A fatal case of acute chest syndrome in a patient with undiagnosed sickle cell trait

We report a fatal case of acute chest syndrome in an African-American male. The patient was hospitalized for respiratory distress, fevers, and pulmonary infiltrates after working in an attic space on a summer day. An extensive work-up failed to reveal an etiology for his respiratory failure. He died of respiratory failure two weeks after admission. Autopsy findings suggested the patient had clinically unrecognized sickle cell trait exacerbated by working in the heat, causing acute chest syndrome with a fatal outcome. Clinicians should consider acute chest syndrome and sickle cell trait in the differential diagnosis of patients with unexplained respiratory failure and pulmonary infiltrates.     

SARS和疑似SARS患者胸部影像学变化的临床研究

目的总结严重急性呼吸综合征(SARS)患者胸部影像学动态变化及其与治疗的关系.方法分析7例SARS和疑似SARS患者X线胸片显示肺部病变的出现时间、部位、范围等影像学特点及其与糖皮质激素和白蛋白加速尿治疗的关系.结果全部患者发病当时X线胸片就显示有肺部病变.6例患者累及双肺、多叶.病灶呈进行性发展,高峰出现在发病第8～10天.4例患者应用糖皮质激素[甲基泼尼松龙,1.5～3 mg*(kg*d)-1]治疗,但不能减轻肺水肿.5例患者应用白蛋白加速尿治疗后,渗出病灶吸收明显.结论SARS患者X线胸片渗出病灶出现早、进展快、范围广.糖皮质激素对X线胸片改变无干预作用.白蛋白加速尿可以明显减轻肺水肿.     

Biochemical diagnosis in sickle cell disease

Seventy-four sicklers with bone pain had blood taken for biochemical analysis of the haemoglobins carried. Sixty-five patients with sickle cell disease diagnosed as Hb S/S disease, five patients diagnosed as Hb A/S and four patients with three haemoglobin bands corresponding to Hb's A, S and C were used in this quantitative reassessment on cellulose acetate. Haemoglobin A2 level was determined in all cases and alkali resistant haemoglobin level in twenty-nine cases. The significance of raised Hb A2 levels in some of these cases is discussed with relation to the possible existence of Hb S/beta-Thalassaemia in the sickle cell disease patients examined.     

Life threatening sickle chest syndrome treated with extracorporeal membrane oxygenation

A young West Indian woman with established sickle cell disease developed a severe episode of sickle chest syndrome. Conventional treatment including exchange transfusions and mechanical ventilation was to no avail, and an infusion of epoprostenol also failed to halt her worsening condition. When her arterial oxygen tension (PaO2) had fallen to 6.5 kPa (49 mm Hg) extracorporeal membrane oxygenation was instituted. Within two days her PaO2 was greatly improved (maximum 11.6 kPa; 87 mmHg), and by the sixth day pulmonary vascular resistance was reduced and angiography showed reperfusion of many vessels. The patient recovered and six months later showed a transfer factor close to the predicted range and normal spirometric values. Extracorporeal membrane oxygenation should be considered for severe sickle chest syndrome when conventional methods of artificial ventilation fail.     

高频通治疗胸部创伤合并急性呼吸窘迫综合征的临床研究

目的探讨高频通气(high frequency ventilation,HFV)治疗胸部创伤合并急性呼吸窘迫综合征(acute respiratory distress syndrome,ARDS)的临床应用价值.方法对该院1999年6月以来胸部创伤合并ARDS的患者随机分为治疗组与对照组,治疗组30例应用HFV,对照组26例应用常频机械通气(conventionalmechnical ventilation,CMV),通过监测氧合指数OI(PaO2/FiO2)、动脉血氧分压(PaO2)、二氧化碳分压(PaCO2)等参数,评估HFV治疗重症胸部创伤合并ARIDS的治疗效果及临床应用价值.结果治疗组应用HFV治疗前后比较,OI、PaO,在应用HFV 4 h后均显著提高(P＜0.01).两组患者对比,OI在治疗前及治疗2h后差异无显著性(P＞0.05),在治疗4、8、16、24 h及48 h后差异有显著性(P＜0,05);PaCO2在治疗前及治疗2 h后差异无显著性(P＞0.05),在治疗4 h后差异有显著性(P＜0.05)、8 h后均有显著差异(P＜0.01).HFV组病死率为40.0%.结论HFV能够促进胸部创伤合并ARDS患者的肺脏通气,有效改善氧合、纠正低氧血症;同CMV对比,HFV改善氧合、提高OI的疗效更加显著,在应用HFV 4 h后可引起CO2的轻度潴留;HFV有助于胸部创伤合并ARDS患者的急救,值得临床推广应用、深入研究.     

高频通气治疗胸部创伤合并急性呼吸窘迫综合征的临床研究

目的 探讨高频通气（high frequency ventilation，HFV）治疗胸部创伤合并急性呼吸窘迫综合征（acute respiratory distress syndrome，ARDS）的临床应用价值。方法 对该院1999年6月以来胸部创伤合并ARDS的患者随机分为治疗组与对照组，治疗组30例应用HFV，对照组26例应用常频机械通气（conventional mechanical ventilation，CMV），通过监测氧合指数OI（PaO2／FiO2）、动脉血氧分压（PaO2）、二氧化碳分压（PaCO2）等参数，评估HFV治疗重症胸部创伤合并ARDS的治疗效果及临床应用价值。结果 治疗组应用HFV治疗前后比较，OI、PaO，在应用HFv4h后均显著提高（P〈0．01）。两组患者对比，OI在治疗前及治疗2h后差异无显著性（P〉0．05），在治疗4、8、16、24h及48h后差异有显著性（P〈0．05）；PaCO2在治疗前及治疗2h后差异无显著性（P〉0.05），在治疗4h后差异有显著性（P〈0.05）、8h后均有显著差异（P〈0.01）。HFV组病死率为40．0％。结论 HFV能够促进胸部创伤合并AR33S患者的肺脏通气，有效改善氧合、纠正低氧血症：同CMV对比，HFV改善氧合、提高OI的疗效更加显著，在应用HFV 4h后可引起CO2的轻度潴留；HFV有助于胸部创伤合并ARDS患者的急救，值得临床推广应用、深入研究。     

Pulmonary manifestations of sickle cell disease

Pulmonary complications account for significant morbidity and mortality in patients with sickle cell disease. Clinical lung involvement manifests in two major forms: the acute chest syndrome and sickle cell chronic lung disease. Acute chest syndrome is characterised by fever, chest pain, and appearance of a new infiltrate on chest radiograph. Sickle cell chronic lung disease, on the other hand, manifests as radiographic interstitial abnormalities, impaired pulmonary function, and, in its most severe form, by the evidence of pulmonary hypertension. Progress has been made in understanding the pathophysiology and management of these complications. In this review the current knowledge of the mechanism, diagnosis, and treatment of pulmonary complications of sickle cell disease are discussed.