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Query: UMLS:C0030193 (pain)
 261,466 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

The acute chest syndrome is a generic term for pulmonary complications of sickle cell diseases with heterogeneous etiologies that include pneumonia, vaso-occlusion of pulmonary arterioles, rib infarction, and fat embolism syndrome. My review summarizes these etiologies, the evidence, and pathophysiology supporting the hypothesis that infarction of segments of ribs by the same vaso-occlusive process responsible for the acute episodes of pain (characteristic of the sickle cell diseases) is often involved in the acute chest structure. Inflammation associated with the infarct then causes splinting, hypoventilation, and hypoxia and further vaso-occlusion. The relationship with adult respiratory distress syndrome and fat embolism is also discussed. Use of the incentive spirometer combined with effective analgesia when chest pain is present is advocated for prevention of the pulmonary infiltrates. Newer understanding of the role of nitric oxide in regulating oxygen transport and its relationship to blood transfusions used in therapy of the acute chest syndrome are discussed.
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PMID:The role of rib infarcts in the acute chest syndrome of sickle cell diseases. 1267 38

High fetal hemoglobin (HbF) levels inhibit the polymerization of sickle hemoglobin (HbS) and reduce the complications of sickle cell disease. Pharmacologic agents that can reverse the switch from gamma- to beta-chain synthesis--gama-globin chains characterize HbF, and sickle beta-globin chains are present in HbS--or selectively increase the proportion of adult erythroid precursors that maintain the ability to produce HbF are therapeutically useful. Hydroxyurea promotes HbF production by perturbing the maturation of erythroid precursors. This treatment increases the total hemoglobin concentration, reduces the vaso-occlusive complications of pain and acute chest syndrome, and attenuates mortality in adults. It is a promising beginning for pharmacologic therapy of sickle cell disease. Still, its effects are inconsistent, trials in infants and children are ongoing, and its ultimate value--and peril--when started early in life are still unknown.
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PMID:Hydroxyurea treatment for sickle cell disease. 1280 65

Previous reports have shown that in more than 40% of adults with acute chest syndrome (ACS), fat droplets suggestive of pulmonary fat embolism were present in alveolar macrophages. To determine whether induced sputum (IS) is a reliable test for detecting this embolism, we compared bronchoalveolar lavage and IS results in 20 patients with ACS. We found a correlation between the number of Oil Red O-stained macrophages in sputum and lavage fluid (Spearman's coefficient: rho = 0.657, p < 0.018). Sputum cytology was then studied in another 60 patients who had sickle cell disease with ACS. An elevated percentage of Oil Red O-stained macrophages was found in the sputum of 37/47 patients, but they did not include any of the patients with sickle cell disease but no clinical symptoms. Patients suffering from ACS with Oil Red O-stained macrophages had more extrathoracic concomitant pain than those without (76 vs. 50%, p < 10-8), had more neurologic symptoms (7 vs. 0%, p < 10-8), a lower differential platelet count (-49 +/- 121 vs. +85 +/- 229, p < 0.04), and higher abnormal transaminase values (28 vs. 17%, p < 0.01). We conclude that IS analysis is a safe, noninvasive, and useful test for fat embolism detection in ACS.
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PMID:Induced sputum versus bronchoalveolar lavage during acute chest syndrome in sickle cell disease. 1296 66

Human parvovirus (HPV) B19 causes significant morbidity and mortality in children with sickle cell disease (SCD), but little data are published about the epidemiology of HPV B19 infection and its associated complications in this patient population. In this study, prevalence and incidence rates of HPV B19 were determined in 633 patients with SCD followed at The Children's Hospital of Philadelphia between November 1996 and December 2001. Thirty percent (30%) were HPV B19 immunoglobulin G (IgG) positive at first testing, and the 70% without evidence of past HPV B19 infection were tested annually. One hundred ten patients developed evidence of HPV B19 infection for an incidence rate of 11.3 per 100 patient years. Sixty-eight episodes of HPV B19-induced transient red cell aplasia occurred with the following clinical events: fever (89.7%), pain (61.8%), acute splenic sequestration (19.1%), and acute chest syndrome (11.8%). Pain, fever, and acute splenic sequestration were more frequent events with acute HPV B19 infections compared with acute events in uninfected patients. The results of this epidemiologic study, the largest and most comprehensive to date, justify the development of HPV B19 prevention strategies to diminish the frequent and often severe complications associated with HPV B19 infections in patients with SCD.
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PMID:Epidemiology of human parvovirus B19 in children with sickle cell disease. 1452 77

The purpose of this study was to determine whether blood counts discriminate between sickle cell pain episodes that lead to successful discharge from the emergency department (ED) and those that result in complications. This retrospective review compared the hemoglobin, reticulocyte count, and white blood cell count with differential during complicated and uncomplicated ED visits. Complicated visits were pain episodes followed by admission, by readmission within 48 hours, by acute chest syndrome, by an aplastic crisis, or by the administration of blood or antibiotics. There were 2 statistically important differences between complicated and uncomplicated pain episodes. Children successfully discharged were younger than those experiencing a complicated visit (8.9 v 11.2, P = 0.04). At a difference of 0.4 g/dL, the change in hemoglobin from baseline among children with complicated versus uncomplicated pain crises was not clinically useful. Routinely performed blood counts do not reliably identify the course of sickle cell pain crises.
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PMID:The utility of screening laboratory studies in pediatric patients with sickle cell pain episodes. 1525 63

Our objective was to determine if physician-diagnosed asthma increases the risk of acute chest syndrome (ACS) in children with sickle-cell disease (SCD) hospitalized for pain. Our study design was a retrospective case-control study of all SCD patients, aged 2-21 years, hospitalized for pain during the interval 1999-2000. Medical records of first admissions during the interval were reviewed to determine the presence of ACS during the admission. Cases were defined as patients with ACS, and controls were patients without ACS. Independently, medical records of admissions prior to the study interval were reviewed for evidence of physician-diagnosed asthma. Sixty-three cases with ACS and 76 controls without ACS were identified. No significant differences in gender, age, and hemoglobin phenotype were found. Patients with physician-diagnosed asthma were 4.0 times (95% CI, 1.7, 9.5) more likely to develop ACS during the admission than patients without asthma. Individuals with physician-diagnosed asthma had a longer hospitalization for ACS, i.e., 5.6 days vs. 2.6 days, respectively (P = 0.01). In conclusion, our preliminary data suggest that asthma in children with SCD admitted to the hospital for pain may be a risk factor for ACS and may increase the duration of hospitalization when compared to children with SCD and without asthma.
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PMID:Asthma and acute chest in sickle-cell disease. 1527 2

Sickle cell disease is an inherited disease characterised by the presence of an abnormal haemoglobin. Sickle cell disease can be complicated by acute vaso-occlusive crisis, which are the major clinical problem prompting admission to hospital and the major cause of death. It mainly manifests by osteo-articular pain and acute chest syndrome and can be complicated by multi-organ failure. The main treatment of severe acute vaso-occlusive crisis is based on transfusion.
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PMID:[Acute complications in sickle cell disease]. 1555 64

Sickle cell disease is an inherited disease characterized by the presence of an abnormal haemoglobin. It is the more prevalent genetic disease at birth in Ile-de France area. Sickle cell disease can be complicated by acute vaso-occlusive crisis, chronic visceral involvement related to ischemic process, and infectious complications. In adults, acute vaso-occlusive crisis is the major clinical problem prompting admission to hospital and the main cause of death. It mainly manifests by osteoarticular pain and acute chest syndrome and can be complicated by multiorgan failure. Acute vaso-occlusive crisis can be also manifested by priapism, ischemic or haemorrhagic stroke, or abdominal pain. The main treatment of severe acute vaso-occlusive crisis is based on transfusion.
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PMID:[Emergencies in adults with sickle cell disease]. 1558 59

BACKGROUND: Asthma and sickle cell disease are common conditions that both may result in pulmonary complications. We hypothesized that children with sickle cell disease with concomitant asthma have an increased incidence of vaso-occlusive crises that are complicated by episodes of acute chest syndrome. METHODS: A 5-year retrospective chart analysis was performed investigating 48 children ages 3-18 years with asthma and sickle cell disease and 48 children with sickle cell disease alone. Children were matched for age, gender, and type of sickle cell defect. Hospital admissions were recorded for acute chest syndrome, cerebral vascular accident, vaso-occlusive pain crises, and blood transfusions (total, exchange and chronic). Mann-Whitney test and Chi square analysis were used to assess differences between the groups. RESULTS: Children with sickle cell disease and asthma had significantly more episodes of acute chest syndrome (p = 0.03) and cerebral vascular accidents (p = 0.05) compared to children with sickle cell disease without asthma. As expected, these children received more total blood transfusions (p = 0.01) and chronic transfusions (p = 0.04). Admissions for vasoocclusive pain crises and exchange transfusions were not statistically different between cases and controls. SS disease is more severe than SC disease. CONCLUSIONS: Children with concomitant asthma and sickle cell disease have increased episodes of acute chest syndrome, cerebral vascular accidents and the need for blood transfusions. Whether aggressive asthma therapy can reduce these complications in this subset of children is unknown and requires further studies.
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PMID:Asthma is a risk factor for acute chest syndrome and cerebral vascular accidents in children with sickle cell disease. 1566 85

We analyzed the records of 153 Guadeloupean children with sickle cell anemia (SCA), for whom clinical and laboratory data were prospectively collected (mean follow-up duration 8.4 +/- 4.6 yr). Prevalence and age-specific frequencies of acute clinical events were determined and correlations between complications, hematological parameters and potential modulating factors investigated. Painful crisis and acute chest syndrome (ACS) were the two most common complications, affecting 65.4% and 58.8% of the patients, respectively. The frequency of acute anemia was 49.7% (acute splenic sequestration 24.8%; acute aplastic anemia 15.0%). Prevalences of septicemia-meningitis and osteomyelitis were 15.7% and 16.3%, respectively. A higher incidence of infections, painful crises and acute anemia was detected in patients who developed ACS. The well-documented protective effect of HbF level on the overall disease expression was observed with higher HbF level in asymptomatic than in symptomatic patients (17.5% +/- 8% vs. 9.9% +/- 6.4%, P = 0.01) with similar ages and sex ratio. It was also confirmed on ACS and, for the first time, further extended to acute anemic events and septicemia. Besides its effect on hematological parameters, alpha-thalassemia seems to have little impact on the prevalence of complications, as do beta(S)-globin haplotypes. Comparison with other series suggests that the natural history of SCA in Guadeloupe is more similar to that in Jamaica with regard to those reported in Europe and the United States, suggesting a potential impact of environmental factors on the clinical course of the disease.
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PMID:Sickle cell anemia in Guadeloupean children: pattern and prevalence of acute clinical events. 1645 94


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