Quality Standards for Diabetes Care Toolkit



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Guidelines

Monogenic diabetes


In the European report on treatment and care of diabetes in children by the SWEET group, it is suggested that while in the past many patients with monogenic forms of diabetes have been diagnosed incorrectly as either type 1 diabetes (T1DM) or type 2 diabetes (T2DM) dependent on the age of hyperglycaemia detection, current diagnostic availability should allow specific diagnosis (SWEET 2010).
Genetic testing should be available in all centres either locally or nationally, allowing for specific treatment depending on the genetic defect. Treatment may be as simple as annual follow-up without diet or medication, to intensive diet and/or sulphonylurea or insulin (SWEET 2010). There may be progressive beta-cell deficiency in some children with transcription factor mutations therefore creating the need for oral hypoglycaemic agents. HNF-1α variants may exhibit sensitivity to small doses of sulfonylureas (with the risk of hypoglycaemia) and may require insulin treatment managed by a specialist team but this is usually in adulthood (International Society for Pediatric and Adolescent Diabetes 2000).
Genetic testing for monogenic diabetes is recommended under the care of an expert diabetologist or endocrinologist (New Zealand Society for the Study of Diabetes 2012).
The NZSSD Monogenic Guidelines can be found here: www.nzssd.org.nz/education/2013%20Monogenic_diabetes_card_with_forms_18%20Dec%20copy.pdf


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