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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...
Original Article
A Clinical Study of Myelodysplastic Syndrome in Children
Seong Hoon Hah, Hyo Seop Ahn
Clin Exp Pediatr. 1993;36(4):556-567.   Published online April 15, 1993
Myelodysplastic syndrome is a heterogeneous disease complex which is basically a clonal disorder and has characteristics of cytopenia of one or more cell series in peripheral blood and of dyspoiesis of precurosirs in bone marrow. Since the nature of this disease in childhooh is very different from that in adults, retrospective clinical study was performed with 17 confirmed patients who...
Effect of Cyclosporin, Indomethacin and Methylprednisolone on Puromycin-Aminonucleoside Induced Nephrosis in Rats
Hong Bae Kim, Hae Lee Chung, Cheol Woo Ko, Ja Hoon Ko, Jung Sik Gwak
Clin Exp Pediatr. 1993;36(4):495-505.   Published online April 15, 1993
This experimental study was conducted to evaluate the effect of indomethacin and methylprednisolone on PAN-induced nephrosis in rats. Sprague-Dawley rats weighing 150∼200gm were used and divided into controls, group Ⅰ(PAN intraperitoneally), group Ⅱ(PAN intraperitoneally, followed by indomethacin peritoneally for 12 days), group Ⅲ(PAN intraperitoneally, followed by methylprednisolone peritoneally for 12 days) and group Ⅳ(PAN intraperitoneally, followed by cyclosporin subcutaneously for...
Case Report
A Case of Monozygotic Twin with Dwn Syndrome
Seong Heon Jeon, Choong Hyun Yoon, Young Wook Kim, Ki Bok Kim
Clin Exp Pediatr. 1993;36(3):434-438.   Published online March 15, 1993
Down syndrome is the most common autosomal chromosomal abnormality characterized by mental and growth retardation, and by various typical features including prominent epicanthal fold, oblique palpebral fissure, flat nasal bridge, short and broad hand, wide toe interspace, etc. The overall incidence has been shown to be 1:800 deliveries, increasing with advancing maternal age. However, twin cses are extremely rare, and...
A Case of Incontinentia Pigmenti
Soo Am Chung, Won Rae Kim, Hyung Kun Nam, Jin Sam No, Jung Hee Park
Clin Exp Pediatr. 1993;36(3):428-433.   Published online March 15, 1993
Incontinentia pigmenti (Bloch-Sulzberger syndrome)is a genetic disease of the skin with generalized ectodermal and mesodermal dysplasia. Skin lesions are uually present at birth or shortly after in the form of eythermatous eruptions with linear vesiculations. The final stage is characterised by irregular macular streaks of brown to slate-grey pigmentation. We experienced five cases of incontinentia pigmenti maternal female relative of a...
A Case of MELAS Syndrome
Clin Exp Pediatr. 1993;36(3):394-402.   Published online March 15, 1993
MELAS syndrome is a rae but distinct clinical entity belonging to a group of mitochondrial encephalomyopathies characterized by the tetrad of mitochondrial myopathy, encephalopaphy, lactic acidosis, and stroke-like episodes. We experienced a case of MELAS syndrome in an 8 year-old boy who showed headache, pain of the eyeball, vomiting, stroke-like episodes such as visual disturbance and dysarthria, myoclonic seizure, confusion,...
Original Article
Surfactant Replacement Therapy in Neonates with Respiratory Distress Syndrome: A Collective Evaluation of Trials from 16 Hospitals
CW Bae, YD Kwon, SJ Ko, KS Kim, HM Kim, WS Park, SH Byun, CS Son, HS Ahn, SG Lee, YP Chang, YJ Chung, KS Cho, KH Cho, KC Choeh, MJ Chey, JH Choi, JK Yoon, CI Ahn, S Chida, T Fujiwara
Clin Exp Pediatr. 1993;36(2):244-265.   Published online February 15, 1993
Surfactant replacement therapy in neonates with respiratory distress syndrome (RDS) has been introduced in our country since May 1990. The purpose of this study was to assess the effect and short-term outcome of surfactant replacement for neonatal RDS using collective data of uncontrolled trials from different hospitals in Korea. For the period May 1990 to Dec. 1991, a total of 68...
A Study on the Atlantoaxial Instability in Children with Down syndrome
Sa Young Kim, Chan Yung Kim
Clin Exp Pediatr. 1993;36(2):179-184.   Published online February 15, 1993
To evaluate the atlantoaxial instability in children with Down syndrome, the authors analyzed the 97 children with Down syndrome attending to 2 schools for handicaped children in Pusan. The results of study were as follows; 1) The incidence of atlantoaxial instability in 97 individuals with Down syndrome was 8(8.3%) in neutral position and in 10(10.3%) in flexion, respectively. 2) The mean atlantoaxial gap...
Case Report
A Case of Prune Belly Syndrome in Female
Zu Heo, Eun Joo Shin, Woo Yeong Chung, Soon Yong Lee
Clin Exp Pediatr. 1992;35(10):1454-1458.   Published online October 15, 1992
We experienced a case of prune belly syndrome in a 2 days old female with deficient abdominal wall musculature and urinary tract anomalies which composed of bilateral multicystic kidney, left vesicoureteral reflux and megaureter with kinking in appearance and marked distended bladder. On ultrasonographic examination, there were no abnormalities in uterus, vagina and overies. A review of literatures was also presented briefly.
Original Article
Diagnostic Accuracy of Stable Microbubble Rating Test for the Early Detection of Neonatal Respiratory Distress Syndrome
Jung-Sam Jun, Chy-Hyung Park, Jong-Woo Bae, Chang-Il Ahn
Clin Exp Pediatr. 1992;35(10):1361-1368.   Published online October 15, 1992
Respiratory distress syndrome(RDS) of preterm infants remains a significant cause of morbidity and mortality despite improvements in neonatal intensive care and artificial ventiratory 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 on RDS infants by Fujiwara and co-workers in 1980. Therefore, exogenous surfactant replacement...
Case Report
Two Cases of Alport Syndrome Developed in Sister
Clin Exp Pediatr. 1992;35(9):1314-1318.   Published online September 15, 1992
The moderate sensory neural hearing loss and decreased visual acuity were accompanied in a 15 year old girl with chronic renal failure, and so we suspected Alport syndrome. In Family study, her younger sister of 12 years old had shown persistent hematuria from 2 years ago and mild hearing loss. HTe ultrasound-guided percutaneous renal biopsy was performed in younger sister...
A Case of Kugelberg-Welander Syndrome
Sun Young Lee, Myung Ik Lee, Soon Wha Kim, Don Hee Ahn, Je Geun Chi
Clin Exp Pediatr. 1992;35(9):1263-1266.   Published online September 15, 1992
We have experienced a 6 year-old female patient who had suffered from progressive muscular atrophy with weakness and loss of voluntary motor action since 3 years of age. She was diagnosed as Kugelberg-Welander syndrome by EMG and muscle bio. Multiple polyphasic motor unit potentials were revealed on EMG and grouped muscle atrophy without fibrosis or degenerative change was found on...
A Case of Stein-Leventhal Syndrome with Severe Obesity
Kyeong Sang Kim, In Hee Jung, Hong Jin Lee, Won Il Park, Kyung Ja Lee
Clin Exp Pediatr. 1992;35(8):1164-1168.   Published online August 15, 1992
We experienced a extremely rare case of Stein-Leventhal Syndrome in 7 year-old female patient. Her chief complaints was severe obesity. We performed specific radiologic and hormonal studies. Diagnosis was suspected by pelvic sono and CT. We performed vertical gastroplasty with Taplon bandage for morbid obesity. A brief review of related literature was made.
A Case of Hypomelanosis of Ito Accompanying Turner Syndrome
Kyung Un No, Dong Wook Kim, Dong Joo Shin, Hyung Ro Moon
Clin Exp Pediatr. 1992;35(8):1157-1163.   Published online August 15, 1992
Hypomelanosis of Ito has been known to show the characteristic hypopigmentation of the skin over the trunk and the extremities following Blaschko lines, and to accompany abnormalities of the central nervous system, the skeletal system, the eye and so on. Lately, a variety of chromosomal anomalies, especially mosaicism, have been reported in association with hypomelanosis of Ito, which is believed...
Seven Cases of Immotile Cilia Syndrome
Sun Young Lee, Myung Ik Lee, Don Hee Ahn, Keun Chan Sohn, Je Geun Chi
Clin Exp Pediatr. 1992;35(8):1127-1134.   Published online August 15, 1992
Immotile cilia syndrome is a disease caused by congenital abnormality of the cilia and symptom complex composed by chronic rhinitis, sinusitis, otitis, male infertility etc. We have experienced 7 cases of immotile cilia syndrome diagnosed by electron microscopic finding of nasal or bronchial mucosal biopsies since 1984. Six cases were girls and type Id was most common (4 cases) on...