Secondary Diabetes Mellitus Other Specific Types

This broad category includes multiple disorders that are associated with either extensive pancreatic destruction or significant insulin resistance. Secondary diabetes as a consequence of decreased insulin production can occur following pancreatectomy, chronic pancreatitis, cystic fibrosis, or hemochro-matosis. In the absence of pancreatic damage, secondary diabetes can result from extreme insulin resistance induced by glucocorticoids (Cushing's syndrome); growth hormone (acromegaly); adrener-gic hormones (pheochromocytoma); other medical conditions, such as uremia, hepatic cirrhosis, or polycystic ovary syndrome; or medications (diuretics or exogenous glucocorticoids).

Included in this category of secondary diabetes are patients who appear to have type 2 diabetes but in whom monogenic molecular defects in either the glucose-sensing or insulin action pathways have been defined. The best established molecular defects are mutations in the gene coding for the enzyme glucokinase, which has a role in the sensing of glucose by the fi cell. Individuals with this autosomal dominant condition usually develop mild diabetes in early adulthood or adolescence. Hence, the condition is known as maturity onset diabetes of the young (MODY). Several other types of MODY have been defined, due to gene defects in fi cell transcription factors. Rare causes of diabetes secondary to insulin resistance include various inborn errors of metabolism (e.g., insulin receptor mutations or type 1 glycogen storage disease), chromosomal abnormalities such as Down's and Turner's syndrome, and muscle diseases (e.g., myotonic dystrophy).

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