Metabolic Health

MODY Diabetes - Causes, Symptoms & Treatment - Sugar.Fit

Reviewed by

Shifa Fathima

MODY Diabetes Causes, Symptoms & Treatment

What is MODY Diabetes?

MODY, Maturity-onset diabetes of the young, is a group of relatively rare kinds of inherited diabetes that manifests itself during teenage or young adulthood. It interferes with adequate insulin production by the pancreas, which results in high blood sugar levels. Among all cases of diabetes, MODY diabetes accounts for only about 2%. It usually runs in successive generations, which means that a grandparent, parent, and child of a family have MODY diabetes. As against adult-onset diabetes (type 2 diabetes), factors such as obesity do not increase the risk of MODY diabetes.

MODY Diabetes is so rare that it often gets misdiagnosed as type 1 diabetes or type 2. Some key features of MODY Diabetes that helps it distinguish from the other type of diabetes are:

  • Diagnosed with diabetes below the age of 25 years.
  • Diabetes running in successive generations.
  • More than average weight at birth (about 4 kg) or abnormally low blood sugar at birth.
  • If you fulfill any of the above criteria, it is advisable to undergo genetic testing, along with the usual blood test used for diagnosis of diabetes, to rule out any MODY gene mutation, and get a correct diagnosis and treatment.

Types of MODY Diabetes

There are different MODY Diabetes types depending upon the gene in which mutation has occurred:

  • MODY 1: Caused by a mutation in the HNF4A gene. It is a less prevalent type of MODY Diabetes.
  • MODY 2: Caused by a mutation in the GCK gene. This type accounts for about 30%-50% of cases of MODY Diabetes.
  • MODY 3: Caused by a mutation in the HNF1A gene. This is one of the most common types of MODY 3, affecting 50%-70% of MODY Diabetes persons.
  • MODY 5: Caused by a mutation in the HNF1B gene. It is a rare type, accounting for just 5%-10% of the cases.
  • Other : There are several other MODY diabetes types, but they are very rare.

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Causes of MODY Diabetes

MODY Diabetes is caused by a mutation (change in a gene) in a single gene. Thus it is a monogenic disorder. On the contrary, other common types of diabetes, such as type 1 and type 2 diabetes, are caused by changes in several genes (polygenic) and external factors such as obesity, lifestyle, and food habits.

If a parent has MODY diabetes, there is a 50% chance that the child will inherit this gene and develop MODY diabetes before the age of 25 (mostly), regardless of the weight and lifestyle status.

Symptoms of MODY Diabetes

The symptoms of MODY Diabetes vary with the gene that is affected. Some people do not have any symptoms. The symptoms appear gradually, and a person may have high blood sugars for years before getting diagnosed. The list of symptoms of MODY Diabetes are:

  • Polydipsia (increased thirst)
  • Polyuria (frequent urination)
  • Fatigue
  • Frequent skin infections
  • Weight loss
  • Blurry vision

If MODY Diabetes remains undiagnosed, over a course of time, the uncontrolled blood sugar levels can result in damage to the nerves, causing neuropathy, and adversely affect the kidneys, resulting in kidney failure, along with affecting other organs such as the heart, eyes, and foot.

Though MODY diabetes symptoms may be similar for different types, certain features are specific for different MODY types.

For example,

  • Infants born with HNF4A gene mutation tend to weigh more than the average babies and may have abnormally low blood sugar levels at birth. However, other signs and symptoms of diabetes manifest only later in childhood or young adulthood in these cases.
  • Individuals with HNF1A gene mutation are at a higher risk of developing benign liver tumours known as hepatocellular adenomas.  
  • GCK-MODY is a very mild form of MODY Diabetes, and generally do not have any symptoms of diabetes. Usually, people having this type just have a slightly high fasting glucose level.
  • People with HNF1B-MODY, also known as RCAD syndrome (renal cysts and diabetes), often have kidney cysts or urinary tract abnormalities and diabetes. They are also at a higher risk of diseases of the liver, pancreas, or gout (a type of arthritis).

MODY Diabetes Treatment

Treatment of MODY Diabetes depends upon the gene that has been affected. Following is the treatment approach for different MODY Diabetes types:

  1. MODY 1 and MODY 4: It is commonly treated with the class of drugs sulfonylureas. It induces increased insulin production from the pancreas. Some persons of MODY 1 and MODY 4 may also require taking insulin.
  2. MODY 2: This type of MODY Diabetes can usually be successfully managed through diet and exercise. Medicines are generally not required.
  3. MODY 3: In the initial stages, this type of diabetes can be controlled with diet. Over the course, though, medicines may be required, followed by insulin.
  4. MODY 5: Insulin is required for the treatment of this type of MODY Diabetes. Complications such as kidney cysts or kidney failure may occur in MODY 5, which will further need to be treated.
  5. MODY 6: It generally occurs in later life, around the 40s, and persons require insulin to treat this type.

In cases where insulin is required for the treatment of MODY Diabetes, they often need smaller doses of insulin than people with other types of diabetes. Similar to all types of diabetes, there is no cure for MODY diabetes. But treatments are available to help control blood sugar levels adequately.

Bottomline

MODY Diabetes (Maturity-onset diabetes of the young) is so-called because its symptoms usually occur in adolescence or early adulthood. It is a rare form of diabetes caused by mutations in a single gene. MODY diabetes is passed down in families and is unrelated to obesity or lifestyle factors. There are several types of MODY diabetes, each caused by a different mutation in the MODY gene. Though there is no cure for MODY diabetes, it can be successfully managed, like other types of diabetes.

FAQs

1. How is MODY diagnosed?

If your blood test shows a high blood sugar level, you may be diagnosed with diabetes. Your physician may then inquire about your family history and do some other blood tests to ascertain the type of diabetes. Based on these results, your doctor may order genetic testing to look for any gene mutations that can cause MODY Diabetes. Thus, genetic testing gives a definitive diagnosis of MODY Diabetes.

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