BABINGTON'S DISEASE
Babington's Disease is an autosomal dominant vascular dysplasia leading to telangiectases and arteriovenous malformations of skin, mucosa, and viscera. Epistaxis and gastrointestinal bleeding are frequent complications. Visceral involvement includes that of the lung, liver, and brain. It is commonly known today as hereditary hemorrhagic telangiectasia (HHT Syndrome) or Osler-Rendu-Weber disease.
History of the name of the disease.
The syndrome was first described in 1864 by B.G. Babington who reported a family who had a tendency toward nosebleeds and gastrointestinal bleeding. A comprehensive report of the syndrome was made by Rendu in 1896. Two other physicians, Sir William Osier and Professor Weber were responsible for recognising the syndrome (thus the name Osler-Weber-Rendu Syndrome). The only previous report that Osler could find in 1901 was that of Rendu dated 1896. Because of his prominence as a physician and author of a textbook, Osler became associated with the disease. Weber described cases later as part of a life-long interest in angiomas and other vascular lesions. The name, although not chronologically accurate, is perhaps justified by the contribution to the nosology of the entity. In the interests of accuracy, my personal pride in the origins of Anglo-Irish medicine and the outstanding contribution of Guy's past persona towards progress, I prefer for the interests of this column to remember all the disease after the physician who discovered it and whom it was first called after.
B.G Babington was born in 1794, the same year that Antoine Lavoisier, the father of modern chemistry was executed by guillotine by the leaders of the French Revolution. He was first educated at Charterhouse, later joined the Royal Navy as a midshipman. After he left the Navy, he joined the Indian Service and moved to the subcontinent where he became ill with cholera and had to return home. He studied medicine at Guy's Hospital where his father William, was already a well-known physician and pharmacological lecturer. Of passing interest is the fact that on Sir Astley Cooper's recommendation, the next lecturer was William Allen, a man obsessed with the purity and quality of the supplies he used for teaching the chemical aspects of medicine. He later became founder of the drug firm of Allen and Hanburys and one of his first products was improved sources of quinine-containing Peruvian bark used to treat intractable fevers.
Babington became interested in clotting problems and suggested in the 1820's that fibrin had to be formed in the blood from a soluble precursor. In 1820, as Danish physicist Hans Oersted was busy discovering that a wire conducting an electric current produces a magnetic field, Babington was occupied inventing the glottiscope, an instrument to examine the larynx. He is remembered as the first person to perform indirect laryngoscpopy. In 1834, he was appointed physician at Guy's, ahead of Thomas Hodgkin's (Hodgkins Disease). Although it was widely reported at the time that Hodgkins was disfavoured because of his liberal tendencies towards immigrants and native aboriginals there is certainly another explanation. Babington's father was a physician in the hospital and his sister was married to Richard Bright, (Bright's Disease) the first physician to describe nephritis. In the end it didn't matter, as it was a period of acrimony between Guy's and St. Thomas's resulting in the transfer of many famous people including Sir Astley Cooper and in 1854, Babington became frustrated and resigned his post.
He was annoyed that medical students were not allowed to have full access to the patients in the hospital. He died in 1866, from the complications of renal disease. It was the same year that the Fenian Brotherhood, an organization of Irish-Americans, planned to capture Canada and hold it until Britain agreed to free Ireland.
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