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Original Article
A Study of Meconium Aspiration Syndrome.
Chae Sup Yoo, Mee Kyung Kim, Keun Lee, Jae Sun Jung
Clin Exp Pediatr. 1988;31(11):1425-1431.   Published online November 30, 1988
The authors observed 51 cases of meconium aspiration syndrome in newborns who were bom at Ewha Womans University Hospital during the period of January 1986 through May, 1987. The results are summarized as follows: 1) The incidence of meconium aspiration syndrome was 1.6% (51 cases). 2) There was a significantly greater incidence of developing meconium aspiration syndrome in meconium stained babies who had toxemia mothers and low...
A Case of Dapsone Syndrome.
Jeong Kyu Seoh, Hye Kyong Bae, Jae Seung Yang, Eung Deok Choi, Baek Keun Lim, Jong Soo Kim
Clin Exp Pediatr. 1988;31(10):1376-1380.   Published online October 31, 1988
Dapsone syndrome is a rare hypersensitivity reaction to Dapsone. The reaction included fever, malaise, dermatitis jaundice with hepatic dysfunction, lymphadenopathy, and hemolytic anemia. We have experienced a case of Dapsone syndrome in a 10 years old male. This patients had taken D.D.S. with 50 mg per day for 1 month to treat an unknown skin disease. After then, above mentioned symptoms and signs were...
A Case of Treacher Collins Syndrome.
B I Choo, Y M Jung, S S Kee, U S Shim, H S Lee
Clin Exp Pediatr. 1988;31(10):1371-1375.   Published online October 31, 1988
The authors report a 3 days old newborn who showed the abnormalities comprising relatively complete form of Treacher Collins syndrome. These include following findings; 1) Antimongoloid slant of palpebral fissures. 2) Coloboma in outer third of lower lid with absence of cilia medial to coloboma. 3) Large nose with narrow nares and flattened frontonasal angle. 4) Macrostomia with cleft palate. 5) Deformed pinna, extra ear tag, blind fistura, absent external auditory canals,...
A Case of 4P-Syndrome.
Hong Kyu Lee, Sung Sik Lee, Soon Il Lee, Young Seok Lee, Kweon Ha Son
Clin Exp Pediatr. 1988;31(10):1366-1370.   Published online October 31, 1988
The first short arm deletion syndrome in human was described by Lejeune et al. in 1963. This has been labelled the 5p- syndrome. It was given the name ‘cri du chat` because of the characteristic kitten like cry of these patients. Two years later, Wolf et al. demonstrated a second short arm deletion syndrome which had no kitten like cry, but had characteristic...
Diagnosis of Meconium Aspiration by Spectrophotometric Analysis of Urine.
Chang Yul Kim, Hye Kyung Bae, Mee Kyung Namgoong, Baek Keun Lim, Joong Soo Kim
Clin Exp Pediatr. 1988;31(10):1275-1280.   Published online October 31, 1988
We measured optical density of aqueous extracts of meconium and meconium stained amniotic fluid at 390 nm, 405 nm, 420 nm, 435 nm, 450 nm by spectrophotometry. We also applied the spectrophotometric method to the urine from 25 infants with clinical sign of meconium aspiration and from normal infants. The absorption band at 450 nm observed on the spectra of urine was estimated as...
Lipoproteinelectropheresis pattern in Nephrotic Syndrome.
Soon Don Hong, Byung Soo Cho, Chang Il Ahn
Clin Exp Pediatr. 1988;31(9):1163-1170.   Published online September 30, 1988
This study was undertaken to eluidate lipoprotein patterns in patients with childhood nephrotic syndrome by response to initial steroid therapy, serum albumin level in MCNS and other histologic classifications. The result were summerized as follows. 1) There were 19 cases (52.8%) of abnormal lipoprotein patterns by Fredrickson classifications, type Ila were 6 cases (16.7%), type lib were 1 cases (2.8%) and type IV were 12 cases...
A Study on Prognostic Factors of Meconium Aspiraion Syndrome.
Oh Kyung Lee, Hong Chul Lee, Wan Seoub Kim, Myung Ho Lee
Clin Exp Pediatr. 1988;31(9):1114-1119.   Published online September 30, 1988
We observed 42 cases of neonates, who were diagnosed as having Meconium aspiration syndrome in neonatal intensive care unit of Presbyterian Medical Center from July 1984 to May 1987. The followings were obtained: 1) The incidence of Meconium aspiration syndrome was 0.6% of all deliveries and 7.4% of meconium stained babies. 2) The geatational age was over 37 weeks in all cases and the body weight was...
A Case of Ectopic ACTH Syndrome Caused by Wilms Tumor.
Dong Hun Chi, Sang Bong Han, Young Jong Woo, Tai Ju Hwang
Clin Exp Pediatr. 1988;31(8):1071-1078.   Published online August 31, 1988
We experienced a extremely rare case of ectopic ACTH syndrome caused by Wilms tumor in a 4 year 8 month-old female patient. We performed specific radiologic and hormonal studies. Diagnosis was confirmed by histologic and biochemical findings of tumor mass after right nephrectomy. A brief review of related literature was made.
A Case of Acute Megakaryoblastic Leukemia in Infant with Down's Syndrome.
Jung Soon Whang, Soon hee Kim, Moon Ja Kim, Keun Lee, Je Geun Chi
Clin Exp Pediatr. 1988;31(8):1064-1070.   Published online August 31, 1988
Acute megakaryoblastic leukemia is an uncommonly recognized disorder that is characterized by rapidly progressive proliferation of atypical megakaryocytes and their precursor cells, and fatal course. Abnormalities in chromosome 21 may have a more than random relationship to acute megakaryoblastic leukemia. The authors reports a case of acute megakaryoblastic leukemia in a 2 month old female patient who was admitted for evaluation of hepatosplenomgaly. Multiple organ infiltration...
Thyriod Function Studies in Children with Nephrotic Syndrome.
In Hoon Lee, Chan Yung Kim
Clin Exp Pediatr. 1988;31(8):1037-1047.   Published online August 31, 1988
To evaluate the influence of nephrotic syndrome on the thyroid function test in children, serum T3, T4, free T4, TSH, TBG and rT3 were measured using radioimmunoassay in 14 clinically euthyroid children with nephrotic syndrome who were admitted to Department of Pediatrics, Pusan National University Hospital from July, 1986 to August, 1987. Control group was 15 children who visit our outpatient department for minor...
A Case of Kasabach-Merritt Syndrome.
Eun Hwa shin, Youn Hong Choi, Ju Hong Cha, Kwang Jun Kim
Clin Exp Pediatr. 1988;31(7):935-941.   Published online July 31, 1988
Kasabach-Merritt Syndrome is a combination of a rapidly enlarging hemangioma and throm- bocytopenia. A 5 day old male newborn who had giant hemangioma associated with thrombocytopenia and anemia is reported with review of literatures. He was placed on prednisolone, blood transfusion, and compression with the elastic bandage over hemangioma. Hemangioma has been somewhat regressed, bleeding tendency has been improved, however, platelt counts are still low.
Study on Diagnostic Methods for Spleen in Complex Heart Disease.
Jung Yun Choi, Yong Soo Yun, Chung Il Noh, Chang Yee Hong, Kyung Mo Yeon, In One Kim
Clin Exp Pediatr. 1988;31(7):833-840.   Published online July 31, 1988
Thirty one cases of congenital heart disease with suspicious splenic anomaly were prospectively studied at Seoul National University Children’s Hopsital from October, 1986 to February, 1988 in order to determine the sensitivity and the specificity of various non-invasive diagnostic methods for splenic status. In most cases, splenic status was confirmed by abdominal aortography. Each case had chest X-ray, abdominal X-ray, bronchus tomography, electrocardiogram, echocardiography,...
A Case of Mucocutaneous Lymph Node Syndrome Complicated by Partial Intestinal Obstruction.
Dong Won Choi, Ki Sup Chung, Jin Suk Suh
Clin Exp Pediatr. 1988;31(6):796-802.   Published online June 30, 1988
Mucocutaneous lymph node syndrome was first described as a new clinical disease by Kawasaki in Japan in 1967. Recently, there have been numerous reports of this disease throughout the world and mucocutaneous lymph node syndrome has become familiar to the pediatrician. Complications previously described include coronary arteritis, carditis and gall bladder hydrops, aseptic meningitis and arthritis. We experienced 1 case of mucocutaneous lymph node syndrome...
Hypoplastic Left Heart Syndrome : Report of a Unique Survivor.
Hang Bo Cho, Seong Ho Kim, Ha Baik Lee, Kyu Hwang Rhee, Chong Moo Park
Clin Exp Pediatr. 1988;31(6):790-795.   Published online June 30, 1988
Hypoplastic left heart syndrome is a rare, and fatal congenital heart disease. Most of the patients with this syndrome die within the 1st month of life. Recently we experienced a hypoplastic left heart syndrome in a child who was unusually living without surgical intervention for 3 and half years. The unique clinical course might result from a number of favorable hemodynamic factors and intact...
Waardenburg's Syndrome in a Boy and Mother.
D H Kim, M Y Kim, C S Suh, S Y Kim, J Y Jung
Clin Exp Pediatr. 1988;31(5):655-659.   Published online May 31, 1988
Waardenburg's syndrome is a rare autosomal dominant disorder which is characterized by (1) lateral displacement of the medial canthi of the eyes and the inferior lacrimal puncta. (2) a broad, prominent root of the nose (3) hyperplasia of the medial portions of the eyebrows (4) white or grey forelock (5) partial or total heterochromia of the irides and (6) deaf-mutism. The authors experienced 2...
A Case of Congenital Nephrotic Syndrome.
Choong Sun Kang, Chang Hee Hwang, Pyung Kil Kim, Hyeon Joo Jeong, In Joon Choi, Jung Hye Choi
Clin Exp Pediatr. 1988;31(5):627-634.   Published online May 31, 1988
Congenital nephrotic syndrome is an uncommon disorder, which is caused by a heterogenous group of renal diseases with variable etiology and natural history. It may be inherited, sporadic, acquird or a part of a general malformation syndrome. This disorder has no single or pathological feature for pathognomonic finding, and there is no ultimate curative treatment except renal transplantation. We experienced a case of congenital...
Burkitt Lymphoma in Children.
Soo Jong Hong, Dong Kyu Jin, Hee Young Shin, Hyo Seop Ahn, Chang Yee Hong, Je G Chi
Clin Exp Pediatr. 1988;31(4):482-491.   Published online April 30, 1988
Seventeen cases of Burkitt lymphoma under 15 years of age who were admitted to Dept, of Ped., Seoul National University Hospital from Jan. 1979 to Dec. 1986 were reviewed and analysed. One hundred and six cases of non-Hodgkin lymphoma were seen during the same period. 1) Burkitt lymphoma accounted for 16% of non-Hodgkin lymphoma. 2) The age ranged from 10/12 year to 15 5/12 years, with...
Physical Features, Karyotypes and Dermatoglyphics of 113 Children with Down Syndrome.
Byung Ho Lim, Kyoung Sim Kim, Ki Bok Kim
Clin Exp Pediatr. 1988;31(4):474-481.   Published online April 30, 1988
The physical features including dermatoglyphic patterns as well as the karyotype patterns were assesed in 113 children with Down Syndrome who had been cytogenetically confirmed in the Department during past 5 years from August 1981. The results are summarized as follows: 1) The sex ratio of the patients was 1.2 male to one female. 2) Cytogenetic examination revealed 21-trisomy in 79% and translocation in 21% of all...
A Case of Kasabach-Merritt Syndrome.
S H Lee, S J Kim, H D Cha, T W Paik, C M Kang
Clin Exp Pediatr. 1988;31(3):398-403.   Published online March 31, 1988
Kasabach-Merritt syndrome (Giant hemangioma associated with thrombo-cytopenia) is a rare disease in infants and ifs hemorrhgic manifestations in these patients are frequently severe enough to threaten life. We experienced a case of this syndrome in a 19 days old boy who had a huge hemangioma on the left upper arm, left anterior chest and back, associated with thrombocytopenia at birth. The patient also had pneumonia...
A Case of Fetal Alcohol Syndrome.
Kee Don Hong, In Chul Yoo, Hung Sub Choi, Dong Hwan Lee, Snag Jhoo Lee
Clin Exp Pediatr. 1988;31(3):375-380.   Published online March 31, 1988
We experienced a case of fetal alcohol syndrome in 9 day old male neonate who delivered at term. On maternal history, his mother had been drunk as much as 64 to 96 cc of alcohol everyday during first trimester and third trimester. The baby had a small for gestational age, microcephaly, bilateral micro-ophthalmia, short palpebral fissure, shallow philtrum, posterior rotation of helix, vocal...
A Case of 18P-Syndrome with Frequent Intection.
Ellen A Kim, Jong Sung Eum, Hee Ju Kim, Sung Ill Ahn, Kyung Yu Park
Clin Exp Pediatr. 1988;31(3):370-374.   Published online March 31, 1988
We herein reported a case of newborn with round face, low nasal bridge, micrognathia, down turning comers of mouth, large protruding ears, high arched palate with webbed short neck: This odd looking newborn was confirmed to have deltion of short arm of chromosome 18 (18P- syndrome). Frequent episodes of pneumonia developed until recently at 6 months of age. The cause of this frequent infection...
Acute Tumor Lysis Syndrome in Burkitt Lymphoma.
Dong Kyu Jin, Soo Jong Hong, Il Soo Ha, Hyo Seop Ahn, Yong Choi, Chang Yee Hong
Clin Exp Pediatr. 1988;31(3):362-369.   Published online March 31, 1988
To assess the tumor lysis syndrome in Burkitt lymphoma, we analyzed the clinical and laboratory findings of 17 patients with Burkitt lymphoma who were admitted to the Department of Pediatrics, Seoul National University Hospital from January 1979 to December 1986. The acute tumor lysis syndrome was diagnosed when two of the five following metabolic abnormal- ities were met: ① hyperuricemia (≥8.0mg/dl), ② hyperphosphatemia (≥5.5 mg/dl),...
Clinical Study on 19 Cases of Neurocutaneous Syndrome in Children.
S H Lee, S J Kim, H D Cha, H S Kim, T C Kwon, C M Kang
Clin Exp Pediatr. 1988;31(3):355-361.   Published online March 31, 1988
The clinical study on 19 cases of neurocutaneous syndrome which were diagnosed at Pediatric Department of Dong San hospital, Keimyung University for last 10 years from Mar. 1975 to Feb. 1985 were performed and following results were obtained. Out of 19 cases, 11 cases were neurofibromatosis, 4 were Tuberous sclerosis and 4 were Sturge- Weber syndrome. 9 cases were male and 10 cases were...
A Case of Cornelia de Lange Syndrome.
Jung Han, Soo Taek Bae, Soon Ok Byun, Ji Sub Oh
Clin Exp Pediatr. 1988;31(2):282-287.   Published online February 28, 1988
The Cornelia de Lange syndrome is characterized by severe growth and mental retardation, typical face, and low-pitched, weak, growling cry, as was first described by Cronelia de Lange in 1933. We have recognized a case of Cornelia de Lange syndrome in a 4 month old Korean girl. The patient showed typical appearance of face with low forehead, bushy eyebrows and synophrys, long curly eyelases,...
A Case of Wilson-Mikity Syndrome.
Jae You Choi, Sang Ho Choi, Byung Hak Lim, Im Ju Kang
Clin Exp Pediatr. 1988;31(2):241-245.   Published online February 28, 1988
We experienced a male newborn patient with Wilson-Mikity Syndrome who showed characteristic X-ray findings and clinical manifestation. We managed the patient conservatively. During in the course, a congestive heart failure with cor pulmonale was developed. He was gradually recovered clinically and radiologically with management. We report one case of Wilson-Mikity Syndrome with review of literature.
A Study on Minimal Change Nephrotic Syndrome in Children.
Yong Choi, Hong Jin Lee, Hae Il Cheong, Kwang Wook Ko, Hyun Soon Lee, Yong Il Kim
Clin Exp Pediatr. 1988;31(2):220-226.   Published online February 28, 1988
To assess the clinical significance of histopathologic variants of minimal change nephrotic syn- drome (MCNS) by light microscopic findings, 148 biopsy proven MCNS patients, who were admitted to Seoul National University Hospital between the period from January 1976 to December 1986, were analyzed according to clinical findings, steroid responsiveness, and follow up status in relation to the pathologic variants. The followings are summary and conclusion. 1) Significantly...
A Case of DiGeorge's Syndrome.
Jong Sik Kim, Dae Young Kim, Kang Ho Kim, Choon Ho Park, Young Bong Park, Kwang Rhun Koo, Chang Soo Ra
Clin Exp Pediatr. 1988;31(1):100-105.   Published online January 31, 1988
DiGeorge’s syndrome is characterized by partial or complete absence of the parathyroid gland and thymus gland and often associated with the cardiovascular and craniofacial anomalies. A-22-days old male infant, delivered without complications at term after a normal pregnancy, developed irritability, feeding difficulties, dyspnea and convulsion. He was characterized by a low-set, malformed right ear, short philtrum of the upper lip, absence of the thymic...
A Case of Congenital Myeloblastic Leukemia Associated with Down's Syndrome.
Ho Jin Oh, Kee Hyoung Lee, Chang Sung Son, Hyun Keum Lee
Clin Exp Pediatr. 1987;30(12):1468-1474.   Published online December 31, 1987
A 4 day old male patient was admitted to our hospital due to jaundice, poor feeding and abdominal distention. His clinical features and peripheral blood smear revealed congenital myeloblastic leuke- mia which confirmed by autopsy. Chromosome study showed 47, XY, +21 in Karyotype. We report this case with review of literatures.
A Case of Short Rib-Polydactyly Syndrome, Type 3.
Yong Woo Park, Jun Ho Kim, Yo Han Kim, Eun Ryong Kim, Soon Jai Lee, Young Jin Kim
Clin Exp Pediatr. 1987;30(12):1439-1445.   Published online December 31, 1987
A case of the Naumoff type (type 3) of short rib-polydactyly syndrome is reported. The skeletal changes included bulging forehead, flat occiput, short cranial base, extremely short horizontal ribs, abnormal vertebrae, and post-axial polydactyly. Microscopically, cartilage showed markedly stunted and disorganized endochondrial ossification. Extraskeletal manifestation were cleft palate, pulmonary hypoplasia, hydropelvis and hydroureter with narrowing of ureterovesical junction. Differentiation from other type of short rib-polydactyly syndrome...
A Case of Larsen Syndrome.
Dong Chul Park, Chan Jun Coe, Duk Jin Yun
Clin Exp Pediatr. 1987;30(11):1305-1310.   Published online November 30, 1987
An infant is descirbed who has multiple joint dislocations, unusual facies, and bony abnormalities, which constitute a syndrome first reported by Larsen. This report expands Larsen syndrome with additional findings such as apparent diminshed rigidity of cartilage in infancy, short nails, unusual juxtacalcaneal “accessory” bone, and mental retardation. The joint lesions and mental retardation require prolonged observation and treatment is limited mainly symptomatic therapy and...
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