Growth hormone therapy in Turner syndrome
Short stature is the single most common physical abnormality in Turner syndrome (TS) with adult stature averaging 20 cm shorter than that of the general population. Randomized, placebo-controlled studies to final adult height have proven that GH therapy is effective in increasing stature in TS. Recently, randomized, controlled studies have demonstrated that adjunctive therapies with low-dose estrogen or low-dose oxandrolone enhance stature further. These therapies may provide benefits beyond height augmentation.
Available from: PubMed Central
- "It was reported that there was lipid deposition in the individuals with TS who have lost weight due to the disease.7,8 The current study revealed a high level of body fat in patients with TS before being treated with rhGH, indicating a lipid accumulation caused by abnormal lipid metabolism. "
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ABSTRACT: Objective: To study the effect of recombinant human growth hormone (rhGH) therapy on carbohydrate, lipid and protein metabolisms of Turner syndrome (TS).
s: Total 45 patients with TS admitted between Jul. 2008 and Jun. 2011 were involved in this study. All patients received the clinical evaluation of body fat, plasma lipids, proteins and oral glucose tolerance test (OGTT) before and after rhGH therapy.
: Our results indicated a significant decrease of body fat (FAT%) from 23.56±4.21 to 18.71±2.23 but no obvious change on the level of fat mass (FM) (p>0.05) was observed after rhGH therapy. We also detected significant changes on plasma high-density lipoprotein cholesterol (HDL-C) from (1.65±0.58 mmol/L) to (2.20±0.65 mmol/L) and low-density lipoprotein cholesterol (LDH-C) from (2.55±0.55 mmol/L) to (2.10±0.54 mmol/L) after rhGH exposure. However, no statistical significance was detected on the level of plasma triglyceride (TG), cholesterol (CHO). Interestingly, the levels of plasma retinol binding protein (RbP) (32.55±4.28mg/L), transferrin (TRF) (2.95±0.40 mg/L), serum albumin (PRE) (250.00±45.50 mg/L) and albumin (propagated) (33.58±4.25 mg/L) were significantly increased. When it goes to the oral glucose tolerance test (OGTT) test, there were 10 impaired glucose tolerance (IGT) cases among all patients before and after rhGH therapy. No significant change was observed on homeostasis model assessment- insulin resistance (HOMA-IR) level during rhGH intervention.
: Abnormal lipid and protein metabolisms of the children with TS can be improved with rhGH therapy for 6 months.
Pakistan Journal of Medical Sciences Online 07/2014; 30(4):731-4. DOI:10.12669/pjms.304.4546 · 0.23 Impact Factor
Available from: Sofia Helena Valente Lemos-Marini
- "Short stature is one of the most common physical features in girls with TS and growth hormone therapy is recommended for normalization of height . According to Davenport , the mean final height in TS girls is 20 cm below the mean of the normal girls. The physiological variations during the growth makes difficult the construction of new height references for TS girls. "
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ABSTRACT: The objective of this study was to review the growth curves for Turner syndrome, evaluate the methodological and statistical quality, and suggest potential growth curves for clinical practice guidelines. The search was carried out in the databases Medline and Embase. Of 1006 references identified, 15 were included. Studies constructed curves for weight, height, weight/height, body mass index, head circumference, height velocity, leg length, and sitting height. The sample ranged between 47 and 1,565 (total = 6,273) girls aged 0 to 24 y, born between 1950 and 2006. The number of measures ranged from 580 to 9,011 (total = 28,915). Most studies showed strengths such as sample size, exclusion of the use of growth hormone and androgen, and analysis of confounding variables. However, the growth curves were restricted to height, lack of information about selection bias, limited distributional properties, and smoothing aspects. In conclusion, we observe the need to construct an international growth reference for girls with Turner syndrome, in order to provide support for clinical practice guidelines.
BioMed Research International 05/2014; 2014(2):687978. DOI:10.1155/2014/687978 · 2.71 Impact Factor
Available from: Alan D Rogol
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Testosterone (T) drives normal male sexual development both in utero and at puberty. Aberrant T exposure manifests as virilization of a female fetus, contrasexual precocity in girls, and isosexual precocity in boys. Evidence of pathologic T exposure warrants a prompt evaluation.
The authors introduce the topic of T exposure in children by reviewing its physiology in the fetus and during childhood and adolescence. Pathologic conditions leading to virilization of a female fetus as well as androgen-mediated gonadotropin-independent precocious puberty in both genders are then discussed. The authors finish by noting exogenous T exposure in children and adolescents, focusing specifically on secondary exposure to topical T preparations.
Contrasexual precocity in a girl or sexual precocity in a boy should prompt evaluation for causes of gonadotropin-independent pubertal changes. Initial biochemical evaluation includes a bone age, T, 17-hydroxyprogesterone, androstenedione, dehydroepiandrosterone sulfate (DHEA-S) and high sensitivity gonadotropin levels. The provider must query exposure to topical androgen-containing preparations as unintentional secondary exposure to topical T must be considered. Hyperandrogenism is temporally related to exposure of topical T and removal of exposure results in a marked decrease in serum T as well as resolution or stabilization of the signs and symptoms.
Expert Opinion on Drug Safety 03/2013; 12(3). DOI:10.1517/14740338.2013.782000 · 2.91 Impact Factor
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