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Original Article
Endocrinology
Comparison of effectiveness of growth hormone therapy according to disease-causing genes in children with Noonan syndrome
Kyo Jin Jo, Yoo Mi Kim, Ju Young Yoon, Yeoun Joo Lee, Young Mi Han, Han-Wook Yoo, Hyang-Sook Kim, Chong Kun Cheon
Clin Exp Pediatr. 2019;62(7):274-280.   Published online December 3, 2018

Purpose: To analyze the growth response to growth hormone (GH) therapy in prepubertal patients with Noonan syndrome (NS) harboring different genetic mutations. Methods: Twenty-three patients with prepubertal NS treated at Pusan National University Children’s Hospital between March 2009 and July 2017 were enrolled. According to the disease-causing genes identified, the patients with NS were divided into 4 groups. Three groups were...
Case Report
Endocrinology
Concomitant occurrence of Turner syndrome and growth hormone deficiency
Jung Yu, Ha Young Shin, Chong Guk Lee, Jae Hyun Kim
Clin Exp Pediatr. 2016;59(Suppl 1):S121-S124.   Published online November 30, 2016

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...

Review Article
Growth hormone treatment and risk of malignancy
Hyun-Wook Chae, Duk-Hee Kim, Ho-Seong Kim
Clin Exp Pediatr. 2015;58(2):41-46.   Published online February 28, 2015

Growth hormone (GH) treatment has been increasingly widely used for children with GH deficiencies as the survival rate of pediatric patients with malignancies has increased. Both GH and insulin-like growth factor-I have mitogenic and antiapoptotic activity, prompting concern that GH treatment may be associated with tumor development. In this review, the authors examined the relationship between GH treatment and cancer...

Efficacy and safety of growth hormone treatment for children born small for gestational age
Il Tae Hwang
Clin Exp Pediatr. 2014;57(9):379-383.   Published online September 30, 2014

Recombinant growth hormone (GH) is an effective treatment for short children who are born small for gestational age (SGA). Short children born SGA who fail to demonstrate catch-up growth by 2-4 years of age are candidates for GH treatment initiated to achieve catch-up growth to a normal height in early childhood, maintain a normal height gain throughout childhood, and achieve...

Original Article
Prader-Willi syndrome: a single center's experience in Korea
Yea Ji Kim, Chong Kun Cheon
Clin Exp Pediatr. 2014;57(7):310-316.   Published online July 23, 2014
Purpose

Prader-Willi syndrome (PWS) is a complex genetic disorder that results from the lack of paternally expressed genes in the chromosome 15q11-q13 region. This study was performed to delineate the clinical features of PWS infants and toddlers and the effects of two-year growth hormone (GH) treatment according to gender and age at the start of treatment.

Methods

The clinical characteristics and the results...

Review Article
Endocrine problems in children with Prader-Willi syndrome: special review on associated genetic aspects and early growth hormone treatment
Dong-Kyu Jin
Clin Exp Pediatr. 2012;55(7):224-231.   Published online July 17, 2012

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,...

Systematic review of the clinical and genetic aspects of Prader-Willi syndrome
Dong Kyu Jin
Clin Exp Pediatr. 2011;54(2):55-63.   Published online February 28, 2011

Prader-Willi syndrome (PWS) is a complex multisystem genetic disorder that is caused by the lack of expression of paternally inherited imprinted genes on chromosome 15q11-q13. This syndrome has a characteristic phenotype including severe neonatal hypotonia, early-onset hyperphagia, development of morbid obesity, short stature, hypogonadism, learning disabilities, behavioral problems, and psychiatric problems. PWS is an example of a genetic condition caused...

Original Article
Prevalence and risk factors of the metabolic syndrome in young adults with childhood-onset hypopituitary growth hormone deficiency
Han Hyuk Lim, Min Jae Kang, In Suk Yun, Young Ah Lee, Choong Ho Shin, Sei Won Yang
Clin Exp Pediatr. 2010;53(10):892-897.   Published online October 31, 2010
Purpose

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).

Methods

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,...

Effects of human growth hormone on gonadotropin-releasing hormone neurons in mice
Janardhan P. Bhattarai, Shin Hye Kim, Seong Kyu Han, Mi Jung Park
Clin Exp Pediatr. 2010;53(9):845-851.   Published online September 13, 2010
Purpose

Recombinant human growth hormone (rhGH) has been widely used to treat short stature. However, there are some concerns that growth hormone treatment may induce skeletal maturation and early onset of puberty. In this study, we investigated whether rhGH can directly affect the neuronal activities of of gonadotropin-releasing hormone (GnRH).

Methods

We performed brain slice gramicidin-perforated current clamp recording to examine the direct...

Effect of GnRH analogue on the bone mineral density of precocious or early pubertal girls
Jeong Sook Lim, Heon-Seok Han
Clin Exp Pediatr. 2009;52(12):1370-1376.   Published online December 15, 2009
Purpose : Treatment of precocity with gonadotropin releasing hormone analogue (GnRHa) might theoretically exert a detrimental effect on the bone mass during pubertal development. We investigated the short-term changes in bone mineral density (BMD) during GnRHa treatment and the enhancement in the changes with the co-administration of GnRHa and human growth hormone (hGH). Methods : Forty girls with precocious or...
Economic evaluation of a weekly administration of a sustained-release injection of recombinant human growth hormone for the treatment of children with growth hormone deficiency
Hye-Young Kang, Duk Hee Kim, Sei-Won Yang, Yoon-Nam Kim, Miseon Kim
Clin Exp Pediatr. 2009;52(11):1249-1259.   Published online November 15, 2009
Purpose : From a societal perspective, we evaluated the cost-effectiveness of a novel sustained-release injection of recombinant human growth hormone (GH) administered on a weekly basis compared with that of the present daily GH injection for the treatment of children with GH deficiency. Methods : Health-related utility for GH therapy was measured based on the visual analogue scale. During July...
Review Article
Treatment of chronic kidney disease in children
Joo Hoon Lee
Clin Exp Pediatr. 2009;52(10):1061-1068.   Published online October 15, 2009
The treatment of pediatric patients with chronic renal disease comprises management of nutritional imbalance, fluid, electrolyte, and acid-base disturbances, mineral bone disease, anemia, hypertension, and growth retardation. The treatment also includes administration of appropriate renal replacement therapy, if required. Adequate dietary intake of carbohydrates, fats, and proteins and caloric intake must be encouraged in such patients to ensure proper growth...
Original Article
Growth responses to growth hormone therapy in children with attenuated growth who showed normal growth hormone response to stimulation tests
Jae Hyun Kim, Hey Rim Chung, Young Ah Lee, Sun Hee Lee, Ji Hyub Kim, Choong Ho Shin, Sei Won Yang
Clin Exp Pediatr. 2009;52(8):922-929.   Published online August 15, 2009

Purpose:The aim was to investigate the clinical characteristics and responses to growth hormone (GH) therapy in children with attenuated growth who showed normal GH responses to GH stimulation tests (GHST). Methods:The study included 39 patients with height velocity (HV) of less than 4 cm/yr and normal GHST results. Clinical characteristics of patients were analyzed retrospectively. Results:Eleven were born as small for gestational age (SGA)...
Effect of growth hormone on neuronal death in hippocampal slice cultures of neonatal rats exposed to oxygen-glucose deprivation
Kyung Sik Hong, Jihui Kang, Myeung Ju Kim, Jeesuk Yu, Young Pyo Chang
Clin Exp Pediatr. 2009;52(5):588-593.   Published online May 15, 2009
Purpose : To investigate whether growth hormone (GH) has a protective effect on neurons in hippocampal slice cultures of neonatal rats exposed to oxygen-glucose deprivation (OGD). Methods : Cultured hippocampal slices of 7-day-old rats were exposed to OGD for 60 min. Then, the slices were immediately treated with three doses of GH (5, 50, or 500 µM) in media. The...
Analysis of cytosine adenine repeat polymorphism of the IGF-I promoter gene in children with idiopathic short stature
Jae Hoon Moon, Woo Yeong Chun
Clin Exp Pediatr. 2009;52(3):356-363.   Published online March 15, 2009
Purpose : A polymorphism in the IGF-I gene promoter region is known to be associated with serum IGF-I levels, birth weight, and body length, suggesting that IGF-I gene polymorphism might influence postnatal growth. The present study aimed to investigate the role of this polymorphic cytosine-adenine (CA) repeat of the IGF-I gene in children with idiopathic short stature. Methods : The...
Factors for persistent growth hormone deficiency in young adults with childhood onset growth hormone deficiency
Young Ah Lee, Hye Rim Chung, Se Min Lee, Jae Hyun Kim, Ji Hyun Kim, Sun Hee Lee, Choong Ho Shin, Sei Won Yang
Clin Exp Pediatr. 2009;52(2):227-233.   Published online February 15, 2009
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 :...
Review Article
Growth and sex differentiation of children born small for gestational age
Se Young Kim
Clin Exp Pediatr. 2009;52(2):142-151.   Published online February 15, 2009
The incidence of small for gestational age (SGA) births is frequent, accounting for 2.3% to 8% of all live births. Several childhood and adult diseases are related to early postnatal growth and birth size, and 10% of children born SGA may have a short stature throughout postnatal life. Additionally, they may have abnormal growth hormone (GH)-insulin like growth factor axis,...
Original Article
Study on the diagnostic utility of serum levels of insulin-like growth Factor-I and insulin-like growth factor binding protein-3 in growth hormone deficiency
Geun Ha Chi, Jeong Nyeo Lee, Woo Yeong Chung
Clin Exp Pediatr. 2008;51(12):1329-1335.   Published online December 15, 2008
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...
Effects and adverse-effects of growth hormone therapy in children with Prader-Willi syndrome: A two year study
Su Jin Kim, Joong Bum Cho, Min Jung Kwak, Eun Kyung Kwon, Kyung Hoon Paik, Dong-Kyu Jin
Clin Exp Pediatr. 2008;51(7):742-746.   Published online July 15, 2008
Purpose : The objective of this study was to evaluate the effects and adverse side-effects of growth hormone (GH) therapy in children with Prader-Willi syndrome (PWS). Methods : Forty-one patients who had been treated with GH for more than two years (24 boys and 17 girls, mean age 7.3?.3 years during treatment initiation) were enrolled for this study. Results : After...
Phenotype-genotype correlations and the efficacy of growth hormone treatment in Korean children with Prader-Willi syndrome
Keun Wook Bae, Jung Min Ko, Han-Wook Yoo
Clin Exp Pediatr. 2008;51(3):315-322.   Published online March 15, 2008
Purpose : Prader-Willi syndrome (PWS) is a complex genetic disorder, caused by the deletion of the paternally derived 15q11-13 region or the maternal uniparental disomy of chromosome 15 (mUPD(15)). In this study, we compared phenotypic differences between those patients whose disease was caused by microdeletion and those caused by mUPD(15). In addition, a comparison of the efficacy of growth hormone...
Clinical, endocrinological and radiological courses in patients who was initially diagnosed as idiopathic central diabetes insipidus
Seung Joon Chung, Seong Yong Lee, Choong Ho Shin, Sei Won Yang
Clin Exp Pediatr. 2007;50(11):1110-1115.   Published online November 15, 2007
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...
Growth promoting effect of combined gonadotropin releasing hormone analogue and growth hormone therapy in early pubertal girls with predicted low adult heights
Eun-Jeong Hong, Heon-Seok Han
Clin Exp Pediatr. 2007;50(7):678-685.   Published online July 15, 2007
Purpose : Recent reports pointed out that gonadotropin releasing hormone analogue (GnRHa) therapy alone is not so promising for improving adult height in precocious puberty. So, that we studied the growth promoting effect of combined therapy with GnRHa and growth hormone (GH) in early pubertal girls. Methods : Twenty three early pubertal girls (9.73?.59 yr) with predicted adult heights (PAH) below...
Growth promoting effect of short-term gonadotropin releasing hormone agonist with or without growth hormone in girls with early puberty
Eun-Ju Lee, Heon-Seok Han
Clin Exp Pediatr. 2007;50(2):198-204.   Published online February 15, 2007
Purpose : The hope that arresting pubertal developement might increase final adult height has led to an attempt to use GnRH agonist (GnRHa) in children with early puberty and poor growth prognosis. We investigated the growth-promoting effect of GnRH agonists with or without growth hormone (GH) in girls with early puberty and decreased predicted adult height (PAH). Methods : Thirty five...
Factors affecting the final adult height in survivors of childhood brain tumors
Kyong-Ah Yun, Young Ah Lee, Choong Ho Shin, Sei Won Yang, Hee Young Shin, Hyo Seop Ahn, Il Han Kim
Clin Exp Pediatr. 2007;50(1):65-73.   Published online January 15, 2007
Purpose : Short stature is an important complication that impairs the quality of life in survivors of childhood brain tumors. We studied their final adult height (FAH) to evaluate risk factors for short stature. Methods : We reviewed the medical data of 95 survivors of childhood brain tumors (64 males and 31 females) who had been followed up from 1982 to...
Review Article
Current use of growth hormone in children
Choong Ho Shin
Clin Exp Pediatr. 2006;49(7):703-709.   Published online July 15, 2006
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...
Original Article
Effects of growth hormone treatment on glucose metabolism in idiopathic short stature
Seung Yeon Kwon, Duk-Hee Kim, Ho-Seong Kim
Clin Exp Pediatr. 2006;49(6):665-671.   Published online June 15, 2006
Purpose : To study the effects of growth hormone(GH) treatment on glucose metabolism and insulin resistance in children with idiopathic short stature(ISS). Methods : Glucose and insulin concentrations were measured during oral glucose tolerance test (OGTT) before and after GH treatment(0.6-0.7 IU/kg/week) in 20 patients with ISS. Insulin resistance was assessed by homeostasis model assessment(HOMA). Results : During OGTT, the mean...
Effect of GnRH analogue on predicted adult height in girls with early puberty
Byung-Hoon Ahn, Heon-Seok Han
Clin Exp Pediatr. 2006;49(5):552-557.   Published online May 15, 2006
Purpose : The recent results observed in precocious puberty and the hope that interrupting puberty might increase adult height have led to an attempt to use GnRH agonist(GnRHa) in children with premature puberty and a poor growth prognosis. We aimed to analyze the growth promoting effect of GnRHa in girls with early puberty and low predicted adult height(PAH). Methods : Thirty...
Effects of NG-monomethyl-L-arginine and L-arginine on cerebral hemodynamics and energy metabolism during reoxygenation-reperfusion after cerebral hypoxia-ischemia in newborn piglets
Sun Young Ko, Saem Kang, Yun Sil Chang, Eun Ae Park, Won Soon Park
Clin Exp Pediatr. 2006;49(3):317-325.   Published online March 15, 2006
Purpose : This study was carried out to elucidate the effects of nitric oxide synthase(NOS) inhibitor, NG-monomethyl-L-arginine(L-NMMA) and nitric oxide precursor, L-arginine(L-Arg) on cerebral hemodynamics and energy metabolism during reoxygenation-reperfusion(RR) after hypoxia- ischemia(HI) in newborn piglets. Methods : Twenty-eight newborn piglets were divided into 4 groups; Sham normal control(NC), experimental control(EC), L-NMMA(HI & RR with L-NMMA), and L-Arg(HI & RR...
The effect of growth hormone treatment in short children born small for their gestational ages
Joo Hee Seo, Duk Hee Kim
Clin Exp Pediatr. 2006;49(3):312-316.   Published online March 15, 2006
Purpose : Growth hormone(GH) has been recognized as an effective treatment for short children born small for their gestational ages(SGA), and nowadays it has been widely used for the treatment of short children born SGA. The aim of this study is to assess the efficacy of GH treatment for the children born SGA. Methods : The study population was made of...
The comparison of predicted adult height change and height gain after gonadotropin-releasing hormone agonist and combined growth hormone treatment in girls with idiopathic central precocious puberty
Ji-Young Seo, In-Suk Yoon, Choong-Ho Shin, Sei-Won Yang
Clin Exp Pediatr. 2006;49(3):305-311.   Published online March 15, 2006
Purpose : GnRH analogues(GnRHa) are used to treat central precocious puberty(CPP). However, in some patients, the GV decrease is so remarkable that it impairs predicted adult height(PAH); and there fore, the addition of growth hormone(GH) is suggested. We analysed the growth changes during two years and final adult height(FAH) in girls with idiopathic CPP treated with combined therapy, compared with...
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