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Case Report
A Case of Antley-Bixler Syndrome
Young Rae Kim, Kook In Park, Choon Sik Yoon, Ran Namgung, Chul Lee, Dong Gwan Han
Clin Exp Pediatr. 1995;38(4):582-585.   Published online April 15, 1995
Antley-Bixler syndrome is a very rare disese of characteristic feature of craniosynostosis, brachycephaly, midface hypoplasia, depressed nasal bridge, radiohumeral synostosis and bowing femur. We presented a case of Antley-Bixtler syndrome with brief review of lituratures.
Two Siblings of Non Salt Losing Congenital Adrenal Hyperplasia due to 21-hydroxylase Deficiency
Seung Cheol Lee, Byung Kyu Suh, Byung Churl Lee
Clin Exp Pediatr. 1995;38(4):574-581.   Published online April 15, 1995
Congenital adrenal hyperplasia in caused by a defect in the biosynthesis of cortisol as a result of deviciency in one of the essential enzymes, most commonly 21-hydroxylase and is an autosomal recessive disease in close genetic linkage with HLA. The common clinical manifestations of congenital adrenal hyperplasia is progressive virilization in both sexes, abnormal external genitalia in the...
A Case of Patent Ductus Arteriosus with Eisenmenger Syndrome Treated by Ligation of PDA
Chang Seon Yoo, Hae Yong Lee, Mee Kyung Namgoong, Jong Soo Kim, Mee Yon Cho, Joong Hwan Oh
Clin Exp Pediatr. 1995;38(2):257-263.   Published online February 15, 1995
We experienced a case of eisenmenger syndrome treated by simple surgical intervention. Eisenmenger syndrome refers to patients with congenital heart defects who have a systemic level of pulmonary arterial pressure and high pulmonary vascular resistance, with right-to-left or bidirectional shunting. Because a progressive rise in pulmonary vascular resistance may be better tolerated with an intracardiac communication, surgical repair in such patients...
G Deletion Syndrome II
Bon Su Koo, Sang Uk Park, Yung Tak Lim, Hee Ju Park
Clin Exp Pediatr. 1995;38(2):240-244.   Published online February 15, 1995
We experienced a case of G deletion syndrome II in a 8 year and 1 month old boy. He showed mental retardation, microcephaly, high arched palate, low set malformed ears, epicanthal fold, thoracic deformity, tracheomegaly and two lumens of esophagus. Chromosomal study showed the deletion of long arm of chromosome 22, kariotypically he was depicted as 46, XY, 22q-. A brief...
Original Article
Sequential Changes of Chest Radiographic Finding after Exogenous Surfactant Replacement Therapy in Neonates with RDS
Hyeok Choi, Chong-Woo Bae, Sa-Jun Chung, Yong-Mook Choi
Clin Exp Pediatr. 1995;38(2):151-158.   Published online February 15, 1995
Purpose : The chest radiograph is useful and reliable in assessing the severity and progres-sion of neonatal respiratory distress syndrome. To evaluate the effect of exogenous surfactant r eplacement therapy, we performed sequential observation of chest radiogratphic findings in ne-onates with respiratory distress syndrome. Methods : Two groups of infant with RDS in mechanical ventilation therapy were studied. S-urfactant(S-Tx) group(n=36) was...
Clinical Differences by Karyotype in Patients with Turner Syndrome
Mi Jung Park, Jin Sung Lee, Kir Young Kim, Duk Hi Kim, Ho Seoung Kim
Clin Exp Pediatr. 1995;38(2):143-150.   Published online February 15, 1995
Purpose : The aim of this study was to evaluate clinical differences by karyotype in Turner Syndrome. Methods : We evaluated 66 patients with Turner syndrome diagnosed at Yonsei University College of medicine from Mar.1985 to Feb.1993. We divided subproups as pure 45,X groups, mosaisism groups and structural aberration groups. Clinical features, serum estrogen, LH, FSH, concentrations, gonadotropin release after GnRH...
Case Report
A Case of Dandy-Walker Syndrome with Complex Cardiac Anomaly
Ssack Joong Yoon, Sung Jin Hong, Hyung Goo Cho, Dong Chul Park
Clin Exp Pediatr. 1995;38(1):110-116.   Published online January 15, 1995
The Dandy-Walker syndrome is a developmental disorder of the brain characterized by cystic dilatation of the fourth ventricle and agenesis or hypoplasia of the cerebellar vermis. We experinced a case of Dandy-Walker syndrome with complex cardiac anomaly in female newborn who presented with apnea at birth. Physical examination showed coloboma on left eye, low estting malformed ear, and high arched...
A Case of Dandy-Walker Syndrome with Chromosomal Abnormality
Hyui Sung Chang, Seok Kyu Lee, Gi Chung Lee, Woo Ki Lee, Kwang Woo Kim
Clin Exp Pediatr. 1994;37(12):1784-1788.   Published online December 15, 1994
The Dandy-Walker syndrome is a developmental disorders of the brain characterized by cystic defromity of the 4th ventricle and agensis of the cerebellar vermis. Other systemic anomalies and chromosomal abnormalities are associated with this syndrome. We are experienced a case in a 9 months old male infant who presented initially with frequent vomiting, low birth weight, On the physical examination, a...
A Case of Remission of Systemic Juvenile Rheumatoid Arthritis (Still's Disease) Treated with High-dose Intravenous Gammaglobulin
Yon Sook Rho, Yun Woo Lee, Sang woo Kim
Clin Exp Pediatr. 1994;37(12):1767-1772.   Published online December 15, 1994
High dose intravenous gammaglobuline (IVIG) therapy is effective in some of the autoimmune diseases. Although the exact mechamism of action of IVIG is uncertain, the action as a neutralizing antibody against unknown etiologic agents, the action of blocking of Fc receptors of effector cells, or the action as a antiidiotypic antibody are suggested. We report a case of 12 year old...
A Case of Sjören-Larsson Syndrome
Kil Joon Lee, Jong Bock Kim, Dong Hwan Lee, Sang Man Shin, Sang Jhoo Lee
Clin Exp Pediatr. 1994;37(12):1757-1761.   Published online December 15, 1994
Sjören-Larsson Syndrome is a rare disorder chracterized by congenital icthyosis, spastic diplegia or tetraplegia and mental retardation. The inheritance is autosomal recessive, due to deficiency of alcohol dehydrogenase activity of fatty acid matabolism. We have experienced a case of Sjören-Larsson Syndrome in a 16 month-old male children who have dried thick skin, developmental delay, and spastic diplegia. He was much improved clinically...
A Case of Wiskott-Aldrich Syndrome
Byoung Geun Lee, Soo Hee Chang, Soo Young Cho, Pyoung Han Hwang, Jung Soo Kim
Clin Exp Pediatr. 1994;37(11):1615-1619.   Published online November 15, 1994
Wiskott-Aldrich Syndrome is an X-linked disorder characterized by recurrent infection, thrombocytopenia and eczema. Various defects in cell-mediated immunity and deficient antibody response to carbohydrate antigens have been described. We experienced a case of Wiskott-Aldrich Syndrome of 28 months old male patient. He has been suffered from multiple petechiae with bleeding, recurrent pyogenic infections and generalized eczema since 3 months of...
A Case of Epstein's Syndrome
Seon Young Choi, Hyun Chul Chae, Hae Young Cho, Hong Bae Kim, Ji Sub Oh
Clin Exp Pediatr. 1994;37(11):1610-1614.   Published online November 15, 1994
Epstein's syndrome is a rare disease whish is characterized by the association of thrombocytopenia, macrothrombocytopathia, nephritis and deafness. We experienced a case of Epstein's syndrome in a 12 years old male patient who was presented with a life long history of bleeding, usually as epistaxis, bilateral sensorineural deafness and hematuria with proteinuria starting in the childhood. Hematologic studies showed thrombocytopenia with...
Adrenogenital Syndrome Combined with Congenital Hypothyroidism
Jong-Woon Choi, Woo-Sik Cheong, In-Kyu Yi
Clin Exp Pediatr. 1994;37(11):1607-1609.   Published online November 15, 1994
Original Article
A Study for Menstruation of School Girl in Ansan
Ae Yeon Lim, Dae Hun Pee, Baik Lin Eun, Sang Hee Park, Soon Kyum Kim
Clin Exp Pediatr. 1994;37(11):1586-1595.   Published online November 15, 1994
Purpose : Menstruation is a great part as the procedure for physiological, psychological development and physical growth, and menstrual disorders are very common problems in adolescence period. These problems can be resolved by interview or appropriate education of normal pubertal development. However unlike other developed countries, systematic report or appropriate education for menstruation may not be performed in our country....
Diagnostic Value of Stable Microbubble Rating Test and Shake Test for the Early Detection of Respiratory Distress Syndrome in Prematurity
Sung Eun Lee, Sang Lak Lee, Chin Moo Kang
Clin Exp Pediatr. 1994;37(11):1500-1507.   Published online November 15, 1994
Respiratory distress syndrome (RDS) of newborn is a disease revealed high morbidity and mortality rate, especially in premature infant. To evaluate the predictive value of Stable Microbubble Rating (SMR) and Shake test on RDS in premature infant, the anthors carried out the gastric aspirates Shake test and SMR test at birth, 3 and 6 hours after birth respectively on 124 premature...
Case Report
A Case of Cerebral Infarcion due to Thrombosis Associated with Focal Segmental Glomerulosclerosis and Steroid Resistant Nephrotic Syndrome
Dae Woo Kim, Heon Lang Park, Sang Man Shin, Eun Mi Kim
Clin Exp Pediatr. 1994;37(9):1317-1324.   Published online September 15, 1994
Focal segmental glomerulosclerosis is the renal histopathologic lesion observed in 10% of children with idopathic nephrotic syndrome. Complications include infection, thrombosis, hypocalcemia and adverse effect of steroid use. The incidnce of thrombosis reported ranges from 10~40%. The pathogenesis are changes in coagulation system, decrease of Antithrombin III, increase platelet aggregability and steroid or diuretics use. These changes take place passively...
A Case of Miescher Syndrome with Insulin-Resistant Diabetes Mellitus
Byung Min Choi, Jong Kwang Lee, Kee Hwan Yoo, Joo Won Lee, Soon Kyum Kim
Clin Exp Pediatr. 1994;37(9):1292-1295.   Published online September 15, 1994
Miescher syndrome comprises congenital acanthosis nigricans, hypertrichosis, failure to thrive and short stature, dysmorphism especially of the jaws and oral cavity. Insulin-resistant diabetes mellitus, and a characteristic general appearance. This report concerns a rare case of 12-year-old girl having insulin resistant diabetic mellitus with Miescher syndrome. The relevant literature was reviewed.
Original Article
The Roles of IgG and Albumin as a Predictor of Frequent Relapse
Jae-Ho Lee, Jong-Gyun Kim
Clin Exp Pediatr. 1994;37(9):1245-1250.   Published online September 15, 1994
The etiology of nephrotic syndrome is unknown. The characterization were proteinuria, hypoalbuminemia, generalized edema and hyperlipidemia. To assess the recurrence factors in the nephrotic syncrome, we measured serum immunoglobulin(IgG, IgA, IgM), albumin, complement, cholesterol and the 24-hour total urine protein at the initial relapse of nephrotic syndrome. Each data of frequent and infrequent relapsed nephrotic syndrome were compared. In total...
Case Report
Three Cases of Apert Syndrome (Acrocephalosyndactyly)
Young Sil Ahn, Jong Won Lee, Jin Keon Bang, Doo Bong Lee
Clin Exp Pediatr. 1994;37(8):1149-1155.   Published online August 15, 1994
Apert syndrome is an uncommon, congenital disorder characterised by malformation of the skull, most often acrocephaly or oxycephaly, in association with symmetrical syndactyly of both hands and feet. It is due to disturbance in the growth of bone and soft tissue affecting principally the head, the hands and the feet. The original description was presented by Troquart in 1886, and...
Original Article
Incidence and Perinatal Risk Factors of Respiratory Distress Syndrome of Newborn
Yun Sil Chang, Beyong Il Kim, Jung-Hwan Choi, Chong Ku Yun
Clin Exp Pediatr. 1994;37(8):1065-1077.   Published online August 15, 1994
Over a continuous 4-year period, from January 1989 till December 1992, 7,100 infants were born in Department of Obstetrics and admitted to Nursery or Neonatal Intensive Care Unit of Department of Pediatrics of Seoul National University Children's Hospital. Among them, 126 cases were diagnosed as respiratory distress syndrome (RDS) of newborn. The total incidence of RDS among all the newborns(inborn)was 1.77%....
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...
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