Intensive insulin therapy by either insulin pump or MDI is safe in children and young adolescents with type 1 diabetes, with similar diabetes control and a very low rate of adverse events. We suggest that both modes be available to the diabetic team to better tailor therapy.

The risk of a mother with 21-OH-deficient NCAH for giving birth to a child affected with CAH is 2.5%; at least 14.8% of children born to these mothers have NCAH.

Continuous subcutaneous insulin infusion improves glycemic control in youth with type 1 diabetes, especially in those with a history of poor glycemic control. This improvement is associated with a decrease in the rate of severe hypoglycemia in the absence of weight gain.

High serum ferritin levels during puberty are a risk factor for hypogonadism, and high serum ferritin levels during the first decade of life predict final short stature. It remains to be determined whether improving chelation by earlier initiation of DFO or by the combined use of DFO and deferiprone will lead to better growth and sexual development without DFO toxicity.

Background: Though autoimmune phenomena have been regularly associated with chronic urticaria in adults, data in children are sparse. Aim: To describe our experience with children and adolescents with chronic urticaria and autoimmunity. Methods and Results: Of 187 patients referred for evaluation of chronic urticaria during a 7.5 year period, eight (4.3%), all females aged 7-17 years, had increased levels of antithyroid antibody, either antithyroid peroxidase antibody (n = 4, >75 IU/ml), antithyroglobulin antibody (n = 2, >150 IU/ml), or both (n = 2). The duration of urticaria was four months to seven years. Five patients were euthyroid, one of whom was found to have increased antithyroid antibody levels five years after onset of the urticaria. One patient was diagnosed with Hashimoto thyroiditis three years before the urticaria, and was receiving treatment with thyroxine. Two other hypothyroid patients were diagnosed during the initial work up for urticaria (thyroxine 9.2 pmol/l, thyroid stimulating hormone (TSH) 40.2 mIU/l) and five years after onset of the urticaria (thyroxine 14 pmol/l, TSH 10.3 mIU/l). Both were treated with thyroxine but neither had remission of the urticaria. Five patients had a low positive titre of antinuclear antibodies. Conclusion: Children with chronic urticaria should be screened periodically for thyroxine, TSH, and antithyroid antibodies, as thyroid autoimmunity and hypothyroidism may appear several years after onset of the urticaria.

ABSTRACT. Background. The use of insulin pumps is becoming a popular technique for insulin delivery among patients with type 1 diabetes mellitus (T1DM), but there is no consensus regarding the guidelines for proper pump use during exercise.Objective. To investigate the physiologic responses and risk of hypoglycemia among children and adolescents with T1DM when exercising with the pump on (PO) (50% of the basal rate) or pump off (PF).Methods. Ten subjects with T1DM (6 female subjects and 4 male subjects), 10 to 19 years of age, performed prolonged exercise (40 -45 minutes) on a cycle ergometer ϳ2 hours after a standard breakfast and an insulin (Lispro) bolus. Complex carbohydrates (20 g) were provided before and after the exercise. Each patient exercised once with PO and once with PF, in a randomized, crossover (single-blind) manner. During exercise and 45 minutes of recovery, subjects were monitored for cardiorespiratory, metabolic, and hormonal responses. Blood glucose concentrations were recorded for 24 hours after exercise, with a continuous glucose monitoring system, to document late hypoglycemic events.Results. During exercise, blood glucose concentrations decreased by 59 ؎ 58 mg/dL (mean ؎ SD: 29 ؎ 24%) with PF and by 74 ؎ 51 mg/dL (35.5 ؎ 18%) with PO (not significant). No significant differences were found in cortisol, growth hormone, or noradrenaline levels between PO and PF. There were no differences in cardiorespiratory parameters, blood lactate concentrations, or free fatty acids concentrations between pump modes. Hypoglycemic events during exercise were asymptomatic and occurred for 2 subjects with PO and 2 with PF. Nine subjects had late hypoglycemia after PO, compared with 6 after PF (not significant).Conclusions. We found no advantage for subjects with either PO or PF during exercise, and we noted that late hypoglycemia was more common than hypoglycemia during exercise. However, PO was associated with a trend of increased risk for late hypoglycemia. We recommend that the pump be removed or turned off during prolonged exercise and that blood glucose concentrations be monitored for several hours after exercise, regardless of the pump mode.

WHAT'S KNOWN ON THIS SUBJECT: XY disorders of sex development have a diverse etiology and often present with atypical genitalia in the newborn period. Sex assignment in those cases in whom this is marked genital ambiguity is a rare, challenging situation that requires multidisciplinary input. WHAT THIS STUDY ADDS:An international registry has shown temporal changes over the last 3 decades in the practice of sex assignment with a greater proportion of severely affected infants being raised as boys, raising the need for long-term monitoring of these children. abstract BACKGROUND AND OBJECTIVE: It is unclear whether the proportion of infants with a disorder of sex development who are raised as male or female has changed over time. The temporal trends in sex assignment of affected cases entered in the International Disorder of Sex Development (I-DSD) Registry were studied. METHODS:Cases of disorders of sex development reported as partial androgen insensitivity syndrome (PAIS; n = 118), disorder of gonadal development (DGD; n = 232), and disorder of androgen synthesis (DAS; n = 104) were divided into those who were born before 1990, 1990-1999, and after 1999. External appearance of the genitalia was described by the external masculinization score. RESULTS:The median (5th-95th percentile) external masculinization scores of those infants with PAIS, DGD, and DAS who were raised as boys were 6 (2-9), 6 (3-9), and 6 (1-12), respectively, and were significantly higher than in those raised as girls (2 [0-6], 2 [0-7], and 0 [0-5], respectively); this difference was maintained in the 3 temporal birth cohorts (P , .01). Of the 118 cases in the pre-1990 cohort, 41 (35%) were raised as boys; of the 148 cases in the 1990-1999 cohort, 60 (41%) were raised as boys; and of the 188 cases in the post-1999 cohort, 128 (68%) were raised as boys.

One-year treatment with DiaPep277 at a dosage of 1 mg is safe for use and well tolerated in children with recent-onset T1DM. However, it appears to have no beneficial effect in preserving beta-cell function or improving metabolic control.