The inclusion of "diabetes mellitus" as part of pediatric endocrinology is a relatively recent concept. The first two editions (1950 and 1957) of Lawson Wilkins' classic text "The Diagnosis and Treatment of Endocrine Disorders in Childhood and Adolescence" only mentioned diabetes mellitus in pregnancy in the context of neonatal hypoglycemia. Wilkins' third edition, published just after his death in 1965 devoted less than a page to diabetes.
Later, we began to recognize "juvenile onset" diabetes as a pediatric-appropriate inclusion in textbooks, and now we realize that that designation includes many subtypes of diabetes, including classic Type 1 but also MODY and other forms. What was known at one time as "adult-onset" or "non-insulin-dependent" diabetes is neither limited to adults, nor is necessarily non-insulin dependent, and is now more appropriately designated Type 2 diabetes.
The editors therefore enlisted a village of experts in the various aspects of "Diabetes Mellitus" to contribute to this chapter, which, of necessity, is given in four parts: (A). Introduction; (B). Type 1 Diabetes; (C). Type 2 Diabetes; and (D). Other specific types of diabetes and causes of hyperglycemia.
Selected Readings will be listed at the end of each part.
A majority of cases of diabetes mellitus fall into one of two categories: (1) type 1 diabetes, which is most commonly caused by autoimmune destruction of the pancreatic islet cells resulting in β-cell failure and an absolute deficiency of insulin (type 1 A diabetes, T1DM), and (2) type 2 diabetes (T2DM), which results from a combination of insulin resistance in target tissues and a lack of an adequate compensatory insulin response to overcome this resistance. The American Diabetes Association (ADA)1 recognizes more than 50 other specific types of diabetes (Table 10-1). All of these disorders collectively account for only 1% to 5% of cases of diabetes (see "Part D" of this chapter later on).
Table 10-1. Classification of Diabetes Mellitus |Favorite Table|Download (.pdf)
Table 10-1. Classification of Diabetes Mellitus
|Initial presentation in patients < 6 mo old|
Genetic defects in insulin action
- Rabson-Mendenhall syndrome
- Donahue syndrome (Leprechaunism)
|Initial presentation in patients > 6 mo old|
Type 1 diabetes
- Slowly progressive type 1 diabetes
Type 2 diabetes
Atypical diabetes (Flatbush)
Maturity onset diabetes of youth (MODY)
Genetic defects in insulin action
- Type A insulin resistance
- Lipoatrophic diabetes
Diseases of the exocrine pancreas
- Cystic fibrosis
- Cushing syndrome
Drug- or chemical-induced
Uncommon forms of immune-mediated diabetes
- "Stiff man" syndrome
- Type B insulin resistance
- Autoimmune hypoglycemia
Genetic syndromes associated with diabetes
- Down syndrome
- Klinefelter syndrome
- Turner syndrome
- Wolfram syndrome
- Friedreich ataxia
- Huntington chorea
- Laurence-Moon-Biedl syndrome
- Myotonic dystrophy
- Prader-Willi syndrome
Gestational diabetes ...
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