MODY: One of Several Hereditary Forms of Diabetes
The most well-known variations of diabetes are type 1 diabetes and type 2 diabetes. Both type 1 and type 2 diabetes are polygenic, meaning that the development of the disease is the result of an interaction among several genes.
What is less known is that there are a variety of related diseases that don’t fall within these two diagnoses, but share many of the characteristics of diabetes. The difference between these variations is that each is the result of a mutation of a single gene (monogenic.)
MODY
MODY refers to Maturity Onset Diabetes of the Young, one of several hereditary forms of diabetes caused by a specific type of genetic mutation. This mutation may occur in any one of a number of different genes, and each gene’s mutation results in a slightly different variation of diabetes.
MODY is the diagnosis in 1 to 5 percent of diabetes cases. While MODY is generally diagnosed in later childhood, adolescence or early adulthood, many cases of MODY are not diagnosed until patients are in their 50s. There is also a significant risk of misdiagnosis. Since the differences in presentation of the disease can be subtle, patients are frequently misdiagnosed, at least initially, as either type 1 or type 2.
Types of MODY
HNF1A (MODY3) is the most common form of MODY. It is inherited and generally shows up early in life. Insulin is usually not immediately needed for treatment, as this disorder responds well to sulfonylureas.
GCK (MODY2) is the second most common type overall of MODY. (It might be the most common type suffered by children.) The presentation of symptoms is mild, with only slightly elevated blood sugars. These elevations, though, are generally present from birth.
HNF4A (MODY1) is an inherited, early-onset form of MODY that responds well to treatment using sulfonylurea pills.
INS (MODY10) can cause neonatal diabetes, antibody negative type 1 diabetes and MODY.
ABCC8 can also cause neonatal diabetes, but rarely leads to MODY. This may present mildly elevated blood glucose levels.
NEUROD1 (MODY6), KLF11 (MODY7), PAX4 (MODY9) and BLK (MODY11) are very rare forms of MODY.
IPF1 (MODY4) is another very rare form of MODY. This version can cause exocrine pancreatic insufficiency, or impairment of the digestive functions of the pancreas. The pancreas might also be very small.
CEL (MODY8) may cause exocrine pancreatic insufficiency, because the digestive functions of the pancreas are impaired by this version of MODY. Like MODY4, the pancreas might also be very small.
HNF1B (MODY5) can cause either diabetes or kidney problems. Individuals diagnosed with MODY5 may go on to develop diabetes or developmental problems with the kidneys (cysts are the most common finding.) The patient may develop diabetes or kidney disease. He or she may also have abnormalities of the reproductive organs.
Sources: National Institutes of Health and University of Chicago (2)
Photo: Pixabay
More Articles
With its slightly nutty flavor, chewy texture, and nutritional punch farro is an ancient whole grain worth a place in our pantry.
Farro...
Having diabetes is cause for concern, however it doesn’t mean that you need to eliminate all of your favorite foods from your diet completely....
With diabetes becoming more of an epidemic each and every day, being knowledgeable about the disease, its effects on the body as well as ways to...
If you’re a diabetic or live with one, it can be tough trying to figure out the best foods to eat because your priority is to control your blood...
In this post, I will show you the 10 best ice creams for diabetics.
When you think of sweetened foods that a diabetic shouldn’t consume,...
More Articles
If you’re living with diabetes, snacking is a necessity because going for more than 3-4 hours without eating can be detrimental to your health....
For diabetics, choosing healthy snacks can be a daunting task.
A good rule of thumb is to pick out snacks that are rich in protein, fiber,...
According to information available through the National Institutes of Health, there’s an estimated 462 million people in the world who are...
Eleven Clinical Studies
After eleven clinical studies and 300,000 participants, researcher Vasanti Malik and her team of researchers...
Diabetic women often have a harder time losing weight than non-diabetic women. A study funded by Jenny Craig proved that diabetic women have an...
Many recent studies have proved that magnesium levels are lower in patients with diabetes than in non-diabetics. This magnesium...
Fluid retention, also known as edema, is a problem that affects many diabetics, especially those with type 2 diabetes...
Some of us might be thrilled if we could manage our blood sugar by sitting in a hot tub or sauna, instead of working up a sweat biking, or using...
Cooking and baking with the ancient cereal grain sorghum has health benefits for people with diabetes, and those with weight control issues....
When it comes to certain foods, there are always questions as to whether or not a diabetic can have them without...
Matcha tea is a rich, creamy, full-bodied beverage with amazing nutritional properties that address several diabetes health concerns.
The...
Salads are good example of foods that type 2 diabetics can enjoy with relatively low guilt. With the right greens and other elements added, salad...
Remaining gainfully employed is important to many people. Those who live with any form of diabetes may find that some lines of work are more...
Learning that you have diabetes does mean making some lifestyle changes. One of the areas that needs attention is your diet. Most people find that...
One of the more challenging aspects of life as a type 2 diabetic is managing your diet. There’s often the temptation to avoid certain foods...