Unexpected Medical Discoveries Found in the Dwarfs of the Andes
Unexpected Medical Discoveries Found in the Dwarfs of the Andes
Brooke Phenicie, Period 6
Due Date - April 22
Found in Ecuador were a group of dwarfs that have a disorder called Laron Syndrome. It causes a stunt in their growth, but also uncovers a surprising fact. These dwarfs are immune to developing cancer or diabetes. They have an insufficiency to human growth hormone that causes a restriction in their growth. A team of researchers have considered producing a medication for normal humans to take that will help regulate their growth hormone and keep them healthy. This process has not been taking into action, but is an exciting idea. These dwarfs could be the answer to increasing the lives of many people.
In 1987, Dr. Guevara-Aguirre, an Ecuadorian physician and diabetes specialist, came across a small group of adult Ecuadorian dwarfs while riding on a horse through the mountains (Wade, 2011, 12)(Calderon, 2011, 5). They lived along the slopes of the Andes Mountains in South America and were from a family of a Spanish background (Theibert, 2011, 3)(Naik, 2011, 3). There are about 300 people total in the world with Laron Syndrome and Aguirre had found one third of them (Naik, 2011, 12). About a hundred of these dwarfs were looked into by the University of Southern California for having Laron Syndrome, which restrains the purpose of growth hormone (Naik, 2011, 1). After seeing this, it was discovered that almost everyone with Laron Syndrome never developed diabetes or cancer (Naik, 2011, 8). Almost all the dwarfs live longer than normal humans too. Facts show that only one person with Laron Syndrome developed cancer and no one developed diabetes, even though many of the dwarfs were overweight (Brown, 2011, 3)(Brown, 2011, 4). Aguirre studied these dwarfs for 22 years along with the help of Valter Longo, who Aguirre found in 2005 (Wade, 2011, 13). Valter Longo was a professor who was interested in the mechanism of cellular protection and aging. They worked together to try to produce a medication or drug for the people with Laron Syndrome to use to help normal humans live disease-free.
Laron Syndrome is an irregularity that affects a person’s growth by 50% and is caused by an alteration called E180 (Brown, 2011, 1). It is believed to be caused by inbreeding and a lack of IGF-1, Insulin-like Growth Factor. Laron Syndrome is know for delayed bone age and being short in size. Most people with Laron Syndrome only reach the height of about 4 foot 10 inches, or less (Jirage, 2010, 4). Even though all the dwarfs with Laron Syndrome never had cancer or diabetes, some of their ancestors without the disorder did. As fascinating as it is, when scientists put the Laron disease into mice, they found that the mice lived almost ten times longer than they normally would (Dunbar, 2008, 5). Researches took serum from Laron individuals and combined it with human cells. They discovered that the serum helped DNA to not break, which would assist in the making of cancer (Brown, 2011, 12). Also found in the serum was increased apoptosis, which is the death of cells that occur as a normal part of an organism's growth (Aguirre, 2011, 5). The serum had a "double protective effect" by protecting DNA against damage and by contributing to the help of killing off injured cells (Marziali, 2011, 10)(Marziali, 2011, 11). If too much hormone was to be put into a normal human, breast, prostate, or bowel cancer could develop (Dunbar, 2008, 7). Plus, high growth factor levels could be a risk for cancer (Marziali, 2011, 15). Longo believes that if there was less IGF-1 there would be less DNA damage in cells (Dunbar, 2008, 8).
Diabetes is caused by having high blood sugar or a too small amount of insulin (Anonymous, 2010, 1)(Anonymous, 2010, 2). The reason why they have high blood sugar is either because their pancreas doesn't make enough insulin or their muscle, fat, and liver cells don't respond to insulin. The role of insulin is to move glucose from the bloodstream to the muscle, fat, and liver cells. Insulin is a huge part of diabetes because if a person stops eating for a few days, their insulin level drops (Naik, 2011, 20). Out of Diabetes 1 and Diabetes 2, Diabetes 2 is more common based on the obesity factor evolving (Anonymous, 2010, 5). If someone is over 45 years old, has family with diabetes, or has heart disease or high cholesterol, they have a better chance of developing diabetes (Anonymous, 2010, 6).Fasting and reducing protein intake can cause the immune system to wear out which aids in the production of diabetes (Naik, 2011, 19). There are 23.6 million people that have diabetes in the United States and it is the sixth leading cause of death since 2006. As deadly as this disease is, the Laron's are lucky to be immune to such a horrifying disorder. Being overweight is a huge contribution to developing diabetes, but even though the dwarfs with Laron Syndrome had this feature, there was still no sign of any type of diabetes (Marziali, 2011, 12).
Adversely, there was little evidence of cancer. Cancer occurs when a group of cells demonstrate uncontrollable growth (Anonymous, 2010, 1). In a normal person's body, cells work differently. If the body needs the cells, they will multiply and if the body doesn't need them, they will die (Anonymous, 2010, 2). There are many different types of cancer and all symptoms of cancer depend on where the cancer is taking place in the body (Anonymous, 2010, 5). Cancer can take place in any organ or tissue, such as the colon, breast, lung, skin, and nerve tissue. Some types of cancer are treatable, but there are still many cancers that have no treatment and remain a mystery. Cancer can be caused by many things including sunlight, drinking alcohol, radiation, toxins, and obesity. Some say that the dwarfs weren’t exposed to things like chemicals and smoke, which we experience everyday that could help this process (Theibert, 2011, 2). Apart from being immune to diabetes, the overall percentage of the dwarf population that had cancer was 17% (Naik, 2011, 10).
Dwarfism is a disorder where someone has a very small body or stature and can be caused by over 200 different conditions, by which most are genetic. A lot of the time, a person's body can be a different size than their arms or legs. Just like Laron Syndrome, adults only grow to be 4 foot 10 inches and under (Anonymous, 2011, 1). Dwarfism is not a disease, but can involve difficulty with hormone and metabolism (Anonymous, 2011, 2). There are three main types of dwarfism; achondroplasia, Turner syndrome, and growth hormone deficiency (Jirage, 2010, 6). In achondroplasia, a child receives one mutated copy of the gene and one normal copy of the gene. In Turner Syndrome, there is a random deletion of the X chromosome in the egg or sperm (Jirage, 2010, 8). In growth hormone deficiency, the cause usually involves genetic mutation (Jirage, 2010, 10). However, dwarfism is associated with the genetic mutation that counteracts to GHR, or human growth hormone (Brown, 2011, 10). GHR has a receptor, or protein, that is fixed in the tissue of cells (Wade, 2011, 3) This receptor also has a shortage of its last eight units in its outside section that doesn't react with GHR, causing the Laron patient's stunt in growth (Wade, 2011, 5). GHR can only be found in Laron subjects and indicates a higher insulin sensitivity, causing the reactions to the syndrome. Along with affecting this genetic mutation, faulty nutrition and hormonal variation adds to the causes of dwarfism (Jirage, 2010, 5). Nutrition is a big part of staying healthy when having a genetic mutation like GHR because if someone goes a short time on an intense diet or period of fasting, it can cause life-threatening affects to that person (Marziali, 2011, 20).
The goal of researchers interested in the Laron subjects is to develop a type of drug to put into humans that will help growth hormone and diseases involved with aging (Brown, 2011, 6). The intention is not to make humans live longer, but to live disease-free and healthy. Scientists did animal studies on blocking growth hormone and they found it caused a "40% life span extension" and reduced the risk of getting tumors (Marziali, 2011, 17)(Marziali, 2011, 18). Scientist also found that a Laron patient's cells will kill off damaged cells that are about to become non useful (Brown, 2011, 5). Based on the positive outcomes of testing the hormone, biologist Valter Longo said he would like to use it for particular people going through chemotherapy, the treatment of disease by using chemical substances. (Marziali, 2011, 19). By using this during chemotherapy, it will help protect the patient's cells from chemical damage. The drug is suppose to be initially used in full grown adults because the hormone hasn't completely developed in children or teens that haven't hit puberty. The growth hormone medication has actually worked better than medications for an established disease (Marziali, 2011, 14). Some researchers were worried about the Food and Drug Administration already having a drug for growth hormone, but it is used to treat giantism, which is when a child grows abnormally large during their childhood due to their growth plates remaining open (Salazar, 2011, 4).
On the other hand, the team involved with the making of this medication say they can't just lower the levels of insulin in humans (Steve, 2011, 1). Side effects can appear such as cardiovascular problems and low energy levels. Researcher had tested the drug on mice and found that it can not make the growth factor receptor, which humans traits match (Steve, 2011, 3). Because of this, the team may need to find a new way to develop this drug or change some qualities of it to make it useable. If they found a way to produce a successful drug, it would have to be given to the patient before he/she has hit puberty. If not, there's no point because the person has already begun to grow and it would be a problem to their hormones causing extra side effects. They want to prove that there is a connection between genetic mutations and a lowered cancer rate (Dunbar, 2008, 9). The drug would initially be aimed towards adults to lower their insulin level to normal (Naik, 2011, 22). The only reason why the team was worried about giving it to adults was because of their strange causes of deaths after being completely satisfied with the drug (Marziali, 2011, 4). Another problem to consider would be the financial aid of making this product and getting it approved.
Researches found that the dwarfs living in Ecuador were not dying of usual deaths. So far, there has only been 30 deaths in the Laron population, "eight from heart disease, one from stroke, and twenty one from non-age related causes" (Brown, 2011, 2). In the group of "non-age related causes", some were even from alcohol, substance abuse, and accidents. In America, the top leading cause of death is heart disease and the 3rd leading cause of death is stroke (Anonymous, 2010, 1). The Laron’s deaths fall perfectly into these categories, but still no one can explain why there were so many deaths involving alcohol and substance abuse.
In conclusion, these Ecuadorian dwarfs have an impressive feature. Not only are they immune to getting cancer or diabetes, they get to live their life disease-free. By having Laron Syndrome, the dwarfs are quite a bit shorter than normal humans, but receive a wonderful benefit on the other side. Hopefully scientists and researchers will be able to produce a successful medication in the future for regulating growth hormone deficiency after all the positive outcomes seen in the recent tests. These dwarfs with Laron Syndrome show an outstanding discovery that in the future, they may bring an end to diabetes and some cancers.