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Original Article
Association of Diagnostic Criteria and Autoantibodies with Juvenile Dermatomyositis in Newly Diagnosed Children
Kyung Sue Shin, Joong Gon Kim
Clin Exp Pediatr. 2003;46(9):898-902.   Published online September 15, 2003
Purpose : To determine the clinical association of diagnostic criteria and the prevalence of autoantibodies in newly diagnosed children with juvenile dermatomyositis(JDM). Methods : Thirty-two children with JDM were identified at Seoul National University Children's Hospital from March 1985 to March 1999 by retrospective review. The diagnosis of JDM was based on the criteria proposed by Bohan and Peter. We investigated...
Case Report
A Case of Intestinal Tuberculosis Concurring with Endobronchial and Laryngeal Tuberculosis
Su Jin Kim, Su Eun Park, Min Gi Lee, Gun Il Kim, Chang Hun Lee, Jae Hong Park
Clin Exp Pediatr. 2003;46(7):714-717.   Published online July 15, 2003
Although the incidence of tuberculosis has been reduced recently because of the advance of anti-tuberculosis chemotherapy, improvements of public health, and early diagnosis, it is still high in developing countries. Intestinal tuberculosis can occur at any age, but young adults and female are more affected. Early diagnosis of childhood intestinal tuberculosis is difficult, largely because of vague symptoms and nonspecific...
Original Article
Sensitization of TNFα and Agonistic FAS/CD95 Antibody-Induced Apoptosis by INFγ on Neuroblastoma Cells
Ho Il Bang, Jong Duck Kim, Du Young Choi
Clin Exp Pediatr. 2003;46(7):702-709.   Published online July 15, 2003
Purpose : IFNγ sentitizes many tumor cells to TNFα and FASL-mediated apoptosis by enhancing the expression of TNF or FAS/CD95 receptor and modulating the activation of caspase and Bcl-2 family. It has been reported that IFNγ and TNFα synergistically caused differentiation and growth inhibition of neuroblastoma cells. Even though some neuroblastoma cell express FASR/FASL on the cell surface, they could...
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...
Original Article
Plasma G-CSF and GM-CSF Concentration and Amount of Their Receptors on the Granulocyte in Kawasaki Disease
Young-Kyoung Yoo, Gibum Lee, Hyun-Hee Kim, Soo-Young Kim, You-Jeong Kim, Wonbae Lee
Clin Exp Pediatr. 2003;46(4):376-381.   Published online April 15, 2003
Purpose : This study aimed to demonstrate the possible pathogenesis of granulopoiesis in patients of Kawasaki disease(KD) using quantitative analysis of G-CSF, GM-CSF and their CSFr. Methods : The plasma levels of G-CSF, GM-CSF, G-CSFr and GM-CSFr were studied in 14 patients in the acute phase of KD; 13 children with normal peripheral white blood cell counts were used as the...
Prognostic Value of Repeated Cerebrospinal Latex Agglutination Testing in Bacterial Meningitis
Soo Jeong Lee, So Hyun Ahn, Su Jin Cho, Hae Soon Kim, Seung Joo Lee
Clin Exp Pediatr. 2003;46(4):345-350.   Published online April 15, 2003
Purpose : Bacterial meningitis is a serious infection of childhood associated with a significant morbidity and mortality. Repeated cerebrospinal fluid(CSF) examination is a useful prognostic indicator and a delayed sterilization is associated with a higher incidence of neurologic abnormalities. In this study we tried to determine the prognostic value of repeated CSF latex agglutination testing. Methods : We retrospectively evaluated 19...
Plasma G-CSF and GM-CSF Concentrations and Expression of their Receptors on the Granulocyte in Children with Leukocytosis
Won Seok Choi, Kyung Hwan Ryu, You Jeong Kim, So Young Kim, Hyun Hee Kim, Wonbae Lee
Clin Exp Pediatr. 2003;46(3):271-276.   Published online March 15, 2003
Purpose : Granulocyte-colony stimulating factor(G-CSF) and granulocyte macrophage-colony stimulating factor(GM-CSF) are principal cytokines in granulopoiesis and their physiologic effects are mediated through binding to specific cell surface receptors. Although it is known that the level of serum G-CSF and GM-CSF, and presentation of the receptors are increased in infectious diseases, there have been no studies to find the correlation between...
Influence of Prognostic Factors on Survival Rate of Medulloblastoma Patient with Chemotherapy
Kyung Mi Shin, Sung Yeon Choi, Sung Chul Won, Chang Hyun Yang, Chuhl Joo Lyu, Chang Ok Suh, Joong Uhn Choi, Byung Soo Kim
Clin Exp Pediatr. 2003;46(2):178-182.   Published online February 15, 2003
Purpose : Brain tumors are the second most common tumor in childhood, and medulloblastomas comprise 15-25% of brain tumors. The well known prognostic factors are age at diagnosis, stage of disease, and extent of surgical excision. In this study, we analysed the prognostic factors in patients who received chemotherapy after excision. Methods : We reviewed the medical records of 61...
Case Report
A Case of Klinefelter Syndrome associated with Unilateral Multicystic Dysplastic Kidney in a Newborn Infant
Kyung A Ha, Sun Mi Chung, Eun Jin Choi, Jin Kyung Kim, Un Seok Nho, Jae Shin Park, Woo Taek Kim, Young Dae Kwon
Clin Exp Pediatr. 2002;45(9):1141-1145.   Published online September 15, 2002
Klinefelter syndrome is the most common chromosomal abnormality, with a 47, XXY karyotype and typical clinical findings of infertility, hypogonadism, reduced body hair, gynecomastia, tall stature, and incresed gonadotropins and decreased testosterone levels. In addition to this classic description, several other diseases have been discribed in Klinefelter syndrome such as unilateral renal aplasia, autoimmune disease, diabetes mellitus, sexual precoxity, renal...
Original Article
A Clinical Study of Children Who Showed Benign Hemophagocytic Histiocytes in Bone Marrow Examination
Jin A Park, Joo Young Park, Ji Min Park, Seat Byoul Park, Heung Sik Kim, Dong Seok Jeon
Clin Exp Pediatr. 2002;45(8):1007-1015.   Published online August 15, 2002
Purpose : The aim of this study is to find a method to diagnose and treat children who showed benign hemophagocytic histiocytes in bone marrow examination. Methods : We analyzed the clinical data of thirty patients retrospectively who showed benign hemophagocytic histiocytes in bone marrow examination from January 1995 to November 2001 at Keimyung University Dong-san Hospital. Bone marrow histiocytes...
Case Report
A Case of Nonspecific Interstitial Pneumonia in a Child
Tae Wan Kim, Dae-Hyun Lym, Jung Hee Kim, Byong Kwan Son, Hye-Seung Han, Young Kyu Shin
Clin Exp Pediatr. 2002;45(4):529-534.   Published online April 15, 2002
Department of Pediatrics†, College of Medicine, Korea University, Seoul, Korea Interstitial pneumonia is a heterogenous group of inflammatory and fibrosing lesions that manifest themselves as infiltrative lung disease. Of these, nonspecific interstitial pneumonia is characterized as a variable degree of interstitial inflammation with or without fibrosis and is distinguished from usual interstitial pneumonia and desquamative interstitial pneumonia, histologically. The influx of...
Original Article
Local Rifampicin Instillation Therapy for Suppurative Bacillus Calmette-Gu rin Lymphadenitis
Mee Jeong Kim, Seong Hee Jang, Young Min Ahn, Mi Kyoung Kang, Sang Jae Kim
Clin Exp Pediatr. 2002;45(4):454-458.   Published online April 15, 2002
Purpose : Bacillus Calmette-Gu rin(BCG) lymphadenitis is one of the most common complications of BCG vaccination. The involved lymph nodes usually subside spontaneously, but they may become enlarged and form an abscess. Treatment of these infants is controversial. The Pan American Health Organization recommends local isoniazid or rifampicin instillation for patients with suppurative BCG lymphadenitis. Methods : The study group comprised...
Therapeutic Effect of Different Doses of Recombinant Human Granulocyte Colony-Stimulating Factor(rhG-CSF) on Neonatal Sepsis Complicated by Neutropenia
Moon Young Choi, Yeon Sook Jung, Dong Woo Son, Hyo Seop Ahn
Clin Exp Pediatr. 2002;45(4):439-448.   Published online April 15, 2002
Purpose : The aim of this study is to determine and compare the effects of adjunctive therapy with different doses of recombinant human granulocyte-colony stimulating factor(rhG-CSF) on reversing sepsis-associated neonatal neutropenia, and their survival rate in a group I/II-type trial. Methods : RhG-CSF was injected subcutaneously to 10 septic-neutropenic neonates with doses of 10 μg/kg from Oct. 1995 to Sep. 1996,...
Short-term Results of Hematopoietic Stem Cell Transplantation for Children with Myelodysplastic Syndrome
Jin Lee, Soh Yeon Kim, Bin Cho, Pil Sang Jang, Nak Gyun Chung, Hack Ki Kim
Clin Exp Pediatr. 2002;45(3):370-375.   Published online March 15, 2002
Purpose : In most cases, myelodysplastic syndrome(MDS) transforms into a more aggressive state or acute myelogenous leukemia; it's prognosis is very poor. It is believed that hematopoietic stem cell transplantation(HSCT) is the only curative treatment of MDS, but available data in children are very sparse. In this report, the short term outcome of HSCT in childhood MDS was analyzed. Methods :...
Case Report
A Case of 4q Deletion with Partial Agenesis of Corpus Callosum
Mi Na Kang, In Suk Lim, Byeong Eui Kim, Myoung Jae Chey, Sang Woo Kim
Clin Exp Pediatr. 2002;45(2):273-277.   Published online February 15, 2002
Syndrome of 4q deletion is characterized by an abnormal shape of the skull, craniofacial dysmorphism, cardiovascular malformations, genitourinary defects, limb and digital anomalies, and developmental delay. We experienced a case of 4q interstitial deletion in a 2 day-old female neonate who showed short extremities, partial agenesis of corpus callosum and congenital heart defects. We report the case with a brief...
A False Diagnosis of Hypoplastic Left Heart Syndrome in a Fetus
Youn Jeong Shin, Sung Hee Jang, Jung Yun Choi, Eun Sook Han
Clin Exp Pediatr. 2002;45(2):267-272.   Published online February 15, 2002
Typical hypoplastic left heart syndrome(HLHS) is a distinct pathologic entity with aortic atresia, mitral atresia, very hypoplastic or absent left ventricle and thread like ascending aorta. Occasionally, the lesser degree of hypoplasia is found and is called hypoplastic left heart complex(HLHC) by some authors. This HLHC is often associated with critical aortic stenosis. Fetal echocardiography has enabled us to observe...
Original Article
Clinical Study of Renal Cystic Diseases in Children
Ja Hyung Kim, You Jeong Kim, Byeong Seon Lee, Tae Sung Ko, Young Seo Park
Clin Exp Pediatr. 2002;45(2):232-239.   Published online February 15, 2002
Purpose : Renal cystic diseases comprise a mixed group of heritable, developmental and acquired disorders. Recently the use of imaging modalities such as ultrasonography and radionuclide scanning has increased the detection rate of renal cystic diseases. We studied to review the clinical features and treatment of renal cystic diseases in children. Methods : This study was performed in 95 children with...
Case Report
Small Intestinal Infarction Associated with Henoch-Schöenlein Purpura
Myung Ki Han, Yu In Park, Jeong Ho Kim, Jung Joo Lee, Hye Young Kang
Clin Exp Pediatr. 2002;45(1):125-130.   Published online January 15, 2002
Henoch-Schöenlein purpura, also known as anaphylactoid purpura, is characterized by palpable purpura, colicky abdominal pain, gastrointestinal hemorrhage, arthralgias, and renal involvement. Histopathologically, the condition represents a vasculitis, and in fact, it may be the most common vasculitis syndrome affecting children. The pathogenesis of Henoch-Schöenlein purpura remains poorly understood, but it is postulated that an unknown antigenic stimulus causes elevation of circulating IgA and that complement...
Original Article
Clinical Spectrum and Lung Pathology in Children with Interstitial Lung Disease
Ji-Hyun Chung, Seung-Ju Ha, Bong-Seong Kim, Soo-Jong Hong
Clin Exp Pediatr. 2002;45(1):79-87.   Published online January 15, 2002
Purpose : Interstitial lung disease(ILD) is a rare and poorly characterized disorder in children with poor prognosis. To understand the ILD in children, we reviewed our experience with 21 patients who were diagnosed interstitial lung disease during 9-year period at Asan Medical Center retrospectively. Methods : Severity-of-illness score was measured by the Denver protocol. We evaluated underlying diseases, clinical manifestations, high resolution computed tomography findings,...
Clinical Features of Acute Scrotum in Children
Ja Yun Yun, Mi Ran Kim, Kon Hee Lee, Kwang Nam Kim
Clin Exp Pediatr. 2001;44(12):1424-1431.   Published online December 15, 2001
Purpose : The acute scrotum is a clinical syndrome that is defined as an acute, painful swelling of the scrotum or its contents. The symptoms and signs of acute scrotum are similar and its pathognomonic features are rarely detected. This study was performed to find a proper way to diagnose and treat acute scrotum promptly and correctly. Methods : From January 1990 through December...
Case Report
A Case of Disseminated Lymphangiomatosis Involving Mediastinum, Bone, Spleen and Retroperitoneum in an Asymptomatic Healthy Child
Kyung Hee Yoon, Seung Ju Ha, In Sook Park, Hyun Woo Goo, Jong Hyun Yoon
Clin Exp Pediatr. 2001;44(11):1326-1329.   Published online November 15, 2001
Lymphangiomatosis, a benign tumor consisting of a cluster of dilated lymphatic channels, is very unusual. Most lymphangiomatoses are found in the neck and head area. Less than 5% are diagnosed intraabdominally and they are very infrequently encountered in the retroperitoneal area. Herein, we report a rare case of asymptomatic lymphangiomatosis of a 13 year-old boy during a routine chest...
A Case of Wernicke's Encephalopathy which Occurred During Chemotherapy in a Child with Acute Myeloblastic Leukemia
Sang-Nam Bae, Bu-Jin Kim, Seong-Shik Park, Sang-Ook Nam, Young-Tak Lim, Hak-Jin Kim
Clin Exp Pediatr. 2001;44(11):1320-1325.   Published online November 15, 2001
Wernicke,s encephalopathy(WE), a neurological disorder caused by thiamine deficiency, is characterized by the triad of ocular symptoms, ataxia, and mental confusion. More than 90% of the cases are observed in chronic alcoholics. Other conditions less frequently associated with WE are anorexia nervosa, prolonged parenteral nutrition, hemodialysis, uremia, hyperemesis gravidarum, gastroplasty for morbid obesity, and acquired immunodeficiency syndrome(AIDS). One of the...
A Case of Chronic Pneumonitis of Infancy in Pediatric Interstitial Lung Disease
Hyun Oh Jang, Se Chang Ham, Young Kyoun Kim, Se Wook Oh, Yong Won Park
Clin Exp Pediatr. 2001;44(9):1085-1090.   Published online September 15, 2001
The interstitial lung diseases(ILD) comprises a large, heterogeneous group of disorders characterized by derangement of alveolar walls and alveolar capillary unit causing restrictive pulmonary dysfunction and disordered gas exchange. It is rare, especially in children, and the opinions on investigation and treatments are controversial. The classification of adult ILD is applied to that of children. But considering occurrence during the developing state of lung...
Three Cases of Acute Scrotal Disorders in the Neonate
Ja Yun Yun, Min Kyoung Kwon, Mi Ran Kim, Kon Hee Lee, Kwang Nam Kim, Ho Hyun Jeong
Clin Exp Pediatr. 2001;44(7):841-846.   Published online July 15, 2001
Acute scrotal disorders(ASD) in neonates-especially the torsion of testes- are rare, and the clinical presentations are quite different from those of adolescents or adults. The neonates with ASD are generally afebrile and do not show typical symptoms. Early diagnosis and proper treatment of ASD are very important but the differentiation of torsion and orchitis is not easy in neonates. The...
Original Article
Colonic Transit Time in Chronic Constipated Patients
Je Eun Choi, Im Jeong Choi, Jung A Lee, Sung Mi Kim, Jin Hwa Jeong, Jeong Ho Lee
Clin Exp Pediatr. 2001;44(7):752-757.   Published online July 15, 2001
Purpose : In most instances, constipation is considered as idiopathic or functional. The total colonic transit time, traced by radio-opaque markers, makes possible the identification of the colon segment that has the motility alteration that causes constipation. We measured the total and segmental colonic transit time in children with chronic idiopathic constipation and compared the results with those without constipation...
Case Report
49, XXXXY Syndrome with Multicystic Kidney in a Neonate
Eun Young Park, Soo Jee Moon
Clin Exp Pediatr. 2001;44(6):714-717.   Published online June 15, 2001
The 49, XXXXY Klinefelter variant is distinctive; classic findings include mental retardation, hypogonadism and radioulnar synostosis. Its incidence is estimated to be 1/80,000 to 1/100,000 male births. We report a case of 49, XXXXY syndrome confirmed by chromosomal study in neonatal period, whose clinical features included a coarse face, short neck, micropenis, and other multiple minor anomalies including simian creases....
Original Article
A Clinical Study for Risk Factors of Cystic Periventricular Leukomalacia
Ji Eun Kim, Su Jin Kim, Woo Taek Kim
Clin Exp Pediatr. 2001;44(6):627-634.   Published online June 15, 2001
Purpose : It is very important to study the individual risk factors which cause periventricular leukomalacia(PVL) and those which affect the process of cystic formation from liquefied periventricular white matter, in order to prevent and permit early diagnosis of cystic PVL that contributes to mortality and neurologic sequelaes in preterm infants. Therefore, the present study was designed to assess the...
Case Report
A Case of Protein-losing Enteropathy in Congestive Heart Failure : Resolution with Prednisolone
Jong-Woon Choi
Clin Exp Pediatr. 2001;44(5):587-591.   Published online May 15, 2001
Protein-losing enteropathy can result from primary or secondary intestinal lymphangiectasia. Secondary intestinal lymphangiectasia develops as a result of lymphatic obstruction or elevated lymphatic pressure. Cardiac lesions such as constrictive pericarditis, post-Fontan procedure state, and chronic congestive heart failure increase lymphatic pressure and may cause intestinal lymphangiectasia and protein-losing enteropathy. An 11-year-old girl who underwent corrective surgery for Tetralogy of Fallot...
Original Article
Studies on Functional Differentiation of Small Intestinal Epithelial Cells
Ho Keun Yi, Su Mi Oh, Dae Yeol Lee, Jung Soo Kim, Pyoung Han Hwang
Clin Exp Pediatr. 2001;44(5):523-532.   Published online May 15, 2001
Purpose : We investigated compounds from food sources given to children that may induce the differentiation of small intestinal epithelial cells in order to signal pathways that induce the proliferation and differentiation of small intestinal epithelial cells. Methods : We analyzed small intestinal epithelial cell differentiation using in vitro IEC-6 cells model. The growth curve of IEC-6 cells was obtained...
Case Report
A Case of de novo Interstitial Deletion of 17 Chromosome
Kyung Hee Yoon, Hee Cheol Lee, Ai Rhan Kim, Ki Soo Kim, Soo Young Pi, Eul Ju Seo, Han Wook Yoo
Clin Exp Pediatr. 2001;44(4):475-479.   Published online April 15, 2001
This is the first reported case of a unique interstitial deletion involving the long arm of chromosome 17 in a Korean male infant born to parents with normal karyotype. The patient presented with multiple congenital malformations suggestive of chromosomal anomaly including round face, upslanted palpebral fissure, hypertelorism, posteriorly rotated low set ear, micrognathia, microcephaly, finger- like thumb, bilateral hearing loss,...
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