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Case Report
A Case of Congenital Dyserythropoietic anemia Type IV
Hwa Young Jeon, Hwang Min Kim, Baek Keun Lim, Duk Woo Park, Myung Seo Kang, Young Kun Deung
Clin Exp Pediatr. 1995;38(5):702-706.   Published online May 15, 1995
Congenital dyserythropoietic anemia(CDA) is characterized by ineffective erythropoiesis an d increased numbers of multinucleated red cell precursors in the marrow. This syndrome has b een subdassified on the basis of morphologic and serologic differences in the red cell pecursors. There are some patients with congenital dyserythropoietic anemia who do not fit into the conventional classification of type I, II, or...
Two Cases of Congenital TBG Deficiency
In Seong Jo, Ha Joo Choi, Young Ah Lee, Woo Gap Chung, Youn Bok Chang
Clin Exp Pediatr. 1995;38(5):697-701.   Published online May 15, 1995
We have experienced 4 and 5-week-old male neonates presented low T4 and normal TSH l-evel by filter paper mothod for neonatal screening, finally diagnosed typical TDG deficiency wit h TBG radioimmunoassay, paper electrophoresis, and autoradiography. Two neonates have ha d no specific complaints and lived well until now. Congenital TBG deficiency is an X chromosome linked inhcritant disorder characterized by l...
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 Congenital Lipoid Adrenal Hyperplasia
Hae Kyung Lee, Dong Hwan Lee, Sang Jhoo Lee
Clin Exp Pediatr. 1995;38(4):567-573.   Published online April 15, 1995
Congenital lipoid adrenal hyperplasia is the rarest type among salt losing types of congenital adrenal hyperplasia. The defect of this disorder is in the cholesterol side chain cleavage enzyme(P450SCC)which converts cholesterol to pregnenolone. We experienced a case of 20,22 desmolse deficiency in a 21-day old phenotypically female who was admitted to our hospital due to lethargy and dark skin pigmentation. The...
A Case of Diabetes Mellitus due to Hemochromatosis in Patient with CPRCA
Eun Hee Kim, Yong Hwa, Ho Sik, Sung Wom Kim
Clin Exp Pediatr. 1995;38(2):280-284.   Published online February 15, 1995
Iron overload from repeated blood transfusions in patients with hematologic problems is a problem of clinical significance, because eventually it can lead to secondary hemochromatosis with diabetes. So it is important to diagnose iron overload early and to restrict blood transfusions in these patients. Recently, we experienced one case of secondary hemochromatosis with diabetes in patient wi th congenital pure red...
A Case of Congenital Pericardial Defect
Byoung Chan Lee, Jong Wan Kim, Kyung Tai Whang
Clin Exp Pediatr. 1995;38(2):271-274.   Published online February 15, 1995
Congenital absence of the pericardium is thought to be uncommon. There are two types, complete and partial, of different clinical features. Complete type is innocuous but partial type is potentially fatal due to herniation. This case was referred for an unusual appearance of chest radiograph taken after admissio n for bronchopneumoia without any cardiac symptoms. Plain chest radiograph showed an unusual...
A Case of Congenital Agammaglobulinemia
Sang Yong Kim, Jong Hyun Kim, Jin Han Kang, Joon Sung Lee, Sang In Shim
Clin Exp Pediatr. 1995;38(1):99-103.   Published online January 15, 1995
Congenital agammaglobulinemia was described first among all human immunodeficiencies, and characterized by defect of B lymphocyte maturation and severe panhypogammaglobulinemia. These patients frequently acquire infections with high-grade extracellular pyogenic pyogenic organisms since infancy. We experienced a case of congenital agammaglobulinemia in a 10 years old male. He had history of recurrent pneumonia and purulent otitis media since late infancy. Also he...
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
Ultrasonographic Diagnosis by Pyloric Volume Measurement in Congenital Hypertrophic Pyloric Stenosis
Soon Kil Lee, Jae Wha Oh, Yeon Kyun Oh, Chang Guhn Kim
Clin Exp Pediatr. 1994;37(11):1595-1599.   Published online November 15, 1994
Real-time ultrasonogram was performed in 31 Pt. With CHPS, who was admitted at the pediatric department of Wonkwang University hospital from January 1991 to June 1993. Those who had positive results of pyloric volume for diagnosis of CHPS and were confirmed by surgery. The results were at follows, 1) The average ultrasonographic measurements of pyloric muscle thickness, pyloric diameter, pyloric length were...
Case Report
Congenital Adrenal Hyperplasia with 21-hydroxylase Deficiencies in Twins
Young Don Kim, Jeong Hwa Choi, Jae Hong Park, Hee Ju Park, Seong Suk Jeon
Clin Exp Pediatr. 1994;37(10):1469-1473.   Published online October 15, 1994
Congenital adrenal hperplasia is inherited disorder of adrenal steroidogenesis. 21-hydroxylase deficiency is the most commone enzymatic defect and is divided into classic and late-onset or nonclassic forms. Both classic and non-classic 21-hydroxylase deficiencies are inherited in a recessive manner as allelic variants. But it is rare that happened in twin infants. Chief complaints of affected twins in our case were ambiguous...
A Case of Congenital T Cell Lymphoblastic Lymphoma
Eun Sun Yoo, Young Mi Hong, Kyung Hee Kim, Hae Soo Gyu, Eun Chul Chung
Clin Exp Pediatr. 1994;37(9):1296-1304.   Published online September 15, 1994
T cell lymphoblastic lymphoma is characterized by immature lymphoid cells that are indistinguishable from the lymphoblasts and prolymphocytes of acute lymphoblastic leukemia. Several characteristic clinical features of lymphoblastic lymphoma, with include a high male-to-female ratio, a relatively high incidence in older children and young adults, the frequent presence of mediastinal involvement at the time of diagnosis. Also, this disease is...
Original Article
Chronic Cough in Children
Bin Cho, Joon Sung Lee, Kyung Tai Whang, Sung Hoon Cho
Clin Exp Pediatr. 1994;37(8):1116-1123.   Published online August 15, 1994
Chromic cough is a symptom frequently encountered by the pediatrician. Although most coughs are self-limited, chronic cough ofter proves to be a frustrating problem. This study was performed at Kangnam St. Mary's Hospital from January 1,1992 to December 31, 1992, and 83 children with chronic cough persisting for longer than 3 weeks was evaluated. We categorized these patients into 5...
Case Report
A Case of Two Giant congenital Aneurysms of the Right Coronary Artery
Yong Kwan Kim, Jo Won Chung, Jong Kyun Lee, Jun Hee Sul, Sung Kyu Lee
Clin Exp Pediatr. 1994;37(6):850-853.   Published online June 15, 1994
Aneurysms of the coronary arteries are rare. They may be due to atherosclerosis, mucocutaneous lymph node syndrome, mycotic emboli, syphilis or trauma and occasionally they are congenital. The prognosis appears to be poor and death can occur suddenly from rupture of the aneurysm, peripheral coronary embolism or bacterial endocarditis. Recently surgical treatment has been successful In a 5 year old patient with...
Original Article
Ultrasonographic Diagnosis of Congenital Hypertrophic Pyloric Stenosis
Chung Hyun Yun, Kwang Sin Kim, Young Wook Kim, Ki Bok Kim
Clin Exp Pediatr. 1994;37(4):494-501.   Published online April 15, 1994
Ultrasonic evaluation of the pylorus was performed in a group of 22 infants clinically suspected of having Congenital Hypertrophic Pyloric Stenosis (CHPS), from march 1990 to July 1991. In every case the diagnosis of CHPS was confirmed by surgery. Ten normal babies served as the control group. 1) In CHPS group, the mean age on admission was 36.7¡¾16.4 days, and male...
Clinical Studies on Congenital Heart Diseases
Kyeong-Ah Lee, Tae-Guen Song, Hyun-Kee Chung
Clin Exp Pediatr. 1994;37(4):472-480.   Published online April 15, 1994
The clinical studies were performed on 304 patients with heart disease who had been received corrective heart surgery at Kosin Medical Center from July, 1984 to December, 1991. The results were follows : 1) Out of 304 patients, 162 cases (53.3%) were male and 142 cases (46.7%) were female and sex ratio was 1.15 : 1.35 cases (11.5%) had clinical cyanosis and...
Case Report
Congenital Lobar Emphysema
Cheal Gee Kim, Do Hee Choi, Jin Bok Hwang, Chang Ho Han, Hye Li Chung, Young Dae Kwon
Clin Exp Pediatr. 1994;37(3):429-433.   Published online March 15, 1994
We had experienced a case of congenital lobar emphysema in a 3months old male infant. Chief symptoms included tachypnea, respiratory difficulty, cyanosis. Chest X-ray or chest CT scan revealed extensive emphysematous changes of the right upper and middle lobes, compression of the right lower lobe and shifted of mediastinum to the left side. This condition was appeared in the absence...
A Case of Congenital Hypoprothrombinemia
Jong Kwang Lee, Hae Won Cheon, Jung Hwa Lee, Kwang Chul Lee, Soon Kyum Kim
Clin Exp Pediatr. 1994;37(3):422-429.   Published online March 15, 1994
Congenital hypoprothrombinemia is a rare congenital coagulation defect. The clinical signs are manifestation of generalized bleeding tendency such as; mucosal bleeding, hypermenorrhea and post tooth extraction hemorrage. It is associated with prolongation of PT and PTT with normal thrombin time and decreased serum prothrombin level. A case with congenital hypoprothrombinemia was experienced by the authors. A 36 days old male baby...
A Case of congenital Diverticulum of Ventricle Associated with Pulmonary Atresia
Kun Taek Kim, Jo Won Jung, Jong Kyun Lee, Jun Hee Sul, Sung Kyu Lee
Clin Exp Pediatr. 1994;37(2):276-281.   Published online February 15, 1994
The congenital diverticulum of ventricle is a rare cardiac malformation arising from the left or right ventricle, the former being more common. Ventricular diverticulum is usually associated with other anomalies including intracardiac, midline thoracic, diaphragmatic and abdominal wall defect. The authors experienced a case of congenital diverticulum of left ventricle in nine month-old female infant. Left Blolock-Taussig shunt operation was done...
A Case of Bart's Syndrome - Epidermolysis Bullosa and Congenital Localized Absence of Skin -
Hong Shin Jeon, Young Jin Hong, Don Hee Ahn, Hee Jun Yoo, Je Geun Chi
Clin Exp Pediatr. 1994;37(1):104-108.   Published online January 15, 1994
A female newborn had the following characteristics: a congenital localized absence of skin over the lower extremities; blistering of the skin or mucous membrane, incited by trauma, which heals without scarring; and congenital absence or deformity of the nails. In respect to the characteristic manifestationhj, clinical course and electron microscopic features, our patient seems to fit well into Bart's syndrome....
A Case of Congenital Dyserythropoietic Anemia, Type II
Won Kyung Yang, Jung Wan Yoo, Hyung Goo Cho, Dong Chul Park, In Sung Lee, Won Yong Lee
Clin Exp Pediatr. 1994;37(1):99-103.   Published online January 15, 1994
Congenital dyserythropoietic anemia Type II (herditary erythroblatic multinuclearity with positive acidfied serum test;HEMPAS) is characterized by binuclearity, multinuclearity, pluripolar mitoses, karyorrhexis of normoblasts, and the presence of abnormla antigens on the red cells. We experienced a case of HEMPAS in a 2 month old girl patient who had an intermittent fever, abdominal distention with palpable liver & spleen, and generalized jaundice. The...
Original Article
Re-evaluation of TSH Screening TEST in Neonates
Jin Young Song, Dong Woo Son, Beyong Il Kim, Sei Won Yang, Jung-Hwan Choi, Chong Ku Yoon, Hyung Ro Moon
Clin Exp Pediatr. 1993;36(11):1502-1506.   Published online November 15, 1993
Five years ago, we made the cut-off value of TSH by dry filter paper method as 15μU/ml to screening congenital hypothyrodism. Since then, 1,210 term neonates, who had no perinatal problems, screening test with this cut-off point. Neonates had been recalled for measurement of serum T4/TSH to rule out congenital hypothyroidism if their TSH value by screening tests reveal more...
Growth Pattern and Final Height in Congeital Adrenal Hyperplasia
Ho Seong Kim, Duk Hi Kim
Clin Exp Pediatr. 1993;36(10):1359-1365.   Published online October 15, 1993
Clinical characteristics and growth pattern were evaluated in 42 patients with classical congenital adrenal hyperplasia treated since diagnosis. And final height was evaluated in 16 patients who had reached final adult height with regard to clinical form, degree of hormonal control, and age of initial treatment. The results were as follows; 1) Among 42 patients with classical congenital adrenal hyperplasia, the male...
Comparative Value of Cardiac MRI and Echocardiography in the Assessment of Congenital Heart Lesions
Young-Hwue Kim, #NAME? Park, Chang-Yee Hong, Shi-Joon Yoo, Tae-Hwan Lim
Clin Exp Pediatr. 1993;36(10):1343-1350.   Published online October 15, 1993
To examine the usefulness of cardiac MRI in assessing patients (pt) with congenital heart diseases (CHD), informations obtained from MRI and echocardiogrphy (echo) were compared in 91 consecutive pt with CHD and was correlated with findings at cardiac catheterization(53pt) and at surgery (71pt). Pt were studied with 1.5Tesla MRI unit and multiplanar images of the heart and great vessels were...
A Clinical Study of Congenital Anomalies in Births associated with Hydramnios
Eun Sil Shin, Min Jeong Kim, Ock Seung Jeong, Son Sang Seo
Clin Exp Pediatr. 1993;36(9):1227-1235.   Published online September 15, 1993
A study was made in the congenital anomalies in 137 babies born to 120 mothers with hydramnios, delivered at Ilsin Christian Hospital between Jan. 1st 1981 and Dec. 31th 1990. The results were as follows; 1) The incidence of hydramnios was 16/1000 deliveries, 120 cases in total 73,129 deliveries. 137 infants was born and of...
Case Report
A Case of Congenital Hypertrophic Pyloric Stenosis in Two Siblings
Yung Min Jang, Mi Ran Park, Sung Yoon Byun, Jae Young Kim, In Sang Jeon, Kwang Jun Kim
Clin Exp Pediatr. 1993;36(7):1025-1029.   Published online July 15, 1993
A Case of Congenital Goiter with Congenital Hypothyroidism due to Organification Defect
Ik Hee Lee, Sung Yong Jung, Thi Hyung Park, Sa Jun Chung, Chang Il Ahn
Clin Exp Pediatr. 1993;36(7):1002-1008.   Published online July 15, 1993
We experienced a case of congenital goiter with congenital hypothyroidism in 45 dayold male, who complained of respiratory difficulty and anterior neck mass. After admission, he was diagnosed congenital hypothyroidism by the clinical manifestations and laboratory tests including biochemistry, radioimmunoassay, radioisotope study, perchlorate discharge test, and bone radiography. We obtained positive finding at the perchlorate discharge test and found that his...
Original Article
A Clinical Review of Congenital Gastrointestinal Anomalies
Dong Hak Shin, Woo Hyun Park, Chul Young Bae
Clin Exp Pediatr. 1993;36(7):944-950.   Published online July 15, 1993
A clinical review was done on 343 infants and children diagnosed and operated at the Department of Pediatric Surgery in Keimyung University, Dong San Medical Center for congenital gastrointestinal anomalies from January, 1988 to December, 1991. The results are summarized as follows; 1) The most prevalent age group of congenital gastrointestinal anomaly was within first week after birth, and infants within 3months...
Case Report
A Case of Congenital Tricuspid Stenosis
Chan Uhng Joo, Soo Young Cho, Soo Chul Cho
Clin Exp Pediatr. 1993;36(5):733-736.   Published online May 15, 1993
Congenital tricuspid stenosis is ancommon congenital heart anomaly. Tricuspid stenosis is usually associated with other anomalies, most commonly severe pulmonary stenosis or atresia with secondary hypoplasia of the right ventricle. Clinically it may be difficult to distinguish from tricuspid atresia. A case of congenital tricuspid stenosis in a 32 day-old-girl is presented. The patient had cyanosis and dyspnea. Echocardiographic study revealed...
Original Article
Growth Outcome in Congenital Hypothyroidism
Mi Jung Park, Ho Seong Kim, Duk Hi Kim
Clin Exp Pediatr. 1993;36(5):713-720.   Published online May 15, 1993
Congenital hypothyroidism is one of the most common endocrine disease in childhood and it causes not only mental retardation but also growth retardation. There were many papers about evaluation of developmental outcome in congenital hypothyroidism, The aim of this study was to evaluate growth outcome in congenital hypothyroidism. We evaluated 65 patients with congenital hypothyroidism diagnosed at yonsei University College...
Detection of Cytomegalovirus DNA in Urine Culture Using Polymerase Chain Reaction
Young Mo Sohn, Won Young Lee
Clin Exp Pediatr. 1993;36(5):626-633.   Published online May 15, 1993
Polymerase chain reaction(PCR) amplication was used to detect cytomegalovirus (CMV) in tissue culture from the ruine of newborns and patients who was suspected CMV infection. Synthetic oligonucleotide primer pairs were used to amplify DNA from the magor immediate-early and the phosphoprotein 150 genes of CMV AD 169. Amplified products were detected by gel electrophoresis and by dot-blot hybridization with oligonucleotide...
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