ESPE Abstracts

GHD diagnosis in neonates and infants is a challenge owing to the fact that GH pharmacological stimulation tests (GHST) are not approved at this age. In a retrospective study, we found that a random GH<6.5 µg/L confirmed GHD diagnosis in neonates with clinical suspicion of GHD with high diagnostic accuracy (1). The accuracy of GH and its surrogates of action have not been set for infants with current standardized immunoassays.Objective</...

Introduction: Growth depends on growth hormone (GH) secretion and on individual sensitivity to its action. The effects of birth parameters on growth and metabolic status are well documented in small-for-gestational-age children, but in children with GH deficiency those associations are not clear. Taking into account that GH-deficient children are not a homogenic group of patients, the importance of an individual approach to GH doses and the assessment of the e...

Background: Pseudopseudohypoparathyroidism (PPHP) is a rare variant of pseudohypoparathyroidism (PHP) type I, with typical anatomical abnormalities known as Albright's hereditary osteodystrophy (AHO)(short stature, brachydactyly particularly involving metacarpals and metatarsals, round face, stocky build, ectopic ossifications and a number of possible associated defects), but with normal calcium, phosphate and PTH levels and normal response to exogenous PT...

Purpose: Our previous study showed no correlation between -202 A/C IGFBP-3 promoter polymorphism and Δheight SDS in children with growth hormone deficiency. We investigated the influences of the -202 IGFBP-3 polymorphism on 1-year follow-up outcomes of GH treatment in Korean children with ISS.Methods: Data was obtained from 81 children with idiopathic short stature (peak serum growth hormone (GH) ≥ 7.0 ng/mL b...

Background: The acid-labile subunit (ALS) is the crucial third player in the tertiary complex for its function of prolonging the half-life of the IGF1-IGFBP3 binary complexes. IGF1 and IGFBP3 are routinely determined during the diagnostic work-up for growth hormone deficiency (GHD). The aim of the study is to evaluate the relevance of serum ALS as an additional biomarker, alone or in combination with IGF1 and IGFBP3, in the diagnosis of GHD.<p class="abste...

According to current recommendations, children with height SDS <-3.0, normal growth hormone (GH) peak in stimulation tests (stimGH) and severe IGF-I deficiency (IGFD) may be diagnosed with primary IGFD and treated with recombinant IGF-I. The need for direct confirmation of GH insensitivity is a matter of discussion. On the other hand, children born small for gestational age (SGA) with no catch-up growth are qualified to GH therapy despite normal GH secretion. The fact that...

The role of insulin-like growth factor-1 (IGF-1) in the regulation of growth of children with intrauterine growth is currently being discussed, but the final point of view has not been achieved.Aim: to study the effect of IGF-1 in the dynamics of postnatal growth of premature infants with SGA in the first 5 years of life.Material and Methods: The prospective study included prematurity with SGA (gro...

Background: Pain represents one of the most stressful experiences for children undergoing medical therapies (Kortesluoma, 2008), but is under represented in literature for what concerns pediatric endocrinology. Children consider Injections one of the most painful, frighteningand distressing procedures (Fassler, 1985). The treatment for patients with Growth Hormone Deficiency requires daily subcutaneous injection, performed by parents or patients themselves. Th...

Background: Severe primary insulin-growth factor-1 (IGF-1) deficiency (SPIGF1D) is a rare cause of growth retardation. Diagnostic criteria include age- and sex-dependent low basal IGF-1 levels (<2.5th percentile), height ≤ -3SDS, absence of growth hormone deficiency and of any secondary causes of growth failure.Objectives: Description of pubertal onset and growth spurt, data on adult or near-adult-he...

Introduction: Growth hormone (hGH) and/or growth factors are thought to play a role in the pathogenesis of diabetic retinopathy. Increased treatment of human growth hormone (hGH) in children rise questions concerning the safety of GH replacement therapy on ophtalmic changes. Adverse effects of hGH treatment (pseudotumor cerebri, papilloedema, retinal changes mimicking diabetic retinopathy, neovascularization) have been reported in some papers. In this study, i...

Background: One of the main concerns in patients with growth disorders is to achieve optimal adherence to growth hormone (GH) treatment. For this it is important to identify patients with low adherence to treatment and to evaluate actions to improve it.The Merck Patient Support Program (PSP) carried out educational actions aimed at patients with low adherence and their parents, to raise their awareness of the importance of good adherence...

IGFALSgene is located in chromosome 16p13.3 encoding acid labile subunit which binds insulin-like growth factors (IGFs) to increase their half-life and vascular localization. The biallelic defect of this gene leads to acid-labile subunit deficiency characterized by postnatal growth retardation, insulin resistance, delayed puberty, and no growth hormone deficiency. A 5-year-old-boy was referred to as low IGF-1 and cortisol level. He presented with obesity and growth re...