Bone marrow transplantation is sometimes difficult to practice due to lack of donors. Faced with this problem, researchers are testing another method, i.e., the transplantation of stem cells extracted from the umbilical cord of newborns. These immature cells are able to regenerate the bone marrow, and it is best when HLA (Human Leucocyte Antigen) compatibility is observed.

These immature cells decrease the graft rejection phenomenon. Moreover, their ability to proliferation is superior to adult bone marrow cells. Finally, it is a very important source because, until this, the umbilical cord was just a “waste”.

Many therapeutic advances, have been noted in children with leukemia, in the field of cord blood transplantation. In adults, this kind of transplant is also possible in some cases. Thousands have been done in the world until 2004, and even more currently.

This transplant which does not require absolute histocompatibility, allows to shorten the waiting time for transplantation, which may be very important in case of emergency (e.g., acute leukemias requiring immediate intervention), because often no compatible bone marrow samples are available readily.

Some studies are continuing in this field, because it would seem that perfect compatibility is not essential for a successful transplant, which gives great hope especially for patients with leukemia waiting for a compatible marrow.

Most diseases benefiting from the therapeutic input of stem cells are treated using bone marrow transplantation or cord blood stem cell transplant (i.e., placental blood). Transplants of Hematopoietic stem cells derived from umbilical cord blood are growing, because of the quite interesting properties characterizing this type of cells, i.e., their immunological immaturity and the advantage of providing a readily available source of cells.

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.

Robert Graves, the son of an episcopal clergyman was born in Dublin in 1797, the same year that Napoleon was leading his armies over the Alps to threaten the citizens of Vienna. He was a brilliant student and graduated with a first class medical degree in the fall of 1818 as Abraham Lincoln’s mother Nancy, lay dying of ‘milk fever’ in a small wooden hut in the untamed forestlands of Indiana. It appears that as well as being an excellent scholar, Graves was also a passionate adventurer and often told his medical friends that ‘there was always something waiting to be discovered if we only took the time to look for it’. It is therefore of no surprise that when Graves completed his medical studies he decided to further his knowledge of the Arts by travelling overland on the continent. Let us remember that this was the European mainland of the 1820’s and a lot of the continent was still considered alien to most people on these islands. The perception of the ancient palaces of Rome or maybe the evening mist settling on the sleepy canals of Venice were often images snatched from lines of poetry or from the canvases of travelling painters.

Many people of this period were highly suspicious of strangers who were often considered to be displaced soldiers wandering around after the recent battle of Waterloo. It was in a little lakeside Austrian village that Graves eventually aroused the distrust of the locals and he was arrested and held as a Prussian spy. It appears that the local authorities refused to believe that an Irishman could speak German so well and the unfortunate scholar had to stay in jail for ten days before he could get verification of his identity sent from Dublin. In 1821, Graves was travelling alone in Switzerland and found himself staying in the same hotel as the famous English painter, J.M.W. Turner. Both men struck up a friendship and they travelled and painted together for many months before finally parting company outside the Vatican in Rome. Many of Turner’s famous storm scenes come from that period of his life and often show dreary afternoon skies heavily streaked with dark cautioning thunderclouds. It is surmised by some that Robert Graves may have been the physical inspiration for one of Turners more imposing storm paintings, The Fishermen at Sea.

In this picture the inky blackness of the night is gathering fast and a sliver of a white moon shows a fishing vessel being tossed and thrown about at sea. It is known that Graves once was caught in a violent storm while on a sailing ship on the Mediterranean. The vessel was at the mercy of a raging tempest and suddenly began to take on water and sink. The bilge pumps were leaking and the crew found themselves unable to save the stricken craft. Amidst the frenzy of the thunderous storm the crew mutinied and abandoned ship by stealing the only lifeboat that was aboard the stricken vessel. Graves was incensed and refused to allow himself or his fellow passengers to be left to the peril of the seas. He ran forward and grabbed a nearby fire axe and holed the lifeboat as the mutinous sailors lowered it into the turbulent waters. He then gathered leather from the shoes of the passengers and proceeded to fix the bilge pumps. The ship was sailed into port the next morning and everybody on board was saved.

Graves returned to Dublin in 1821, in the year that Napoleon died on a small British outcrop of rock in the south Atlantic, and he became chief physician at the Meath Hospital. He continued his idea of believing that ‘everything was waiting to be discovered if you only look for it’ and before long he had described hyperthyroidism, scleroderma, pontine haemorrhages, angioneurotic oedema as well as pathological fractures and the paraneoplastic syndrome, erythromelalgia. He was also a great teacher and taught in English, which was unusual and most medical classes in the 1820’s still taught their pupils in a sort of Latin and his clinical notes were used by Trousseau (Trousseau’s sign) in Paris in 1825. When Trousseau wrote a clinical text some years later it was translated into English and used in Dublin. Graves became a good friend of William Stokes and also became passionate about the introduction of the stethoscope into clinical examination of the chest and abdomen.

They both shared ward rounds in the Meath Hospital and often spent long hours teaching medical students the signs of illness. In the midst of a busy round Graves once joked to his residents that Stoke’s’ epitaph should be ‘He fed fevers’. He died in 1853, as Guiseppe Verdi’s Il Traviata was having its premiere in La Scala in Milan. I often feel sad that people like Robert Graves are not alive today as they certainly would brighten the world of Irish medicine and enjoy the clinical benefits we now possess. Maybe somebody will remember one of our present consultants in a century and a half from now in the highly technologised society our own children will help to create.