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MODY gene is

A HNF 1alfa
B HNF 4 alfa

C glucokinase
D All

Ans. D

Recent Advances:

Other categories of Diabetes Mellitus

1). Potential Diabetes (PNQ): Person himself at present is non-diabetic but has a strong family history Q of type 2 diabetes.

2). Latent Diabetes (PNQ): A person becomes diabetic under stressful conditionsQ like pregnancy. Person again becomes non-diabetic when stress is removed.

3). Brittle Diabetes (PNQ): It is seen in children with type 1 diabetes. Sometimes patient’s sugar becomes very high leading to DKA, sometimes patient goes into hypoglycemiaQ.

4). Acute Fulminant diabetes mellitus (PNQ)

It is acute onset diabetes, can occur in any age, occurs after viral infection. Serum insulin level are reduced but there are no antibodies against insulin or beta cell. (PNQ)

The viral infections associated with diabetes are :­

Mumps, Measles, Coxsackie virus, Cytomegalovirus, Rubella, EB virus

5). Maturity onset diabetes of the young (MODY) is an autosomal dominant Q form of Type 2 DM affecting young people with a Definite positive family history. (AIPG 2009)

There is impaired glucose – induced secretion of insulin.

Six type of MODY have been described. Except for MODY 2, in which a glucokinase gene is defective, all other types involve mutations of a hepatocyte nuclear transcription factor (HNF) that regulates islet gene expression. (MODY 1,3,5 )

MODY 3 is the most common form – accounts for two-thirds of all MODY cases. It is due to HNF-1 alpha mutation.

Features of MODY: (AIIMS May 2013)

1). No antibodies

2). No obesity

3). Glucokinase gene defect Q

4). DKA uncommon Q

5). Insulinopenia Q

6). No hypertension

7). No Insulin resistance

8). No Hyperlipidemia

6). Malnourished diabetes (PNQ)

It is seen in malnourished children. Serum insulin level are reduced but there are no antibodies against insulin or beta cell. But in these patients DKA is uncommon.

7). Latent autoimmune diabetes of adults (LADA) is a form of Type 1 DM, with slower progression to insulin dependence in elderly person. So in this case also serum insulin level is reduced & auto antibodies are there.

8). Gestational Diabetes: This term includes gestational impaired glucose tolerance (GIGT) and gestational diabetes mellitus (GDM). These women are at risk of later developing diabetes, with Type 1 versus Type 2 diabetes mellitus

It is defined as

a). Fasting plasma glucose ≥ 126mg/dl or

b). After 100gm glucose- fasting ≥ 95(Carpenter/Clouston diagnostic criteria)

i. 1hr later plasma glucose ≥ 180mg/dl.or

ii. 2hr later plasma glucose ≥ 155mg/dl.or

iii. 3hr later plasma glucose ≥ 140mg/dl

9). Tropical diabetes: Diabetes mellitus associated with chronic malnutrition and, sometimes, chronic pancreatitis. Also called malnutrition-related diabetes. Chronic pancreatitis patient are usually in Africa where they consume Casava. That leads to pancreatic calcification and pain abdomen.

10). Mitochondrial Gene defects: Point mutations in mitochondrial DNA are sometimes associated with DM and deafness. A form of IDDM, associated with mitochondrial mutations, is the wolfram syndrome. Wolfram syndrome is characterized by diabetes insipidus, DM, optic atrophy, and deafness – thus, the acronym DIDMOAD.

11). Genetic defects of insulin action:

a). Leprechaunism

b). Rabson Mendenhall syndrome

c). Cystic fibrosis

AutoImmune disease having associated diabetes

a). Chronic lymphocytic thyroiditis (Hashimoto)

b). Celiac disease

c). Multiple endocrine deficiency syndrome

Endocrine Flashcard List

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