'An account of an hemorrhagic disposition existing in certain families'
'An account of an hemorrhagic disposition existing in certain families'
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DOI：
10.1046/j.1365-2516.1997.00050.x
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发表时间：
1997-04-01
期刊：
影响因子：
3.9
通讯作者：
Hilgartner, MW
中科院分区：
文献类型：
--
作者：
Hilgartner, MW
1995年10月出版的《血友病》杂志[1]上发表了莱恩先生的报告《一个粗壮的年轻女子的血》，英格拉姆博士在这篇报告上的注释使我想起了美国第一篇关于血友病的文章，作者是约翰·C.费城的奥托1803年发表在《医学知识库》上的关于某些家族中存在出血倾向的描述。在纽约出版的《医学知识库》是对美国医学、外科和科学分支出版物的回顾，由医学博士塞缪尔·莱瑟姆·米切尔和医学博士爱德华·米勒建立。Mitchill博士是哥伦比亚学院自然史、植物学、化学和药物学专业的著名医生，1796年至1817年在纽约医院工作。他是一个兴趣广泛的人，在哲学和科学方面发表了大量文章，是美国参议员，帮助制定了检疫法，改变了破布的进口关税，改善了纸张的制造并使其更便宜，通过印刷更好地帮助知识的传播。约翰·康拉德·奥托博士于1774年3月14日出生在新泽西的伍德伯里，父亲是一位德国移民医生，曾在革命军担任军官。他在普林斯顿的新泽西学院接受教育，1792年毕业，在费城师从本杰明·拉什博士（Benjamin Rush）学习医学。拉什博士是当时费城最重要的医生，也是宾夕法尼亚大学的临床医学教授。他于1796年获得医学博士学位，并在宾夕法尼亚药房工作，直到拉什博士于1813年去世，当时他被任命为宾夕法尼亚医院的STA [3]。在后来的职位上，他成为了一个有力而清晰的作家和临床教师。Shephard家族在这篇文章中介绍了Otto博士关于某些家庭的出血性倾向，这些家庭是一位名叫Smith的妇女的后代，她于1729年在新罕布什尔州的普利茅斯定居[4]。本文介绍了多种止血治疗的尝试，在出血发作的男性成员的家庭与出血的处置。最有效的治疗方法是用普通泻药量的硫酸钠连续2 ~ 3天给药。试图解释这一宝贵补救办法的运作方式可能会引起许多猜测。由于处方是在Shephard家庭拥有，由医生看到的情况是罕见的，因此，关于其行为方式的正确结论是不可能的。然而，关于出血者的遗传和他们在出血发作之间的良好健康状况进行了准确的观察。虽然这是美国医学文献中记录该疾病的第一篇论文，但来自美国不同地区（马里兰州、宾夕法尼亚州和新罕布什尔州）的四个家庭记录说明了该疾病的多个家庭起源。来自缅因州的创始人与新罕布什尔州的史密斯家族有着潜在的家庭关系，目前还不能否认。然而，这种关系似乎不太可能，因为FVIII在美国其他州更普遍。奥托博士似乎对这种出血素质产生了浓厚的兴趣，他在1805年发表了第二篇论文，详细描述了马里兰州的本杰明·宾尼（Benjamin Binny）家族发生的四例致命的遗传性出血病例。
Dr Ingram's note on Mr Lane's reportThe blood of a stout young woman'published in the October 1995 issue of Haemophilia [1] brought to mind the® rst article written in America about haemophilia by John C. Otto of Philadelphia entitledAn account of a hemorrhage disposition existing in certain families' published in the Medical Repository in 1803. The Medical Repository printed in New York was a review of American Publications on Medicine, Surgery, and The Auxillary Branches of Science established by Samuel Latham Mitchill, MD and Edward Miller, MD. Dr Mitchill was a well-known physician on the sta of Columbia College in Natural History, Botany, Chemistry and Materia Medica and physician at The New York Hospital from 1796 to 1817. He was a man of diverse interests who published extensively in Philosophy and Science, was a US Senator, helped to frame the quarantine laws, and alter the import duties on rags, to improve andrender the manufacture of paper cheaper, to better aid the di usion of knowledge by printing'. Dr John Conrad Otto was born in Woodbury, New Jersey, 14 March, 1774, the son of an immigrant German physician who was an o cer in the Revolutionary Army [2]. He was educated at the College of New Jersey in Princeton, graduating in 1792, and studied medicine in Philadelphia under Dr Benjamin Rush, the foremost physician in the city at that time and professor of clinical medicine at the University of Pennsylvania. He obtained his MD degree in 1796 and worked in the Pennsylvania Dispensary until Dr Rush's death in 1813 when he was appointed to the sta of the Pennsylvania Hospital [3]. In this later position, he became known as a forceful and clear writer and clinical teacher. The Shephard family presented Dr Otto in this article pertaining to a haemorrhagic disposition in certain families are the descendants of a woman named Smith who settled in Plymouth, New Hampshire, in the 1729s [4]. The paper presents multiple therapeutic attempts for haemostasis during bleeding episodes in those male members of the families with the haemorrhagic disposition. The most e ective therapeutic measure was to sulphate of soda given in theordinary purging doses administered two or three days in succession. An attempt to explain the mode of operation of this valuable remedy might give birth to much speculation. As the prescription was in the possession of the Shephard family, the cases that were seen by a physician were rare and, therefore, correct conclusions as to its manner of acting could not be made'. However, accurate observations were made concerning the genetic inheritance of thebleeders', and their good health in between their haemorrhagic episodes. Although this is the® rst paper in the US medical literature documenting the disease, the four families documented from di erent parts of the country, states of Maryland, Pennsylvania and New Hampshire, illustrates a multiple family origin of the disease. The potential family relationship of the founder from Maine whose family had FIX de® ciency to the Smith family in New Hampshire cannot be negated at the present time. However, a relationship would seem unlikely as FVIII is more prevalent in other states in this country. Dr Otto seems to have developed a keen interest in this bleeding diathesis as he published a second paper in 1805 in which he detailed the history of four fatal cases of hereditary haemorrhage occurring in the family of Benjamin Binny of Maryland.