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

Get Free Diabetic Supplies

Get free diabetic supplies delivered to your door. You will get a free glucose meter, lancing device, 100 test strips, 100 lancets, control solution and carrying case. Just pay shipping.

More Articles

The medical community relies heavily on the goodwill of its citizens, as giving blood and organ donations help save thousands of lives every year...

There are several misconceptions about Diabetes. Learn more about the top misconceptions vs. facts surrounding Type 2 Diabetes below.

86...

It is estimated that 80,000 children and adolescents each year are diagnosed with juvenile diabetes. Juvenile diabetes is diagnosed when the body...

As the summer months come to an end, we throw aside our swimsuits and bring out our favorite team jerseys! In America, fall means football, and...

Evidence is mounting that consuming black cumin helps to manage type 2 diabetes. The spice lowered blood glucose...

More Articles

It's not uncommon to spend several years living with type 2 diabetes and not even know you have the condition. Despite regular check-ups with your...

Type 2 diabetes has become so common in Western culture and media that this often lifelong, serious condition doesn't really raise an eyebrow...

Limiting our intake of highly processed foods, and eating more whole and lightly processed fare can boost our nutrient and fiber intake, lower...

Lantus is a once a day insulin injection that is taken at the same time every day. It replaces anywhere from the average three to five insulin...

Improving your A1C reading requires you to maintain consistently healthy blood...

Just because you have diabetes doesn't mean you can't help your community by being a blood donor. When persons with diabetes maintain good control...

Most of us do not regularly keep the fruit that scientists call Fortunella margarita in our refrigerator. That these small, yellow-orange...

A diagnosis of diabetes usually comes with a list of recommended lifestyle changes. To make these diet and exercise...

Having diabetes affects your body’s ability to handle hot, humid weather, and steamy temperatures can degrade diabetes supplies. But all of this...

Fashion and function come together in clothing developed by AnnaPS, making life a bit more comfortable and convenient for insulin users. The...

Not only is dry skin uncomfortable, but scratched or cracked skin is also susceptible to bacterial and fungal infections – common medical...

One way to ensure our body gets a variety of nutrients is eating nutrient-dense foods, and one of the most nutrient-dense foods on our planet is...

Financial aid and scholarship money are available for people with diabetes, but acquiring it requires an investment of time and patience. Whether...

Any sweetener you use to replace table sugar is a sugar substitute. One type of sugar substitute is artificial sweetener. Three other types...

Smoking is a proven health hazard for everyone, but it is an especially dangerous habit for those with diabetes. Research shows that smokers with...