Gastronomic-Paradise

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Research reveals that a rare genetic disorder associated with small stature may offer safeguard against diabetes, heart ailments, and other health issues.

SymClub
May 1, 2024
6 min read
Newswellness
Find exercise you will enjoy and do regularly, said Freeman.
Find exercise you will enjoy and do regularly, said Freeman.

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Experience life in a 4-foot frame free from persistent health issues.

Torres, aged 42, expressed her frustration over people staring, commenting, and even taking photos of her, saying "It makes me feel very bad." Measuring only 4ft 2in (127cm) tall, Torres, being a "little person," isn't accustomed to this level of attention. Growing up in Quito, Ecuador, she reckoned nobody brought up her small size. Instead, she held a job at a car dealership and felt embraced by her community, viewing her stature as a source of opportunity rather than a hindrance.

In her own words, she shared, "Since my teenage years, I've seen my height as a chance for development - the height problem is all part of my personality." Additionally, her unique condition serves as a safety net. Torres is afflicted with Laron syndrome, a rare genetic mutation that thwarts growth, but endows her body with constant protection from chronic conditions like cancer, which typically takes many lives prematurely.

Prof. Valter Longo, an expert on gerontology and biological sciences from the University of Southern California, Los Angeles, remarked, "I wonder what those who crack jokes at Nathaly would say if they knew her body could one day offer insights into how to live a health-promoting life, free of disease."

Prof. Longo has invested close to two decades in studying Laron syndrome. He stated, "We have observed that people with Laron suffer disease infrequently - including cancers, diabetes, and cognitive decline. Brain scans suggest they retain brains that resemble someone 20 years younger." Furthermore, Laron syndrome-diagnosed people may be safeguarded from heart disease too, according to the professor, who was also one of the study's authors. He went on to add, "Laron syndrome is able to shield them from these diseases, but it doesn't make them immune."

The disorder first surfaced in the 1950s within communities of Jewish immigrants hailing from the Middle East. Currently, an estimate puts the number of people with Laron syndrome between 350 and 500 in countries like the US, Ecuador, Israel, Croatia, and Ireland.

Dr. Jaime Guevara-Aguirre, the person who first identified this rare syndrome in the 1980s, discovered 100 individuals scattered across rural communities of Ecuador, his home country, afflicted with the condition. These people shared a common genetic anomaly in their human growth hormone receptor gene, which shut down the body's mechanism for utilizing insulin-like growth factor – a vital hormone responsible for growth in bones and tissues. People with Laron have trace amounts of IGF-1 in their tissues, which come in handy during times of injury, but their bloodstreams levy little to none. Conversely, this deficiency both restricts the uncontrolled growth of cancer cells and heightens insulin sensitivity, preventing diabetes.

In 2005, Dr. Guevara-Aguirre sought out Prof. Longo, who was studying a similar mutation in yeast cells that protracted their lifespans. "Incorporating these yeast cells, worms, flies, and mice with the same mutation all enjoy life spans three times longer than others," Longo claimed. "Mice with a similar human mutation we're investigating have set the mark for the longest-lived mice. These animals live 40% longer than average and 50% of them develop no disease at all."

The pair soon teamed up to pinpoint a drug that could potentially regulate circulating IGF-1 in the larger populace, unlocking anti-aging or disease-preventive benefits for all of us. "The intent would be for individuals with high circulating IGF-1 levels to receive drugs that lower the IGF-1, like our current practice for cholesterol,” Longo explained.

Interestingly, a diet extensive in protein and sugar has been discovered to skip IGF-1 levels sky-high - a scenario that contributes to accelerated aging. Dr. Longo highlighted, "There's this 'fasting-mimicking diet' approach, where you fast for five days each month. Research indicates decreased IGF-1 levels persist for a while after that."

Understanding Laron Syndrome

These special individuals encountered in Israel in 1958 revealed they'd outlived the norm when plagued by diseases. Some experts believe there are up to 350-500 individuals worldwide afflicted by Laron syndrome, including countries like the US, Ecuador, Israel, Croatia, Ireland, and other European nations. In the late 1980s, Dr. Jaime Guevara-Aguirre, then an emerging physician, identified handfuls of people in Ecuador with Laron syndrome.

Exhibiting a mutation in their human growth hormone receptor gene was the common factor among these little people. The disruption in this genetic code buries their ability to use insulin-like growth factor (IGF-1), a hormone established predominantly in the liver to bolster bone and tissue growth - hence, they experienced growth stunting. However, despite a plummeting growth hormone, their tissues still accommodate IGF-1 for healing and other bodily processes.

The absence of circulating IGF-1 fosters a mind-boggling advantage: it curbs cancer cell growth and reinforces insulin sensitivity against diabetes.

In 2005, Dr. Guevara-Aguirre got wind of Prof. Valter Longo, who was digging deep into the relationship between IGF-1 and longevity in yeast cells. Longo had uncovered that the latter organisms could live astonishingly long lives when faced with a similar mutation.

Curious about potential applications for humans, the researchers started collaborating on the prospect of creating a drug that could regulate IGF-1 in the public, which could pave the way for anti-aging benefits and disease prevention. "The goal is to provide people with high circulating IGF-1 levels with medication to lavish them with benefits matching restricted intake," Prof. Longo said.

Notably, a Western diet oversees soaring IGF-1 levels, compounding the aging process. Prof. Longo elaborated, "IGF-1 reducers, like fasting for five days a month, can maintain lowered IGF-1 circulation."

Nathaly Paola Castro Torres, 42, has Laron syndrome, which stunts her height but provides her body with protection from major disease.

Following substantial research, the team published their findings in Med on exactly how Laron syndrome shields its carriers from cardiovascular issues while dropping their risk of several diseases. "Those with Laron syndrome launch their efforts to escape disease at an earlier stage, making it vital for us to comprehend them," they said. Undeniably, their condition ushers in a promising new front to fight disease and aging.

Guevara-Aguirre and Longo are trying to raise funds to give IGF-1-enhancing shots to children born in Ecuador with Laron syndrome. This treatment is believed to stimulate growth during childhood, after which it can be stopped to let the mutation's protective effects against illness take over.

Currently, the Ecuadorian government and pharmaceutical firms are refusing to pay for it. Hence, they are having to deal with it more gradually, using diets instead, with some results so far.

Laron syndrome can lead to severe obesity, which is a risk factor for diabetes, heart disease, cancer, and other health issues. Furthermore, several of the individuals Longo researches "rarely exercise" and often consume alcohol, smoke, and reside in underprivileged neighborhoods with inadequate healthcare, according to him.

Surprisingly, instead of dying from chronic diseases related to obesity, Laron syndrome patients have a higher rate of alcohol- and accident-related fatalities, as well as convulsive disorders.

With their low IGF-1 levels being linked in studies to a higher risk of cardiovascular disease, it was assumed that those with Laron would have a higher incidence of heart and cardiovascular problems, especially given their obesity.

To determine this, Longo and Guevara-Aguirre analyzed Torres and 23 other people with Laron syndrome and compared them to 27 of their first-degree relatives without the disease, most of whom resided in Ecuador.

The researchers discovered that individuals with Laron didn't have an increased incidence of cardiovascular disease despite their obesity and their lives in impoverished circumstances, even though signs of cardiovascular wear and tear were absent from the study, prompting some questions, according to Freeman.

Freeman, a Denver-based cardiovascular prevention and wellness specialist who wasn't involved in the research, thinks the study is fascinating and holds potential.

“This study opens the door to understanding how we can reduce the harms that come with age,” he said.

Although considered overweight at 100 pounds (45.4 kg), Torres was healthy, with no signs of diabetes or cardiovascular disease, and had no signs of diabetes or heart disease during a recent checkup.

Her doctor advised her that she could lose weight if she wanted but wasn't necessarily in danger. Torres intends to stay in the United States to take advantage of educational opportunities and pursue a better career. Despite being fortunate to have this advantage, she's still aware of the importance of paying attention to her diet.

"I've learned that even though my body is a bit more adaptable, I still have to be cautious about my diet," she said. "We don't know for sure how much this will protect us."

She is also proud to be part of research on Laron syndrome.

"The goal is to develop medical assistance to help those who are fighting with diabetes and cancer, two of the most tragic illnesses," she said. "This is my hope."

Find exercise you will enjoy and do regularly, said Freeman.
Rather than help weight loss, the study suggests smoking leads to more abdominal fat.

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    Source: edition.cnn.com

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