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Case Report
A Case of Superior Mesenteric Artery Aneurysm associated with Marfan Syndrome
Ick Ho Sung, Sang Hee Kim, Min Seop Song, Chul Ho Kim
Clin Exp Pediatr. 1998;41(7):984-988.   Published online July 15, 1998
Superior mesenteric artery aneurysm is very rare complication of Marfan syndrome, especially in children. A 11 years old male patient was admitted to the hospital because of fluctuating high fever and diagnosed as infective endocarditis and Marfan syndrome. During antibiotics therapy, fever was slowly decreased but abdominal pain was developed and pulsatile abdominal mass was palpable in the midline of...
Original Article
Molecular Genetic Diagnosis in Korean Patients with Myoclonic Epilepsy with Ragged Red Fiber(MERRF) Syndrome
Tae-Sung Ko, Sang-Ahm Lee, Gheeyoung Choe, Han-Wook Yoo
Clin Exp Pediatr. 1998;41(7):941-952.   Published online July 15, 1998
Purpose : Myoclonic epilepsy with ragged red fiber(MERRF) syndrome is a disease of the mitochondrial encephalomyopathies, characterized by progressive myoclonus(action), epilepsy, cerebellar ataxia, intention tremor, muscle weakness, progressive dementia, sensorineural hearing loss and optic atrophy. Its inheritance is maternally inherited mitochondrial mutation, and its pathologic finding is characterized by ragged red fibers(RRF). Biochemically its defects are diverse. This study was...
Developmental Morphology of the Atrial Venous Component in the iv/iv Mouse
Young-Hwue Kim, Yong-Soo Yun, Jeong-Wook Seo
Clin Exp Pediatr. 1998;41(7):931-940.   Published online July 15, 1998
Purpose : Developmental processes of atria in embryos or fetuses of the iv/iv mouse were studied to find the differences between right and left atrial chambers and to identify the characteristics of atrial isomerism in the abnormal laterality syndrome. Methods : Seventy-three embryos from SI/Col iv/iv mice(Jackson's laboratory, USA) or fetuses at the 10th-13th day were used. They were examined using...
Case Report
Meckel-Gruber syndrome
Jung Seo Park, Ji Yoen Lee, Sun Chan Bae, Moon Sung Park, Jung Seon Kim, Si Houn Hahn, Chang Ho Hong
Clin Exp Pediatr. 1998;41(6):856-860.   Published online June 15, 1998
Meckel-Gruber syndrome is a multiple malformation syndrome featuring occipital meningoencephalocele, multicystic dysplasia of kidney, cystic and fibrotic change of liver, polydactyly, and other characteristics inherited by the autosomal recessive trait. We exprienced a case of Meckel-Gruber syndrome in a newborn male diagnosed clinically and confirmed pathologically. Abnormalities of the fetus were found prenataly by ultrasonogram, and subsequently the baby was...
Original Article
A Clinical Study on Lennox-Gastaut Syndrome
Yong Hun Choi, Gyo Dang Koo, Yeong Ho Rah, Sa Jun Chung
Clin Exp Pediatr. 1998;41(5):663-668.   Published online May 15, 1998
Purpose : Lennox-Gastaut syndrome is an intractable epilepsy which is comprised of several types of etiology and generalized seizures. It also combines with mental retardation. The present study was made to ascertain the clinical study on Lennox-Gastaut syndrome in Korea. Methods : From January, 1985 to December, 1995, 31 children, diagnosed with Lennox-Gastaut syndrome at Kyung Hee University Hospital, were analyzed...
Case Report
A Case of Berry Syndrome associated with Syndactyly
Sang Chun Jung, Kyoung Sim Kim, Yong Wook Kim, Ki Bok Kim
Clin Exp Pediatr. 1998;41(4):543-547.   Published online April 15, 1998
Berry syndrome is a rare association of congenital heart anomalies which consists of a distal aortopulmonary window with aortic origin of the right pulmonary artery and hypoplasia or interruption of the aortic arch. This defect can be corrected only by immediate surgical intervention, so accurate preoperative diagnosis and detailed anatomic depiction of this syndrome are important in prognosis. We experienced a case of Berry...
Original Article
Preparation and in vitro Physical Activities of Crude Natural Surfactant and Artificial Pulmonary Surfactant Containing Synthetic Peptide and Phospholipid Mixtures
Joo Hyun Kang, Song Yub Shin, Cheol-Young Maeng, Kil Lyong Kim, Chong-Woo Bae, Kyung-Soo Hahm
Clin Exp Pediatr. 1998;41(4):472-480.   Published online April 15, 1998
Purpose : In this study, natural pulmonary surfactant was extracted from bovine lung lavage and its surface activity was determined. To investigate the usefulness of synthetic peptides reconstituted with phospholipid as artificial surfactant, truncated peptides from surfactant protein (SP)-B were synthesized and restored the surface tension lowering activities when appropriately recombined with phospholipid. Methods : Crude natural surfactant(CNS) was isolated from lung lavage by centrifugation...
Case Report
A Case of Congenital Nephrotic Syndrome due to Diffuse Mesangial Sclerosis
Jung-Jin Yu, Dong Kyu Jin, Hae Il Cheong, Hyun Soon Lee, Yong Choi
Clin Exp Pediatr. 1998;41(3):415-419.   Published online March 15, 1998
Diffuse mesangial sclerosis(DMS) is one of the underlying pathology of congenital and infantile nephrotic syndrome. Infants with DMS develop nephrotic syndrome before 2 years of age and progress to end stage renal disease within 3 years of age. The authors experienced a case of isolated DMS in a 4-month-old male infant who had nephrotic syndrome for 1 month. The diagnosis...
A Case of Alagille Syndrome
Eun Kyung Hwang, Gwang Hoon Lee, Eell Ryoo, Kang Ho Cho, Gil Hyun Kim, Hak Soo Lee, Ji Hye Kim, Sung Hae Park, Hee Sup Kim
Clin Exp Pediatr. 1998;41(3):410-414.   Published online March 15, 1998
Alagille syndrome is characterized by chronic cholestasis, posterior embryotoxon, skeletal abnormalities, cardiovascular abnormalities, and a typical face with prominent forehead and pointed chin. Its histological feature includes paucity of interlobular bile ducts. We experienced a 49-day-old female infant presenting with frequent upper respiratory tract infection and persistent jaundice. She had a typical face and chronic cholestasis. Echocardiograms revealed peripheral pulmonary...
A Case of DiGeorge Syndrome
Young Joo Son, Yu Sik Jeon, Soon Lee Jung, Kyuchul Choeh
Clin Exp Pediatr. 1998;41(3):390-395.   Published online March 15, 1998
We experienced a case of DiGeorge syndrome in a 25-day-old male infant presented with micrognathia, short neck, fish-shaped mouth and intractable seizures with a loading dose of phenobarbital & dilantin. The serum calcium level was 3.7mg/dl, ionized calcium level was 0.62mmol/L, and parathyroid hormone carboxy-terminal level was 0.01ng/ml. We treated with it calcium gluconate infusion, low phosphorous formula milk feeding, and...
Original Article
Treatment of Supraventricular Tachycardia by Catheter Ablation Using Radiofrequency Currents in Children and Adolescents
Sung Jae Lee, Mi Jin Jung, Dieter Gonska
Clin Exp Pediatr. 1998;41(2):186-191.   Published online February 15, 1998
Purpose : Catheter ablation using radiofrequency currents have been proven to be an effective and safe tool for the treatment of adult patients with accessory atrioventricular pathways. This study was designed to analyze the efficacy of this method in childern and adolescents. Methods : Using radiofrequency currents, cathether ablation of an accessory pathway was performed in 54 patients(mean age 11.8 years)...
Effects of Early Dexamethasone Therapy in Neonatal Respiratory Distress Syndrome
Hyeon-Soo Lee
Clin Exp Pediatr. 1998;41(1):19-25.   Published online January 15, 1998
Purpose : To examine the clinical effects of early dexamethasone treatment from postnatal age of 1 week to ventilated preterm infants with respiratory distress syndrome. Methods : 14 one-week-old ventilator-dependent infants were enrolled. Seven received dexamethasone and another 7 did not receive. The doses of dexamethasone used were 0.25mg/kg twice daily for 3 days, 0.125mg/kg twice for 1 day, then 0.125mg/kg...
A Study of Serum Cytokines in Nephrotic Syndrome
Mea Young Chang, Jae Ho Lee
Clin Exp Pediatr. 1997;40(12):1701-1706.   Published online December 15, 1997
Purpose : It has been reported that patients with nephrotic syndrome have high serum IgE value and IL-4 involve in IgE synthesis, IL-6 is an autocrine growth factor for the proliferation of mesangial cells. We studied association between serum cytokines and of nephrotic syndrome. Methods : We measured serum IL-6, IL-4, IFN-Υ, CD23 in 14 children with nephrotic syndrome and 3 healthy children by...
Influences of the Intrapulmonary R→L Shunt on PaO2 and PaCO2 after Instillation of Exogenous Pulmonary Surfactant to Premature Neonates with Respiratory Distress Syndrome
Yoon Hyung Park, Sei Woo Chung, Seung Baik Han, Young Se Kwon, Ji Youn Kim, Woo Sik Cheong, Dae Hyun Lim, Byong Kwan Son
Clin Exp Pediatr. 1997;40(11):1508-1519.   Published online November 15, 1997
Purpose : We are inclined to analyze the relationship between the intrapulmonary right-to-left shunt and the PaO2/PaCO2 after endotracheal single-dose surfactant instillation to premature neonates with respiratory distress syndrome within 6 hours after birth. Methods : From Jan. 1993 to Jun. 1995, we have conducted a clinical trial of surfactant replacement therapy to the premature neonates with respiratory distress syndrome at the neonatal intensive care unit...
Case Report
A Case of Pena-Shokeir Phenotype in Trisomy 18 Syndrome
Ki Hun Song, Jee Yeon Song, In Kyung Sung, Kyong Su Lee
Clin Exp Pediatr. 1997;40(9):1303-1308.   Published online September 15, 1997
Pena-Shokeir syndrome is a rare, often lethal disease, characterized by intrauterine growth retardation, craniofacial anomalies, limb ankylosis, polyhydramnios and pulmonary hypoplasia. This autosomal recessive disease should be differentiated from trisomy 18, which the second most common multiple congenital malformation syndrome. It is therefore clear that the two syndromes have certain features in common, the most consistent being craniofacial and limb abnormalities and intrathoracic pathology. Therefore,...
Original Article
Prediction of Steroid Responsiveness in the Primary Nephrotic Syndrome Using Urinary β2-Microglobulin Level and N-Acetyl-β-D-Glucosaminidase Activity
Kwang In Lee, In Seok Lim, Eung Sang Choi
Clin Exp Pediatr. 1997;40(9):1285-1292.   Published online September 15, 1997
Purpose : Considering that renal biopsy is not routinely indicated in nephrotic syndrome in children and the risk of the procedure, we studied that it is possible to predict steroid responsiveness in nephrotic syndrome and the difference in responsivenss is related with the histopathologic type using urinary β2-microglobulin and N-Acetyl-β-D-Glucosaminidase activity as a safe and noninvasive method. Methods : We measured serum creatinine, albumin, cholesterol...
Case Report
A Case of Unusual (1q;21q) Translocation Down Syndrome Inherited from a t(1q;21q) Balanced Carrier Mother
Dong Hoon Yi, Jae Ock Park, Sang Mann Shin, You Kyoung Lee, Won Bae Kim, Won Bae Lee, Sung Sup Park, Han-Ik Cho
Clin Exp Pediatr. 1997;40(8):1156-1161.   Published online August 15, 1997
Down syndrome is the most common autosomal syndrome in man. The incidence of trisomy 21 due to translocation is about 3.5-5%. Translocations are usually centric fusions between a 21 and a D group (54.2%) or a G group (40.9%) chromosome. Since the short arm of 21 carries no phenotypically active genes, even if the short arm of 21 is lost, usually there is...
Morquio's Syndrome Accompanied by Respiratory Failure
Jun Hur, Hyeon Jeong Lee, Jeong Hwa Whang, Hee Jin Kim, Chan Yung Kim
Clin Exp Pediatr. 1997;40(7):1015-1020.   Published online July 15, 1997
Authors experienced a case of Morquio’s syndrome accompanied by respiratory failure in a aged fifteen year old boy. Patient showed normal intelligence, but stunted growth with short trunk and unproportionately large head, pectus carinatum, kyposcoliosis, muscular weakness, paralysis and genu valgum. No other member of family was affected with this syndrome. There were radiological findings of subluxation between first and second cervical vertevrae, central beaking with...
Original Article
Seizure Types and EEG Findings of Juvenile Onset Idiopathic Generalized Epilepsy
Hyunmi Kim, Won Seop Kim, Jong Shin Kim, Kangho Cho, Ki Joong Kim, Yong Seung Hwang
Clin Exp Pediatr. 1997;40(7):991-998.   Published online July 15, 1997
Purpose : Juvenile myoclonic epilepsy, juvenile absence epilepsy, and epilepsy wth generalized tonic clonic seizure(GTCS) on awakening are the three syndromes of idiopathic generalized epilepsy of adolescent onset currently included in the classification of epilepsy syndromes of the International League Against Epilepsy(ILAE). Although they differ in their predominant seizure types, the syndromes share several seizure types. Also, there are no unique electrophysiologic or genetic markers....
A Study of Relation between Stable Microbubble Rating and Surfactant Derivatives(SurfactenⓇ : Phospholipid :, ExosurfⓇ : Dipalmitoyl-Phosphatidyl Choline) Concentration in Vitro
Myung Ho Oh, Kee Hyuck Kim, Jae Gun Sim, Min Hee Kim
Clin Exp Pediatr. 1997;40(7):939-944.   Published online July 15, 1997
Purpose : With the recent advent of surfactant replacement therapy, there is an increasing need for a rapid and reliable test to predict respiratory distress syndrome (RDS) immediately before or at birth. There are many investigations and methods for the detection of RDS in prenatal or postnatal period. The stable microbubble rating test (SMR-test) developed by pattle et al. is rapid and simple test...
Extrachromosome 21 in Korean with Down Syndrome Using DNA Haplotyping
Jin-Sung Lee, Won Kyu Choi
Clin Exp Pediatr. 1997;40(7):917-924.   Published online July 15, 1997
Purpose : Down syndrome, the most common single cause of mental retardation, is usually due to meiotic nondisjunction leading to trisomy 21. In order to understand the mechanisms of meiotic nondisjunction including parental origin of an extrachromosome and the meiotic stage of nondisjunction, we have studied DNA polymorphisms at loci on the long arm of chromosome 21 in 36 families with free trisomy 21. Methods...
Case Report
A Case of Congenital Pulmonary Lymphangiectasia in Noonan Syndrome
Dong Hee Lee, Won Bae Kim, Jung Hye Choi, Su Nam Lee
Clin Exp Pediatr. 1997;40(6):877-882.   Published online June 15, 1997
Congenital pulmonary lymphangiectasia is a rare and perphaps underestimated cause of fatal respiratory distress in the neonate or infant. Pathologically, this condition is characterized by wide lymphatic channels in the subpleural and peribronchovascular spaces and the interlobular septa. Noonan syndrome is characterized by a phenotype similar to Turner syndrome but with a normal karyotype. Both pulmonary and intestinal lymphangiectasia have been reported in patients...
Erratum
A Case of Joubert Syndrome
Hye-Young Kim, Sun-Jun Kim, Jung-Soo Kim
Accepted January 1, 1970  
Joubert syndrome is frequently associated with developmental delay and mental retardation, neonatal tachypnea and apnea, abnormal eye movements, and ataxia. It is inherited by an autosomal recessive trait. The most significant and constant neuropathological finding is partial or total agenesis of cerebellar vermis. Absence of the vermis results in a triangular-shaped mid-fourth ventricle and a "bat-wing" shaped fourth ventricle superiorly. The superior cerebellar peduncles...
Case Report
Pena-Shokeir I Syndrome in a Newbonrn Infant
Myung Gil Han, Kyu Young Kim, Dong Woo Son, Bo Young Yoon, Kyung Hee Park, Do Hyun Kim, Hyung Ro Moon
Clin Exp Pediatr. 1997;40(5):721-725.   Published online May 15, 1997
Pena-Shokeir I syndrome is a multiple malformation syndrome displaying characteristics of camptodactyly, multiple ankylosis, severe muscle weakness, facial anomalies (low set ears, hypertelorism, depressed tip of nose), polyhydramnios, fetal growth retardation & pulmonary hypoplasia which are inherited by autosomal recessive trait. We experienced 1 case of Pena-Shokeir I syndrome in a neonate (41 weeks, 2.08Kg). This patient suffered from dyspnea. Respiratory...
Original Article
Clinical Features of Adrenocortical Neoplasms
Jung-Eun Lee, So-Chung Chung, Duk-Hi Kim, Ho-Sung Kim
Clin Exp Pediatr. 1997;40(5):680-689.   Published online May 15, 1997
Purpose : Adrenocortical tumors are uncommon in children and comprise only a small proportion of primary adrenal neoplasms. The biologic behavior of these tumors may be very difficult to predict, and their rarity has hindered identification of clinical characteristics. Patients with functioning tumors have excessive steroid hormone production, and the clinical manifestation depends on the predominant hormone produced. The detection of...
A Study of Vascular Reactivity Change in Nephrotic Syndrome Children Using Plethysmography
Jae Won Shim, Byoung Hoon Yoo, In Suk Lim
Clin Exp Pediatr. 1997;40(5):650-659.   Published online May 15, 1997
Purpose : The nephrotic syndrome is characterized by proteinuria, hypoproteinemia, edema and hyperlipidemia. These can change body homeostasis and cause hypertension. This study was designed to determine the relationship between the forearm vasodilating capacity and serum cholesterol level of nephrotic syndrome patients. Methods : 15 Nephrotic syndrome patients and 17 normal control children who visited Chung-ang University Youngsan Hospital from Sep....
Case Report
A Case of Congenital Acute Megakaryoblastic Leukemia with Down Syndrome
Nan Yee Kye, Kon Hee Lee, Jae Kook Cha, Hye Sun Yoon, Won Keun Song
Clin Exp Pediatr. 1997;40(4):578-583.   Published online April 15, 1997
We experienced a case of congenital acute megakaryoblastic leukemia with Down syndrome. The patient was admitted due to characteristic facial figure of Down syndrome and abdominal distension. Acute megakaryoblastic leukemia was diagnosed with abundant megakaryoblast in peripheral blood smear, severe myelofibrosis in bone marrow biopsy and positive platelet glycoprotein Ⅲa receptor. On third hospital day, the patient expired due to DIC...
A Case of Severe Pulmonary Complication and ARDS Secondary to Peanut Aspiration in 15 Month Old Male
Sung Keun Moon, Chang Keun Kim, Churl Young Chung
Clin Exp Pediatr. 1997;40(3):423-428.   Published online March 15, 1997
Foreign body aspiration(especially peanut) is the leading cause of accidental death in children under 1 year of age and most cases of serious aspiration occur between the age of 1 and 3. We report a case of severe pulmonary complication and adult respiratory distress syndrome(ARDS) secondary to peanut aspiration in 15 month old male in spite of all removal(9 pieces) by ventilating bronchoscope. Chest...
A Case of Dup(3q) Syndrome
Yo Seop Pahn, Mi Yeon Choi, Young Ah Lee, Woo Kap Chung, Keun Young Lee, Sook Kyoung Oh
Clin Exp Pediatr. 1997;40(3):408-412.   Published online March 15, 1997
We have experienced a case of dup(3q) syndrome in the neonate who had a multiple congenital anomalies of hypertrichosis, hypertelorism, upslanting palpaberal fissures, anteverted nostrils, long philtrum, micrognathia, downturned corners of the mouth, highly arched palate, short, webbed neck, clinodactyly, rocker-bottom feet, dermal sinus. Cytogenetic studies showed a duplication 3q21→qter regions. Chromosome study of relatives is extremely important for counseling because...
Original Article
Clinical Study of Symptoms and Various Anomalies of Patients with Joubert Syndrome
Hang Bo Jeong, Se Hee Hwang, Ki Joong Kim, Yong Seung Hwang, Seung Cheol Kim, In-One Kim
Clin Exp Pediatr. 1997;40(3):385-392.   Published online March 15, 1997
Purpose : Joubert syndrome is known tobean autosomal recessive disorder characterized by cerebellar vermian dysgenesis with many symptoms and variety of other malformations. We studied the relevant symptoms and various malformations of seven patients diagnosed as Joubert syndrome at our institution. Methods : Seven children with cerebellarvermian dysgenesis consistent with Joubert syndrome were included in ourstudy. Each child was diagnosed at the Seoul National University Children's...
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