Connect
MJA
MJA

Paediatric diabetes — which children can gain insulin independence?

Shubha Srinivasan and Kim C Donaghue
Med J Aust 2007; 186 (9): . || doi: 10.5694/j.1326-5377.2007.tb00991.x
Published online: 7 May 2007

Molecular genetics can facilitate a successful switch to oral diabetes therapy

The increase in type 1 and type 2 diabetes in childhood has been well documented worldwide and in Australia.1,2 In addition, the separate entity of monogenic diabetes is increasingly recognised in paediatric diabetes, and now encompasses neonatal diabetes mellitus and maturity onset diabetes of the young (MODY)3 (Box 1). Monogenic diabetes is defined as diabetes caused by a single gene defect. A diagnosis of monogenic diabetes should be considered in a child who is diabetes-associated-autoantibody negative, is diagnosed with diabetes in the first 6 months of life, has a parent with diabetes, and/or is not markedly obese. Although uncommon — its frequency is estimated to be 1%–3% of all childhood diabetes3 — the clinical relevance of this condition is that at least some of those affected (in particular, those with MODY1 and MODY3) can achieve very good diabetes control with sulfonylurea rather than insulin therapy.


  • 1 Institute of Endocrinology and Diabetes, The Children’s Hospital at Westmead, Sydney, NSW.
  • 2 University of Sydney, Sydney, NSW.


Correspondence: KimD@chw.edu.au

  • 1. Taplin CE, Craig ME, Lloyd M, et al. The rising incidence of childhood type 1 diabetes in New South Wales, 1990–2002. Med J Aust 2005; 183: 243-246. <MJA full text>
  • 2. McMahon SK, Haynes A, Ratnam N, et al. Increase in type 2 diabetes in children and adolescents in Western Australia. Med J Aust 2004; 180: 459-461. <MJA full text>
  • 3. Craig ME, Hattersley A, Donaghue K; International Society for Pediatric and Adolescent Diabetes. ISPAD clinical practice consensus guidelines 2006–2007. Definition, epidemiology and classification. Pediatr Diabetes 2006; 7: 343-351.
  • 4. von Muhlendahl KE, Herkenhoff H. Long-term course of neonatal diabetes. N Engl J Med 1995; 333: 704-708.
  • 5. Gloyn AL, Pearson ER, Antcliff JF, et al. Activating mutations in the gene encoding the ATP-sensitive potassium-channel subunit Kir6.2 and permanent neonatal diabetes. N Engl J Med 2004; 350: 1838-1849.
  • 6. Proks P, Arnold AL, Bruining J, et al. A heterozygous activating mutation in the sulphonylurea receptor SUR1 (ABCC8) causes neonatal diabetes. Hum Mol Genet 2006; 15: 1793-1800.
  • 7. Babenko AP, Polak M, Cave H, et al. Activating mutations in the ABCC8 gene in neonatal diabetes mellitus. N Engl J Med 2006; 355: 456-466.
  • 8. Pearson ER, Flechtner I, Njolstad PR, et al; Neonatal Diabetes International Collaborative Group. Switching from insulin to oral sulfonylureas in patients with diabetes due to Kir6.2 mutations. N Engl J Med 2006; 355: 467-477.
  • 9. Zung A, Glaser B, Nimri R, Zadik Z. Glibenclamide treatment in permanent neonatal diabetes mellitus due to an activating mutation in Kir6.2. J Clin Endocrinol Metab 2004; 89: 5504-5507.
  • 10. Hattersley A, Bruining J, Shield J, et al; International Society for Pediatric and Adolescent Diabetes. ISPAD clinical practice consensus guidelines 2006–2007. The diagnosis and management of monogenic diabetes in children. Pediatr Diabetes 2006; 7: 352-360.

Author

remove_circle_outline Delete Author
add_circle_outline Add Author

Comment
Do you have any competing interests to declare? *

I/we agree to assign copyright to the Medical Journal of Australia and agree to the Conditions of publication *
I/we agree to the Terms of use of the Medical Journal of Australia *
Email me when people comment on this article

Online responses are no longer available. Please refer to our instructions for authors page for more information.