Case 17-2005: Acute Chest Syndrome and ARDS
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《新英格兰医药杂志》
To the Editor: The case described by Medoff et al. (June 9 issue)1 and a similar one published in the Journal eight years ago2 illustrate how rapidly the acute respiratory distress syndrome (ARDS) related to sickle cell anemia can become irreversible. In the current case, aggressive transfusions and exchange transfusions, which are the specific treatment for sickle cell disease–related ARDS,3 were ineffective 25.5 hours after severe hypoxemia was first detected (5.5 hours after the first admission). In the previously published case, exchange transfusions were performed 24 to 48 hours after the initial documentation of the values for oxygen saturation and fraction of inspired oxygen that were indicative of ARDS, but they also failed. Most hematologists who treat patients with sickle cell anemia are aware of the urgency of the problem and institute transfusion or exchange-transfusion therapy, or both, on an emergency basis in acutely hypoxemic patients. It is a pity that exchange transfusions were not listed as specific treatment (in Table 4 of the article) and that they do not appear anywhere in the management algorithm (Figure 2 of the article), even though the patient in the report died of sickle cell disease–related ARDS. All of the steps recommended in the ARDS algorithm are necessary, but without emergency transfusions they will remain insufficient in patients with sickle cell disease.
Oswaldo Castro, M.D.
Howard University Center for Sickle Cell Disease
Washington, DC 20059
olcastro@aol.com
Mark T. Gladwin, M.D.
National Institutes of Health Clinical Center
Bethesda, MD 20892
References
Case Records of the Massachusetts General Hospital (Case 17-2005). N Engl J Med 2005;352:2425-2434.
Case Records of the Massachusetts General Hospital (Case 34-1997). N Engl J Med 1997;337:1293-1301.
Acute chest syndrome and other pulmonary complications. In: Division of Blood Diseases and Resources. The management of sickle cell disease. 4th ed. Bethesda, Md.: National Heart, Lung, and Blood Institute, June 2002:103-10. (NIH publication no. 02-2117.)
To the Editor: In the clinical and pathological discussion of ARDS and acute chest syndrome in the case described in the June 9 issue, there is an important omission. Physicians should always suspect tension pneumothorax as the cause of sudden hemodynamic collapse in patients with catastrophic ARDS. The application of extreme levels of positive end-expiratory pressure (28 cm of water was the level documented before the patient's death) and the appearance of the endotracheal tube just above the origin of the right mainstem bronchus (as shown in Figure 1 of the article) increase the risk of lung overdistention and pneumothorax. We wonder if needle or tube thoracentesis was performed during the cardiac arrest concurrently with the "maximal treatment with norepinephrine, vasopressin, phenylephrine, dopamine, epinephrine, and atropine," or whether it should be performed in similar scenarios.
Petros Kopterides, M.D.
Attikon University Hospital
12462 Athens, Greece
petkop@ath.forthnet.gr
Richard Fremont, M.D.
Emory University School of Medicine
Atlanta, GA 30322
The discussant replies: Drs. Castro and Gladwin point out an important omission in Table 4 in that exchange transfusion should have been listed in the specific-treatment column for miscellaneous causes of ARDS. As they note, and as I indicated in the text of the article, exchange transfusions should be considered early in patients who have acute chest syndrome with rapid decline in their respiratory status. Unfortunately for the patient in this case, this therapy was not available until late in her course of illness. The management algorithm in Figure 2 of the article was meant to outline a general approach to patients with catastrophic ARDS and thus did not include specific therapies such as exchange transfusions.
The development of a tension pneumothorax can be an important cause of hemodynamic collapse and of a rapid decline in respiratory status in critically ill patients with ARDS, as Drs. Kopterides and Fremont note. The patient in question did not receive needle or tube decompression during cardiac arrest; however, at autopsy she did not have a pneumothorax noted. High pleural pressures can complicate the management of critically ill patients with ARDS by leading to increased lung collapse and hemodynamic compromise. An increase in pleural pressures can result from a pneumothorax, from rapidly expanding effusions secondary to empyema or hemothorax, or from a decrease in chest-wall compliance — as might be seen in abdominal compartment syndrome. In such patients, placement of a chest tube or abdominal decompression can dramatically improve oxygenation and hemodynamics and should be considered in the proper clinical setting.1
Benjamin D. Medoff, M.D.
Massachusetts General Hospital
Boston, MA 02114
References
Medoff BD, Harris RS, Kesselman H, Venegas J, Amato MB, Hess D. Use of recruitment maneuvers and high-positive end-expiratory pressure in a patient with acute respiratory distress syndrome. Crit Care Med 2000;28:1210-1216.
Oswaldo Castro, M.D.
Howard University Center for Sickle Cell Disease
Washington, DC 20059
olcastro@aol.com
Mark T. Gladwin, M.D.
National Institutes of Health Clinical Center
Bethesda, MD 20892
References
Case Records of the Massachusetts General Hospital (Case 17-2005). N Engl J Med 2005;352:2425-2434.
Case Records of the Massachusetts General Hospital (Case 34-1997). N Engl J Med 1997;337:1293-1301.
Acute chest syndrome and other pulmonary complications. In: Division of Blood Diseases and Resources. The management of sickle cell disease. 4th ed. Bethesda, Md.: National Heart, Lung, and Blood Institute, June 2002:103-10. (NIH publication no. 02-2117.)
To the Editor: In the clinical and pathological discussion of ARDS and acute chest syndrome in the case described in the June 9 issue, there is an important omission. Physicians should always suspect tension pneumothorax as the cause of sudden hemodynamic collapse in patients with catastrophic ARDS. The application of extreme levels of positive end-expiratory pressure (28 cm of water was the level documented before the patient's death) and the appearance of the endotracheal tube just above the origin of the right mainstem bronchus (as shown in Figure 1 of the article) increase the risk of lung overdistention and pneumothorax. We wonder if needle or tube thoracentesis was performed during the cardiac arrest concurrently with the "maximal treatment with norepinephrine, vasopressin, phenylephrine, dopamine, epinephrine, and atropine," or whether it should be performed in similar scenarios.
Petros Kopterides, M.D.
Attikon University Hospital
12462 Athens, Greece
petkop@ath.forthnet.gr
Richard Fremont, M.D.
Emory University School of Medicine
Atlanta, GA 30322
The discussant replies: Drs. Castro and Gladwin point out an important omission in Table 4 in that exchange transfusion should have been listed in the specific-treatment column for miscellaneous causes of ARDS. As they note, and as I indicated in the text of the article, exchange transfusions should be considered early in patients who have acute chest syndrome with rapid decline in their respiratory status. Unfortunately for the patient in this case, this therapy was not available until late in her course of illness. The management algorithm in Figure 2 of the article was meant to outline a general approach to patients with catastrophic ARDS and thus did not include specific therapies such as exchange transfusions.
The development of a tension pneumothorax can be an important cause of hemodynamic collapse and of a rapid decline in respiratory status in critically ill patients with ARDS, as Drs. Kopterides and Fremont note. The patient in question did not receive needle or tube decompression during cardiac arrest; however, at autopsy she did not have a pneumothorax noted. High pleural pressures can complicate the management of critically ill patients with ARDS by leading to increased lung collapse and hemodynamic compromise. An increase in pleural pressures can result from a pneumothorax, from rapidly expanding effusions secondary to empyema or hemothorax, or from a decrease in chest-wall compliance — as might be seen in abdominal compartment syndrome. In such patients, placement of a chest tube or abdominal decompression can dramatically improve oxygenation and hemodynamics and should be considered in the proper clinical setting.1
Benjamin D. Medoff, M.D.
Massachusetts General Hospital
Boston, MA 02114
References
Medoff BD, Harris RS, Kesselman H, Venegas J, Amato MB, Hess D. Use of recruitment maneuvers and high-positive end-expiratory pressure in a patient with acute respiratory distress syndrome. Crit Care Med 2000;28:1210-1216.