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Original Article
A Case of Congenital Cystic Adenomatoid Malformation (Type1) of the Lung.
Dong Sik Kim, Hwang Min Kim, Jae Seung Yang, Baek Keun Kim, Jong Soo Kim, Dong Hwan Shin
Clin Exp Pediatr. 1990;33(6):830-834.   Published online June 30, 1990
A rare form of congenital cystic lung disease, characterized by the presence of one or usually multiple interconnecting cyst, is called congenital cystic adenomatoid malformation of the lung. This disease almost invariably presents either in live premature or stillborn infants, and death in those infants bom alive usually occurs within a few hours of birth. Infants with congenital cystic adenomatoid malformation usually have tachypnea,...
Clinical Studies on Congenital Anomalies.
Hyeon Joo Chai, Kyung Hee Kim
Clin Exp Pediatr. 1990;33(6):754-761.   Published online June 30, 1990
Modem medical science has been developed advanced rapidly in the past years, but congenital anomalies are known as a major cause of neonatal death. So, we studied the incidence of congenital malformations among 11,119 babies deliveried or admitted at EWUH during a period of five years starting from June 1983. The results were as follows: 1) The incidence of congenital malformations among 11,119 deliveries was 1.5%(1.5%...
A Case of Congenital Hypothyroidism.
Woo Hyon Kwon, Soon Young Song, Chang Ho Han, Hyae Ri Chung, Soo Yong Kim, Young Dae Kwon
Clin Exp Pediatr. 1990;33(5):708-713.   Published online May 31, 1990
Recently, we experienced a case of congenital hypothyroidism in 3month-old girl, who came with chief complaints of prolonged jaundice for 2 months after birth. After admission, she was diagnosed congenital hypothyroidism by the laboratory tests, including biochemistry, radioimmunoassay, radioisotope study, and bony radiography, and since then treated with sodium-L-thyroxine orally. The case was presented with brief review of literatures.
Congenital Pericardial Defect, A Report of Two Cases.
Young Sook Lee, Chang Youn Lee, Young Hwue Kim, Jung Yun Choi
Clin Exp Pediatr. 1990;33(5):680-683.   Published online May 31, 1990
This report describes two confirmed cases of partial pericardial defect which presented in complete- ly different way. The first case was referred with the chief complaint of chest pain. This case showed typical chest X-Ray and echocardiographic findings. In view of frequent chest pain, this case was operated on and the defect was closed with patch. In the second case, defect was incidentally found...
A Clinical Study on Hematological Findings in Children with Cyanotic Congenital Heart Disease.
Seong Ho Kim, Heung Jae Lee, Hahng Lee
Clin Exp Pediatr. 1990;33(5):642-650.   Published online May 31, 1990
Children with cyanotic congenital heart disease (CCHD) and secondary polycythemia exhibit increased susceptibility to hemorrhage or thrombosis. The hemostatic disorder manifests itself by the occurrence of excessive hemorrhage after surgery, and by increased incidence of cerebrovascular accident of a thrombotic nature during life. To assess whether the severity of polycythemia in children with CCHD is correlated with coagula- tion abnormalities, hematologic studies with preoperative and...
Prognosis according to Etiology and Age at Diagnosis in Congenital Hypothyroidism.
Myoung Sook Nam, Young Jong Oh, Byung Hee Kim, Young Jong Woo, Young Youn Choi, Tai Ju Hwang
Clin Exp Pediatr. 1990;33(4):506-513.   Published online April 30, 1990
A retrospective study on patients with congenital hypothyroidism was undertaken. The following results were obtained from 17 patients, on whom follow up investigations were available. 1) Males outnumbered females. The etiologic classification revealed: thyroid agenesis (64.7%), dyshormonogenesis (23.5%), and ectopic thyroid (11.8%). 2) In two-thirds of cases the diagnosis were made under 1 year of age, with 23.5% of cases being diagnoses below 3 month and 41.2%...
A clinicostatistical Study of Congenital Intestinal Obstruction.
Min Young Lee, Young Sook Hong, Se Jin Kang, Soon Kyum Kim, Pyung Hwa Choe
Clin Exp Pediatr. 1990;33(4):456-462.   Published online April 30, 1990
A clinicostatistical assessment of the 134 patients with congenital intestinal obstruction who were admitted to the department of pediatrics and pediatric surgery. Korea university medical center from Feb. 1979 to June. 1988 was performed. The results were as follows. 1) Congenital hypertrophic pyloric stenosis (43 cases) was the' most common congenital intestinal obstruc- tion, and congenital megacolon (24 cases), imperforate anus (23 cases), omphalocele (11 cases),...
A case of congenital dyserythropoietic Anemia.
Sang Oh Na, Seong Hoon Ha, Hong Hoe Koo, Hee Young Shin, Il Soo Ha, Hyo Seop Ahn, Doek Ja Oh, Myoung Hee Park
Clin Exp Pediatr. 1990;33(3):410-415.   Published online March 31, 1990
The congenital dyserythropoietic anemia refers to a group of hereditary disorders of erythropoiesis characterized by ineffective erythropoiesis, multinuclearity of erythroblasts, and secondary hemo- chromatosis. The authors report a case of anemia in a 4 month-old girl whose bone marrow showed dyserythropoietic features. A brief review of referential literatures was made.
Two Cases of Congenital Dislocation of the knee.
Hwa Jin Byun, Hye Kyung Lee, Hee Ju Kim, Sung Il Ahn, Chang Soo Ra, Woo Ku Chung
Clin Exp Pediatr. 1990;33(3):404-409.   Published online March 31, 1990
Congenital dislocation of the knee is rare deformity. Since its first description by chatelaine in 1822, many authors have been proposed. The authors experienced two cases of congenital dislocation of the both knees and a brief review of the related literatures was made.
A Case of Congenital Cysitic Adenomatoid Malformation of Lung.
Youe Kawn Kim, Deuk Hwan Jun, Bae Young Kim, Won Il Park, Kyung Ja Lee
Clin Exp Pediatr. 1990;33(2):225-228.   Published online February 28, 1990
Congenital cystic adenomatoid malformation is a rare variant of pulmonary cystic disease char- acterized by a mass of cysts lined by proliferating bronchial or cuboidal epithelium. The onset of symptoms, which are cyanosis, and tachypnea, usually occurs during the first week of life. We have experienced a case of congenital cystic adenomatoid malformation in a 9 month-old female. The diagnosis was mady by chest...
A Case of Congenital Esophageal Duplication Cyst.
Kyoung Hoon Choi, Chung Ah Whang, Byung Ju Jeaung, Kyu Earn Kim, Ki Young Lee
Clin Exp Pediatr. 1990;33(1):111-116.   Published online January 31, 1990
A case of congenital esophageal duplication cyst has been studied in a 10-month old male Korean infant. He had suffered from cough, fever and dyspnea since 4 months of age, and he had been admitted 4 times to other hospitals before he visited severance hospital due to aggravated cough and dyspnea. An esophagogram and computed tomogram of chest were done and a post-tracheal mass...
Congenital Systemic Cytomegalic Inclusion Disease.
Dong Beom Lee, Dong Hyun Kim, Jung Sik Min, Chang Hee Choi, Je Geun Chi
Clin Exp Pediatr. 1990;33(1):100-106.   Published online January 31, 1990
The authors experienced a case of congenital systemic cytomegalic inclusion disease in a newborn baby. This case showed the clinical features of low birt wight (2,200 gm), numerous petechiae, severe jaundice, hepatosplenomegaly, hypotonia and respiratory difficulty with cyanosis and Apgar score of 3 at 1 minute at birth. In the clinical course, he showed hypotonia, repeated spell of apnea. These symptoms progressively worsened and he...
Clinical Studies on Congenital Heart Diseases.
Hee Young Chun, Dae Churl Chung, In Kyung Sung, Kyong Su Lee, Du Bong Lee
Clin Exp Pediatr. 1990;33(1):66-74.   Published online January 31, 1990
A retrospective study of 10 years* experience with surgical treatment of congenital heart disease in the St. Mary’s Hospital from 1978 to 1987 was made. Data were analyzed on 188 children with congenital heart disease who had received corrective heart surgery under the age of 18 years. The results were as follows: 1) Out of 188 patients, ventricular septal defect(54.2% of all) was the most...
A Case of Congenital Anterior Urethral Valve.
Chang Ok Soh, Min Seop Song, Woo Yeong Chung, Soon Young Lee
Clin Exp Pediatr. 1989;32(12):1749-1751.   Published online December 31, 1989
We experienced a case of congenital anterior urethral valve in a 13-month old male child. He had recurrent fever and urinary dribbling. The diagnosis was based on radiologic and cystoscopic findings, and excision of the valve with suprapubic cystostomy was performed. A review of literatures was also presented briefly.
A Clinicostatistical Study of Congenital Digestive tract Anomalies.
Young Ok Park, Ju Hwi Kim, Young Wook Kim, Ki Bok Kim
Clin Exp Pediatr. 1989;32(12):1686-1705.   Published online December 31, 1989
A clinicostatistical review was performed on 151 patients of congenital digestive tract anomalies in infants and children diagnosed and treated at the Department of Pediatrics and Surgery of Kwangju Christian Hospital from January, 1983 to June, 1988. The results are summarized as follows: 1) The most frequently encountered congenital anomaly of the digestive tract was congenital hypertrophic pyloric stenosis with 41 cases(25.9%), followed by obstruction of...
A Case of Congenital Cranial Dermal Sinus.
Jae Min Choi, Woo Jung Kim, Hyeun Chung Kim, Ha Joo Choi, Woo Kap Chung
Clin Exp Pediatr. 1989;32(10):1442-1447.   Published online October 31, 1989
Congenital cranial dermal sinus is a depression or an epithelial lined tract that forms a abnormal communication between the scalp and the deeper tissues within the cranial cavity. This is a dysraphic condition resulting from incomplete seperation of cutaneous structures and neuroectoderm in early gestation. The clinical importance of the lesion lies in its associated neurological complications which usually present in early childhood. These complications include...
A Study on the Change of Plasma Renin Activity(PRA) and Aldosterone Concentration(PAC) before and after Heart Operation in Children with Congenital Heart Disease.
Jae Hong You, Jae Ho Lee
Clin Exp Pediatr. 1989;32(9):1232-1239.   Published online September 30, 1989
Thes is a study on changes of plasma renin activity (PRA) and aldosterone concentration (PAC) which was obtained before and after heart operation in 26 normotensive children with congenital heart diseases without renal disease admitted in Chungnam National University Hospital for cardiac catheterization with angiogram and heart operation from Apr. 1988 to Aug. 1988 to confirm any differences of PRA and PAC between groups...
Urinary Tract Anomalies in Congenital Heart Disease.
Chang Youn Lee, Young Seo Park, Hae Il Cheong, Jung Yun Choi, Young Soo Yun, Yong Choi, Kwang Wook Ko, In One Kim, Kyung Mo Yeun, Kwang Myung Kim, Hwang Choi
Clin Exp Pediatr. 1989;32(6):804-808.   Published online June 30, 1989
A variety of urinary tract anomalies were detected in 1,569 children with congenital heart disease during their cardiac evaluation with cardiac cineangiography in the Department of Pediatrics, Seoul National University Children’s Hospital within a period from October 1985 to April 1988. The incidence, nature and course of these urinary tract anomalies were analyzed and the results were as follows; 1) The number of male children...
Ultrasonographic Diagnosis of Congenital Hypertrophic Pyloric Stenosis.
Shin Heh Kang, Chul Lee, Ran Namgung, Dong Gwan Han, Ki Keun Oh, Seung Hun Choi
Clin Exp Pediatr. 1989;32(6):756-764.   Published online June 30, 1989
The diagnosis of congenital hypertrophic pyloric stenosis has traditionally been made on the basis of history and physical examination in the majority of patients. Imaging procedures such as UGI series have been reserved for vomiting infats in whom the diagnosis was obscure. More recently ultrasonography with the lack of hazards associated with radiation provides an additional method of investigation which is accurate and diagnose...
A Case of Congenital Hypoplastic Anemia.
Jae Wook Lee, Soon Ok Kang, Jee Sung Kim, Im Ju Kang, Seh Yoon Jeong
Clin Exp Pediatr. 1989;32(5):724-729.   Published online May 31, 1989
We observed a case of congenital hypoplastic anemia in a 6 months old female patient who was admitted because of severe anemia & failure to thrive and diagnosed through bone marrow aspiration. This patient had reticulocytopenia & increased M:E ratio (57.5:1). His hematologic improvement was followed by administration of prednisone. A brief review of related literature was made.
Two Siblings of Congenital Hypothyroidsm due to Organification Defect.
Young Kie Park, Kwang Sun Park, Byung Churl Lee
Clin Exp Pediatr. 1989;32(4):582-588.   Published online April 30, 1989
Congenital hypothyroidism due to a variety of defects in the biosynthesis of thyroid hormone is detected in 1/30,000-50,000 live births. In the iodide organification defect which is one of defect in the biosynthesis of thyroid hormone, iodide is not organified and may be rapidly discharged from the thyroid by administration of perchlorate. We experienced two siblings who had short stature, goiter, mental retardation, constipation and other...
A Case of Recurrent Bacterial Meningitis due to Congenital Cribriform Plate Defect.
Hong Chul Lee, Jong Leam Choi, Wan Soeb Kim, Myung Ho Lee
Clin Exp Pediatr. 1989;32(3):412-417.   Published online March 31, 1989
One patient with a history of recurrent bacterial meningitis was examined with skull and sinus radiographs, routine cranial computed tomography and immunologic evaluation. None of these studies were diagnostic. Thin-section (1-2 mm) direct coronal computed cranial tomography demonstrated congenital cribriform plate defect. This patient was repaired surgically and no further meningitis has developed for 16 months
A Case of Congenital Megakaryoblastic Leukemia Accompanied by Down Syndrome Which was Diagnosed by Autopsy Findings.
Il Kyung Kim, Dong Beom lee, Jung Sik Min, Chang Hee Choi, Je Geun Chi
Clin Exp Pediatr. 1989;32(2):262-269.   Published online February 28, 1989
Many cases of acute megakaryonlastic leukemia have been reported in childhood or adults, but congenital form or the cases occuring in neonatal period is very rare throughout the world. In Korea only one case was reported previously by Chung et al in 1987. The authors experienced a case of congenital megakaryoblastic leukemia accompanied by Down' s syndrome, which was confirmed by autopsy and positive...
Severe Iron Deficiency Anemia as a Result of Congenital Hiatal Hernia.
H Y Lee, H G Ham, M K Namgoong, B K Lim, J S Kim
Clin Exp Pediatr. 1989;32(2):250-254.   Published online February 28, 1989
Congenital diaphragmatic anomaly is a rare congenital malformation. A 6 year old boy with pale appearance and complaining of weakness for several months was diagnosed as having iron deficiency anemia as a result of congenital hiatal hernia. The diagnosis was confirmed by chest X.ray, esophagogram, upper gastrointestinal series and bone maπow biopsy After the hernia was successfully corrected by operation, the anemia was disappeared. Congenital...
11 q Deletion Syndrome.
Y B Im, S B Park, B Y Pyun, J O Park, S J Lee, S Y Moon
Clin Exp Pediatr. 1989;32(2):239-243.   Published online February 28, 1989
llq deletion syndrome is a rare chromosomal anomaly. The authors experienced a case of llq deletion syndrome with congenital glaucoma and infantile spasm in a female child. She showed delayed psychomotor development, flat occiput, hypertelorism, low set malformed ears, flat nasal bridge, short neck, simian line on right palm and throm. bocytopenia. Chromosomal study showed the deletion of the long arm of chromosme 11, karyotypically...
A Clinical Study of Congenital Hypertrophic Pyloric Stenosis.
Kwang Sun Park, Young Ki Park, Jong Wan Kim, Chang Kyu Oh, Mahn Kyoo Yang
Clin Exp Pediatr. 1989;32(1):27-33.   Published online January 31, 1989
We experienced 23 patients with congential hypertrophic pyloric stenosis from Dec. 1978 to Aug. 1987 The age and sex distribution, family and birth history, clinical symptoms with prognosis etc. were reviewed. The results were as follows. 1) Most of the patients (20 cases, 87%) represented symptoms within 6 weeks of age. 2) Male was predominated than female by the ratio of 19:4. 3) The first baby was...
2 Cases of Congenital Bronchial Anomalies Associated with Recurrent Pneumonia in Children.
Choong Sun Kang, Kyu Earn Kim, Ki Young Lee
Clin Exp Pediatr. 1988;31(12):1657-1662.   Published online December 31, 1988
We have experienced 2 cases of congenital bronchial anomalies in children. In one case, the right middle lobe bronchus originated from the right upper lobe bronchus, and all branches of the bronchial tree including the right main bronchus showed severe deformity. In another case, two upper lobe bronchi originated from the right main bronchus. These bronchial anomalies were associated with chronic and recurrent respiratory...
A Case of Acute Lymphocytic leukemia in a Newborn.
Young Jin Min, Myoung Hee Cha, Kyeng Sook Cho, Jong Dai Jo
Clin Exp Pediatr. 1988;31(9):1197-1201.   Published online September 30, 1988
The case of a 13 day-old baby is reported who satisfied the usual diagnostic criteria for congenital leukemia.
Plasma Renin Activity (PRA) in Peripheral Vein and Renal Veins in Normotensive Children with Cyanotic Congenital Heart Disease.
Sang Kyu Park, Hae Il Cheong, Chung Il Noh, Yong Soo Yun, Yong Choi, Kwang Wook Ko
Clin Exp Pediatr. 1988;31(8):1021-1027.   Published online August 31, 1988
PRA’s in peripheral vein, both renal veins, and inferior vena cava (IVC) were measured in 25 normotensive children without renal disease undergoing cardiac catheterization for their underlying cyanotic congenital heart disease. And we presented the results as reference values/ranges of PRA in children. 1) There was no significant difference in PRA between both side renal veins at any absolute PRA levels with a mean ratio (RT/LT)...
Evaluation of Pulmonary and Systemic Blood Flow by Doppler Echocardiography in Pediatric Congenital Heart Disease.
Jae Hong Park, Chun Dong Kim, Jin Gon Jun
Clin Exp Pediatr. 1988;31(8):1014-1020.   Published online August 31, 1988
This study was designed to assess the accuracy of pulmonary (Qp) and systemic blood flow (Qs) and the ratio of pulmonary to systemic blood flow (Qp/Qs) measured by non-invasive range-gated pulsed Doppler echocardiography. Thirty five children with congenital heart disease, aged 4 months to 15 years, admitted for cardiac operation to this hospital, were studied. Cardiac outputs were measured by 2-dimensional range-gated pulsed Doppler...