The incidence of overweight and obese children has been increasing worldwide over the past several decades. 1-3 Additionally, health problems caused by obesity, such as type 2 diabetes mellitus (T2D) and metabolic syndrome (MS), have become a serious problem. 1,2 In Japanese children, the incidence of obesity tripled between the 1970s and around 2000, although a tendency towards a slight
Heterozygous activating mutations of KCNJ11 (Kir6.2) are the most common cause of permanent neonatal diabetes mellitus (NDM), and successful glycemic control has been obtained in several cases with oral sulfonylureas (SU). We have verified a lack of clinical response for both glycemic control and neurological features in an infant with permanent neonatal diabetes mellitus and DEND syndrome due to a V59A mutation in the KCNJ11 gene. Thus, our case reinforces that most cases with DEND syndrome are insensitive to SU.
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