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The diagnosis is… Mysteries of the disease Mycobacterium leprae , the organism responsible for leprosy, is a relative of M. A bacillus is a rod-shaped bacterium. Scientists theorize that the leprosy bacillus enters the body through a break in the skin or through the mucous membranes of the nose. The disease can be transmitted from person to person by prolonged close contact, but even today scientists are uncertain of the exact mechanism. Much about leprosy remains mysterious, at least in part because the bacillus has never been grown in tissue culture and because the only tools for studying its transmission have been a limited number of animal models, chiefly armadillos and mice. The bacillus can grow in the footpads of mice, which provides an important mechanism for bacillus cultivation for experimental study. The geographic distribution of the leprosy bacillus is another mystery. Some scientists suspect that the organism exists in the soil in many parts of the world, but, again, because it cannot be grown in a laboratory culture , the only evidence of its presence in a given region is the appearance of the disease itself in humans or other animals that are susceptible to it. Besides humans, the only animals known to develop leprosy in nature are New World armadillos and African primates. A study of wild nine-banded armadillos Dasypus novemcinctus and people living with leprosy in the southern United States found that the armadillos and humans were infected with a nearly identical strain of the leprosy bacillus. A subset of the infected individuals recalled having been in direct contact with armadillos. The findings suggested that, in that region of the world, leprosy may be a zoonotic disease an illness transmitted between humans and other vertebrates. Given that the organism that causes leprosy is widely distributed and the disease is contagious , it seems logical to ask why leprosy is not a great deal more prevalent than it is. The answer is that the infection apparently is quite difficult to contract. The vast majority of people 95 percent or so simply are not susceptible to the bacillus and, even under repeated exposure, will never develop the disease. Among the few individuals who do contract the disease, in most cases it will be self-limiting and disappear before any symptoms become evident. Even in cases where early symptoms of leprosy develop, most patients will self-heal. This rather unusual pattern of infection, along with a three- to five-year incubation period the time that elapses between contact with the bacillus and the onset of symptoms , makes the epidemiology of leprosy particularly difficult to study. Transmission In the many centuries since leprosy was first described, a number of theories have been proposed to explain how the bacillus is transmitted. In the 19th century leprosy was believed to be a hereditary ailment. This made sense, as it frequently occurred in households among individuals who were members of a single family. In , however, G. Armauer Hansen, a physician working in a leprosy hospital in Bergen, Norway, discovered the leprosy bacillus in a sample of tissue from one of his patients. Hansen was able to identify the organism under the microscope because its propensity to collect iron caused it to appear brownish in colour compared with the tissue itself. His discovery demonstrated that leprosy is an infectious disease propagated by a specific microorganism. The route of transmission of leprosy remained a matter of debate. The prevailing opinion for many years was that the illness spread via prolonged skin-to-skin contact. Then the theory of respiratory transmission became popular; it posited that the bacillus entered the human body through the lining of the nose. For a time scientists even entertained the possibility of transmission by insect bites. In the late 20th century, experiments with a mouse model of the disease showed that transmission is indeed possible through the intact lining of the nose and through breaks in the skin but not via the mouth, lungs, or digestive tract or through unbroken skin. And, although leprosy can be produced in mice by exposing them to the bacillus, the disease cannot be transmitted from an infected to an uninfected mouse. In one type of reaction, immune cells crowd into the area in an attempt to seal off the bacterium, and in these areas very few bacilli can be found. This gives rise to a form of leprosy known as tuberculoid leprosy because of the hard nodules, or tubercles, that form in the skin. The intense cellular reaction involves all of the thicknesses of the skin and the tissues under it, the sweat glands , the hair follicles, and the nerve fibres that end in the skin. The cellular reaction continues to spread into the main trunk of the involved nerve, so that nerve impulses cannot be transmitted, which thereby causes loss of sensation and decreased circulation in the affected part. This is most commonly seen in the forearm or lower leg, and it leads to claw hand and gross deformity of the foot. Paralysis of muscles of the face, eye, and neck may also occur. The patient is unable to feel pain , and minor injuries remain unnoticed. Large eroding ulcers can form, causing loss of fingers and toes; sometimes the condition of the limb is so bad that amputation is necessary. It is ironic that this form of leprosy occurs in people whose tissues resist the disease, for the intense cellular response is a reaction of resistance—successful insofar as it prevents local multiplication of the leprosy bacillus and its spread throughout the body but unsuccessful in that it destroys vital tissues in the invaded areas. The disease spreads via the nerves but does not adhere to them as in the tuberculoid form. It spreads very often to the skin of the face, where it causes thickening and corrugation of the skin and a typical leonine appearance. Soft nodules appear on the ears, nose, and cheeks and sometimes erode into discharging sores. The nose often is teeming with bacilli, and this sometimes leads to destruction of the septum of the nose and the palate. The progress of leprosy is slow. It may be years before a child infected by a parent shows the first sign of disease, frequently a vague, scarcely noticed patch on the skin. Often the child has grown to an adult before the disease is recognized. Persons with leprosy have occasional bouts of fever , but the course of the disease is mainly one of increasing disability and disfigurement, slowly progressing through the years even though it does not usually cut life short. Therapy The current treatment of leprosy is extremely effective, halting the progress of the disease. The bacilli can be killed rapidly, and multidrug therapy—the use of two or more antileprosy drugs in combination—prevents the development of drug-resistant strains. Indeed, multidrug therapy—a practice widely adopted in the treatment of tuberculosis and AIDS —was first proposed after scientists observed that some cases of leprosy were becoming resistant to sulfones, the earliest class of antileprosy drugs. A multidrug regimen developed by WHO is the current standard of treatment. For patients with localized forms of leprosy and relatively few leprosy bacilli in their bodies, two drugs, dapsone and rifampicin , are given for a total of six months. For patients with more widespread disease and relatively large numbers of bacilli, three drugs—dapsone, clofazimine, and rifampicin—are given for 24 months. Most patients are able to tolerate the drugs well, but a few experience undesirable side effects or even exacerbations of the symptoms. Relapses, in general, are rare, occurring in fewer than 1 per 1, treated patients. Occasionally the infection persists despite continued therapy. Killing the bacillus has no effect on body tissues that have already been damaged or destroyed. Up to a point, nerve function can be restored by antileprosy drugs. Once the disease has progressed beyond that point, the loss of function is permanent. A small percentage of newly diagnosed leprosy patients have visible deformity or damage to their hands or feet or impaired vision. These disabilities can interfere with their ability to earn a living and otherwise lead a normal life. If the damage is unsightly, and it frequently is, then they also must cope with loss of social acceptance. History People tend to think of leprosy as a tropical disease because most cases today are found in less-developed countries, which are mainly in the tropics. This has not always been the case. In ce an estimated 19, leprosy hospitals existed all over Europe. The disease is much older than that, however, and it is believed to have originated on the Indian subcontinent. Indeed, the most ancient evidence of leprosy comes from a 4,year-old human skeleton uncovered in India in The skeleton was found to have erosion patterns similar to those found in skeletons of lepers in Europe dating to the Middle Ages. Thus, there is evidence that leprosy existed in India by bce, and this coincides with what is suspected to be the first textual reference to leprosy—in an ancient Sanskrit sacred work known as the Atharvaveda. There are many difficulties in interpreting ancient medical writings, and the descriptive terms used by ancient authors for clinical conditions are often misleading. An illness that fits the description of leprosy almost certainly appears in the Sushruta-samhita, a medical work from India that dates to about bce. A similar ailment is described in a Chinese medical text from bce. Tradition has it that members of the army of Alexander the Great contracted the illness when they invaded India in the 4th century bce and carried it into the Middle East and then throughout the eastern Mediterranean upon their return home. It is also traditionally believed that Roman soldiers in the army of Pompey took the disease from Egypt to Italy in the 1st century bce and that Roman legionnaires later took the disease as far as the British Isles. On the other hand, genetic analysis of the leprosy bacillus indicates that Mycobacterium leprae may have evolved some , years ago in eastern Africa or southwestern Asia. From there it seems to have migrated eastward and westward, developing one distinct subtype in Asia and another subtype in Europe and North Africa. Leprosy appears to have been introduced by Europeans or North Africans into western Africa , where the bacillus evolved yet another distinct subtype. The disease was brought to the Americas by European colonists and western African slaves. So acute was the suffering of those infected by leprosy that the disease was thought to be highly contagious. Persons with leprosy not wealthy enough to live at home in isolation were segregated in what came to be called lazarets or leprosaria. Outside these hospices they were feared and ostracized, frequently condemned to wander the roads wearing signs and ringing bells to warn healthy people of their approach. Like many diseases, leprosy was considered to be a form of divine punishment for worldly sins, and the outward signs of the disease were taken as proof that leprosy victims were utterly embroiled in sin. Rather abruptly and for unknown reasons, the incidence of leprosy began to decline in Europe—with the exception of Scandinavia—between and In Norway the disease persisted until the 20th century but then disappeared. It was taken to what is now Louisiana , in the southern United States, by French Canadians Acadians who were expelled from Canada in Another immigrant group known to include people with leprosy moved to the United States from Scandinavia, mainly Norway, in the middle of the 19th century and settled principally in Minnesota. The disease was transmitted and has persisted in Louisiana, where occasional new cases appear even today among people of Acadian descent, but the disease was not transmitted in Minnesota and has completely disappeared there. It was in Norway that Hansen identified the leprosy bacillus in At that time leprosy affected about 2. This great discovery made possible the modern era of treating the disease itself, rather than merely containing it or treating the symptoms. Early in the 20th century, the Carville home was transferred to U. Leprosy treatment centre at Kalaupapa, on the island of Molokai, Hawaii. Werner Stoy from Camera Hawaii For centuries an oil derived from the seeds of the chaulmoogra tree genus Hydnocarpus had been used to treat leprosy and other skin conditions in India and China. In Frederic John Mouat, an English doctor working in Calcutta Kolkata , reported the possible utility of chaulmoogra oil in treating leprosy, and by the s the oil or derivatives of the oil were the principal medication available. However, the drug was not effective when applied locally to affected areas, produced nausea when taken orally, and caused pain upon injection. Its real value was never fully accepted by authorities, and it was finally abandoned upon introduction of the sulfones, the first truly effective leprosy drug, in the s. In doctors at Carville began to test a derivative of the compound , called promin, on patients. The most effective one was a medicinal form of DDS called dapsone, which quickly replaced chaulmoogra oil as the standard medication for leprosy. 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8 Comments

  1. Occasionally the infection persists despite continued therapy. In the early s experts assembled by WHO issued the recommendation that all leprosy patients receive combination multidrug therapy and that all leprosy treatment be strictly limited in duration. The bacillus can grow in the footpads of mice, which provides an important mechanism for bacillus cultivation for experimental study.

  2. Given that the organism that causes leprosy is widely distributed and the disease is contagious , it seems logical to ask why leprosy is not a great deal more prevalent than it is. Once the disease has progressed beyond that point, the loss of function is permanent.

  3. The first historically unquestionable [2] papal renunciation is that of Benedict IX in In the 19th century leprosy was believed to be a hereditary ailment. In , however, G.

  4. Once the disease has progressed beyond that point, the loss of function is permanent. From there it seems to have migrated eastward and westward, developing one distinct subtype in Asia and another subtype in Europe and North Africa.

  5. The skeleton was found to have erosion patterns similar to those found in skeletons of lepers in Europe dating to the Middle Ages. The diagnosis is… Mysteries of the disease Mycobacterium leprae , the organism responsible for leprosy, is a relative of M.

  6. He lived two more years as a hermit and then prisoner of his successor Boniface VIII and was later canonised.

  7. Michael's sister Christina Julie Benz , a single mother struggling to care for her troubled teenaged son Milo Liam Aiken , believes in the supernatural and is thrilled with the idea that Anna is back in Michael's life, as she insists he was a better person when she was around. This had the effect of reducing the official numbers of people with leprosy from millions to only hundreds of thousands. Even in cases where early symptoms of leprosy develop, most patients will self-heal.

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