Please fill-out the enrollment form, if
1) you have been diagnosed with MODy- or MIDD diabetes OR
2) you/ your doctor/ your diabetes nurse has thought that you could have MODY or MIDD diabetes or OR
3) your close relative has been diagnosed with MODY- or MIDD diabetes and you are not aware if you have the disease too OR
4) you want to contact us in other matter concerning MODY- or MIDD diabetes
MODY (abbrevied for Maturity Onset Diabetes of the Young) comprises a group of monogenic diabetes arising from pathogenic variants (or mutations) in one of known MODY genes.
Glucokinase-MODY and HNF1A-MODY are the two most common subtypes of MODY. A mild subtype of MODY, glucokinase-MODY, might remain undiagnosed for decades, as the symptoms are generally mild or non-existent. The life-spanning feature of glucokinase-MODY is to have a constantly elevated fasting glucose level. Other subtypes of MODY usually emerge later in life; patients are typically young at diagnosis. Many MODY patients – but not all – present with a strong family history of diabetes (several relatives with diabetes). As MODY is quite rare (about 1-2% of all diabetes), some patients remain undiagnosed or misdiagnosed as type 1 or of type 2 diabetics. Today, the number of known MODY genes has exceeded 10.
Glucokinase-MODY (GCK-MODY or MODY2)
In glucokinase-MODY, fasting plasma glucose level is constantly elevated (5.5-8 mmol/l). HbA1c rarely increases markedly. The initiation of antihyperglycemic agents is unnecessery to treat fasting hyperglycemia in glucokinase-MODY. With non-existent to mild symptoms, hyperglycemia is often revealed in routine screening.
Glucokinase (GCK) is the enzyme which sensors glucose level in pancreatic beta cells. If glucose reaches the threshold of the enzyme, the pancreas releases its insulin. MODY variants (=mutations) in GCK raise this threshold level.
HNF1A-MODY and HNF4A-MODY (or MODY3 and MODY1)
In early stages of HNF1A and HNF4A, fasting glucose can remain relatively normal for years. Hyperglycemia is initially evident post-prandially (=after the meals) only. Both HNF1A- and HNF4A-MODY present with insulin deficiency, which is milder than in type 1 diabetes. A core component of diet is to balance carbohydrate content of meals. Some patients rely on glidines (“meal tablets”) which release insulin from the pancreas. DPP4 inhibitors might alleviate hyperglycemia. At times, fast-acting insulin is initiated. No placebo-controlled drug trials, however, exist so far. The long-affecting insulin is less often required, as it readily causes hypoglycaemia among the patients (=too low a blood glucose).