Louis-Gustave A, Louis-Gustave R
Arch Mal Coeur Vaiss. 1977 Feb;70(2):135-40.
Sickle cell disease is the commonest of the haemoglobinopathies. The pathophysiology consists on the one hand of chronic haemolytic anaemia whose course is punctuated by acute crises with pain, fever and severe haemolysis, and on the other of a tendency to microthrombotic accidents. Of all the very varied clinical features of the disease, cardiac signs are both common and variable. The authors report a series of 92 cases, and compare their findings with those in the literature.
镰状细胞病是最常见的血红蛋白病。其病理生理学一方面表现为慢性溶血性贫血,病程中伴有疼痛、发热和严重溶血的急性发作,另一方面表现为微血栓形成事件的倾向。在该疾病所有非常多样的临床特征中,心脏体征既常见又多变。作者报告了一系列92例病例,并将他们的发现与文献中的发现进行了比较。