hrp0089p1-p150 | GH & IGFs P1 | ESPE2018

Total Sum of Growth Hormone Values Obtained from Growth Hormone Stimulation Test May be Useful in the Diagnosis of Prepubertal Children with Idiopathic Growth Hormone Deficiency

Lee Su-Jeong , Moon Jung-Eun , Lee Gi-Min , Kim Hyeon-A , Ko Cheol-Woo

Background and objective: The peak GH value plays a crucial role in the diagnosis of idiopathic growth hormone deficiency (iGHD). However, the prediction of peak GH in iGHD diagnosis is known to be limited. The purpose of this study was to evaluate the clinical and diagnostic usefulness of the total sum of GH values obtained from the GH stimulation test.Materials and methods: We retrospectively reviewed 178 prepubertal children who were diagnosed with iG...

hrp0089p3-p218 | GH & IGFs P3 | ESPE2018

Bone Age Maturation During the Three Years of GH Treatment in Patients with Idiopathic GH Deficiency and Idiopathic Short Stature: Analysis of Data from LG Growth Study

Shim Young Suk , Hwang Il Tae , Yang Seung , Kim Eun Young

Background: Although the beneficial effects of GH treatment on statural growth are well known, the impacts on skeletal maturation are not fully understood. In the current study, we aimed to investigate the progression of bone age (BA) in children with idiopathic GH deficiency (iGHD) and idiopathic short stature (ISS) based on a LG Growth Study (LGS). We also evaluated the progression rate of BA relative to chronologic age (CA) between iGHD and ISS and to find their associated ...

hrp0089p3-p296 | Pituitary, Neuroendocrinology and Puberty P3 | ESPE2018

Analysis of Influencing Factors on Bone Maturation in Girls with Central Precocious Puberty (CPP)

Lee Gi-Min , Moon Jung-Eun , Lee Su-Jeong , Kim Hyeon-A , Ko Cheol-Woo

Background & objective: The occurrence of CPP seems to be increasing in our clinical practice thesedays. It is known that CPP causes socio-psychological disturbances relating to early pubertal changes and finally leads to a significant decrease in the final adult height because of premature closure of the growth plate. This study was conducted to see major factors affecting to the bone maturation, which is closely related to the final adult height in girls with CPP.<p ...

hrp0089p1-p246 | Sex Differentiation, Gonads and Gynaecology or Sex Endocrinology P1 | ESPE2018

Clinical Phenotypes and Mutation Spectrum of Patients with Isolated Gonadotropin-Releasing Hormone Deficiency in a Single Academic Center

Yoo Han-Wook , Seo Go Hun , Oh Arum , Kim Gu-Hwan , Choi Jin-Ho

Background: Isolated gonadotropin-releasing hormone (GnRH) deficiency (IGD) is caused by a deficiency in GnRH production, secretion or action. IGD is a highly heterogeneous disorder with wide phenotypic spectrum including Kallmann syndrome (KS) with anosmia and normosmic idiopathic hypogonadotropic hypogonadism (nIHH). More than 30 different causative genes have been identified in several studies. However, there are no data on the prevalence, clinical characteristics, and mole...

hrp0086rfc3.1 | Pituitary | ESPE2016

Endocrinopathy in Childhood Intracranial Germ Cell Tumours is Predicted by Disease Location not Treatment: 30 year Experience from a Single Tertiary Centre

Serra-Caetano Joana , Dimitrakopoulou Eftychia , Ederies Ash , Phipps Kim , Spoudeas Helen Alexandra

Background: Childhood pineal and/or suprasellar intracranial germ cell tumours (IGCT) are highly curable (>90%) with neuraxial radiation alone; international (SIOP) trials have aimed to decrease late radiation-induced neuroendocrine morbidity by substituting chemotherapy. However, without longitudinal study, disease and treatment contributions to long term outcomes remain unknown.Objective: To define tumour and treatment factors implicated in neuroen...

hrp0086rfc6.5 | Syndromes: Mechanisms and Management | ESPE2016

Abnormal Videofluoroscopic Swallow Studies (VFSS) in Infants with Prader-Willi Syndrome Indicate a High Rate of Silent Aspiration

Salehi Parisa , Chen Maida , Beck Anita , McAfee Amber , Kim Soo-Jeong , Herzig Lisa , Leavitt Anne

Background: Prader-Willi Syndrome (PWS), due to loss of expression from genes within the PWS imprinted region at chromosome 15q11.2-13, is characterized by hypotonia and feeding intolerance in infancy with later development of hyperphagia and obesity. Growth hormone improves tone, body composition, and height and can be started in infancy. Morbidity and mortality in PWS include those secondary to hyperphagia and respiratory illness as well as a 17% reported incidence of sudden...

hrp0086p2-p91 | Adrenal P2 | ESPE2016

Corticosteroid-Induced Adrenal Insufficiency in a Child with T Cell Lymphoblastic Lymphoma

Lee Sang Hyun , Son Kyung Lae , Sim Yeji , Kim Heung Sik

Background: Glucocorticoids play a major role in the treatment of lymphoblastic lymphoma. However, supraphysiological glucocorticoid therapy may cause the secondary adrenal insufficiency.Presentation of case: A 11-year-old boy with T cell lymphoblastic lymphoma, treated according to COG A5971 protocol, experienced sudden onset of tremor and general weakness in the first day after tapering 28 days of glucocorticoid therapy. He had a moon face and pigmenta...

hrp0086p1-p127 | Bone &amp; Mineral Metabolism P1 | ESPE2016

Transient Pseudohypoaldosteronism and Failiure to Thrive in A 5-Month-Old Infant

Soon Kim Hae , Lee Yoonsuk , Won Lee Jung , Jin Cho Su

Hyponatremia with hyperkalemia in infancy may be seen in many endocrinologic and metabolic disorder such as congenital adrenal hyperplasia, congenital adrenal hypoplasia, and other forms of hypoadrenalism in infancy. Here, we report a infants who presented with hyponatremia with hyperkalemia finally diagnosed as pseudohypoaldosteronism (PHA) due to urinary tract infection (UTI) with reflux nephropahy. A 5-month-old female initially was transferred for poor weight gain for 2 mo...

hrp0086p1-p741 | Pituitary and Neuroendocrinology P1 | ESPE2016

Serum Anti-Mullerian Hormone Levels in Precocious Puberty Girls According to the Timing of GnRH Agonist Treatment

Nam Hyo-Kyoung , Kim Hye Ryun , Rhie Young Jun , Lee Kee-Hyoung

Background: Few studies have investigated the long-term effects of gonadotropin releasing hormone (GnRH) agonist treatment on reproductive function.Objective and hypotheses: We assessed ovarian function by analyzing serum anti-mullerian hormone (AMH) levels in central precocious puberty (CPP) girls according to GnRH agonist-treatment timing.Method: Our study included 505 CPP girls subdivided into five groups based on the timing of ...

hrp0086p2-p849 | Syndromes: Mechanisms and Management P2 | ESPE2016

Genetic Variability in Patients with Noonan Syndrome in the Republic of Macedonia

Kocova Mirjana , Sukarova-Angelovska Elena , Kacarska Rozana , Lee Beom Hee , Kim Jae-Min

Background: Noonan syndrome is autosomal dominantly inherited disease with an incidence of 1:1000 to 1:2500 newborns. It is caused by different gene mutations involved in the RAS/MAP kinase signaling pathway in the cells. Phenotype including expression of dysmorphic features and visceral organ affection is variable. Different gene mutations are found in approximately 60–70% of tested patients.Objective and hypotheses: To report mutational analysis i...