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Original Article
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...
Effects of Insulin Level on Dyslipidemia in Children with Simple Obesity
Jung Sub Lim, Choong Ho Shin, Sei Won Yang
Clin Exp Pediatr. 2002;45(6):764-772.   Published online June 15, 2002
Purpose : Obesity is closely related to insulin resistance, compensatory hyperinsulinemia and dyslipidemia in adults. We identified the effect of obesity measured by BMI and insulin resistance on dyslipidemia in children and adolescents. Methods : The fasting serum insulin, glucose, total cholesterol, triglyceride, HDL- and LDL-cholesterol were measured and insulin resistance(HOMA-IR) was calculated in 35 children with simple obesity(age :...
Clinical Characteristics of Type 2 Diabetes in Children and Adolescents
Seong Yong Lee, Choong Ho Shin, Sei Won Yang
Clin Exp Pediatr. 2002;45(6):754-763.   Published online June 15, 2002
Purpose : The incidence of type 2 diabetes in children and adolescents has been reported to increase recently. The aim of this study is to investigate the clinical features of type 2 diabetes developing during childhood and adolescent period. Methods : The medical records of 33 patients with type 2 diabetes were reviewed. We analysed clinical manifestations, demographic data, and modes...
Changes of Bone Metabolism Markers and Bone Mineral Density with Improvement of Thyroid Function in Children and Adolescents with Hyperthyroidism
Min Ho Jung, Choong Ho Shin, Sei Won Yang, Byung Churl Lee
Clin Exp Pediatr. 2002;45(6):743-753.   Published online June 15, 2002
Purpose : Dynamics of bone mineral density(BMD) and bone metabolism in children and adolescents with hyperthyroidism have not been thoroughly investigated. The aim of this study was to study how the improvement of thyroid function with antithyroid treatment influenced bone metabolism and BMD in children and adolescents with hyperthyroidism. Methods : Serum levels of osteocalcin(OC), bone-specific alkaline phosphatase(b-ALP), and carboxyterminal telopeptide...
Case Report
A Case of Citrullinemia Diagnosed at the Neonatal Period
Seung Kyu Song, Kyung Chang Oh, Mi Ae Hong, Hee Taeg Kim, Hye Jung Shin, Soon Young Kim, Jin Keun Chang, Heui Seung Jo, Beyong Il Kim, Sei Won Yang, Jung-Hwan Choi
Clin Exp Pediatr. 2002;45(4):524-528.   Published online April 15, 2002
Citrullinemia is a rare inborn error of metabolism of the urea cycle, and was first reported by McMurray, et al. in 1962. It is inherited as an autosomal recessive trait. The normal synthesis of argininosuccinic acid is blocked in this disease due to a deficiency of argininosuccinic acid synthetase(AS), which has been demonstrated in liver cells and fibroblasts. The clinical...
Original Article
Study on the Frequency of Receptor Insensitivity to Androgen Using the Androgen-receptor Binding Assay in the Genetic Male Children with Abnormalities of Sexual Differentiation
Choong Ho Shin, Sei Won Yang
Clin Exp Pediatr. 2002;45(1):95-102.   Published online January 15, 2002
Purpose : The development of external genitalia in genetic male is dependent on the transcriptional regulatory activity of dihydrotestosterone(DHT)-androgen receptor complex in the genital skin. The abnormality of androgen receptor encompasses a wide range of phenotypes. We investigated the androgen receptor binding capacity of genetic males with ambiguous genitalia(grade was determined by Prader grade) for the availability as screening test. Methods : The binding capacity...
Factors Related to the Development of Microalbuminuria in Children and Adolescents with Type 1 Diabetes Mellitus
Min Ho Jung, Pei Tai Wan, Choong Ho Shin, Sei Won Yang
Clin Exp Pediatr. 2001;44(5):577-586.   Published online May 15, 2001
Purpose : We observed the prevalence rate of microalbuminuria(MA) and its relationship with the various clinical factors in children and adolescents with type 1 diabetes mellitus(DM). Methods : Seventy two children and adolescents(26 males and 46 females, 17.1±3.0 years) were included. MA was defined as 24 hour urine microalbumin >30mg/day(20μg/min). Subjects were classified into three groups(normoalbuminuria, transient MA, or persistent MA)....
Clinical Characteristics of Williams Syndrome
Choong Ho Shin, Ho Sung Kim, Sei Won Yang, Jung Yun Choi
Clin Exp Pediatr. 2001;44(4):443-449.   Published online April 15, 2001
Purpose : Williams syndrome(WS) is an autosomal dominant disorder resulting from a submicroscopic deletion of contiguous genes on the long arm of chromosome 7. We evaluated the physical, neurodevelopmental, and behavioral characteristics of children and adults with WS confirmed by fluorescence in situ hybridization. Methods : The clinically suspicious twelve cases with cardiac anomaly were confirmed as WS with fluorescence in...
Factors Affecting the Presence of Menarche and Final Adult Height in Adult Female Patients with Congenital Adrenal Hyperplasia of 21-Hydroxylase Deficiency
Seong Yong Lee, Chae Kyoung Song, Min Ho Jung, Jin Soon Hwang, Sei Won Yang
Clin Exp Pediatr. 2001;44(1):75-82.   Published online January 15, 2001
Purpose : Amenorrhea and short stature are serious complications in patients with congenital adrenal hyperplasia of 21-hydroxylase deficiency(CAH). We analysed several factors influencing the presence of menarche and final adult height in these patients. Methods : We retrospectively reviewed medical records of 24 female patients with CAH. We analysed possible factors affecting the menarche and final adult height, such as clinical...
Factors Influencing the Onset of Honeymoon Period in Children with Type I Diabetes Mellitus
Jin Soon Hwang, Sei Won Yang
Clin Exp Pediatr. 2000;43(8):1106-1110.   Published online August 15, 2000
Purpose : Type I diabetes mellitus(DM) is an autoimmune disease which decreases insulin secretion of pancreatic β-cell. The honeymoon period in type I DM is known to be related to the partial recovery of C-peptide and preservation of pancreatic β-cell function. The aim of this study was to determine factors influencing the onset of honeymoon period in children with type...
Case Report
A Case of Hepatoblastoma with Precocious Puberty
Chang Won Choi, Hyun Jin Park, Ki Woong Sung, Eun Sun Yoo, Hee Young Shin, Sei Won Yang, Hyo Seop Ahn
Clin Exp Pediatr. 2000;43(2):288-293.   Published online February 15, 2000
Hepatoblastoma is the most common primary malignant liver tumor in childhood. Most cases are boys generally below three years of age. Hepatoblastoma are associated with various non-metastatic syndromes. Rarely, human chorionic gonadotropin may be produced by the tumor. Ectopic gonadotropin production from this source is a rare cause of precocious puberty in boys. Since a report by Behrendt in 1931,...
Original Article
Re-Evaluation of Neonatal Screening Tests for Inborn Errors of Metabolism with Dried Filter Paper Blood Spots
Kye Shik Shim, Jin Soon Hwang, Jung Sub Lim, Se Young Kim, Choong Ho Shin, Sei Won Yang, Jung Hwan Choi, Chong Ku Yun, Jung-Han Song
Clin Exp Pediatr. 1999;42(12):1639-1644.   Published online December 15, 1999
Purpose : There have been high rates of false positive and recall in neonatal screening test using the cut-off points set by the manufacturing company. So, it is necessary to re-evaluate the cut off values to minimize the false positive rates. Methods : We collected capillary blood in dry filter paper from 996 healthy neonates on the third day in cases...
Role of Polymorphism in HLA DQ-α and -β Chain Loci in the Pathophysiology of Autoimmune Thyroid Disease in Children with and without Turner Syndrome
Kye Shik Shim, Kyu Chul Choeh, Sei Won Yang, Sa Jun Chung, Jin Sung Lee, Kyung Tae Lee, Sung Ho Goh, Yong Sung Kim
Clin Exp Pediatr. 1999;42(7):980-990.   Published online July 15, 1999
Purpose : About 10% of girls with Turner syndrome may have autoimmune thyroid disease(AIT), but the disease's pathophysiology has not yet been elucidated. Accordingly, this study was performed to observe whether the pathogenesis of AIT in children with Turner syndrome and without Turner syndrome correlate with special loci of DQ α and β chain in HLA. Methods : Blood samples were...
Renal Problems in Wilson Disease
Il Soo Ha, Jun Ho Lee, Yeon Ho Choe, Hae Il Cheong, Sei Won Yang, Young Yull Koh, Jeong Kee Seo, Yong Choi
Clin Exp Pediatr. 1999;42(6):817-824.   Published online June 15, 1999
Purpose : Wilson disease is a hereditary metabolic disorder characterized by an accumulation of copper in tissues which sometimes results in renal problems. This study was performed to evaluate the prevalences of the renal problems in Wilson disease and related risk factors. Methods : In 31 patients with Wilson disease, we looked for renal problems by history taking, physical examination...
Study on Changes in Bone Metabolism with Growth Hormone Treatment in Children with Growth Hormone Deficiency - Changes in the Serum Levels of Bone Markers -
Eun Young Kim, Sei Won Yang
Clin Exp Pediatr. 1999;42(5):704-710.   Published online May 15, 1999
Usefulness of Minimal Model Analysis : Measurement of Insulin Sensitivity, Glucose Effectiveness, and Insulin Secretory Capacity from Pancreatic Islet Cells in Children with Simple Obesity or Type 2 Diabetes Mellitus
Se Young Kim, Sei Won Yang
Clin Exp Pediatr. 1999;42(4):561-570.   Published online April 15, 1999
Purpose : It is known that insulin resistance and compensatory hyperinsulinemia from pancreatic islet cells to overcome insulin resistance could develop in children with simple obesity. When insulin resistance is aggravated by decompensation of high insulin secretion, obese children frequently progress to overt type 2 diabetes mellitus(DM). The purpose of the present study was to measure insulin sensitivity, glucose effectiveness,...
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...
Clinical Characteristics, Including Endocrinological Changes, of Optic Glioma in Children
Jee Suk Yu, Se Young Kim, Choong Ho Shin, Ki Joong Kim, Sei Won Yang, Yong Seung Hwang
Clin Exp Pediatr. 1998;41(8):1111-1119.   Published online August 15, 1998
Clinical Lecture
Failure to Thrive
Sei Won Yang
Clin Exp Pediatr. 1998;41(2):147-153.   Published online February 15, 1998
Case Report
A Pediatric Case of MELAS Syndrome Associated with Insulin-dependent Diabetes Mellitus
Kyung Ran Park, Hye Won Park, Tae Sung Ko, Hae Il Cheong, Sei Won Yang, Young Seung Hwang, In Won Kim, Je Geun Chi
Clin Exp Pediatr. 1996;39(10):1461-1465.   Published online October 15, 1996
MELAS(mitochondrial myopathy, encephalopathy, lactic acidosis, and sroke like episodes) is a major subgroup of heterogeneous mitochondrial encephalopathy. Recent advances in molecular genetics revealed specific mutations in the mitochondrial DNA causing MELAS. We described clinical and molecular genetic findings of a 13-year-old boy with MELAS syndrome associated IDDM(insulin dependent diabetes mellitus). For molecular genetic studies, DNAs from peripheral blood nucleated cells were used. And the A→G...
A Case of Mauriac's syndrome
Se Young Kim, Choong Ho Shin, Il Soo Ha, Hae Il Cheong, Sei Won Yang, Yong Choi, Hyung Ro Moon
Clin Exp Pediatr. 1996;39(7):1020-1024.   Published online July 15, 1996
Mauriac's syndrome was described in the 1920s, as a triad of poorly controlled insulin dependent diabetes mellitus, profound growth retardation, and hepatomgaly. Following the wide availability of insulin and intensification of diabetic control, this entity has become quite rare. A 9-year-old female child was transferred to pediatric OPD of SNUCH because of hyperglycemia, short stature, and visual disturbance. Five years prior to admission, she...
Original Article
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...
The Changes of the Bone Mineral Density in the Girls with Turner Syndrome, using Recombinant Human Growth Hormone
Sei Won Yang
Clin Exp Pediatr. 1996;39(6):839-845.   Published online June 15, 1996
Purpose : Estrogen deficiency causes sexual infantilism in Turner syndrome, which could decrease the bone mineral density(BMD) since birth. This decreased BMD might be contributed by the decreased growth hormone(GH) secretion. To improve the decreased BMD, estrogen therapy is recommended, especially after the pubertal age, but estrogen therpay during childhood can accelerate the epiphyseal fusion, resulting in shorter final height. There is...
Study on the Thyroid Function of Neonate Born to Mother with Hyperthyroidism
Choong Ho Shin, Se Young Kim, Sei Won Yang, Jung Hwan Choi, Chong Ku Park
Clin Exp Pediatr. 1996;39(1):106-114.   Published online January 15, 1996
Purpose : In newborns from mothers with Graves' disease, neonatal transient hyperthyroidism or hypothyroidism may develop early in life. We evaluated the incidence and prognosis of neonatal thyroid dysfunction in neonates born to mothers with hyperthyroidism during pregnancy. Methods : We measured blood T4, T3, TSH levels and TSH-R-Ab titer in 48 hyperthyroid mothers and their babies between 1988 and 1994. Results :...
Cushing Syndrome in Children and Adolescents
Choong Ho Shin, Sei Won Yang, Hyung Ro Moon
Clin Exp Pediatr. 1995;38(12):1677-1685.   Published online December 15, 1995
Purpose : Cushing syndrome occurs rarely in children and adolescents. The clinical manifestation is variable and growth is usually adversely affected. Methods : We retrospectively analyzed 8 patients with Cushing syndrome between the ages of 1 and 14 year who were admitted to the pediatric department of Seoul National University Children's Hospital during the period from 1978 to 1995. Results : 1)...
Upper Limit of Normal Value for Antistreptolysin O Titer of Healthy Children in Seoul
Sung Ho Cha, Yong Mook Choi, Sei Won Yang, Dong Hwan Lee, Chong Guk Lee, Chang Ho Hong
Clin Exp Pediatr. 1995;38(8):1061-1068.   Published online August 15, 1995
Purpose : We have found somewhat high titer of antistreptolysin O (ASO) accidentally when we had delineated normal carrier rate of beta hemolytic streptococci in rural area of Kangwon Province in 1991(1). We would like to know upper limit of normal value of ASO and re-valuated results which might be changed with different geographic areas, age, and prevalent rate...
Case Report
Two Cases of Tetrahydrobiopterin Deficiency
Jee Won Choi, Sei Won Yang, Hyung Ro Moon, Se Chin Cho
Clin Exp Pediatr. 1995;38(3):397-403.   Published online March 15, 1995
Tetrahydrobiopterin(BH4) deficiency is a rare type of hyperphenylalaninemia and usually leads to a progressive neurologic deterioration despite early dietary control of blood phenylalanine concentration. We experienced two cases of BH4 deficiency in brother and sister, confirmed by biochemical study of blood and urine. They had suffered from a progressive neurologic illness such as mental retardation, severe hypotonia, seizure, and athetotic movements...
Original Article
The Effect of Long-Acting Gonadotropin-Releasing Hormone (GnRH) Analog on the Growth Velocity and Final Adult Height in the Children with True Precocious Puberty
Sei Won Yang, Hyung Ro Moon
Clin Exp Pediatr. 1994;37(3):309-316.   Published online March 15, 1994
The final adult height in the children with true precocious puberty are destined to be short due to excessive bone maturation, compared to the growth velocity, regardless of its etiologies. To improve this final shortness, long-acting GnRH analog have been tried to the children with true precocious puberty. We evaluated the parameters of the growth, including the growth velocity, height...
The Correlation between the Bone Age and Predicted Adult height, Final Adult Height in the Children with Congenital Adrenal Hyperplasia, Especially 21-Hydroxylase Deficiency
Sei Won Yang, Hyung Ro Moon
Clin Exp Pediatr. 1994;37(3):300-308.   Published online March 15, 1994
We studied the correlation between the bone age and the predicted adult height, final adult height in the 69 children (30 salt losing form and 39 non-salt losing form) diagnosed as 21-hydroxylase deficiency, retrospectively. The results were as belows; 1) The bone age was similar to the chronological age in the children with salt-losing form (5.3±3.5 years, respectively), but the bone age...
The Growth Hormone Response to Growth Hormone-Releasing Hexapeptide (GHRP-6) in the Sprague Dawley Rat with Hypothyroidism
Sei Won Yang
Clin Exp Pediatr. 1994;37(1):89-98.   Published online January 15, 1994
We studied the effects of growth hormone to subthreshold, threshold, or suprathershold dose of growth hormone-releasing hexapeptide (GHRP-6) in conscious and unconscious (anesthesized with ketamine and phenobarbital) female Sprague Dawley rats with hypothyroidism, induced with methimazole and compared these results with those in conscious and unconscious rats with euthyroidism. The results were as below; 1) In conscious rats, the peak GH response...
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