ESPE Abstracts

McCune-Albright syndrome (MAS) is a rare disease defined by the triad of precocious puberty (PP), café au lait spots, and fibrous dysplasia (FD). There are only a few patients with MAS in Korean because of the rarity of this disease. We reported the various clinical and endocrine manifestations and genetic analysis of 14 patients with MAS in Korea. It is a retrospective cohort study of patients’ clinical data including about peripheral PP, FD. Also, treatment experie...

Background: Prenatal androgen exposure can lead to variable virilization of external female genitalia. The lack of a consensus definition of clitoromegaly and the limited data available on normal steroid levels in female neonates makes its diagnosis difficult.Objective and hypotheses: The aims of this study were (i) to define reference sizes for external female genitalia in term and preterm neonates as a function of gestational age and birth weight; and ...

Background, Objective and hypotheses: GX-H9 is a hybrid Fc-based long-acting recombinant human growth hormone (hGH). The safety, tolerability, and PK/PD of single ascending dose in healthy volunteers were assessed to determine GX-H9 doses capable of normalizing IGF-1 level. The PK/PD, safety, efficacy and tolerability of multiple sequential doses of GX-H9 in adult growth hormone deficiency (AGHD) were compared to that of a daily recombinant hGH in AGHD. A Phase 2 study in pedi...

Background: The weekly sustained-release growth hormone (GH) has been approved for treatment in growth hormone deficiency (GHD). It provides a practical strategy for improving adherence.Objective and hypotheses: To evaluate the long-term safety and effectiveness of two formulations of daily (Eutropin®) and weekly (EutropinPlus®) GH in Korean pediatric GHD patients.Method: A multicenter, long-term, pr...

Background: Hematopoietic stem cell transplantation (HSCT) has improved the prognosis of children with malignant hematologic disease. However, it has had significant adverse effects on the endocrine system, including bone health. Limited studies are available to assess osteoporosis in survivors of adolescents after HSCT.Objective and hypotheses: We investigate the bone mineral density (BMD) and endocrinopathy/treatment factors associated with low BMD in ...

Background: Central precocious puberty (CPP) may result from organic lesions, but it is most frequently of idiopathic origin in girls.Objective and hypotheses: The objective of the study was to identify central nervous system abnormalities in girls with CPP.Method: This retrospective study was performed in 3-tertiary care hospitals between 2005 and 2015. During this period, 200 girls with CPP performed brain magnetic resonance imag...

Background: South Korea is one of the adequate or excessive iodine nutritional state countries and high-iodine intake is related to papillary thyroid cancer. The prevalence of thyroid cancer of South Korea has increased and the proportion of thyroid papillary cancer has increased as 97.9–98.3% in 2010. Childhood thyroid cancer is rare, and the prevalence of thyroid cancer in pediatric population was reported as 20–26% of thyroid nodules worldwide. However, there have...

Background: Over 4 years, 1,526 patients received Eutropin® and EutropinPlus® (recombinant human growth hormone (GH), LG Life Sciences, Ltd.) while enrolled in the LG Growth Study (LGS), designed to monitor the long-term effectiveness and safety of GH. We present LGS experience for GH treatment during 4 years in growth hormone deficiency (GHD)Objective and hypotheses: To evaluate the long-term safety and efficacy of Eutrop...

Background: SOX2 is an early developmental transcription factor implicated in pituitary development; heterozygous SOX2 mutations have been reported in patients with a severe ocular phenotype and hypogonadotrophic hypogonadism (HH) with/without associated abnormalities. SOX2 physically interacts with β-catenin, a member of the Wnt-signalling pathway, via its carboxyl-terminus and it represses in vitroβ-catenin mediated activation.<p class="abstex...

Background: Nesfatin-1, a recently discovered anorexigenic neuropeptide, seems to play an important role in hypothalamic pathways regulating food intake and energy homeostasis.Objective and hypotheses: The aim of this study is to evaluate the relation of serum nesfatin-1 level with anthropometric and metabolic parameters in children and adolescents.Method: This study included 78 Korean children and adolescents (42 obese/overweight ...