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Chest Syndrome (chest + syndrome)
Kinds of Chest Syndrome Selected AbstractsHospital admissions for acute painful crisis in Trinidad and Tobago.INTERNATIONAL JOURNAL OF LABORATORY HEMATOLOGY, Issue 5 2006Are the British Committee for Standards in Haematology (BCSH) guidelines applicable? Summary We observed consecutive hospital admissions for acute painful crisis (APC) among adults with Sickle Cell Disease (SCD) over a 6-month period in Trinidad and Tobago. Episodes (111) of APC resulted in 82 admissions of 59 patients. The most common site for pain was the trunk. Patients ranged in age from 17 to 53 years (median: 25). Median length of hospital stay was 4 days. Total dose of Pethidine given per admission ranged from 100 to 1650 mg (median: 525). The mean dose of morphine was 70 mg. Six (7%) of patients were readmitted within 10 days of discharge. Twenty-five (30%) of patients had chest pain at presentation of whom 10 (12%) had consolidation on chest X-ray, defining the acute chest syndrome (ACS). There was one death caused by biliary sepsis. The study revealed seemingly low opiate usage for in-hospital treatment of APC with acceptable rates of readmission. The BCSH 2003 guidelines seemed applicable apart for the choice and route of fluid for rehydration and opiate analgesia. [source] Foetal haemoglobin in homozygous sickle cell disease: a study of patients with low HBF levels*INTERNATIONAL JOURNAL OF LABORATORY HEMATOLOGY, Issue 5 2001A. Donaldson High foetal haemoglobin (HbF) levels are believed to ameliorate the manifestations of homozygous sickle cell (SS) disease. The corollary implies that patients with low HbF levels should have more severe clinical courses. We investigated this in a retrospective study of 50 Jamaican patients with steady-state HbF levels below 1% compared with a control group (A) of 54 subjects with steady-state HbF levels between 2.5 and 3.4% (around the 25th centile for our population), and a second control group (B) of 60 patients with steady-state HbF levels between 4.6 and 5.2% (around the 50th centile). Comparisons across the groups indicated significantly fewer females in the study group (16, 50 and 57%, respectively). Examination for haematological trends across the groups showed positive linear trends for haemoglobin (Hb) (P=0.004), packed cell volume (PCV) (P=0.01), mean cell volume (MCV) (P=< 0.001), mean cell haemoglobin (MCH) (P=< 0.001) and a negative trend for haemoglobin A2 (P=0.03). Clinically, there were no differences in the incidence of painful crises, abdominal crises and the acute chest syndrome, but leg ulcers were significantly less frequent in the study group (P=0.04). Therefore low HbF levels do not appear to increase the clinical severity of SS disease and may be protective against leg ulceration. [source] Sickle Cell Trait Mimicking Multiple Inflicted Injuries in a 5-Year-Old BoyJOURNAL OF FORENSIC SCIENCES, Issue 5 2009Charis Kepron M.D. Abstract:, Sickle cell disease (SCD) and sickle cell trait (SCT) can be associated with sudden unexpected death in the pediatric population, usually due to pulmonary complications occurring within the acute chest syndrome (ACS). Musculoskeletal complications can occur and are classically limited to bone infarcts. The occurrence of bone pathology centered upon the epiphyseal growth plate in SCD/SCT is extremely rare, and multiple such injuries in a single patient have not been previously reported. Herein, we describe a case of sudden unexpected death in a 5-year-old child with undiagnosed SCT due to the ACS, with widespread epiphyseal and periosteal bone lesions mimicking multiple inflicted injuries at autopsy. This case highlights the importance of clinicopathological correlation and is the first to describe SCT pathology as a mimic of nonaccidental injury. [source] Erratum: "Corticosteroids for acute chest syndrome in children with sickle cell disease: Variation in use and association with length of stay and readmission" by Sobota et al., Am J Hematol 2010 DOI number 21565AMERICAN JOURNAL OF HEMATOLOGY, Issue 5 2010Amy Sobota No abstract is available for this article. [source] Acute chest syndrome in sickle cell disease due to the new influenza A (H1N1) virus infection,AMERICAN JOURNAL OF HEMATOLOGY, Issue 4 2010Charlotte F. J. van Tuijn No abstract is available for this article. [source] 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 HEMATOLOGY, Issue 1 2010Amy Sobota Acute chest syndrome (ACS) causes significant morbidity and mortality in sickle cell disease. The role of corticosteroids is unclear. The objectives of our study were to examine the variation between hospitals in their use of corticosteroids for ACS, describe characteristics associated with corticosteroids, and investigate the association between corticosteroids, length of stay, and readmission. We performed a retrospective examination of 5,247 hospitalizations for ACS between January 1, 2004, and June 30, 2008, at 32 hospitals in the Pediatric Health Information System database. We used multivariate regression to examine the variability in the use of corticosteroids adjusting for hospital case mix, identify factors associated with corticosteroid use, and evaluate the association of corticosteroids with length of stay and 3-day readmission rates controlling for propensity score. Corticosteroid use varied greatly by hospital (10,86% among all patients, 18,92% in patients with asthma). Treatment with corticosteroids was associated with comorbid asthma (OR 3.9, 95% CI: 3.2,4.8), inhaled steroids (OR 1.4, 95% CI: 1.1,1.7), bronchodilators (OR 3.2, 95% CI: 2.5,4.2), nitric oxide (OR 2.4, 95% CI: 1.2,5.0), oxygen (OR 2.3, 95% CI: 1.8,2.9), ICU (OR 1.7, 95% CI: 1.3,2.3), ventilation (OR 2.0, 95% CI: 1.4,2.8), APR-DRG severity level (OR 1.4, 95% CI: 1.2,1.6), and discharge year (OR 0.86, 95% CI: 0.80,0.92). Corticosteroids were associated with an increased length of stay (25%, 95% CI: 14,38%) and a higher 3-day readmission rate (OR 2.3, 95% CI: 1.6,3.4), adjusted for confounding. Hospitals vary greatly in the use of corticosteroids for ACS, even in patients with asthma. Clear evidence of the efficacy and toxicity of corticosteroid treatment in ACS may reduce variation in care. Am. J. Hematol. 2010. © 2009 Wiley-Liss, Inc. [source] Functional capacity in children and young adults with sickle cell disease undergoing evaluation for cardiopulmonary disease,AMERICAN JOURNAL OF HEMATOLOGY, Issue 10 2009Robert I. Liem Although cardiopulmonary disease is associated with decreased functional capacity among adults with sickle cell disease (SCD), its impact on functional capacity in children with SCD is unknown. We evaluated 6-min walk (6MW) distance in 77 children and young adults with SCD undergoing screening for cardiopulmonary disease. Of 30 subjects who also underwent cardiopulmonary exercise testing, we found evidence for decreased exercise capacity in a significant proportion. Exercise capacity was related to baseline degree of anemia and was significantly lower in subjects with a history of recurrent acute chest syndrome. We found that 6MW distance adjusted for weight and body surface area was shorter in subjects with restrictive lung disease but that only 6MW adjusted for weight remained significantly shorter when we controlled for baseline hemoglobin. Exercise capacity was not significantly different in subjects with and without cardiopulmonary disease. We conclude that restrictive lung disease is associated with shorter 6MW distances in children and young adults with SCD, but that variables associated with decreased exercise capacity, other than anemia, remain unclear. Our study underscores the importance of further delineating the direct pathophysiologic processes that contribute to decreased exercise capacity observed among individuals with SCD and cardiopulmonary disease. Am. J. Hematol., 2009. © 2009 Wiley-Liss, Inc. [source] Life threatening parvovirus B19 and herpes simplex virus associated acute myocardial dysfunction in a child with homozygous sickle cell diseasePEDIATRIC BLOOD & CANCER, Issue 7 2007Lakshmanan Krishnamurti MD Abstract Human parvovirus (HPV) B19, a common infection, frequently causes transient red cell aplasia in children with hemolytic anemia, such as sickle cell disease (SCD). It was considered to be a self-limited condition, easily treated with blood transfusion. However, acute splenic sequestration, acute chest syndrome, nephrotic syndrome, and stroke have been reported in SCD patients following HPV B19 infection. We report a 3-year-old child with SCD who developed fulminant myocarditis following HPV B19-related aplastic crisis. The diagnosis of myocarditis should be considered in a patient with hemolytic anemia with an infection with HPV B19 who develops signs of cardiopulmonary failure despite correction of anemia. Pediatr Blood Cancer 2007;49:1019,1021. © 2006 Wiley-Liss, Inc. [source] Early blood transfusions protect against microalbuminuria in children with sickle cell diseasePEDIATRIC BLOOD & CANCER, Issue 1 2006Ofelia Alvarez MD Abstract Background Microalbuminuria (MA) is an early indicator for glomerulopathy in sickle cell disease (SCD). Procedure We reviewed the medical records of asymptomatic patients ages 4,20 with sickle hemoglobinopathies, who were screened for MA in order to find out its prevalence and risk factors. Results Nineteen of 120 (15.8%) screened patients had MA detected by spot urine (mean albumin absolute value 6.95,±,0.56 mg/dl) and abnormal albumin to creatinine ratios (79.8,±,0.62 mg/g creatinine). Twenty four-hour urine collections confirmed 57% of MA cases by spot urine. There was no difference in hyperfiltration between positive and negative patients. From the MA-positive patients, 15 had SS (16.8% of SS group) and 4 had SC (18% of SC group). Nineteen percent of children 10 years of age or older had MA, as compared to 8% of the younger children (P,=,0.018), demonstrating that increasing age is a risk factor for MA. There was a positive correlation between MA and acute chest syndrome. Young age at start of chronic transfusions was inversely related to MA and therefore renoprotective (P,=,0.03). We did not see a protective effect in the group of patients taking hydroxyurea for a relatively short time, mean age at start of treatment 12,±,5 years; however the sample was small. Conclusions We conclude that: (1) children with sickle cell hemoglobinopathies 10 years or older should be screened for MA and (2) chronic transfusions starting at an early age may be renoprotective. © 2005 Wiley-Liss, Inc. [source] Lower airway obstruction is associated with increased morbidity in children with sickle cell disease,PEDIATRIC PULMONOLOGY, Issue 3 2009Jessica H. Boyd MD Abstract Rationale The association between pulmonary function and morbidity in children with sickle cell disease (SCD) has not been previously evaluated. Our objective was to study the relationship between abnormalities in pulmonary function and morbidity as represented by the rate of hospitalizations for pain or acute chest syndrome (ACS) in children with SCD. Methods Results of pulmonary function tests obtained for clinical indications in children ages 6,18 years were classified as lower airway obstruction (forced expiratory volume in 1 sec/forced volume capacity <95% confidence interval adjusted for age, gender, race, and height), restriction (total lung capacity <80% predicted adjusted for gender, age, race, and height), and normal lung function. Incidence rates of pain or ACS were compared between children with lower airway obstruction or restriction and children with normal lung function. Results A total of 102 children, mean age at evaluation 12.0 years with follow-up of 3.8 years, were included. Children with lower airway obstruction had twice the rate of morbidity compared to children with normal lung function (2.5 vs. 1.2 hospitalizations for pain or ACS per patient-year, P,=,0.003) (Risk ratio: 2.0; 95% CI: 1.3,3.3). Children with restriction did not have different rates of future morbidity compared to children with normal lung function (1.4 vs. 1.2 hospitalizations for pain or ACS per patient-year, P,=,0.68) (Rate ratio: 1.1; 95% CI: 0.6,2.1). Conclusions We conclude that children with SCD who have lower airway obstruction should have increased surveillance for future morbidity. Pediatr Pulmonol. 2009; 44:290,296. © 2009 Wiley-Liss, Inc. [source] Airway hyperresponsiveness and acute chest syndrome in children with sickle cell anemiaPEDIATRIC PULMONOLOGY, Issue 3 2007Karl P. Sylvester PhD Abstract To determine the occurrence and magnitude of airway hyperresponsiveness (AHR) in children with sickle cell anemia (SCA) who had or had not had acute chest syndrome (ACS) episodes. A subsidiary aim was to determine whether cold air and exercise challenge testing gave similar results in children with SCA. AHR would be greater in SCA children who had had an ACS episode compared to those who had not. Prospective observational study. Forty-two SCA children (median age of 11.5 [range 6.1,16.8] years); 12 children had been previously hospitalized for an ACS episode. AHR was assessed by the change in forced expiratory volume in 1 sec (FEV1) to a cold air challenge and in a subset of the children to an exercise challenge. A positive result to either challenge was deemed to have occurred if the FEV1 fell by at least 10% from the pre-challenge baseline. The magnitude of change in FEV1 following the cold air challenge was similar in children who had or had not had an ACS episode. Six children had a positive response to the cold air challenge (AHR); none had had an ACS hospitalization. Similar proportions of children responded to the cold air and exercise challenge and the magnitude of response to the two tests was similar. Some children, however, responded only to a cold air challenge and others only to an exercise challenge. SCA children who had had an ACS hospitalization episode compared to those who had not were not more likely to respond to a cold air challenge. Importantly, if AHR is to be correctly diagnosed, some SCA children will require to undergo both cold air and exercise challenge testing. Pediatr Pulmonol. 2007; 42:272,276. © 2007 Wiley-Liss, Inc. [source] Temporal relationship of asthma to acute chest syndrome in sickle cell diseasePEDIATRIC PULMONOLOGY, Issue 2 2007Karl P. Sylvester PhD Abstract Acute chest syndrome (ACS) is an important cause of mortality and morbidity in children with sickle cell disease (SCD). An association between asthma and ACS has been reported. Our aims were to determine whether asthma was more common in SCD children than controls and the relationship of the timing of the SCD children's first ACS episode to a diagnosis of asthma. One hundred and sixty-five SCD children median age 8.2 (range 0.3,17.3) years and 151 similar ethnic origin and aged controls were prospectively recruited into the study and a detailed history was taken from all of the children to determine if they were taking anti-asthma medication. The medical records of the SCD children were examined to assess whether they had an ACS episode, the age this episode occurred and when any diagnosis of asthma had been made. A similar proportion of the SCD children and controls were taking anti-asthma medication (7% and 9%). Thirty-three SCD children had at least one ACS episode. More of the children who had an ACS compared to those who had not were taking anti-asthma medication (P,=,0.02). The ACS children had been diagnosed as asthmatic at a median of 3.5 (range 0.5,7) years prior to their first ACS episode. In conclusion, these results suggest asthma exacerbations may predispose to ACS episodes. Pediatr Pulmonol. 2007; 42:103,106. © 2006 Wiley-Liss, Inc. [source] Asthma management: Reinventing the wheel in sickle cell disease,AMERICAN JOURNAL OF HEMATOLOGY, Issue 4 2009Claudia R. Morris Asthma is a common comorbidity in sickle cell disease (SCD) with a reported prevalence of 30,70%. The high frequency of asthma in this population cannot be attributed to genetic predisposition alone, and likely reflects in part, the contribution of overlapping mechanisms shared between these otherwise distinct disorders. There is accumulating evidence that dysregulated arginine metabolism and in particular, elevated arginase activity contributes to pulmonary complications in SCD. Derangements of arginine metabolism are also emerging as newly appreciated mechanism in both asthma and pulmonary hypertension independent of SCD. Patients with SCD may potentially be at risk for an asthma-like condition triggered or worsened by hemolysis-driven release of erythrocyte arginase and low nitric oxide bioavailability, in addition to classic familial asthma. Mechanisms that contributed to asthma are complex and multifactorial, influenced by genetic polymorphisms as well as environmental and infectious triggers. Given the association of asthma with inflammation, oxidative stress and hypoxemia, factors known to contribute to a vasculopathy in SCD, and the consequences of these factors on sickle erythrocytes, comorbid asthma would likely contribute to a vicious cycle of sickling and subsequent complications of SCD. Indeed a growing body of evidence documents what should come as no surprise: Asthma in SCD is associated with acute chest syndrome, stroke, pulmonary hypertension, and early mortality, and should therefore be aggressively managed based on established National Institutes of Health Guidelines for asthma management. Barriers to appropriate asthma management in SCD are discussed as well as strategies to overcome these obstacles in order to provide optimal care. Am. J. Hematol., 2009. © 2008 Wiley-Liss, Inc. [source] Urinary cysteinyl leukotriene E4 is associated with increased risk for pain and acute chest syndrome in adults with sickle cell disease,AMERICAN JOURNAL OF HEMATOLOGY, Issue 3 2009Joshua J. Field Leukotriene E4 (LTE4) levels are associated with rate of pain episodes in children with sickle cell disease (SCD). Because complications of SCD manifest differently in adults than children, we examined a cohort of adults with SCD to determine the relationship between baseline LTE4 and SCD-related morbidity. Baseline LTE4 levels were associated with increased rates of pain and acute chest syndrome (ACS) episodes, when those with LTE4 values in the highest tertile were compared with those in the lowest tertile (pain: risk ratio 7.1, 95% CI 1.8,27.5, P = 0.005; ACS: risk ratio 12.2, 95% CI 2.1,69.8, P = 0.005). Am. J. Hematol., 2009. © 2009 Wiley-Liss, Inc. [source] Echocardiographic abnormalities in sickle cell diseaseAMERICAN JOURNAL OF HEMATOLOGY, Issue 3 2004Shahid Ahmed Abstract Echocardiographic abnormalities in patients with sickle cell disease (SCD) were determined, and pulmonary arterial systolic pressure (PASP) was estimated. Clinical data and echocardiograms of 38 adult hospitalized patients with SCD at two tertiary care hospitals were reviewed. Fisher's exact test was performed to determine correlation between pulmonary hypertension and various clinical variables. Pulmonary hypertension was the most common abnormality identified in 22 (58%) patients. The estimated mean PASP was 37.5 ± 10.9 mmHg. Older age and prior history of acute chest syndrome were significantly correlated with an increased prevalence of pulmonary hypertension (P < 0.05). Patients with hemoglobin levels <8 g/dL had PASP 43.2 ± 0.5 compared to a mean PASP of 33.3 ± 6.0 in patients with hemoglobin ,8 g/dL (P = 0.01). Eight (21%) patients had evidence of a hyperdynamic left ventricle. Left heart abnormalities included dilated atrium in 14 (37%), dilated ventricle in 5 (13%), ventricle hypertrophy in 5 (13%), and ventricle dysfunction in 3 (9%) patients. Right heart abnormalities included dilated atrium in 9 (24%), dilated ventricle in 6 (16%), and ventricle dysfunction in 3 (9%) patients. Despite an increased incidence of abnormal flow across the valves on Doppler analysis, no patient had structurally abnormal valves. A majority of patients with SCD had evidence of pulmonary hypertension, which correlated with older age and history of acute chest syndrome. Other structural and functional echocardiographic abnormalities were less common. Am. J. Hematol. 76:195,198, 2004. © 2004 Wiley-Liss, Inc. [source] Sickle cell leg ulcers: a frequently disabling complication and a marker of severityBRITISH JOURNAL OF DERMATOLOGY, Issue 2 2008M. Halabi-Tawil Summary Background, Leg ulcers are a poorly known and underestimated complication of sickle cell disease (SCD), but in our experience they often appear as a severely disabling condition, associated with the most severe forms of the disease. Objectives, To assess the characteristics, complications, repercussion on quality of life and associations of SCD ulcers. Methods, Case series of 20 patients followed in a French referral centre for SCD and who had previous/present leg ulcers. Results, Median ulcerated area was 12 cm² and median time spent with ulcers was 29·5 months. Locoregional infections developed in 85%, ankle stiffness in 50% and mood disorders in 85%. Ninety per cent of patients needed analgesics, 20% being opioids. Median loss of time from work was 12·5 months. The Short Form 36 Health Survey showed physical and mental component summary scores of 41·5 and 40·7, respectively, indicating severe alteration close to that found in lung cancer or haemodialysis. Two categories of SCD leg ulcers were distinguished, defined by a 1-year duration cut off. The ,prolonged' ulcers had larger surfaces, tended to recur more frequently and led to more infection and depression. Several SCD complications were associated with leg ulcers, notably priapism, pulmonary hypertension, stroke and acute chest syndrome. Conclusions, Leg ulcers are a major complication of SCD, given their severe consequences and frequent association with other specific organ damage, and they constitute in their ,prolonged' form a severely disabling condition that remains an important therapeutic challenge. [source] Myocardial infarction following sickle cell chest syndromeBRITISH JOURNAL OF HAEMATOLOGY, Issue 1 2006Mark A. Tanner No abstract is available for this article. [source] Are there clinical phenotypes of homozygous sickle cell disease?BRITISH JOURNAL OF HAEMATOLOGY, Issue 4 2004Neal Alexander Summary The distribution of clinical features was examined in subjects with homozygous sickle cell (SS) disease in the Jamaican Cohort Study to determine whether there is evidence of distinct clustering of symptoms or clinical phenotypes. A twofold model yielded groups that could be interpreted as painful crisis or leg ulcer phenotypes and 78% of patients were classified with 95% confidence into one of these. The painful crisis phenotype also manifested higher frequencies of dactylitis, meningitis/septicaemia, acute chest syndrome and stroke. Attempts to define a three-group model were less convincing although 43% of patients could be allocated with 95% confidence. The three-group model essentially divided subjects with the leg ulcer phenotype into subgroups with higher and lower frequencies of painful crisis, dactylitis, meningitis/septicaemia and acute chest syndrome. In the three-group model, the painful crisis phenotype had lower total haemoglobin, fetal haemoglobin, mean cell volume and higher reticulocytes but there was no apparent influence of alpha thalassaemia or beta globin haplotype. Both environmental and genetic factors are likely to contribute to most manifestations of SS disease and the evidence for different clinical phenotypes suggests that a search for associated genetic polymorphisms may provide insights into the mechanisms of clinical variability in SS disease. 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