MODY diabetes puzzles docs

Prabeerkumar Sikdar


A Rare Hereditary Form, It Is Often Confused With Type-1 And Type-2 Diabetes

While most people are familiar with Type-1 and Type-2 diabetes, there is a third type called Maturity Onset Diabetes of the Youth (MODY) which city medical experts have encountered, but face difficulties in identifying because clinical features mimic the first two.

Known to surface only in one per cent of diabetic population, especially in the age group of 10 to 30 years, the MODY form of diabetes is often prone to misdiagnosis. A rare hereditary form, it has nine different sub-types depending upon the specific defective gene involved.

A misdiagnosis case can result in the affected patient getting wrong treatment meant for either Type-1 (insulin dependent) or Type-2 (noninsulin dependent) diabetes with disastrous consequenc es. Though city experts report treating such cases, there hasn't been a case recorded in recent past in the city.

“Research suggests that treating MODY patients with drugs meant for Type-2 diabetes appears to lead to destruction of insulin-secreting beta cells that regulate blood sugar,“ said Dr V Mohan, chairman, Dr. Mohan's Diabetes Specialties Centers, adding that about eight to 10 referral cases have come from Hy derabad to their Madras Dia betes Research Foundatio for diagnosis.

While adding that ge netic tests are mandatory fo MODY diagnosis, he said tha earlier those below 25 years were considered vulnerable, but now latest guidelines say that patients less than 40 years can take the test.

The diagnosis for MODY involves a genetic test to find out which par ticular gene is responsible for the condition, but city-based endocrinologists Dr Shyam Kalavalapalli, director Institute of Diabetes, Endocrinology and Adiposity (IDEA) termed it a costly affair.

“For the vast majority of diabetic population some of who could be a suspected patient of rare MODY type of diabetes, it may be difficult to afford a genetic screening test that could cost between Rs 50,000 to Rs 1 lakh. I would suggest a cost effective method of diagnosis that in volves giving the suspect dose of s u l p h o ny l u re a s (generic name) to see if they respond to it or not,“ said Dr Shyam. In fact, Dr Shyam, a practising endocrinologist in the city, said his last confirmed MODY patient was a Londonbased pregnant lady , a case he detected five years ago during his practise in the UK.“She was detected with Mody 2, a sub-type that's caused due to mutation in GCK gene that is known to take affect during pregnancy . What's interesting is that this form of diabetes does not need any medication as the blood sugar level remains constant without posing any danger if it remains unmanaged,“ says Dr Shyam.



For endocrinologists, a potential Mody case arises when they are faced with a diabetic patient with a family history of diabetic patients. Someone who is battling the disorder could be as young as 10-20 years, could be someone not obese, but with high blood sugar, and one with a previous history of a Mody patient in the family