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Case Report
Autosomal dominant hypocalcemia with Bartter syndrome due to a novel activating mutation of calcium sensing receptor, Y829C
Keun Hee Choi, Choong Ho Shin, Sei Won Yang, Hae Il Cheong
Clin Exp Pediatr. 2015;58(4):148-153.   Published online April 22, 2015

The calcium sensing receptor (CaSR) plays an important role in calcium homeostasis. Activating mutations of CaSR cause autosomal dominant hypocalcemia by affecting parathyroid hormone secretion in parathyroid gland and calcium resorption in kidney. They can also cause a type 5 Bartter syndrome by inhibiting the apical potassium channel in the thick ascending limb of the loop of Henle in the...

Original Article
Urinary 6-sulfatoxymelatonin level in girls and its relationship with obesity
Jieun Lee, Juyoung Yoon, Jin A Lee, Seong Yong Lee, Choong Ho Shin, Sei Won Yang
Clin Exp Pediatr. 2012;55(9):344-349.   Published online September 14, 2012
Purpose

Short sleep duration is associated with obesity. Urinary 6-sulfatoxymelatonin (6-OHMS), the principal metabolite of melatonin, is closely related with sleep. We evaluated the difference in urinary 6-OHMS levels between obese girls and normal weight girls, and the relationship of urinary 6-OHMS with other hormones regulating body weight and metabolism.

Methods

A total of 79 girls (6.3 to 12.4 years) were included in...

Excess of leptin inhibits hypothalamic KiSS-1 expression in pubertal mice
Sung Yeon Ahn, Sei Won Yang, Hee Jae Lee, Jong Seon Byun, Ji Yeon Om, Choong Ho Shin
Clin Exp Pediatr. 2012;55(9):337-343.   Published online September 14, 2012
Purpose

Leptin has been considered a link between metabolic state and reproductive activity. Defective reproductive function can occur in leptin-deficient and leptin-excessive conditions. The aim of this study was to examine the effects of centrally injected leptin on the hypothalamic KiSS-1 system in relation to gonadotropin-releasing hormone (GnRH) action in the initial stage of puberty.

Methods

Leptin (1 µg) was injected directly into...

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,...

Remission rate and remission predictors of Graves disease in children and adolescents
Sun Hee Lee, Seong Yong Lee, Hye Rim Chung, Jae Hyun Kim, Ji Hyun Kim, Young Ah Lee, Sei Won Yang, Choong Ho Shin
Clin Exp Pediatr. 2009;52(9):1021-1028.   Published online September 15, 2009
Purpose : Medical therapy is the initial treatment for children with Graves disease to avoid complications of other treatments. However, optimal treatment for childhood Graves disease is controversial because most patients require relatively long periods of medical therapy and relapse is common after medication discontinuation. Therefore, this study aimed to search clinical or biochemical characteristics that could be used as...
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)...
Development of metabolic syndrome and its correlation with insulin resistance in adult patients with Turner syndrome
Joo Hwa Kim, Min Jae Kang, Choong Ho Shin, Sei Won Yang
Clin Exp Pediatr. 2009;52(3):370-375.   Published online March 15, 2009
Purpose : The risk of metabolic syndrome (MS) and cardiovascular disease in Turner syndrome (TS) patients is high. We analyzed metabolic factors in adults with TS and evaluated the metabolic risk of insulin resistance. Methods : Forty-three adults with TS were enrolled. The frequency of MS and the values of the metabolic factors were analyzed. Patients were divided into insulin...
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 :...
A study of the development of macrovascular complications and factors related to these complications in young adults with childhood/adolescence-onset type 1 diabetes mellitus
Min Jae Kang, Joo Hwa Kim, Hye Rim Chung, Young Ah Lee, Choong Ho Shin, Sei Won Yang, You Yeh Kim, Seon Mi Jin, Chung Il Noh
Clin Exp Pediatr. 2009;52(2):220-226.   Published online February 15, 2009
Purpose : Macrovascular complications are the main cause of mortality in type 1 diabetes mellitus (T1DM). The purpose of this study was to clarify the presence of early vascular changes and to assess the risk factors of macrovascular complications in young adults with T1DM diagnosed in childhood and adolescence. Methods : Seventy-two patients (23.9¡¾2.4 years) with T1DM diagnosed before 18 years...
Case Report
A case of testicular adrenal rest tumor in a male child with congenital adrenal hyperplasia
Joo Hwa Kim, Kyong Ah Yun, Choong Ho Shin, Sei Won Yang
Clin Exp Pediatr. 2008;51(9):1018-1022.   Published online September 15, 2008
Testicular adrenal rest tumors are a well-known complication in male patients with congenital adrenal hyperplasia. Corticosteroid suppressive therapy usually results in the regression of these tumors. We describe a patient with 21-hydroxylase deficiency who developed bilateral testicular masses. Despite steroid suppressive therapy, the tumors did not regress and hormonal control was poor. Consequently, bilateral partial orchiectomies were performed.
Erratum
ERRATUM: Epidemiologic characteristics of type 1 diabetes in children aged 14 years or under in Korea, 1985-2000.
Choong Ho Shin
Clin Exp Pediatr. 2008;51(8):896-897.   Published online August 15, 2008
Case Report
A case of simultaneously identified glycogen storage disease and mucopolysaccharidosis
Ju Young Lee, Jeong Ok Shim, Hye Ran Yang, Ju Young Chang, Choong Ho Shin, Jae Sung Ko, Jeong Kee Seo, Woo Sun Kim, Gyeong Hoon Kang, Jeong Han Song, Jong Won Kim
Clin Exp Pediatr. 2008;51(6):650-654.   Published online June 15, 2008
Glycogen storage disease (GSD) and mucopolysaccharidosis (MPS) are both independently inherited disorders. GSD is a member of a group of genetic disorders involving enzymes responsible for the synthesis and degradation of glycogen. GSD leads to abnormal tissue concentrations of glycogen, primarily in the liver, muscle, or both. MPS is a member of a group of inherited lysosomal storage diseases, which...
Original Article
Interpretation of screening for congenital adrenal hyperplasia in preterm infants
Hye Rim Chung, Choong Ho Shin, Sei Won Yang, Kyong Ah Yun, Young Ah Lee, So Eun Park, Chang Won Choi, Byung Il Kim, Jung Hwan Choi, Junghan Song
Clin Exp Pediatr. 2008;51(6):616-621.   Published online June 15, 2008
Purpose : This study was undertaken to identify factors that influence 17-OHP levels in preterm infants and to suggest a reasonable follow-up schedule of screening for congenital adrenal hyperplasia (CAH) in preterm infants. Methods : The 17-OHP concentrations in filter paper blood spots of 427 preterm infants were obtained. The effects of gestational age (GA), systemic diseases, and antenatal dexamethasone...
Review Article
Epidemiologic characteristics of type 1 diabetes in children aged 14 years or under in Korea, 1985-2000
Choong Ho Shin
Clin Exp Pediatr. 2008;51(6):569-575.   Published online June 15, 2008
Type 1 diabetes mellitus (T1DM) develops in genetically susceptible individuals as a result of progressive autoimmune destruction of beta cells. There is a large global variation in incidence among children aged 0-14 years. The incidence of T1DM in Korea is very low. The latest survey in Korea was conducted in 2001 by the Korean Society of Pediatrics to analyze ...
Case Report
Pheochromocytoma associated with cyanotic congenital heart disease
Seung Joon Chung, Young Ah Lee, Choong Ho Shin, Sei Won Yang, Eun Jung Bae, Jung Il Noh
Clin Exp Pediatr. 2008;51(1):93-97.   Published online January 15, 2008
Pheochromocytoma is a rare tumor of childhood, arising from adrenal medullary and chromaffin tissue. Because chronic hypoxia may induce pheochromocytoma, there have been several reports of pheochromocytoma development in cyanotic patients after corrective or palliative cardiac surgery. The variable clinical presentation of pheochromocytoma is obscured by both underlying heart disease and medications. If sudden hypertension, aggravation of a heart condition,...
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