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阵发性夜间血红蛋白尿于1882年由保罗·施特鲁宾首次描述:临床与基础研究合作的一个实例。

Paroxysmal nocturnal hemoglobinuria first described in 1882 by Paul Strübing: an example of cooperation between clinical and basic research.

作者信息

Wilmanns J C

出版信息

Blut. 1982 Dec;45(6):367-73. doi: 10.1007/BF00320547.

DOI:10.1007/BF00320547
PMID:6753969
Abstract

The 100th anniversary of the first description of paroxysmal nocturnal hemoglobinuria by Paul Strübing presents an opportunity to analyze the premises valid for the description of this disease in addition to an attempt at an extensive pathophysiological analysis. Strübing's two papers of 1882 were way ahead of his time, when pathophysiology was just at its beginning, particularly considering the fact that neither Marchiafava, who is still commonly credited wit the first description of this disease (1911) and its recognition as a clinical entity (1928), nor his student Micheli analyzed the PNH syndrome in pathophysiological terms as carefully as Strübing. Both of the former names were given to the disease, which is generally referred to as the Marchiafava-Micheli Anemia. William Crosby, who in 1951 in a historical review of PHN first pointed out the pioneering achievement of Strübing, suggested that it was mainly due to the lack of the right "intellectual climate" at the time that so little attention was paid to his work. Still another important aspect of the early history of PNH will be described in the present paper. The analysis of Strübing's publications leads to the conclusion that he was only able to make his important contribution to medical science because he not only had the appropriate clinical setting but also the scientific backup of the famous physiologist Leonhard Landois and his institute at the University of Greifswald, which is an excellent example of scientific progress through cooperation between a clinician and a research scientist.

摘要

保罗·施特鲁宾首次描述阵发性夜间血红蛋白尿一百周年,这为分析该疾病描述所依据的前提提供了契机,同时也尝试进行广泛的病理生理分析。施特鲁宾1882年的两篇论文远远领先于他所处的时代,当时病理生理学才刚刚起步,尤其要考虑到,尽管马尔恰法瓦(人们仍普遍认为是他于1911年首次描述了这种疾病,并于1928年将其确认为一种临床实体)及其学生米凯利都未像施特鲁宾那样仔细地从病理生理学角度分析阵发性夜间血红蛋白尿综合征。前两人的名字都被用于命名这种疾病,该病通常被称为马尔恰法瓦 - 米凯利贫血。威廉·克罗斯比在1951年对阵发性夜间血红蛋白尿的历史回顾中首次指出了施特鲁宾的开创性成就,他认为当时很少有人关注施特鲁宾的工作,主要是因为缺乏合适的“学术氛围”。本文还将描述阵发性夜间血红蛋白尿早期历史的另一个重要方面。对施特鲁宾出版物的分析得出的结论是,他之所以能够对医学做出重要贡献,是因为他不仅有合适的临床环境,还得到了著名生理学家莱奥纳德·兰多伊斯及其在格赖夫斯瓦尔德大学的研究所的科学支持,这是临床医生与科研人员合作推动科学进步的一个绝佳范例。

相似文献

1
Paroxysmal nocturnal hemoglobinuria first described in 1882 by Paul Strübing: an example of cooperation between clinical and basic research.阵发性夜间血红蛋白尿于1882年由保罗·施特鲁宾首次描述:临床与基础研究合作的一个实例。
Blut. 1982 Dec;45(6):367-73. doi: 10.1007/BF00320547.
2
[Paul Strübing, the discoverer of paroxysmal nocturnal hemoglobinuria (Strübing-Marchiafava anemia)].[保罗·施特鲁宾,阵发性夜间血红蛋白尿症(施特鲁宾-马尔恰法瓦贫血症)的发现者]
Dtsch Med Wochenschr. 1959 Feb 20;84(8):335-6. doi: 10.1055/s-0028-1113605.
3
[History of paroxysmal nocturnal hemoglobinuria].
Acta Medica (Hradec Kralove) Suppl. 2002;45(1):11-7.
4
Paroxysmal nocturnal hemoglobinuria: an historical overview.
Hematology Am Soc Hematol Educ Program. 2008:93-103. doi: 10.1182/asheducation-2008.1.93.
5
[Paroxysmal nocturnal hemoglobinuria. Strübing-Marchiafava-Micheli anemia].
Med Welt. 1966 Sep 24;39:2043-50.
6
[Carotid occlusion in paroxysmal nocturnal hemoglobinuria (Strübing-Marchiafava-Micheli)].
Nervenarzt. 1982 Oct;53(10):576-9.
7
Evolution of clinical understanding: paroxysmal nocturnal hemoglobinuria as a paradigm.临床认识的演变:以阵发性夜间血红蛋白尿为例
Am J Hematol. 1993 Jan;42(1):122-6. doi: 10.1002/ajh.2830420124.
8
Paroxysmal nocturnal hemoglobinuria (PNH): an historical perspective.
Hematology Am Soc Hematol Educ Program. 2008:92. doi: 10.1182/asheducation-2008.1.92.
9
[Permanent intrabasal hemolysis (Strübing-Marchiafava disease)].
Klin Med (Mosk). 1972 Nov;50(11):5-9.
10
Chronic hemolytic anemia with paroxysmal nocturnal hemoglobinuria (Marchiafava-Micheli syndrome).伴有阵发性夜间血红蛋白尿的慢性溶血性贫血(马尔恰法瓦-米凯利综合征)。
Am J Med. 1950 Sep;9(3):414-21. doi: 10.1016/0002-9343(50)90475-x.

本文引用的文献

1
Paroxysmal nocturnal hemoglobinuria; a classic description by Paul Strübling in 1882, and a bibliography of the disease.阵发性夜间血红蛋白尿;1882年保罗·施特布林的经典描述以及该疾病的文献目录。
Blood. 1951 Mar;6(3):270-84.
2
[Paul Strübing, the discoverer of paroxysmal nocturnal hemoglobinuria (Strübing-Marchiafava anemia)].[保罗·施特鲁宾,阵发性夜间血红蛋白尿症(施特鲁宾-马尔恰法瓦贫血症)的发现者]
Dtsch Med Wochenschr. 1959 Feb 20;84(8):335-6. doi: 10.1055/s-0028-1113605.
3
[Paroxysmal nocturnal hemoglobinuria. Strübing-Marchiafava-Micheli anemia].
Med Welt. 1966 Sep 24;39:2043-50.