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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...
2 Cases with Adult Respiratory Distress Syndrome in Infant and Young Child
Hye Jung Joo, Im Jae Park, Joon Soo Lee, Soo Young Lee, Byeung Ju Jeoung, Kyu Earn Kim, Ki Young Lee, Hyunee Yim, Soon Won Hong, Woo Hee Jung
Clin Exp Pediatr. 1992;35(8):1118-1126.   Published online August 15, 1992
Adult respiratory distress syndrome has been described as a clinical syndrome of respiratory distress and need for mechanical ventilation with positive end-expiratory pressure, diffuse pulmonary infiltration on thoracic roentgenogram, impaired pulmonary compliance, decreased lung volumes and capacities, increased alveoloarterial oxygen gradient and hyaline membrane formation. It is the clinical manifestation of injury to the terminal alveolocapillary unit as a result...
A Case of Prune Belly Syndrome ssociated with Turner Syndrome
Chang Soo Oh, Sang Muk Choi, Jae Ock Park, Chang Hwi Kim, Sang Jhoo Lee
Clin Exp Pediatr. 1992;35(7):978-983.   Published online July 15, 1992
The Prune belly syndrome is a rare congenital anomaly characterized by lax, wrinkled abdominal wall, cryptorchidism and urinary tract anomalies. But it has a wide spectrum of clinical presentation and combine with other anomalies such as pulmonary, skeletal, digestive, cardiovascular and other system. We experienced a case of prune-belly syndrome associated with Turner syndrome in a 18 month old female...
Hutchinson-Gilford Progeria Syndrome
Moon Whan Lee, Byoung Geun Lee, Pyung Han Hwang, Dae Yeol Lee, Jung Soo Kim
Clin Exp Pediatr. 1992;35(7):971-977.   Published online July 15, 1992
Hutchinson-Gilford Progeria Syndrome is an extremely rare condition that was initially reported by Jonathan Hutchinson in 1886 and further described by Hastings Gilford in 1904. Clinical manifestations are evident by the first or second year of life with the physical characteris-tics of the elderly. Progeric patients ordinarily develop atherosclerosis and die of cardiac or cerebral vascular disease between 7 and 27...
Original Article
A Clinical Aspect of the Hemolytic Uremic Syndrome
Hye Won Park, Tae Sun Ha, Il Soo Ha, Hae Il Cheong, Yong Choi, Kwang Wook Ko
Clin Exp Pediatr. 1992;35(7):909-920.   Published online July 15, 1992
We reviewed the medical records of 14 children (8 girls, 6 boys), diagnosed as hemolytic uremic syndrome at Seoul National University Children뭩 Hospital from 1981 to 1990. The age at presentation ranged from 1 month to 10 years, with a mean age of 2.7 years. Only eight (57.1%) of the children had diarrheal prodrome and five (35.7%) had grossly bloody...
Case Report
A Case of McKusick-Kaufman Syndrome
Han Joo Kong, Young Mi Jee, In Kyung Sung, Byung Churl Lee
Clin Exp Pediatr. 1992;35(6):829-833.   Published online June 15, 1992
We experienced a case of Mckusick-Kaufman syndrome in a 14 day-old female neonate. She had hydrometrocolpos with vaginal atresia and polydactyly of feet associated with bilateral hydronephrosis and umbilical hernia. Hydronetrocolpos with vaginal atresia was corrected by abdominoperineal-vaginal pull through operation on 21st day of life. A brief review of related literatures was made.
Original Article
Clinical Observation on Reye syndrome According to the Onset of Age in Children
Kyung Hee Kim, Baik Hee Lee, Myung Ik Lee, Don Hee Ahn, Keun Chan Sohn
Clin Exp Pediatr. 1992;35(6):788-794.   Published online June 15, 1992
A clinical observation according to the age of onset was made on 40 children with Reye syndrome who had been admitted to pediatric department of National Medical Center during 11 year period from January 1979 to December 1989. 1) Annual distribution showed the largest number in 1982. There was no significant difference in seasonal distribution. 2) Cases of infant comprised 22 (55.0%)...
Case Report
A Case of D13 Ring Chromosome Syndrome
Sung Lae Park, Ho Jun Im, Jae Hun Shin, Hahng Lee, Myung Soo Lyu, Yong Kyun Paik
Clin Exp Pediatr. 1992;35(5):713-717.   Published online May 15, 1992
A case of D13 Ring Chromosome Syndrome, confirmed by chromosome study, was described. She was 8 years and 4 months old, and menifested facial abnormalities, psychomotor retardation and other multiple congenital abnormalities.
Two Cases of Cornelia de Lange Syndrome
Yoon Jong Yoo, Ki Chan Na, Ho Seong Yoo, Sang Kee Park, Yong Bong Park
Clin Exp Pediatr. 1992;35(5):684-690.   Published online May 15, 1992
The Cornelia de Lange syndrome is characterized by severe growth and mental retardation, typical face, and low-pitched growling cry. We had experienced two cases of Cornelia de Lange syndrome in korean male infants. The baby showed microbrachycephaly, typical appearance of face with low forehead, bushy eyebrows, long curely eyelashs, thin lip with cleft palate, low set ears, generalized hirsutism and growth retardation...
A Case of Prader Willi Syndrome
K.N. Kim, Y.T. Jang, S.Y. Choi, W.S. Kim
Clin Exp Pediatr. 1992;35(5):678-683.   Published online May 15, 1992
Prader willi syndrome is characterized by infantile hypotonia, mental retardation, hyperphagia with obesity, and hypogonadism. The etiology of Prader Willi syndrome is still unknown; however, a hypothalamic defect has been postulated and recently, the deletion of long arm of chromosome 15 has been postulated and recently, the deletion of long arm of chromosome 15 has been considered as a cause...
A Case of WDHA(Watery Diarrhea Hypokalemia Achiorhydria) Syndrome
Nam Seon Beck, Jun Jai Kim, Beom Soo Park, Jeong Kee Seo, Hyo Seop Ahn, Hyung Ro Moon, Je Geun Chi
Clin Exp Pediatr. 1992;35(4):575-580.   Published online April 15, 1992
VIP secreting tumors are rare in children but they produce a dramatic clinical picture, the most prominent feature of which is profuse, watery diarrhea and hypokalemia. A 5-year-old girl was brought to Seoul National University Children's Hospital for evaluation of profuse watery diarrhea. She presented with watery diarrhea, hypokalemia, and stunted growth, and had experienced these problems for four years...
Original Article
The Incidence of Hypothyroidism in Children with Down Syndrome
Seong Hyeon Jeon, Chun Ho Cho, Kyoung Sim Kim, Ki Bok Kim
Clin Exp Pediatr. 1992;35(4):534-538.   Published online April 15, 1992
A review of Thyroid function tests was performed on 32 children with Down syndrome confirmed by cytogenetic examination at the Dept. of Pediatrics, Kwangju Christian Hospital from Jan.1989 to Feb.1990. 1) Twenty among 32 children with Down syndrome were male, with the sex ratio being 1.7 : 1. Their ages ranged from 3 days to 5 years, with the mean age...
Clinical Observation of Meconium Aspiration Syndrome: Prognostic Implication of Early Meconium Suctioning
Dae Hyun Kim, Dong Hoon Ko, Young Jong Woo, Young Youn Choi, Tae Ju Hwang
Clin Exp Pediatr. 1992;35(4):484-491.   Published online April 15, 1992
The authors observed the clinical findings of 36 patients with meconium aspiration syndrome(MAS) to evaluate the effect of early appropriated meconium suctioning on the morbidity and mortality of the syndrome. Patients in the study group, 20 babies born in Chonnam university Hospital, received appropriate meconium suction through endotracheal tube during and rig-ht after the delivery, while the patients in the...
Case Report
A Case of 7q-Syndrome
Ja Hyun Park, Baek Gil Lee, Il Kyung Kim, Jung Sik Min, Chang Hee Choi
Clin Exp Pediatr. 1992;35(3):422-427.   Published online March 15, 1992
7q-syndrome, although rare, is a well defined syndroime which usually arises de novo. Characteristic clinical features include severe growth and metal retardation, microcephaly, low birth weight, broad nasal bridge with bulbous nasal tip, large, low set ears and genital anomalies. We experienced a male newborn with clinical features suggestive of chromosomal anomaly which was confirmed to be a 7q-(q32-qter) syndrome...
Adult Respiratory Distress Syndrome in Infancy
Hoo Jae Hann, Young Mi Hong, Seung Joo Lee
Clin Exp Pediatr. 1992;35(2):245-251.   Published online February 15, 1992
Adult Respiratory distress Syndrome (ARDS) is a clinical entity characterized by respiratory distress and diffuse pulmonary infiltrates due to increased permeability after various kinds of insult to alveolar-capillary membrane. We experienced a case of ARDS in a 3-1/2 month old girl after Pseudomonas pneumonia, cardiac arrest and generalized convulsion. This is the report on her clincal and autopsy findings with brief review...
A Cases of Goldenhar's Syndrome
Jin Eun Hyun, Eun Hee Park, Hee Young Jeon, Whwa Jin Byeun, Young Mok Hwang
Clin Exp Pediatr. 1992;35(1):135-139.   Published online January 15, 1992
Goldenhar's syndrome is a syndrome consisting of epibulbar dermid or lipodermoid, preauricular skin appendices and blind ended fistulas and vertebral anomalies. We experienced a case of Goildenhar's syndrome is a one month-old boy who showed asymmetric face, preauricular skin tag, hemiertebrae, rib anomaly and cyanotic congenital heart disease. A review of the references concerning Goldenhar's syndrome was made birdefly.
Original Article
A case of transient myeloproliferative disorder in Down's syndrome.
Dong Uk Kim, Woo Ki Lee, Eung Won Park, Kwang Woo Kim
Clin Exp Pediatr. 1991;34(12):1740-1744.   Published online December 31, 1991
Transient myeloproliferative disorder has been reported in infants with Down’s syndrome. This disorder is clinically and hematologically indistinguishable from congenital leukemia. In contrast to congenital leukemia, complete clinical and hematological recovery occurs within weeks to months of diagnosis without antileukemic treatment. We experienced a case of Transient myeloproliferative disorder with Down’s syndrome. This 4-day-old female neonate showed jaundice, hepatomegaly, fever, dyspnea, mongoloid face and congenital heart...
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