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Review Article
Pathophysiology and management of disorders in water metabolism
Dong Un Kim
Clin Exp Pediatr. 2007;50(5):430-435.   Published online May 15, 2007
Even though we drink and excrete water without recognition, the amount and the composition of body fluid remain constant everyday. Maintenance of a normal osmolality is under the control of water balance which is regulated by vasopressin despite sodium concentration is the dominant determinant of plasma osmolality. The increased plasma osmolality (hypernatremia) can be normalized by the concentration of urine,...
Case Report
Joubert syndrome with peripheral dysostosis - A case report of long term follow-up -
Jung Tae Kim, Sun Jun Kim, Chan-Uhng Joo, Soo Chul Cho, Dae-Youl Lee
Clin Exp Pediatr. 2007;50(3):315-318.   Published online March 15, 2007
This report describes the long-term follow-up of a 10-year-old female patient with Joubert syndrome with short stature and brachydactyly. She presented with hyperpnea alternated with hypopnea, uncontrolled jerking eye movements, and hypotonia during early infancy. She was diagnosed with Joubert syndrome based on clinical symptoms and typical MRI findings at 5 months of age. Abnormal ventilation and eye movements disappeared...
Original Article
Cord blood IL-10, IL-12 in preterm newborns as predictors of respiratory distress syndrome and bronchopulmonary dysplasia
Jee Yoon Park,, Ji Young Kim, Soo Jin Cho, Young Ju Kim, Hye sook Park, Eun Hee Ha, Eun Ae Park
Clin Exp Pediatr. 2007;50(3):248-254.   Published online March 15, 2007
Purpose : Inflammation plays a major role in the pathogenesis of RDS and BPD in the immature lung. We investigated the possible role of IL-10 and IL-12 in the cord blood of preterm newborns with RDS or BPD. Methods : Forty preterm newborns whose mothers received antenatal care at Ewha Womans University Mokdong Hospital between January 2003 to June 2005, and...
Clinical application of D-dimer in Kawasaki Disease
Jae Joon Han, Hong Ki Ko, Young Yoo, JungHwa Lee, Kwang Chul Lee, Chang Sung Son, Joo Won Lee
Clin Exp Pediatr. 2007;50(2):205-208.   Published online February 15, 2007
Department of Pediatrics, College of Medicine, Korea University, Seoul, Korea Purpose : Vascular endothelial cell damage and alteration of a fibrinolytic system was suggested to play a role in the development of coronary artery abnormalities in Kawasaki disease (KD). D-dimer is one of the markers of endothelial damage and fibrinolysis. We evaluated the clinical usefulness of D- dimer to differentiate KD...
The effects of high-dose intravenous immunoglobulin on plasma protein and lipid levels in the patients with Kawasaki disease
Keun Young Lee, Dong-Un Kim, Hyun Seung Lee, Pil Sang Jang, Young-Hoon Kim, Jin Tack Kim, Hyun Hee Kim, Kyung-Yil Lee, Joon-Sung Lee
Clin Exp Pediatr. 2006;49(12):1348-1353.   Published online December 15, 2006
Purpose : The reticuloendothelial system is composed of sinusoidal capillaries, through which even large protein molecules are freely movable between plasma and interstitial space, including the lymphatic system. Therefore, high-dose intravenous immunoglobulin (IVIG) would cause a redistribution of proteins between two compartments. To investigate this hypothesis, we measured plasma protein and lipid levels in patients with Kawasaki disease before and...
Short-term follow up of thyroid function after pediatric hematopoietic stem cell transplantation
Seon-Ju Lee, Jae-Wook Lee, Dae-Hyoung Lee, Young-Joo Kwon, Young-Shil Park, Hui Sung Hwang, Sun Young Kim, Ji Kyoung Park, Pil-Sang Jang, Min-Ho Jung, Nak-Gyun Chung, Dae-Chul Jeong, Bin Cho, Hack-Ki Kim, Byung-Churl Lee
Clin Exp Pediatr. 2006;49(11):1211-1215.   Published online November 15, 2006
Purpose : In this study, we analyzed the short term changes of thyroid function, incidence and risk factors of thyroid dysfunction soon after allogeneic hematopoietic stem cell transplantation (HSCT) in children. Methods : We enrolled 80 pediatric patients following allogeneic HSCT, at the Catholic HSCT center between January, 2004 and February, 2006. Serum TSH (thyroid stimulating hormone), total serum thyroxine and...
Modified Tei index in patients with Kawasaki disease by tissue doppler imaging
Hee Jung Kim, Jung Hwa Cha, Young Mi Hong
Clin Exp Pediatr. 2006;49(11):1202-1210.   Published online November 15, 2006
Purpose : A quantitative and easily measured Doppler index of combined systolic and diastolic ventricular myocardial performance (Tei index) was recently proposed as a potentially useful predictor of global myocardial performance. However, presence of heart rate fluctuation makes it unreliable. Therefore, the modified Tei index was introduced by using tissue Doppler imaging (TDI) which enables measuring contraction and relaxation velocities...
A case of hereditary pancreatitis with a N29I mutation in the cationic trypsinogen gene
Jee Youn Shin, You Jeong Kim, Jeong Kee Seo
Clin Exp Pediatr. 2006;49(10):1067-1072.   Published online October 15, 2006
Purpose : The purpose of this study was to examine the clinical courses and long-term outcomes of children with Allagille syndrome in Korea, and to evaluate the prognostic potentials of identified variables. Methods : We reviewed the clinical manifestations and outcomes of 30 children with Alagille syndrome, investigated from 1984 to 2006 until the end of this study (defined as death...
Case Report
Infliximab treatment for a patient with refractory Kawasaki disease
Hyo-Jung Yu, Soo-Jin Lee, Sejung Sohn
Clin Exp Pediatr. 2006;49(9):987-990.   Published online September 15, 2006
Intravenous immunoglobulin (IVIG) infusion is an effective therapy for acute Kawasaki disease (KD). Nonetheless, approximately 10 percent to 20 percent of patients have persistent or recrudescent fever despite IVIG treatment, leading to a higher risk for coronary artery aneurysms (CAA). This unresponsiveness may pose a challenge to the clinicians. Tumor necrosis factor-α levels are elevated in the acute phase of...
Review Article
Giant platelet syndrome
Hoon Kook
Clin Exp Pediatr. 2006;49(8):833-838.   Published online August 15, 2006
Giant platelet syndrome is a group of unique disorders characterized by the presence of abnormally large platelets, and usually accompanied by thrombocytopenia. Most cases of giant platelets are encountered in idiopathic thrombocytopenic purpura(ITP). In contrast, inherited giant platelet disorders, a group of heterogeneous diseases, are rare. Bernard-Soulier syndrome and its variants, and MYH9 related diseases have been defined at the...
Type 2 diabetes mellitus and metabolic syndrome
Jin Soon Hwang
Clin Exp Pediatr. 2006;49(7):710-717.   Published online July 15, 2006
Type 2 diabetes mellitus in children and adolescents has been increasing for last 10 years. The increase in frequency of type 2 diabetes appears to parallel the increase in prevalence and severity of obesity in children and adolescents. The metabolic syndrome, cluster of potent risk factors for atherosclerotic cardiovascular disease and type 2 diabetes, consists of insulin resistance, obesity, hypertension...
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...
Case Report
A case of Tolosa-Hunt syndrome
Do Gyun Kim, Young Ok Kim, Young Jong Woo
Clin Exp Pediatr. 2006;49(6):696-699.   Published online June 15, 2006
소아에서 드물게 보고되는 토로사-헌트 증후군은 둔하면서 지속적인 안와 주위의 통증과 안구운동 장애 및 해면동 주위의 뇌신경 침범을 특징으로 하는 질환으로 비특이적 염증조직에 기인한 것으로 알려져 있다. 이는 자연 치유도 가능하나 대개 스테로이드가 증상의 회복에 효과적이며 신속한 호전을 유도한다. 토로사-헌트 증후군은 그 예후가 양호하다고 알려져 있으나 일부는 치료 후에 재발하기도 한다. 우리는 토로사-헌트 증후군으로 진단받고 스테로이드 치료 후 특별한 휴우증 없이...
Original Article
Serum high sensitivity C-reactive protein levels in obese middle school boys
Jae-Ho Jeong, Jae-Woo Lim, Eun-Jeong Cheon, Kyong-Og Ko, Young-Hyuk Lee
Clin Exp Pediatr. 2006;49(6):617-622.   Published online June 15, 2006
Purpose : High-Sensitivity C-reactive protein(hs-CRP) has been recognized as a very useful and sensitive predictor of the future risk of myocardial infarction. But the clinical significance of hs-CRP in children remains uncertain. To confirm the existence of obesity-induced vascular inflammation and the association between metabolic syndromes and elevation of CRP in children, we investigated the relationship among CRP, obesity, blood...
A survey of infant sleep positions associated with sudden infant death syndrome
Dong Jun Lee, So Ick Jang, Eun Jung Shim, Do Jun Cho, Dug Ha Kim, Ki Sik Min, Ki Yang Yoo
Clin Exp Pediatr. 2006;49(6):602-609.   Published online June 15, 2006
Purpose : As the prone position is thought to be an important factor in sudden infant death syndrome(SIDS), this study was conducted to contribute to reducing SIDS by analyzing sleeping positions of infants. Methods : A face-to-face questionnaire was carried out with a total of 170 parents with a baby aged less than 6 months. Results : A total of 170 infants...
Case Report
A case of Rothmund-Thomson syndrome
Seung Hyo Kim, Choong Ho Shin, Sei Won Yang
Clin Exp Pediatr. 2006;49(5):565-569.   Published online May 15, 2006
Rothmund-Thomson syndrome (RTS), is a rare autosomal recessive disorder, characterized by : skin photosensitivity, poikiloderma, sparse hair, sparse eyebrows/lashes, short stature, skeletal abnormalities, cataracts, and an increased risk of malignancy. Skeletal abnormalities include : dysplasia, absent or malformed bones, such as absent radii, osteopenia, and delayed bone formation. RTS is thought to result from chromosomal instability, and children with RTS...
Original Article
Jagged1 mutation analysis in Alagille syndrome patients
Jae Sung Ko, Hye Ran Yang, Kyung Mo Kim, Jeong Kee Seo
Clin Exp Pediatr. 2006;49(5):519-522.   Published online May 15, 2006
Purpose : Alagille syndrome is an autosomal dominant disorder with developmental abnormalities affecting the liver, heart, eyes, vertebrae, and craniofacial region. The Jagged1(JAG1) gene, which encodes a ligand of Notch, has been found mutated in Alagille syndrome. The aim of the study was to investigate the mutation analysis of JAG1 gene in Korean patients with Alagille syndrome. Methods : Genomic DNA...
Case Report
A case of acute respiratory distress syndrome treated with surfactant and low dose methylprednisolone
Bo Yeon Choi, Kyong Mo Kim, Jong Seo Yoon, Joon Sung Lee
Clin Exp Pediatr. 2006;49(4):455-459.   Published online April 15, 2006
The major pathogenesis of acute respiratory distress syndrome (ARDS) is an inflammatory process that results from a diversity of injuries to the body. Due to the various cytokines and vasoactive peptides released from the endothelium, the vascular permeability is increased; the migration of inflammatory cells and the leakage of plasma proteins then occur and edema develops in the alveolus. There...
A case of Dyke-Davidoff-Masson syndrome in Korea
Jun Hwa Lee, Zee Ihn Lee, Ho Kyun Kim, Soon Hak Kwon
Clin Exp Pediatr. 2006;49(2):208-211.   Published online February 15, 2006
Dyke-Davidoff-Masson Syndrome (DDMS) is a rare condition characterized by asymmetry of cerebral hemispheric growth with atrophy on one side, ipsilateral compensatory osseous hypertrophy, and contralateral hemiparesis. We experienced a 17 month-old male who presented with left focal clonic or tonic-clonic seizures accompanied by left hemiparesis and developmental delay. Brain MRIs demonstrated progressive atrophy of the right cerebral hemisphere with dilatation...
Development of Crohn disease in patients with myelodysplastic syndrome : report of two children
Jeong Ok Shim, Jeong Kee Seo, Hye Ran Yang, Jae Sung Ko, Hee Young Shin, Hyo Seop Ahn, Woo Sun Kim, Gyeong Hoon Kang
Clin Exp Pediatr. 2006;49(1):107-111.   Published online January 15, 2006
Crohn disease (CD) is rare, but the incidence of CD has been increasing over the past ten years. We found two cases of CD, associated with myelodysplastic syndrome (MDS), for the first time in children. In the first patient, MDS was diagnosed at three years of age and CD developed later at eight years of age. The patient presented with...
Original Article
A Cytogenetic Study in Patients with Sex Chromosome Abnormalities
Hyun Ji Seo, Ji Hye Lee, Heung Kyo Lee, Seung Hee Jung, Kun Soo Lee
Clin Exp Pediatr. 2005;48(12):1317-1323.   Published online December 15, 2005
Purpose : This study was performed to evaluate the recent frequency of karyotypes in different sex chromosome abnormalities and to evaluate the age and clinical manifestations at diagnosis. Methods : Peripheral blood leukocytes were obtained from subjects who were clinically suspected to have sex chromosome abnormalities and referred to the cytogenetic laboratory in the Department of Pediatrics, Kyungpook National University Hospital...
Serum Ferritin as a Risk Factor in Type 2 Diabetes Mellitus
Jung Hyun Kim, Ho Seong Kim, Duk Hee Kim
Clin Exp Pediatr. 2005;48(11):1239-1243.   Published online November 15, 2005
Purpose : Iron accumulation interferes with hepatic insulin extraction and affects insulin synthesis and secretion. The purpose of this study is to investigate the correlation between serum ferritin and type 2 diabetes mellitus. Methods : We compared the serum ferritin level among 18 patients in an impaired glucose tolerance (IGT) group, 36 in a type 1 diabetes group, eight in...
Clinical and Epidemiologic Study of Kawasaki Disease in Children 8 Years of Age and Older
Yong Won Park, Ji Whan Han, In Sook Park, Chang Hwi Kim, Sung Ho Cha, Jae Sook Ma, Tae Chan Kwon, Sang Bum Lee, Chul Ho Kim, Heung Jae Lee, Yong Soo Yun
Clin Exp Pediatr. 2005;48(10):1139-1142.   Published online October 15, 2005
Purpose : The aim of this study was to investigate the epidemiologic and clinical profiles of Kawasaki disease(KD) in children 8 years of age and older. Methods : For the epidemiologic study of KD in Korea, data of total 15,692 KD patients were collected from 1994 to 2002 on a 3 year basis, by the retrospective survey. Among them, data of...
A Study on the Effects of Early Surfactant Replacement and Gentle Ventilation in the Treatment of Respiratory Distress Syndrome
Yong Suk Lee, Ji Hye Lee, Sang Geel Lee
Clin Exp Pediatr. 2005;48(10):1096-1101.   Published online October 15, 2005
Purpose : Recently, early surfactant replacement and tidal volume based gentle ventilation has been a fundamental treatment of respiratory distress syndrome(RDS). The aims of this study were to survey the changes in ventilator care duration and rate of complication in RDS groups. Methods : We performed a retrospective study of 255 newborn infants less than 1,500 g admitted to the...
Comparison of Clinical Progress between Single- and Multiple-dose Surfactant Treatment in Neonatal Respiratory Distress Syndrome
Chang Hee Kil, Ho Sang Jeon, Chong Woo Bae
Clin Exp Pediatr. 2005;48(10):1090-1095.   Published online October 15, 2005
Purpose : In the case of serious respiratory distress syndrome(RDS) or relapse of clinical appearances after single treatment, we obtained more effective results with multiple-dose surfactant replacement therapy. We carried out this investigation for comparing and observing clinical progress between single-dose(group S) and multiple-dose(group M) pulmonary surfactant treatment group of neonatal RDS. Methods : We investigated 48 neonates who were...
Clinical Lecture
Can We Predict How Often Nephrotic Syndrome will Relapse into the Patients?
Mee Kyung Namgoong
Clin Exp Pediatr. 2005;48(10):1033-1037.   Published online October 15, 2005
Pediatric nephrotic syndrome is a well-known illness for its high relapsing rate. If we can predict the relapsing rate and the responses to the steroid therapy of individual patients with nephrotic syndrome, the predictability will be helpful in building a therapeutic plan. Here is my review of research articles on the risk factors for the prediction of relapsing nephrotic syndrome.
Case Report
A Case of Young-Simpson Syndrome
Ja-Young Hwang, Se-Young Seo, Seong-Hoon Hahn, So-Young Kim, Hyun-Hee Kim, Wonbae Lee
Clin Exp Pediatr. 2005;48(9):1016-1018.   Published online September 15, 2005
Young Simpson syndrome is a rare malformation syndrome characterized by congenital hypothyroidism, dysmorphic face, mental retardation, severe postnatal growth retardation, hypotonia and congenital heart abnormalities. In the present study, we report a case of 4-year-old girl with Young Simpson syndrome for the first case in Korea.
Giant Coronary and Axillary Aneurysms in an Infant with Kawasaki Disease Associated with Thrombocytopenia
Sei Young Seo, Jin Hee Oh, Jong-Hyun Kim, Ji-Whan Han, Kyung-Yil Lee, Dae Kyun Koh
Clin Exp Pediatr. 2005;48(8):901-906.   Published online August 15, 2005
Kawasaki disease (KD) is a leading cause of acquired heart disease in children. Yet the etiology of KD is still unknown and diagnosis depends on the exclusion of other diseases and the clinical manifestations meeting the defined criteria. Young infants frequently show atypical clinical courses and are frequently complicated with coronary aneurysms. Some cases show thrombocytopenia, which is known as...
Original Article
Clinical Outcome and Prognostic Factors of Acute Respiratory Distress Syndrome in Children
Jung-Min Ko, Eun-Ju Ha, Eun-Hee Lee, So-Youn Lee, Hyo-Bin Kim, Soo-Jong Hong, Seong-Jong Park
Clin Exp Pediatr. 2005;48(6):599-605.   Published online June 15, 2005
Purpose : The purpose of this study was to examine the causes, clinical courses and outcomes in children with acute respiratory distress syndrome(ARDS), and evaluate the physiologic variables as prognostic factors in the patients. Methods : Retrograde medical chart review was carried out in 24 patients who were diagnosed with ARDS at the pediatric intensive care unit(PICU) during 20-month period. Results...
Case Report
A Case of Wiskott-Aldrich Syndrome with Novel Mutation in Exon 2 of the WASP Gene
Hyuk Lee, Jung In Park, Sun Young Kim,, Kyeung Hee Moon, Ho Keun Yi, Pyoung Han Hwang
Clin Exp Pediatr. 2005;48(5):551-556.   Published online May 15, 2005
Wiskott-Aldrich syndrome(WAS) is an X-linked recessive immunodeficiency characterized by thrombocytopenia with small platelet volume, eczema, and recurrent infections, and is also characterized by increased incidence of auto immune diseases and malignancies. The phenotype observed in this syndrome is caused by mutation in the Wiskott-Aldrich syndrome protein(WASP) gene localized to the proximal short arm of the X chromosome and recently isolated...
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