Duplication of the DAX1 gene on the X chromosome with normal sex determining region of Y (SRY) results in 46 XY sex reversal. This was inherited from the mother who had normal ovarian function. Additional problems include growth failure, mental retardation and multiple congenital anomalies. The baby did not have a mutation or deletion of DAX1, which would have caused adrenal insufficiency and hypogonadism.

Despite the recent advances in treatment and technology, intensive insulin therapy for treatment of type 1 diabetes remains a challenge in children due to developmental issues, wide glycemic fluctuations and unpredictable eating and activity patterns. Many children with diabetes nowadays use a basal-bolus regimen by means of either multiple daily injections or CSII in an attempt to mimic closely the physiological insulin and serum glucose profiles in order to achieve optimal glycemic control. This article reviews the different regimens employed to treat type 1 diabetes in children, using short-and intermediateacting insulins, newer insulin analogues, as well as continuous subcutaneous insulin infusion (CSII). With the ongoing development of new technologies allowing better monitoring of blood glucose and insulin delivery, it is hoped that in the near future a ''closed loop system'' will help in achieving normal glycemic control while minimizing side effects related to administration of exogenous insulin. Drug Dev Res 69: 158-164, 2008.

The metabolic syndrome is a cluster of metabolic disturbances that result in an increased risk of type 2 diabetes and cardiovascular disease in adults. Despite the lack of a uniform definition of the syndrome for children, several studies have reported an overall prevalence of 3 to 4% among children. Among obese adolescents, the prevalence can be as high as 30 to 50%. Besides insulin resistance and obesity, the intrauterine environment and genetic factors also seem to play a role in the pathogenesis of the syndrome in children. In view of the current obesity epidemic and since an increasing amount of evidence shows that obesity during adolescence is significantly associated with insulin resistance, abnormal serum lipid levels, and elevated blood pressure during adulthood, there is a great need for a clear definition, for the development of screening guidelines, and for appropriate prevention and treatment strategies for the metabolic syndrome in the pediatric population. Drug Dev. Res. 67:602-606, 2006.

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