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Endocrine Reviews, doi:10.1210/er.2007-0029
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Endocrine Reviews 29 (3): 265-291
Copyright © 2008 by The Endocrine Society

Neonatal Diabetes Mellitus

Lydia Aguilar-Bryan and Joseph Bryan

Pacific Northwest Diabetes Research Institute, Seattle, Washington 98122

Correspondence: Address all correspondence and requests for reprints to: Lydia Aguilar-Bryan and Joseph Bryan, Pacific Northwest Diabetes Research Institute, 720 Broadway, Seattle, Washington 98122. E-mail: lbryan{at}pnri.org or jbryan{at}pnri.org

An explosion of work over the last decade has produced insight into the multiple hereditary causes of a nonimmunological form of diabetes diagnosed most frequently within the first 6 months of life. These studies are providing increased understanding of genes involved in the entire chain of steps that control glucose homeostasis. Neonatal diabetes is now understood to arise from mutations in genes that play critical roles in the development of the pancreas, of β-cell apoptosis and insulin processing, as well as the regulation of insulin release. For the basic researcher, this work is providing novel tools to explore fundamental molecular and cellular processes. For the clinician, these studies underscore the need to identify the genetic cause underlying each case. It is increasingly clear that the prognosis, therapeutic approach, and genetic counseling a physician provides must be tailored to a specific gene in order to provide the best medical care.




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Copyright © 2008 by The Endocrine Society