Auxological and endocrinological features in internationally adopted children.

In internationally adopted children disorders of linear growth, puberty development, thyroid function, and bone metabolism are frequently reported. It is important that these children receive careful auxological and endocrinological evaluations and follow-up.Pediatricians and other healthcare providers should be aware that auxological and endocrinological problems are common in newly arrived international adoptees.

Bone modeling, remodeling, and skeletal health in children and adolescents: mineral accrual, assessment and treatment.

The modeling and remodeling process of the bone is fundamental to maintaining its integrity and mechanical properties. Many physical and biochemical factors during childhood and adolescence are crucially important for the development of healthy bones. Systemic conditions, such as hormonal status, nutrition, physical inactivity, or many pharmacological treatments, as well as a local variation in the load, can influence bone turnover and, consequently, the attainment of a proper peak bone mass. However, many diseases affecting children and adolescents can be associated with a reduction in bone accrual or a loss of bone mass and quality, which leads to an increased risk of fracture over one's life. In this review, we examine the effects of genetics, physical activity, chronic diseases and pharmacological treatments, and dietary factors on bone integrity in children and adolescents. We also briefly describe the specific tools that are currently used in assessing bone health.

HYDROCORTISONE THERAPY AND GROWTH TRAJECTORY IN CHILDREN WITH CLASSICAL CONGENITAL ADRENAL HYPERPLASIA.

OBJECTIVE: Poor linear growth is one of the main concerns in children with congenital adrenal hyperplasia (CAH). We aimed to analyze factors affecting growth trajectory in children with classical CAH. METHODS: Clinical records of children followed from infancy up to the end of growth at two Italian tertiary referral hospitals were reviewed. Fifty-seven patients (31 males), treated with hydrocortisone and fludrocortisone only, were included. Clinical observations were divided into three groups: 0 to 2 years, 172 observations; from 2 years to puberty onset, 813 observations; after puberty onset, 527 observations. Height velocity, pubertal growth spurt, and final height were evaluated as outcomes. RESULTS: Final height standard deviation score (SDS) was lower than target height SDS (-0.74 ± 1.1 versus -0.31 ± 1.01; P<.001). Target-adjusted final height SDS was -0.44 ± 1.8 in males and -0.13 ± 1.1 in females (P = .001). Total pubertal growth was 21.9 ± 7.3 cm in males and 19.2 ± 8.2 cm in females (P = .19). Hydrocortisone dose increased and height-velocity SDS decreased during puberty. At multivariable analysis, height-velocity SDS was adversely affected by hydrocortisone dose (P = .038) and directly related to adrenocorticotropic hormone (ACTH) levels (P = .023). Target-adjusted final-height SDS was adversely affected by hydrocortisone dose (P<.001) and positively related to mineralocorticoid therapy (P = .001) and ACTH levels (P = .02). Total pubertal growth (cm) was positively related to ACTH levels (P = .01). CONCLUSION: Height outcome of CAH patients is now better than previously reported. During puberty, the lowest effective dose of hydrocortisone should be used to optimize pubertal growth spurt and final height. ABBREVIATIONS: 17-OHP = 17-alpha-hydroxyprogesterone ACTH = adrenocorticotropic hormone BMI = body mass index CAH = congenital adrenal hyperplasia GH = growth hormone HPA = hypothalamus-pituitary-adrenal PRA = plasma renin activity SDS = standard deviation score SV = simple virilizing SW = salt-wasting.

Long-term first line medical treatment in a 4-year-old girl with Xq26.3 microduplication-negative somatotropinoma. Case report and literature review.

Growth hormone (GH) secreting adenoma represents a therapeutic challenge in childhood. Because of its rarity no treatment guidelines are available and pediatric management often results from recommendations issued for adults. We report a case of a 4-year-old girl with somatotropinoma successfully treated with only medical treatment. She presented with tall stature and history of growth acceleration. Imaging and laboratory confirmed the diagnosis of GH secreting macroadenoma. She started medical treatment with a somatostatin analogue and a dopamine agonist. During an 8-year follow-up period, a good clinical control of the disease and a shrinkage of the adenoma have been demonstrated. At the last observation she achieved normal near-adult height and pubertal development. According to our experience and limited literature evidences, first line treatment with somatostatin analogues can be attempted in patients with somatotropinoma. This approach seems to be able to control the clinical course of the disease, allowing to postpone transphenoidal surgery to adult age or to avoid it.