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Original Article
The corrected QT (QTc) prolongation in hyperthyroidism and the association of thyroid hormone with the QTc interval
Ye Seung Lee, Joong Wan Choi, Eun Ju Bae, Won Il Park, Hong Jin Lee, Phil Soo Oh
Clin Exp Pediatr. 2015;58(7):263-266.   Published online July 22, 2015
Purpose

Ventricular repolarization is assessed using the QT interval corrected by the heart rate (QTc) via an electrocardiogram (ECG). Prolonged QTc is associated with an increased risk of arrhythmias and cardiac mortality. As there have been few reports regarding the effects of hyperthyroidism on ventricular repolarization, we studied the association between serum free thyroxine (free T4 [fT4]) and thyroid stimulating hormone...

Case Report
Identification of a novel mutation in a patient with pseudohypoparathyroidism type Ia
Ye Seung Lee, Hui Kwon Kim, Hye Rim Kim, Jong Yoon Lee, Joong Wan Choi, Eun Ju Bae, Phil Soo Oh, Won Il Park, Chang Seok Ki, Hong Jin Lee
Clin Exp Pediatr. 2014;57(5):240-244.   Published online May 31, 2014

Pseudohypoparathyroidism type Ia (PHP Ia) is a disorder characterized by multiform hormonal resistance including parathyroid hormone (PTH) resistance and Albright hereditary osteodystrophy (AHO). It is caused by heterozygous inactivating mutations within the Gs alpha-encoding GNAS exons. A 9-year-old boy presented with clinical and laboratory abnormalities including hypocalcemia, hyperphosphatemia, PTH resistance, multihormone resistance and AHO (round face, short stature, obesity, brachydactyly...

Transient carnitine transport defect with cholestatic jaundice: report of one case in a premature baby
Hyun-Seok Cho, Young Kwang Choo, Hong Jin Lee, Hyeon-Soo Lee
Clin Exp Pediatr. 2012;55(2):58-62.   Published online February 14, 2012

Carnitine (β-hydroxy-γ-trimethylaminobutyric acid) is involved in the transport of long-chain fatty acids into the mitochondrial matrix and the removal of potentially toxic acylcarnitine esters. Transient carnitine transport defect is a rare condition in newborns reported in 1/90,000 live births. In this paper, we describe a case of transient carnitine transport defect found in a premature baby who had prolonged cholestatic...

Original Article
Inherited metabolic diseases in the urine organic acid analysis of complex febrile seizure patients
Hee Jeong Cheong, Hye Rim Kim, Seong Soo Lee, Eun Joo Bae, Won Il Park, Hong Jin Lee, Hui Chul Choi
Clin Exp Pediatr. 2009;52(2):199-204.   Published online February 15, 2009
Purpose : Seizure associated with fever may indicate the presence of underlying inherited metabolic diseases. The present study was performed to investigate the presence of underlying metabolic diseases in patients with complex febrile seizures, using analyses of urine organic acids. Method : We retrospectively analyzed and compared the results of urine organic acid analysis with routine laboratory findings in 278...
Reye syndrome after acute enteritis during the neonatal period
Jun Suck Bang, Sang Jung Nam, Kyung Hwa Lee, Eun Joo Bae, Won Il Park, Hyun Sook Lee, Bae Young Son, Hwan Suck Choi, Hong Jin Lee
Clin Exp Pediatr. 2006;49(3):273-277.   Published online March 15, 2006
Purpose : The reinfection rate of H. pylori reported before 13C-urea breath test(13C-UBT) era was higher than that of the post 13C-UBT era. Children are usually reluctant to receive invasive endoscopic evaluation for the reinfection of H. pylori, particularly when they are asymptomatic. The aim of the study is to discover the reinfection rate by different diagnostic tests, and to...
Organic acidemias in Korea - Eight years experience of organic acid analysis -
Jun Suck Bang, Si Hong Lim, Kyung Hwa Lee, Eun Joo Bae, Won Il Park, Hong Jin Lee
Clin Exp Pediatr. 2006;49(3):258-267.   Published online March 15, 2006
Purpose : We have done this retrospective study to know the relative incidence and clinical manifestations of organic acidopathies in Korea during 8 years(from Jul. 1997 to May 2005). This results of organic acid analysis of 1,787 patients were compared with the results of organic acid analysis that were published three years ago. Methods : The results of quantitative organic acid...
Quantification of Organic Acids in Amniotic Fluid for Prenatal Diagnosis of Organic Acidemia
Chang Bum Koh, Dong Soo Shin, Eun Joo Bae, Won Il Park, Kyeung Ja Lee, Hong Jin Lee, Young Jo, Seung Won Lee, Ui Sun Noh
Clin Exp Pediatr. 2004;47(10):1047-1052.   Published online October 15, 2004
Purpose : Since 1997, the quantification of organic acids in urine has become possible in Korea. This helped to diagnose a great variety of inborn errors of metabolism. However, we still don't know the normal value of organic acids in amniotic fluid, therefore it is impossible for doctors to make a correct diagnosis of inborn errors of metabolism in prenatal...
Clinical Lecture
Organic Acidemias in Korea
Hong Jin Lee
Clin Exp Pediatr. 2002;45(12):1459-1476.   Published online December 15, 2002
Original Article
Organic Acidemias in Korea - Three Years Experience of Organic Acid Analysis -
You Kyoung Choi, Eung Seok Kim, Eun Joo Bae, Won Il Park, Kyung Ja Lee, Hong Jin Lee
Clin Exp Pediatr. 2002;45(11):1346-1358.   Published online November 15, 2002
Purpose : We have done this retrospective study to know the relative incidences and clinical manifestations of organic acidopathies in Korea. Methods : The results of quantitative organic acid analysis of 1,125 samples of 712 patients, referred from Jul. 1997 to Jun. 2000, were analyzed retrospectively according to four age groups (-2 mon, 3 mon-2 year, 3 years-12 years, over...
Case Report
A Case of Dominantly Inherited β Thalassemia Due to Hb Dieppe
You Kyoung Choi, Hong Jin Lee, Won Il Park, Kyung Ja Lee, Sung Ha Kang, Ji Yeon Kim, Sung Sup Park
Clin Exp Pediatr. 2002;45(5):659-663.   Published online May 15, 2002
β thalassemias are usually transmitted as autosomal recessive traits. However, some dominant forms of β thalassemia have been identified in individuals who have inherited a single copy of an abnormal β globin gene. Thalassemia intermedia with mild anemia, jaundice, and splenomegaly has been observed in these patients. Electrophoresis has shown elevated Hemoglobin(Hb) A2 and Hb F levels. In particular, there...
Original Article
Quantification of Age-related Reference Values for Urinary Organic Acids in the Healthy Korean Population
Yong Jae Choi, In Cheol Kim, You Kyoung Choi, Hong Jin Lee, Bong Sung Kim, Won Il Park, Kyoung Ja Lee, T. Giudic, M. Blaskovics
Clin Exp Pediatr. 2001;44(12):1413-1423.   Published online December 15, 2001
Purpose : For the accurate diagnosis of organic acidopathies, quantification of urinary organic acid should be done and we should know the normal ranges of each organic acid excreted in the urine. The amount of organic acids excreted in the urine shows wide variability according to ethnic group, diet and age. We have quantified 82 organic acids to make a Korean reference value. Methods...
Case Report
A Case of Maple Syrup Urine Disease Controlled by Peritoneal Dialysis and Diet
Ju Wan Kim, June Huh, Won Il Park, Kyung Ja Lee, Hong Jin Lee
Clin Exp Pediatr. 2001;44(1):94-98.   Published online January 15, 2001
Maple syrup urine disease is an autosomal recessive disease caused by a deficiency of the branched-chain α-ketoacid dehydrogenase complex. The disease is often suspected because of the peculiar odor of maple syrup in urine. Maple syrup urine disease is usually confirmed by amino acid analysis and urine organic acid analysis showing marked elevations of leucine, isoleucine, valine, and respective ketoacids in...
A Case of Propionic Acidemia
Woo Seop Yeoum, Kwang Wook Lee, Byeong Ho Chae, Baek Keun Lim, Hong Jin Lee
Clin Exp Pediatr. 1999;42(8):1159-1164.   Published online August 15, 1999
Propionic acidemia is an autosomal-recessive inborn error of branched-chain amino acid metabolism. It is caused by deficient activity of propionyl-coenzyme A carboxylase and is characterized by a spectrum of clinical and biochemical findings. It usually manifests in the neonatal period or early infancy. Since Childs et al first described the propionic acidemia of infants in 1961, it has rarely been...
Erythrocyte Band 7 Integral Protein Defectin Congenital Hemolytic Anemia: Hereditary Stomatocytosis
Chang Seoup Sim, Sang Joo Han, Hong Jin Lee, Woo Ill Park, Kyung Ja Lee, So Young Chung, Sechan Woo, Eui Yul Choi
Clin Exp Pediatr. 1997;40(2):260-264.   Published online February 15, 1997
Hereditary stomatocytosis is a rare congenital hemolytic anemia, named after mouth shaped or stomatocytic erythrocyte morphology. In this report, we present a case of a hereditary stomatocytosis in a 1 month old boy. During the initial identification process, we overlooked the morphology of the RBC in peripheral blood smear and tentatively diagnosed it to be a hereditary spherocytosis case. In order to...
Original Article
Age Related Ca, Phosphorus, Uric acid concentration in Serum & Urine and Ca/Cr ratio, Cua/Ccr & TRP
Jah In Kim, Sang Joo Han, Hong Jin Lee, Won Il Park, Kyung Ja Lee
Clin Exp Pediatr. 1996;39(10):1405-1413.   Published online October 15, 1996
Purpose : There are many methods for the evaluation of the renal function. The proximal tubule of the nephrons have a role of reabsorption of the materials such as water, electrolytes, glucose, amino acids, and small molecular weight protein through the glomerular filtration. Therefore if abnormality is in the proximal tubule, these materials can be changed both in serum & urine. It is very...
A Clinical Study of Benign Epilepsy of Childhood with Centrotemporal Spikes
Byung Il Lee, Sang Ju Han, Hong Jin Lee, Won Il Park, Kyung Ja Lee
Clin Exp Pediatr. 1994;37(9):1251-1256.   Published online September 15, 1994
Benign epilepsy of childhood with centrotemporal spikes(BECCT) is an electroclinical syndrome characterized by noctural seizure that remit spontaneouly before adulthood, and belong to idiopathic age and location related epilepsies. We reviewed the medical records to analyse the seizure pattern, and also inspect the EEG recording to identify topography of the epileptiform discharge of 24 patient who met the following criteria: 1)presence...
Clinical Observation of urinary Tract Anomalies
In Hee Jung, Hong Jin Lee, Sang Joo Han, Won Il Park, Kyung Ja Lee
Clin Exp Pediatr. 1994;37(8):1092-1096.   Published online August 15, 1994
Urinary tract anomaly is the most common anomaly in childhood. We reviewed medical records of 45 patients who were confirmed urinary tract anomalies on radiologic studies as Chuncheon Sacred Hospital, from Dec. 1984 to Sep. 1992. We analyzed incidence and clinical characteristics of urinary tract anomalies. The results obtained were as follows: 1) The age distrtibution were: 17 cases (37.8%) under...
Effect of Different Levels of the Dietary Protein on Long Term Prognosis of Experimental Renal Damage
Choon Haeng Lee, Sang Joo Han, Hong Jin Lee, Won Il Park, Kyung Ja Lee, Young Eui Park, Min Chul Lee, Tae Heon Yoon
Clin Exp Pediatr. 1993;36(6):820-829.   Published online June 15, 1993
We examined the effect of various levels of dietary protein on long term prognosis of Adriamycinephropathy of S-D rat, fed with high protein(30%), intermediately low(10%), and strictly low(5%) protein diet for 15 weeks 1) In rats fed with strictly low protein diets(5%), proteinuria and serum creatinine decreased and creatinine clearance and histological changes were relatively well preserved. But hypoproteinmia and weight...
Case Report
A Case of Stein-Leventhal Syndrome with Severe Obesity
Kyeong Sang Kim, In Hee Jung, Hong Jin Lee, Won Il Park, Kyung Ja Lee
Clin Exp Pediatr. 1992;35(8):1164-1168.   Published online August 15, 1992
We experienced a extremely rare case of Stein-Leventhal Syndrome in 7 year-old female patient. Her chief complaints was severe obesity. We performed specific radiologic and hormonal studies. Diagnosis was suspected by pelvic sono and CT. We performed vertical gastroplasty with Taplon bandage for morbid obesity. A brief review of related literature was made.
Original Article
HDL Cholesterol, Copper, Ceruloplasmin, Zinc, Iron Values of the Blood in Newborn
Kyeong Sang Kim, Chun Hang Lee, Hong Jin Lee, Won Ill Park, Kyung Ja Lee, Tae Hyon Yoon, Won Chan Tae
Clin Exp Pediatr. 1992;35(8):1096-1101.   Published online August 15, 1992
HDL choleaterol, copper, ceruloplasmin, zinc, and iron values of the blood of 85 newborns delivered at hallym University hospital from September, 1989 to February, 1991 were analysed. The results were as follows: 1) The mean value of HDL cholesterol in 50 normal newborns was 31.37¡¾9.47mg/dl, that of copper 80.54¡¾17.54ug/dl, that of ceruloplasmin 11.34¡¾6.37mg/dl, that of zinc 92.81¡¾79.74ug/dl, that of iron 198.98¡¾86.07 ug/dl. 2)...
Clinical study on epileptiform discharge in children.
Wonil Park, Hong Jin Lee, Kung Ja Lee
Clin Exp Pediatr. 1991;34(11):1559-1564.   Published online November 30, 1991
Seizures are the most common neurologic manifestation in childhood. The demonstration of epileptiform disshonge on EEG not only support clinical diagnosis but also aid the classification of epilepsy. 174 patients with recurrent seizure were taken EEG studies in Chuncheon Sacred Heart Hospital from Jan. 1985 to Dec. 1990. and 71 patients (40.8%) of them occured epilepiform discharge in inital EEG recording. The incidence of epilepiform pattern...
Seventeen Years Experience on Inborn Errors of Metabolism A Study for Future Development of Inborn Errors of Metabolism in Korea.
Hong Jin Lee, Hyung Ro Moon
Clin Exp Pediatr. 1990;33(8):1031-1036.   Published online August 31, 1990
In Korea, the study of inborn errors of metabolism is very primitive. Even the incidence and prevalence of the disease of inborn errors of metabolism are not known in Korea. We have done this study of inborn errors of metabolism in Korea and concluded that: 1) The varieties and incidences of inborn errors of metabolism in Korea might be relatively diverse and high. 2) Nation wide mandatory...
Late Infantile Metachromatic Leukodystrophy-Arylsulfatase A Assay in 24h Urine.
Hong Jin Lee, Yong Joon Shin, Yong Seung Hwang, Hyung Ro Moon, Jeong Seon Seo
Clin Exp Pediatr. 1989;32(7):978-983.   Published online July 31, 1989
A 2 year 5 month old female infant with late infantile metachromatic leukodystrophy was diagnosed by typical clinical and laboratory findings and Arylsulfatase A assay in 24h urine. The patient’s mother was confirmed as a heterozygote by Arylsulfatase A activities in 24h urine which was about one half of normal control groups.
Urinary Red Blood Cell Morphology in Hematuria.
Hong Jin Lee, Chang Yeon Lee, Hae Il Cheong, Yong Choi, Kwang Wook Ko, Myung Hee Park
Clin Exp Pediatr. 1988;31(11):1460-1467.   Published online November 30, 1988
We have studied morphological alterations in urinary RBC as a tool for the differential diagnosis of hematuria in children, that is, differentiation of glomerular bleeding from nonglomerular bleeding, and the results were as follows; 1) Urinary RBCs from glomerular diseases showed higher rate of dysmorphism (84.3±16.8%), and those from nonglomerular diseases did lower one (15.2 ±12.9%) with phase contrast microscopic examination. 2) Simple light microscopic examination after Wright’s...
A Clinicopathologic Study on Postinfectious Glomerulonephritis.
Hong Jin Lee, Kwang Wook Ko
Clin Exp Pediatr. 1988;31(6):723-730.   Published online June 30, 1988
38 biopsy proven postinfectious glomerulonephritis patients were analyzed retrospectively in view of clinicopathological correlation. 1) Of the 38 patient reviewed, 23 cases were male and 15 female and the male to female ratio was 1.5:1. The mean age of onset was 9.8±3.2 years old with 2 peaks at 7 and 12 years of age. 2) The clinical presentations on admission were acute nephritic syndrome (15 cases,...
A Case of Familial Dysautonomia.
Hong Jin Lee, Dong Kyu Jin, Sei Won Yang, Jeong Kee Seo, Hyung Ro Moon, Je Geun Chi
Clin Exp Pediatr. 1988;31(5):648-654.   Published online May 31, 1988
Few documented cases of Familial dysautonomia fulfilling current diagnostic criteria have been recognized in non-Jews especially in orientals. In our case diagnosis was established in 8 year old Korean girl. She fulfilled 8 out of 9 essential diagnostic criteria of Riley. It represents a report of this syndrome with achalasia and improved with modified Heller’s myotomy.
A Study on Minimal Change Nephrotic Syndrome in Children.
Yong Choi, Hong Jin Lee, Hae Il Cheong, Kwang Wook Ko, Hyun Soon Lee, Yong Il Kim
Clin Exp Pediatr. 1988;31(2):220-226.   Published online February 28, 1988
To assess the clinical significance of histopathologic variants of minimal change nephrotic syn- drome (MCNS) by light microscopic findings, 148 biopsy proven MCNS patients, who were admitted to Seoul National University Hospital between the period from January 1976 to December 1986, were analyzed according to clinical findings, steroid responsiveness, and follow up status in relation to the pathologic variants. The followings are summary and conclusion. 1) Significantly...
A Case of Type A Niemann Pick Disease.
Hyo Nam Cho, Hong Jin Lee, Jae Won Song, Jung Hwan Choi, Hyung Ro Moon, Je Geun Ji, Myeong Hee Park
Clin Exp Pediatr. 1987;30(12):1461-1467.   Published online December 31, 1987
We experienced a case of type A Niemann Pick disease in 13 month female baby. She showed marked hepatosplenomegaly, developmental regression and apathy. Fundoscopic examination revealed a cherry red spot in the macula. Numerous foamy histiocyte were seen in bone marrow. Autopsy finding revealed that liver, spleen, thymus, lymph node, lung and brain were involved. Liver showed fatty change. Generalized muscular atrophy and patch...
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