The Effects of 5 Years of Growth Hormone Treatment on Growth and Body Composition in Patients with Temple Syndrome

INTRODUCTION: Temple syndrome (TS14) is a rare imprinting disorder caused by maternal uniparental disomy of chromosome 14, paternal deletion of 14q32.2, or an isolated methylation defect. Most patients with TS14 develop precocious puberty. Some patients with TS14 are treated with growth hormone (GH). However, evidence for the effectiveness of GH treatment in patients with TS14 is limited. METHODS: This study describes the effect of GH treatment in 13 children and provides a subgroup analysis of 5 prepubertal children with TS14. We studied height, weight, body composition by dual-energy X-ray absorptiometry, resting energy expenditure (REE), and laboratory parameters during 5 years of GH treatment. RESULTS:In the entire group, mean (95% CI) height SDS increased significantly during 5 years of GH treatment from -1.78 (-2.52; -1.04) to 0.11 (-0.66; 0.87). Fat mass percentage SDS decreased significantly during the first year of GH, and lean body mass (LBM) SDS and LBM index increased significantly during 5 years of treatment. IGF-1 and IGF-BP3 levels rose rapidly during GH treatment, and the IGF-1/IGF-BP3 molar ratio remained relatively low. Thyroid hormone levels, fasting serum glucose, and insulin levels remained normal. In the prepubertal group, median (interquartile range [IQR]) height SDS, LBM SDS, and LBM index also increased. REE was normal at start and did not change during 1 year of treatment. Five patients reached adult height and their median (IQR) height SDS was 0.67 (-1.83; -0.01). CONCLUSION: GH treatment in patients with TS14 normalizes height SDS and improves body composition. There were no adverse effects or safety concerns during GH treatment.</p

Morfoanatomia e histoquímica da semente de sororoca (Phenakospermum guyannense (Rich.) Endl. - Strelitziaceae)

Phenakospermum guyannense, popularly known in the Amazon as sororoca, is usually found along rivers and in ombrophilous environments. The objective of this study was to describe the morpho-anatomy and histochemistry of mature seeds of P. guyannense collected at the Urubuí Waterfall, Presidente Figueiredo, Amazonas state, Brazil. Seed anatomy was studied using a light and a scanning electron microscopes (SEM). Histochemical tests were performed to identify phenolic compounds, starch, protein and lipids. The mature seed of P. guyannense is stenospermic, with a black seed coat composed of several layers of different cell types, with most containing phenolic compounds. The hilum is punctiform, surrounded by cells, which form the aryl. The endosperm is solid, formed by tetrahedral cells containing starch and protein. The embryo, which is cylindrical and located in the proximal region, is basal capitate, with cells containing lipids and proteins and is composed of a slightly dilated hypocotyl-radicle axis. The haustorium is flattened and located in the distal region