The International Society for Mannosidosis & Related Diseases

ISMRD: A global approach to Oligosaccharide Storage Diseases

 
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Our Mission

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ISMRD is the leading advocate for families worldwide affected by an Oligosaccharide Storage Disease . Through partnerships built with medicine, science and industry, we seek to detect and cure these diseases, and to provide a network of support and information.

 

 

ISMRD
  • A quarterly newsletter, Pathways, distributed at no charge to interested parties.
  • Our informative website.
  • Informational brochures about our organization and the Oligosaccharide Diseases .
  • Parent matching.

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ISMRD FEIN #: 52-2164838
State of Maryland Charity
Registration #: 6421

How to contact us:
3210 Batavia Avenue
Baltimore, MD 21214 USA
410.254.4903
info@mannosidosis.org

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Alpha-Mannosidosis

Alpha-Mannosidosis is a lysosomal storage disorder. Lysosomes are particles that are enclosed by membranes in cells that are found in the digestive tract, and they function as digestive units. The enzymes found inside the lysosomes break down particular nutrients including complex molecules made up of glycoproteins – sugar that is attached to protein.

Sometimes, the alpha-mannosidase enzyme could become inactive or reduce in supply, and this could result in abnormal accumulation of compounds in the metabolic pathway in cells of the infected individuals, and this comes with unfavorable outcomes.

Symptoms

The progression, symptoms, and the severity of alpha-mannosidosis vary from one person to another and even in the siblings who have the same mutation. In some people, the symptoms show up immediately after one is born and this could result in life-threatening complications during infancy or even early childhood. On the other hand, some individuals may develop mild symptoms before the age of 10 years. The symptoms may also show up during adulthood like unexplained weight gain or loss of testosterone levels in men.

The Lysosomal storage disorder is a condition that can be inherited and it is a metabolic disorder that is characterized by an entirely abnormal build-up of different toxic substances in the body cells, a condition resulting from enzyme deficiency. There are close to 50 various forms of the alpha-mannosidosis, and they tend to affect different parts of a human body which include heart, brain, skeleton, skin, and the nervous system.

Still, new forms of alpha-mannosidosis disorder are being identified. Though there are clinical trials in progress in an attempt to find a cure for this condition, so far, there is approved a cure for the alpha-mannosidosis health condition.

Researchers are still studying gene therapy, with the hope that it can shed light on the treatment of the lysosomal storage disorder. Gene therapy involves replacing the defective gene in patients with another normal gene and this enables the production of the active enzyme in the affected areas, and this slows down as well as preventing the progression of alpha-mannosidosis. Read more on ALPHA-MAN Project.

With the replacement of the defective gene with a gene that functions well, this form of therapy seems to be the anticipated cure for alpha-mannosidosis. However, as of now, we can't really conclude that scientists have arrived at the cure of alpha-mannosidosis. Numerous technical difficulties need to be resolved to make sure that the gene replacement therapy is successful.

To deal with unexplained gain, some phentermine/topiramate drug like Qsymia or herbal alternatives can be tried.

Does Mannosidosis effect Sex Life And Testosterone

One of the things that may shock many men is learning the fact that sex is an excellent way of enhancing testosterone, a fact that was revealed by many scientists. A good example is a study that was carried out in 1992 and which examined four different couples on the days that they had sexual intercourse and the days they didn’t. However, Mannosidosis can have huge impact on sex lives of many,in later years of ther life.

The obvious results were that the testosterone levels went up for the nights the couples had sex and decreased in the nights the couples missed sex. Therefore, according to this study, it is clearly evident that testosterone levels vary according to one’s sexual activities.

One thing that was very interesting in the 1992 study is that males were instructed to take either Viagra or the Cialis, yet it is known that Cialis contain more testosterone as compared to Viagra.

These two drugs are PDE5 inhibitors and therefore, they enhance the level of nitric oxide, resulting insufficient blood supply in the male sex organs. However, it is worth noting that Cialis has a longer half-life, and this is crucial in comprehending variations in the testosterone levels.

For most guys, Cialis is effective for close to 36 hours in one evening, and the researchers pointed out that sexual intercourse frequency was higher in the couples who took Cialis.

It can be concluded that the higher levels of testosterone associated with Cialis lead to more confidence and better erection as compared to the Viagra group. According to the researchers, Cialis and Viagra have no different direct impact on the pituitary-testis axis, and this effect could be due to frequent full sexual intercourse.

There are natural foods that enhance sex drive, and such foods help men to have a better erection. It is believed that such foods correct the imbalances in the human body. For those who wish to get the best out of these foods, they should shun bad habits such as smoking and other drugs.

To cure sexual problems caused by mannosidosis, natural foods and herbs that enhance sex drive in men and certain antioxidants supplements are recommended.

Here is a list of foods that are known to increase sex drive in men as well as helping them to maintain an erection as desired.

1. Caffeine

Caffeine is useful for enhancing blood flow and therefore, it is recommended to take coffee or tea two to three times a day. Also, caffeine improves body metabolism as well as burning excess fat in the body.

2. Foods rich in zinc

Seeds, nuts, and oysters are good sources of zinc and vitamin B. the two nutrients are crucial in the production of sex hormone –testosterone, which heightens sex desire in men.

3. Potassium-rich foods

Potassium is essential in ensuring a smooth blood flow and a healthy heart. It also lowers the levels of sodium in the body and significantly reduces the risk of high blood pressure. For better levels of potassium, dwell on foods such as watermelon, bananas, and other foods that contain this nutrient.

4. Fresh fruits

Fresh fruits supply your body with the essential nutrients that you might have missed in the main meals. They are not only rich in vitamin but also act as body cleansing agents. They remove any deposits in your blood arteries, and this ensures smooth blood flow.

5. Thiamine-rich foods

Thiamine is essential for the better functioning of the nervous system. Since the erection process begins in the brains, then, you need a healthy nervous system for good erection. Whole-grain bread is good sources of thiamine.

6. Seafood

Seafood is an excellent source of a low-fat diet, and it is known to be one of the bets cures for erectile dysfunction. It enhances blood circulation, and this means that that blood can flow well to the male sex organ resulting in a better erection.

7. Foods rich in fiber

It is recommended to take at least 3mg of fiber three to five times a day. It controls the levels of cholesterol in the body, and this enhances blood circulation.

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URL: www.mannosidosis.org 
Last updated:
Friday, March 21, 2003