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Clinical Investigations: DIFFUSE LUNG DISEASE |

Acute Chest Syndrome in Adults With Sickle Cell Disease*: Therapeutic Approach, Outcome, and Results of BAL in a Monocentric Series of 107 Episodes FREE TO VIEW

Bernard Maitre, MD; Anoosha Habibi, MD; Françoise Roudot-Thoraval, MD; Dora Bachir, MD; Dominique Desvaux Belghiti, MD; Frederic Galacteros, MD; Bertrand Godeau, MD
Author and Funding Information

*From the Sickle Cell Disease Center (Drs. Habibi, Bachir, Galacteros, and Godeau), Unit of Pulmonary Diseases (Dr. Maitre), Department of Public Health (Dr. Roudot-Thoraval), and Department of Pathology (Dr. Belghiti), Hôpital Henri Mondor, A.P.H.P., Créteil, France.

Correspondence to: Bernard Maitre, MD, Unité de Pneumologie, Service de Réanimation Médicale, Hôpital Henri Mondor, A.P.H.P. 94 010 Créteil, France; e-mail: bernard.maitre@hmn.ap-hop-paris.fr



Chest. 2000;117(5):1386-1392. doi:10.1378/chest.117.5.1386
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Study objectives: Acute chest syndrome (ACS) is a frequent and potentially severe pulmonary illness in sickle cell disease (SCD). The aim of the study was to report the clinical features and outcome of consecutive ACS episodes in adult patients in a French SCD center. All patients were treated according to an uniform therapeutic protocol applying transfusion only in the more severe clinical form of ACS.

Results: There were 107 consecutive episodes in 77 adult patients (mean age, 29 ± 7 years; 78% hemoglobin [Hb] SS; 14% Hb SC; and 8% Hb Sβ + thalassemia) over a 6-year period. Seventy-eight percent of our patients had an associated vaso-occlusive crisis that preceded the chest signs in half of the cases. Comparison between acute and baseline levels showed a statistically significant difference in Hb levels (drop of 1.6 to 2.25 g/dL depending on Hb genotype), WBC count (increase of 9.2 ± 8.3 × 109/L); platelet count (increase of 67 ± 209 × 109/L); and lactate dehydrogenase values (increase of 358 ± 775 IU/L) in ACS patients. Hypercapnia was detected in 42% of patients without sign of narcotic abuse. We identified a high percentage of alveolar macrophages containing fat droplets in 31 of 43 (77%) patients who underwent BAL. Bacterial culture findings were almost always negative, but were performed after starting antibiotic therapy that was administered in 96 episodes. Transfusion was required in 50 of 107 ACS events (47%). Five patients died, and all were transfused.

Conclusions: These results confirm that fat embolism is probably a frequent mechanism of ACS in adult patients. However, fat embolism was not associated with a more severe clinical course, suggesting that bronchoscopy and BAL have little impact on the management of these patients. Restricting transfusion to the most severe ACS cases does not seem to increase the mortality rate.

Figures in this Article

Acute chest syndrome (ACS) is a complication of sickle cell disease (SCD), defined by occurrence of chest symptoms, new pulmonary infiltrate on chest radiograph, and in some cases fever. Several pathologic processes have been recognized to cause ACS, including infectious diseases, in situ thrombosis, hypoventilation secondary to chest pain, and fat embolism.110 Postmortem studies have confirmed the detection of fat emboli from bone marrow infarction in severe forms of ACS.11Vichinsky et al12and our group13 investigated the presence of fat droplets in alveolar cells recovered by BAL in ACS episodes. The results of both studies suggest that fat embolism is frequent, but its prognostic value is unknown. Various studies have described the clinical characteristics and prognosis of ACS, but most have been multicentric, concerning mostly pediatric populations, and providing little comment about treatment issues.5,1419 Transfusion, in addition to symptomatic treatment, has been shown to be very effective in decreasing respiratory symptoms in children with ACS, but the benefit of this treatment is unknown. We determined the clinical course, treatment, and outcome of ACS in adult SCD patients in a monocentric study of 107 consecutive ACS episodes in 77 adult patients. We determined the frequency and prognostic value of fat embolism in ACS in adult patients by investigating whether fat droplets were present in alveolar cells recovered by BAL in 43 ACS episodes.

Patients

All patients were recruited from the Sickle Cell Disease Center at the Henri Mondor University Hospital, Créteil, France, which follows a cohort of 800 adult patients. All consecutive ACS episodes in this cohort were recorded from 1991 to 1997. Hemoglobin (Hb) phenotype was determined by standard procedure to be homozygous Hb SS, Hb SC, or Hb Sβ-thalassemia. β-globin cluster haplotypes were determined as previously described.20 The diagnosis of ACS was based on the presence of fever or chest pain, associated with new pulmonary infiltrates on chest radiograph. Clinical events, including fever, chest pain, prior painful episodes, cough, hemoptysis, and crackles on lung auscultation, were recorded. The laboratory tests recorded were hematologic values, urea nitrogen, creatinine, glucose, plasma electrolytes, bilirubin, aspartate and alanine aminotransferases, alkaline phosphatase, and lactate dehydrogenase (LDH). Precritical values for each individual were those recorded > 1 month away from any clinical event, and 3 months from the last transfusion. For the corresponding ACS values, the most pathologic biological value in the first 72 h of hospitalization was selected. Blood and urine samples for culture were taken from each patient on admission. Blood tests were routinely performed for Mycoplasma, Chlamydia, Legionella spp, and Coxiella burnetti, as were indirect immunfluorescence tests for Legionella pneumophila in bronchial aspirate. Room air arterial blood gas was generally determined once during hospitalization, and chest radiographs were recorded on the day of ACS diagnosis.

Bronchoscopy Procedure

Forty-eight patients underwent bronchoscopy. Twenty-five consecutive BALs were performed to evaluate the value of neutral fat detection in ACS.13 Thereafter, BAL were performed only in the most severe episodes. Bronchoscopy was performed using an Olympus breath frequency P 30 fiberoptic bronchoscope (Olympus France; Rungis, France). A plugged telescoping catheter (PTC) procedure was performed for the setting up of bacterial quantitative cultures, in an area pathologic in appearance on chest radiograph, as previously described.21BAL (three aliquots of 50 mL) was then performed in the same area. Quantitative BAL bacterial cultures were performed as previously described.22 The second and third aliquots of BAL fluid were pooled and immediately processed for cytologic examination. The cells were stained by the May-Grunwald-Giemsa, Papanicolaou, and Perls methods. To detect neutral fat, staining using the Oil-Red-O (ORO) method was performed as previously described.13 The cutoff, determined in a previous study using two control groups, was set at 5% ORO-stained macrophages.13

Treatment

A uniform standardized treatment protocol was applied for all patients. Symptomatic treatment included IV rehydration (30 mL/kg/d, not > 2,000 mL/d); nasal-administered oxygen, unless oxygen saturation measured by pulse oximetry was > 95%; and analgesia with IV-administered proparacetamol (1 g q6h), complemented if necessary with narcotics (controlled-release morphinomimetics). Antibiotic treatment was started immediately after blood and urine samples were taken for culture, in patients with fever on admission. The decision to treat patients with antibiotics was based on British and French guidelines for the management of community-acquired pneumonia.2324

RBC transfusion was administered only in the following circumstances: (1) without delay for patients with severe hypoxemia and rapid worsening of symptoms (< 24 h) with acute respiratory failure; (2) after a follow-up of 3 days for patients with initially mild to moderate hypoxemia but chest pain worsening and/or new infiltrate on chest radiograph. Patients received blood from donors testing negative for Hb S, that had been phenotyped and leukodepleted. Depending on Hb level, simple RBC transfusion was performed to attain individual precritical Hb levels, or partial-exchange transfusion was performed to achieve a total of < 40% Hb S.

Statistical Analysis

All results are presented as mean ± SD. Categorical variables were compared with the χ2 or Fisher’s Exact Test. Quantitative variables were compared using the Kruskall-Wallis nonparametric test. Results were considered to be significantly different for p values < 0.05.

One hundred seven consecutive episodes of ACS were considered in 77 patients. Of these patients, 52 had one ACS episode, 17 had two episodes, and 7 had three episodes. For 14 patients (20%), ACS was the first severe acute event related to SCD. Table 1 shows the baseline characteristics of the 77 patients before the first episode. The haplotype distribution of patients presenting ACS was similar to that of the total patient population followed at our center. Only three patients received chronic hydroxyurea treatment, and seven patients were treated with a chronic transfusion protocol for severe repeated vaso-occlusive crises (VOC).

Clinical, Radiographic, and Laboratory Findings

As shown in Table 2 , VOC were very frequently observed a few days before the onset of ACS. Cough, wheezing, and hemoptysis were noted in few cases. However, we detected no severe CNS symptom suggesting systemic fat embolism syndrome in this group of patients. The most frequent clinical signs were bilateral rales on auscultation and fever. On chest radiographs, lower lobe diseases were the most frequent and half the patients had bilateral infiltrates or pleural effusion. Laboratory test values (Table 3 ) revealed abnormalities usually observed in VOC (anemia, leukocytosis, increase in LDH value). Arterial blood gases indicated hypoxemia (Pao2 < 80 mm Hg) in 88% of patients, and, surprisingly, normocapnea/hypercapnia was detected in 46% of patients.

Bacteriology

Bacteremia was observed in only one ACS episode (unclassified Streptococcus). Urine culture was positive (Escherichia coli) for one patient. There was no bacterial growth in secretion samples obtained by BAL and PTC, at a concentration≥ 103 cfu/mL for PTC, or 104 cfu/mL for BAL. Bronchoscopy was, however, always carried out after antibiotic treatment, which was frequently started before hospital admission. All serologic studies for atypical microorganisms were negative.

BAL

Forty-eight bronchoscopies with BAL were performed, and complete data (ORO staining and cell counts) are available for 41 episodes. ORO staining was not performed in five cases, and in four cases, BAL differential cell count showed a low total cell count and a high percentage of bronchial epithelial cells, suggesting poor lavage recovery and bronchial contamination. BAL cell count results are given in Table 4 . Patients with fat embolism defined by percentage of stained alveolar macrophages > 5% had a statistically higher total cell count, differential cell count of lymphocytes, and polymorphonuclear neutrophil (PMN) cells. A correlation was also found between the percentage of PMN and the percentage of ORO-stained alveolar macrophages (ρ = 0.52, p = 0.001). We avoided having to define a cutoff for the percentage of ORO-stained cells, by arbitrarily assigning patients to three groups: group I, ≤ 5% ORO + macrophages; group II, 5% ≤ ORO + < 40%; group III, ORO + ≥ 40%. No significant difference in ACS characteristics was observed between groups of patients, except that the mean length of hospitalization was longer in group III (14.6 vs 8.3 days, p < 0.05; Table 5 ).

Treatment

In 96 of 107 episodes, the patients were treated with antibiotics. Twenty-nine patients received two antibiotics, and 3 received tritherapy. Eighty-seven patients received amoxicillin or amoxicillin/clavulanate, 17 received macrolides, 16 received fluoroquinolones, and 12 received cephalosporins. RBC transfusions were given in 50 of the 107 ACS episodes (46.7%). Simple transfusion was carried out in 16 ACS episodes and partial-exchange transfusion in 34 ACS episodes. Transfusion was administered in the 48 h following ACS onset in 19 patients (35%), and from 3 to 14 days after onset in the other 75 cases (65%; Fig 1 ). We compared the baseline and clinical characteristics of two groups of patients: patients who underwent secondary transfusion, and patients who did not undergo transfusion (Table 6 ). As expected, clinical characteristics at the time of ACS were more severe in the group of patients who received transfusion. Low Hb level was the only precritical factor for which individual values were related to transfusion requirement.

Hospital Course

The mean length of stay in hospital was 11.4 days. Admission to the ICU was required in 29 cases (28%). As expected, none of the patients in the nontransfused group died, whereas five patients who received transfusions died (death rate, 4.6%). Three of the five patients who died were homozygous (SS) for the sickle cell trait, and two were heterozygous (SC). Only one had a history of ACS and frequent VOC, and was treated by chronic transfusion. The thoracic symptoms of four of the five patients began in a surgical context (cesarean section, abortion, abdominal surgery). All patients suffered respiratory failure within 48 h of the onset of clinical symptoms. All patients were treated with broad-spectrum antibiotics, and no sepsis was detected during hospitalization. Four of the five patients underwent partial exchange transfusion during the first 12 h after acute respiratory failure. The patient who received a simple transfusion died 16 h after the first chest symptom, and the primary cause of death was cardiac failure. The autopsy report was available for only one patient; it recorded edema, alveolar wall necrosis, and massive fat embolism in the lungs (Fig 2 ).

The aim of this study was to determine the characteristics and outcome of ACS in adult patients. Recent studies, particularly the report of the Cooperative Study of Sickle Cell Disease (CSSCD), have described ACS clinical features in large numbers of patients.5,1419 However, most of these studies have focused on patients < 16 years old. In contrast, our monocentric study included only adult patients treated according to an uniform protocol in which transfusion was used only for the most severe ACS episodes. BAL, with ORO staining for macrophages, was performed in a large number of patients, enabling us to evaluate the clinical characteristics of fat embolism syndrome.

Our patients had similar clinical and biological characteristics to the 252 adult patients of the CSSCD cohort.14 The decrease of Hb levels and increase of WBC counts are well known to be prognostic factors for acute events in SCD, and have been found in our study and the CSSCD cohort. We also observed an increase in platelet count and LDH concentration in ACS patients. However, the CSSCD study described VOC in only 25% of adult patients, whereas in our study, 78% of patients had at least one lower-extremity finding consistent with an associated VOC, and this symptom preceded chest abnormalities in half the cases. These, and our BAL results, confirm previous reports that associated bone marrow infarction is frequently suspected in adult patients. In a study of ACS in 27 children, Vichinsky et al12 found a frequency of 40% fat embolism. As we performed BAL in only 39 ACS events, we cannot estimate the frequency of fat embolism in ACS. The characteristics of the patients with and without BAL were similar (data not shown), suggesting that the observed percentage of fat embolism is probably accurate. None of the patients received IV lipid infusion, or had a history of aspiration pneumonia or gastroesophageal reflux before ACS onset, which may induce the detection of neutral fat in BAL.

The clinical and prognostic value of pulmonary fat embolism in ACS events is unclear. Vichinsky et al12 concluded from a study of 27 patients that ACS may be more severe in cases with pulmonary fat embolism. In a previous study including 20 adult patients and in this study, we found no difference in severity of ACS between patients with and without fat embolism, but the clinical course tended to be longer for patients with fat embolism. Fat embolism occurs in most trauma patients, but fat embolism syndrome is clinically diagnosed in only about 10% of such patients.25 This may account for the absence of petechial rash, mental status changes, and decreases in platelet and Hb counts in the population studied. The higher platelet counts recorded here differ from those observed by Vichinsky et al12 in a pediatric series, but this difference in results may be due to the functional asplenism observed in almost all adult patients.

The detection of hypercapnia in 46% of these hypoxemic patients is surprising. Such high levels of hypercapnia have never before been reported. There was no correlation between the severity of ACS, Pao2, and Paco2 levels. The severity of ACS may be related to hypoventilation due to rib infarcts or the use of narcotic analgesia. Rucknagel et al1 showed that rib infarcts detected by 99m-technetium-diphosphonate bone scan were frequently associated with ACS events. Although an association between the severity of ACS and the use of narcotic analgesia has been suggested,26 no direct effect of hypoventilation leading to atelectasis and then to local hypoxia and pulmonary intravascular sickling has been demonstrated. None of our patients developed signs of narcotic abuse, and various studies have suggested that controlling pain secondary to rib infarct and incentive spirometry are very important in the control of hypoventilation in ACS patients.1,2627 For these reasons, we think it is preferable to provide rapid pain relief to obtain a better thoracic vital capacity in these patients.

The clinical course and outcome of our patients were similar to those of patients in previous studies. These results support our choice for the management of ACS in hospitalized patients. Two treatment aspects are of particular interest: antibiotics and transfusion. We systematically administered antibiotics to febrile patients, but detected no microorganisms in serologic and BAL studies. However, bronchoscopy was always performed after empiric antibiotic administration, and this may explain why no bacteria were detected in lung samples. Our data seem to be consistent with the only previous prospective study28 on bronchial secretions from adults with ACS, suggesting that bacterial pneumonia is not frequent in adult episodes of ACS. However, we still recommend the use of amoxicillin as the first-line antibiotic treatment in adult patients with ACS.

In this study, we decided to perform transfusion only in the most severe cases. These were defined as patients with early acute respiratory failure and patients with mild respiratory distress that worsened after 3 days. Transfusions are often recommended for the treatment of ACS and were performed in 75% of episodes in a large American study,29but no controlled studies have been performed to evaluate the effect of transfusion on lung function and mortality. Transfusion may have side effects, such as pulmonary edema, blood-borne infections, and alloimmunization, so this treatment should not be used for all ACS episodes.3031 Using clinical criteria, transfusion was not required in about half the patients in our study, and none of the patients in the untreated group died, which demonstrates that transfusion should be restricted to the more severely affected patients.

Thus, we have presented herein BAL results and the response to uniform treatment of adult patients admitted to hospital for ACS. We recorded a high frequency of fat embolism detected by BAL in these patients. However, the presence of fat in alveolar macrophages seems to be unrelated to the severity of symptoms, biological abnormalities, and clinical outcome. All these results suggest that bronchoscopy and BAL have little impact on the management of these patients, and may therefore lead to a reduction in the use of such investigations. Restricting transfusions to the most severe ACS cases, based mainly on the standard clinical evaluation of these patients, does not increase the mortality rate. Future studies should focus on the more accurate characterization of severe patients, so that the therapeutic approach can be further improved.

Abbreviations: ACS = acute chest syndrome; CSSCD = Cooperative Study of Sickle Cell Disease; Hb = hemoglobin; LDH = lactate dehydrogenase; ORO = Oil-Red-O; PMN = polymorphonuclear neutrophil; PTC = plugged telescoping catheter; SCD = sickle cell disease; VOC = vaso-occlusive crises

Table Graphic Jump Location
Table 1. Baseline Characteristics of Patients Presenting With ACS*
* 

Data are presented as mean ± SD or No. (%) unless otherwise indicated; CAR = Central African Region.

 

Normal value < 250 IU/L.

Table Graphic Jump Location
Table 2. Clinical and Radiographic Findings of Patients With ACS*
* 

Data are presented as No. (%) unless otherwise indicated.

Table Graphic Jump Location
Table 3. Laboratory Values of ACS Patients and Comparison With Baseline Values*
* 

Data are presented as mean SEM unless otherwise indicated.

 

Normal value < 250 IU/L.

Table Graphic Jump Location
Table 4. BAL Cell Counts in ACS Patients Separated in Two Groups Depending on the Percentage of Macrophages ORO +*
* 

Data are presented as mean ± SD unless otherwise indicated; MAC = alveolar macrophages; LYM = lymphocytes.

 

p < 0.01.

 

p < 0.005.

§ 

p < 0.001, comparison between groups I and II.

Table Graphic Jump Location
Table 5. Comparison of Patients Who Underwent Bronchoscopy Depending of the Percentage of ORO + Alveolar Macrophages in BAL*
* 

Data are presented as mean ± SD unless otherwise indicated.

 

Normal value < 250 IU/L.

 

p < 0.05, comparison between groups I and III.

Figure Jump LinkFigure 1. Time interval between ACS onset and transfusion in the 50 patients. Dark columns correspond to all patients, and hatched columns to patients who died from ACS.Grahic Jump Location
Table Graphic Jump Location
Table 6. Comparison of ACS Events in Patients Who Underwent Transfusion Therapy vs Patients Who Did Not Undergo Transfusion Therapy*
* 

Data are presented as mean ± SD or No. (%) unless otherwise indicated.

 

Normal value < 250 IU/L.

Figure Jump LinkFigure 2. Postmortem pulmonary biopsy: massive pulmonary fat embolism detected by ORO staining (arrows).Grahic Jump Location
Rucknagel, D, Kalinyak, K, Gelfand, M (1991) Rib infarcts and acute chest syndrome in sickle cell disease.Lancet337,831-833. [CrossRef] [PubMed]
 
Miller, S, Hammerschlag, M, Chirgwin, K, et al Role ofChlamydia pneumoniaein acute chest syndrome of sickle cell disease.J Pediatr1991;118,30-33. [CrossRef] [PubMed]
 
Lowenthal, E, Wells, A, Emanuel, P, et al Sickle cell acute chest syndrome associated with parvovirus B19 infection.Am J Hematol1996;51,207-213. [CrossRef] [PubMed]
 
Israel, R, Salipante, J Pulmonary infarction in sickle cell trait.Am J Med1979;66,1787-1792
 
Haynes, J, Kirkpatrick, MB The acute chest syndrome of sickle cell disease.Am J Med Sci1993;305,326-330. [CrossRef] [PubMed]
 
Godeau, B, Dhainaut, JF, Bachir, D, et al Pulmonary fat embolism after prostaglandin infusion in sickle cell disease with fatal outcome despite exchange blood transfusion.Am J Hematol1993;43,330-331
 
Gelfand, M, Daya, S, Rucknagel, D, et al Simultaneous occurrence of rib infarction and pulmonary infiltrates in sickle cell disease patients with acute chest syndrome.J Nucl Med1993;34,614-618. [PubMed]
 
Dreyer, Z Chest infections and syndromes in sickle cell disease of childhood.Semin Respir Infect1996;11,163-172. [PubMed]
 
Cockshott, P Rib infarcts in sickling disease.Eur J Radiol1992;14,63-66. [CrossRef] [PubMed]
 
Bhalla, M, Abboud, M, McLoud, T, et al Acute chest syndrome in sickle cell disease: CT evidence of microvascular occlusion.Radiology1993;187,45-49. [PubMed]
 
Haupt, HM, Moor, GW, Bauer, TW The lung in sickle cell disease.Chest1982;81,332-337. [CrossRef] [PubMed]
 
Vichinsky, E, Williams, R, Das, M, et al Pulmonary fat embolism: a distinct cause of severe acute chest syndrome in sickle cell anemia.Blood1994;83,3107-3112. [PubMed]
 
Godeau, B, Schaeffer, A, Bachir, 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 Med1996;153,1691-1696. [PubMed]
 
Vichinsky, EP, Styles, LA, Colangelo, LH, et al Acute chest syndrome in sickle cell disease: clinical presentation and course.Blood1997;89,1787-1792. [PubMed]
 
Van Agtmael, M, Cheng, J, Nossent, H Acute chest syndrome in adult Afro-Carribean patients with sickle cell disease.Arch Intern Med1994;154,557-561. [CrossRef] [PubMed]
 
Sprinkle, R, Cole, T, Smith, S, et al Acute chest syndrome in children with sickle cell disease.Am J Pediatr1986;8,105-110
 
Davies, S, Luce, P, Win, A, et al Acute chest syndrome in sickle cell disease.Lancet1984;7,36-38
 
Barret Connor, E Acute pulmonary disease and sickle cell anemia.Am Rev Respir Dis1971;104,159-165. [PubMed]
 
Castro, O, Brambilla, D, Thorington, B, et al The acute chest syndrome in sickle cell disease: incidence and risk factors.Blood1994;84,643-649. [PubMed]
 
Sutton, M, Bouhassira, E, Nagel, R Polymerase chain reaction amplification applied to the determination of beta-like globin gene cluster haplotypes.Am J Hematol1989;32,66-69. [CrossRef] [PubMed]
 
Pham, L, Brun-Buisson, C, Legrand, P, et al Diagnosis of nosocomial pneumonia in mechanically ventilated patients: comparison of a plugged telescoping catheter with the protected specimen brush.Am J Respir Dis1991;143,1055-1061
 
Chastre, J, Fagon, J, Bornet-Lecso, M, et al Evaluation of bronchoscopic techniques for the diagnosis of nosocomial pneumonia.Am J Respir Crit Care Med1995;152,231-240. [PubMed]
 
British Thoracic Society.. Guidelines for the management of community-acquired pneumonia in adults admitted to hospital.Br J Hosp Med1993;49,346-350. [PubMed]
 
Société de Pathologie Infectieuse de Langue Française: les infections des voies respiratoires. Med Mal Infect 1991; 21:1–8.
 
Chan, K, Tham, K, Chiu, H, et al Post-traumatic fat embolism: its clinical and subclinical presentations [abstract]. J Trauma. 1984;;24 ,.:45. [CrossRef] [PubMed]
 
Palmer, J, Broderick, K, Naiman, J Acute lung syndrome during painful sickle cell crisis: relation to site of pain and narcotic requirement [abstract]. Blood. 1983;;62 ,.:59A
 
Bellet, P, Kalinyak, K, Shukla, R, et al Incentive spirometry to prevent acute pulmonary complications in sickle cell disease.N Engl J Med1995;333,699-703. [CrossRef] [PubMed]
 
Kirkpatrick, M, Haynes, J, Bass, J Results of bronchoscopically obtained lower airway cultures from adult sickle cell disease patients with the Acute Chest Syndrome.Am J Med1991;90,206-210. [PubMed]
 
Vichinsky, E Acute chest syndrome associated with neurologic findings: an often fatal complication of sickle cell disease [abstract]. Blood. 1998;;92 ,.:159A
 
Vichinsky, EP, Earles, A, Johnson, RA, et al Alloimmunization in sickle cell anemia and transfusion of racially unmatched blood.N Engl J Med1990;322,1617-1621. [CrossRef] [PubMed]
 
Vichinsky, E Transfusion therapy. Embury, S Hebbel, R Mohandas, Net al eds.Sickle cell disease: basic principles and clinical practice1994,781-793 Raven Press. New York, NY:
 

Figures

Figure Jump LinkFigure 1. Time interval between ACS onset and transfusion in the 50 patients. Dark columns correspond to all patients, and hatched columns to patients who died from ACS.Grahic Jump Location
Figure Jump LinkFigure 2. Postmortem pulmonary biopsy: massive pulmonary fat embolism detected by ORO staining (arrows).Grahic Jump Location

Tables

Table Graphic Jump Location
Table 1. Baseline Characteristics of Patients Presenting With ACS*
* 

Data are presented as mean ± SD or No. (%) unless otherwise indicated; CAR = Central African Region.

 

Normal value < 250 IU/L.

Table Graphic Jump Location
Table 2. Clinical and Radiographic Findings of Patients With ACS*
* 

Data are presented as No. (%) unless otherwise indicated.

Table Graphic Jump Location
Table 3. Laboratory Values of ACS Patients and Comparison With Baseline Values*
* 

Data are presented as mean SEM unless otherwise indicated.

 

Normal value < 250 IU/L.

Table Graphic Jump Location
Table 4. BAL Cell Counts in ACS Patients Separated in Two Groups Depending on the Percentage of Macrophages ORO +*
* 

Data are presented as mean ± SD unless otherwise indicated; MAC = alveolar macrophages; LYM = lymphocytes.

 

p < 0.01.

 

p < 0.005.

§ 

p < 0.001, comparison between groups I and II.

Table Graphic Jump Location
Table 5. Comparison of Patients Who Underwent Bronchoscopy Depending of the Percentage of ORO + Alveolar Macrophages in BAL*
* 

Data are presented as mean ± SD unless otherwise indicated.

 

Normal value < 250 IU/L.

 

p < 0.05, comparison between groups I and III.

Table Graphic Jump Location
Table 6. Comparison of ACS Events in Patients Who Underwent Transfusion Therapy vs Patients Who Did Not Undergo Transfusion Therapy*
* 

Data are presented as mean ± SD or No. (%) unless otherwise indicated.

 

Normal value < 250 IU/L.

References

Rucknagel, D, Kalinyak, K, Gelfand, M (1991) Rib infarcts and acute chest syndrome in sickle cell disease.Lancet337,831-833. [CrossRef] [PubMed]
 
Miller, S, Hammerschlag, M, Chirgwin, K, et al Role ofChlamydia pneumoniaein acute chest syndrome of sickle cell disease.J Pediatr1991;118,30-33. [CrossRef] [PubMed]
 
Lowenthal, E, Wells, A, Emanuel, P, et al Sickle cell acute chest syndrome associated with parvovirus B19 infection.Am J Hematol1996;51,207-213. [CrossRef] [PubMed]
 
Israel, R, Salipante, J Pulmonary infarction in sickle cell trait.Am J Med1979;66,1787-1792
 
Haynes, J, Kirkpatrick, MB The acute chest syndrome of sickle cell disease.Am J Med Sci1993;305,326-330. [CrossRef] [PubMed]
 
Godeau, B, Dhainaut, JF, Bachir, D, et al Pulmonary fat embolism after prostaglandin infusion in sickle cell disease with fatal outcome despite exchange blood transfusion.Am J Hematol1993;43,330-331
 
Gelfand, M, Daya, S, Rucknagel, D, et al Simultaneous occurrence of rib infarction and pulmonary infiltrates in sickle cell disease patients with acute chest syndrome.J Nucl Med1993;34,614-618. [PubMed]
 
Dreyer, Z Chest infections and syndromes in sickle cell disease of childhood.Semin Respir Infect1996;11,163-172. [PubMed]
 
Cockshott, P Rib infarcts in sickling disease.Eur J Radiol1992;14,63-66. [CrossRef] [PubMed]
 
Bhalla, M, Abboud, M, McLoud, T, et al Acute chest syndrome in sickle cell disease: CT evidence of microvascular occlusion.Radiology1993;187,45-49. [PubMed]
 
Haupt, HM, Moor, GW, Bauer, TW The lung in sickle cell disease.Chest1982;81,332-337. [CrossRef] [PubMed]
 
Vichinsky, E, Williams, R, Das, M, et al Pulmonary fat embolism: a distinct cause of severe acute chest syndrome in sickle cell anemia.Blood1994;83,3107-3112. [PubMed]
 
Godeau, B, Schaeffer, A, Bachir, 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 Med1996;153,1691-1696. [PubMed]
 
Vichinsky, EP, Styles, LA, Colangelo, LH, et al Acute chest syndrome in sickle cell disease: clinical presentation and course.Blood1997;89,1787-1792. [PubMed]
 
Van Agtmael, M, Cheng, J, Nossent, H Acute chest syndrome in adult Afro-Carribean patients with sickle cell disease.Arch Intern Med1994;154,557-561. [CrossRef] [PubMed]
 
Sprinkle, R, Cole, T, Smith, S, et al Acute chest syndrome in children with sickle cell disease.Am J Pediatr1986;8,105-110
 
Davies, S, Luce, P, Win, A, et al Acute chest syndrome in sickle cell disease.Lancet1984;7,36-38
 
Barret Connor, E Acute pulmonary disease and sickle cell anemia.Am Rev Respir Dis1971;104,159-165. [PubMed]
 
Castro, O, Brambilla, D, Thorington, B, et al The acute chest syndrome in sickle cell disease: incidence and risk factors.Blood1994;84,643-649. [PubMed]
 
Sutton, M, Bouhassira, E, Nagel, R Polymerase chain reaction amplification applied to the determination of beta-like globin gene cluster haplotypes.Am J Hematol1989;32,66-69. [CrossRef] [PubMed]
 
Pham, L, Brun-Buisson, C, Legrand, P, et al Diagnosis of nosocomial pneumonia in mechanically ventilated patients: comparison of a plugged telescoping catheter with the protected specimen brush.Am J Respir Dis1991;143,1055-1061
 
Chastre, J, Fagon, J, Bornet-Lecso, M, et al Evaluation of bronchoscopic techniques for the diagnosis of nosocomial pneumonia.Am J Respir Crit Care Med1995;152,231-240. [PubMed]
 
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