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与多纳-兰斯泰纳抗体相关的自身免疫性溶血

Autoimmune haemolysis associated with Donath-Landsteiner antibodies.

作者信息

Sokol R J, Hewitt S, Stamps B K

出版信息

Acta Haematol. 1982;68(4):268-77. doi: 10.1159/000206992.

Abstract

The clinical pattern of haemolysis associated with Donath-Landsteiner antibodies has undergone a change over the years. In the current study 13 patients developed the acute form of the disease whilst only 1 presented with the classical picture of chronic paroxysmal cold haemoglobinuria. The acute illness typically occurred in young children with a male predominance of about 2.5:1; 10 patients were less than 5 years old. There was often a history of a preceding infection, the onset was sudden, prostration, haemoglobinuria and pallor were prominent. The patients were very ill but rapid and complete recovery usually occurred within a few days; however, 1 patient died. Treatment consisted of rest and warmth; in addition blood transfusion was needed in 7 patients. The chronic disease (which was non-syphilitic in origin) followed a benign course, warmth and avoidance of cold being all that were necessary to maintain the patient's well-being. It is felt that the general term for this disorder should be Donath-Landsteiner haemolysis rather than the traditional paroxysmal cold haemoglobinuria.

摘要

多年来,与多纳-兰德斯泰纳抗体相关的溶血临床模式发生了变化。在当前研究中,13例患者出现了该病的急性形式,而只有1例表现为慢性阵发性冷血红蛋白尿的典型症状。急性疾病通常发生在幼儿中,男性占主导,比例约为2.5:1;10例患者年龄小于5岁。常有前驱感染史,起病突然,虚脱、血红蛋白尿和面色苍白较为突出。患者病情很重,但通常在几天内迅速完全康复;然而,有1例患者死亡。治疗包括休息和保暖;此外,7例患者需要输血。慢性疾病(非梅毒起源)病程良性,保暖和避免寒冷是维持患者健康所需的全部措施。人们认为,这种病症的通用术语应为多纳-兰德斯泰纳溶血,而非传统的阵发性冷血红蛋白尿。

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