Turner syndrome (TS) is a genetic disorder in phenotypic females that has characteristic physical features and presents as partial or complete absence of the second sex chromosome. Growth hormone deficiency (GHD) is a condition caused by insufficient release of growth hormone from the pituitary gland. The concomitant occurrence of TS and GHD is rare and has not yet been reported... |
Prader-Willi syndrome (PWS) is a complex multisystem genetic disorder characterized by hypothalamic-pituitary dysfunction. The main clinical features include neonatal hypotonia, distinctive facial features, overall developmental delay, and poor growth in infancy, followed by overeating with severe obesity, short stature, and hypogonadism later in development. This paper reviews recent updates regarding the genetic aspects of this disorder. Three mechanisms (paternal deletion,... |
This study evaluated the prevalence of the metabolic syndrome (MetS) and risk factors for metabolic derangement in young adults with childhood-onset hypopituitary growth hormone deficiency (ACOHGHD). Thirty patients with ACOHGHD who were treated with hormone-replacement therapy, aged 18 to 29 years, who visited the Seoul National University Children's Hospital between September 2009 and February 2010 were enrolled. Height, weight, waist circumference,... |
Purpose : Growth hormone (GH) replacement after retesting is necessary because impairment of body composition and cardiovascular health has been more severe in adult patients with persistent GH deficiency (GHD) from childhood to adulthood. This study aimed to investigate the factors for persistent GHD and define a highly probable group of persistent GHD in young adults with childhood-onset GHD. Methods :... |
Purpose : This study aimed to determine the best cutoff line for insulin-like growth factor (IGF)-I and insulin-like growth factor binding protein (IGFBP)-3 to discriminate between growth hormone deficiency (GHD) patients and the control group. Methods : Two hundred thirty subjects with normal controls (129 boys and 101 girls, aged 7-15 years), 14 patients with complete GHD (12 boys and... |
Purpose : Idiopathic central diabetes insipidus (CDI) is defined in CDI patients without definite etiology. Some patients initially diagnosed as idiopathic CDI progressed to organic causes. We reviewed clinical, endocrinological, and radiological courses of 20 patients who was initially diagnosed as idiopathic CDI, to assess the predicting factors for progression to brain tumors. Methods : We reviewed the medical data and... |
Since the advent of growth hormone(GH), children with a wide variety of growth disorders have received GH treatment. In GH deficiency(GHD), Turner syndrome, chronic renal failure, children born small for gestational age, Prader-Willi syndrome, and idiopathic short stature, the therapeutic effects and safety profile of GH are reviewed. GH therapy has been clearly shown to improve height velocity and final... |
Central diabetes insipidus is a rare disorder that can result as a consequence of diverse etiologies, including malformations, autoimmune, infiltrative(e.g. neoplastic or histiocytosis) or traumatic processes, as well as mutations in the gene encoding arginine vasopressin. Idiopathic central diabetes insipidus is a diagnosis of exclusion, one that has been made less frequently through the decades. Idiopathic central diabetes insipidus in... |
Purpose : This study was designed to evaluate the effect of growth hormones on children with growth hormone deficiency(GHD) or idiopathic short stature(ISS). Methods : Between January 1988 to July 2003, 45 patients(M26, F19) with GHD and 24 patients (M13, F11) with ISS were enrolled in this study. Height standard deviation score(Ht SDS) for chronological age(CA) and Ht SDS for bone... |
Purpose : The purpose of this study was to evaluate the factors affecting the final adult height and total height gain in idiopathic and organic growth hormone deficient(GHD) children after growth hormone(GH) treatment. Methods : Thirteen patients with idiopathic GHD and 22 patients with organic GHD who had been treated with GH and attained adult final height were included in this... |
Purpose : Craniopharyngiomas are often accompanied by severe endocrine disorders. Although there is universal growth hormone deficiency(GHD), the resulting growth pattern is very heterogeneous. We report the growth and endocrine outcome of 44 children with craniopharyngioma, with emphasis on initial symptoms, growth before and during growth hormone(GH) treatment and spontaneous growth in spite of GHD. Methods : We performed a... |
Purpose : To investigate the effect of growth hormone(GH) secretion on serum concentration of leptin, we evaluated the differences in serum concentration of leptin between patients with growth hormone deficiency(GHD) and GH normal short stature in prepubertal male children, and the relationship between GH and serum leptin level. Also, we analyzed the correlation between serum concentrations of leptin and insulin-like growth factor-I(IGF-I), insulin-like growth... |
Purpose : In this study, efforts have been made to evaluate the sella MRI in 14 patients with growth hormone deficiency to define more clearly the structural abnormalities of the hypothalamohypophyseal axis, examine frequency of these abnormalities, and determine whether a relationship exists between the sella MRI findings and types of endocrine dysfunction. Methods : Sella MRI was performed in 14 patients who were... |
Purpose : To assess whether pretreatment IGF-I SDS and pretreatmental bone age are useful indicators in the response of rhGH therapy in children with GH deficiency(GHD), the relationship between pretreatment IGF-I SDS, delay in bone age and the growth response during the first year of rhGH treatment was analyzed. Methods : This study included 12 children with GHD. We measured IGF-I levels by RIA... |
Purpose : The aim of this study was to evaluate the long-term growth response to growth hormone therapy in 18 children with naive growth hormone deficiency. Methods : The subjects of this study were aged 3-13.5 years and each patient continuously received GH(EutropinⓇ) 0.45-0.7IU/kg/week, intramuscularly or subcutaneously in 3-7 divided dose for up to 7 years. Standard auxologic assessment was carried... |
Purpose : For diagnosis of growth hormone(GH) deficiency in short stature, peak growth hormone levels after pharmacologic stimulation are usually used. In this study, we measured serum IGFBP-3, which is a major binding protein in serum and is considered to be GH-IGF-I axis dependent, levels by radioimmuno assay(RIA) in sera from normal short stature(NSS) children, and patients with GH deficiency children to clarify the... |
Purpose : The insulin like growth factors (IGFs) circulate complexed to IGF-binding proteins(IGFBPs). IGFBP-3 is the major circulating IGFBP and is found primarily as a 150 kDa complex which contains an acid labile subunit(ALS), IGFBP-3, and IGF-I or IGF-II and is considered to be growth hormone(GH) dependent. In this study, we measured serum IGF-I, IGFBP-3 and 150 kDa levels in sera of growth hormone... |
Purpose : At present, to determine growth hormone(GH) deficiency in short stature, many provocative tests using various pharmacologic agents such as clonodine, L-dopa, insulin should be done. These are not only complicated, but also misleading in some patients. And so, in search of a simple method of detecting GH deficiency that may replace the complicated stimulation test, we measured basal... |
Purpose : To investigate the diagnostic significance of urinary growth hormone measurement and compare with the urinary GH values in different methods of urine collection, this study was performed in patients with short stature. Methods : Measurement of urinary GH excretion was performed in 9 children with complete growth hormone deficiency (GCD), in 19 children with partial growth hormone deficiency (GPD)... |
Urinary growth hormone (GH) excretion was quantitated in 12-hr overniaght urine collections obtained from 13 children with complete growth hormone deficiency(CGHD), 6 children with partial growth hormone deficiency(PGHD), 5 children with short stature and normal GH provocation tests(NSC), and 5 normal control children (NC) to investigate whether the measurement of urinary GH can clearly separate the PGHD and CGHD groups... |
Twelve-hour nocturnal urine was collecte in 5 patients diagnosed of having complete GH deficiency, 6 of having partial GH deficiency and 7 persons as normal control group. The GH levels in urine collections were evaluated, and the results are as follows. 1) The GH level of normal control group was 16.76¡¾1.91ng/12hr, 59.00¡¾6.92ng/gCr, 16.24¡¾2.52ng/m2, 0.58¡¾0.12ng/kg. 2) The GH level of complete GH deficiency... |
Fifteen GH deficient dwarfs were treated with LBD-003, the first year-derived rhGH produced in Korea. The growth promoting effect of LBD-003 was excellent and similar to that of the pituitary extract GH or the previous imported rhGHs. The serum concentrations of IGF-1 at 6th and 12th month of treatment showed significant elevation (p< 0.005, p< 0.001 respectively), but no significant correlation was observed between the... |
Since the success of human growth hormone (hGH) synthesis by recombinant DNA technology, these GH products are used in the treatment of growth hormone deficiency. Recently, methionyl-GH has been produced in the yeast, Saccharomyces cerevisiae 2150 by the use of a recombinant method. In this paper, the clinical efficacy and. immunogenicity of this GH was studied during therapy of 1 year in 20 children... |