ISPAD Clinical Practice Consensus Guidelines 2018: The diagnosis and management of monogenic diabetes in children and adolescents

ISPAD Clinical Practice Consensus Guidelines 2018: Definition, epidemiology, and classification of diabetes in children and adolescents

“…Characteristic features of youth onset type 1 diabetes in comparison with type 2 diabetes and monogenic diabetes are shown in Table . Type 2 diabetes and monogenic diabetes are more completely discussed in the ISPAD guidelines on these conditions …”

“…The most common form is associated with mutations in the transcription factor hepatocyte nuclear factor (HNF)‐1α (also known as HNF1‐MODY). Mutations in the glucokinase gene ( GCK ) and HNF4A contribute to the majority of remaining cases, while rare forms result from mutations in other transcription factors, including HNF‐1B , pancreatic‐duodenal homeobox ( PDX‐1 ), and NeuroD1 ; for further detail see ISPAD guideline on Monogenic diabetes …”

“…A monogenic form of diabetes in the first 6 months of life is known NDM, although cases may present as late 9 to 12 months of age . Further details of the genetic basis of NDM are provided in the ISPAD guideline on Monogenic Diabetes …”

Identification of insulin gene variants in neonatal diabetes

“…The diagnosis of PNDM was in accordance with the classification of the American Diabetes Association based on history and clinical features [16]; patients were ketosis prone, lacked endogenous insulin and required insulin to control their hyperglycaemia. HbA1c was measured using the turbidimetric inhibition immunoassay (TINIA) principle on Dimension Xpand Plus (Siemens Healthcare, Germany); the %HbA1c results were standardised to the results obtained in the Diabetes Control and Complications Trial (DCCT).…”

ISPAD Clinical Practice Consensus Guidelines 2018: What is new in diabetes care?

“…At the same time, the different types of insulin analogs have increased, albeit at considerable expense, and technology use, including pumps, sensors, and automated insulin delivery systems, has risen in all age groups in countries where these treatment modalities are accessible. Advances in the genetic diagnosis of atypical diabetes have guided decisions regarding the best treatment for many children . Type 2 diabetes in youth, a consequence of the obesity epidemic, has become widespread in many regions of the world and the evidence base for treatment of this disorder has expanded significantly since 2014 .…”