages. A significantly larger percentage of sputum samples were uninterpretable (42 percent, P<0.001), in most cases because of contamination with epithelial cells from the oropharynx or an inflammatory exudate.
Of the 27 different pathogens identified, C. pneumoniae was the most frequent, followed by M. pneumoniae and respiratory syncytial virus (Table 5Table 5
Infectious Pathogens Isolated in 671 Episodes of the Acute Chest Syndrome.). Parvovirus was isolated from a bronchial sample in 10 episodes. Severe reticulocytopenia was present in 5 of these 10 episodes. In total, 249 pathogens were identified in 216 episodes. A single infectious pathogen was identified in 172 episodes, multiple pathogens were identified in 25 episodes, and fat embolism was diagnosed together with an infectious agent in 19 episodes.Comparison of Pulmonary Fat Embolism, Infection, and Infarction
We compared the characteristics of 53 patients with fat embolism as a cause of a first episode of the acute chest syndrome, 157 with infection as a cause, and 82 with infarction as the presumed cause. More patients in the fat-embolism group than in the infection group or the infarction group were 20 years of age or older (30 percent vs. 22 percent and 13 percent, P=0.003). The patients in the fat-embolism group also had a lower mean oxygen saturation at presentation (89 percent vs. 94 percent and 91 percent, P=0.02) and were more likely to have upper-lobe infiltrates during hospitalization (45 percent vs. 38 percent and 24 percent, P=0.04). The overall rates of complications were similar in the three groups, except for a higher incidence of vaso-occlusive events in the fat-embolism group (74 percent vs. 54 percent and 68 percent, P=0.02).
Comparison of Chlamydia and Mycoplasma Infections
We also compared the characteristics of the patients who were infected with the two most common pathogens. As compared with patients with mycoplasma infections, patients with chlamydia infections were older (mean age, 17.8 vs. 11.3 years; P=0.02), were less likely to be taking prophylactic antibiotics (43 percent vs. 77 percent, P=0.006), and were more likely to have a vaso-occlusive event during hospitalization (65 percent vs. 39 percent, P=0.04). At diagnosis, patients with chlamydia infections had higher mean hemoglobin levels than did patients with mycoplasma infections (8.0 vs. 7.0 g per deciliter, P=0.03).
In seven episodes M. hominis was the only agent identified. The patients with M. hominis infections were older than those with M. pneumoniae infections (mean age, 19.6 vs. 10.2 years) but were similar in other respects.
Discussion
Although there is an increased awareness that the acute chest syndrome is the leading cause of death in patients with sickle cell disease, the diagnosis is often delayed, the optimal treatment is unknown, and the cause is usually not determined. The longer patients with sickle cell disease live, the higher the frequency of recurrent pulmonary disease and resultant chronic lung disease.21 Despite the young age of our patients, more than two thirds had a history of the acute chest syndrome, and many had multiple episodes during the study.
The symptoms at presentation were age-dependent; wheezing, cough, and fever were most common among patients who were younger than 10 years of age, whereas pain in the arms and legs and dyspnea were more common among the adults.22 Almost half the patients in whom the acute chest syndrome developed were admitted for other reasons, including pain, and had radiographic and clinical symptoms within three days after hospitalization. Clearly, pain is a prodrome of the acute chest syndrome,22,23 and a single physical examination or radiograph may not be adequate for early diagnosis.
Laboratory values worsened after the diagnosis of the syndrome despite aggressive intervention. As in previous studies, we found that involvement of the lower lobes predominated,9,22 radiographic abnormalities progressed, and oxygenation and hemoglobin levels declined. These findings indicate that serial monitoring is necessary.
The average length of hospitalization was more than 10 days and was longer than that reported in previous studies.8,9 An older age, pain in the arms and legs at presentation, fever, a low platelet count at diagnosis, extensive radiographic abnormalities, transfusion therapy, and respiratory failure were associated with prolonged hospitalization. At the time of diagnosis of the acute chest syndrome, patients who went on to have respiratory failure were more likely to have a history of cardiac disease, to have four or more lobes affected, and to have platelet counts of 0 to 199,000 per cubic millimeter at base line. In the general population, similar findings have been found to be predictive of the severity of pneumonia, the acute respiratory distress syndrome, and the acute multiorgan-failure syndrome.24-26
There is a strong relation between the acute chest syndrome and the occurrence of neurologic complications. Neurologic complications developed in 22 percent of the adults in our study. In nearly half these patients, respiratory failure subsequently developed. A recent history of a pulmonary event is the non-neurologic risk factor that is the most highly predictive of stroke.27 The mechanism most likely involves sudden decreases in oxygenation in the vascular bed of the central nervous system.27,28 Although our statistical model included only a small number of neurologic events, the occurrence of relative thrombocytopenia has been associated with neurologic ischemic insults and reflects the presence of hypoxia, tissue damage, and platelet consumption.29,30 In addition, fat embolism and infection with chlamydia or mycoplasma may also play a direct part in brain injury.31,32
Our study evaluated interventions such as transfusions, bronchodilators, antibiotics, and mechanical ventilation. Almost three quarters of the patients received transfusions because of a worsening clinical course. Both exchange and simple transfusions improved oxygenation, providing evidence to support studies that demonstrate that limited transfusions alleviate organ dysfunction.33-35 The use of phenotypically matched units resulted in a 1 percent rate of alloimmunization, which is lower than the rate of 7 percent that is associated with standard transfusions,33 and pulmonary function improved with bronchodilator therapy. The 81 percent rate of recovery after mechanical ventilation is better than the rate among patients with the acute respiratory distress syndrome.36
The cause of the acute chest syndrome was established in 38 percent of episodes, and pulmonary infarction was the presumed cause in another 16 percent. In contrast to prior studies, in which specific causes were seldom identified,2,22 infection and emboli were common causes in our study. We examined bronchoalveolar-lavage fluid because of previous reports of the successful identification of bacterial pathogens and pulmonary fat embolism in such specimens.10-12,37,38 Bronchoscopy produced higher-quality samples than did the collection of sputum, although this procedure was associated with a risk of complications. However, in 85 percent of samples, adequate material was collected and the diagnostic yields were high.
We identified a large number of organisms that have not usually been associated with the acute chest syndrome. Chlamydia was the most common isolate and was associated with an increased rate of vaso-occlusive events, although the rate of infection with this organism was similar to that in the general population.39,40 Mycoplasma and viral pneumonia, including that caused by parvovirus, occurred in all age groups, but predominated among young children.6 We also found that M. hominis was responsible for seven episodes of the acute chest syndrome and legionella for four. These organisms have not been considered causes of the syndrome, but they should be.
As reported in previous studies,11,12 pulmonary fat embolism was common in our study and the clinical course of patients with this cause varied. Although the catalyst of the syndrome may be pulmonary fat embolism or infection, these events precipitate sickling, regional hypoxia, and further ischemic damage.41-44 An environment ideal for sickle cell–related injury is created by altering cellular adhesive molecules, damaging the vascular endothelium, and stimulating cytokines.11,23,41,43-45
In order to minimize lung injury in patients with sickle cell disease, a comprehensive plan aimed at both the catalyst and the host response is needed. Patients should receive the influenza and pneumococcal vaccines, and they should be evaluated for respiratory syncytial virus vaccine. Those admitted for painful crises should be considered to be in the prodromal phase of the acute chest syndrome; they require incentive spirometry and daily monitoring for pulmonary disease.
Once the acute chest syndrome has been diagnosed, broad-spectrum antibiotics, including a macrolide, are required. Airway hyperreactivity should be assumed to be present, even if the patient is not wheezing, and treatment with bronchodilators should be initiated. Routine, early transfusions are indicated for patients at high risk for complications, including adults and those who have a history of cardiac disease and severe pain in the arms and legs at presentation. Those who present with severe anemia, thrombocytopenia, or both and multilobar pneumonia should receive a transfusion before respiratory distress develops. In most patients with anemia, treatment with leukocyte-depleted, matched, simple transfusions is safe and effective.
Bronchoscopy is recommended in patients with no response to initial therapy because of the potential for organisms to be missed by analysis of sputum samples. Preliminary reports suggest that corticosteroids and nitric oxide are beneficial in severe cases that have not responded to other treatments.45,46 Given the high rate of success with mechanical ventilation in our study, aggressive ventilatory support, including extracorporeal membrane oxygenation, may be necessary. After recovery, patients must be evaluated for lung injury. Hydroxyurea, transfusion therapy, or bone marrow transplantation may be indicated in patients with recurrent events.
Supported by grants (HL-20985, HL-99-016, and M-01RR01271) from the National Institutes of Health.
We are indebted to Shanda Robertson and Dana Kelly for data management, to Karen Seth and Christy Andrews for assistance in the preparation of the manuscript, to Dr. Julias Schachter for performing chlamydia cultures and determining antibody titers, and to Dr. William Klitz and Dr. Dennis Black for statistical support.
Source Information
From the Departments of Hematology–Oncology (E.P.V., L.D.N., A.N.E.) and Pathology (R.W.), Children's Hospital Oakland, Oakland, Calif.; Virolab, Berkeley, Calif. (E.T.L.); the Department of Medicine, University of California School of Medicine at San Francisco, San Francisco (D.D.); the Department of Pediatric Pulmonary Medicine, Children's Hospital of Orange County, Orange, Calif. (B.N.); the Department of Hematology, University of North Carolina, Chapel Hill (E.O.); the Department of Pediatric Hematology–Oncology, Medical College of Georgia, Augusta (V.M.); the Department of Medicine, New York Methodist Hospital, Brooklyn (R.B.); the Department of Pediatric Hematology–Oncology, East Carolina University, Greenville, N.C. (C.D.); and the Department of Pathology, University of Southern Alabama Doctors' Hospital, Mobile (E.A.M.).
Address reprint requests to Dr. Vichinsky at Children's Hospital Oakland, 747–52nd St., Oakland, CA 94609, or at evichinsky@mail.cho.org.
Other members of the National Acute Chest Syndrome Study Group are listed in the Appendix.
Other authors were Miguel Abboud, M.D. (Medical University of South Carolina, Charleston); Mark Moncino, M.D. (Scottish Rite Children's Medical Center, Atlanta); Samir Ballas, M.D. (Thomas Jefferson University, Philadelphia); and Russell Ware, M.D. (Duke University Medical Center, Durham, N.C.).
Appendix
The following investigators also participated in the National Acute Chest Syndrome Study Group: C. Daeschner (East Carolina University), P. Groncy (Memorial Miller Children's Hospital), R. Iyer (University of Mississippi), T. Kinney (Duke University Medical Center), M. Koshy (University of Illinois), W. Rackoff (Indiana University Medical Center), C. Pegelow (University of Miami), H. Hume (St. Justine Hospital), J. Parke (Carolinas Medical Center), L. McMahon (Boston Medical Center), L. Benjamin and M. Bestak (Albert Einstein College of Medicine–Montefiore Hospital), F. Little and Y. Ming-Yang (University of South Alabama), P. Waldron (University of Virginia), D. Wethers and G. Ramirez (St. Luke's–Roosevelt Hospital), N. Grossman (Columbus Children's Hospital), S. Embury and W. Mentzer (San Francisco General Hospital), M. Grossi (Children's Hospital of Buffalo), S. Claster (Summit Medical Center), L. Guarini (Interfaith Medical Center), M. Koehler (Children's Hospital of Pittsburgh), J. Eckman and T. Adamkiewicz (Emory University), E. Lowenthal (University of Alabama at Birmingham), P. Swerdlow (Medical College of Virginia), and C. Johnson (University of Southern California Medical Center).
- References
References
1
Vichinsky E . Comprehensive care in sickle cell disease: its impact on morbidity and mortality. Semin Hematol 1991;28:220-226
Web of Science | Medline2
Castro O ,Brambilla DJ ,Thorington B , et al. The acute chest syndrome in sickle cell disease: incidence and risk factors: the Cooperative Study of Sickle Cell Disease. Blood 1994;84:643-649
Web of Science | Medline3
Platt OS ,Brambilla DJ ,Rosse WF , et al. Mortality in sickle cell disease: life expectancy and risk factors for early death. N Engl J Med 1994;330:1639-1644
Full Text | Web of Science | Medline4
Castro O . Systemic fat embolism and pulmonary hypertension in sickle cell disease. Hematol Oncol Clin North Am 1996;10:1289-1303
CrossRef | Web of Science | Medline5
Bellet PS ,Kalinyak KA ,Shukla R ,Gelfand MJ ,Rucknagel DL . Incentive spirometry to prevent acute pulmonary complications in sickle cell diseases. N Engl J Med 1995;333:699-703
Full Text | Web of Science | Medline6
Lowenthal EA ,Wells A ,Emanuel PD ,Player R ,Prchal JT . Sickle cell acute chest syndrome associated with parvovirus B19 infection: case series and review. Am J Hematol 1996;51:207-213
CrossRef | Web of Science | Medline7
Miller ST ,Hammerschlag MR ,Chirgwin K , et al. Role of Chlamydia pneumoniae in acute chest syndrome of sickle cell disease. J Pediatr 1991;118:30-33
CrossRef | Web of Science | Medline8
Poncz M ,Kane E ,Gill FM . Acute chest syndrome in sickle cell disease: etiology and clinical correlates. J Pediatr 1985;107:861-866
CrossRef | Web of Science | Medline9
Sprinkle RH ,Cole T ,Smith S ,Buchanan GR . Acute chest syndrome in children with sickle cell disease: a retrospective analysis of 100 hospitalized cases. Am J Pediatr Hematol Oncol 1986;8:105-110
Medline10
Kirkpatrick MB ,Haynes J Jr ,Bass JB Jr . Results of bronchoscopically obtained lower airway cultures from adult sickle cell disease patients with the acute chest syndrome. Am J Med 1991;90:206-210
Web of Science | Medline11
Vichinsky E ,Williams R ,Das M , et al. Pulmonary fat embolism: a distinct cause of severe acute chest syndrome in sickle cell anemia. Blood 1994;83:3107-3112
Web of Science | Medline12
Godeau B ,Schaeffer A ,Bachier D , et al. Bronchoalveolar lavage in adult sickle cell patients with acute chest syndrome: value for diagnostic assessment of fat embolism. Am J Respir Crit Care Med 1996;153:1691-1696
Web of Science | Medline13
Hutchinson RM ,Merrick MV ,White JM . Fat embolism in sickle cell disease. J Clin Pathol 1973;26:620-622
CrossRef | Web of Science | Medline14
Landry ML, Hsiung GD. Primary isolation of viruses. In: Specter S, Lancz G, eds. Clinical virology manual. 2nd ed. New York: Elsevier Science Publishing, 1992:43-69.
15
Waites KB, Taylor-Robinson D. Mycoplasma and Ureaplasma. In: Murray PR, ed. Manual of clinical microbiology. 7th ed. Washington, D.C.: ASM Press, 1999:782-806.
16
Lennette ET, Lennette DA. Immune adherence hemagglutination. In: Specter S, Lancz G, eds. Clinical virology manual. 2nd ed. New York: Elsevier Science Publishing, 1992:251-61.
17
Kajigaya S ,Shimada T ,Fujita S ,Young NS . A genetically engineered cell line that produces empty capsids of B19 (human) parvovirus. Proc Natl Acad Sci U S A 1989;86:7601-7605
CrossRef | Web of Science | Medline18
Lennette ET. Epstein-Barr virus. In: Murray PR, ed. Manual of clinical microbiology. 7th ed. Washington, D.C.: ASM Press, 1999:912-8.
19
Schachter J. Chlamydiae. In: Balows A, Hausler WJ Jr, Hermann KL, Isenberg HD, Shadomy HJ, eds. Manual of clinical microbiology. 5th ed. Washington, D.C.: ASM Press, 1991:1045-53.
20
Lietman T ,Brooks D ,Moncada J ,Schachter J ,Dawson C ,Dean D . Chronic follicular conjunctivitis associated with Chlamydia psittaci or Chlamydia pneumoniae. Clin Infect Dis 1998;26:1335-1340
CrossRef | Web of Science | Medline21
Powars D ,Weidman JA ,Odom-Maryon T ,Niland JC ,Johnson C . Sickle cell chronic lung disease: prior morbidity and the risk of pulmonary failure. Medicine (Baltimore) 1988;67:66-76
Web of Science | Medline22
Vichinsky EP ,Styles LA ,Colangelo LH ,Wright EC ,Castro O ,Nickerson B . Acute chest syndrome in sickle cell disease: clinical presentation and course: Cooperative Study of Sickle Cell Disease. Blood 1997;89:1787-1792
Web of Science | Medline23
Styles LA ,Schalkwijk CG ,Aarsman AJ ,Vichinsky EP ,Lubin BH ,Kuypers FA . Phospholipase A2 levels in acute chest syndrome of sickle cell disease. Blood 1996;87:2573-2578
Web of Science | Medline24
Bone RC ,Balk R ,Slotman G , et al. Adult respiratory distress syndrome: sequence and importance of development of multiple organ failure. Chest 1992;101:320-326
CrossRef | Web of Science | Medline25
Jacobs ER ,Bone RC . Clinical indicators in sepsis and septic adult respiratory distress syndrome. Med Clin North Am 1986;70:921-932
Web of Science | Medline26
Cook DJ ,Walter SD ,Cook RJ , et al. Incidence of and risk factors for ventilator-associated pneumonia in critically ill patients. Ann Intern Med 1998;129:433-440
Web of Science | Medline27
Ohene-Frempong K ,Weiner SJ ,Sleeper LA , et al. Cerebrovascular accidents in sickle cell disease: rates and risk factors. Blood 1998;91:288-294
Web of Science | Medline28
Prohovnik I ,Pavlakis SG ,Piomelli S , et al. Cerebral hyperemia, stroke, and transfusion in sickle cell disease. Neurology 1989;39:344-348
Web of Science | Medline29
Tohgi H ,Suzuki H ,Tamura K ,Kimura B . Platelet volume, aggregation, and adenosine triphosphate release in cerebral thrombosis. Stroke 1991;22:17-21
CrossRef | Web of Science | Medline30
Kohelet D ,Perman M ,Hanna G ,Ballin A . Reduced platelet counts in neonatal respiratory distress syndrome. Biol Neonate 1990;57:334-342
CrossRef | Medline31
Cook PJ ,Honeybourne D ,Lip GY ,Beevers DG ,Wise R ,Davies P . Chlamydia pneumoniae antibody titers are significantly associated with acute stroke and transient cerebral ischemia: the West Birmingham Stroke Project. Stroke 1998;29:404-410
CrossRef | Web of Science | Medline32
Narita M ,Matsuzono Y ,Togashi T ,Kajii N . DNA diagnosis of central nervous system infection by Mycoplasma pneumoniae. Pediatrics 1992;90:250-253
Web of Science | Medline33
Vichinsky EP ,Haberkern CM ,Neumayr L , et al. A comparison of conservative and aggressive transfusion regimens in the perioperative management of sickle cell disease: the Preoperative Transfusion in Sickle Cell Disease Study Group. N Engl J Med 1995;333:206-213
Full Text | Web of Science | Medline34
Mallouh AA ,Asha M . Beneficial effect of blood transfusion in children with sickle cell chest syndrome. Am J Dis Child 1988;142:178-182
Web of Science | Medline35
Emre U ,Miller ST ,Gutierez M ,Steiner P ,Rao SP ,Rao M . Effect of transfusion in acute chest syndrome of sickle cell disease. J Pediatr 1995;127:901-904
CrossRef | Web of Science | Medline36
Wyncoll DL ,Evans TW . Acute respiratory distress syndrome. Lancet 1999;354:497-501
CrossRef | Web of Science | Medline37
Chastre J ,Fagon JY ,Soler P , et al. Bronchoalveolar lavage for rapid diagnosis of the fat embolism syndrome in trauma patients. Ann Intern Med 1990;130:583-58838
Frankel LR ,Smith DW ,Lewiston NJ . Bronchoalveolar lavage for diagnosis of pneumonia in the immunocompromised child. Pediatrics 1988;81:785-788
Web of Science | Medline39
Bates JH ,Campbell GD ,Barron AL , et al. Microbial etiology of acute pneumonia in hospitalized patients. Chest 1992;101:1005-1012
CrossRef | Web of Science | Medline40
Chirgwin K ,Roblin PM ,Gelling M ,Hammerschlag MR ,Schachter J . Infection with Chlamydia pneumoniae in Brooklyn. J Infect Dis 1991;163:757-761
CrossRef | Web of Science | Medline41
Smolinski PA ,Offermann MK ,Eckman JR ,Wick TM . Double-stranded RNA induces sickle erythrocyte adherence to endothelium: a potential role for viral infection in vaso-occlusive pain episodes in sickle cell anemia. Blood 1995;85:2945-2950
Web of Science | Medline42
Aldrich TK ,Dhuper SK ,Patwa NS , et al. Pulmonary entrapment of sickle cells: the role of regional alveolar hypoxia. J Appl Physiol 1996;80:531-539
Web of Science | Medline43
Hebbel RP ,Visser MR ,Goodman JL ,Jacob HS ,Vercellotti GM . Potentiated adherence of sickle erythrocytes to endothelium infected by virus. J Clin Invest 1987;80:1503-1506
CrossRef | Web of Science | Medline44
Ibe BO ,Morris J ,Kurantsin-Mills J ,Raj JU . Sickle erythrocytes induce prostacyclin and thromboxane synthesis by isolated perfused rat lungs. Am J Physiol 1997;272:L597-L602
Web of Science | Medline45
Gladwin MT ,Schechter AN ,Shelhamer JH ,Ognibene FP . The acute chest syndrome in sickle cell disease: possible role of nitric oxide in its pathophysiology and treatment. Am J Respir Crit Care Med 1999;159:1368-1376
Web of Science | Medline46
Bernini JC ,Rogers ZR ,Sandler ES ,Reisch JS ,Quinn CT ,Buchanan GR . Beneficial effect of intravenous dexamethasone in children with mild to moderately severe acute chest syndrome complicating sickle cell disease. Blood 1998;92:3082-3089
Web of Science | Medline
- Citing Articles (189)
Citing Articles
1
Scott Roberts. 2012. Sickle Cell Anemia. , 93-97.
CrossRef2
Janet I. Malowany, Jagdish Butany. (2012) Pathology of sickle cell disease. Seminars in Diagnostic Pathology 29:1, 49-55
CrossRef3
Deepika S. Darbari, Onyinye Onyekwere, Mehdi Nouraie, Caterina P. Minniti, Lori Luchtman-Jones, Sohail Rana, Craig Sable, Gregory Ensing, Niti Dham, Andrew Campbell, Manuel Arteta, Mark T. Gladwin, Oswaldo Castro, James G. Taylor, Gregory J. Kato, Victor Gordeuk. (2012) Markers of Severe Vaso-Occlusive Painful Episode Frequency in Children and Adolescents with Sickle Cell Anemia. The Journal of Pediatrics 160:2, 286-290
CrossRef4
Patrick J.H. Kim, Michael S. Pollanen. (2012) Osmium impregnation detection of pulmonary intravascular fat in sudden death: A study of 65 cases. Journal of Forensic and Legal Medicine
CrossRef5
Jean L. Raphael, Brigitta U. Mueller, Marc A. Kowalkowski, Suzette O. Oyeku. (2012) Shorter hospitalization trends among children with sickle cell disease. Pediatric Blood & Cancern/a-n/a
CrossRef6
Sofia Mouttalib, Henry E. Rice, Denise Snyder, Justin S. Levens, Audra Reiter, Pauline Soler, Jennifer A. Rothman, Courtney D. Thornburg. (2012) Evaluation of partial and total splenectomy in children with sickle cell disease using an internet-based registry. Pediatric Blood & Cancern/a-n/a
CrossRef7
Lori C. Jordan, James F. Casella, Michael R. DeBaun. (2012) Prospects for primary stroke prevention in children with sickle cell anaemia. British Journal of Haematologyno-no
CrossRef8
David C. Rees, John S. Gibson. (2011) Biomarkers in sickle cell disease. British Journal of Haematologyno-no
CrossRef9
Britta Panda, Jeffrey Ecker. 2011. Sickle cell disease. , 108-112.
CrossRef10
Ruby A. Khoury, Khaled M. Musallam, Salman Mroueh, Miguel R. Abboud. (2011) Pulmonary Complications of Sickle Cell Disease. Hemoglobin 35:5-6, 625-635
CrossRef11
Samir K. Ballas. (2011) Defining the Phenotypes of Sickle Cell Disease. Hemoglobin 35:5-6, 511-519
CrossRef12
Jerônimo Gonçalves Araújo, Carlos André Araújo-Melo, Osvaldo Alves de Menezes-Neto, Diego Fernandes Chagas da Silveira, Jailson Barros Correia, Rosana Cipolotti. (2011) Risk Factors for Acute Chest Syndrome in Patients From Low Socioeconomic Background. Journal of Pediatric Hematology/Oncology 33:7, 484-486
CrossRef13
Samuel O Anim, Robert C Strunk, Michael R DeBaun. (2011) Asthma morbidity and treatment in children with sickle cell disease. Expert Review of Respiratory Medicine 5:5, 635-645
CrossRef14
G. Quéré, A. Tempescul, F. Couturaud, N. Paleiron, C. Leroyer, L. De Saint-Martin. (2011) Le syndrome thoracique aigu de l’adulte drépanocytaire. Revue de Pneumologie Clinique
CrossRef15
Robyn T. Cohen, Anusha Madadi, Morey A. Blinder, Michael R. DeBaun, Robert C. Strunk, Joshua J. Field. (2011) Recurrent, severe wheezing is associated with morbidity and mortality in adults with sickle cell disease. American Journal of Hematology 86:9, 756-761
CrossRef16
Rabindra N. Paul, Oswaldo L. Castro, Anita Aggarwal, Patricia A. Oneal. (2011) Acute chest syndrome: sickle cell disease. European Journal of Haematology 87:3, 191-207
CrossRef17
Carlo Brugnara, Lucia De Franceschi. 2011. Transfusion Therapy in β Thalassemia and Sickle Cell Disease. , 179-191.
CrossRef18
Paul T. Telfer. (2011) Management of sickle cell disease: acute episodes in the community and in hospital. Paediatrics and Child Health 21:8, 363-368
CrossRef19
Fahd A. Ahmad, Charles G. Macias, Joseph Y. Allen. (2011) The Use of Incentive Spirometry in Pediatric Patients With Sickle Cell Disease to Reduce the Incidence of Acute Chest Syndrome. Journal of Pediatric Hematology/Oncology 33:6, 415-420
CrossRef20
Elana Y. Poulter, Piotr Truszkowski, Alexis A. Thompson, Robert I. Liem. (2011) Acute chest syndrome is associated with history of asthma in hemoglobin SC disease. Pediatric Blood & Cancer 57:2, 289-293
CrossRef21
Ward Hagar, Claudia R. Morris. 2011. Adults with Sickle-Cell Disease: Implications of Increasing Longevity. , 115-140.
CrossRef22
Jean L. Raphael, Patricia L. Kavanagh, C. Jason Wang, Brigitta U. Mueller, Barry Zuckerman. (2011) Translating scientific advances to improved outcomes for children with sickle cell disease: a timely opportunity. Pediatric Blood & Cancer 56:7, 1005-1008
CrossRef23
Ping An, Emily A. Barron-Casella, Robert C. Strunk, Robert G. Hamilton, James F. Casella, Michael R. DeBaun. (2011) Elevation of IgE in children with sickle cell disease is associated with doctor diagnosis of asthma and increased morbidity. Journal of Allergy and Clinical Immunology 127:6, 1440-1446
CrossRef24
S. T. Miller. (2011) How I treat acute chest syndrome in children with sickle cell disease. Blood 117:20, 5297-5305
CrossRef25
Emma Drasar, Norris Igbineweka, Nisha Vasavda, Matthew Free, Moji Awogbade, Marlene Allman, Aleksandar Mijovic, Swee Lay Thein. (2011) Blood transfusion usage among adults with sickle cell disease - a single institution experience over ten years. British Journal of Haematology 152:6, 766-770
CrossRef26
N.R. Shilo, L.C. Lands. (2011) Asthma and Chronic Sickle Cell Lung Disease: A Dynamic Relationship. Paediatric Respiratory Reviews 12:1, 78-82
CrossRef27
Chairs Elliott P. Vichinsky, Kwaku Ohene-Frempong, Regional Chairs Swee Lay Thein, Clarisse Lopes de Castro Lobo, Adlette Inati, Alexis A. Thompson, Other Faculty Kim Smith-Whitley, Janet L. Kwiatkowski, Paul S. Swerdlow, John B. Porter, Peter W. Marks. (2011) Transfusion and Chelation Practices in Sickle Cell Disease: A Regional Perspective. Pediatric Hematology-Oncology 28:2, 124-133
CrossRef28
Mary M. Reagan, Michael R. DeBaun, Melissa J. Frei-Jones. (2011) Multi-modal intervention for the inpatient management of sickle cell pain significantly decreases the rate of acute chest syndrome. Pediatric Blood & Cancer 56:2, 262-266
CrossRef29
Kenneth I. Ataga, Julia E. Brittain, Susan K. Jones, Ryan May, John Delaney, Dell Strayhorn, Payal Desai, Rupa Redding-Lallinger, Nigel S. Key, Eugene P. Orringer. (2011) Association of soluble fms-like tyrosine kinase-1 with pulmonary hypertension and haemolysis in sickle cell disease. British Journal of Haematology 152:4, 485-491
CrossRef30
Jean L. Raphael, Minghua Mei, Brigitta U. Mueller, Thomas Giordano. (2011) High resource hospitalizations among children with vaso-occlusive crises in sickle cell disease. Pediatric Blood & Cancern/a-n/a
CrossRef31
Kathryn Blake, John Lima. (2011) Asthma in Sickle Cell Disease: Implications for Treatment. Anemia 2011, 1-15
CrossRef32
Julie A. Panepinto. 2011. Fever or acute illness in a child with sickle cell disease. , 608-611.
CrossRef33
Brad Talley. 2011. Sickle cell disease. , 290-295.
CrossRef34
James E. Jacobs, Keith Quirolo, Elliott Vichinsky. (2011) Novel influenza a (H1N1) viral infection in pediatric patients with sickle-cell disease. Pediatric Blood & Cancer 56:1, 95-98
CrossRef35
Finn Hawkins, Noelle Ebel, George P. Sorescu, Lillian McMahon, Philippa Sprinz, Elizabeth S. Klings. (2011) Keeping it in the family: Three relatives with HbSC disease and simultaneous acute pulmonary emboli. American Journal of Hematologyn/a-n/a
CrossRef36
J. Bradley Ball, Samina Y. Khan, Nathan J.D. McLaughlin, Marguerite R. Kelher, Rachelle Nuss, Laura Cole, Xiayuan Liang, Christopher C. Silliman. (2011) A two-event in vitro model of acute chest syndrome: The role of secretory phospholipase A2 and neutrophils. Pediatric Blood & Cancern/a-n/a
CrossRef37
M.A. Bender, Katie R. Nielsen. 2011. Hemoglobinopathies. , 1191-1206.
CrossRef38
Stephen John Cico, Carolyn A. Paris, George A. Woodward. (2010) Miscellaneous Causes of Pediatric Chest Pain. Pediatric Clinics of North America 57:6, 1397-1406
CrossRef39
Samir K. Ballas, Robert L. Bauserman, William F. McCarthy, Oswaldo L. Castro, Wally R. Smith, Myron A. Waclawiw. (2010) Hydroxyurea and Acute Painful Crises in Sickle Cell Anemia: Effects on Hospital Length of Stay and Opioid Utilization During Hospitalization, Outpatient Acute Care Contacts, and at Home. Journal of Pain and Symptom Management 40:6, 870-882
CrossRef40
Jennifer M. Knight-Madden, Terrence S. Forrester, Norma A. Lewis, Anne Greenough. (2010) The Impact of Recurrent Acute Chest Syndrome on the Lung Function of Young Adults with Sickle Cell Disease. Lung 188:6, 499-504
CrossRef41
David C Rees, Thomas N Williams, Mark T Gladwin. (2010) Sickle-cell disease. The Lancet 376:9757, 2018-2031
CrossRef42
J. J. Strouse, M. E. Reller, D. G. Bundy, M. Amoako, M. Cancio, R. N. Han, A. Valsamakis, J. F. Casella. (2010) Severe pandemic H1N1 and seasonal influenza in children and young adults with sickle cell disease. Blood 116:18, 3431-3434
CrossRef43
Ashutosh Lal, Elliott P Vichinsky. 2010. Sickle Cell Disease. , 109-125.
CrossRef44
Xandra W. van den Tweel, Johanna H. van der Lee, Harriët Heijboer, Marjolein Peters, Karin Fijnvandraat. (2010) Development and validation of a pediatric severity index for sickle cell patients. American Journal of Hematology 85:10, 746-751
CrossRef45
Michelle Y. Owens, James N. Martin. 2010. Sickle Cell Crisis. , 391-399.
CrossRef46
Keith Quirolo. (2010) How do I transfuse patients with sickle cell disease?. Transfusion 50:9, 1881-1886
CrossRef47
Jack L. Bartram, Swee L. Thein, Kate Gardner, Yaya Egberongbe, Pam D’Silva, Susan E. Height, Moira C. Dick, Sandra O’Driscoll, David C. Rees. (2010) Outcome of children with sickle cell disease admitted to intensive care - a single institution experience. British Journal of Haematology 150:5, 614-617
CrossRef48
Muriel Fartoukh, Yannick Lefort, Anoosha Habibi, Dora Bachir, Frédéric Galacteros, Bertrand Godeau, Bernard Maitre, Laurent Brochard. (2010) Early intermittent noninvasive ventilation for acute chest syndrome in adults with sickle cell disease: a pilot study. Intensive Care Medicine 36:8, 1355-1362
CrossRef49
R. T. Cohen, M. R. DeBaun, M. A. Blinder, R. C. Strunk, J. J. Field. (2010) Smoking is associated with an increased risk of acute chest syndrome and pain among adults with sickle cell disease. Blood 115:18, 3852-3854
CrossRef50
G. A. Laurie. (2010) Acute chest syndrome in sickle cell disease. Internal Medicine Journal 40:5, 372-376
CrossRef51
Charlotte F. J. van Tuijn, Erfan Nur, Eduard J. van Beers, Hans L. Zaaijer, Bart J. Biemond. (2010) Acute chest syndrome in sickle cell disease due to the new influenza A (H1N1) virus infection. American Journal of Hematology 85:4, 303-304
CrossRef52
Riten Kumar, Shahina Qureshi, Prita Mohanty, Sreedhar P. Rao, Scott T. Miller. (2010) A Short Course of Prednisone in the Management of Acute Chest Syndrome of Sickle Cell Disease. Journal of Pediatric Hematology/Oncology 32:3, e91-e94
CrossRef53
T.F. Mendonça, M.C.V.C. Oliveira, L.R.S. Vasconcelos, L.M.M.B. Pereira, P. Moura, M.A.C. Bezerra, M.N.N. Santos, A.S. Araújo, M.S.M. Cavalcanti. (2010) Association of variant alleles of MBL2 gene with vasoocclusive crisis in children with sickle cell anemia. Blood Cells, Molecules, and Diseases 44:4, 224-228
CrossRef54
J. E. Brittain, B. Hulkower, S. K. Jones, D. Strayhorn, L. De Castro, M. J. Telen, E. P. Orringer, A. Hinderliter, K. I. Ataga. (2010) Placenta growth factor in sickle cell disease: association with hemolysis and inflammation. Blood 115:10, 2014-2020
CrossRef55
Leslie P. Scheunemann, Kenneth I. Ataga. (2010) Delayed Hemolytic Transfusion Reaction in Sickle Cell Disease. The American Journal of the Medical Sciences 339:3, 266-269
CrossRef56
Robert I. Parker. (2010) Thrombosis in the pediatric population. Critical Care Medicine 38, S71-S75
CrossRef57
Dunia Alhashimi, Zbys Fedorowicz, Fatima Alhashimi, Saeed Dastgiri, Dunia Alhashimi. 2010. Blood transfusions for treating acute chest syndrome in people with sickle cell disease. .
CrossRef58
Radha Raghupathy, Deepa Manwani, Jane A. Little. (2010) Iron Overload in Sickle Cell Disease. Advances in Hematology 2010, 1-9
CrossRef59
M Arkuszewski, E R Melhem, Krejza J. (2010) Neuroimaging in assessment of risk of stroke in children with sickle cell disease. Advances in Medical Sciences 1:-1, 1-15
CrossRef60
Melissa Rayburg, Karen A. Kalinyak, Alexander J. Towbin, Peter B. Baker, Clinton H. Joiner. (2010) Fatal bone marrow embolism in a child with hemoglobin SE disease. American Journal of HematologyNA-NA
CrossRef61
Zbigniew M. Szczepiorkowski, Jeffrey L. Winters, Nicholas Bandarenko, Haewon C. Kim, Michael L. Linenberger, Marisa B. Marques, Ravindra Sarode, Joseph Schwartz, Robert Weinstein, Beth H. Shaz. (2010) Guidelines on the use of therapeutic apheresis in clinical practice-Evidence-based approach from the apheresis applications committee of the American Society for Apheresis. Journal of Clinical Apheresis 25:3, 83-177
CrossRef62
Catherine Booth, Baba Inusa, Stephen K. Obaro. (2010) Infection in sickle cell disease: A review. International Journal of Infectious Diseases 14:1, e2-e12
CrossRef63
M. Paulina Velasquez, Mary M. Mariscalco, Stuart L. Goldstein, Gladstone E. Airewele. (2009) Erythrocytapheresis in children with sickle cell disease and acute chest syndrome. Pediatric Blood & Cancer 53:6, 1060-1063
CrossRef64
Donna K. McClish, Wally R. Smith, Bassam A. Dahman, James L. Levenson, John D. Roberts, Lynne T. Penberthy, Imoigele P. Aisiku, Susan D. Roseff, Viktor E. Bovbjerg. (2009) Pain site frequency and location in sickle cell disease: The PiSCES project. PAIN 145:1-2, 246-251
CrossRef65
Charis Kepron, Gino R. Somers, Michael S. Pollanen. (2009) Sickle Cell Trait Mimicking Multiple Inflicted Injuries in a 5-Year-Old Boy. Journal of Forensic Sciences 54:5, 1141-1145
CrossRef66
Mariane de Montalembert. (2009) Current strategies for the management of children with sickle cell disease. Expert Review of Hematology 2:4, 455-463
CrossRef67
Reem Ameen, Salem Al Shemmari, Abdulaziz Al-Bashir. (2009) Red blood cell alloimmunization among sickle cell Kuwaiti Arab patients who received red blood cell transfusion. Transfusion 49:8, 1649-1654
CrossRef68
Robert E. Richard. (2009) The management of sickle cell pain. Current Pain and Headache Reports 13:4, 295-297
CrossRef69
Paul G. Firth. (2009) Anesthesia and Hemoglobinopathies. Anesthesiology Clinics 27:2, 321-336
CrossRef70
Michael B. Stone. (2009) Acute chest syndrome diagnosed by lung sonography. The American Journal of Emergency Medicine 27:4, 516.e5-516.e6
CrossRef71
(2009) Transfusion in sickle cell anemia revisited. Transfusion 49:5, 821-823
CrossRef72
Ardie Pack-Mabien, Johnson Haynes. (2009) A primary care provider’s guide to preventive and acute care management of adults and children with sickle cell disease. Journal of the American Academy of Nurse Practitioners 21:5, 250-257
CrossRef73
Jeff M. Turner, Jason B. Kaplan, Hillel W. Cohen, Henny H. Billett. (2009) Exchange versus simple transfusion for acute chest syndrome in sickle cell anemia adults. Transfusion 49:5, 863-868
CrossRef74
Dunia Alhashimi, Fatima Alhashimi, Saeed Dastgiri, Zbys Fedorowicz, Mona Nasser, Dunia Alhashimi. 2009. Blood transfusions for treating acute chest syndrome in people with sickle cell disease. .
CrossRef75
Claudia R. Morris. (2009) Asthma management: Reinventing the wheel in sickle cell disease. American Journal of Hematology 84:4, 234-241
CrossRef76
Pasquale Niscola, Francesco Sorrentino, Laura Scaramucci, Paolo de Fabritiis, Paolo Cianciulli. (2009) Pain Syndromes in Sickle Cell Disease: An Update. Pain Medicine 10:3, 470-480
CrossRef77
N. Mahdi, A. M. Al-Subaie, K. Al-Ola, A. Q. Al-Irhayim, M. E. Ali, Z. Al-Irhayim, W. Y. Almawi. (2009) HLA DRB1*130101-DQB1*060101 haplotype is associated with acute chest syndrome in sickle cell anemia patients. Tissue Antigens 73:3, 245-249
CrossRef78
Shannon Wahl, Keith C Quirolo. (2009) Current issues in blood transfusion for sickle cell disease. Current Opinion in Pediatrics 21:1, 15-21
CrossRef79
Jessica Knight-Perry, Michael R DeBaun, Robert C Strunk, Joshua J Field. (2009) Leukotriene pathway in sickle cell disease: a potential target for directed therapy. Expert Review of Hematology 2:1, 57-68
CrossRef80
Brian Boulmay, Richard Lottenberg. (2009) Cocaine Abuse Complicating Acute Painful Episodes in Sickle Cell Disease. Southern Medical Journal 102:1, 87-88
CrossRef81
Raul C. Ribeiro, Carlos Rodriguez-Galindo, Guillermo Chantada. 2009. Pulmonary Manifestations of Hematologic and Oncologic Diseases. , 135-169.
CrossRef82
Amy Sobota, Dionne A. Graham, Matthew M. Heeney, Ellis J. Neufeld. (2009) Corticosteroids for acute chest syndrome in children with sickle cell disease: Variation in use and association with length of stay and readmission. American Journal of HematologyNA-NA
CrossRef83
Gladwin, Mark T., Vichinsky, Elliott, . (2008) Pulmonary Complications of Sickle Cell Disease. New England Journal of Medicine 359:21, 2254-2265
Full Text84
Nikolaos A. Maniatis, Anastasia Kotanidou, John D. Catravas, Stylianos E. Orfanos. (2008) Endothelial pathomechanisms in acute lung injury. Vascular Pharmacology 49:4-6, 119-133
CrossRef85
Robert C. Strunk, Michael Scott Brown, Jessica H. Boyd, Pamela Bates, Joshua J. Field, Michael R. DeBaun. (2008) Methacholine challenge in children with sickle cell disease: A case series. Pediatric Pulmonology 43:9, 924-929
CrossRef86
A.E. Fawibe. (2008) Managing acute chest syndrome of sickle cell disease in an African setting. Transactions of the Royal Society of Tropical Medicine and Hygiene 102:6, 526-531
CrossRef87
Rajmony Pannu, Jun Zhang, Richard Andraws, Annemarie Armani, Praful Patel, Peter Mancusi-Ungaro. (2008) Acute Myocardial Infarction in Sickle Cell Disease. Critical Pathways in Cardiology: A Journal of Evidence-Based Medicine 7:2, 133-138
CrossRef88
Justin Hering, Stefano Schena, Barney Dillard, James Doherty, Don Fishman, Steven Salzman. (2008) Acute chest syndrome following blunt liver laceration. Injury Extra 39:6, 216-218
CrossRef89
J. A. LEIFERT. (2008) Anaemia and cigarette smoking. International Journal of Laboratory Hematology 30:3, 177-184
CrossRef90
John J. Strouse, Clifford M. Takemoto, Jeffrey R. Keefer, Gregory J. Kato, James F. Casella. (2008) Corticosteroids and increased risk of readmission after acute chest syndrome in children with sickle cell disease. Pediatric Blood & Cancer 50:5, 1006-1012
CrossRef91
Nathalie Sabaa, Lucia de Franceschi, Philippe Bonnin, Yves Castier, Giorgio Malpeli, Haythem Debbabi, Ariane Galaup, Micheline Maier-Redelsperger, Sophie Vandermeersch, Aldo Scarpa, Anne Janin, Bernard Levy, Robert Girot, Yves Beuzard, Christophe Leboeuf, Annie Henri, Stéphane Germain, Jean-Claude Dussaule, Pierre-Louis Tharaux. (2008) Endothelin receptor antagonism prevents hypoxia-induced mortality and morbidity in a mouse model of sickle-cell disease. Journal of Clinical Investigation 118:5, 1924-1933
CrossRef92
Michael S. Isakoff, J. Alyssa Lillo, J. Nathan Hagstrom. (2008) A Single-Institution Experience With Treatment of Severe Acute Chest Syndrome: Lack of Rebound Pain With Dexamethasone Plus Transfusion Therapy. Journal of Pediatric Hematology/Oncology 30:4, 322-325
CrossRef93
N. Marrouche, F. Rassi, N. Kanj, A. Taher. (2008) Hydatid cyst mimicking acute chest syndrome in a sickle thalassemia patient. Annals of Hematology 87:4, 331-332
CrossRef94
Ward Hagar, Elliott Vichinsky. (2008) Advances in clinical research in sickle cell disease. British Journal of Haematology 0:0, 080313115903451-???
CrossRef95
Amani Al Hajeri, Graham R Serjeant, Zbys Fedorowicz, Amani Al Hajeri. 2008. Inhaled nitric oxide for acute chest syndrome in people with sickle cell disease. .
CrossRef96
C. T. Quinn, N. J. Lee, E. P. Shull, N. Ahmad, Z. R. Rogers, G. R. Buchanan. (2008) Prediction of adverse outcomes in children with sickle cell anemia: a study of the Dallas Newborn Cohort. Blood 111:2, 544-548
CrossRef97
Laura S. Inselman. 2008. Pulmonary Manifestations of Systemic Disorders. , 1053-1079.
CrossRef98
Tae W Kim. (2008) Perioperative Care of the Patient With Sickle Cell Disease. ASA Refresher Courses in Anesthesiology 36:1, 61-74
CrossRef99
Zakari Y. Aliyu, Gregory J. Kato, James Taylor, Aliyu Babadoko, Aisha I. Mamman, Victor R. Gordeuk, Mark T. Gladwin. (2008) Sickle cell disease and pulmonary hypertension in Africa: A global perspective and review of epidemiology, pathophysiology, and management. American Journal of Hematology 83:1, 63-70
CrossRef100
Eduard J. van Beers, Charlotte F.J. van Tuijn, Pythia T. Nieuwkerk, Philip W. Friederich, Jan H. Vranken, Bart J. Biemond. (2007) Patient-controlled analgesia versus continuous infusion of morphine during vaso-occlusive crisis in sickle cell disease, a randomized controlled trial. American Journal of Hematology 82:11, 955-960
CrossRef101
R. Ward Hagar, Jennifer G. Michlitsch, Jennifer Gardner, Elliott P. Vichinsky, Claudia R. Morris. (2007) Clinical differences between children and adults with pulmonary hypertension and sickle cell disease. British Journal of Haematology 0:0, 071003043944004-???
CrossRef102
Nicola Conran, Camila B. Almeida, Carolina Lanaro, Renata Proença Ferreira, Fabiola Traina, Sara T. O. Saad, Fernando F. Costa. (2007) Inhibition of caspase-dependent spontaneous apoptosis via a cAMP-protein kinase A dependent pathway in neutrophils from sickle cell disease patients. British Journal of Haematology 139:1, 148-158
CrossRef103
Harvey G Klein, Donat R Spahn, Jeffrey L Carson. (2007) Red blood cell transfusion in clinical practice. The Lancet 370:9585, 415-426
CrossRef104
Jason K. Graham, Marina Mosunjac, Randy L. Hanzlick, Mario Mosunjac. (2007) Sickle Cell Lung Disease and Sudden Death. The American Journal of Forensic Medicine and Pathology 28:2, 168-172
CrossRef105
G. Madani, A.M. Papadopoulou, B. Holloway, A. Robins, J. Davis, D. Murray. (2007) The radiological manifestations of sickle cell disease. Clinical Radiology 62:6, 528-538
CrossRef106
Zbigniew M. Szczepiorkowski, Nicholas Bandarenko, Haewon C. Kim, Michael L. Linenberger, Marisa B. Marques, Ravindra Sarode, Joseph Schwartz, Beth H. Shaz, Robert Weinstein, Ashka Wirk, Jeffrey L. Winters. (2007) Guidelines on the use of therapeutic apheresis in clinical practice—Evidence-based approach from the apheresis applications committee of the American society for apheresis. Journal of Clinical Apheresis 22:3, 106-175
CrossRef107
Fenella J Kirkham. (2007) Therapy Insight: stroke risk and its management in patients with sickle cell disease. Nature Clinical Practice Neurology 3:5, 264-278
CrossRef108
Arturo J Martí-Carvajal, Lucieni O Conterno, Jennifer M Knight-Madden, Arturo J Martí-Carvajal. 2007. Antibiotics for treating acute chest syndrome in people with sickle cell disease. .
CrossRef109
Eileen M. Finnegan, Aslihan Turhan, David E. Golan, Gilda A. Barabino. (2007) Adherent leukocytes capture sickle erythrocytes in an in vitro flow model of vaso-occlusion. American Journal of Hematology 82:4, 266-275
CrossRef110
Tami D. Benton, Judith A. Ifeagwu, Kim Smith-Whitley. (2007) Anxiety and depression in children and adolescents with sickle cell disease. Current Psychiatry Reports 9:2, 114-121
CrossRef111
Laurie Duckworth, Lewis Hsu, Hua Feng, Jianwei Wang, James E. Sylvester, Niranjan Kissoon, Eric Sandler, John J. Lima. (2007) Physician-diagnosed asthma and acute chest syndrome: Associations with NOS Polymorphisms. Pediatric Pulmonology 42:4, 332-338
CrossRef112
Winfred C. Wang. (2007) Central Nervous System Complications of Sickle Cell Disease in Children: An Overview. Child Neuropsychology 13:2, 103-119
CrossRef113
Lakshmanan Krishnamurti. (2007) Hematopoietic cell transplantation for sickle cell disease: state of the art. Expert Opinion on Biological Therapy 7:2, 161-172
CrossRef114
Pablo Bartolucci, Minh-Triet Ngo, Yves Beuzard, Fr??d??ric Galact??ros, Guitanouch Saber, Dominique Rideau, Saadia Eddahibi, Bernard Maitre, Serge Adnot, Christophe Delclaux. (2007) Decrease in lung nitric oxide production after peritonitis in mice with sickle cell disease*. Critical Care Medicine 35:2, 502-509
CrossRef115
Arturo J Martí-Carvajal, Ivan Solà, Arturo J Martí-Carvajal. 2007. Treatment for anemia in people with AIDS. .
CrossRef116
M. R. DeBaun, J. J. Field. (2007) Limitations of Clinical Trials in Sickle Cell Disease: A Case Study of the Multi-center Study of Hydroxyurea (MSH) Trial and the Stroke Prevention (STOP) Trial. Hematology 2007:1, 482-488
CrossRef117
Lily P H Yang, Susan J Keam, Gillian M Keating. (2007) Deferasirox. Drugs 67:15, 2211-2230
CrossRef118
Lori A Styles, Miguel Abboud, Sandra Larkin, Margaret Lo, Frans A. Kuypers. (2007) Transfusion prevents acute chest syndrome predicted by elevated secretory phospholipase A2. British Journal of Haematology 136:2, 343-344
CrossRef119
Priscilla Dike. 2007. Emergency Care of Children with Sickle Cell Disease: One Family's Experience. , 162-170.
CrossRef120
Dany Elsayegh, Janet M. Shapiro. (2007) Sickle Cell Vasoocclusive Crisis and Acute Chest Syndrome at Term Pregnancy. Southern Medical Journal 100:1, 77-79
CrossRef121
Kenneth Ataga, Richard Lottenberg. 2007. Hemostatic Aspects of Sickle Cell Disease. , 723-735.
CrossRef122
Steven J. Ambrusko, Sriya Gunawardena, Allison Sakara, Beth Windsor, Lizabeth Lanford, Peter Michelson, Lakshmanan Krishnamurti. (2006) Elevation of tricuspid regurgitant jet velocity, a marker for pulmonary hypertension in children with sickle cell disease. Pediatric Blood & Cancer 47:7, 907-913
CrossRef123
R. Dickerhoff. (2006) Sichelzellkrankheit in Deutschland. Monatsschrift Kinderheilkunde 154:11, 1081-1089
CrossRef124
Casey W. Melton, Johnson Haynes. (2006) Sickle Acute Lung Injury: Role of Prevention and Early Aggressive Intervention Strategies on Outcome. Clinics in Chest Medicine 27:3, 487-502
CrossRef125
Hilali HASSAN, Inam MUGHAL, Javeed DAR, Taj E. AL MEKKI, Zivani CHAPUNDUKA, Ahmad HADDAD, Imad S. A. HASSAN. (2006) Significant bronchospasm during sickle cell painful crises is associated with a lower peripheral eosinophil count. Respirology 11:5, 633-637
CrossRef126
Arturo J Martí-Carvajal, Lucieni O Conterno, Arturo J Martí-Carvajal. 2006. Antibiotics for treating community acquired pneumonia in people with sickle cell disease. .
CrossRef127
Muharrem Inan, Gilbert Chan, Kirk Dabney, Freeman Miller. (2006) Heterotopic Ossification Following Hip Osteotomies in Cerebral Palsy. Journal of Pediatric Orthopaedics 26:4, 551-556
CrossRef128
Fenella J. Kirkham, Avijit K. Datta. (2006) Hypoxic adaptation during development: relation to pattern of neurological presentation and cognitive disability. Developmental Science 9:4, 411-427
CrossRef129
Alice Ackerman. (2006) Noninvasive ventilation in the pediatric intensive care unit: Is the time now?*. Pediatric Critical Care Medicine 7:4, 391-393
CrossRef130
Jonathan A. Staser, Tariq Alam, Kimberly Applegate. (2006) Calcified pulmonary thromboembolism in a child with sickle cell disease: value of multidetector CT in patients with acute chest syndrome. Pediatric Radiology 36:6, 561-563
CrossRef131
Nathalie Dauphin-McKenzie, Jerry M. Gilles, Elvire Jacques, Thomas Harrington. (2006) Sickle Cell Anemia in the Female Patient. Obstetrical & Gynecological Survey 61:5, 343-352
CrossRef132
Robert Dunlop, Kyle CLB Bennett, Robert Dunlop. 2006. Pain management for sickle cell disease in children and adults. .
CrossRef133
Vanessa Tarer, Maryse Etienne-Julan, Jean-Pierre Diara, Marie Sylvaine Belloy, Martin Mukizi-Mukaza, Jacques Elion, Marc Romana. (2006) Sickle cell anemia in Guadeloupean children: pattern and prevalence of acute clinical events. European Journal of Haematology 76:3, 193-199
CrossRef134
Claudia R Morris. (2006) New Strategies for the Treatment of Pulmonary Hypertension in Sickle Cell Disease. Treatments in Respiratory Medicine 5:1, 31-45
CrossRef135
Lydia Foucan, Didier Ekouevi, Maryse Etienne-Julan, L Rachid Salmi, Jean-Pierre Diara. (2006) Early onset dactylitis associated with the occurrence of severe events in children with sickle cell anaemia. The Paediatric Cohort of Guadeloupe (1984-99). Paediatric and Perinatal Epidemiology 20:1, 59-66
CrossRef136
Aaron L. Zuckerberg, Myron Yaster. 2006. Anesthesia for Pediatric Orthopedic Surgery. , 737-769.
CrossRef137
A. Ferster, A. Kentos, C. Bradstreet, F. Vertongen, B. Gulbis, B. Gulbis. (2005) Clinique de la drépanocytose. Journal Européen des Urgences 18:4, 230-233
CrossRef138
Iris D. Buchanan, Maribel Woodward, George W. Reed. (2005) Opioid selection during sickle cell pain crisis and its impact on the development of acute chest syndrome. Pediatric Blood & Cancer 45:5, 716-724
CrossRef139
Cage S. Johnson. (2005) The Acute Chest Syndrome. Hematology/Oncology Clinics of North America 19:5, 857-879
CrossRef140
Charles T. Quinn, Naveed Ahmad. (2005) Clinical correlates of steady-state oxyhaemoglobin desaturation in children who have sickle cell disease. British Journal of Haematology 131:1, 129-134
CrossRef141
Sam O. Wanko, Marilyn J. Telen. (2005) Transfusion Management in Sickle Cell Disease. Hematology/Oncology Clinics of North America 19:5, 803-826
CrossRef142
Cabot, Richard C.Harris, Nancy Lee, Shepard, Jo-Anne O., Ebeling, Sally H.Ellender, Stacey M.Peters, Christine C., Medoff, Benjamin D., Shepard, Jo-Anne O., Smith, R. Neal, Kratz, Alexander, . (2005) Case 17-2005. New England Journal of Medicine 352:23, 2425-2434
Full Text143
Nghia C. Dang, Cage Johnson, Mahmoud Eslami-Farsani, L. Julian Haywood. (2005) Bone marrow embolism in sickle cell disease: A review. American Journal of Hematology 79:1, 61-67
CrossRef144
Jane Hankins, Michael Jeng, Sylvia Harris, Chin-Shang Li, Tiebin Liu, Winfred Wang. (2005) Chronic Transfusion Therapy for Children With Sickle Cell Disease and Recurrent Acute Chest Syndrome. Journal of Pediatric Hematology/Oncology 27:3, 158-161
CrossRef145
Duane Robina Bonds. (2005) Three decades of innovation in the management of sickle cell disease: the road to understanding the sickle cell disease clinical phenotype. Blood Reviews 19:2, 99-110
CrossRef146
Carolyn Hoppe. (2005) Defining stroke risk in children with sickle cell anaemia. British Journal of Haematology 128:6, 751-766
CrossRef147
Charles T. Quinn, Scott T. Miller. (2004) Risk factors and prediction of outcomes in children and adolescents who have sickle cell anemia. Hematology/Oncology Clinics of North America 18:6, 1339-1354
CrossRef148
LeRoy M. Graham. (2004) The Effect of Sickle Cell Disease on the Lung. Clinical Pulmonary Medicine 11:6, 369-378
CrossRef149
Marie J Stuart, Ronald L Nagel. (2004) Sickle-cell disease. The Lancet 364:9442, 1343-1360
CrossRef150
Fenella J. Kirkham, Michael R. deBaun. (2004) Stroke in children with sickle cell disease. Current Treatment Options in Neurology 6:5, 357-375
CrossRef151
Jessica H. Boyd, Asif Moinuddin, Robert C. Strunk, Michael R. DeBaun. (2004) Asthma and acute chest in sickle-cell disease. Pediatric Pulmonology 38:3, 229-232
CrossRef152
Paul G. Firth, C Alvin Head. (2004) Sickle Cell Disease and Anesthesia. Anesthesiology 101:3, 766-785
CrossRef153
Mark W. Crawford, Melanie Speakman, Edmund D. Carver, Peter C. W. Kim. (2004) Acute chest syndrome shows a predilection for basal lung regions on the side of upper abdominal surgery. Canadian Journal of Anesthesia/Journal canadien d'anesthésie 51:7, 707-711
CrossRef154
Robert I. Liem, Maurice R. O'Gorman, Deborah L. Brown. (2004) Effect of red cell exchange transfusion on plasma levels of inflammatory mediators in sickle cell patients with acute chest syndrome. American Journal of Hematology 76:1, 19-25
CrossRef155
Boris Zavizion, Andrei Purmal, John Chapman, Bernadette Alford. (2004) Inactivation of mycoplasma species in blood by INACTINE PEN110 process. Transfusion 44:2, 286-293
CrossRef156
LeRoy M. Graham. (2004) Sickle cell disease: Pulmonary management options. Pediatric Pulmonology 37:S26, 191-193
CrossRef157
Heather J. Zar. (2004) Etiology of sickle cell chest. Pediatric Pulmonology 37:S26, 188-190
CrossRef158
Kyung W. Park. (2004) Sickle Cell Disease and Other Hemoglobinopathies. International Anesthesiology Clinics 42:3, 77-93
CrossRef159
Lawrence Feldman, Robert Gross, Jack Garon, Anitha Nallari, Navleen Kaur, Bharat Motwani, Sunitha Sukumaran, Sandra Allen, Maxwell Westerman. (2003) Sickle cell patient with an acute chest syndrome and a negative chest X-ray: Potential role of the ventilation and perfusion (V/Q) lung scan. American Journal of Hematology 74:3, 214-215
CrossRef160
Paul G. Firth, Yoshihiko Tsuruta, Yogish Kamath, Walter H. Dzik, Christopher S. Ogilvy, Robert A. Peterfreund. (2003) Transfusion-related acute lung injury or acute chest syndrome of sickle cell disease? — A case report. Canadian Journal of Anesthesia/Journal canadien d'anesthésie 50:9, 895-899
CrossRef161
Margaret R. Hammerschlag. (2003) Pneumonia due toChlamydia pneumoniae in children: Epidemiology, diagnosis, and treatment. Pediatric Pulmonology 36:5, 384-390
CrossRef162
Jennifer M Knight-Madden, Ian R Hambleton, Jennifer M Knight-Madden. 2003. Inhaled bronchodilators for acute chest syndrome in people with sickle cell disease. .
CrossRef163
Warwick A. Marchant, Isabeau Walker. (2003) Anaesthetic management of the child with sickle cell disease. Pediatric Anesthesia 13:6, 473-489
CrossRef164
&NA;. (2003) A multipronged approach needed for successful management of sickle cell anaemia. Drugs & Therapy Perspectives 19:5, 10-13
CrossRef165
Persis J. Amrolia, Antonio Almeida, Sally C. Davies, Irene A. G. Roberts. (2003) Therapeutic challenges in childhood sickle cell disease Part 2: a problem-orientated approach. British Journal of Haematology 120:5, 737-743
CrossRef166
Reda E. Girgis, Mohammed A. Qureshi, Judith Abrams, Paul Swerdlow. (2003) Decreased exhaled nitric oxide in sickle cell disease: Relationship with chronic lung involvement. American Journal of Hematology 72:3, 177-184
CrossRef167
Thomas J. Reid. (2003) Hb-based oxygen carriers: are we there yet?. Transfusion 43:2, 280-287
CrossRef168
Deborah Dean, Lynne Neumayr, Dana M. Kelly, Samir K. Ballas, Klara Kleman, Shanda Robertson, Rathi V. Iyer, Russell E. Ware, Mabel Koshy, Wayne R. Rackoff, Chuck H. Pegelow, Peter Waldron, Lennette Benjamin, Elliott Vichinsky. (2003) Chlamydia pneumoniae and Acute Chest Syndrome in Patients With Sickle Cell Disease. Journal of Pediatric Hematology/Oncology 25:1, 46-55
CrossRef169
Sophie Lanzkron, Alison R. Moliterno, Edward J. Norris, Steven A. Gould, Jodi Segal, Eric L. Nuermberger, Paul M. Ness. (2002) Polymerized human Hb use in acute chest syndrome: a case report. Transfusion 42:11, 1422-1427
CrossRef170
Thomas P. Nifong, Ronald E. Domen. (2002) Oxygen Saturation and Hemoglobin A Content in Patients with Sickle Cell Disease Undergoing Erythrocytapheresis. Therapeutic Apheresis and Dialysis 6:5, 390-393
CrossRef171
Ki Hyeong Lee, Virgil C. McKie, Elizabeth A. Sekul, Robert J. Adams, Fenwick T. Nichols. (2002) Unusual Encephalopathy After Acute Chest Syndrome in Sickle Cell Disease: Acute Necrotizing Encephalitis. Journal of Pediatric Hematology/Oncology 24:7, 585-588
CrossRef172
Oswaldo Castro, S. Gerald Sandler, Patricia Houston-Yu, Sohail Rana. (2002) Predicting the effect of transfusing only phenotype-matched RBCs to patients with sickle cell disease: theoretical and practical implications. Transfusion 42:6, 684-690
CrossRef173
Elliott Vichinsky. (2002) Reply. Transfusion 42:5, 659-660
CrossRef174
Tammara L. Jenkins. (2002) Sickle Cell Anemia in the Pediatric Intensive Care Unit: Novel Approaches for Managing Life-threatening Complications. AACN Clinical Issues: Advanced Practice in Acute and Critical Care 13:2, 154-168
CrossRef175
Linda S Franck, Marsha Treadwell, Eufemia Jacob, Elliott Vichinsky. (2002) Assessment of Sickle Cell Pain in Children and Young Adults Using the Adolescent Pediatric Pain Tool. Journal of Pain and Symptom Management 23:2, 114-120
CrossRef176
Constance F.M. Danielson. (2002) The Role of Red Blood Cell Exchange Transfusion in the Treatment and Prevention of Complications of Sickle Cell Disease. Therapeutic Apheresis and Dialysis 6:1, 24-31
CrossRef177
Samir K. Ballas. (2002) Sickle Cell Anaemia. Drugs 62:8, 1143-1172
CrossRef178
Lauren Rome, Ganesan Murali, Michael Lippmann. (2001) NONRESOLVING PNEUMONIA AND MIMICS OF PNEUMONIA. Medical Clinics of North America 85:6, 1511-1530
CrossRef179
Samir K. Gupta, George A. Sarosi. (2001) THE ROLE OF ATYPICAL PATHOGENS IN COMMUNITY-ACQUIRED PNEUMONIA. Medical Clinics of North America 85:6, 1349-1365
CrossRef180
KCLB Bennett, R Dunlop, J Lau, LJ Benjamin, DB Carr, Kyle Bennett. 2001. Drug treatments for pain in sickle cell disease. .
CrossRef181
Marilyn J Telen. (2001) Principles and problems of transfusion in sickle cell disease. Seminars in Hematology 38:4, 315-323
CrossRef182
Samir K Ballas. (2001) Sickle cell disease: Current clinical management. Seminars in Hematology 38:4, 307-314
CrossRef183
Elliott P. Vichinsky, Naomi L.C. Luban, Elizabeth Wright, Nancy Olivieri, Catherine Driscoll, Charles H. Pegelow, Robert J. Adams, . (2001) Prospective RBC phenotype matching in a stroke-prevention trial in sickle cell anemia: a multicenter transfusion trial. Transfusion 41:9, 1086-1092
CrossRef184
T. Frietsch, I. Ewen, K. F. Waschke. (2001) Anaesthetic care for sickle cell disease. European Journal of Anaesthesiology 18:3, 137-150
CrossRef185
Marie J. Stuart, B.N. Yamaja Setty. (2001) Acute chest syndrome of sickle cell disease: new light on an old problem. Current Opinion in Hematology 8:2, 111-122
CrossRef186
Charles H. Pegelow. (2001) Stroke in Children With Sickle Cell Anaemia. Paediatric Drugs 3:6, 421-432
CrossRef187
(2000) Acute Chest Syndrome in Sickle Cell Disease. New England Journal of Medicine 343:18, 1336-1337
Full Text188
Kenneth I. Ataga, Eugene P. Orringer. (2000) Bone Marrow Necrosis in Sickle Cell Disease:. The American Journal of the Medical Sciences 320:5, 342-347
CrossRef189
Platt, Orah S., . (2000) The Acute Chest Syndrome of Sickle Cell Disease. New England Journal of Medicine 342:25, 1904-1907
Full Text
- Letters
