Definition, epidemiology, diagnosis and classification

Other specific types

Diseases of endocrine pancreas

Cystic fibrosis-related diabetes

  • Longer survival of cystic fibrosis (CF) patients results in 10–30% of 15- to 25-year-olds developing diabetes
  • Screening for hyperglycemia, glycosuria and/or HbA1c is recommended as part of the annual review of CF patients and particularly if steroid treatment is used
  • Insulin treatment will improve hyperglycemia and help to prevent catabolic weight loss in CF especially during intercurrent infections
  • High dietary energy intake is recommended including high fat and high complex carbohydrate

Thalassemia

  • Iron overload affecting beta-cell function and the decreasing insulin sensitivity of puberty are thought to contribute to the risk of diabetes in young people with thalassemia
  • Some studies suggest that treatment with high doses of insulin is required if iron levels remain high

Fibrocalculous pancreatopathy and other diabetes in developing countries

  • Atypical diabetes in young people occurs in some developing countries
  • Previously named malnutrition-related diabetes mellitus (MRDM), it may be characterized by lean body mass, relative ketosis resistance and superimposed nutritional deficiencies
  • There may be calcification of the pancreas, or protein deficiency pancreatic diabetes
  • The ketosis ‘resistance’ may reflect later onset, less severe type 1 diabetes in children who survive long enough to reach medical attention

Other genetic syndromes associated with diabetes

Diabetes Insipidus, Diabetes Mellitus, Optic Atrophy, Deafness (DIDMOAD) syndrome (Wolfram syndrome)

  • Insulin-dependent diabetes mellitus usually presenting in the first decade
  • Insidious onset of diabetes insipidus, deafness and visual impairment
  • Non-autoimmune genetic condition, usually autosomal recessive
  • Potentially progressive deterioration in neurological function with cerebral atrophy and psychological disturbances in the third or fourth decade
  • Gene localized to chromosome 4p

Prader-Willi syndrome

  • Severe obesity is associated with a high incidence of secondary diabetes in the second decade
  • Weight reduction is extremely difficult
  • The diabetes is type 2, usually responding poorly to oral hypoglycemic agents; most patients require insulin


 
 
Consensus Guidelines 2000
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IDF Type 1 Guidelines
IDF Type 2 Guidelines