A Familial Hemorrhagic Condition Associated With Prolongation of the Bleeding Time

Minot, George R.

doi:10.1097/00000441-192803000-00001

Cited by 56 publications

(16 citation statements)

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“…Further investigation suggested that the bleeding was due to a platelet abnormality (von Willebrand and Jurgens, 1933) although later (Blackburn, 1961), capillary abnormalities were described. A similar clinical disorder had been noted in America by Minot (1928). Von Willebrand's disease had seemed quite distinct from haemophilia because ofthe autosomal, dominant inheritance, both sexes being equally affected and transmission being predominantly through affected persons, and because of the long bleeding time which was seldom found in classical haemophilia.…”

Section: The Royal Haemophiliamentioning

confidence: 54%

The history of haemophilia.

Ingram¹

1976

J Clin Pathol

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Section: The Royal Haemophiliamentioning

confidence: 54%

The history of haemophilia.

Ingram¹

1976

J Clin Pathol

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“…In 1926, von Willebrand described a hereditary bleeding disorder affecting both sexes among the inhabitants of the Aland Islands in the mouth of the Gulf of Bothnia, in which also the bleeding time was prolonged[70]. Further investigation suggested that the bleeding was due to a platelet abnormality[71] although later, capillary abnormalities were described[72].A similar clinical disorder had been noted in America byMinot[73].von Willebrand's disease had seemed quite distinct from haemophilia because of the autosomal dominant inheritance, both sexes being equally affected and transmission being predominantly through affected persons, and because of the long bleeding time which was seldom found in classical haemophilia. It was therefore with great surprise, in the 1950s, that a reduction in factor VIII was found to occur also in von Willebrand's disease [74-77].…”

mentioning

confidence: 80%

The history of haemophilia*,†

Ingram¹

1997

Haemophilia

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The history of haemophilia shows the human mind attempting to define and encompass a mysterious yet fascinating phenomenon; and also the human heart responding to the challenge of repeated adversity. These responses have not remained isolated but have frequently interacted. As doctors have studied haemophilia in order to try to do more for their patients, their investigations have notably advanced our general understanding of haemostasis; and as haemophiliacs have benefited from improvements in their treatment, they have been eager to help by giving blood samples for experiments as well as in raising funds for research. The mutation giving rise to haemophilia is probably of great age because it occurs in at least three Orders of placental mammals, the Perissodactyla (Ungulata) the Fissipedia (Carnivora), and the Anthropoidea (Primates); for haemophilia has been described in the horse [1] and in nine breeds of dog [2-4] as well as in various races of man. The Orders to which these groups belong may have been distinct from the end of the Cretaceous, say 65 million years ago; and the mutation has probably recurred independently many times, in all three, since it must be nearly lethal in the wild state. The mutation rate in man has been estimated at about 1-4 χ ΙΟ" 5 [5]. Early references The earliest written references to what may have been human haemophilia are attributed to Jewish writings of the 2nd century AD. A ruling of Rabbi Judah the Patriarch exempts a woman's third son from being circumcized if his two elder brothers had died of bleeding after circumcision [6, 7]; and Rabbi Simon ben Gamaliel forbade a boy to be circumcized because the sons of his mother's three elder sisters had died after circumcision (Rothschild 1882 in [8], [9]). There are subsequent similar Rabbinic references; and these features of fatal bleeding after minor surgery in brothers or in maternally related boy cousins are characteristic of the "Opening lecture to the Third European Regional Congress of the World Federation of Haemophilia, London, 17 April 1976, marking the Silver Jubilee of the British Haemophilia Society. 'kind permission of the British Medical Association. disorder we call haemophilia. In the 10th century, males of a certain village who bled to death from trivial wounds were described by Khalaf ibn Abbas, known to us as Albucasis. In the 12th century, Maimonides applied the Rabbinic ruling to the sons of a woman who was twice married [9]: and down the years there are other scattered records of bleeding disorders more or less closely agreeing with the clinical picture we know. The first descriptions which we may say probably do refer to haemophilia are from the end of the 18th century. Although William Hewson, despite his interest in abnormalities of the blood, does not appear to describe the disorder, the anonymous obituarist of Isaac Zoll, writing in 1791 (in [10]), Consbruch in 1793 and 1810 [11], Rave in 1796 [12], and Otto in 1803 [13] all described families in which males suffered abnormally prolonged post-traumat...

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“…Von Willebrand gave detailed descriptions of the disease in several publications [1,3,4] and although 70 years old, this description is still relevant for the disease. Two years later, in 1928, the clinical picture was described independently by four Americans, including Minot [5]. In 1933 von Willebrand began to collaborate with the German physician Rudolf Jürgens.…”

Section: The History Of Von Willebrand Disease and Scientific Visits mentioning

confidence: 99%

von Willebrand disease: an update in the Åland islandsSummary of a Nordic Symposium on von Willebrand disease, 24–25 September 1998, Mariehamn, Åland

1999

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In the past decades von Willebrand disease (vWD) has, in several respects, fallen into the shadow of classical haemophilia due to all problems that have faced those dealing with congenital bleeding disorders, not least regarding blood-borne diseases. The time has come to revisit and refocus on vWD, probably the most common bleeding disorder. Accordingly, a number of Nordic physicians and scientists working in this field organized a meeting on the ferry boat from Stockholm to Mariehamn, in the Aland islands, the home of the index families with this disease, as described by Erik von Willebrand in 1926. The objective of the meeting was to make a comprehensive survey of vWD as seen from the situation in the Nordic countries. In order to achieve all goals some scientists of note from countries outside the Nordic area were invited as well. This paper gives a summary of the symposium, mainly based on the separate papers published in this issue.

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A Familial Hemorrhagic Condition Associated With Prolongation of the Bleeding Time

Cited by 56 publications

References 0 publications

The history of haemophilia.

The history of haemophilia.

The history of haemophilia*,†

von Willebrand disease: an update in the Åland islandsSummary of a Nordic Symposium on von Willebrand disease, 24–25 September 1998, Mariehamn, Åland

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