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Original Article
A Study of Relation between Stable Microbubble Rating and Pulmonary Surfactant Concentration in vitro
Seong Yong Jung, Thi Hyung Park, Chong Woo Bae, Chang Il Ahn
Clin Exp Pediatr. 1994;37(8):1048-1054.   Published online August 15, 1994
Respiratory distress syndrome (RDS) of preterm infants remains a significant cause of morbidity and mortality despite improvements in neonatal intensive care and artificial ventilatory techniques. After identification of the deficiency of pulmonary surfactant is major pathophysiologic basis in RDS, artificial surfactant replacement therapy in RDS was first successfully tested by Fujiwara and co-workers in 1980. Therefore, exogenous surfactant replacement produced exellent...
Case Report
A Case of Idiopathic Hypereosinophilic Syndrome
Bin Cho, Jin Tack Kim, Joon Sung Lee, Kyoo Hong Cho
Clin Exp Pediatr. 1994;37(7):1020-1027.   Published online July 15, 1994
The idiopathic hypereosinophilic syndrome(HES) represents a heterogenous group of disorder characterized by prolonged eosinophilia of undetectable cause and multiorgan system dysfunction. Bone marrow is the most frequentry involved organ, but the most severe clinicopathologic involvement is heart. The major cause of death in patients with the HES is cardiac dysfunction especially congestive heart failure resulted from endocardial fibrosis and restrictive cardiomyopathy. We...
A Case of Steven-Johnson Syndrome Associated with Cholestatic Hepatitis
Tae-Hee Park, Ran-Ju Kim, Byoung-Geun Lee, Soo-Chul Cho, Dae-Yeol Lee
Clin Exp Pediatr. 1994;37(7):1016-1019.   Published online July 15, 1994
A12-year-old boy developed cholestatic hepatitis with Steven-Johnson syndrome following the use of amoxicillin. The skin lesion and general condition were improved over 2 weeks, but jaundice was gradually aggrevated. We performed liver biopsy, on 30th hospital day, which showed cholestatic hepatitis. The patient improved gradually and liver function was normalized 5 months later.
A Case of Goltz Syndrome
Won Rae Kim, Hyang Joo Kim, Ghee Young Jung, Jin Gun Bang, Du Bong Lee, Jung Hee Park
Clin Exp Pediatr. 1994;37(7):994-998.   Published online July 15, 1994
Goltz syndrome is known as a rare mesoectodermal hereditary disease, characterized by focal dermal atrophies with hernias of adipose tissue and also associated with a multitude of possible skeletal, dental, ophthalmological and other abnormalities. We experienced a case of Goltz syndrome. An one day old female newborn had focal atrophic and telangiectatic skin lesions, microphthalmia, syndactyly and urinary tract abnormality. The finding...
A Case of Incomplete Drash Syndrom
Im Jae Park, Hyunee Yim, Jae Seung Lee, Hyeon Joo Jeong, Woo Hee Jung
Clin Exp Pediatr. 1994;37(6):872-879.   Published online June 15, 1994
Drash syndrome, which was first reported by Denys et al. In 1967 is a complex disorder which associates a nephropathy, Wilms?tumor, and male pseudohermaphroditism. The common denominator is a nephropathey. The nephropathy may be associated with either genital abnormalities or Wilms?tumor, and these associations are called incomplete form of Drash syndrome. This syndrome appears early in life and the first...
Two Cases of Toxic Shock Syndrome (TSS) in Infants
Jin Kil Park, Hong Ju Choi, Hee Tag Im, Jae Sam Kim, Hyo Kyung Shin, Chul Ho Koo
Clin Exp Pediatr. 1994;37(6):861-871.   Published online June 15, 1994
Much has been learned of the pathogenesis and pathophysiology of the toxic shock syndrome (TSS) sinde the initial description in 1978 by Dr. James K, Todd. The clinical illness in defined by the criteria listed in the case definition formulated for epidemiologic studies. With the advent of widespread recognition of TSS, there have been numerous published reports describing the clinical...
A Case of Rett Syndrome Observed with Video-EEG Monitoring
Hyun Mi Kim, Young Ah Lee, TaecSung Ko, Hyung Nam Moon, Chang Yee Hong
Clin Exp Pediatr. 1994;37(5):718-725.   Published online May 15, 1994
Rett syndrome is progressive neurodegenerative disorder in female patients, characterized by autistic behavior, mental retardation, loss of purposeful hand skills, sterotypic hand movement, breathing dysfunction, severely impaired language, ataxia, and seizure. The diagnosis of Rett syndrome is based on its characteristic clinical manifestation and course. The electroencephalographic(EEG) findings of Rett syndrome are nonspecific, but a progressive deteriorationin the EEF, characterized...
Original Article
Occult Mediastinal Ganglioneuroblastoma Presenting with Myoclonic Encephalopathy as Paraneoplastic Syndrome
Hahng Lee, Dong Ki Han, Jae Won Oh, In Joon Seol, Eun Kyung Hong, Seok Chol Jeon
Clin Exp Pediatr. 1994;37(5):695-700.   Published online May 15, 1994
Ganglioneruroblastoma and neuroblastoma are among commonest types of childhood malignancy and a number of unique paraneoplastic syndromes have been associated with both localized and disseminated neuroblastoma. The coincidence of neuroblastoma and myoclonic encephalopathy or other paraneoplastic syndromes occurs relatively rare, and therefore, failure to recognize this association could result in delays in both diagnosis and treatment, and the result...
Diagnostic value and Relationship of the between Stable Microbubble rating Test and Shake Test for the Prediction of Neonatal Respiratory distress Syndrome
Seong Jin Ha, Dong Kyun Ryu, Oh Kyung Lee, Wan Seob Kim
Clin Exp Pediatr. 1994;37(5):620-627.   Published online May 15, 1994
Respiratory distress syndrome (RDS) in the newborn infants remains a major cause of mortality and morbidity in the newborn period despite much improvements in neonatal intensive care and artificial ventilatory techniques. Gastric fluid was obtained from 151 patients within 6 hours after delivery. The sensitivity, specificity, and predictive value of the simple shake test (133 cases) and stable microbubble rating...
Case Report
A Case of Prader-Willi Syndrome with Diabetes Mellitus
Hee Ran Choi, Eun Ha Choi, Seong Hee Jang, Young Min Ahn
Clin Exp Pediatr. 1994;37(4):565-572.   Published online April 15, 1994
Prader-Willi syndrome is characterized by infantile hypotonia, mental retardation, hyperhagia, hypogonadism and obestiy. Approximately 60% of all PLW syndrome show an interstitial deletion of chromosome 15, 37% have apparently normal chromosome, and 3.6% have a variety of other abnormalities involving chromosome 15. Diabetes mellitus has been considered a component of PLW syndrome and the incidence is about 7%. We...
A Case of Rotor Syndrome
Jin Nyoung Park, In Joon Seol
Clin Exp Pediatr. 1994;37(3):410-415.   Published online March 15, 1994
We experienced a case of Rotor syndrome in an 8 year 6 month old boy who presented with icteric sclera and icteric skin on whole body. His clinical and laboratory characteristics were as follows; 1) Jaundice appeared from several months ago before admission without any clinical disturbance. 2) Direct bilirubin was more increased than indirect bilirubin. 3) Plasma indocyanine green (ICG) kinetics test...
A Case of Bart's Syndrome - Epidermolysis Bullosa and Congenital Localized Absence of Skin -
Hong Shin Jeon, Young Jin Hong, Don Hee Ahn, Hee Jun Yoo, Je Geun Chi
Clin Exp Pediatr. 1994;37(1):104-108.   Published online January 15, 1994
A female newborn had the following characteristics: a congenital localized absence of skin over the lower extremities; blistering of the skin or mucous membrane, incited by trauma, which heals without scarring; and congenital absence or deformity of the nails. In respect to the characteristic manifestationhj, clinical course and electron microscopic features, our patient seems to fit well into Bart's syndrome....
Original Article
Cyclosporin A Treatment of Minimal Change Nephrotic Syndrome and Focal Segmental Glomerulosclerosis
Ik Jun Lee
Clin Exp Pediatr. 1993;36(12):1740-1746.   Published online December 15, 1993
In this study I assesed the efficacy and tolerance of cyclosporin A (CyA) in the treatment of steroid-dependent MCNS (minimal change nephrotic cyndrome) and steroid resistant FSGS (focal segmental glomerulosclerosis). The results showed, that 1) CyA was effective in sustaining a remission in steroid-dependent MCNS without prednisone treatment, and therefore, could be an alternative therapeutic choice. 2) After discontinuation of CyA, relapses reccurred...
Clinical Observation of Meconium Aspiration Syndrome : In Relation to Fetal Heart Rate Monitoring
Young Ran Yoon, Jin Sook Kim, Mi Kyung Kim, Sun Young Kho, Seong Ja Lee, Mi Jung Kim, Hye Kyung Han, Kyung Hoon Choi, Mi Na Lee
Clin Exp Pediatr. 1993;36(12):1656-1662.   Published online December 15, 1993
To identify the neonates at the risk of having meconium aspiration syndrome, the perinatal characteristics and finding of fetal heart rate monitorings in 366 neonates who had meconium-stained amniotic fluid were retrospectively reviewed. The results were as follows ; 1) Overall incidence of MAS was 0.7%. 2) Mortality rate of MAS was 5.8%. 3) The mean Apgar scores at 1-and 5-minute were...
Case Report
A case of Aberrant Right Subclavian Artery Associated with Innominate Artery Compression Syndrome
Kyung Ho Ko, Young-Ill Park, Churl Young Chung
Clin Exp Pediatr. 1993;36(11):1626-1629.   Published online November 15, 1993
An aberrant right subclavian artery, the most common congenital anomaly of the aortc arch, is rarely symptomatic during the infancy, if an anomalous origin of a right common carotid artery is also associateda varient of innomiate artery comperssion syndrome. We experienced a case of an aberrant right subclavian artery associated with an anomalous origin of the right common carotid artery in...
Two Cases of Virus Associated Hemophagocytic Syndrome
Jae Hee Han, Hyun Chul Lee, Hwang Min Kim, Jong Soo Kim, Kyung Won Lee
Clin Exp Pediatr. 1993;36(10):1458-1465.   Published online October 15, 1993
Virus associated hemophagocytic syndrome, class II histiocytoses, characterized by high fever, severe constitutional symptoms, abnormal liver function and coagulation, peripheral blood pancytopenia and histiocytic hyperplasis with prominent hemophagocytosis in bone marrow and lymph nodes has been reported and associated with active viral infection. It is non-malignant and reversible. It must be differentiated from histiocytic medullary reticuosis because of the inappropriateness...
A Case of Roberts Syndrome
Young Choi, Yo Han Chung, In Seok Lim, Chul Ha Kim, Dong Keun Lee, Seong Nam Kim, Sang Yong Song, Je Geun Chi
Clin Exp Pediatr. 1993;36(10):1447-1451.   Published online October 15, 1993
Roberts syndrome is an autosomal recessive disorder accompanied by limb defects, craniofacial abnormalities, pre-and postnatal growth retardation. Patients with Roberts syndrome have characteristic premature separation of heterochromatin of many chromosomes and abnormalties in celldivision cycle. We have experienced a case of Roberts syndrome in an immature neonate. The patients showed characteristic clinical features of multiple encephaloceles. Severe facial mid-line clefts, and...
One Case of Bronchiolitis Obliterans Associated with Stevens-Johnson Syndrome due to Mycoplasma pneumoniae In fection
Soo Kyung Lee, Joon Soo Lee, Soo Young Lee, Byung Ju Jeoung, Kyu Earn Kim, Ki Young Lee
Clin Exp Pediatr. 1993;36(10):1441-1446.   Published online October 15, 1993
Bronchiolitis obliterans is an infrequent disorder characterized by partial or complete obstruction of bronchi and bronchioles by fibrous tissue following an insult to the lower respiratory tract. In the pediatric population, Bronchiolitis obliterans has most frequently been preceded by respiratory tract infection by adenovirus, influenza, measles. But it may occur in young adults after mycoplasma infection. We experienced the case...
Original Article
Nephrotic Syndrome under 2 Years of Age
Jae Sung Ko, Kang Mo Ahn, Hye Won Park, Il Soo Ha, Hae Il Cheong, Yong Choi, Kwang Wook Ko
Clin Exp Pediatr. 1993;36(10):1395-1401.   Published online October 15, 1993
There had been total 20 patients with early onset (4 months~2 years) primary nephrotic syndrome in the Deparment of Pediatrics, Seoul National University Children's Hospital, during the period from March 1987 to February 1993. We analysed clinical courses, response to treatment, pathological findings and prognosis of the patients And the results were as follows; 1) The initial responders to steroid treatment...
Case Report
A Case of Aicardi Syndrome with Cleft Lip and Palate
In Seok Yang, Gyung Og Yu, Soo Ahn Chae, Dug Ha Kim, Chong Young Park, Ik Won Kang
Clin Exp Pediatr. 1993;36(9):1325-1330.   Published online September 15, 1993
A case of Aicardi syndrome with cleft lip and palate was experienced at the Department of Pediatrics, College of Medicine, Hallym University, and the patient's infantile spasm was treated with ACTH. In previous studies, four cases of Aicardi syndrome accompanied by cleft lip and palate were reported. We present the fifth case of Aicardi syndrome with cleft lip and palate...
A Case of Bartter's Syndrome
Jun Ho Yoon, Jeong Eun Kwon, Jae Ouk Lee, Sang Gil Lee
Clin Exp Pediatr. 1993;36(9):1308-1314.   Published online September 15, 1993
Bartter's syndrome is a rare tubular disorder characterized by hypokalemic, hypochloremic metabolic alkalosis, hyperreninemic hyperaldosteronism, hyporesponsiveness to pressor agents, and juxtaglomerular apparatus hyperments In addition to a case report, brief review of related literatures was done.
Original Article
The Relationship between Spontaneous Diuresis and Changes of Pulmonary Function in Infants with Respiratory Distress Syndrome
Byeong Hyun Kim, Jae Kag Choi, Yeon Kyun Oh, Kwang Soo Oh, Hyang Suk Yoon, Chong Cuk Kim
Clin Exp Pediatr. 1993;36(8):1094-1100.   Published online August 15, 1993
To evaluate the relationship between spontaneous diuresis and improvement of pulmonary function in respiratory distress syndrome, 15 premature neonates requiring mechanical ventilation for RDS, who admitted in NICU of Wonkwang University Hospital from January 1990 to June 1992, were studied. The results were as follows(results are mean¡¾SEM) 1) Mean birth weight of study infants was 1.82¡¾0.08Kg, and mean gestational age was 32.80¡¾0.54...
Case Report
A Case of Syndrome Associated with Colonic Atresia
Si Whan Koh, Joon Soo Park, Kyung Hwan Oh, Dong Hwan Lee, Sang Jhoo Lee, Chul Moon
Clin Exp Pediatr. 1993;36(7):1030-1033.   Published online July 15, 1993
The association of colonic atresia in patients with Down syndrome is a rare anomaly. The incidence of congenital atresia of the gastrointestinal tract has been estimated to be about one in 1500 births. Colonic atresia is rarer still, and is throut to comprise about 5% to 10% of this group. This intestinal atresia occurs in about 30% to 50% of...
Original Article
Comparison and diagnostic Accuracy of Stable Microbubble Rating Test and Shake Test for the Early Detection of Respiratory Distress Syndrome
Hyeon Gon Kim, Sang Hyun Byun, Young Hun Chung
Clin Exp Pediatr. 1993;36(7):913-918.   Published online July 15, 1993
Respiratory distress syndrome of preterm infants remains a significant cause of morbidity and mortality. Early, just after birth, prediction and recognition of RDS is so important. The precision and reliability of the stable microbuble test (SMR) and shake test as a predictor of respiratory distress syndrome were studied. A 11-=neonate who was born at Chungnam National University Hospital between November 1991...
Studies on the Prognostic Marker in Minimal Change Nephrotic Syndrome
Byoung-Soo Cho
Clin Exp Pediatr. 1993;36(7):906-912.   Published online July 15, 1993
Childhood minimal change nephrotic syndrome (MCNS) is often associated with hyper-sensitivity reactions and considered to be caused by immune dysfunction. The elevated serum IgE levels and atopic symptoms have been frequently associated in these patients. The present therapy for MCNS mainly depends on corticosteroids, alkylating agents such as chlorambucil, cyclophosphamide and cyclosporin A (Cy-A). Howerver, frequent relapses and severe side effects...
Case Report
A Case of Rett Syndrome
Kim Hye Soon, Lee Keun
Clin Exp Pediatr. 1993;36(5):743-747.   Published online May 15, 1993
Rett syndrome is a newly characterized developmental disorder that affect girls exclusively. These gils are born clinically normal, but their psychomotor development stagnates and deteriorates between the age of 6 months and 2 years. The full syndrome compriseslaquired microcephaly, severe dementia, autism, loss of purposeul use of the hands, characteristic hand-wringing sterotypes, jerky ataxia of the trunk epilepsy with various types...
A Case of Fanconi Syndrome
Young Woon Lee, In Seok Lim, Chul Ha Kim
Clin Exp Pediatr. 1993;36(5):737-442.   Published online May 15, 1993
Fanconi syndrome is a complex of renal tubular dysfunction defined by glycosuria without diabetes, generalized aminoaciduria, phosphaturia, bicarbonaturia, uric aciduria, and renal tubular acidosis. It is often associated with hypokalemia, hypophosphatemia, rickets and osteomalacia. We have experienced one case of Fanconi syndrome with chronic tubulointerstitial nephritis. The patient was a 4 year and boy and his chief complaints were polyruia, polydipsia,...
Original Article
Clincal Observation on Guillain-Barré Syndrome in Children
In Cheol Park, In Seung Park, Jeh Hoon Shin, In Joon Seol, Ha Baik Lee
Clin Exp Pediatr. 1993;36(5):634-642.   Published online May 15, 1993
Clinical observation was performed on 23 children with Guillain-Barré syndrome which were hospitalized at the Pediatric ward of Han Yang University Hospital, from July 1975 through May 1991. The results were as follows: 1)The ratio of male patient to female was 2.8:1 and 47.8% of total GBS patients were between the ages 1to 5 years. 2)The highest seasonal incidence of GBS was seen...
Case Report
A Case of VATER Syndrome
Seong Jin Ha, Ki Hyun Chung, Oh Kyung Lee, Wan Seob Kim, Jae Seung Yang
Clin Exp Pediatr. 1993;36(4):583-588.   Published online April 15, 1993
The VATER syndrome is a group of congenital anomalies with a nonrandom tendency for concurrence. Defects include vertebral, anorectal malformation, tracheoesophageal fisutla with esophageal atresia, radial-limb, vascular, and renal abnormalities. The critical period of organogenesis is at or before the sixth or seventh week of gestation. We experienced one case of VATER syndrome in a 1 day old male neonate having...
A Case of Cerebrocostomandibular Syndrome with Congenital Heart Disease
Sang Heui Song, Kook In Park, Hye Jung Joo, Ran Namgung, Chul Lee, Dong Gwan Han
Clin Exp Pediatr. 1993;36(4):574-578.   Published online April 15, 1993
Cerebrocostomandibular Syndrome is characterized by micrognathia, cleft palate, multiple thorax deformity and frequently, mental deficiency. Respiratory compromise is a common cause of death. We experienced a case of cerebrocostomandibular syndrome with congeniyal heart disease in a 2 day old female baby with the chief complaint of cyanosis and respiratory difficulty since birth, She was delivered by cesarian section due to delayed...
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