Thyrotoxic, hypokalemic periodic paralysis (THPP) in adolescents.

INTRODUCTION: Periodic paralysis associated with hyperthyroidism and hypokalemia is an uncommon disorder reported primarily in Asian males and rarely in children. We report three Hispanic adolescent males who were seen with Graves' disease (GD) and THPP. METHODS: The method used was chart review. RESULTS: Two of these boys presented with episodes of paralysis and were diagnosed with GD. The third was initially seen with hyperthyroidism and developed weakness and paralysis when his disease progressed because of therapeutic noncompliance. Hypokalemia was documented in two of the three patients with the third not seen during paralysis. Intravenous K⁺ was required in only one case. All three boys were treated with antithyroid medications and ß blockers, and the musculoskeletal symptoms resolved in all three when hyperthyroidism was controlled after 2 weeks of treatment. The duration for each episode of weakness and paralysis varied in each case and resolved within 15 min to 2 h in case 1, 1-5 h in case 2, and 24 h in case 3. CONCLUSIONS: THPP is considered uncommon except in Asian males and rare in childhood and adolescence. Its occurrence in these three Hispanic boys suggests that it may occur more frequently in the young and in the USA than has been suspected, especially with the changing national demographics. We believe that our experience should raise the awareness of THPP among pediatric care providers.

Unawareness of the effects of soy intake on the management of congenital hypothyroidism.

It has been established that soy products can interfere with thyroid hormone absorption resulting in continued hypothyroidism in individuals receiving recommended levothyroxine replacement. It has also been reported that achievement of euthyroidism in hypothyroid patients using soy products requires increased doses of levothyroxine. We have observed 2 patients with congenital hypothyroidism who continued to manifest clinical hypothyroidism while receiving recommended doses of hormone and ingesting soy products. The first patient was diagnosed by newborn screening (thyroid-stimulating hormone [TSH] =169 µIU/mL) and treated with 50 µg of levothyroxine since 6 days of age while simultaneously starting soy formula. At 3 weeks of age, she was clinically and biochemically hypothyroid (thyroxine = 4.0 µg/dL, TSH = 216 µIU/mL). We stopped her soy formula and decreased her levothyroxine dose. Three weeks later signs of hypothyroidism were resolving, and, by 10 weeks of age, she was clinically and biochemically euthyroid. Another patient was diagnosed by newborn screening, received levothyroxine, and did well. She was lost to us for 2 years. During this interval she began consuming soy milk and became profoundly hypothyroid (free thyroxine <0.4 ng/dL, TSH = 248 µIU/mL), even though the primary care physician had increased her levothyroxine dose to 112 µg/day. She was switched to cow milk, and her thyroid function slowly normalized with decreasing doses of levothyroxine. These 2 patients reinforce the importance of remembering that soy products interfere with levothyroxine absorption and can endanger infants and young children with congenital hypothyroidism who are at risk for developmental and growth delay.

The "other" forms of diabetes in children.

The large majority of children with diabetes seen by any pediatrician or in any pediatric diabetes clinic will fall into the two major categories of type 1 (T1DM) and, more recently in adolescents, type 2 (T2DM). But one has only to look at the diabetes classifications currently proposed by the American Diabetes Association or the World Health Organization to see that these forms account for only two of the many forms of diabetes listed. The goal of this chapter is to discuss, very briefly, the other classes of diabetes which occur in children and are of importance to physicians caring for adolescents.

Role of obesity in complicating and confusing the diagnosis and treatment of diabetes in children.

The alarming increase in the prevalence of obesity in children in the United States and globally raises major concerns about its future adverse impact on public health. One outcome of this disturbing trend that is already evident is the rapidly increasing incidence of type 2 diabetes at all ages. This disease, once thought to be nonexistent in children, is increasing coincident with obesity. This article addresses the role that obesity plays in type 2 diabetes and also explores its effects on other types of diabetes that occur in childhood. The new challenges for physicians who formulate a differential diagnosis of diabetes in children are discussed. Also examined are modifications of traditional diabetes treatment that can be helpful in combating the insulin resistance associated with obesity and that use medications that are not traditionally used in this age group. Cases are presented to illustrate certain points. An underlying thesis suggests that specific classification may not be as important to the clinician as the understanding of pathophysiologic factors that contribute to hyperglycemia in individual patients. Recommendations are offered to the practitioner for diagnosing and treating the obese child or adolescent with diabetes.

Endocrine abnormalities in patients with Jacobsen (11q-) syndrome.

Jacobsen syndrome (JS), a rare disorder with multiple dysmorphic features, is caused by the terminal deletion of chromosome 11q. Short stature has been reported in this syndrome, however very few of these patients have undergone endocrine evaluation. Serum insulin-like growth factor-1 (IGF-1) levels are an indirect indicator of growth hormone activity and are a useful initial screening tool in the assessment of an individual's growth hormone axis. We studied nine children with JS, eight of whom had short stature. Four out of eight children with short stature (50%) had low IGF-1 values, with three low for age and one low for Tanner stage. Four out of six males (67%) had cryptorchidism, a potential sign of hypogonadism. We conclude that low IGF-1 is common in patients with JS and short stature, and that growth hormone status and possibly hypothalamic-pituitary function should be evaluated in this patient population.

Virilization of young children after topical androgen use by their parents.

Children were virilized by contact with adults using cutaneous steroid preparations. Parents were unaware of the dangers of passive transfer. Laboratory data were consistent with exogenous androgen exposure. Each child had opportunity for passive exposure, and discontinuation of contact resulted in a decrease of androgen levels or regression of symptoms.

Type 2 diabetes mellitus in adolescence: lipid and cardiovascular risk factors.

This review discusses the important consequences of dyslipidemia and arteriosclerosis in type 2 diabetes as documented in studies in adults. It then examines the relatively recent upsurge in type 2 diabetes in children and adolescents, its characteristics, and its importance in directing our attention to cardiovascular risk factors in this age group. The discussion concludes with an examination of the information available about arteriosclerosis in the young and about the treatment of hypercholesterolemia in children and adolescents.

Effect of metformin in pediatric patients with type 2 diabetes: a randomized controlled trial.

OBJECTIVE: Metformin is the most commonly prescribed oral antidiabetic agent in the U.S. for adults with type 2 diabetes. The incidence of type 2 diabetes in children has increased dramatically over the past 10 years, and yet, metformin has never been formally studied in children with type 2 diabetes. RESEARCH DESIGN AND METHODS: This study evaluated the safety and efficacy of metformin at doses up to 1,000 mg twice daily in 82 subjects aged 10-16 years for up to 16 weeks in a randomized double-blind placebo-controlled trial from September 1998 to November 1999. Subjects with type 2 diabetes were enrolled if they had a fasting plasma glucose (FPG) levels > or =7.0 and < or =13.3 mmol/l (> or =126 and < or =240 mg/dl), HbA(1c) > or =7.0%, stimulated C-peptide > or =0.5 nmol/l (> or =1.5 ng/ml), and a BMI > 50th percentile for age. RESULTS: Metformin significantly improved glycemic control. At the last double-blind visit, the adjusted mean change from baseline in FPG was -2.4 mmol/l (-42.9 mg/dl) for metformin compared with +1.2 mmol/l (+21.4 mg/dl) for placebo (P < 0.001). Mean HbA(1c) values, adjusted for baseline levels, were also significantly lower for metformin compared with placebo (7.5 vs. 8.6%, respectively; P < 0.001). Improvement in FPG was seen in both sexes and in all race subgroups. Metformin did not have a negative impact on body weight or lipid profile. Adverse events were similar to those reported in adults treated with metformin. CONCLUSION: Metformin was shown to be safe and effective for treatment of type 2 diabetes in pediatric patients.