Intractable neonatal seizures: an unusual presentation of congenital hypothyroidism.

Congenital hypothyroidism is the most common treatable cause of mental retardation. We report an unusual case of congenital hypothyroidism presenting as intractable seizures in an infant delivered to a mother known to have autoimmune hypothyroidism and who was noncompliant with therapy. To our knowledge, this rare presentation of congenital hypothyroidism has not been reported previously.

Effect of insulin with oral nutrients on whole-body protein metabolism in growing pubertal children with type 1 diabetes.

Insulin treatment of children with insulin-dependent diabetes mellitus improves whole body protein balance. Our recent study, conducted in pubertal children with type 1 diabetes with provision of both insulin and amino acids, indicated a positive effect of insulin on protein balance, primarily through decreased protein degradation. The current study was undertaken to assess the effect of insulin on protein metabolism in adolescents with type 1 diabetes during oral provision of a complete diet. Whole-body protein metabolism in six pubertal children (13-17 y) with type 1 diabetes mellitus was assessed with L-[1-13C]leucine during a basal (insulin-withdrawn) period and during infusion of 0.15 U/kg/h regular insulin with hourly meals to meet protein and energy requirements. Net leucine balance was significantly higher with insulin and nutrients (13.1 +/- 6.3 micromol leucine/kg/h) than in the basal state (-21.4 +/- 2.8, p < 0.01) with protein degradation decreased from 138 +/- 5.6 mumol leucine/kg/h to 108 +/- 5.9 (p < 0.01) and no significant change in protein synthesis. Even with an ample supply of nutrients, insulin does not increase whole-body protein synthesis in pubertal children with type 1 diabetes mellitus and positive protein balance is solely due to a substantial reduction in the rate at which protein is degraded.

Accuracy of self-reported height measurements in parents and its effect on mid-parental target height calculation.

BACKGROUND: Clinical determination of mid-parental height is an important part of the assessment of a child's growth, however our clinical impression has been that parents cannot be relied upon to accurately report their own heights. Therefore, we conducted this study to assess the accuracy of parental height self-reporting and its effect on calculated mid-parental target height for children presenting to a pediatric endocrinology office. METHODS: All parents bringing their children for an initial evaluation to a pediatric endocrinology clinic over a period of nine months were questioned and then measured by a pediatric endocrinologist. Parents were blinded to the study. Mid-parental target heights, based on reported and actual height were compared. RESULTS: There were 241 families: 98 fathers and 217 mothers in our study. Mean measured paternal height was 173.2 cm, self reported 174.9 cm (p < 0.0001), partner reported 177 cm (p = 0.0004). Only 50% of fathers and 58% of mothers reported their height within +/- 2 cm of their measured height, while 15% of fathers and 12% of mothers were inaccurate by more than 4 cm. Mean measured maternal height was 160.6 cm, self-reported 161.1 cm (NS), partner reported 161.7 cm (NS). Inaccuracy of height self-report had a small but significant effect on the mean MPTH (0.4 cm, p = 0.045). Analysis showed that only 70% of MPTH calculated by reported heights fell within +/- 2 cm of MPTH calculated using measured heights, 24% being in +/- 2-4 cm range, and 6% were inaccurate by more than 4 cm. CONCLUSION: There is a significant difference in paternal measured versus reported heights with an overall trend for fathers to overestimate their own height. A large subset of parents makes a substantial error in their height self-report, which leads to erroneous MPTH. Inaccuracy is even greater when one parent reports the other parent's height. When a child's growth is in question, measured rather than reported parental heights should be obtained.