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Case Report
A Case of Cystinosis
Min Ho Jung, Seung On Keun, Soon Ju Lee, Byung Churl Lee
Clin Exp Pediatr. 2003;46(6):615-619.   Published online June 15, 2003
Cystinosis, an autosomal recessively inherited lysosomal storage disease, results from impaired transport of the amino acid cystine out of cellular lysosomes. The consequent accumulation and crystallization of cystine destroys tissues, causing growth retardation, Fanconi syndrome, renal failure, eye problems, and endocrinopathies. The gene for cystinosis, CTNS, was mapped to chromosome 17p13. The diagnosis of cystinosis was made by measuring the...
A Case of Partial Inverted Duplication of Chromosome 7q
Hyun Ji Kim, So Young Lee, Mi Young Kim, Chung Sun Baek, Won Duck Kim, Dong Seok Lee, Doo Kwun Kim, Sung Min Choi
Clin Exp Pediatr. 2003;46(5):510-513.   Published online May 15, 2003
Duplication of chromosome 7q has been reported as either partial or complete. Partial 7q duplication was first described by Carpentier in 1972. Pure partial duplication of the long arm of chromosome 7 is extremely rare and only 16 cases with a pure partial duplication of different 7q segment have been described in the literature. Pure partial duplication of the long...
Original Article
Overnight Growth Hormone Secretions and Sleep Patterns in Idiopathic Short Stature Children
Sang Young Seo, Kee Hyoung Lee, Baik Lin Eun, Chang Sung Sohn, Young Chang Tockgo, Chol Shin, Baek-Hyun Kim
Clin Exp Pediatr. 2003;46(4):363-369.   Published online April 15, 2003
Purpose : Pharmacologic provocation test of growth hormone(GH) is a non-physiologic method and has several limitations for diagnosing growth hormone(GH) deficiency. Spontaneous GH release studies could be important in understanding the pathophysiology of children with poor growth but normal responses to GH provocation tests. Also, the relationship between nocturnal GH secretions and sleep patterns in short stature children is poorly...
Endocrine Function and Growth in Children with Craniopharyngioma
Yoo Mi Chung, Choong Ho Shin, Sei Won Yang
Clin Exp Pediatr. 2003;46(3):277-283.   Published online March 15, 2003
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...
Hypoxia Induced Expression of Vascular Endothelial Growth Factor in Rat Pulmonary Artery Smooth Muscle Cells
Un Seok Nho, Yeo Hyang Kim, Myung Chul Hyun, Sang Bum Lee
Clin Exp Pediatr. 2003;46(2):167-172.   Published online February 15, 2003
5% CO2-95% air atmosphere. The smooth muscle cells were confirmed by immunostaining with smooth muscle myosin and α-smooth muscle actin antibodies. The VEGF gene expression in the hypoxic group was compared with the one in control the group as well as the one in the starved group by RT-PCR and Northern blot hybridization. Results : There was no statistically significant difference...
Thymus Size and Its Relationship to Perinatal Diseases; Respiratory Distress Syndrome and Intrauterine Growth Retardation
Sun Mi Chung, Woo Taek Kim
Clin Exp Pediatr. 2002;45(7):855-861.   Published online July 15, 2002
Purpose : Thymus size can be affected by several factors and perinatal diseases can be estimated by its size. The purpose of this study was to search for a relationship between cardiothymic/ thoracic(CT/T) ratio and perinatal diseases such as neonatal respiratory distress syndrome(RDS) and intrauterine growth retardation(IUGR) by measuring the width of the cardiothymic shadow at the level of the...
Type 2 Diabetes Mellitus in Children
Jee Min Park, Eun Gyong Yoo, Duk Hee Kim
Clin Exp Pediatr. 2002;45(5):646-653.   Published online May 15, 2002
Purpose : The incidence of type 2 diabetes mellitus in children has been increasing worldwide recently, which is thought to be related to the increasing prevalence of obesity. We investigated to evaluate the incidence and the characteristics of type 2 diabetes mellitus in children and also analysed the relationship between intrauterine growth retardation and type 2 diabetes mellitus. Methods :...
The Study of Serum Vascular Endothelial Growth Factor in the Acute Febrile Phase of Kawasaki Disease
Minshik Kim, Kyungsook Kim
Clin Exp Pediatr. 2002;45(1):114-119.   Published online January 15, 2002
Purpose : Kawasaki Disease(KD) is a febrile disease with acute multisystemic vasculitis associated with early development of acute myocarditis and coronary artery abnormalities. The cause and etiologic agents are still unknown. Clinical studies suggest that KD is one of the autoimmune disorders caused by infectious agent, but this is not proved yet. The study was performed to investigate the levels of vascular endothelial growth...
Growth and Neurodevelopmental Outcome at 15 Months of Corrected Age in Very Low Birth Weight Infants with Chronic Lung Disease
Seon Young Kim, Chang Yee Cho, Young Youn Choi
Clin Exp Pediatr. 2001;44(10):1112-1118.   Published online October 15, 2001
Purpose : This study was performed to compare the growth and neurodevelopmental outcome at 15 months of corrected age in very-low-birth weight infants between chronic lung disease(CLD) group and the control group. Methods : Very-low-birth-weight infants who were admitted and survived in the NICU(neonatal intensive care units) of Chonnam Univeristy Hospital from Jan. 1997 to Jan. 2000 were divided into...
Case Report
Two Cases of Growth Hormone Therapy in Idiopathic Dilated Cardiomyopath
Jeong Won Shin, Hong Ryang Kil, Jae Hong Yu
Clin Exp Pediatr. 2001;44(8):942-947.   Published online August 15, 2001
Growth hormone receptor gene is expressed in the myocardium and growth hormone(GH) administration has been shown to increase cardiac insulin-like growth factor-I(IGF-1) contents. The cardiac myocyte express IGF-I receptors and IGF-I promotes cardiac hypertrophy. Furthermore GH therapy has been reported to have beneficial effects in the treatment of dilated cardiomyopathy in adults. We have studied the effect of short term...
Original Article
Growth Hormone Receptor Mutation and Partial Growth Hormone Insensitivity in Children with Idiopathic Short Stature
So Chung Chung, Ran Namgung, Duk Hee Kim
Clin Exp Pediatr. 2001;44(8):922-935.   Published online August 15, 2001
Purpose : Children with idiopathic short stature(ISS) are classified on the basis of exclusion criteria. Short stature with normal or increased circulating growth hormone(GH) and low IGF-I levels indicates that partial growth hormone insensitivity(GHI) may play a role in ISS. The present study was performed to investigate whether partial GHI is observed in children with idiopathic short stature and whether...
Case Report
A Case of Apert`s Syndrome(Acrocophalosyndactyly) with Fibroblast Growth Factor Receptor 2 Exon Ⅲa Mutation
Hyung Su K, Pyl Soon Yang, Jee Yeoun Kang, Ok Young Kim, Chul Hae Ku, Wha Mo Lee
Clin Exp Pediatr. 2000;43(7):1006-1011.   Published online July 15, 2000
Apert syndrome is an uncommon congenital disorder characterized by malformation of the skull in association with symmetrical syndactyly of both hands and feet. This syndrome is autosomal dominant. The original description was presented by Apert in 1906. Since then more than 200 cases have been reported in the world. Recently, we experienced a case of newhorn male infant with congenital...
Original Article
Insulin-like Growth Factor(IGF)-I and IGF-Binding Protein-3 in Relation to Hemoglobin Concentration in Healthy Infants
Yoon Ki Kang, Dae-Yeol Lee
Clin Exp Pediatr. 2000;43(7):967-973.   Published online July 15, 2000
Purpose : Insulin-like growth factor(IGF-I) and IGF binding protein(IGFBP)-3 is thought to play an important role in fetal erythropoiesis. The objective of this study was to establish a relation between IGF-I, free IGF-I, IGFBP-1, and -3 with hemoglobin level in healthy term, 3-month, and 12-month old infants. Methods : Healthy term infants(n=20) were enrolled at birth, as well as 3 months(n=30) and 12 months(n=30) of...
Effect of Growth Hormone Secretion on Serum Concentration of Leptin
Se-Eun Kim, Jeong-Nyeo Lee, Sang-Ook Nam, Woo-Yeong Chung
Clin Exp Pediatr. 2000;43(7):959-966.   Published online July 15, 2000
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...
Changes in Fat Tissue and Growth Hormone Receptor mRNA after Growth Hormone Therapy
Hye Jung Shin, Duk Hee Kim, So Chung Chung, Kyungyoon Kam
Clin Exp Pediatr. 2000;43(2):247-252.   Published online February 15, 2000
Purpose : Growth hormone(GH) is a powerful inhibitor of lipoprotein lipase and is known to decrease fat cell mass. The lipolytic effect has more pronounced influence on visceral fat than subcutaneous fat. The effects of GH therapy on GH receptor in fat tissue are not clear. We investigated the changes in fat tissue and GH receptor mRNA in adipose tissue...
Effects of Growth Hormone Treatment on Alterations of Growth and Bone Metabolism in Rats Induced by Dexamethasone Administration
Woo Yeong Chung, Jeong Nyu Lee, Hye Kyoung Yoon
Clin Exp Pediatr. 1999;42(7):991-1002.   Published online July 15, 1999
Purpose : The aim of this study was to investigate the alterations of growth and bone metabolism in SDR induced by dexa administration and to evaluate the effects of GH treatment in dexamethasone(dexa) induced growth and bone metabolism in SDR. Methods : Forty-five female Sprague-Dawley rats(weight 150-170gm) were divided in 3 groups: Group 1(n=15) received normal saline as control, Group 2(n=15)...
Final Height in Idiopathic Growth Hormone(GH) Deficient Children Treated with GH
Byung Churl Lee
Clin Exp Pediatr. 1999;42(6):852-857.   Published online June 15, 1999
Purpose : Improved adult height is a major goal in the treatment of children with short stature due to GH deficiency(GHD). The purpose of this study was to evaluate final height in idiopathic GH deficient children after long-term GH treatment Methods : GHD was diagnosed in 9 male patients by at least two stimulation tests(maximum GH level≤5ng/ml). Other pituitary functions were...
Serum Levels of Insulin-Like Growth Factor-Ⅰ and Insulin-Like Growth Factor Binding Protein-3 between Short Children with or without Intrauterine Growth Retardation
Kye Shik Shim, Se Young Kim, Choong Ho Shin, Sei Won Yang
Clin Exp Pediatr. 1999;42(3):390-396.   Published online March 15, 1999
Purpose : Postnatally, 20% of children with intrauterine growth retardation(IUGR) has no catch- up growth, This might be dependent on the serum level of Insulin-like growth factor I(IGF-I), resulting in shortness. We measured serum levels of IGF-I and Insulin-like growth factor binding protein-3(IGFBP-3) in children with IUGR and normal short stature variants(NSSV) to observe whether they play a role in...
Case Report
A Case of Ulcerative Colitis with Growth Disturbance
Ki Hyun Jeon, Dong Hyun Choi, Eun Yung Seol, Kyung Eun Oh, Mun Ki Cho, Chul Son, Jong Soon Kim
Clin Exp Pediatr. 1999;42(2):289-295.   Published online February 15, 1999
Ulcerative colitis is a chronic relapsing inflammatory disease of the colonic and rectal mucosa, which affects children as well as adults. Inflammation caused by ulcerative colitis is limited to the colon and rectum, to the mucosa and submucosa, and is involved continuously, with varying degrees of ulceration, hemorrhage, edema, and regenerating epithelium. Ulcerative colitis most commonly presents with the onset...
A Case of FGFR2 Exon Ⅲc Mutation in Crouzon Syndrome
Seon Chan Bae, Eun Ha Lee, Moon Sung Park, Si Houn Hah, Chang Ho Hong
Clin Exp Pediatr. 1998;41(12):1717-1721.   Published online December 15, 1998
Crouzon syndrome, an autosomal dominant disorder, has characteristic features of craniosynostosis, hypertelorism, exophthalmos, maxillary hypoplasia and relative mandibular prognathism. Mutations of fibroblast growth factor receptor 2(FGFR2) gene are associated with craniosynostotic conditions, such as Crouzon syndrome, Jackson-Weiss syndrome, Pfeiffer syndrome, Apert syndrome and Beare-Stevenson cutis gyrata. We found one child with common morphological features of Crouzon syndrome. Interestingly, she...
Original Article
Profile of Insulin, Growth Hormone and Insulin-like Growth Factors in Human Cord Blood According to Birth Weight
Hye Young Kim, Dae Yeol Lee, Soo Chul Cho
Clin Exp Pediatr. 1998;41(11):1484-1490.   Published online November 15, 1998
Purpose : Insulin-like growth factors(IGFs) are mitogenic peptides that are essential for fetal and maternal tissue growth during pregnancy. They circulate primarily with serum IGF-binding protein(IGFBP) which regulates the availability of IGFs to their specific target tissue. This study was performed to examine the relationships between birth weight and IGFs, insulin and growth hormone in the sera of cord blood...
Short Term Effect of Recombinant Human Growth Hormone Therapy in Children with Chronic Renal Failure
Si-Whan Ko, Dong-Kyu Ji
Clin Exp Pediatr. 1998;41(9):1256-1261.   Published online September 15, 1998
Purpose : Growth retardation is one of the major side effects of chronic renal failure. In this study, the short-term growth promoting effect, effect on the chemical parameters, and probable side effects of growth hormone treatment to child patients with chronic renal failure were examined. Methods : The research sample was 5 child patients diagnosed with chronic renal failure and receiving...
Growth Status and Levels of Growth Factors in Children with Insulin-dependent Diabetes Mellitus
So Chung Chung, Duk Hee Kim
Clin Exp Pediatr. 1998;41(8):1102-1110.   Published online August 15, 1998
Purpose : It is well known that the linear growth velocity in children with insulin-dependent diabetes mellitus(type 1 DM) is closely related to metabolic control and onset age of the disease. Many studies have demonstrated growth impairment in children with type 1 DM, regardless of the degree of metabolic control, whereas other studies have found no growth retardation. Therefore, we...
Abnormalities of Hypothalamic-Pituitary MR Imaging and Pituitary Function in Patients with Growth Hormone Deficiency
Hun-Gy Kim, Han-Wook Yoo
Clin Exp Pediatr. 1998;41(6):791-798.   Published online June 15, 1998
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...
The Relationships of Pretreatment IGF-I SDS, Delay in Bone Age and Response to Recombinant Human Growth Hormone(rhGH) Therapy in Children with Growth Hormone Deficiency
Seon-Young You, Ki-Joong Kim, Jeh-Hoon Shin, Jin-Nyoung Park, Hee-Jeong Ahn, Yong-Joo Kim
Clin Exp Pediatr. 1998;41(1):90-98.   Published online January 15, 1998
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...
Cardiovascular Effects of Growth Hormone Treatment in Idiopathic Short Stature
Hyang Ok Woo, Dong Wook Kim
Clin Exp Pediatr. 1997;40(8):1149-1155.   Published online August 15, 1997
Purpose : An excess of GH causes various problems within the cardiovascular system including cardiac hypertrophy and abnormalities of left ventricle(LV) function. To evaluate the cardiovascular effects due to GH treatment in idiopathic short stature (ISS), we measured the cardiovascular function. Methods : Twenty-two echocardiographic studies were performed in ISS who were admitted from Jan. 1994 through Jul. 1996, and they were divided into two...
Long-term Growth Response to Growth Hormone Therapy in Children with Growth Hormone Deficiency
Byung Churl Lee
Clin Exp Pediatr. 1997;40(5):672-679.   Published online May 15, 1997
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...
TheAnalytic Review of Standard Growth Valuein School-Age Children in Seoul
Joo Young An, Jin Seop Kang, Young Jin Hong, Don Hee Ahn, Sung Jae Suh
Clin Exp Pediatr. 1996;39(12):1669-1679.   Published online December 15, 1996
Purpose : It hasn't been developed a new standard growth value for children in Korea since 1985. This study was designed to compare the difference of standard growth value of height, weight and chest circumference for school-age children in Seoul between 1985 and 1994. Methods : Height, weight and chest circumference were measured in 6,308 students, aged 6-17 years, of 12...
The Characteristics of Insulin-like Growth Factor Binding Protein-3(IGFBP-3) in Serum; The Diagnostic Utility of IGFBP-3 in Growth Hormone Deficiency
Ki-Joong Kim, Jeh-Hoon Shin, Young-Ik Lee
Clin Exp Pediatr. 1996;39(11):1594-1599.   Published online November 15, 1996
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...
The Effect of Recombinant Human Growth Hormone on the Growth in the Children with Chronic Renal Failure
Sei Won Yang, Young Choi
Clin Exp Pediatr. 1996;39(7):997-1004.   Published online July 15, 1996
Purpose : We observed the effect of three year-administration of recombinant human growth hormone(rhGH) on the growth in the children with chronic renal failure(CRF). Methods : Five prepubertal children(three boys and two girls) with CRF, who had been managed by conservative methods, were given rhGH(1 U/kg/week) for three years. Growth parameters, such as growth velocity, height standard deviation score(SDS), and predicted final adult height, obtained...
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