Volume 2

Treasury of human inheritance / edited by Karl Pearson, R.A. Fisher, L.S. Penrose.

Date:
1909-
Catalogue details

Licence: Attribution-NonCommercial 4.0 International (CC BY-NC 4.0)

Credit: Treasury of human inheritance / edited by Karl Pearson, R.A. Fisher, L.S. Penrose. Source: Wellcome Collection.

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    (Pedigree No. 440). In this way he was able to give a very complete and accurate account of haemophilia which he raised to its appointed rank in the nosological system. From the data before him he was able to assert that males alone are the subjects of haemophilia, and that the disease is transmitted by normal females through their marriages with normal males. “ An ihnen (the women) selbst und iiberhaupt an einer weiblichen Person jener Familien ausserte sich eine solche Neigung (to haemorrhage) niemals.” The affection of the male sex, and the trans mission of the disease through normal unaffected females, is frequently described in the literature as “ Nasse’s law,” although prior to Nasse all the classical writers on haemophilia had emphasised this mode of transmission. The publication of Nasse’s paper came about the time when the teaching of J. L. Schônlein was revolutionising German medicine, and from it dates the onset of the flood of publications on haemo philia which has continued down to the present day. Among the more interesting of the publications of succeeding years may be mentioned those of Elsaesser 881 (1824), Davis'* 1 (1826), Chelius 1481 (1827), Coates' 61 ’ (1828), Rieken' 601 (1829), Hughes' 66 ' 71 ’ (1831), Escherich <S8 ’ (1835), Yieli 1164 ’ (1846), Wachsmuth' 181) (1849), and Lange' 193 ’ (1851). In addition appeared a large number of communications which, a study shows, contained nothing new or were of little value. The concept of the disease, based upon such communications, tended to become more and more obscured, and drifted farther and farther from the clear statements of the original accounts. Dr Ludwig Grandidier, a Brunnenarzt at Nenndorf and practising in Cassel, made the collection of these data the main interest of his life, and for more than forty years contributed collective accounts on the subject of haemophilia, his chief work being a monograph Die Haemophilie oder die BluterJcrankheit, Leipzig, 1855 (2nd edit. 1877). It cannot be denied that without Grandidier’s labours a large mass of literature would have been lost in oblivion. While this, in our opinion, would have been desirable in a very large number of instances, it must be admitted that the balance of his data is of value. Nevertheless, it must be stated that his figures and statistics, which have been freely copied by all subsequent writers without criticism, can only be described as largely erroneous. A personal study of practically the whole of the haemophilie literature has led us to the belief that in many instances he had not seen the original papers, his information being derived from abstracts in journals and year-books, and suffering, no doubt, in transcription. Subsequently to the appearance of Grandidier’s monograph the production of material, good and bad, continued. In 1872 Wickham Legg' 331 ’, the next notable writer, published his scholarly monograph. He appears to have been the first to attempt anything like a critical survey of the data published up to this time. His monograph was followed by Immermann’s <401) exhaustive article in Ziemssen’s Cyclopaedia in 1876, this being one of the most authoritative accounts. The second edition of Grandidier’s book, published in 1877 shortly before his death, may be taken as the last of the classical works on the disease. As has been stated above, Otto’s paper (1803) was the origin of our know T - ledge of haemophilia. It remains however to notice the cases which have been